Paediatrics and Child Health May2012 by manuel Montellanos

EgyptianPediatrics Yahoo Group http://health.groups.yahoo.com/group/ EgyptianPediatrics/

Paediatrics

and Child Health

Paediatrics and Child Health is the continuously updated review of paediatrics and child health (formerly Current Paediatrics) Paediatrics and Child Health is an authoritative and comprehensive resource that provides all paediatricians and child health care specialists with up-to-date reviews on all aspects of hospital/community paediatrics and neonatology, including investigations and technical procedures in a 4-year cycle of 48 issues. The emphasis of the journal is on the clear, concise presentation of information of direct clinical relevance to both hospital and community-based paediatricians. Contributors are chosen for their recognized knowledge of the subject. The journal is abstracted and indexed in Current Awareness in Biological Sciences. The layout of the journal, including the design and colour, enables fast assimilation of key information. For ease of reference, Paediatrics and Child Health is available in print and online formats.

Editor-in-Chief Patrick Cartlidge

DM FRCP FRCPCH

Senior Lecturer in Child Health and Honorary Consultant Neonatologist, Wales College of Medicine, Cardiff University, Cardiff, UK

Founding Editor Richard Wilson

MB FRCP FRCPCH DCH

Associate Editors Allan Colver MA MD FRCPCH Professor of Community Child Health, Sir James Spence Institute, Newcastle University, Newcastle, UK Harish Vyas DM FRCP FRCPCH Professor in PICU and Respiratory Medicine, Queenâ&#x20AC;&#x2122;s Medical Centre, Nottingham University Hospital, UK Doug Simkiss BMedSci MBChB DCH DTMH MSc FRCP (Ed) FRCPCH FHEA Associate Professor in Child Health, Warwick Medical School, Warwick, UK Honorary Consultant Paediatrician, Birmingham Community HealthCare NHS Trust and Sandwell and West Birmingham NHS Trust, Birmingham, UK

Nicholas Mann MD FRCP FRCPCH DCH Consultant Paediatrician, Department of Paediatrics, Royal Berkshire Hospital, Reading, UK Alistair Thomson MA MD BChir FRCPCH FRCP DCH DRCOG Consultant Paediatrician, Leighton Hospital, Crewe, UK Colin Powell MBChB DCH MRCP FRACP FRCPCH MD Consultant Paediatrician, University Hospital of Wales, Cardiff, UK Peter Heinz MD State Exam Med FRCPCH Consultant Paediatrician, Addenbrookeâ&#x20AC;&#x2122;s Hospital, Cambridge, UK

International Advisory Board R Adelman (Phoenix, USA) Z Bhutta (Karachi, Pakistan) MC Chiu (Kowloon, Hong Kong) P Malleson (Vancouver, Canada)

A Bagga (New Delhi, India) H Buller (Rotterdam, The Netherlands) M Hassan (Islamabad, Pakistan) A Martini (Genova, Italy)

A Moosa (Saffat, Kuwait)

C Morley (Carlton, Australia)

BJC Perera (Colombo, Sri Lanka)

J Pettifor (Johannesburg, South Africa)

M Uchiyama (Niigata, Japan)

M van de Bor (Nijmegen, The Netherlands)

Paediatrics and Child Health has an eminent editorial board and a wide array of authors, all of whom are recognized experts in their field. Visit our website at: www.paediatricsandchildhealthjournal.co.uk for previous issues, subscription information and further details.

SYMPOSIUM: SOCIAL PAEDIATRICS

The role of the medical expert in care proceedings

The starting point has to be Part 25 of the Family Procedure Rules 2010, Practice Direction 25A and the Practice Direction for Experts in Family Proceedings relating to Children. The objective is for the parties to provide the court with early information to enable it to determine whether it is necessary or practical to ask an expert to assist the court in identifying and narrowing the issues between the parties, and to provide an opinion about questions that are not within the skill and experience of the court, and if it is for the parties to identify questions that need to be answered by the experts. The Practice Direction identifies the duties of the expert: the overriding duty to the court that we are all now so familiar with. To confine the opinion to matters that are material to the issues between the parties and that are within the expert’s expertise. In expressing an opinion to take into consideration all the material factors and to explain what material they have relied upon in reaching their conclusion. Where a question has been put which falls outside the expert’s expertise, to state this at the earliest opportunity and to volunteer an opinion as to whether another expert is required to bring expertise not possessed by those already involved or, in the rare case, as to whether a second opinion is required on a key issue and, if possible, what questions should be asked of the second expert; to inform those instructing the expert without delay of any change in the opinion and of the reason for the change. The expert must remember that he/she is there to assist the court in identifying, narrowing and resolving the issues between the parties and they must confine their opinion to that alone. Before that can be done effectively you have to first identify the issues the court is going to be asked to decide. In public law cases there are often two distinct stages: the fact-finding stage upon which threshold is based and, once that has been determined disposal where the welfare of the child is paramount. At the fact-finding stage the expert is, or should be, clearly instructed that where the parties do not agree on the facts they should not attempt to resolve the areas in dispute, but they should advise in the alternative. Having said that the paediatric overview almost inevitably involves a consideration of the plausibility of any explanation proffered by the parent as to the possible cause of the injury in question. Where expert reports are sought for the second stage the “matters that are material to the issues” when it comes to the facts are those that have been found to be so by the court or by agreement in the first stage. It does not include any further findings or inferences that the expert may feel is justified when preparing the report. The Judge has to approach the issues at the welfare stage on a certain factual matrix and if the experts are not doing the same then they are not confining their opinion to matters material to the issues between the parties and they are therefore not assisting the court as they should. This has been settled law since Re M and R (Child Abuse: Evidence) [1996] 2 FLR 195 where it was held that a decision by the local authority not to pursue allegations of sexual abuse meant that if the threshold criteria was satisfied on a different basis (i.e. neglect and emotional harm) then, at the welfare or disposal stage, the court could not assess risk on the basis either (a) that there was sexual abuse or (b) that there was a suspicion that there might have been sexual abuse, as alleged by the younger boys. In Re R (Care Disclosure Nature of Proceedings) 2002 1 FLR 755

James Tillyard QC

Abstract The purpose of the Article is to explain the respective roles of the trial judge and the medical expert in care proceedings, the circumstances under which the court requires expert evidence and the way in which it fits into the “wider canvas”. It also considers how the court system is striving to improve the way in which the evidence is received in order to avoid unnecessary demands on the valuable resource of the medical expert.

Article 8(1) of the European Convention for the Protection of Human Rights and Fundamental Freedoms 1950 declares a right of privacy of family life. Parents should be permitted to bring up their children as they wish without intervention by the state. It is not an unqualified right and Article 8(2) specifies circumstances in which it may be infringed, and that includes intervention by the Court pursuant to section 31(2) of the Children Act 1989, but as Hedley J said in Re L (CARE: THRESHOLD CRITERIA) 2007 1 FLR 2050: Art 8(2) and s 31(2) contemplate the exceptional rather than the commonplace. It is a draconian power that must be exercised with great caution. The legislation is to found at section 31(2) of the Act: A court may only make a Care Order or Supervision Order if it is satisfied; (a) that the child concerned is suffering or is likely to suffer significant harm; and (b) that the harm or likelihood of harm is attributable to the care given to the child or likely to be given to him if the order were not made, not being what it would reasonable to expect a parent to give him; or (c) the child’s being beyond parental control. In sub-section 9 certain definitions of relevant terms are set out including: “harm” means ill-treatment or the impairment of health or development, including, for example, impairment suffered from seeing or hearing the ill-treatment of another. “development” means physical, intellectual, emotional, social or behavioural development. There is no doubt that the medical expert plays a vital role in the determination of care proceedings, but it must always be remembered that it is the Judge who decides cases not the expert. It is he (or she) who has to reach a conclusion on the totality of the evidence, and it is always open to him to come to a different conclusion to that of the expert. That being so why are you asked by Counsel and Judges alike “on the balance of probabilities do you think this is a non-accidental injury?” In the words of Shakespeare: “that is not the question.”

James Tillyard QC is a practicing barrister who specializes in the area of public law proceedings and who is based in Cardiff, UK. Conflict of interest: none.

PAEDIATRICS AND CHILD HEALTH 22:5

177

SYMPOSIUM: SOCIAL PAEDIATRICS

Charles J said that a similar but not identical situation would have arisen if the allegations of sexual abuse had been pursued and the court had been unable to make findings that there had been sexual abuse, or who the perpetrators were, to the requisite standard of proof. The courts are very anxious that doctors should be given the widest discretion, and encouragement to identify the possible abuse of children. The House of Lords made that abundantly clear in the House of Lords decision of D v East Berkshire Community NHS Trust 2005 2 FLR 284. The case was to do with claims relating to professional negligence, and involved the question of whether doctors owed a duty of care to parents when identifying sexual abuse of children, and they found that healthcare and other child care professionals did not owe a common law duty of care to parents not to make negligent allegations of child abuse. They did however make it clear that there was such a duty owed to the child. The duty, of course, is to exercise the standard of skill and care expected of any reasonable professional in the circumstances e it is not a duty to get it right. In the course of their Judgements their Lordships commented on the vital part doctors have to play in combating child abuse. Per Lord Nicholson paragraph 85:

becomes apparent that a second expert in a particular field is necessary. This has been settled law in Civil litigation since Daniels v Walker (Practice Note) [2000] 1 WLR 1382, CA, but was only considered in family proceedings in 2005 in the case of W v OLDHAM METROPOLITAN BOROUGH COUNCIL [2006] 1 FLR 543. If involved an alleged shaken baby and the interpretation of the MRI scans by a jointly instructed neuro-radiologist. On an application for leave to appeal Thorpe LJ released the papers to a second expert who fundamentally disagreed with the first and supported the parents’ case that the injuries had an innocent explanation. The appeal was allowed and remitted for re-hearing before Ryder J. In passing, at the re-hearing the learned Judge felt it appropriate to instruct a third expert. The court of appeal gave clear guidance as to when a second expert in any particular field should be permitted (from the headnote): In cases in which certain medical evidence became pivotal, for example, in cases involving non-accidental head injury or pathologically ‘unascertained’ infant death, and in which such evidence was, by its nature, difficult to challenge in the absence of further expert opinion, the court should be slow to decline an application for a second expert. It was clear in this case that there was only one medical expert capable of assessing the MRI scan and that the apparent medical consensus on which the judge relied depended upon the analysis of that expert being correct. In such circumstances, the parents were entitled to a second opinion (see paras [10], [31]e[32]). Usually the party applying for a second expert should first pose questions to the jointly instructed expert directed at the area in dispute. In Merton London Borough Council v K 2005 2 FLR 422 Baron J comments that medical experts are a rare resource and should not be required to waste time at court unless their attendance was absolutely necessary. It was the on-going duty of counsel to consider the necessity of the attendance of witnesses. If matters could be dealt with in writing then questions should be posed in that manner even after the commencement of trial. Since the advent of the Public Law Outline (PLO) Judges have been far more vigilant about this. The new Family Procedure Rules require the advocates to identify the issues the experts are to address. If the experts are in agreement a party has to say whether or not they accept the agreed position. This gives the court a much clearer picture at the Issue Resolution Hearing (IRH) as to whether or not a witness is required to give evidence at the final hearing and if so why. After the meeting of experts it is often apparent that there is broad agreement between them and there is nothing to be gained by calling every one and so the court may restrict it to one expert or one from each discipline, in order to give the dissenting party an opportunity to put their case. Video link is now the norm rather than the exception. That being so I have to say that I find it extraordinary that they remain so unreliable. In A County Council v K D and L 2005 1 FLR 1 Charles J suggested that whilst an expert is entitled to give his or her opinion on the “ultimate issue” it might be better not to ask experts in a case concerning children to express a view by reference to the balance of probabilities in order to make clear the respective roles of the expert and the judge who is the decision-maker (paragraph 85). He went on to say:

But when considering whether something does not feel ‘quite right’, a doctor must be able to act single-mindedly in the interests of the child. He ought not to have at the back of his mind an awareness that if his doubts about intentional injury or sexual abuse prove unfounded he may be exposed to claims by a distressed parent. Lord Brown paragraph 132: Those comparatively brief passages from the evidence highlight what in any event seems plain. Doctors have a vital part to play in combating the risk of child abuse. Nothing must be done to discourage them in that task. ‘The easy option’ (Professor Craft), ‘the line of least resistance’ (Mary Marsh), will always be for the doctor to accept the explanations given and to suppress his doubts. What is needed, however, is that doctors should act with ‘courage’ (Mary Marsh), when they feel that something ‘is not quite right’ (Professor Craft), although there is ‘nothing specific to support that feeling’ (Mary Marsh), and when ‘the evidence of child abuse is at best tentative’. (Professor Craft). In these cases ‘the interests of the child and the parent do not coincide’ (Professor Craft); ‘for children at risk of abuse, especially those at the most ominous risk, there may not be congruity of interest but rather conflict of interest’ (Mary Marsh). That is however, a duty that doctors are expected carry out in their capacity as treating doctors and not as expert witnesses. Single joint expert e when considering whether to give permission for the parties to rely on expert evidence and whether that evidence should be from a single joint expert the court will take into account all the circumstances set out in the Practice Direction but in practice the starting point is that the instruction of a single joint expert is likely to assist the parties and the court to resolve the issue more speedily and in a more cost-effective way than separately instructed experts. This is certainly the case at first instance but there are occasions when it then

PAEDIATRICS AND CHILD HEALTH 22:5

178

SYMPOSIUM: SOCIAL PAEDIATRICS

[89] Nonetheless it seems to me that in civil cases concerning children it might (a) assist all involved, (b) better reflect the roles of the expert and the judge, and (c) demonstrate that the expert is not the decision-maker as to whether the relevant death, injuries or harm is the result of non-accidental human agency and whether the threshold is satisfied, and does not have all the relevant information, if the medical experts were not asked to express a view as to the cause of the relevant death, injuries or harm on the balance of probabilities but were asked to:

that the injury had a natural cause or was accidental. Equally in a case where the medical evidence was that there was nothing diagnostic of a non-accidental injury, the court could nonetheless reach a finding on the totality of the evidence that there had been a non-accidental injury and that the threshold was established. It was open to the judge to reach a conclusion on the totality of the evidence that was different to or did not accord with the conclusion reached by the medical experts. This was followed by the decision of Ryder J in Re X Y Z [2005] 2 FLR 129 where he said:

(i) identify possible causes of the relevant death, injuries or harm setting out in respect of each the reasons why it might be a cause and thus why it should be considered; (ii) state their views as to the likelihood of each possibility being the cause of the relevant death, injuries or harm and the reasons why they include or reject it as a reasonable (as opposed to a fanciful or merely theoretical) possible cause; (iii) compare the likelihood of the cause (or causes) identified as reasonable possibilities being the actual cause of the relevant death, injuries or harm; (iv) state whether they consider that a cause (or causes) is (are) the most likely cause (or causes) of the relevant death, injuries or harm and their reasons for that view; and (v) to state whether they consider that a cause (or causes) is (are) more likely than not to be the cause (or causes) of the relevant death, injuries or harm and their reasons for that view.

The court had to make a factual decision based on all available materials to be judged in context, and to have regard to the wide range of social, emotional, ethical and moral factors that would inform the decision on whether the threshold was satisfied for the making of a care order. The court had to be cautious of declining to follow un-contradicted expert evidence but it was not bound by it. The assessment of adult credibility as to the responsibility for harming a child remained the function of the court. The learned Judge emphasized at para [44]: “a factual decision must be based on all available materials, ie be judged in context and not just upon medical or scientific materials, no matter how cogent they may in isolation seem to be. Just as best interests are not defined only by medical or scientific best interests likewise, investigations of fact should have regard to the wide context of social, emotional, ethical and moral factors.” When considering the ‘wide canvas’ of evidence the following section of the speech of Lord Nicholls in Re H and R (Child Sexual Abuse: Standard of Proof) [1996] 1 F.L.R. 80 remains relevant (at 101B):

Disputed medical evidence e specific guidance has been given by the Court of Appeal in respect of cases involving controversial areas of medicine in the aftermath of the criminal cases of Cannings and Clarke. The position is summarized by Butler Sloss P in Re U; Re B (Serious Injury: Standard of Proof) 2 FLR 263 at paragraph 23: In the brief summary of the submissions set out above there is a broad measure of agreement as to some of the considerations emphasized by the judgement in R v Cannings that are of direct application in care proceedings. We adopt the following: (a) The cause of an injury or an episode that cannot be explained scientifically remains equivocal. (b) Recurrence is not in itself probative. (c) Particular caution is necessary in any case where the medical experts disagree, one opinion declining to exclude a reasonable possibility of natural cause. (d) The court must always be on guard against the overdogmatic expert, the expert whose reputation or amour propre is at stake, or the expert who has developed a scientific prejudice. (e) The judge in care proceedings must never forget that today’s medical certainty may be discarded by the next generation of experts or that scientific research will throw light into corners that are at present dark. In A County Council v. K D & L [2005] 1 FLR 851 Charles J said:

“I must now put this into perspective by noting, and emphasising, the width of the range of facts which may be relevant when the court is considering the threshold conditions. The range of facts which may properly be taken into account is infinite. Facts including the history of members of the family, the state of relationships within a family, proposed changes within the membership family, parental attitudes, and omissions which might not reasonably have been expected, just as much as actual physical assaults. They include threats, and abnormal behaviour by a child, and unsatisfactory parental responses to complaints or allegations. And facts, which are minor or even trivial if considered in isolation, taken together may suffice to satisfy the court of the likelihood of future harm. The court will attach to all the relevant facts the appropriate weight when coming to an overall conclusion on the crucial issue.” This attracted an echo in Re U; Re B in which the Butler Sloss P referred to the: “wide canvas, including a detailed history of the parents’ lives, their relationship and their interaction with professionals. There will be many contributions to this context, family members, neighbours, health records, as well as the observation of professionals such as social workers, health visitors and children’s guardian.”

It is the role of the court to take into account and weigh the expertise and speciality of expert witnesses. In a case where the medical evidence was to the effect that the likely cause of an injury was non-accidental the court was entitled to find

PAEDIATRICS AND CHILD HEALTH 22:5

179

SYMPOSIUM: SOCIAL PAEDIATRICS

Charles J, who has been at the forefront of the development of the law in relation to expert witnesses in family proceedings, returned to the subject in Lancashire County Council v D AND E [2010] 2 FLR 196 e (Headnote): 1. The factual examination, and the forensic process relating to it, could not properly rest only on a chain of reasoning based on: (a) the view of medical experts that the most likely cause of the injuries was shaking or shaking and impact; and (b) the identification of a pool of possible perpetrators by reference to the carers of the child. The correct position was that in determining whether the child was the victim of an inflicted injury, a medical view as to the most likely cause of injuries clearly established the cause in question as a real possibility which had to be considered, in all the circumstances of the case, together with other possibilities. The exercise of identifying a perpetrator or the pool of perpetrators then formed part of the exercise of considering whether there had been an inflicted injury; it was not the case that someone with the opportunity to injure a child had to show that he or she had not done so (see paras [36], [41]). 2. When no evidence emerged concerning an accident that might have caused the child to suffer the injury, it was not open to the court to make a finding as to what the accident was or might have been (see para [42]). 3. If parents impressed the court and others as credible witnesses, this was a factor in the overall factors to be taken into account, which effectively reversed the degrees of likelihood placed upon the likely causative event identified by medical experts (see para [155]). 4. In relation to possible causes of injury in this case, the experts had not ruled out either accidental handling of a kind that would not alert the handler that there was a problem, or something unknown, and both could properly be regarded as real possibilities, from a medical perspective, as opposed to fanciful possibilities. Given that the court accepted the parents’ evidence that neither of them had injured the child by shaking the child, and that neither were concealing a specific cause of the injuries, a conclusion could be reached that, notwithstanding the well-reasoned medical view that the most likely cause of the injuries was shaking or shaking and impact, on the totality of the evidence, the cause was more likely than not something that had not been appreciated by either parent (see paras [2], [145], [146], [150], [169], [207], [208], [210]). It is apparent from the authorities that there is a considerable difference between an explanation as to how the injuries were caused being inconsistent with the medical evidence and one that may be consistent but remains very unlikely. Provided the experts agree, in the first case the medical evidence will almost certainly determine the outcome. In the second the court will consider the wider canvas, and in particular whether or not the Judge finds the alleged perpetrators to be credible witnesses. That is not to ignore the expert evidence as to the most and least likely cause but it also takes into account the fact that unlikely events do occur. It goes a little further than that, because not only do they occur, when they occur professionals are suspicious because of the unlikelihood of the event and so they often end up in the criminal and family courts.

PAEDIATRICS AND CHILD HEALTH 22:5

The term ‘Munchausen’s Syndrome by Proxy’ has for some time now been consigned to the history books by both the legal and (as I understand it) the medical profession. Ryder J considered the term “Factitious (and Induced) Illness (by Proxy)” in A County Council v X Y and Z and considered that it should only be used as a factual description of a series of events or behaviour that should then be accurately set out and that it must not be used as a substitute for factual analysis and risk assessment. He said: “[175] The terms ‘Munchausen’s Syndrome by Proxy’ and ‘Factitious (and Induced) Illness (by Proxy)’ are child protection labels that are merely descriptions of a range of behaviours, not a paediatric, psychiatric or psychological disease that is identifiable. The terms do not relate to an organised or universally recognised body of knowledge or experience that has identified a medical disease (ie an illness or condition) and there are no internationally accepted medical criteria for the use of either label. [176] In reality, the use of the label is intended to connote that in the individual case there are materials susceptible of analysis by paediatricians and of findings of fact by a court concerning fabrication, exaggeration, minimisation or omission in the reporting of symptoms and evidence of harm by act, omission or suggestion (induction). Where such facts exist the context and assessments can provide an insight into the degree of risk that a child may face and the court is likely to be assisted as to that aspect by psychiatric and/or psychological expert evidence.” The fact is that evidence as to the existence of FII in any individual case is at best evidence of mere propensity which would be inadmissible at the fact-finding stage in any event (see Re CB and JB (Care Proceedings: Guidelines) [1998] 2 FLR 211). It follows that however useful FII may apparently be to the child protection practitioner, one should caution against its use other than as a factual description of a series of incidents or behaviours that should then be accurately set out. There has been a very recent and interesting development in the way experts may be required to give evidence in family proceedings in the future, particularly in relation to welfare. In A LOCAL AUTHORITY v A (NO 2) [2011] 2 FLR 162 Ryder J heard oral evidence from the three experts commissioned to analyze the key welfare issues by means of “hottubbing”, whereby the court drew up an agenda of topics upon which it sought assistance. All the witnesses were sworn together and were asked the same questions taking a topic at a time. They were asked to add or explain their, or other, evidence so that “a healthy discussion ensue” and they were cross-examined by counsel. The learned Judge felt that by this means the court acquired coherent evidence directed at the key issues and avoided adversarial point scoring. It also meant that evidence that would otherwise have taken 2 days of court time took 4 h. This is a small but important step forward from the meeting of experts and the schedule of what is and what is not agreed. Its merits are obvious but, may I suggest, that it requires a strong and robust chair without which the proceedings could easily descend into chaos. Ryder J is obviously up to the task. We will have to wait and see whether others follow suit. A

180

SYMPOSIUM: SOCIAL PAEDIATRICS

Advocacy and the paediatrician

Definitions of advocacy C C

Tony Waterston

C C

Abstract Advocacy means speaking out for the disempowered. It is as important a skill for paediatricians as communication but is less well taught. Advocacy applies to the paediatrician’s everyday work with child patients and also to the field of prevention in relation to a population of children. Paediatricians are powerful advocates because of their position and their perceived lack of bias, and they have ready access to those in positions of power. Advocacy has a long history in paediatrics and examples are given from the UK and USA. Its underpinning principles are drawn from the UN Convention on the rights of the child, which requires states to offer provision, protection and participation to children and young people. Its articles provide guidance on the scope of advocacy. Current issues requiring advocacy in the UK include special needs children in the education system, adolescents in hospital and outpatients, corporal punishment in the home, and child mental health and violence against children. Advocacy must be integrated into paediatric training as it is likely to be required even more in the future as services become more complex.

Box 1

good communication and accurate diagnosis, this requires thought, analysis and above all practice. Advocacy begins with the child in front of you and I will offer examples in the next section. However most paediatricians recognize that the same problem is prone to recurring, and that more often than not the system is at fault. This requires what is known as ‘system’ or public health advocacy, which is beyond the scope of a single paediatrician and requires working together with other sectors and organizations. It is most helpful if the paediatrician’s professional association can take this up and examples are given later of how effective this can be.

Why is advocacy part of the work of the paediatrician? Some examples may help to explain this. Nathan is a 7-year-old boy who has been attending the enuresis clinic for a couple of years. He has been treated for a urinary infection on one occasion and investigated for reflux, with normal findings. He has been treated with a star chart and an alarm is on order. One day, his mother mentioned that he has occasional wetting during the day which upsets him quite a lot. The paediatrician asks him about using the toilet at school and he says he is not allowed out during class, and doesn’t like going in the break as the toilets are smelly, there is not enough paper, there are no locks on the door and he is called names when he goes there as he is known to wet the bed. He goes home at lunch and uses the toilet then, but sometimes can’t wait. The paediatrician is shocked. What can she do to improve the situation in the school toilets? Advocacy role: write to head teacher; propose to parents that they take this up with the school governors; suggest that survey of school toilets be a task for a trainee on the local advocacy module for the Msc in child health. Gail is an 8-year-old girl with congenital hypothyroidism. She presents to the clinic with a letter from her GP saying that she has not been at the clinic for 3 years as she has needle phobia and is reluctant to attend, but has now agreed. She had a series of bad experiences when attending the clinic at your hospital where there is no paediatric venepuncture service and she attended the adult service. She had to be restrained during the blood test and became extremely upset. The GP also attempted to take blood but failed with further misery to the child. The paediatrician takes blood with little difficulty using Emla cream and finds the T4 level is below the normal range and her treatment needs to be increased. However, there is still an adult

Keywords advocacy; children’s rights; discrimination; participation

What is advocacy? There are a number of definitions of advocacy, some of which are given in Box 1, but the fundamental meaning is speaking out for the disempowered. This is especially necessary in paediatrics and child health since children are usually unable to speak out for themselves, and sometimes their parents find it difficult too. Advocacy means going beyond the everyday task to support a child’s needs, or to protect the health of children in the population. For many perhaps most paediatricians, this is what they do every day, so is advocacy really something special? I believe it is, particularly in the modern world, when children’s lives are constrained in so many ways and when we as paediatricians work with so many other sectors which have different ways of working. If it is hard for us to understand the bureaucracy of social services and education, imagine what is must be like for parents. So advocacy means unblocking officialdom, removing injustice, combating discrimination, increasing participation, and above all ensuring that children and young people receive respect from all the professionals they meet. It is because this is not easy, and requires additional skills and knowledge, that this article is necessary. We can all be effective advocates for children, but like

Tony Waterston MD FRCPCH is a Retired Consultant Paediatrician and Senior Lecturer in Child Health from Newcastle upon Tyne, UK. Conflicts of interests: none.

PAEDIATRICS AND CHILD HEALTH 22:5

“Speaking out in a cause” “Promoting the rights of oneself or others” “Giving power to the powerless” “Persuasive communication and targeted actions in support of a cause or issue that seek to change policies, positions, and programmes”

181

SYMPOSIUM: SOCIAL PAEDIATRICS

venepuncture service only and she realizes that children are often upset by this. Surely there should be a paediatric service in outpatients? Advocacy role: talk to manager of the service and find out scope for change and make a business case for a paediatric venepuncture service. Obtain colleagues’ and parents support for this and consider taking it to the Trust CEO as this is an example of very poor practice, with children’s needs not being adequately met. These true examples (the names have been changed) show that every day, paediatricians are liable to come across situations requiring advocacy. A common response from the stressed out paediatrician is to think e oh no e I can’t do anything about that. Our experience with child protection tells us that this response is not acceptable. We can do something, and we may have to. And it is much easier to do something, if we have past experience, if we understand how the system works, and if we work together with our colleagues. Paediatricians are extremely powerful advocates, (see Box 2), and with the right approach you will have a good chance of success.

Paediatric advocates C

History of paediatric advocacy For many years, there have been paediatricians who have spoken up for their patients and for social conditions which will benefit children. Some of those who are best known are shown in Box 3 but there are many others who are known to their colleagues and the parents they work with.

Box 3

What does the Convention have to say about child health? Almost all of the 41 articles apply to health, some of them (for example, Article 12 the right to express a view) to every consultation and others (for example Article 36 on protection against exploitation) only occasionally. The convention covers three main fields: provision, protection and participation. The state need to provide all children with an education and health service, with support in times of difficulty, and with the wherewithal for a reasonable lifestyle. The state will

Underpinning principles for advocacy What are the principles underlying advocacy by paediatricians? Sometimes it is obvious e as in the examples above. Sometimes the law is behind us, particularly in the child protection field e in this respect, the Children Act in England give us powerful backing. GMC guidance is also important and is in line with the UN Convention on the Rights of the Child. For me, the latter offers the most significant backing for my advocacy work with children, yet most paediatricians have had little training in its application. A child rights approach encourages us to think first of children as members of society and only second as patients with medical problems. The Convention also encourages us to see children and young people as autonomous individuals who are active participants in the world e and this implies in the consultation too.

One paediatrician and a thermometer In the 1970s an American paediatrician’s experience of children being scalded by hot tap water led him to question why such seemingly preventable injuries had occurred. An investigation of national statistics showed that around a sixth of all serious childhood scalds were caused by tap water. Dr Ken Feldman, his wife and an assistant from his Seattle clinic started knocking on doors in the neighbourhood and measuring tap water temperatures. They discovered that 80% of domestic hot water supplies were routinely set (often pre-set by the manufacturer) at a temperature that could cause full-thickness burns in under 30 s. Lowering the temperature by even five degrees would raise this burn-time to 10 min. A local public education campaign in conjunction with the utility companies resulted in widespread lowering of water temperatures in the area. Feldman also led a campaign for a law requiring heaters to be pre-set at the factory or installation at a safe temperature. Florida became the first state to enact such a law in 1983 and others followed; manufacturers increasingly volunteered to lower the settings. The national incidence of tap burns in children decreased.

Why paediatricians are powerful advocates C C C

C C C C

They are seen as lacking self interest They can take an independent view of children’s needs They are known to take a balanced view and make use of evidence, hence less likely to be biased They are well informed on how children’s services operate They are well respected by parents They carry influence within the power structure They are likely to be offered media space owing to their position

Box 2

PAEDIATRICS AND CHILD HEALTH 22:5

James Spence (1950s): pressed for parents to be allowed to stay in hospital with their children, and this eventually became the norm Donald Court (1960s): ensured that parents were represented on a committee for reforming children’s health services, and after this it became common to consult with parents Hugh Jackson (1970s): publicized the harm done by children ingesting adult medicines and became a fervent advocate of injury prevention with the eventual result of the enforcement of child-proof containers Dr Ken Feldman (USA, 1970s): worked for legislation to prevent scalds by lowering water heater temperature settings [see Box 4 below] Al Aynsley Green (2000s): Prof of Child Health at ICH, London then became the English Children’s Commissioner and took up the case of children in detention in the criminal justice system

Box 4

182

SYMPOSIUM: SOCIAL PAEDIATRICS

Securing effective advocacy services for children; Undertaking strategic research and data collection; Securing a cost-analysis to show the financial benefits of the goal being advocated; Piloting innovative models of practice; Disseminating proven models of good practice; Building up coalitions, alliances and action groups seeking a common end; Using the media to publicize the issue and to inform and influence opinion; Using the law to claim rights; Disseminating the message through publications, training materials, conferences etc.; Cultivating relationships within local and central government and other decision-making bodies; Joining committees, task groups etc.; Responding to consultations on proposed changes; Lobbying local and national politicians, administrators and decision-makers.

also protect against abuse and neglect, against exploitation of various kinds, and at times of stress such as when separated from parents. Participation rights include the right to information (and this includes information on diseases and treatment), to participate in society (and this includes disabled children) and to express a view. At the simplest level, in a consultation a paediatrician should listen to the child; should provide information on the examination being carried out and the findings, should explain the nature of the condition in terms the child can understand, should ensure the child is protected from violence, and should ascertain the childâ&#x20AC;&#x2122;s view on what has been said. There are plenty of opportunities for advocacy in the consultation and in the first place, the paediatrician needs to ensure that the environment (and other staff working there) is child centred and the same principles are applied. Box 5 lists some of the key articles of the Convention in relation to child health but it is important to recognize that almost all the articles do apply in the health service. Currently the UNCRC is not law in the UK, whilst the Children Act is a legal instrument. Hence enforcement of the Convention requires good will rather than the justice process. The UK Government has ratified the Convention and this requires it to make 5 yearly submissions to the UN Committee on the Rights of the Child on its progress. The committee then makes a report on UK performance. NGOs may also submit a report which can be critical of the government and these are considered by the UN at the same time. However there is no procedure for examination of individual cases. Each of the delegated countries in the UK has a Commissioner for children whose responsibility it is to ensure that the voice of children is heard and that childrenâ&#x20AC;&#x2122;s issues are accorded high priority. The English Commissioner does not have the power to examine individual cases but will address policy.

Current pressing issues for advocacy in the UK and globally Iâ&#x20AC;&#x2122;ll give a short list of what I think are pressing issues for child health advocacy in 2011. These are listed in Box 6 and I offer

Advocacy priorities in UK in 2011 Health services C Child health to be prioritized in present re-organization and oppose privatization C Within child health: adolescent health, child mental health, disability services C Children and young peopleâ&#x20AC;&#x2122;s views to be heard

Methods and techniques for advocacy

Education C Better facilities for special needs pupils especially behavioural and learning difficulties C More and better trained school nurses in secondary schools C More parenting support especially for teenagers

Methods and techniques for effective advocacy (both direct and public policy) include: Taking direct action on behalf of individual children by writing letters, making telephone calls, giving evidence, attending meetings, arranging expert assistance or second opinions; Facilitating self-advocacy by children and their families or local groups;

Child health C Legislation on corporal punishment at home C Stop the detention of children (asylum seekers and others) C Unit pricing for alcohol C Tougher legislation on food labelling and food marketing on television C Education of parents on control of television for children C Universal parenting programmes for all first parents

Key articles of UNCRC for child health Article 5 e The right of children to respect for their evolving capacities Article 12 e The right to be listened to and taken seriously Article 13 e Freedom of expression e the right to seek, receive and impart information and ideas of all kinds Article 16 e The right to privacy and respect for confidentiality Article 19 e Protection from physical or mental violence, injury or abuse

Global C C C

Box 6

Box 5

PAEDIATRICS AND CHILD HEALTH 22:5

Paediatric advocacy on health impact of climate change Raising awareness of violence against children Ensure that child rights are considered in all policies

183

SYMPOSIUM: SOCIAL PAEDIATRICS

Health impact of climate change

a short explanation of some of them below. This is an individual list and others may prioritize differently.

What needs to be done? A review of the statementing system with paediatric input and better support for special needs pupils, particularly those with behavioural problems and learning difficulties. Who needs to do it and how? Paediatricians and paediatric organizations together with special needs support organizations. There will be no quick solution here as funding is required as well as organizational reform. However the delays in proper assessment and the limited involvement of paediatricians at an early stage are leading to much suffering and loss of education by children with intrinsic disabilities. How feasible? Certainly possible but requires sustained lobbying supported but not lead by paediatricians.

What needs to be done? Climate change is still not at the top of the agenda for the present government, despite the evidence that it is the greatest global health threat of the 21st century. Highlighting the health impact could ensure that there is public support for early government action including personal carbon quotas and a carbon tax, as well as much greater emphasis on domestic and industrial use of renewable fuels and a modal shift from car and air traffic to public transport and bicycle use. Who needs to do it and how? Both individual action by paediatricians and lobbying by the RCPCH is required, in addition to the current efforts of the Climate and Health council. How feasible? Progress is definitely feasible but the problem is the current political and public climate which is not prioritizing climate change, owing to mis-information given out by the media. How should these topics be prioritized by a paediatric organization? At the present, topics taken up tend to be reactive and depend on individual interest: for example the present support by RCPCH for legislation on parents smoking inside cars. In my view this is of dubious significance in relation to its impact on child health and will be very difficult to enforce. Whereas unit pricing of alcohol is eminently achievable and could have an immediate impact. I would suggest the following criteria for an organization to select topics proactively to take up in the public sphere: Is there potentially widespread support among the general public? Is the measure achievable within the political system? Can the paediatric organization bring added strength to a coalition seeking change? Is there a child rights perspective on the topic at issue? Is there a role for individual paediatricians to campaign as well as the organization? This would add greatly to the force and effectiveness of the lobby.

Corporal punishment in the home

Whistle blowing

What needs to be done? There is a need for legislation to make it not permissible for parents to use corporal punishment on their own children. At present they are allowed to do so as long as there is no mark to the skin. Paediatricians consider this to be unacceptable and the RCPCH has written a Position Statement which recommends legislation, following the lead of many European countries. Who needs to do it and how? The Government could quite easily bring in an amendment to the Children Act and efforts have been made by the Children are Unbeatable Alliance of which the RCPCH is a member, to support this. However up to now no Government has been prepared to back such legislation, even though the majority of the children sector backs a change in the law. How feasible? Legislation to make corporal punishment analogous to assault could be introduced easily and has support from many MPs. A strong lobby by voluntary organizations including the RCPCH is likely to be successful as soon as a government is elected which is more favourably inclined to the needs of children.

Currently there is much concern about doctors being able to speak out in relation to problems within their Trust. Some of those who have spoken out have lost their jobs, and at the same time the GMC and others are encouraging this role. Hence there is a great need for understanding among doctors about how to carry out this type of advocacy.

Adolescent health What needs to be done? Improved facilities in hospital wards, paediatric outpatients and GP surgeries. Who needs to do it and how? Paediatricians and paediatric organizations together with the voluntary sector. There needs to be an introduction of quality standards relating to adolescents and these should then be brought to the attention of all Trusts. Teenagers should be consulted over this. This topic should be a priority for paediatric organizations. Governmental support is also essential. How feasible? Considerable improvement could be brought about through staff education and modest funding. A longer term perspective would lead to more adolescent wards and separate facilities in outpatients.

Special needs pupils

PAEDIATRICS AND CHILD HEALTH 22:5

Training of paediatricians in advocacy I have covered this topic in a previous article. There is a list of competencies on advocacy topics in the RCPCH curriculum but at present there is little formal teaching. The key competencies are listed in Box 7. These competencies are difficult to cover in normal teaching sessions and are best fulfilled through a project, as is the case in the MMed Sci teaching programme in the Yorkshire deanery. The Advocacy module in this programme is exemplary in offering both knowledge and skills and is based on real situations. Students keep a diary at the beginning of the module to identify occasions in which advocacy is necessary. They then develop one topic as a project. Recent advocacy projects include access to toilets in

184

SYMPOSIUM: SOCIAL PAEDIATRICS

you call for legislation against corporal punishment in the home, or restriction of food marketing to children). Avoid this by using objective evidence and keep away from Party politics. Third, you can’t advocate for everything e pick your case, and try to prioritize those issues where the need is greatest and where you have the best chance of success. Fourth, you may disempower parents by taking over their role. In many cases, parents are the best advocates for their children. Work side by side with them as an advocate and consult with them over the steps you are proposing to take.

RCPCH competencies in relation to advocacy and related areas C

C C

Develop a commitment to a policy of advocacy for a healthy lifestyle in children and young people and for the protection of their rights Know the principles of the UN Convention on the Rights of the Child, apply these in their own practice and work for the protection of these rights To take on an advocacy role with regard to the best interests of the patient; to ensure appropriate care for patients; to be able to think independently and to challenge decisions or protocols where appropriate Demonstrate an understanding of and respect for legal and ethical issues relating to consent and confidentiality in paediatrics, including the Fraser ruling (formerly known as Gillick competence) Know the law with regard to consent to treatment and the right to refuse treatment, and be aware of variability in Scotland, Wales and Northern Ireland, continuing changes in the law and its interpretation Be aware of child health exploitation issues including child prostitution, child labour and children in combat Be aware of the effect of non-health policies on child health Understand the role of the paediatrician in advocating for children at individual, community, national and international levels Understand (be aware of ) the key determinants of child health and well-being Develop a commitment to advocacy on behalf of disabled children and their families Understand the importance of seeking the views of all children to inform decisions about their individual care and about planning services

The future It is likely that advocacy will become even more necessary in the future as our health service in the UK is under threat and children’s service are becoming more complex. Hence it will be vital for paediatricians to integrate advocacy with their already excellent skills in communication, diagnosis and disease management. To do this they will need the knowledge, practical skills and experience which are outlined in this article. Children will benefit and paediatricians will be able to take pride in their achievement. The role of paediatric associations is critical, the RCPCH and the American Academy of Pediatrics have shown leadership and it is time for other associations with a child health and international perspective to follow in their footsteps. A

FURTHER READING General Medical Council. Guidance for all doctors on children 0e18 years. London: GMC, 2007. Katcher M. Tap water scald prevention: it’s time for a worldwide effort. Inj Prev 1998; 4: 167e8. RCPCH. Advocating for children RCPCH, London 2008. Royal College of Paediatrics and Child Health. A framework of competencies for basic specialist training in paediatrics. London: Royal College of Paediatrics and Child Health, 2004. Rudolf MCJ, Bundle A, Damman A, et al. Exploring the scope for advocacy by paediatricians. Arch Dis Child 1999; 81: 515e8. UN Convention on the Rights of the Child. www.unicef.org/crc (accessed 1 Sep 2011). Waterston T. Teaching and learning about advocacy. Arch Dis Child Educ Pract Ed 2009; 94: 24e8. Waterston T, Goldhagen J. Why children’s rights are central to international child health. Arch Dis Child Feb 2007; 92: 176e80. Waterston T, Haroon S. Advocacy and the paediatrician. Paediatr Child Health 2008; 18: 213e9. Waterston T, Tonniges T. Advocating for children’s health: a US and UK perspective. Arch Dis Child 2001; 85: 180e2. http://www.rcpch.ac.uk/news/new-position-statement-corporalpunishment-8-october-2008 (accessed 1 Sep 2011). http://www.childrenareunbeatable.org.uk/ (accessed 1 Sep 2011). http://www.thelancet.com/series/health-and-climate-change (accessed 1 Sep 2011). www.climateandhealth.org (accessed 1 Sep 2011).

Box 7

a secondary school, developing a children’s venepuncture service, and setting up advocacy teaching in medical school. Attachment to voluntary sector organizations is another means of helping trainees to gain experience of advocacy in a practical way.

Pitfalls of advocacy Are there any pitfalls involved in taking up advocacy for patients or for children’s health? Indeed there are. First, you may be seen as biased or showing poor judgement. Avoid this by gaining the opinion and support of colleagues, and ensuring you have a good evidence base. Advocacy carried out on behalf of a group of paediatricians is more powerful than that done by a single person. Second, you may be seen as being overtly political (for example if

PAEDIATRICS AND CHILD HEALTH 22:5

185

SYMPOSIUM: SOCIAL PAEDIATRICS

Childrenâ&#x20AC;&#x2122;s attachments

The quality of childrenâ&#x20AC;&#x2122;s parental attachments has profound, far-reaching implications because immaturity means that they depend on these to progress safely towards independence. Attachment develops through parental attunement to infantsâ&#x20AC;&#x2122; needs, establishing their preconceptions of relationships and foundations of verbal and non-verbal communication. In giving meaning to feelings and body signals, attuned parenting builds selfawareness. By modulating stress, it contributes to programming of the stress systems, with life-long implications for regulation, behaviour and physical and psychological health, including major causes of mortality. Suboptimal attachment falls on a spectrum ranging from mildly insecure to profoundly dysfunctional. Quality of attachment is relevant to symptom presentation, illness behaviour, service use, and optimal paediatric care. Unsatisfactory childhood attachment frequently underlies intergenerational parenting problems. It is an important consideration in all child protection decisions and their implementation. Enabling children to achieve adequate parental attachment is an overriding priority in working with children in care and in adoptive homes, and is a priority for every child.

a need for approval or attention being channelled into, perhaps, caring or performance, a tendency to â&#x20AC;&#x2DC;avoidanceâ&#x20AC;&#x2122; of relationships into abstract skills such as computing or mathematics, for example. Secure attachment is, however, associated with greater ease of relationships and more positive self-esteem. The â&#x20AC;&#x2DC;adverse childhood experiencesâ&#x20AC;&#x2122; studies show an association between dysfunctional early attachment and adult physical and psychological wellbeing, including major causes of mortality. The more prolonged and extreme the dysfunctional parenting, the greater are the secondary consequences. These characteristically interrelate into vicious circles of which poor self-esteem is integral. Difficulties with peer relationships, emotional dysregulation, impulsivity and inattention are common, compounded by failure at home and school. While resilience varies markedly, children in care, whose attachments are particularly vulnerable, are at increased risk of: mental health problems early parenthood substance abuse lack of qualification unemployment homelessness involvement in crime as victims or perpetrators. The parents of most children adopted from care experienced inadequate early attachment as, frequently, did their own parents.

Keywords adoption; attachment; attention deficit hyperactivity disorder;

Foundations of attachment

Corinne Rees

Abstract

attunement; autism; behaviour; children in care; parenting; stress regulation

Attachments form at every age. However, clinical and neurobiological evidence supports Bowlbyâ&#x20AC;&#x2122;s view that early foundations are particularly important. As in a wall, the quality of foundations of attachment remains important whatever follows. Neural plasticity allows moulding by subsequent experiences, but these build on, rather than replacing, preconceptions derived from the first relationship, which resurface most prominently under stress. Attunement to the needs and feelings of others is the route to building and sustaining any attachment at any age (Box 1). Stern demonstrated by video studies subtle maternal attunement to babiesâ&#x20AC;&#x2122; overtures, involving tone, pitch and rhythm of voice, posture, facial expression, movement and touch, providing a mirror in which their â&#x20AC;&#x2DC;inner worldâ&#x20AC;&#x2122; is reflected, given meaning and regulated, moulding development of the right pre-frontal cortex. This process requires the baby to show their needs and the parent to respond, in a two-way, mutually reinforcing process. As in any relationship, fatigue, stress, distraction and inadequate one-to-one time adversely affect parental attunement. Mental health problems, drugs and alcohol similarly affect it, as do poor personal foundations of attachment. Babies learn through this relationship that others can recognize and respond to their needs, that their behaviour influences others, and that moderate expression of need elicits a response. Foundations are established of perceptions of the value of relationships, of verbal and non-verbal communication, of understanding the â&#x20AC;&#x2DC;inner worldâ&#x20AC;&#x2122; of emotions, body signals and thought, and of trust. Assumptions develop of the extent to which it is safe to relinquish attention and control.

Definition Attachment is the enduring emotional closeness which binds families, to protect children and prepare them for independence and parenthood. Because immaturity makes children dependent, the quality of their parental attachments underpins their physical and psychological wellbeing, growth and development. The length of childhood indicates the complexity of the task and the far-reaching implications of dysfunctional parenting. Early attachment establishes childrenâ&#x20AC;&#x2122;s intuitive preconceptions of the value, reliability, safety and use of relationships, with life-long implications for the extent of emotional self-sufficiency, and for behaviour in relation to others. The responses of attached parents give meaning to a childâ&#x20AC;&#x2122;s â&#x20AC;&#x2DC;inner worldâ&#x20AC;&#x2122;, and facilitate safety, stress regulation and resilience. Inadequate attachment can be highly costly to individuals, to public services, and to wider society. Suboptimal attachment falls on a spectrum, ranging from the mildly suboptimal to the seriously dysfunctional e a problem if it is a problem. 35e40% of nonclinical populations show insecure attachment. A degree of insecurity of attachment may sometimes have value for society e

Corinne Rees MA MB ChB FRCP MRCPCH is Medical Adviser for Adoption at North Bristol NHS Trust, Community Child Health Partnership, Westgate House, Southmead Hospital, Southmead Road, Westbury-on-Trym, Bristol BS10 5NB, UK. Conflict of interest: none.

PAEDIATRICS AND CHILD HEALTH 22:5

Protection is the most prominent evolutionary role of parental attachment. It allows physical and emotional safety, and the

186

SYMPOSIUM: SOCIAL PAEDIATRICS

visually, then through language as mobility develops. Attachment provides the secure base which, paradoxically, facilitates increasing distancing from parents with maturation and underpins safe adolescent separation. It enables children to handle the emotional â&#x20AC;&#x2DC;separationâ&#x20AC;&#x2122; of discipline, accept authority and develop self-worth.

Basics of attachment C

Attachment forms and is sustained by parental attunement to the babyâ&#x20AC;&#x2122;s overtures, through tone, pitch, speed of voice, facial expression, touch and movement Attunement provides a mirror reflecting the babyâ&#x20AC;&#x2122;s feelings and body signals Early attachment establishes foundations for: Preconceptions of relationships Perceptions of the safety of relinquishing attention and control Preconceptions of self Behaviour Verbal and non-verbal communication Emotional awareness Stress system programming

Patterns of attachment Terminology used to describe patterns of relationships, or â&#x20AC;&#x2DC;attachment stylesâ&#x20AC;&#x2122;, varies. The patterns merge, and are not entirely fixed, although tendencies persist. Understanding and describing how children use relationships, and the implications, matters more than labelling. Secure attachment depends on infantsâ&#x20AC;&#x2122; ability to elicit a response and parentsâ&#x20AC;&#x2122; ability to respond sensitively and consistently (Box 1). Relationships are experienced as valuable, reliable and safe, and attention as readily gained when needed, so safely relinquished.

Attunement is affected by stress, fatigue, drugs, alcohol, learning difficulties, poor early parenting Preconceptions deriving from initial relationships remain the most intuitive

Anxious attachment arises if, for example, fatigue, stress, conflicting demands, mental health problems or substance abuse interrupt otherwise good attunement. Children learn that, although valuable, relationships are unreliable. They become insecure in their ability to hold and regain attention, so are liable to learn any behaviour, desirable or otherwise, which achieves it. This ranges from smiling or over-compliance to disruptiveness, soiling, food refusal, or endangerment. They struggle with sharing attention and handling the emotional â&#x20AC;&#x2DC;separationâ&#x20AC;&#x2122; of discipline. Fear of rejection colours relationships. Stress is poorly regulated, and often managed by seeking control and resisting change.

Box 1

experience that these are achievable through relationships. Attachment is relevant to both involuntary and cognitive means of stress regulation, and may influence immunity, healing and the intestinal mucosal barrier. Stress regulation depends, for infants, on parentsâ&#x20AC;&#x2122; ability to recognize and respond to their needs. Their effectiveness in doing so contributes to programming the hypothalamuse pituitaryeadrenal (HPA) and autonomic systems of stress regulation e a process which is thought to start in utero, with lifelong implications, albeit modified by subsequent experience. Regulation of stress matters for exploration, learning, independence and effective relationships. Unregulated stress affects concentration, and â&#x20AC;&#x2DC;readingâ&#x20AC;&#x2122; of relationships. It predisposes to: anxiety and depression conduct disorder aggression attention deficit hyperactivity disorder (ADHD) post traumatic stress disorder â&#x20AC;&#x2DC;psychosomaticâ&#x20AC;&#x2122; illness chronic pain. While suboptimal attunement frequently generates exaggerated stress responses, serious abuse sometimes suppresses them, leading to fearlessness, and autonomic underarousal (e.g. relative bradycardia), correlating sometimes with â&#x20AC;&#x2DC;avoidantâ&#x20AC;&#x2122; infant attachment behaviour. Neuronal plasticity, developing cognition and experience modulate stress responses during childhood around a baseline influenced by the first relationship.

Ambivalence about the value or safety or relationships is learnt through parenting which is, for example, variably adequately attuned and aggressive. Closeness is wanted but also frightening. Childrenâ&#x20AC;&#x2122;s resulting ambivalence produces confusing behaviour. Confrontation tends to be focussed on those to whom they are closest. They may crave attention but, having achieved it, reject it. They sometimes seek and sometimes avoid closeness. Hypervigilance to parental mood affects concentration and causes over-reading of disapproval. Fear of intimacy may persist into adult relationships. Avoidance of relationships may be learnt from consistently unattuned parenting (â&#x20AC;&#x2DC;e.g. if parentsâ&#x20AC;&#x2122; own foundationsâ&#x20AC;&#x2122; for attachment were poor). Children can become â&#x20AC;&#x2DC;avoidantâ&#x20AC;&#x2122; loners, inept at understanding non-verbal cues and the subtleties of language, often seeking control through â&#x20AC;&#x2DC;samenessâ&#x20AC;&#x2122; to regulate anxiety. The resulting picture resembles â&#x20AC;&#x2DC;innateâ&#x20AC;&#x2122; autistic spectrum disorder. Some gorge to the point of vomiting, disregard ambient temperature, ignore pain, and overlook toilet needs because their â&#x20AC;&#x2DC;inner worldâ&#x20AC;&#x2122; has not been given meaning. Disorganized patterns arise if pervasive abuse leaves children ineffective both in self-sufficiency and in using relationships, lacking understanding of their own and othersâ&#x20AC;&#x2122; feelings. Safe independence is unlikely and criminality in adulthood common without recovery.

Breaks in attunement: inevitable beaks in the immediate availability of even the most sensitive of parents allow controlled exposure to stress, and trust. Children develop confidence that separation from their parents is safe, initially maintaining contact

PAEDIATRICS AND CHILD HEALTH 22:5

187

SYMPOSIUM: SOCIAL PAEDIATRICS

Considering attachment in paediatric clinics

dysfunctional attachment. Concentration is unsafe for children facing unpredictable aggression; protective hypervigilance precludes concentration. Diagnostic profiles for ADHD may be atypical if attachment difficulty makes symptoms more troublesome at home than school.

Attachment is always relevant to paediatric care e the questions are in what way, and to what extent. Noticing the quality of parental relationships should be as routine as noticing whether or not a child looks ill e as much a subconscious background awareness as a conscious analysis. The degree of security of attachment e of both child and parent e may colour use of professional services, response to symptoms and ‘illness behaviour’. It sometimes entirely determines children’s needs (e.g. in safeguarding and substitute care). It always warrants consideration in assessing and managing problems with feeding, toileting, crying and behaviour which involve interaction, as a primary or a secondary issue, or both.

The origins of ADHD symptoms cannot necessarily be determined precisely and may be immaterial to management, which demands pragmatism focussed on individual priorities, of which adequate parental attachment overrides all. Whether cause, effect, or both of attachment difficulty, ADHD symptoms need to be addressed promptly because of the risk of escalating difficulty. Attachment to impulsive children can be difficult. In care, they are at risk of placement breakdown, and of failure to achieve stability. ADHD symptoms characteristically increase after moves, when new parents and children lack a safety-net of mutual attachment. Inattention affects emotional awareness and reading of non-verbal communication, influencing all relationships. Poorly attached children lack self-esteem, and untreated ADHD compounds this. Vulnerability to rejection and hypervigilance to disapproval reduce their resilience to the disciplinary consequences of their behaviour; success at home and school can be elusive.

Problems with eating and toileting relate to attachment in several ways. Children who are anxious about parental ‘availability’ are drawn to behaviour that is difficult to ignore (e.g. food refusal, soiling, wetting). Those ambivalent about the safety of close relationships may use these behaviours to ‘get at’ parents. Chaos and neglect make children take control, including through eating and toileting issues. Over-attuned parenting encourages dependence. Children may not develop age-appropriate self-care, holding onto the security of immaturity. Insecurity after moves (e.g. into foster care) may prompt regression of self-help. Persistently unattuned or frightening parenting, causing avoidance of relationships, can lead to gorging to the point of vomiting, soiling or wetting because of failure to recognize body signals. Over-eating may fulfil an emotional need, or represent primitive ‘clinging’ to food after deprivation. Chaotic eating, affecting the gastrocolic reflex, and absent toileting routine may accompany inadequate attachment. Toilet training may be lacking, punitive or associated with abuse. Altered gastrointestinal flora reflecting disturbed attachment may affect bowel function, as may altered diet after moves. Regardless of their origin, eating or toileting problems in children with insecure attachment are often perpetuated as a source of attention, which is an important consideration in management.

Managing ADHD in the context of vulnerable attachment requires broad-based pragmatism (Box 2). Medication, when used, is an adjunct to, and facilitator of, attachment-related behavioural strategies, and a means of enabling children to use

Principles of managing ADHD type symptoms in relation to attachment Principles C Pragmatism focussed on priorities [stable home, adequate attachment, self esteem] C Broad-based, multifaceted care C Medication can facilitate other approaches

Crying: the precise origins of infant crying are often uncertain and sometimes immaterial to its management. Maternal fatigue and depression affect attunement, allowing unregulated infant stress and crying, further exacerbating parental stress. Help includes explanation, social support, treating depression, calming strategies for parent and child (e.g. infant massage), and explanation of attunement. Similar sequences may contribute to irritability of children whose neurological difficulties affect attunement, and contribute to parental depression and fatigue.

Practical approaches C Exposure to calm environment; gradual introduction to high stimulus environments C Calming through relationships e e.g. massage C Routine to reduce anxiety C One-to-one time with minimal distraction C Physical exercise C ‘Calm corner’ created by the child Aims of medication C Facilitation of emotional awareness, reading of non-verbal communication and attachment C Enabling attention to be achieved positively C Helping the regulation of stress and emotion C Helping peer relationships C Facilitating psychological therapeutic work

Attachment and ADHD Symptoms of ADHD can be cause or effect of attachment difficulty e often both. They relate also through common risk factors and as comorbidities. Familial risk of ADHD is high in families experiencing multigenerational abuse. Consequences of parental ADHD may affect attachment: ADHD may underlie parental substance abuse or temper problems. Impulsive parents struggle to parent impulsive children safely. Antenatal exposure to drugs, alcohol and stress are risk factors for ADHD and for

PAEDIATRICS AND CHILD HEALTH 22:5

Box 2

188

SYMPOSIUM: SOCIAL PAEDIATRICS

family, school and professional psychological support to achieve recovery. â&#x20AC;&#x2DC;Slowing downâ&#x20AC;&#x2122; can help childrenâ&#x20AC;&#x2122;s emotional awareness, enable them more readily to achieve attention by desirable means, and encourage mutual attachment. It can help to interrupt vicious circles. The principles of prescribing are those generally applicable to ADHD, although specific evaluation is needed of options and priorities in the context of attachment difficulty.

precipitate uncontrollable outbursts with seemingly little immediate provocation. When children are learning to live with safe relationships, having honed their behavioural strategies in response to abuse, seemingly unreasonable reactions to â&#x20AC;&#x2DC;reasonableâ&#x20AC;&#x2122; parenting can provoke rejection, reinforcing their already negative self-image. The extent to which children see relationships as valuable, safe and trustworthy is a key issue. Those who avoid relationships may seem, in a new home, â&#x20AC;&#x2DC;easyâ&#x20AC;&#x2122; in their lack of demand, but parents describe difficulty in feeling â&#x20AC;&#x2DC;not neededâ&#x20AC;&#x2122;. Those who value relationships but are insecure in their continuing availability tend to seek and hold attention by whatever means work. Their carers can be overwhelmed by their insatiable demands. The question is which factors in the child, parents and environment are contributory or perpetuating, and which can be changed. Explanation of the childâ&#x20AC;&#x2122;s behaviour enables carers to see through the childâ&#x20AC;&#x2122;s eyes. Minimizing parental fatigue and stress is a priority to allow sufficient stamina for effective attunement and calm boundary setting. Parents must be reassured that perfection is neither attainable nor necessarily desirable. Traumatized children frequently drive new parents to responding in less than perfect ways, causing profound guilt. Such times can be used to model repair and build the childâ&#x20AC;&#x2122;s sense of unconditional acceptance (Table 1).

Autism or attachment? Children who have learnt to avoid non-attuned or frightening parenting frequently fulfil criteria for autistic spectrum disorder, a pattern observed following emotional deprivation in orphanages. Children may show: little eye contact isolated play poor social and communication skills literal interpretation of language poor emotional regulation intolerance of change stereotypical play executive function difficulties. Whatever the origins of autistic patterns, attachments are inevitably relevant, even if not causative, and broad-based care is warranted, considering which contributory factors in the child, parents and environment can be changed, how, and how soon, and the implications of failing to do so. When early attachment is an adequate, if not the only plausible explanation for these attributes, care is needed to avoid â&#x20AC;&#x2DC;fixingâ&#x20AC;&#x2122; the child in expectations. Labels, once given, are not readily shed, and their benefits in the short and longer term should outweigh disadvantages. Developing areas of ability is as important as helping deficits e for example actively building non-people skills which are the probable long-term route to achievement.

Safeguarding Attachment should always be a central consideration in safeguarding children (Table 2). It is frequently not the injury but the disturbed relationship it represents that causes greater long-term damage. An accident may be as significant as non-accidental injury if it reflects inadequate supervision or indifference to danger accompanying poor attachment. Emotional abuse is the result of, and causes, dysfunctional attachment. Like a familiar painting, it is more readily recognized than defined. Identifying it resembles the construction of a jigsaw from pieces of varying clarity and certainty, all needed to build the whole picture. Childrenâ&#x20AC;&#x2122;s attachments should be a key consideration in the timing and manner of implementing child protection decisions. Children may have important attachment to unsafe parents, and abuse may be longstanding. Protecting emotional safety may entail accepting a degree of physical risk. The trauma of emergency removal must be balanced against the risk of delay to allow preparation.

Behaviour management Behaviour is inextricably linked to attachment, as cause and effect. It serves a purpose, and generally makes sense in terms of childrenâ&#x20AC;&#x2122;s preconceptions of themselves, relationships and the world, although coloured by sensory and cognitive ability and impulse control. It is prominently influenced by their intuitive perceptions of the safety, or otherwise, of relinquishing attention and control, which are founded in the earliest experiences of attachment. Children learn behaviour which works in the relationships with which they are, or were, living. They learn from the responses which their behaviour elicits, and by role model. The earliest patterns of relationships remain the most instinctive. A child whose behaviour was self-protective from parental aggression may, even if adopted into a secure household, revert under pressure to patterns which used to work e their â&#x20AC;&#x2DC;first languageâ&#x20AC;&#x2122;. The â&#x20AC;&#x2DC;second languageâ&#x20AC;&#x2122; of behaviour which suits calm relationships comes less naturally and needs gradually to be learnt. Following dysfunctional early attachment, behaviour may be coloured by difficulties of regulation caused by overactive early programming of involuntary responses, often compounded by failure to learn cognitive strategies to regulate these. For some, trauma contributes to dysregulation: difficult feelings may

PAEDIATRICS AND CHILD HEALTH 22:5

Substitute parenting Removing children from home introduces new risks and major new professional responsibilities. The purpose should be clearly defined, and tasks necessary to achieve this identified, with an eye always on the overriding priority of achieving adequate permanent parental attachment. â&#x20AC;&#x2DC;Careâ&#x20AC;&#x2122; is a therapeutic task, integral to safeguarding. Working together is as important as when identifying risks: service structures should reflect this. Paediatricians may not technically be â&#x20AC;&#x2DC;corporate parentsâ&#x20AC;&#x2122;, but effectively they are, and commitment should be commensurate with this (Table 2). Achieving the adequate psychological care which will help to determine childrenâ&#x20AC;&#x2122;s future wellbeing requires professional continuity, commitment, and pragmatism. It

189

SYMPOSIUM: SOCIAL PAEDIATRICS

Managing behaviour

Considering attachment in safeguarding and substitute care

Usually relevant: Making safeguarding decisions

Parental responses Need for attention Need for control

Are there parental risks to attachment? Dysfunctional personal foundations of attachment Drugs, alcohol, mental health problems, learning difficulties, temper dysregulation What factors may allow safe parenting despite parental risks? How would the child be likely to understand relationships judging from: C C

Often relevant: Anxiety Fear of failure and rejection; hypervigilance to disapproval Impulsivity Difficulty regulating stress and emotion Lack of concept of safety Lack of experience of boundaries Lack of familiarity with success Poor sleep pattern

Parental risk factors Quality of observed parental attunement How does the child’s observed and described behaviour fit with expected attachment? To whom are the child’s key relationships within and outside the family? What is the quality of sibling relationships? What role does the child have in the family? (e.g. ‘parental’) What would be the purpose of foster care? What are the risks? C C

Management: Parental self-care e a responsibility, not a luxury Attention for desired behaviour Structure the day to maximize success (e.g. one-to-one time) Clear, consistent boundaries and consequences Empathy for feelings underlying behaviour and evoked by discipline Manage control issues: simple choices, diversion Reduce anxiety: routine, environmental calm, exercise

Planning moves How is the child likely to view a move? What are his/her preconceptions of adults/closeness? How is he/she therefore likely to behave? What are the implications for:

Education:

how adopters will feel family dynamics C preparation and support needed for child and family? Do the benefits of emergency placement justify the risks? How can relatives/carers be enabled to support a move? Can a trusted adult accompany the child? C

Model and teach regulation of stress, emotion, temper Teach social skills Encourage all means of success to help self-esteem Generalize: convey the learning of behaviour as a normal, universal need

Table 1

Assessing long-term attachment needs Is it likely that long-term attachment needs can be met at home? Which risks to attachment could be changed? How soon? Is adequate change likely sufficiently soon? What are the risks of delay? Is the child likely to form adequate attachment away from home? What is needed to achieve this? Which relationships must be protected? How? What does the quality of sibling relationships mean for placement plans? What are the advantages and risks of placement together? What are the implications of the child’s attachment pattern for the choice of family?

requires seeing beyond form-filling and definable targets to the daunting reality of the greater task. When adoption offers the opportunity of stability and recovery, considerable, often longterm, professional support may be needed to achieve these. Professional services should reflect the profound vulnerability of children who lack stable relationships. Parenting without mutual attachment is difficult and frightening. Crises are inevitable and often costly. Placement breakdown is highly traumatic for all concerned. Every move adds to the risk of a trajectory of repeated failed placements, and dysfunctional adult life characterized perhaps by unsustained relationships, parenting problems, substance abuse and criminality. Cornerstones of practice are continuity, commitment, availability and rapid responses. Services which do not allow these cannot meet children’s needs. The cost of adequate provision must be balanced against that of failure to achieve stability.

Table 2

Common sense based on experience of everyday adult relationships goes a long way in understanding childhood attachment: simple principles benefit all although some also need more specialized support. Risks to attachment should be identified antenatally and preventive advice offered. Early intervention when there are lesser degrees of difficulty can prevent escalation into the vicious circles and fixed patterns which readily develop.

Working with attachment Noticing the quality of parental attachment should be integral to paediatrics. Sometimes it is a sufficient concern to be at the core of necessary work. Often it is merely a consideration.

PAEDIATRICS AND CHILD HEALTH 22:5

190

SYMPOSIUM: SOCIAL PAEDIATRICS

When attachment is a central issue, for example in safeguarding, the task of understanding childrenâ&#x20AC;&#x2122;s attachments is one of constructing a jigsaw of pieces of varying clarity (Table 3): what is known (or can be assumed) of parental foundations of attachment perinatal and postnatal risks the childâ&#x20AC;&#x2122;s experiences at home, of moves and in other placements the childâ&#x20AC;&#x2122;s described current behaviour the childâ&#x20AC;&#x2122;s observed use of relationships. Consequences of attachment difficulty frequently merge with difficulties related to their cause (e.g. intrauterine exposure to drugs), and consequences of associated abuse. Simple strategies may be all that is required to help attachment. However, for many, broad-based, often multidisciplinary work is needed, addressing remediable factors relating to the

Manifestations of suboptimal attachment Presenting problems Postnatal depression; infant crying/â&#x20AC;&#x2DC;colicâ&#x20AC;&#x2122;; feeding problems; sleep problems; poor eye contact Failure to thrive; poor head growth Delayed verbal and non-verbal communication; generalized developmental delay Injury (accidental/non-accidental) Behaviour problems; ADHD; autism; temper; school exclusion; running away Soiling, wetting, over-eating, pica Adolescent substance abuse, lawlessness, risk taking, promiscuity, pregnancy Depression, eating disorder, obsessive compulsive disorder History Failure to seek closeness, â&#x20AC;&#x2DC;cuddle inâ&#x20AC;&#x2122;, respond to physical affection Indiscriminate friendliness to, or seeking affection from strangers Seeking attention more, or less, than usual; demanding or disruptive behaviour if needing to share attention Poor emotional awareness:

Basics of helping attachment difficulty Building attachment Facilitate attunement by: Parental self-care: rest, relaxation, recharging time, minimizing practical demand One-to-one time Calm closeness: massage, stroking Shared fun Talking time: activities allowing but not demanding conversation (e.g. dog walking) Explanation to parents of the process of attachment, the basis of the childâ&#x20AC;&#x2122;s behaviour etc Facilitate emotional safety

expressed feelings inappropriate to circumstances in quality and extent; excessive tantrums; â&#x20AC;&#x2DC;artificialâ&#x20AC;&#x2122; feelings C lack of empathy C lack of awareness of words for feelings C difficulty in understanding facial expression Inability to recognize body signals (e.g. hunger, satiety, temperature, full bladder, pain) Lack of sense of danger Running off in public places without looking back Difficulty in initiating and sustaining peer relationships; controlling behaviour with other children Difficulty in handling change in routine, unexpected events etc; â&#x20AC;&#x2DC;clinging to samenessâ&#x20AC;&#x2122; C

Routine Clear, consistent boundaries and consequences Preparation for change in routine Structure the day to enable success

Observations

Anticipate and address secondary problems

Parents: poorly attuned; unmodulated voice; little participation in the childâ&#x20AC;&#x2122;s play; rough handling Poor eye contact; excessive eye contact Not seeking as much attention, affection, comfort, reassurance or help from parents as would be expected Not calmed by, or relaxing into cuddles Seeking attention excessively; clinginess; difficulty in sharing attention; â&#x20AC;&#x2DC;in your faceâ&#x20AC;&#x2122;; talking excessively Lack of stranger awareness (>6 months); over-friendly to strangers Running off without looking to parents Difficulty relinquishing control â&#x20AC;&#x2DC;Artificialâ&#x20AC;&#x2122; feelings Poor regulation of stress and emotion; temper Excessive anxiety Lack of affection in pretend play Poor symbolic and role play Poor concentration

Teach through play, stories, role model: Stranger safety Temper regulation C Social skills e negotiation, conflict resolution C Awareness of emotion and body signals C Non-verbal communication Identify and develop areas of ability; address remediable developmental difficulty Manage attention deficit hyperactivity disorder C C

Building resilience Normalize feelings and difficulty by generalizing, stories, 3rd party approach Teach and model calming, stress-release and coping strategies Encourage expression through creativity Build self-esteem Develop a coherent life story Provide a mentor/trusted adult

Table 3

PAEDIATRICS AND CHILD HEALTH 22:5

Table 4

191

SYMPOSIUM: SOCIAL PAEDIATRICS

Gillespie CF, Nemeroff CB. Corticotropin-releasing factor and the psychobiology of early-life stress. Curr Dir Psychol Sci 2007; 16: 85e9. Maunder RG, Hunter JJ. Attachment and psychosomatic medicine: developmental contributions to stress and disease. Psychosom Med 2001; 63: 556e67. Perry BD. Childhood experience and the expression of genetic potential: what childhood neglect tells us about nature and nurture. Brain Mind 2002; 3: 79e100. Rees CA. Thinking about children’s attachments. Arch Dis Child 2005; 90: 1058e65. Rees CA. Understanding emotional abuse. Arch Dis Child 2010; 95: 59e67. Rees CA. All they need is love? Helping children to recover from neglect and abuse. Arch Dis Child 2011; 96: 969e76. Schore AN. Back to basics. Attachment, affect regulation, and the developing right brain. Linking developmental neuroscience to paediatrics. Pediatr Rev 2005; 26: 204e17. Stern DN. The first relationship. Cambridge, MA: Harvard University Press, 1977.

parents, child and environment. Parents need support in managing behaviour; and encouraging social and communication skills, emotional awareness, stress regulation and resilience (Table 4). Paediatricians must be equipped to work to the edge of mental health services, referring children and families to more specialized services as necessary. The multifaceted nature of work with attachment makes services vulnerable if financial constraints encourage evidence to become a rationing tool. Crucial outcomes can be a generation and more away and difficult to quantify. Resource allocation should reflect the far-reaching and costly implications of inadequate early attachment and the false economy of short-term saving. Enabling children to achieve relationships of sufficient quality to equip them for independence, secure adult relationships and safe parenting should be a fundamental priority of paediatrics. A

FURTHER READING Anda RF, Felitti VJ, Bremner JD, et al. The enduring effects of abuse and related adverse experiences in childhood: a convergence of evidence from neurobiology and epidemiology. Eur Arch Psychiatry Clin Neurosci 2006; 256: 174e86. Cairns K. Attachment, trauma and resilience. London: BAAF, 2002. Davies KA, Macfarlane GJ, McBeth J, Morriss R, Dickens C. Insecure attachment style is associated with chronic widespread pain. Pain 2009 June; 143: 200e5. De Bellis MD. The psychobiology of neglect. Child Maltreat 2005; 10: 150. Fahlberg V. A child’s journey through placement. London: BAAF, 1994. Felitti VJ. Relationship of childhood abuse and household dysfunction to many of the leading causes of death in adults. The adverse childhood experiences (ACE) study. Am J Prev Med 1998; 14: 245e58. Gerhardt S. Why love matters: how affection shapes a baby’s brain. Hove: Brunner-Routledge, 2004.

PAEDIATRICS AND CHILD HEALTH 22:5

Practice points C

192

Protecting parental attachment is a responsibility which should be integral to paediatric practice because of its importance for all aspects of children’s wellbeing Work with attachment need not be complex to be useful: factors which help and hinder closeness in any relationship are equally relevant to parent-child attachment Simple observation goes far in indicating when the quality of children’s parental attachments warrants more detailed assessment Attachment is a central consideration in the assessment of abuse or neglect

SYMPOSIUM: SOCIAL PAEDIATRICS

Community care of children with complex health needs

Learning objectives C

Douglas E Simkiss C

Abstract Children with complex health needs include children with disabilities, children with assisted technology and children with palliative care needs. Increasingly care for these children happens outside of hospital. Key issues are effective assessment without duplication and coordinated care involving multi-disciplinary and inter-agency teams. Joint commissioning, key working and integrated care pathways are important developments which, with a focus on listening to children and their families as they express their needs, are resulting in improved care. An understanding of the role of other practitioners and agencies is an essential component to good care for these children.

Other key policy initiatives include the Every Child Matters: change for children programme (2003) which aims to improve and integrate childrenâ&#x20AC;&#x2122;s services and Together from the Start which proposes â&#x20AC;&#x2DC;an identified worker or agency who would work with, advice, be a source of information and support to the family, facilitate more joined up working and alleviate some of the stress for familiesâ&#x20AC;&#x2122;. In 2008 the Department of Health published Better care: Better lives, a strategy for children with life-limiting and lifethreatening conditions including disabled children. The drive to improve care for children with complex health needs has seen ÂŁ340 million in NHS allocations between 2008 and 2011 focussed on improving access to short breaks, community equipment, wheelchairs and childrenâ&#x20AC;&#x2122;s palliative care.

Keywords complex health needs; disabled children; life-limiting conditions; life-threatening conditions; palliative care

Introduction Historically paediatrics, like other clinical disciplines, has focussed on curative medicine. A professional emphasis on the care of children with long term disability, including children who are expected to die, has developed more recently. In 1976 the Committee on Child Health Services, chaired by Professor Donald Court, published â&#x20AC;&#x2DC;Fit For the Futureâ&#x20AC;&#x2122; a far-sighted report which proposed a move from care of children in hospital to care in the community. Many of the recommendations from â&#x20AC;&#x2DC;Fit for the Futureâ&#x20AC;&#x2122; were not initially welcomed by the medical profession, but over the decades since, almost all of them have been implemented. The report is still worth reading today. However, community care for children with complex health needs is still far from all that it could be and new recommendations emerge to strengthen the capacity of community services to meet the demand for care. Standard 8 in the National Service Framework for Children, Young People and Maternity Services is about Disabled children and young people and those with complex health needs. The document says that these children should receive treatment, care and support that match their needs, coordinated by the NHS and other agencies and which helps them and their families live as ordinary lives as possible. The standard has five themes: Services promote inclusion for disabled children Services for disabled children are easy to use Services are child-centred Services support and strengthen families Partnership with children and their parents or carers, and other agencies

Which children have complex health needs? One of the issues in this topic is definitions. There is no universally agreed definition of children with complex health needs but it includes three overlapping groups; children with disabilities, children with assisted technology and children with palliative care needs. Clearly, an individual child could simultaneously, or over a period of time, be in two or all three of these groups. One good working definition is â&#x20AC;&#x2DC;children with on-going healthcare needs related to the use of medical technology and who require the support of a range of professionals and agenciesâ&#x20AC;&#x2122;. The medical technology includes home ventilation, tracheostomy and home oxygen, nasogastric feeding or gastrostomy with enteral nutrition, parenteral nutrition, ambulatory peritoneal dialysis and home renal dialysis. The list continues to expand because of technological advances in diagnosis, suitable equipment for use at home and improved outcomes from critical illnesses. But issues with agreement to funding, equipment costs, organizing home care support, respite and education continue to commonly cause delay in discharge planning from hospital. The emphasis on multi-disciplinary teams and inter-agency working is important and the government guidance is intended to encourage health, education and social care to collectively commission and provide services for children whose needs are greater than the provision available in one service. The Children Act 2004 required every local authority to appoint a commissioner of childrenâ&#x20AC;&#x2122;s services and a board (Childrenâ&#x20AC;&#x2122;s Trusts) to coordinate and commission local childrenâ&#x20AC;&#x2122;s services. Other terms that need defining are â&#x20AC;&#x2DC;life-limiting conditionsâ&#x20AC;&#x2122; e diseases where there is no reasonable hope of cure and from

Douglas E Simkiss FRCP (Ed) FRCPCH FHEA is Associate Professor of Child Health in the Division of Mental Health and Wellbeing, Warwick Medical School, University of Warwick, Coventry, UK. Conflict of interest: none.

PAEDIATRICS AND CHILD HEALTH 22:5

â&#x20AC;&#x2DC;children with complex health needsâ&#x20AC;&#x2122; is an imprecise term but includes children with disability, life-threatening and life-limiting conditions there is a network of support for these children and their families outside of hospital across the health, education, social work and voluntary sectors. To work best, this support has to be coordinated by a key worker listening to the wishes of the child and family integrated care pathways are a tool to facilitate care for these children and their families

193

Ă&#x201C; 2011 Published by Elsevier Ltd.

SYMPOSIUM: SOCIAL PAEDIATRICS

which children or young people will die and â&#x20AC;&#x2DC;life-threatening conditionsâ&#x20AC;&#x2122; in which curative treatment may be feasible but can fail, such as cancer. Children with these conditions often have complex healthcare needs.

ED NE NT AL

LO PM E

Stimulation

â&#x20AC;&#x2122;S

CHILD Safeguarding and promoting welfare

ILD

Guidance and boundaries

Selfcare skills

Emotional warmth

Social presentation

Identity Family and social relationships

Because of the imprecise definition, it is perhaps not surprising that there are not clear statistics on the number of children with complex care needs. The Lifetime project in Bath defined a non malignant life-threatening or life-limiting condition as â&#x20AC;&#x2DC;any condition developed in childhood (before the age of 19 years) whereby the child is likely to die prematurely (before the age of 40 years), or any condition developed in childhood that, without major intervention, will cause the child to die prematurelyâ&#x20AC;&#x2122;. Using this definition, there was a prevalence of 1.28/1000 (0e19 year olds) in that district and a prevalence of malignant disease under treatment of 0.65/1000 children and young people. This is an important group of children in need.

Epidemiology

Ensuring safety PA

Emotional and behavioural development

Basic care

Health Education

Stability

FAMILY AND ENVIRONMENTAL FACTORS y or ng s t ni hi t io il y c m f un Fa nd ly a mi fa er id W g in u s ent Ho m oy pl Em e l ia m oc co In â&#x20AC;&#x2122;s s n o il y t i m ra Fa teg in i t y un m es m urc Co s o re

Needs based assessments and care coordination

Figure 1 The common assessment framework.

In addition to having to cope with dramatic changes in their expectation and lifestyles, parents of children with complex health needs have to learn how to access the necessary services for the child and themselves. This can be very confusing. Indeed one of the frequent complaints of families in this situation is how difficult it is to know what support is available and how to get it. They may be known to many services including health, social services, social security and education and aspects of their needs may be assessed many times but families can be left waiting too long for services they desperately need. A strategy to address repeated assessment and uncoordinated care is to use a common assessment framework and nominate a single person with the role of coordinating care. In the UK this is called a key worker; in North America â&#x20AC;&#x2DC;service coordinatorâ&#x20AC;&#x2122; is used. The key tasks of this role are: Providing information and advice. Families want this to be relevant and timely at significant points in their childâ&#x20AC;&#x2122;s life and development Identifying and addressing needs. In the UK the Framework for the Assessment of Children in Need was developed to identify needs and is a useful starting point (see Figure 1) Accessing and coordinating services for the family Providing emotional support Acting as an advocate The National Service Framework summarized the evidence on key working as â&#x20AC;&#x2DC;studies of key workers consistently report positive effects on relationships with services, fewer unmet needs and greater family wellbeingâ&#x20AC;&#x2122; and over time more key working projects have emerged across the country. A second important development in care coordination for children with complex health needs are Integrated Care pathways. The Association for Childrenâ&#x20AC;&#x2122;s Palliative Care (ACT) has produced care pathway templates including a neonatal pathway for babies with palliative care needs, integrated multi-agency care pathways for children with life-threatening and life-limiting conditions, a family companion to the ACT Care Pathway and a Transition Care Pathway.

The Neonatal Pathway for Babies with Palliative Care Needs has been developed primarily to help support professionals working in maternity, obstetric and neonatal units who care for babies with life-threatening or life-limiting conditions. It aims to encourage practitioners and commissioners to ensure that the appropriate care is available in the right place, at the right time for each of these babies. The Integrated Multi-agency Care Pathways for Children with life-threatening and life-limiting conditions provides a framework to develop dedicated pathways for children with palliative care needs. It identifies the common needs families have caring for a child with a life-limiting condition and presents a pathway to engage with these needs. It will help ensure that all the necessary people and equipment are in place, so that families can access the appropriate support when it is needed. In 2004 a special issue of Child: care, health and development was dedicated to supporting children with complex health needs in the community. The issue contains an article written by the parents of Andrew, a boy who died from Neimann Pick Disease, which describes their personal experience and what families want from services. ACT has developed a family companion to guide families through the complex and often confusing processes that take place following diagnosis. The Family Companion explains what they should expect at each of the five key stages of the ACT Care Pathway: Breaking news Planning for going home Multi-agency assessment of a familyâ&#x20AC;&#x2122;s needs A multi-agency care plan End of life plan The range of people who may be involved with a child with complex health needs in the community is illustrated in Figure 2. This emphasizes the importance of a key worker. A new classification system called the International Classification of Functioning, Disability and Health (Children and Youth Version) or ICFCY was published by the World Health Organization in 2007. This conceptualizes disability and health as

PAEDIATRICS AND CHILD HEALTH 22:5

194

Ă&#x201C; 2011 Published by Elsevier Ltd.

SYMPOSIUM: SOCIAL PAEDIATRICS

Figure 2 Key services in the team around the child.

dynamic processes and bridges the social and medical models of disability in a biopsychosocial model. ICFCY has three domains; body structure and function, activity and participation and research has demonstrated that interventions for children with cerebral palsy that promote participation are as effective in promoting wellbeing as medical interventions to correct the body structure or function problems. This tool will change the way we develop services for children with complex health needs.

educational needs which funds either their special school placement or provides financial support to a mainstream school to meet the child’s needs. This budget to the school is usually spent on teaching assistants who work with the child for some or all of the school day. Some children have residential school placements and for others home schooling is provided. This is another complex process that families can find frustrating. Sometimes moving to an assessment of special educational needs needs evidence of the impact of interventions offered by the school at school action and other agencies (such as speech and language therapy or educational psychology) at school action plus and so time is necessarily spent at these earlier stages, though if a child’s situation has changed quickly these processes can be bypassed. There is support for families from Parent Partnership, a service provided by each local authority area but delivered by families of children with special educational needs.

Education advice There is a universal entitlement to education and the government has a policy of inclusion meaning that some children with complex health needs are included into mainstream school. The Disability Discrimination Act applies to educational establishments and adaptations should be provided to facilitate access for these children. Parents usually have a choice of educational provision and special schools are available for children with a variety of needs including sensory impairments, physical disabilities and severe and profound learning difficulties. The government is consulting on changes to special educational needs provision at present, but currently there is a staged response to children’s educational needs; school action, school action plus, assessment of special educational needs and provision of a statement of special educational needs. Most children with complex health needs will have a statement of special

PAEDIATRICS AND CHILD HEALTH 22:5

Social support It is clear that having a child with complex health needs may bring financial costs; many parents are not able to combine caring for their child with paid employment and outgoings may be increased by travel to appointments and purchasing equipment. Social support of families who are carers struggles to keep up with medical advances and the benefit system does not fully compensate parents for loss of income; families should be

195

Ó 2011 Published by Elsevier Ltd.

SYMPOSIUM: SOCIAL PAEDIATRICS

encouraged to apply for benefits, many do not know they are entitled to the support. Disability Living Allowance is a tax-free benefit for disabled children to help with extra costs. Children are entitled if they have a physical or mental disability and needs a lot more help or supervision than other children of the same age. Parents can claim for care needs before a child is aged 3 months but benefit will not be paid before 3 months unless the child is terminally ill. Mobility needs can be claimed from 3 years of age if the child is unable, or virtually unable, to walk or would be at risk if they tried to walk. The Family Fund was established by the UK Government in 1973 to give practical help to families with severely disabled children under the age of 16. It is the largest independent grant-giving organization for families raising children with complex health needs. Families often apply for essential items such as washing machines, but young people aged 17 and under can be helped with grants for other needs such as equipment for college. In 2009e10, the Fund distributed ÂŁ32.8 million to 55,000 families. Direct payments give families a budget to arrange care and services themselves. After an assessment, direct payments can be used to purchase services including; short breaks, specialist nursery placements or personal care. Being a parent and carer is not a routine nine-to-five, 5 days a week job but often 24 h a day every day. This on-going demand can lead to social isolation and prevents parents joining in â&#x20AC;&#x2DC;normalâ&#x20AC;&#x2122; life. Access to respite or holidays is an important part of caring for the carers; there is insufficient respite care to meet demand. It comes in different forms; Short breaks provided by family and friends e viewed as flexible but parents worry about imposing on relatives. Access tends to diminish as children grow up. Short breaks provided by an agency (usually in the home) e allows parents to attend to household chores, shopping and socializing with the other children or partner. Overnight in home breaks e allows parents to have a break and the child to remain at home, prevents child from feeling like a burden. Respite facility placement e enables parents to rest and recover from the pressure of care and allows the rest of the family to engage in activities together.

The wish list of a young person with complex health needs

Box 1

moving to adulthood and independence. The transition care pathway developed by ACT provides a framework to help young people, families and professionals prepare in a carefully considered way for adult services. It emphasizes the importance of empowering young people to take control of their lives, and describes the need for an early collaboration of paediatric and adult services to improve transition. It includes a clear template; modelled around six standards, to guide through the transition process.

Interventions

Summary

There is a wide range of specific medical interventions that may benefit children with different complex health needs and this article does not describe these. Palliative care is a growing and important service for some children with complex health needs. There are e-learning modules based on the RCPCH curriculum that aim to equip practitioners and complement existing knowledge and skills in this area.

Real progress has been made in the care of children with complex health needs but much still needs to be done. Key working and coordinated and timely care is vital. Joint commissioning and care pathways are important developments. It is of first importance to listen to the children and families themselves to identify their priorities and needs. The wish list of one young person with complex health needs is shown in Box 1 and would be the best place to start to meet his needs. A

Transition Young people with complex health needs including many supported by medical technologies will survive into adulthood. They face many transitions, some occurring concurrently. As well as moving from childhood to adulthood, there are organizational and illness transitions and the transition from parental care to self care. It is clear that too often transition is focussed on service transition and transfer alone and not seen as part of the holistic process of

PAEDIATRICS AND CHILD HEALTH 22:5

FURTHER READING Association for Childrenâ&#x20AC;&#x2122;s Palliative Care (ACT) e http://www.act.org.uk/. Committee on Child Health Services. Fit for the future. London: HMSO, 1976. Department of Education and Skills and Department for Health. Together from the start e practical guidance for professional working with

196

Ă&#x201C; 2011 Published by Elsevier Ltd.

SYMPOSIUM: SOCIAL PAEDIATRICS

disabled children (birth to 3rd birthday) and their families. London, UK: DfES and DoH, 2003. Department of Health. National service framework for children, young people and maternity services: disabled children and young people and those with complex health needs. London, UK: DoH, 2004. Department of Health. Better care: better lives. London, UK: DoH, 2008. Fauconnier J, Dickinson HO, Beckung E, et al. Participation in life situations of 8e12 year old children with cerebral palsy: cross sectional European study. Br Med J 2009; 338: b1458. HM Government. Every child matters, change for children. London, UK: HM Government, 2004. HM Treasury and Department for Education and Skills. Aiming high for disabled children: better support for families. London, UK: HM Treasury and DfES, 2007. Jardine E, Wallis C. Core guidelines for the discharge home of the child on long term ventilation in the United Kingdom. Thorax 1998; 53: 763e7.

PAEDIATRICS AND CHILD HEALTH 22:5

Kirk S. Transitions in the lives of young people with complex healthcare needs. Child Care Health Dev 2008; 34: 567e75. Lewis M. Establishing a service: a whole population approach. Child Care Health Dev 2004; 30: 221e9. Margolan H, Fraser J, Lenton S. Parental experience of services when their child requires long term ventilation. Implications for commissioning and providing services. Child Care Health Dev 2004; 30: 257e64. NHS West Midlands e-learning resource on paediatric palliative care e http://westmidlandspalliative.westmidlands.nhs.uk/. The NHS Confederation. Aiming high for disabled children: delivering improved health services. London, UK: The NHS Confederation, 2009. World Health Organisation. International classification of functioning, disability and health e children and youth. World Health Organisation, http://www.who.int/classifications/icf/en/; 2007. Contains public sector information licensed under the Open Government Licence v1.0.

197

Ă&#x201C; 2011 Published by Elsevier Ltd.

SYMPOSIUM: SOCIAL PAEDIATRICS

New insights into air pollution and children’s health

impairment of brain function is unclear. Indeed, the possibility of a confounding variable (e.g. noise) cannot be excluded. However, there are proof-of-concept studies suggesting that airway PM affects brain function. For example, Yokota et al instilled diesel exhaust PM into the nose of 2-week old infant rats once a week for 4 weeks and found that PM-treated animals showed a lower avoidance performance than control animals given sham-instillation. Furthermore, there was a trend for levels of dopamine in the medial mammillary nucleus of the brain to be lower in the PMtreated animals. Histological examination of the brain showed no evidence that PM had penetrated into the nervous system e suggesting that this effect was mediated by indirect mechanisms. The effect of maternal inhalation of traffic-derived pollution on the developing fetus has been identified in recent studies with sufficient power to detect independent effects. In a study of over 70,000 singleton births in Canada, Brauer et al estimated exposure to air pollution at mothers’ residence and found that living within 50 m of a main road was associated with a 22% increase in low-birth weight. When exposure to air pollutants was modelled individually, all air pollutants other than ozone, were associated with small for gestational age. An effect of air pollution on preterm birth was also found e albeit only for PM2.5. Similarly in a US study, Wihelm and Ritz reported an association between modelled exposure of mothers to traffic-derived PM- and risk for preterm birth and term low-birth weight. How PM impacting in the lungs of mothers could influence the developing fetus is unclear. A possible mechanism is that PM alters the pattern of cytokines in the maternal and fetal circulation. Some evidence for this phenomenon was recently provided by Latzin et al who studied a birth cohort of 265 healthy term-born neonates and found that mean PM10 over the last 3 months of pregnancy was associated with increased interleukin-1 beta in cord blood. In summary, although the evidence base is limited these epidemiological associations are of concern and establishing biological plausibility in animal models is urgently needed.

Jonathan Grigg

Abstract Air pollution remains a major threat to children’s health. In high-income countries, most outdoor air pollution is from fossil fuel combustion, and most indoor pollution is from cooking and environmental tobacco smoke (ETS). Outdoor pollution in medium- and low-income countries is a mix of fossil-fuel, solid fuel (e.g. coal) and biomass (wood), and indoor pollution is from biomass smoke, solid fuels and ETS. Over the last decade, new data suggest that both biomass smoke and ETS increases the vulnerability of children to bacterial pneumonia, and that fossil-fuel and biomass smoke impair children’s neurocognitive development. Further research is needed to establish biological plausibility for these associations.

Keywords air pollution; biomass; children; environmental tobacco smoke; fossil-fuel; neurocognitive impairment; pneumonia

Children are especially vulnerable to the adverse effects of environmental pollution. Pollutants are either inhaled, or ingested, or absorbed through the skin. There are many potential environmental threats to children’s health, and for most the evidence base is unclear. For example, teasing out the independent effect of a single chemical amongst thousands of compounds is nearly impossible. For chemicals, regulatory agencies must therefore take a precautionary approach i.e. to not wait for definitive proof to protect children’s health. One area where causal association, is certain, yet exposure of children still occurs, is air pollution. A previous review in this journal of the effects of traffic-derived air pollution, focused on inhalable particulate matter (PM less than 10 microns in aerodynamic diameter; PM10), and addressed lung growth and prevalence of respiratory symptoms. The aim of this review is to focus on new insights into the adverse effects of air pollution, both indoor and outdoor, on children’s health.

Indoor air pollution Indoor air pollutants either penetrate into the home from the outside or are generated within the home. In high-income countries, penetrating indoor pollution is mainly from trafficderived emissions. Thus both PM and vehicular-emitted gases such as nitrogen dioxide (NO2) are higher in homes near main roads than those near to less heavily used roads. For example, Esplugues et al found higher concentrations of NO2 in homes located on streets with a high frequency of vehicle traffic. The major sources of indoor-generated air pollutants are cooking and parental smoking e and both sources produce PM and NO2. PM in homes with smokers is up to 15 mg/m3 higher than nonsmoking homes. Although there is substantial body of evidence showing that exposure of children to passive environmental tobacco smoke (ETS) is associated with increased respiratory symptom prevalence e including cough and wheeze, there has always been the question whether exposure of children to secondhand smoke causes asthma. Strong evidence for a causal link between ETS and asthma has recently been provided by a systematic review of data from eight cohort studies. This metaanalysis showed that the adjusted predicted relative risk for a secondhand smoke effect on incident asthma is 1.33 (95% CI, 1.14e1.56) e an association strongest in school-age children.

Traffic-derived air pollution Recent studies suggest that inhalation of traffic-derived pollutants has non-respiratory effects on the developing nervous system. Franco Suglia et al, estimated the exposure of 202 school-age children to black carbon (i.e. traffic-derived soot) at the home address, and found that black carbon was independently associated with decreases in a range of neurocognitive variables such as vocabulary, and composite intelligence quotient. To date, the mechanism that links deposition of black carbon PM in the lung to

Jonathan Grigg MD is Professor of Paediatric Respiratory and Environmental Medicine at the Centre for Paediatrics in the Blizard Institute, Barts and the London School of Medicine, London, UK. Conflict of interest: none.

PAEDIATRICS AND CHILD HEALTH 22:5

198

SYMPOSIUM: SOCIAL PAEDIATRICS

The effect of maternal ETS exposure on perinatal outcomes is less obvious. In a recent systematic review and meta-analysis, Salmasi et al identified publications covering 48,439 ETSexposed women and 90,918 unexposed women and found that ETS-exposed infants weigh less, with a trend towards increased low-birth weight. Using a similar approach, Leonardi-Bee et al reported that exposure of non-smoking pregnant women to ETS reduces mean birth weight by at least 33 g, and increases the risk of birth weight below 2500 g by 22%. Since both ETS and trafficderived emissions have components in common, such as carbonaceous PM, these data also provide indirect support for an association between traffic-derived PM and birth weight (discussed above). Another important area on the global scale is the interaction between ETS and vulnerability to infection. In a recent metaanalysis Lee et al assessed the association between ETS and invasive bacterial disease in children. They found a consistent association between invasive meningococcal and Hib disease with the adjusted odds ratio (OR) for meningococcal disease of 1.2 (1.5e2.6). For invasive pneumococcal disease the OR was positive, but not significant. An explanation for this non significant finding is that pneumococcal pneumonia, although the major cause of bacterial pneumonia in children is frequently associated with negative blood cultures. The strongest evidence that ETS is a major vulnerability factor for pneumococcal pneumonia was provided by a study from Vietnam. Suzuki et al assessed hospital admissions for pneumonia among children aged less than 5 years in the previous 12 months in a populationbased cross-sectional survey that included all residents of 33 in a central area of Vietnam. Exposure to ETS was associated with hospital admissions for pneumonia (OR 1.55, 95% CI, 1.25 e1.92). This OR may seem relatively low, but since the prevalence of ETS in Vietnam was 70%, it was estimated that 28% of childhood pneumonia in this community is attributable to ETS with 44,000 excess hospital admissions in young children per year. Biological plausibility for this association was provided by Phipps et al who exposed mice to cigarette smoke for 5 weeks then instilled S. pneumoniae into the airway. Smoke-exposed mice had increased lung bacterial load at both 24 and 48 h after infection, and more clinical illness. Even more important on the global scale, is the association between exposure to biomass and coal smoke (e.g. wood, coal) and infection. Dherani et al in a meta-analysis and systematic review found an overall pooled OR of 1.78 (95% CI, 1.45e2.18) for exposure of children to solid fuel smoke and risk of pneumonia in young children. It is now widely accepted by policy makers that reduction of household indoor air pollution from solid fuels would represent a major contribution to the prevention deaths in young children living in low-income countries. Indeed, these data have contributed to the realization than indoor air pollution is a major environmental cause of death for both women and young children. In response to this, the United Nations Foundation launched the Global Alliance for Clean Cookstoves e which aims for 100 million homes to adopt clean stoves by 2020. In 2011, Hillary Clinton made this initiative a major part of her Global Partnerships Initiative. Since both ETS and indoor solid fuel pollution are associated with pneumonia in young children e could traffic-derived PM also increase vulnerability to infection? Bacterial pneumonia is

PAEDIATRICS AND CHILD HEALTH 22:5

relatively uncommon in high-income countries as are, fortunately, deaths. Since it is difficult to perform adequately powered epidemiological studies into air pollution and infection in high-income countries, studies suggesting biological plausibility become more important. For example, my group recently developed a model for the effect of PM on the adherence of pneumococci to lower airway epithelial cells (Figure 1). Adherence of bacteria to airway lining cells is a prerequisite step for establishing invasive disease, and environmental factors that upregulate adhesion are putative vulnerability factors for bacterial pneumonia. We found that both PM10 from a UK city (where most pollution is from traffic), and from Accra (a city with a mix of traffic-derived and solid fuel PM10), up-regulated pneumococcal adhesion to lower airway epithelial cells in vitro. Furthermore, we found that the mechanism for both fossil-fuel PM and mixed source PM was the same, i.e. upregulation of the ligand for pneumococcal adhesion on the surface of epithelial cells e the platelet activating factor receptor (PAFR). PAFR, by chance, happens to express proteins that interact with proteins in the pneumococcal cell wall. Pneumococci therefore adhere to this receptor e and are then moved into the cell as the receptor is internalized. In vitro studies comparing the effects of ETS, biomass PM and traffic-derived PM may therefore help to clarify whether there are common mechanisms underlying the association between air pollution and bacterial infection. From our data, we hypothesize that children exposed to high levels of air pollution (biomass, fossil-fuel and ETS) constitutively express increased levels of PAFR in their lower airway epithelial cells. By linking in vitro observations to valid markers of vulnerability in vivo (such as PAFR expression) it may be possible to generate sufficient data for policy makers to act on the presumption that traffic-derived PM increases pneumonia vulnerability without waiting for expensive and difficult-to-perform epidemiological studies.

Figure 1 Pneumococci adhering to a monolayer of alveolar epithelial cells imaged by scanning electron microscopy. Bacteria are bead-like structures. Adherence is mediated by epithelial platelet activating factor receptor interacting with ligands in the bacterial cell wall.

199

SYMPOSIUM: SOCIAL PAEDIATRICS

carbon monoxide, a marker for exposure to woodsmoke. Neurotoxicology 2011. Sep 24th [Epub ahead of print]. Esplugues A, Ballester F, Estarlich M, et al. Indoor and outdoor concentrations and determinants of NO2 in a cohort of 1-year-old children in Valencia, Spain. Indoor Air 2010; 20: 213e23. Kulkarni N, Grigg J. Air pollution and children’s health. Paediatric child health 2008; 18: 287e91. Latzin P, Frey U, Armann J, et al. Exposure to moderate air pollution during late pregnancy and cord blood cytokine secretion in healthy neonates. PLoS One 2011; 6: e23130. Lee CC, Middaugh NA, Howie SR, Ezzati M. Association of secondhand smoke exposure with pediatric invasive bacterial disease and bacterial carriage: a systematic review and meta-analysis. PLoS Med 2010; 7: e1000374. Leonardi-Bee J, Smyth A, Britton J, Coleman T. Environmental tobacco smoke and fetal health: systematic review and meta-analysis. Arch Dis Child Fetal Neonatal Ed 2008; 93: F351e61. Martin 2nd WJ, Glass RI, Balbus JM, Collins FS. Public health. A major environmental cause of death. Science 2011; 334: 180e1. Mushtaq N, Ezzati M, Hall L, et al. Adhesion of Streptococcus pneumoniae to human airway epithelial cells exposed to urban particulate matter. J Allergy Clin Immunol 2011; 127: 1236e12422. Phipps JC, Aronoff DM, Curtis JL, Goel D, O’Brien E, Mancuso P. Cigarette smoke exposure impairs pulmonary bacterial clearance and alveolar macrophage complement-mediated phagocytosis of Streptococcus pneumoniae. Infect Immun 2010; 78: 1214e20. Salmasi G, Grady R, Jones J, McDonald SD. Environmental tobacco smoke exposure and perinatal outcomes: a systematic review and meta-analyses. Acta Obstet Gynecol Scand 2010; 89: 423e41. Suglia SF, Gryparis A, Wright RO, Schwartz J, Wright RJ. Association of black carbon with cognition among children in a prospective birth cohort study. Am J Epidemiol 2008; 167: 280e6. Suzuki M, Thiem VD, Yanai H, et al. Association of environmental tobacco smoking exposure with an increased risk of hospital admissions for pneumonia in children under 5 years of age in Vietnam. Thorax 2009; 64: 484e9. Vork KL, Broadwin RL, Blaisdell RJ. Developing asthma in childhood from exposure to secondhand tobacco smoke: insights from a meta-regression. Environ Health Perspect 2007; 115: 1394e400. Wilhelm M, Ritz B. Local variations in CO and particulate air pollution and adverse birth outcomes in Los Angeles County, California, USA. Environ Health Perspect 2005; 113: 1212e21. Yokota S, Takashima H, Ohta R, et al. Nasal instillation of nanoparticlerich diesel exhaust particles slightly affects emotional behavior and learning capability in rats. J Toxicol Sci 2011; 36: 267e76.

Discovering common mechanisms underlying the effects of air pollution from different sources is a major research challenge for the next decade. For example, does the association between trafficassociated black carbon and neurocognitive effects in children mean that wood smoke is also associated with effects on the brain? Some evidence for this was provided by the Randomized Exposure Study of Pollution Indoors and Respiratory Effects (RESPIRE) stove intervention trial in San Marcos, Guatemala e one of the most important studies in the field of indoor air pollution. As part of this stove intervention study, Dix-Cooper et al estimated early life exposure to solid fuel smoke using carbon monoxide (CO) as marker of exposure. The advantage of CO is that it can be measured by passive monitoring e a method that is both cheap and portable. During the trial, personal CO measures were collected every 3 months from pregnant mothers. Children underwent neurodevelopmental assessment when they reached 6e7 years of age. The study showed an inverse association between personal CO exposure of mothers during their third trimesters and their child’s neuropsychological performance. Clearly, further research is needed to confirm these data, but the signal for an association between air pollution and neurocognitive function is concerning.

Future research We are just beginning to understand the true burden of air pollution on children’s health. From what we know to date, it is very likely that the health burden of air pollution exceeds the burden from threats from manufactured chemicals such as endocrine disruptors. What is clear is that current environmental policy does not sufficiently protect children from the adverse health effects of air pollution. For example, London consistently exceeds the current EU air quality standards. Questions that still need further basic and epidemiological research include; (i) what is the effect of air pollution (both indoor and outdoor) on viralinfections and viral-triggered bacterial interactions?, (ii) how does individual behaviour influence individual exposure of children e.g. does travelling to school on a heavily used road significantly increase personal exposure to traffic-derived PM10?, (iii) how can we protect families from the effects of carbonneutral biomass smoke (carbon neutral) but not increase carbon emissions (e.g. by switching to gas cooking)? A

FURTHER READING BeruBe KA, Sexton KJ, Jones TP, Moreno T, Anderson S, Richards RJ. The spatial and temporal variations in PM10 mass from six UK homes. Sci Total Environ 2004; 324: 41e53. Brauer M, Lencar C, Tamburic L, Koehoorn M, Demers P, Karr C. A cohort study of traffic-related air pollution impacts on birth outcomes. Environ Health Perspect 2008; 116: 680e6. Dherani M, Pope D, Mascarenhas M, Smith KR, Weber M, Bruce N. Indoor air pollution from unprocessed solid fuel use and pneumonia risk in children aged under five years: a systematic review and meta-analysis. Bull World Health Organ 2008; 86: 390e8. Dix-Cooper L, Eskenazi B, Romero C, Balmes J, Smith KR. Neurodevelopmental performance among school age children in rural Guatemala is associated with prenatal and postnatal exposure to

PAEDIATRICS AND CHILD HEALTH 22:5

Practice points C

200

Exposure of children to air pollution directly affects the lung and more distant organs. Recent data suggest an association between traffic- and biomassderived air pollution and neurocognitive impairment in children. Vulnerability to bacterial infection is increased in children exposed to biomass smoke and to ETS. In vitro studies suggest that pollution-mediated vulnerability to infection is mediated by upregulation of epithelial platelet activating factor receptor.

OCCASIONAL REVIEW

Long-term effects of cancer therapy in children e organs, systems and tissues

later. The wide range of late adverse effects (LAEs) that may occur and their causes, as well as the great variability in timing of onset, severity and complexity, is highlighted in Figure 1. Many contemporary treatment protocols strive to cure children with the least possible late toxicity, but some malignancies still have relatively poorer cure rates, especially in the context of metastatic or relapsed disease, and hence still require aggressive treatment strategies leading to higher rates of LAEs. Long-term follow-up (LTFU) is now acknowledged as an important aspect of care of survivors, and survivorship is recognized as an increasingly important aspect of their future care. The first in this series of two reviews describes some of the commoner and more serious LAEs that may affect specific organs, physiological systems and tissues, including the nervous, sensory, endocrine, reproductive, cardiovascular, respiratory, gastrointestinal/hepatobiliary, urinary tract and musculoskeletal systems, as well as the teeth and skin. The second paper of the series will describe LAEs that affect neuropsychological, metabolic and immunological function, growth, quality of life, and consider the risk of secondary malignant neoplasms. It will also describe the impact of late toxicity in survivors of central nervous system (CNS) tumours and haemopoietic stem cell transplants (HSCTs), and highlight the risks of premature mortality in childhood cancer survivors (CCS). Finally it will emphasize the growing focus on the provision of optimal LTFU and quality of survivorship.

Roderick Skinner

Abstract Almost one in 700 young adults is a survivor of childhood malignancy, but about 60% of long-term survivors have at least one and 30e40% at least two chronic medical problems whilst 30% have a severe, life-threatening or disabling adverse effect. Such late adverse effects have a wide range of presentations and may lead to potentially far-reaching consequences, not only during the important period of childhood and adolescent growth and development but also during future adult life. The first part of this series of two reviews highlights some of the commoner and more serious late effects of treatment for childhood and adolescent malignancy by focussing on toxicity affecting specific organs, physiological systems and tissues, including the nervous, sensory, endocrine, reproductive, cardiovascular, respiratory, gastrointestinal/hepatobiliary, urinary tract and musculoskeletal systems, as well as damage to the teeth and skin.

Keywords cancer; chemotherapy; child; late sequelae; leukaemia; radiotherapy

Late adverse effects affecting specific organs, systems and tissues Nervous system Long-term neurological toxicity is the commonest organ/systemspecific LAE reported in the CCSS, occurring in 27% of survivors, with a relative risk (RR) of 3.3. Survivors of CNS tumours, and to a lesser extent Hodgkin disease (HD) and acute leukaemia (AL), are at the highest risk. Chronic neurological toxicity may be severe and is sometimes progressive. The manifestations of neurological toxicity may include leucoencephalopathy, vasculopathy (from stenosis or occlusion), myelopathy and secondary CNS tumours (e.g. malignant gliomas, neuroectodermal tumours and meningiomas), with a wide range of life-threatening or disabling clinical consequences. In contrast, peripheral nervous system (PNS) toxicity usually presents as peripheral neuropathy. Leucoencephalopathy may present with focal motor signs, spasticity, seizures, ataxia and neuropsychological dysfunction causing functional motor disabilities and cognitive impairment affecting intelligence, attention and learning skills, memory, verbal and visual-spatial skills. Vasculopathy may cause seizures, transient ischaemic attacks, cerebrovascular accidents or dementia. Myelopathy may lead to para- or quadriplegia and sphincter dysfunction. Most neurological toxicity is related to the consequences of CNS tumours or caused by cranial radiotherapy (CRT) and/or CNS-directed chemotherapy (intrathecal chemotherapy, or highdose intravenous methotrexate or cytarabine). Leucoencephalopathy occurs predominantly in survivors of CNS tumours or AL treated with CRT or CNS-directed chemotherapy, and is commoner and more severe in patients treated at a younger age. Other aspects of neurotoxicity are related more to specific

Introduction As a result of the increasing long-term survival rates of children with malignancy, now reaching 75e80% in developed countries, almost one in 700 young adults is a survivor of a childhood malignancy. However, this success has required greater use of multiple treatment modalities, especially multi-agent and often high-dose chemotherapy in addition to radiotherapy and surgery, and has been achieved at the cost of increased chronic toxicity. Several studies have shown that about 60% of long-term survivors have one or more chronic medical problems (as defined by the need for continuing medical intervention or advice, or the presence of the likely future development of disability or dysfunction) whilst 30e40% have two or more. In addition, the Childhood Cancer Survivor Study (CCSS), a large North American cohort study, found that about 30% of survivors had a severe (grade 3) or life-threatening/disabling (grade 4) chronic condition. Such chronic late effects may lead to disturbed growth and development during childhood and adolescence and have important adverse effects on health status years or even decades

Roderick Skinner MB ChB PhD FRCPCH is Consultant and Honorary Clinical Senior Lecturer in Paediatric and Adolescent Oncology/Bone Marrow Transplantation, and Children’s BMT Unit, Great North Children’s Hospital, Royal Victoria Infirmary, Newcastle upon Tyne, UK.

PAEDIATRICS AND CHILD HEALTH 22:5

201

OCCASIONAL REVIEW

Causes • Chemotherapy – glomerular and/or renal tubular impairment due to ifosfamide, cisplatin, carboplatin • Radiotherapy – primary hypothyroidism • Surgery – increased risk of encapsulated bacterial infection following splenectomy • Supportive care drugs – ototoxicity due to aminoglycosides • Multifactorial – haemorrhagic cystitis may be due to either (or both of): – Chemotherapy (ifosfamide or cyclophosphamide) – Radiotherapy – And may be precipitated by GvHD or viral infection in HSCT patients • CNS tumours – late adverse effects may be due to damage caused by: – The tumour itself – Treatment – including any/all of surgery, radiotherapy or chemotherapy – And may be modulated by developmental stage at time of damage • Post-HSCT – numerous factors may be relevant including prior and conditioning chemotherapy/ radiotherapy, chronic GvHD, supportive care drugs

Timing of onset • Early – nephrotoxicity due to ifosfamide (usually during treatment or first year afterwards) • Late – secondary malignancies (may occur decades after treatment)

Late adverse effects may include Organs/systems/tissues • Nervous system – Central nervous system – Peripheral nervous system • Sensory system, especially – Vision – Hearing • Dental/craniofacial • Endocrine system, including – Hypothalamus/pituitary – Thyroid • Reproductive system – Gonads – Uterus • Cardiovascular system – Heart – Vascular • Respiratory system • Gastrointestinal system, including – Hepatobiliary system • Spleen • Urinary tract system – Kidneys – Bladder/lower urinary tract • Skin • Musculoskeletal system – Muscular – Bone, including spine Functions • Neuropsychological • Metabolic (including pancreatic) • Immunological • Growth • Quality of life

Severity • Mild – dental caries following chemotherapy or cranial/facial radiotherapy • Moderate – cataract following radiotherapy to a field including the eye • Severe – pulmonar y fibrosis due to carmustine • Potentially life-threatening – secondary malignancies due to radiotherapy or chemotherapy (especially alkylating agents or epipodophyllotoxins)

Complexity • Simple – bilateral high-frequency hearing impairment due to cisplatin – NB ‘simple’ toxicity may still have severe consequences, eg speech impairment, educational difficulties) • Complex – cardiotoxicity – anthracycline-induced cardiomyopathy → congestive cardiac failure – radiotherapy-induced ‘early onset’ coronary heart disease – more rarely - myocardial damage - valvular disease - pericarditis

Others • Secondary malignancy

Figure 1 Categories of late adverse effects that may occur in long-term survivors of childhood malignancy, with examples to illustrate the wide range of causes and variability in timing of onset, severity and complexity. CNS, central nervous system; GvHD, graft-versus-host disease; HSCT, haemopoietic stem cell transplantation. Adapted with permission from Skinner et al. Arch Dis Child 2007; 92: 257e260.

treatments components, for example vasculopathy is associated with previous CRT, myelopathy with spinal radiotherapy (RT) and peripheral neuropathy with cisplatin or vincristine chemotherapy. General management includes physiotherapy and/or occupational therapy assessment in survivors with physical disabilities, and careful educational/neuropsychological assessment with provision of extra school and employment support in those with leucoencephalopathy. Specific treatment options may include surgery in selected cases of vasculopathy, or drug treatment for painful neuropathy.

(including total body irradiation [TBI] in haematological malignancies) may cause dry eyes due to impaired lacrimal gland tear production and hence predispose to corneal ulceration and scarring. Visual impairment may be due to the underlying disease in survivors of CNS tumours or to posterior subcapsular cataracts related to RT or less commonly prolonged steroid treatment. CCSS studies have reported severe visual impairment in 13% of CNS tumour and 3% of all survivors, whilst up to 30% of HSCT survivors develop cataracts after TBI. Rarer adverse effects include keratitis, uveitis, chorioretinopathy and optic neuritis. Very rarely, chemotherapy may cause severe visual toxicity, e.g. visual loss after high dose fludarabine. Treatment strategies include artificial tear replacement for dry eyes and surgical removal of cataracts (with insertion of a lens implant), but established visual loss is

Sensory system Disorders of hearing, speech and vision were reported in 12% of CCSS survivors (RR 2.5). Any RT field that includes the eye

PAEDIATRICS AND CHILD HEALTH 22:5

202

OCCASIONAL REVIEW

usually irreversible and will require appropriate community and educational support. Chronic hearing loss not corrected by a hearing aid was reported in 2% of CCSS survivors, and any hearing impairment in 12% of CNS tumour survivors. The important consequences of deafness, especially in younger children, include delayed speech development and impaired educational/social functioning. The major cause of ototoxicity is platinum chemotherapy, particularly cisplatin and less commonly high-dose carboplatin. The main risk factors are higher cumulative cisplatin dose and younger age at treatment, with total dose more than 400 mg/m2 and age less than 5 years associated with clinically significant bilateral sensorineural hearing loss in 40% of children. Highdose RT including the middle ear may cause mixed sensorineural and conductive hearing loss, but deafness is uncommon in survivors who have not received platinum treatment. Hearing loss may also be exacerbated or caused by other ototoxic treatment (e.g. aminoglycosides). Children with hearing loss should be assessed by pure tone audiography, or by behavioural audiometry or otoacoustic emissions in younger children, and referred for ENT or audiometric/speech therapy assessment in the presence of symptomatic hearing loss. Early evaluation is important in infants treated with cisplatin or high-dose carboplatin, whilst education and community paediatric services should be informed about children with significant hearing impairment.

particularly in girls, and its most worrying consequence is its potential to mask GH deficiency and therefore ultimately increase the risk of reduced final height. Gonadotrophin deficiency is commoner with the higher doses of CRT used for CNS tumours. It is important to monitor both growth and pubertal development closely, and regular measurement of LH, FSH and the sex hormones (testosterone or oestradiol) will allow recognition of hypo- or hypergonadotrophic hypogonadism. Coupled with clinical recognition of precocious puberty, this will allow appropriate management with hormone replacement treatment (for delayed puberty) or antagonist treatment (for precocious puberty). TSH and especially ACTH deficiency are seen less commonly but may complicate higher dose CRT, occurring in up to 10% of survivors after doses higher than 30 Gy. Gonadal/reproductive system The gonads are responsible for pubertal development and reproductive function in both sexes. In males, these functions are performed by two different cell types with differential susceptibilities to chemotherapy and RT, whereas in females the oocyte undertakes both roles. Sertoli cells nurture the development of germ cells into mature spermatozoa, whilst Leydig cells are responsible for male gonadal endocrine function by secreting testosterone. Sertoli and germ cells are very sensitive to damage by RT (even at doses as low as 1.2 Gy) and chemotherapy, leading to oligo- or azoospemia which is usually permanent. Any cytotoxic drug may harm germ cells, and the risk varies according to the precise combinations, doses and schedules used, but the alkylating agents (particularly higher doses of cyclophosphamide), cisplatin, cytarabine, dacarbazine and procarbazine are regarded as the most likely to cause significant damage. Overt Leydig cell damage, manifest by pubertal delay or failure associated with reduced testosterone and raised LH concentrations, occurs only with higher doses of RT (more than 20e30 Gy), and should be treated with replacement testosterone. Leydig cell failure is rare after chemotherapy alone, although raised LH concentrations may be seen after alkylating agent treatment reflecting subclinical toxicity. In contrast, in females germ cell and endocrine failure occur concomitantly following RT or chemotherapy. As with males, any cytotoxic drug may harm female germ cells and the effects are modulated by drug combinations, doses and schedules, but the alkylating agents (especially cyclophosphamide), cisplatin, cytarabine, dacarbazine and procarbazine are most damaging. The clinical presentation varies with age at treatment, ranging from pubertal failure and primary amenorrhoea in younger girls to secondary amenorrhoea, irregular periods or premature menopause in post-pubertal females. Investigations reveal raised FSH and LH but low oestradiol concentrations. Appropriate hormone treatment may be needed to induce puberty or to minimize the long-term consequences (e.g. on cardiovascular or bone health) of oestrogen deficiency. However, ovarian failure may recover in some CCS so it is appropriate to temporarily withdraw hormone replacement treatment periodically to assess whether spontaneous periods restart. Age at treatment is an important risk factor for gonadal damage in females, with age less than 10 years being partially

Endocrine system Endocrine complications are the second commonest group of LAEs, reported in 18% of CCSS survivors (RR 5.9), and have many diverse consequences including hypothalamic-pituitary axis (HPA) dysfunction (which itself may lead to impaired growth and pubertal development), gonadal damage and impaired fertility, thyroid dysfunction and metabolic complications. The tumour itself may damage endocrine tissue directly (especially with brain tumours involving the HPA), but the commonest causes of endocrinopathies are chemotherapy and especially RT. Hypothalamic-pituitary axis CRT is the major risk for anterior pituitary dysfunction, but the threshold dose for and speed of onset of hormone deficiency varies, being lowest and quickest for growth hormone (GH), followed by the gonadotrophins (luteinizing hormone [LH] and follicle-stimulating hormone [FSH]), then adrenocorticotrophin (ACTH) and finally thyroid-stimulating hormone (TSH). The speed of onset of hormone deficiency shortens with increasing CRT dose and the incidence increases with dose and with longer time since treatment. GH deficiency and growth impairment are described in the second paper in this series. The onset of gonadotrophin secretion triggers the development of secondary sexual characteristics and reproductive potential as well as a growth spurt during puberty, whilst continued secretion during adulthood maintains fertility and hormonal function of the gonads. CRT may disturb the timing of puberty by causing early (precocious puberty) or late (delayed puberty) gonadotrophin release. Delayed puberty is commoner, but precocious puberty may follow lower dose CRT (18e24 Gy) given in acute lymphoblastic leukaemia (ALL) treatment,

PAEDIATRICS AND CHILD HEALTH 22:5

203

OCCASIONAL REVIEW

protective presumably due to the higher number of primordial follicles at the time of the toxic insult. However, age has little influence on the risk of toxicity in males. Even when the prognosis for fertility appears very poor, both males and females should be advised to undertake contraceptive precautions if pregnancy is not desired. Referral to a reproductive medicine specialist should be offered in individuals with or at risk of impaired fertility to allow further discussion and, where appropriate, investigation and treatment.

cardiomyopathy. However, general management should include advice to prevent excessive strain on the heart (e.g. avoidance of arduous weight lifting, and of prolonged labour during childbirth), and minimization of other cardiovascular risk factors, such as hormone deficiencies, metabolic problems such as dyslipidaemias, obesity and smoking. Respiratory system Chronic respiratory problems were reported in 12% of survivors in the CCSS (RR 2.8). Restrictive pulmonary disease is usually due to either chemotherapy (especially busulfan, carmustine or lomustine) or RT including lung tissue. It may be subclinical, manifest only by abnormal pulmonary function tests (PFTs), or present with progressive breathlessness and respiratory failure due to pulmonary fibrosis. In contrast, obstructive lung disease occurs most commonly in survivors of HSCT and is associated with chronic graft-versus-host disease (GvHD). Previous thoracic surgery may also impair lung function, whilst bronchiectasis may follow previous lower respiratory tract infections. PFTs should be performed in at-risk patients after completing treatment and regularly thereafter in patients with symptoms or abnormal results. Pneumococcal and annual influenza immunization should be recommended in patients with established lung disease.

Thyroid Any RT field that includes the thyroid gland is associated with a dose-related risk of functional LAEs and an increased incidence of thyroid neoplasms. The functional consequences, which may also be caused by 131iodine-meta-iodobenzylguanidine (MIBG) therapy as used in neuroblastoma, may present as early as 6 months or as late as decades post-treatment and include hypothyroidism, or much less commonly hyperthyroidism or autoimmune thyroiditis. Regular measurement of thyroid function tests will allow timely biochemical detection of hypothyroidism which may be clinical (high TSH and low thyroxine, T4) or more often subclinical (high TSH but normal T4), and may occur in up to 50e60% of CCS treated with higher RT doses to fields including the thyroid (e.g. mantle or craniospinal fields for HD or CNS tumours, respectively). Thyroxine replacement treatment is indicated in clinical hypothyroidism and is usually recommended in persistent subclinical hypothyroidism since the risk of thyroid malignancy is believed to be higher in the presence of a consistently raised TSH. Thyroid neoplasms may be benign (usually adenomas) or malignant (carcinomas) and usually respond well to timely treatment. Regular neck examination is recommended with further investigation of palpable thyroid nodules with ultrasound imaging followed by fine needle biopsy of suspicious lesions.

Gastrointestinal/hepatobiliary system Chronic gastrointestinal conditions were reported in 4% of survivors in the CCSS (RR 3.7). Surgery and RT to the gastrointestinal tract may cause subsequent strictures and malabsorption, especially with higher RT doses or more extensive fields. Hepatitis due to viral infection after blood transfusion is now much less common due to better blood product surveillance. LTFU should include enquiry about symptoms of swallowing or intestinal dysfunctional, examination for signs of liver disease such as hepatosplenomegaly or jaundice, and liver function tests if indicated.

Cardiovascular Cardiac diseases were reported in 13% of CCCS survivors (RR 1.9), the third commonest category. The major risk factors are treatment with anthracyclines and/or RT to the heart, whilst young age at treatment, female gender and longer follow-up also increase the risk. The magnitude of treatment-related risk is dose-dependent with respect to anthracyclines and congestive cardiac failure/cardiomyopathy, and RT and coronary artery disease, myocardial infarction, pericardial disease and valvular abnormalities. Given the seriousness of cardiac LAEs, primary prevention by avoidance or reduction of cardiotoxic treatment whenever possible is desirable but complete avoidance is often impractical given the importance of anthracyclines and RT in many treatment strategies for a wide range of childhood malignancies. Dexrazoxane, an iron chelator that prevents myocyte damage by reducing oxidative stress is effective in reducing cardiomyopathy in breast cancer patients, but it is not widely used in paediatric oncology due to uncertainty about the riskbenefit ratio. Treatment with angiotensin converting enzyme (ACE) inhibitors appears to postpone but not prevent cardiomyopathy. Agreement is lacking about how and how often to monitor CCS at risk of developing cardiac disease, but most strategies are based on echocardiographic surveillance at defined intervals in patients at risk of anthracycline-induced

PAEDIATRICS AND CHILD HEALTH 22:5

Urinary tract system Renal: renal problems were reported in 10% of survivors in the CCSS (RR 1.5), and may comprise glomerular or tubular damage, or both, and occasionally hypertension. Renal failure or the requirement for dialysis is uncommon, occurring in 0.5% of survivors in the CCSS, but has serious implications. Glomerular toxicity is often subclinical, being revealed only by an elevated serum creatinine, but may occasionally lead to chronic or rarely end-stage renal failure. Tubular damage may also be subclinical, but clinically overt proximal tubulopathy may occur in 25e30% of children treated with ifosfamide or cisplatin. Chronic tubular toxicity after ifosfamide may cause hypophosphataemia and even hypophosphataemic rickets (HR), whilst cisplatin (and less commonly carboplatin) may cause hypomagnesaemia, which if severe may result in convulsions, tetany or cardiac arrhythmias. Ifosfamide may also rarely cause renal tubular acidosis (RTA) and/or nephrogenic diabetes insipidus (NDI) due to distal tubular damage. The main risk factors for nephrotoxicity are the specific chemotherapy received, RT including renal tissue and previous nephrectomy, whilst tumour-related urinary tract obstruction or pre-existing renal dysfunction may exacerbate subsequent

204

OCCASIONAL REVIEW

damage. Ifosfamide nephrotoxicity is commoner in patients receiving a cumulative dose more than 80g/m2. The increased frequency and severity of ifosfamide nephrotoxicity in children less than 5 years old at treatment reported in several studies has not been a universal finding especially with longer follow-up. Platinum nephrotoxicity is commoner in children treated with a higher cisplatin dose rate (more than 40 mg/m2/day) or higher cumulative carboplatin doses, and those treated at an older age. Other cytotoxic drugs that may cause or contribute to nephrotoxicity in children include melphalan, high-dose methotrexate and the nitrosoureas (carmustine, lomustine, semustine), as well as supportive care drugs including aminoglycosides and amphotericin, and immunosuppressive agents such as ciclosporin. Patients at risk of or with nephrotoxicity should undergo regular surveillance including measurement of blood pressure and growth, urinalysis for proteinuria and biochemical monitoring. Electrolyte supplementation or specific treatment of HR, RTA and NDI may be required.

decades. CRT may indirectly affect BMD by interfering with GH and sex hormone secretion, whilst prolonged steroid treatment and antimetabolites also reduce BMD in a dose-dependent manner. HSCT survivors are at particularly high risk of skeletal toxicity due to the combination of multiple endocrine and gonadal adverse effects of TBI and high-dose chemotherapy, and the consequences of reduced levels of physical activity. Although the sale of the risk is not clear, CCS with reduced BMD are expected to be at higher risk of bone fractures. DEXA examination should be performed at the end of puberty in at-risk survivors. Effective treatment of reduced BMD is difficult, but vitamin D supplementation and optimal dietary calcium intake should be recommended. In severe and symptomatic osteopenia, treatment with bisphosphonates may also be considered in post-pubertal survivors. Avascular necrosis of bone (AVN) may occur during or after therapy, particularly with prolonged steroid treatment and/or RT, usually causing pain and functional impairment in limb joints. Management is usually conservative initially, comprising pain control, physiotherapy and surgery to encourage joint preservation, but in established AVN joint damage is usually progressive and ultimately severe. Although usually delayed as much as possible in younger patients, joint replacement is frequently required.

Bladder/lower urinary tract: chronic lower urinary tract toxicity may follow previous acute haemorrhagic cystitis (HC) due to either RT or oxazaphosphorine (cyclophosphamide or ifosfamide) chemotherapy, leading to persistent or recurrent urinary frequency, dysuria, irritability and/or urgency, incontinence and occasionally retention due to bladder fibrosis and dysfunction. Rarely, bladder malignancy may follow previous severe HC, and regular urine cytology surveillance in advisable in such individuals. Bladder damage due to the original malignancy or its treatment (with surgery or RT) may result in a small volume bladder or a urethral stricture. Urinary tract symptoms should be evaluated by urinalysis, microscopy and culture, and referral to an urologist for cystoscopy or urodynamic studies may be helpful.

Dental/craniofacial The most important risk factor is RT, although chemotherapy may contribute to impaired dental development. Complications are more prevalent in patients treated at a young age. Permanent teeth may be absent due to prior damage to the developing dentition, whilst damage during later development may result in small, brittle teeth. Root damage may also lead to tooth loss. Good mouth and dental care is very important during and after RT and/or chemotherapy to reduce both acute and chronic oral and dental toxicity. Irradiation of the growing skull and face will lead to hypoplasia of the treated area, which is more pronounced in younger children and with higher RT doses. In unilateral RT fields, hypoplasia becomes more obvious and asymmetrical with time as normal growth of the contralateral side occurs. Surgical correction of such skeletal complications is difficult and complex.

Musculoskeletal system Neurotoxicity due to vincristine usually presents with painful sensorimotor peripheral neuropathy during treatment. Although the sensory element generally improves after completion of treatment, chronic neuromuscular impairment is described. Survivors of ALL demonstrate a reduced range of lower limb joint movements (especially at the ankle) and muscle strength, with impaired physical performance in walking distance tests. Bone metabolism may be disturbed by malignancy itself, reduced physical activity or inadequate nutrition, as well as RT or chemotherapy. The most severe adverse effect on linear growth is caused when RT is given before bone growth has been completed. Exposure of bones to RT doses more than 20 Gy, especially when including long bone epiphyseal plates, leads to reduced bone growth and potentially to asymmetrical limb growth. If the spine is involved in the radiation field, vertebral bodies will display impaired growth leading to decreased final height and disproportionately reduced sitting height. Asymmetrical RT to paravertebral fields may cause scoliosis. It is important to optimize the normal physiological accretion of bone mass (measurable as bone mineral density [BMD] by dual-X ray absorptiometry [DEXA] scanning) which usually peaks in early adulthood, with subsequent decay over later

PAEDIATRICS AND CHILD HEALTH 22:5

Skin LAEs affecting the skin include non-melanomatous skin cancer (NMSC) (most commonly basal cell but also squamous cell carcinoma), melanoma, alopecia and sparse/patchy hair, scarring, atrophy and pigmentation disorders. NMSC is rarely fatal but can cause substantial cosmetic problems if diagnosed and treated late. These tumours also occur at a much younger age in CCS, sometimes even presenting before 10 years age. RT is the main risk factor, with 90% occurring within the RT field, although young age at treatment also increases the risk. Higher RT dose is a risk factor for malignant melanoma, as is treatment with alkylating agents. Chemotherapy may also increase the frequency of pigmented naevi which may be precursors to malignant transformation. CCS should be advised to use suncreams and avoid excess sunlight whilst careful examination of irradiated areas during LTFU is important. A 205

OCCASIONAL REVIEW

FURTHER READING 1 Children’s Oncology Group (COG) Long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers (version 3.0, 2008) (www.survivorshipguidelines.org/). 2 Curry HL, Parkes SE, Powell JE, Mann JR. Caring for survivors of childhood cancers: the size of the problem. Eur J Cancer 2006; 42: 501e8. 3 Diller L, Chow EJ, Gurney JG, et al. Chronic disease in the Childhood Cancer Survivor Study cohort: a review of published findings. J Clin Oncol 2009; 27: 2339e55. 4 Goldsby RE, Lui Q, Nathan PC, et al. Late occurring neurologic sequelae in adult survivors of childhood acute lymphoblastic leukemia: a report from the Childhood Cancer Survivor Study. J Clin Oncol 2010; 28: 324e31. 5 Green DM, Kawashima T, Stovall M, et al. Fertility of female survivors of childhood cancer: a report from the Childhood Cancer Survivor Study. J Clin Oncol 2009; 27: 2677e85. 6 Green DM, Kawashima T, Stovall M, et al. Fertility of male survivors of childhood cancer: a report from the Childhood Cancer Survivor Study. J Clin Oncol 2010; 28: 332e9. 7 Gurney JG, Kadan-Lottick NSD, Packer RJ, et al. Endocrine and cardiovascular late effects among adult survivors of childhood brain tumors: Childhood Cancer Survivor Study. Cancer 2003; 97: 663e73. 8 Mertens AC, Yasui Y, Liu Y, et al. Pulmonary complications in survivors of childhood and adolescent cancer. A report from the Childhood Cancer Survivor Study. Cancer 2002; 95: 2431e41. 9 Mulrooney DA, Yeazel MW, Kawashima T, et al. Cardiac outcomes in a cohort of adult survivors of childhood and adolescent cancer: retrospective analysis of the Childhood Cancer Survivor Study cohort. BMJ 2009; 339: b4606. 10 Oeffinger KC, Mertens AC, Sklar CA, et al. Chronic health conditions in adult survivors of childhood cancer. N Engl J Med 2006; 355: 1572e82. 11 United Kingdom Children’s Cancer Study Group (UKCCSG). Late Effects Group Therapy based long term follow up practice statement. 2nd Edn, http://www.cclg.org.uk/treatmentandresearch/content.php? 3id¼29&2id¼19; 2005.

PAEDIATRICS AND CHILD HEALTH 22:5

12 Wallace WHB, Green DM, eds. Late effects of childhood cancer. London: Arnold, 2004. 13 Wallace WHB, Kelnar CJH, eds. Endocrinopathy after childhood cancer treatment. Basel: Karger, 2009.

Practice points C

206

Almost one in 700 young adults is a long-term survivor of childhood malignancy, but about 60% have one or more chronic medical problems and 30% a severe or lifethreatening/disabling chronic condition Most chronic conditions in survivors are caused by their previous treatment, although the malignancy itself may sometimes contribute, especially after central nervous system tumours Neurological late adverse effects are the commonest organ/ system-specific chronic conditions, followed by endocrine, cardiac, respiratory and renal toxicities Chronic neurological toxicity has many wide-ranging and potentially life-threatening or disabling manifestations, including leucoencephalopathy, vasculopathy, myelopathy and secondary brain tumours, whilst visual and hearing impairment may cause significant social and educational difficulties Endocrine late adverse effects have many diverse consequences including hypothalamic-pituitary dysfunction leading to impaired growth and pubertal development, gonadal damage and reduced fertility, and thyroid dysfunction Referral to a reproductive medicine specialist should be offered to survivors with or at risk of impaired fertility Since the commonest cardiac late effects are anthracyclineinduced cardiomyopathy and radiotherapy-induced coronary artery disease, it is important to minimize other cardiovascular risk factors such as hormone deficiencies, metabolic abnormalities including dyslipidaemias, obesity and smoking

PERSONAL PRACTICE

Rhythmic movement disorder: managing the child who head-bangs to get to sleep

Diagnostic criteria for RMD A The patient exhibits repetitive, stereotyped and rhythmic motor behaviours B The movements involve large muscle groups C The movements are predominantly sleep related, occurring near nap or bedtime, or when the individual appears drowsy or asleep D The behaviours result in a significant complaint as manifest by at least one of the following:

Philippa M Haywood Catherine M Hill

1. Interference with normal sleep 2. Significant impairment of daytime function 3. Self-inflicted bodily injury that requires medical treatment (or would result in injury if preventable measures were not used E The rhythmic movements are not better explained by another current sleep disorder, medical or neurological disorder, mental disorder, medication use, or substance use disorder.

Abstract Rhythmic movements, such as head-banging, at sleep onset are common in infancy and usually resolve by school entry. For some children however they can persist and cause daytime sleepiness. Noise from the movements can also disrupt the sleep of other family members. This article outlines a practical approach to the evaluation and management of this disorder.

Table 1

Keywords rhythmic movement disorder; jactatio capitis nocturna; Table 1). There are a number of clinical subtypes of rhythmic movement disorder, each associated with characteristic movements, generally occurring at a rate of 0.5 to 2 per second and lasting less than 15 minutes (Table 2). The majority of children with RMD are healthy and typically developing. However, it does have an association with developmental disorders such as autistic spectrum disorders (ASD) or learning difficulties (LD). In this group of children RMD is more likely to be problematic and persistent and its stereotyped movements can also occur during the day.

jactatio corporis nocturna; head-banging

Introduction Those who spend a lot of time with young infants will recognize the phenomenon of sleep-related rhythmic movements. Up to 60% of infants aged 9 months will rock their bodies, bang or roll their heads, or use other repetitive movements at sleep onset in order to settle themselves off to sleep. In the vast majority of children, this behaviour, termed ‘rhythmic movement disorder’ begins prior to age 12 months. It subsides rapidly with age, being seen in only 5% of 5 year olds. Less commonly, it persists into adolescence and adulthood although classically the movements become less violent with increasing age. Rhythmic movement disorder (RMD) is defined in the 2nd Edition of the International Classification of Sleep Disorders (ICSD-2) (2005) as ‘comprising a group of stereotyped, repetitive movements involving large muscles, usually of the head and neck; the movements typically occur immediately prior to sleep onset and are sustained into light sleep’. The duration of the movements can be from a few minutes, to several hours. The ICSD-2 also contains the diagnostic criteria for RMD (see

Case history Robbie, aged 8, is referred to the paediatric clinic as his persistent head-banging at night is causing sleep disturbance for the whole family. Robbie himself is too embarrassed to sleepover with friends and is worried about next year’s school holiday to a multi-activity centre.

Key points to elicit in the history Is this RMD? RMD is a diagnosis of exclusion and other concurrent sleep disorders, as well as medical, neurological and psychiatric disorders should be considered and excluded. Reviewing a home

Clinical subtypes of RMD (ICSD-2) Philippa M Haywood MA (Cantab) MB BChir MRCPCH is a Consultant Paediatrician in Community Child Health at the Royal Hampshire County Hospital Winchester, UK. Conflicts of interest: none declared.

Head-banging type: the head is forcibly moved, striking an object Body rocking type: the whole body is rocked while on the hands and knees Head rolling type: the head is moved laterally typically while in a supine position Other type: includes body rolling, leg rolling and leg banging Combined type: includes 2 or more of the individual types

Catherine M Hill BM MSc MRCP FRCPCH is Honorary Consultant Paediatrician for the Solent NHS Trust and Southampton Children’s Sleep Disorder Service, Southampton and Senior Lecturer in Child Health at the University of Southampton Faculty of Medicine, UK. Conflicts of interest: none declared.

PAEDIATRICS AND CHILD HEALTH 22:5

Table 2

207

Ó 2012 Published by Elsevier Ltd.

PERSONAL PRACTICE

How is the family managing the RMD? What are their beliefs about it? Parents and siblings may have their sleep disturbed by the sound of the child’s rhythmic movements. Parental anxiety about the risk of head injury or about an underlying developmental or emotional disorder is common. Both of these contribute to a tendency for parents to inadvertently reinforce the RMD behaviours. This can occur when parents attempt to stop the behaviours by waking the child up, thus giving attention as well as causing the child to start the RMD cycle again in order to resettle. Alternatively, parents often use other methods such as lying with or stroking the child to soothe them to sleep, causing the child to associate the parental intervention with sleep onset and then to demand the same intervention when they arouse normally during the night.

video recording of an episode can increase diagnostic certainty. Clinicians should be aware of local information governance policies on proper usage and storage of such images. Atypical, prolonged or particularly violent episodes should prompt specific questioning to rule out epilepsy. Any tonic-clonic movements, incontinence, eye deviation or tongue/cheek biting during the episodes, or other risk factors suggesting epilepsy, should trigger further neurological evaluation. Is there any reason why Robbie may be waking more than usual during the night? As RMD is a learnt behavioural strategy to aid settling to sleep, it will be more prolonged or frequent in the presence of an associated condition causing either difficulty settling to sleep (e.g. restless legs syndrome) or increased night wakings (e.g. obstructive sleep apnoea). Sleep comprises distinct stages: rapid eye movement (REM), or dream sleep and slow wave (non-REM) sometimes known as deep sleep, interspersed with lighter stage 2 sleep. A normal night’s sleep is characterized by alternating cycles of the two types of sleep, with slow wave sleep predominating during the first half of the night and REM sleep during the latter half of the night (Figure 1). There are usually brief behavioural arousals at the end of each sleep cycle and ideally these should be followed by a rapid return to sleep. However, in RMD, the child associates their preferred rhythmic movement with being able to fall back to sleep again and so they tend to engage in the behaviour when they arouse from sleep. While most RMD occurs during the transition from drowsy wakefulness to sleep, polysomnographic studies show that it can occur during stage 2 and deep sleep but is very unusual in REM sleep when the muscles are profoundly relaxed.

Further points elicited in Robbie’s history Robbie is a typically developing, healthy boy with no daytime behavioural concerns. In the clinic he appears to be sociable and to have a nice bond with his mother, Amy. He tells you that he wishes he could go to sleep without banging his head as he wants to be able to go on sleepovers and school visits without feeling embarrassed. Robbie’s episodes have remained the same since he was 10 months of age. After being put to bed, he gets onto his hands and knees and bangs his head repeatedly onto his headboard or wall until he is asleep. A few months ago, his parents started being been woken in the night by the sound of him head-banging. Robbie’s father is a lorry-driver and the family is concerned that his sleep should not be disturbed. Amy has therefore started going into Robbie’s bedroom when he head-bangs overnight and stroking him back to sleep. She tells you that she is exhausted, as in the last few weeks, Robbie has been waking up to four times each night, head-banging and calling out for her. Amy becomes tearful and says that she is worried that Robbie is going to become brain-damaged from the head-banging. She worries that the problem stems from the bout of post-natal depression which she suffered after his birth.

Is Robbie developing normally? Are there any daytime behavioural or emotional concerns? A developmental history is important to rule out an underlying developmental disorder such as autism or learning difficulties. Whilst, in our experience, most children presenting to paediatric clinics with the condition are healthy, typically developing and well-cared for, it can also be seen in children who have suffered abuse, neglect or psychological distress and this possibility should be considered in severe or persistent cases and where the behaviour also occurs during the day.

Management Figure 2 shows a flowchart for the diagnosis and management of RMD. There are currently no trials of different therapeutic approaches for RMD and therefore the following approaches are based on clinical experience and anecdotal case reports. Parental education and reassurance The key aspect of RMD management is parental education about the nature of the condition. In a typically developing child under 4, with no apparent daytime behavioural or social issues, parents should be reassured that the condition is common, usually selflimiting and represents a learnt behaviour that their child uses to soothe themselves to sleep. Eliciting parental beliefs about the condition and explaining how they may be inadvertently reinforcing RMD behaviours often gives parents the confidence to ignore the behaviour which can by itself lead to improvement. As with any sleep disorder, reinforcing the importance of a calming and consistent bedtime routine with clear limits will help to avoid bedtime battles.

WAKE Stage of sleep

REM 1 2 3 0

Sleep (hours) Figure 1 Hypnogram illustrating typical sleep architecture during the night.

PAEDIATRICS AND CHILD HEALTH 22:5

208

Ó 2012 Published by Elsevier Ltd.

PERSONAL PRACTICE

isolated case reports of more significant injuries. Families should ensure that screws and bolts in the bed are tightened regularly and that the child is protected from falls, using bedrails if necessary. We advise using padding only in cases where there is evidence of harm to the child. Moving the bed away from the wall or sleeping the child on a mattress on the floor as well as encouraging parents to use ear-plugs to block out the noise will lessen the impact on the sleep of other family members.

Repetitive movements at night

Yes

Evidence of epilepsy?

Further evaluation necessary

Management of co-morbidities that disrupt sleep or prolong sleep onset RMD can co-exist with other sleep disorders such as OSA or RLS. RLS is characterized by an uncomfortable sensation in the lower limbs at times of inactivity, such as lying in bed prior to sleep. The sensations, often described by older children as â&#x20AC;&#x2122;pins and needlesâ&#x20AC;&#x2122;, â&#x20AC;&#x2122;creepy crawliesâ&#x20AC;&#x2122; or â&#x20AC;&#x2122;growing painsâ&#x20AC;&#x2122; are associated with the urge to move the legs which then relieves the discomfort. In very young children, restlessness when settling to sleep may be the only sign. There is an association with low iron stores, as reflected in low serum ferritin levels (even when the haemoglobin is normal) and iron supplementation may relieve symptoms. Our practice in all cases of RMD where the rhythmic behaviour is occurring both at bedtime and throughout the night is to look for evidence of obstructive sleep apnoea (OSA) through taking a careful clinical history. Other options include the use of a validated tool such as the Pediatric Sleep Questionnaire (Chervin). If clinical assessment suggests possible OSA, we actively screen for this with overnight pulse oximetry and, where indicated, respiratory polysomnography. Consideration should also be given as to whether other disorders such as ear infections, gastro-oesophageal reflux or other causes of pain could be triggering head-banging or body-rocking.

Typical history for RMD? No Yes Diagnose RMD Explain RMD to family Address parental belief about RMD Give safety advice Advise parents to avoid reinforcing RMD behaviours Access childâ&#x20AC;&#x2122;s motivation to change

Clinical evidence of OSA or RLS?

Yes

Investigate, treat and review

No Age â&#x2030;¤ 4 and developmentally normal?

No RMD resolved?

Yes Discharge

Yes Consider other treatments: Behavioural Psychological Pharmacological

Discharge

Behavioural and psychological approaches Stimulus substitution: replacing the rhythmic movements with other less intrusive repetitive stimuli has been successful in some of our cases. For example, in a learning disabled child, head-banging was gradually replaced, first by the parent patting the childâ&#x20AC;&#x2122;s back and then by use of a metronome set to a similar rhythm. The latter was then gradually withdrawn and over a period of weeks the childâ&#x20AC;&#x2122;s RMD was abolished. Aversive therapy involves interrupting the unwanted behaviour with an aversive experience. Clearly it is inappropriate to â&#x20AC;&#x2DC;punishâ&#x20AC;&#x2122; a child and it is key that the child understands the treatment goals. We have had success in one motivated child where the parent was instructed to gently remove the child from his bed and calmly walk him around his bedroom every time he began to head bang. This is highly demanding of the carer who also needs to be motivated. In some cases it would be more readily achieved in an in-patient setting. This can be combined with mild sleep deprivation to allow the child to experience falling asleep without needing RMD behaviours.

Figure 2 Flowchart illustrating suggested investigation and management of rhythmic movement disorder.

Safety advice Typical cases of RMD pose minimal risk of serious injury to the child. Some children may develop a forehead callus or ecchymosis from repeated head-banging (Figure 3) but there have been only

Pharmacological treatment In severe or very persistent cases, drug treatment can be considered. There is a paucity of research into the pharmacological treatment of RMD in children. Our personal practice is to firstly use a time-limited course of clonazepam at a dose of 1e2 mg orally at night. Our experience is that this is successful in around

Figure 3 Forehead callus in 3 year old with persistent RMD.

PAEDIATRICS AND CHILD HEALTH 22:5

209

Ă&#x201C; 2012 Published by Elsevier Ltd.

PERSONAL PRACTICE

FURTHER READING Khan A, Auger RR, Kushida CA, Ramar K. Rhythmic movement disorder. Sleep Med 2008; 9: 329e30. Mindell J, Owens J. A clinical guide to pediatric sleep: diagnosis and management of sleep problems. 2nd edn. Lippincott Williams and Wilkins, 2009. Sheldon SH, Ferber R, Kryger MH. Principles and practice of pediatric sleep medicine. Elsevier Saunders, 2005.

50% of cases. This failure rate reflects recurrence of the RMD once the clonazepam is discontinued. Caution needs to be exercised in gradual dose reduction We have also had some success using alimemazine tartrate orally at a dose of 1e2 mg/kg at night to consolidate sleep and reduce the number of night wakings. Combined approaches Some success has been reported by other centres using a technique which combined controlled mild sleep restriction with use of a hypnotic. This aimed to rebuild confidence in the ability to fall asleep without needing RMD behaviour.

Case outcome

Practice points

Robbieâ&#x20AC;&#x2122;s family was informed of the diagnosis of RMD and reassured that this was not a refection of psychological damage but primarily a learnt behaviour. Amy was advised to allow Robbie to sleep on a mattress on the floor and to ignore his RMD behaviours as far as possible. A careful history revealed that Robbie had symptoms of obstructive sleep apnoea and this was confirmed on overnight oximetry. He had a tonsillectomy, after which his overnight oximetry improved and the RMD behaviours were only observed at sleep onset. A 4 week trial of clonazepam produced remission of the RMD which was maintained after graded discontinuation of medication. This allowed Robbie to enjoy sleepovers with friends and look forward to his upcoming school trip. A

PAEDIATRICS AND CHILD HEALTH 22:5

210

RMD is common in infancy and in most cases remits spontaneously by 4 years of age Parental reassurance, education and safety advice are key to successful management Underlying conditions which may be disrupting sleep should be actively sought and managed Persistent RMD may respond to specific behavioural, psychological or pharmacological approaches but the current evidence base for these approaches is limited

Ă&#x201C; 2012 Published by Elsevier Ltd.

SELF-ASSESSMENT

Self-assessment Questions

He was born in Nepal and moved to the UK 2 years ago. He has a BCG scar, and his TST shows induration of 20 mm. What THREE assessments/investigations would you chose to do next? (a) Chest and spine X-rays (b) Sinogram (c) Bone scintigram (d) Swab sinus and send for acid fast bacilli test (e) Neurological review (f) HIV test (g) MRI of shoulder and spine (h) CT chest (i) Repeat tuberculin skin test

Case 1 1. A 14-year-old Nepalese boy presented to his GP with a 6-week history of painful swelling with reduced range of movement of the left shoulder. There was no history of injury. He was noted to be of short stature, below the 0.4th centile, and reported that he had been intermittently pyrexial, with night sweats. There was no rash and there had been no previous episodes. What are the THREE most likely diagnoses? (a) Juvenile Idiopathic Arthritis (JIA) (b) Leukaemia (c) Staphylococcal osteomyelitis/Septic Arthritis (OM/ SA) (d) Reactive Arthritis (e) Trauma (f) Osteosarcoma (g) Foreign body (h) Tuberculosis (TB)

4. Results of investigations are shown below (normal ranges in parentheses): Bloods: FBC: Hb 12.3 g/dL (13.0e17.0 g/dL), WCC 12.7 109/L (4.0e11.0 109/litre), Neutrophils 7.2 109/litre (2.0e7.5 109/L), Platelets 422 109/litre (150e400 109/L); CRP 43 mg/L (less than 3 mg/L), ESR 23 mm/h (1 e19 mm/h). Chest X-Ray (Figure 1).

2. Which THREE investigations would you request first? (a) Chest X-ray (b) MRI of the shoulder (c) Full blood count/ESR/CRP (d) Tuberculin skin test (TST) and interferon gamma release assay (IGRA) (e) Rheumatoid factor (f) Serum ferritin (g) X-ray of shoulder (h) Joint aspiration for culture and microscopy

Culture of pus from the sinus grew no organisms. Repeat interferon gamma release assay (IGRA) was positive. Which ONE investigation would do you do next? (a) Request orthopaedic review (b) Request CT guided bone biopsy (c) Bone marrow aspirate (d) Investigate for immunodeficiency (e) Induced sputum culture

3. Two weeks later he re-presented with a discharging sinus on the anterior aspect of his left shoulder. IGRA was indeterminate. Further questioning reveals he has also had back pain for several years, which is worsening.

MRI scan showed multifocal spinal disease. On spinal examination a marked kyphoscoliosis was noted. Neurological examination was normal. He was referred to the orthopaedic team for assessment and further investigation, and further imaging was requested (Figure 2): Following this scan a biopsy was taken from T12/L1 vertebrae and sent for histology. The results were not suggestive of malignancy, and culture for mycobacterium tuberculosis was negative as was staining for acid-fast bacilli. Treatment was started empirically on the basis of positive TST and IGRA. He was discharged on quadruple therapy for 2 months and 2-drug therapy for a further 10 months.

Priya Sukhtankar MBChB, BSc, MRCPCH is Clinical Research Fellow and ST6 in Paediatrics, at the NIHR Wellcome Trust Clinical Research Facility, University of Southampton, University Hospital Southampton NHS Foundation Trust, UK. Conflict of interest: none. Marc Tebruegge DTM&H, DLSHTM, MRCPCH, MSc, MD is Clinical Lecturer, at the NIHR Wellcome Trust Clinical Research Facility, University of Southampton, University Hospital Southampton NHS Foundation Trust; and Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, UK. Conflict of interest: none.

5. Which of the following FOUR drugs are used in standard quadruple therapy in the UK? (a) Amoxicillin (b) Ethambutol (c) Isoniazid (d) Moxifloxacin

Saul N Faust MRCPCH, PhD, FHEA is Consultant in Paediatric Infectious Diseases, at the NIHR Wellcome Trust Clinical Research Facility, University of Southampton, University Hospital Southampton NHS Foundation Trust; Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, UK. Conflict of interest: none.

PAEDIATRICS AND CHILD HEALTH 22:5

211

SELF-ASSESSMENT

Figure 1 Chest X-ray

(e) (f) (g) (h)

Pyrazinamide Rifabutin Rifampicin Streptomycin

6. Which ONE of the following is most important? (a) Refer to social services (b) Refer family for TB screening (c) Inform school nurse (d) Refer to dietician (e) Monitor isoniazid level

This patient continued to be followed up in clinic for a further year. Public health was notified. He required ongoing physiotherapy and orthotic input for a spinal brace. M. tuberculosis specific PCR on biopsy samples confirmed spinal TB.

Case 2 A 7-year-old boy presents with fever and otalgia, worsening over 1 week despite treatment with clarithromycin. His temperature continued to spike to 39 C and he developed a cough. He received 1 week of intravenous antibiotic therapy in hospital for right sided pneumonia, but made little improvement and was transferred to tertiary centre. On arrival he looked unwell and temperature on admission was 40 C. His heart rate was 150/min, blood pressure 82/60 mmHg, respiratory rate 44/min, oxygen saturation 91% in air. He had dullness to percussion on the right side and reduced air entry on auscultation. 1. As the admitting doctor, which THREE of the following are most important in initial management? (a) Obtain intravenous access and give 20 ml/kg 0.9% saline (b) Start dopamine infusion at 10 mcg/kg/min (c) Give 20 mg/kg paracetamol (d) Apply high flow oxygen via facemask (e) Contact anaesthetic team for intubation (f) Insert chest drain (g) Full blood count (h) 50 mg/kg IV cefuroxime (i) Capillary blood gas

Figure 2 Further imaging: bone scan.

PAEDIATRICS AND CHILD HEALTH 22:5

212

SELF-ASSESSMENT

Figure 3 Chest X-ray on arrival at tertiary hospital.

His chest X-ray (Figure 3) and blood tests are shown below. Full blood count: Hb 9.4 g/dL (13.0e17.0 g/dL), WCC 10.9 109/L (4.0e11.0 109/L), Neutrophils 6.4 109/ L (2.0e7.5 109/L, Lymphocytes 4.3 109/L (1.5e4.0 109/L). CRP 169 mg/L (<3 mg/L). Renal profile: Na 136 mmol/L (135e145 mmol/L), K4.6 (3.5e5.0 mmol/L, Urea 6.7 mmol/L (2.9e7.1 mmol/L), Creatinine 50 mmol/L (35e95 mmol/L). Blood cultures are pending.

3. What further investigation is most appropriate? Chose ONE of the following: (a) Ultrasound scan of chest (b) CT scan of chest (c) Bronchoscopy (d) Sputum culture (e) Pleural aspirate 4. What further management may be appropriate? (a) Ventilation on PICU (b) Treatment with 50 mg/kg intravenous cefuroxime (c) Treatment with oral co-amoxiclav and observation (d) Insert percutaneous chest drain (e) Video Assisted Thoracoscopy

2. What is your diagnosis? Choose ONE of the following: (a) Interstitial pneumonia (b) Pneumonia with pleural effusion (c) Tuberculosis (d) Lymphoma (e) Pulmonary abscess

PAEDIATRICS AND CHILD HEALTH 22:5

Pleural effusion was confirmed but was too small for drain insertion. He was treated with cefuroxime. He initially improved and was discharged home on oral co-amoxiclav. In spite of this he continued to spike high temperatures,

213

SELF-ASSESSMENT

and re-presented 1 week later. Repeat chest X-ray showed worsening pneumonia and pleural effusion and he was started on intravenous meropenem and vancomycin. On further questioning he had a past medical history of perianal abscess requiring surgical drainage aged 5 years, but nothing else of note. 5. What investigations would you do next? Choose THREE: (a) Pleural fluid culture and histology (b) Repeat ultrasound scan of chest (c) CT scan of chest (d) Bronchoscopy (e) Immunoglobulin levels, specific response to vaccinations and T-cell subsets (f) Mantoux test (g) Echocardiogram (h) Spirometry (i) Ultrasound scan of abdomen

should be considered given the history of nights sweats, short stature and ethnicity. Leukaemia and JIA are also important differentials. JIA is the most common cause of monoarthritis in this age group. 2. C, D, E Initial investigations should include basic blood tests with inflammatory markers. X-ray of the shoulder is important to exclude trauma, and is an inexpensive and quick imaging test to look for bony abnormalities. Since this child is from a high TB prevalence country, has short stature and an history of night sweats it is also important to exclude tuberculosis, so IGRA and tuberculin skin test should be performed. 3. A, D, G Indeterminate IGRA is not uncommon and does not convey any information regarding TB infection status. This should be repeated, as the history is highly suspicious of TB. TST induration of greater than 15 mm in a child with BCG vaccination is suggestive of TB infection. Chest and spine X-ray are indicated as chronic back pain is a worrying feature suggestive of tuberculous osteomyelitis of the spine, which may result in vertebral collapse and neurological compromise. This should be followed up with MRI of the spine and shoulder. As the sinus is now discharging culture and microscopy of pus is indicated.

He continued to spike high temperatures. Blood results showed normal immunoglobulin levels, normal antibody response to diphtheria and tetanus vaccinations, normal T-cell subsets, but abnormal neutrophil oxidative burst by flow cytometry. 6. What is your diagnosis? Choose one (a) Leucocyte adhesion defect (b) Chronic granulomatous disease (c) Severe combined immunodeficiency (d) Normal immune function, with acute phase reaction (e) WiskotteAldrich Syndrome

4. B Bone scintigraphy shows a significant lesion in the thoracic spine. So far pus swab and blood culture has been sterile. Whilst the most likely cause is TB, other infections and malignancy have not yet been excluded. CT guided biopsy of the spinal lesion is indicated and samples should be sent for TB culture and microscopy for acid fast bacilli as well as histology. In this case the diagnosis was made on PCR. Culture and microscopy were negative.

Loculated pleural effusion was confirmed on repeat imaging and was successfully drained. Two further weeks of IV cefuroxime were completed and the child recovered fully from his pneumonia. Follow up continues. 7. What follow up is appropriate at his next clinic appointment? Choose three (a) Refer for bone marrow transplant (b) 13-valent pneumococcal vaccination (c) Monthly MRSA swabs (d) Prophylactic itraconazole and co-trimoxazole (e) Repeat neutrophil oxidative burst flow cytometry (f) Monthly immunoglobulin replacement (g) Screening of parents and siblings for CGD carrier status/disease (h) Nebulised colomycin (i) Repeat chest X-ray

5. B, C, E, G Standard treatment of tuberculosis is with quadruple therapy for 2 months with rifampicin, isoniazid, pyrazinamide and ethambutol, followed by a further 4 months of rifampicin and isoniazid. If exposure to multi-drug resistant (MDR) TB is suspected this would require modification. (See NICE guideline for tuberculosis. http://publications.nice.org.uk/tuberculosis-cg117) Tuberculous osteomyelitis is uncommon, but should be suspected in patients from countries where TB is endemic, especially if there is a history of night sweats. Many cases of osteoarticular tuberculosis have no history of pulmonary TB. 50% of all osteoarticular TB affects the spine, particularly in adolescents. These patients are at risk of vertebral collapse and spinal cord compression causing paraplegia so orthopaedic input and orthotic support is essential.1,2

Answers Case 1 1. C, F, H The duration of illness, bony pain and intermittent pyrexia are consistent with a diagnosis of osteosarcoma, which should be excluded. Non-tuberculous osteomyelitis/septic arthritis (OA/SA), should be considered in view of fever and joint pain and stiffness. This is most commonly caused by Staphylococcus aureus in those more than 6 years old. Tuberculosis as a cause of OA/SA

PAEDIATRICS AND CHILD HEALTH 22:5

214

SELF-ASSESSMENT

Case 2 1. A, D, G Despite antibiotic therapy at the referring hospital this child has arrived hypotensive, tachypnoeic and tachycardic. Facemask oxygen, fluid resuscitation and repeat full blood count and inflammatory markers are appropriate. The patient should then be reassessed. It may also be important to modify antibiotic therapy. 2. B The CRP is 169 mg/dL although the WCC is normal. This child is also anaemic with haemoglobin of 94 g/dL. The chest X-ray shows right middle lobe pneumonia with a pleural effusion.

In view of the unusual course of pneumonia, and previous admission with perianal abscess, immune deficiency should be considered. This child had immune response to vaccinations tested, as well as lymphocyte subsets, and neutrophil burst tested by flow cytometry. Immunoglobulin and complement levels were also checked. 6. B The neutrophil burst test is expressed as a percentage and this child had low percentage initially, followed by absent neutrophil burst on repeat. This gives a diagnosis of chronic granulomatous disease, which is a deficiency in one of the five subunits of the phagocyte NADPH oxidase molecule which generates reactive oxidase species in response to infection.

3. A Ultrasound scan will allow the depth and nature of the effusion to be assessed without exposing the child to ionizing radiation. This allows the team to assess whether the effusion requires drainage. Bronchoscopy, pleural aspirate and sputum culture are unlikely to be of value at this stage.

7. B, D, G CGD is a disorder of the NADPH oxidase system causing phagocyte dysfunction. Children with CGD are at risk of catalase-positive bacterial and fungal infection, and abscesses. The 13-valent pneumococcal vaccination greatly reduces the risk of pneumococcal infection. Prophylactic co-trimoxazole provides additional protection against catalase-positive bacteria, and fluconazole prevents aspergillus and yeast infections. CGD is X-linked and the family should be screened for carrier or disease status.3

4. B At this stage there has been no organism identified and there is persistent pyrexia. It is appropriate to continue high dose broad spectrum intravenous antibiotic therapy. The child is otherwise clinically stable and does not require PICU admission. A chest drain may be necessary, but the effusion requires ultrasound assessment and is not currently causing any lung collapse.

REFERENCES 1 De Vuyst D, Vanhoenacker F, Gielen J, Bernaerts A, De Schepper AM. Imaging features of musculoskeletal tuberculosis. Eur Radiol 2003; 13: 1809e19. 2 Teo HE, Peh WC. Skeletal tuberculosis in children. Pediatr Radiol 2004; 34: 853e60. 3 Heyworth PG, Cross AR, Curnutte JT. Chronic granulomatous disease. Curr opinion Immunology 2003; 15: 578e84.

5. B, C, E This is now the 3rd presentation with pyrexia and shortness of breath despite adequate antibiotic therapy. It is likely that the effusion has re-accumulated, or extended and ultrasound scan should be repeated to assess this. CT scan of the chest is also of benefit to assess the effusion and pneumonia.

PAEDIATRICS AND CHILD HEALTH 22:5

215