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Pioneer Spring/summer 2014

Size matters The challenges of paediatric surgery

The child first and always

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Contents 04 Around the hospital

Latest news from the hospital

06 The Pioneer interview: Liz Morgan

Welcome to the latest edition of Pioneer. This issue focuses on one of Great Ormond Street Hospital’s original principles: that children need to be cared for differently to adults. Over 160 years ago, the founder of the hospital, Dr Charles West, realised the importance of not just treating children as mini adults, and this principle still guides the hospital today.

Our lead feature looks at the challenges faced by our expert surgeons in operating on children from birth. Surgery is a complicated science in any hospital, but when faced with tiny newborn children, the task becomes even more difficult.

It’s hard not to be full of admiration for our wonderful surgery teams as you comprehend the pressures they face.

In our interview with Liz Morgan, Chief Nurse, the importance of training specialist paediatric nurses becomes evident, as does the hospital’s prominent role in doing so. Nursing at Great Ormond Street Hospital is a unique challenge given the complications many of our children face, and Liz and her team do a remarkable job.

We also hear from George, just one of many children who visit the hospital for whom the word ‘unique’ really is apt. He has a variety of a condition yet be named, but his positivity in the face of adversity is an inspiration.

Great Ormond Street Hospital’s Chief Nurse tells Pioneer about the changing face of nursing and the challenges of caring for children at the hospital

09 Me and my dietitian

Three children introduce us to the dietitians who help them with their nutritional needs

14 Feature: surgery

18 Meet the team: Psychosocial and Family Services

We meet nine members of this diverse team who support children, families and staff to cope with the illness, treatment and, in the worst case, loss of a child

22 Milk – guiding the way

10 I have ectodermal dysplasia

Sixteen-year-old George tells Pioneer about his experience of living with an incredibly rare skin condition

13 Why I support the charity: Andrew Taee

The Executive Chairman of Dial Partners LLP talks about his long-standing support of the charity

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Pioneer takes an in-depth look at the challenges that the surgery team face when operating on children

Pioneer looks at the work of Dr Neil Shah, who has worked to introduce nationwide guidelines to help diagnose the most common food allergy in children

25 Working out

How Great Ormond Street Hospital is working with private gyms for the benefit of children with cystic fibrosis

26 A parent’s perspective: in rude health

Sam, father of Rudy, tells Pioneer about the moment doctors told him his son had a brain tumour

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I do hope you enjoy this edition of Pioneer.

Tim Johnson Chief Executive, Great Ormond Street Hospital Children’s Charity 02 Pioneer

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Around the hospital…

Interim Chief Executive Julian Nettel has been appointed as the Interim Chief Executive of Great Ormond Street Hospital, after Jan Filochowski announced his retirement late last year. Mr Nettel joined the Trust in January and comes with more than 20 years’ experience at senior level in the NHS. He has held chief executive positions in three large acute London trusts and been the Managing Director of the NHS Institute for Improvement and Innovation.

Mr Nettel was Operations Director at King’s Healthcare NHS Trust before being appointed as Chief Executive of Ealing Hospital NHS Trust. In 1999, he was appointed CEO of St Mary’s NHS Trust where he led the organisation to achieve the highest clinical and governance performance ratings, which contributed to it being named the inaugural Dr Foster Hospital of the Year. Mr Nettel was also Chief Executive at Barts and the London NHS Trust where he was instrumental in changing the organisation so it could better carry out translational research.

Mr Nettel said: “I am very excited about joining Great Ormond Street Hospital and using my knowledge and experience to help it continue to thrive as one of the world’s leading children’s hospitals.

I have visited the hospital on a number of occasions and have always been impressed by the dedication of the staff and the quality of care delivered.

“I am looking forward to working with them to further foster innovation and improve the care and treatment the hospital offers to some of the country’s sickest children.”

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New research programme targeting brain tumours

New study shows how stem cells could be used in facial reconstruction The effectiveness of human stem cell therapies for facial reconstruction has been investigated in a new study led

by Great Ormond Street Hospital and the UCL Institute of Child Health (ICH).

The paper, published in the journal Nanomedicine, shows how stem cells could pose a viable alternative to current approaches to facial cartilage reconstruction such as ear and nose reconstruction.

The work was carried out at the Newlife Birth Defects Research Centre (BDRC), which is based at the ICH. Newlife Foundation for Disabled Children also funded this ground-breaking research.

The hospital has an excellent track record of successfully treating patients born with a malformed or missing ear, a condition known as microtia. The twostage ear reconstruction takes cartilage from the patient’s ribs, and from this a new scaffold is moulded and placed beneath the skin.

Both the clinical and cosmetic results of this procedure have been very good.

However, as Patrizia Ferretti, Head of

Developmental Biology Unit at the ICH, and her co-authors demonstrate in their study, the potential application of human stem cells and tissue engineering could further improve results and would prevent the need for this invasive part of the procedure, which leaves a permanent defect in the donor site.

Dr Ferretti explains: “We used stem cells (hADSCs) harvested from the abdominal tissue of young patients affected by craniofacial conditions to explore, in our laboratories, how these might be used in future surgery. The use of stem cells from the paediatric patients themselves circumvents the issue of rejection and would overcome the need for immunosuppressive therapies.”

A ground-breaking research programme aiming to beat childhood brain tumours has been launched. The £4 million study, co-funded by grants from Great Ormond Street Hospital Children’s Charity (with thanks to the Dorothy and Spiro Latsis Benevolent Trust and The Isla Cecil Brighter Future Fund), the Brain Tumour Charity and Children with Cancer UK, will look at new ways to treat some of the deadliest brain tumours affecting children.

Teams from the UCL Institute of Child Health (ICH) and the Institute of Cancer Research, London (ICR) will use cuttingedge screening techniques to identify critical genetic and biochemical features of aggressive brain tumours in young patients. The ultimate aim is to assign patients to different clinical trials according to the genetic make-up of their tumour, developing new drugs that will target the specific mutations found in each tumour.

Researchers at the ICH and the ICR will work in tandem with a team at Newcastle University under the umbrella of INSTINCT, a network that brings together the work of leading scientists and clinicians in the field of high-risk paediatric brain cancer.

The aim of INSTINCT is to ensure that paediatric brain tumour research studies translate as quickly and effectively as possible into new treatments. Between them, the clinical centres working with INSTINCT in Newcastle and London treat more than one in three young brain tumour patients in the UK. Dr Darren Hargrave, who is leading the research strand at the ICH, says the INSTINCT team hopes ultimately to analyse tumour samples from every patient treated in one of its clinical centres and tailor their treatments accordingly. “Our first aim is to improve survival rates among children with highrisk brain tumours,” Dr Hargrave says.

Archbishop Desmond Tutu visits of the brain). This was detected in the womb, and following her birth Isla was taken to Great Ormond Street Hospital where she has been ever since.

Neil Bulstrode, Consultant Plastic Surgeon at the hospital and an author on the paper, said: “It is such an exciting prospect with regard to the future treatment of these patients and many more. Currently, I take the rib cartilage from the chest to make an ear, but if we could produce a block of cartilage using stem cells and tissue engineering, it would be the Holy Grail for our field.”

Archbishop Desmond Tutu made his first visit to Great Ormond Street Hospital in January. He met children and families on Koala Ward, a neurosciences ward, and Bear Ward, which cares for children with cardiac conditions. Both wards are in the Morgan Stanley Clinical Building, within the Mittal Children’s Medical Centre. One of the children he met was Isla who is two months old and has vein of Galen malformation (a rare type of vascular malformation

The Archbishop also visited the hospital’s chapel, St Christopher’s and was introduced to the multi-faith chaplaincy team which provides support and spiritual care for families. The grade II listed chapel, built in 1875, offers children, families and staff a place for prayer and contemplation.

Archbishop Tutu said: “I was very impressed with the dedication of the staff and their warmth and friendliness. Institutions can be cold, but it was clear that this is a wonderful place to be for children – you could almost smell the warmth in the air.

In many ways it is a happy place.

Reception revamp The hospital’s refurbished main reception will open its doors at the end of April to families and staff. The newly designed space will be brighter and friendlier with designated waiting areas and a quiet zone, plus interactive entertainment for children and young people.

The new reception counter has a nautical theme as it’s been built in the shape and colour of a boat. Each reception workstation has been designed for flexibility, allowing staff to deal with multiple requests, reduce waiting times and congestion. Kiosks for a 3D interactive wayfinding system are also being installed to help guide families around the hospital.

The views of patients, families and staff have been integral to the design of the new reception. As well as being consulted on the furniture to be installed, more than 100 patients took part in workshops to create an artwork for the main entrance. Each participant painted a fish that will be gathered to create a huge sea scene in keeping with the nautical theme. The fish represent the number and diversity of our patients.

Finally, a special piece of artwork has been commissioned to form part of the main entrance canopy. Glass artist Chris Wood has created an inspiring and beautiful artwork that recognises and celebrates organ donation and transplantation. Chris works with optical materials to create stunning sculptures that harness light and reflect colour.

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The Pioneer interview Liz Morgan, Chief Nurse, talks to Pioneer

Liz Morgan has been Chief Nurse at Great Ormond Street Hospital for the past three-and-a-half years. Prior to taking up the role, she held the same position at Birmingham Children’s Hospital and worked as a policy advisor in the Department of Health.

This is Liz’s second stint at Great Ormond Street Hospital, having first joined as a nurse in 1976. She lives in Birmingham with her husband, a radiologist, and has one daughter. She enjoys reading, walking, skiing and travelling in her spare time.

How did you come to be Chief Nurse at Great Ormond Street Hospital?

I entered nursing immediately after leaving school. Having enjoyed a paediatric rotation during my training at St Barts, I completed a second qualification in children’s nursing and came to the hospital in 1976. Although I’ve been in nursing for 38 years, not all of it has been at Great Ormond Street Hospital. I was eager to get a broad range of experience, so I’ve worked in a number of paediatric units across the country.

I was Director of Nursing at Birmingham Children’s Hospital for five years and then moved to the Department of Health as a professional advisor in children and young people’s healthcare. Three-anda-half years ago this fantastic job came up at Great Ormond Street Hospital and it’s been a privilege to come back to the place where I started nursing.

What does the Chief Nurse do?

I’m accountable for anything to do with nursing at the hospital – setting standards, quality and monitoring, and providing direction and guidance to the Trust Board about nursing-related issues. We have approximately 1,250 full and part-time posts across the Trust, which fall under my remit.

I’m also the Executive Lead For Safeguarding because we have a responsibility to always ask ourselves whether a child could be experiencing abuse in their private lives.

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Finally, I look after improving the patient experience, trying to fully understand what it’s like to be a family at Great Ormond Street Hospital and improving their journey.

As Chief Nurse, how do you keep on top of what nurses are experiencing on the front line?

It would be very easy to get disconnected, so I put a great deal of effort into ensuring I know what our nurses experience on a daily basis. Having been a nurse for many years undoubtedly helps, and I don’t believe you could do this job without that experience, but I still have dedicated time in my diary to get out onto the wards and speak to staff. I often visit the wards in the evenings as it’s a good time to speak to people properly when it’s quieter. I also mentor a number of nurses and make myself as accessible as possible. It’s my favourite part of the job. Getting out on the wards and talking to families and nurses is where I draw most inspiration from.

What is Great Ormond Street Hospital’s role in educating nurses?

In partnership with London South Bank University, we’re the largest training hospital for children’s nurses in the country. We can have at least 150 student nurses here at any one time doing their clinical placement, which is a vital part of their education. Given the large number of student nurses, we have to invest a lot in supervision and mentorship to ensure we produce the high-quality nurses of the future. By delivering a top-class education, the nurses are more likely to stay with us in the future and if they don’t then they’ll still be taking the high standards set by Great Ormond Street Hospital to different hospitals around the country.

What makes a good nurse?

We’ve developed a vision for nursing that reflects the Chief Nurse for England’s Six Cs – care, compassion, competence, communication, courage and commitment.

From speaking to children, families and nurses we’ve established the essential characteristics of a Great Ormond Street Hospital nurse. I think the most important thing is compassion. Families can be living here for long periods under a lot of stress.

It’s our job to make it as smooth and easy as possible and that’s what really drives me on a daily basis. An ability to show compassion, to recognise the

stresses a parent may be under when their child is ill – these things are more important than exemplary exam results.

How is the nursing department performing?

In last year’s annual patient satisfaction survey, 95 per cent of families reported that they had trust and confidence in our nursing staff. I’m very proud of that – very proud to achieve that level of satisfaction and that is what keeps me motivated.

I want to maintain Great Ormond Street Hospital’s history of excellence and push it even further.

How is nursing different in England compared to across the world?

While hospitals naturally have a power hierarchy, here in Britain nurses’ relationships with doctors are more equal. This is partly down to the great investment we make in nurses’ education. I think it’s really important that nurses are able to speak up when they feel it’s the right thing to do as they’re the people at the bedside on a day-to-day basis. In a hospital like Great Ormond Street Hospital, where many consultants can be world leaders, it can be daunting for our nurses but we encourage them to speak up for the benefit of the children in our care.

Also, Britain is quite unique as we are the only country to train nurses to specifically look after children and young people.

Other countries are now looking to follow suit but it’s one of our founding principles at Great Ormond Street Hospital. Children need to be cared for differently to adults and we need to train staff to be able to care for them in this distinct way.

What are the specific challenges of nursing children?

A key challenge is learning to nurse in a partnership with the child and family.

You need to really get to know the family and understand how things are for them.

However, it’s also important we keep an appropriate distance. Our role is to be the professional voice rather than a friend.

We need to help the family on the journey to caring for their child themselves. A part of this is that the children often can’t communicate what the problem is in the same way an adult could. As such, our nurses have to be able to step back and ask themselves whether this is

“Families can be living here for long periods under a lot of stress. It’s our job to make it as smooth and easy as possible and that’s what really drives me on a daily basis.”

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Me and my…

normal behaviour for the child given their age and condition.

Another challenge is that we encourage families to be involved in their child’s care and be in the hospital as much as they wish. As a result, a lot of our nurses operate in a public environment where their work is constantly under scrutiny.

How does nursing at Great Ormond Street Hospital differ from other children’s hospitals?

Many of our children have had a long journey through the healthcare system before they get here, so families can often be at their wits’ end with worry. As such, our nurses have to build a relationship with families very quickly so they can relieve that anxiety as soon as possible.

Sometimes families can have an expectation that the hospital will be able to fix everything. Sadly, that’s not always true, but that expectation comes from working in a very famous institution. It’s vital that our nurses help manage expectations.

How do you collaborate and learn from other hospitals?

I am a member of the Association of Chief Children’s Nurses, which is an association of senior nurses that meets quarterly to debate key issues that are important in nursing. This has led to developments such as the Chief Nurse for England having a senior children’s nurse as part of her team to offer specialist paediatric advice when it comes to policy development. I also work very closely with children’s hospitals both in the UK and abroad. For example, we are currently involved in an international study looking at the impact of nursing on the patient experience that is being run at Melbourne Children’s Hospital in Australia.

How do you think nursing has changed since you first came to Great Ormond Street Hospital in 1976?

The core values are the same in terms of compassion and ‘the child first and always’, but otherwise it’s changed immeasurably. The relationship with doctors I mentioned before has become more equal and this has led to nurses working in roles that I would never have imagined nearly four decades ago.

Positions such as nurse consultants and advanced nurse practitioners didn’t exist back then. These new roles see nurses doing some work that doctors used to do. It’s more efficient as doctors are a rare resource and should be employed appropriately. By training these nurses to take on more complex work but retain 08 Pioneer

“In partnership with London South Bank University, we’re the largest training hospital for children’s nurses in the country.” the core values of nursing, children will be treated better.

The other big change is the complexity of our nursing work. Many children that we now treat wouldn’t have survived 40 years ago. Through research and technological advancements we’re now able to care for these children; however, they can have complex regimes of care and our nurses have to manage the challenge.

What is the biggest challenge facing nursing?

You won’t be surprised to hear that resource is the biggest issue. Funding is being squeezed in the NHS and traditionally education is one of the first things to lose out. If we want to continue to educate and develop nurses – as we should – we will need to look at smarter and more efficient ways of working so there is enough resource to go around.

How easy is it to recruit high-quality nurses?

Overall, attracting the number of nurses we need at Great Ormond Street Hospital isn’t an issue. The only area we sometimes have a problem is in highly specialised areas such as intensive and neonatal intensive care. As a country we don’t have enough of these nurses, so we look to countries such as Ireland and Portugal to fill the gap. Despite the excellent quality of these nurses, I still want to move to a situation where the country is training

enough of these specialised staff.

The likelihood is that overseas nurses will always return home eventually, so long-term we need to move to a more sustainable model.

Why did the hospital put together a manual for children’s nursing practices?

As a world-class children’s hospital, it felt pertinent to put together a book about what good children’s nursing is, with guidance on policies, procedures and clinical practice guidelines. We’re often contacted by other hospitals for advice, so by putting together an evidence-based manual, drawn on years of experience, we’re able to help more children. The book is aimed at healthcare professionals and the whole ethos focuses on children and young people having different needs to adults and should be treated as such.

“Positions such as nurse consultants and advanced nurse practitioners... see nurses doing some work that doctors used to do. It’s more efficient as doctors are a rare resource and should be employed appropriately.”

The Dietetics team at Great Ormond Street Hospital support children when nutrition and special diets can be used as part of their treatment. The team work alongside all clinical specialties, helping to improve a child’s health, growth and development through their dietary needs. Here, three children introduce themselves and their dietitians:

Hinda, sevenyears-old, Eagle Ward Dietitian name: Kathy Reason I come to the hospital: To help the problems with my gut Hopes: To eat normal foods again Favourite food: Coffee cupcakes Tips: To eat little and often Cool fact: I have five sisters and two brothers

rs- old, a e -y e in n , a z i Fa Elephant Ward

rs-old, Grace, five-yea Rainforest Ward Bahee Dietitian name: body has the hospital: My Reason I come to g down protein difficulty breakin melon and ited food: Water Favourite unlim strawberries and pasta lled food: Chips Favourite contro my and ing with my mum Hobbies: Swimm ers playing with stick at I can Talking about wh Sessions include: ight my height and we eat and plotting n! fu ve a lot of Best thing: We ha

e: James Dietitian nam l: I have to the hospita Reason I come y chest r a tumour in m d growing talle g on weight an in tt Pu : ts en Achievem cream : Chocolate ice Favourite food pecially Oggy es – ing television ch at W y: bb Ho aches and the Cockro s and t different food Next goal: To ea eding tube get rid of my fe

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I have ectodermal dysplasia

George in his hometown of Southend-on-Sea

Sixteen-year-old George has ectodermal dysplasia, a rare genetic disorder affecting the development or function of the teeth, hair, nails and sweat glands. He tells Pioneer his story

What is ectodermal dysplasia?

Ectodermal dysplasia (ED) is not a single disorder, but a group of closely related conditions of which more than 150 different syndromes have been identified. It’s a very rare condition that affects approximately 7,000 people worldwide. ED is caused by altered genes that may be inherited, or normal genes that have become defective (mutated) at the time of conception.

Before a developing baby is large enough to be seen, a layer of cells covers the outside of the body. This surface layer of cells is called the ectoderm. From these cells the skin, hair, nails, teeth, nerve cells, sweat glands, parts of the eye and ear, and parts of some other organs develop. Each of these parts of the body is then called an ectodermal structure.

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“I was only very little, maybe two or three years old, when the first symptoms of my condition became evident. I was having nose bleeds and my parents kept finding small clumps of hair in the bath. Within three weeks it all fell out. They took me to my local hospital in Southend-on-Sea before I was referred to Great Ormond Street Hospital. It was then that I was diagnosed with ED. “ED is not a single disorder but a group of closely related conditions. My variety is so rare there are only two people in the whole country who have been diagnosed with it and they don’t yet have a name for it. ED makes a difference to my daily life in a few ways. I have alopecia and my hair hasn’t grown back since it fell out when I was little. I only started to get a few hairs on my arms and legs midway through secondary school, and eyebrows are only a very recent addition.

“My body struggles to control its body temperature so I can easily overheat or get too cold. Because of this, migraines or passing out due to dehydration is a very real possibility. It means I have to be prepared for the day. If I left the house in a rush before school without a bottle of water then I could become ill. Similarly, in the winter I wear a huge number of layers to deal with the intense cold that my body feels.

“I also can’t grow regular teeth. When they do come through they tend to be joined together so look like much bigger teeth. I’ve got one or two adult teeth but I won’t get any more now.

“My hair, teeth and temperature problems are serious issues but some of the other symptoms can be a bit more left-field.

For example, in contrast to my hair, which doesn’t really grow, I have unusually quick and strong growing nails. I’m forever cutting them back. If I give them a trim on a Sunday, they’ll need redoing by the middle of the next week.

“I’ve been going to Great Ormond Street Hospital for nearly my whole life.

My earliest recollection is of the long train journeys from Southend-on-Sea and then my mum taking me to a museum or gallery afterwards. The hospital has done so much for me. From an early age I developed needle phobia, an acute fear of injections and needles due to the large number I was receiving. Thanks to counselling and the psychologists at the 12 Pioneer

hospital, I’m starting to deal with this issue. Getting behind-the-scenes tours of the hospital where they do injections has helped normalise it and they’ve taught me methods of dealing with the situation, such as meditation. I’m still nervous about needles but I’ve learnt ways to calm myself down if I get panicked.

“The hospital has really helped with my confidence. At primary school I had a few problems, as children sometimes don’t understand how people can be different.

However, the team at the hospital have taught me that not everyone will understand and that’s ok. School is so much better now and I’m doing a BTEC in performing arts at my local college.

Acting is something I really enjoy and I’ve done a few performances as part of the National Youth Theatre. When I’m acting

“The hospital has done so much for me. From an early age I developed needle phobia...Thanks to counselling and the psychologists at the hospital, I’m starting to deal with this issue.”

I’m able to show people that I’m not defined by my condition by transforming myself into different roles.

“Now that I’m 16 I’ll soon be transferring from Great Ormond Street Hospital to Guy’s and St Thomas’. Initially I was very nervous, as I’ve become so familiar with the staff at the hospital. I was worried that the transition would be really difficult.

However, I had an introductory meeting at the new hospital last year that has put my mind at ease.

“As well as acting I’m an avid reader, particularly crime, drama and fantasy.

My favourite author is David Landy who wrote the Skulduggery series, which is set in Ireland and all about magic. I’m also really open to new opportunities.

Thanks to Great Ormond Street Hospital Children’s Charity I had a behind-thescenes pass at the F1 weekend, which was an amazing experience.

“I’ve never known life without ED but I won’t let it rule my life. For example, just because it’s particularly hot or cold I’m not going to let that get in the way of seeing friends or family. In the future I’d love to be a theatre actor like Alex Kingston, who I really admire for her dedication towards the craft of acting. If not, then I’d like to be a drama teacher. Acting has had such a positive influence on my life and I’d like to pass that on to other people.”

George and his mum, Laura

Why I support the charity

By Andrew Taee

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t was actually a chance meeting that led to me supporting the charity for the past 20 years. Nearly two decades ago I was CEO of one of England’s publicly quoted healthcare companies.

As part of my job I attended a lecture at the UCL Institute of Child Health, Great Ormond Street Hospital’s research partner.

As I was attending a lecture next door, the hospital and charity’s chief executives invited me for a tour of the facilities. What I saw shocked me – you had these very ill children and fantastic staff working in archaic conditions. As someone who was building new healthcare facilities at that time, I was horrified that our premier children’s hospital didn’t benefit from similar levels of building stock. As a young father too, I sat in my car after the tour quite emotional at what I’d seen. It was at that moment that I made the decision that Great Ormond Street Hospital Children’s Charity would be my charity of choice.

“I’ve supported the charity in a number of ways over the years. My longest-standing contribution has been setting up the Bishopsgate School Ball and Bishopsgate Clay Pigeon Shoot. As Chair of Governors at Bishopsgate School, I managed to bring the redevelopment needs of both institutions together into a mutually beneficial relationship. Indeed, it’s the fact that both the school and hospital

“I’ve recently included Great Ormond Street Hospital Children’s Charity in my will. I think it’s important as someone who asks other people to support the hospital that I set an example to encourage others.”

benefit from our fundraising that has helped the relationship prosper for so long.

This year will be the 17th annual clay pigeon shoot. Charities are increasingly entering into these strategic partnerships with other organisations, from schools to business, and I think it’s a good thing.

It’s vital that long-term fundraising streams are established. As medicine, research and equipment continues to evolve it requires charity support to ensure Great Ormond Street Hospital retains its world-leading position as a centre of excellence.

“Another way I enjoy helping the charity is introducing friends and work contacts to the amazing work that the hospital does. Very recently I was able to bring Grahame Chilton into the fold as the new Chair of the Tick Tock Club, a fundraising membership club that I helped found.

Bringing influential people like Grahame into the team isn’t easy and the process of asking is an art that the charity’s former Executive Chairman, Nigel Clark, taught me. The reason it’s so difficult is that everyone has their favourite charity so what you’re trying to do is transfer their thought process from what they want to do, to what you want them to do. When approaching someone as successful as Grahame it’s important to show them how good the hospital is, how efficient the charity is, how big the need is and how he can make a real difference. Everyone wants to be associated with something that is successful, doing good for others and knowing that they can add value that really counts.

“As I mentioned before, all successful relationships have benefits for both sides, and my relationship with the charity is no different. I get a lot of pleasure from walking around the hospital and seeing fantastic new facilities that I played a part in funding. As part of the former Redevelopment Advisory Board I was also able to meet HRH The Prince of Wales, which was a great pleasure, and I enjoy

Andrew Taee is Executive Chairman of Dial Partners LLP, an independent finance firm. Formerly he was Deputy Chairman and Co-Founder of Carlton Partners, a firm co-owned with Halifax Bank of Scotland. He lives in Surrey with his wife and has four children. meeting and talking to the world-class medical staff from the hospital. The events I help put on are also great fun. While the main objective is always to raise money for the hospital, it’s important that the guests enjoy themselves so that they keep coming back. It is a lot of hard work and pressure, but with the fantastic team I have helping me it’s something that gives me great satisfaction.

“I’ve really enjoyed the past two decades with the charity, and as a Fundraising Patron I look forward to many more years of helping in the future. I’ve included Great Ormond Street Hospital Children’s Charity in my will. I think it’s important as someone who asks other people to support the hospital that I set an example to encourage others. We all need to help create a real legacy at the hospital so it can maintain its position as a worldleading centre of excellence.”

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W

e all face challenges in the workplace.

They make us work harder, present new opportunities to test our potential, and dare us to take that next leap forward.

This is certainly the case for the surgical teams at Great Ormond Street Hospital, who face new challenges every day. The pressures of work take their toll on us all, but when faced with operating on a small child, that pressure is taken to the extreme. For many, the thought of having to perform any sort of operation would be unfathomable. Could you imagine travelling to work on a busy commute, knowing that in a few short hours, you’ll be performing potentially life-saving surgery on a tiny baby, barely days old, whose life depends on your skill and experience?

How do the surgical teams go about preparing themselves for such a task?

“Approaching a straightforward operation or a complicated operation, it’s the same mind-set,” says Peter Cuckow, Consultant Paediatric Urologist. “It all starts with the first cut or the first stitch, and you just break it down into the steps you are going to take. If anyone stood at the foothill of Mount Everest and said ‘I am going to go straight to the top’, they would probably faint. To achieve anything, you break it down into manageable bits – that way, we can all do it.”

Peter Cuckow, Consultant Paediatric Urologist

Size matters Surgery can be a high-risk experience. At Great Ormond Street Hospital, where surgical teams often employ pioneering approaches to operate on tiny children, the stakes are raised considerably. Pioneer takes a look at the challenges unique to operating on such small patients, and how surgical staff at the hospital deal with the pressures of the job 14 Pioneer

The surgical environment in paediatrics is made all the more stressful by the speed at which things can quickly deteriorate should something go wrong. Anaesthetists in particular have to be prepared to react quickly, before things spiral out of control. “Children are perceived to be more difficult,” says Tim Liversedge, Consultant Paediatric Anaesthetist. “Things can go wrong very quickly: there are lower thresholds from side effects and drugs, and you have to be more precise. But it’s all about attention to detail and working

Tim Liversedge, Consultant Paediatric Anaesthetist

to quite exact standards, which is not to say that adult anaesthetics isn’t like that, but it takes it to an extreme.

“If you think about the most basic thing like putting in a cannula [a flexible tube containing a needle to deliver or remove fluid], in an adult you have a relatively big vein, yet it can still occasionally be challenging. A small child or baby has tiny veins, so inserting the cannula can be more difficult. Working in paediatrics makes all the simple things that we do a bit more challenging. But it’s fun and extremely rewarding to work with kids.”

Fun is the operative word. Talk to any member of the surgical staff at the hospital and they’ll tell you how much they enjoy working with children. But in order to communicate with the patients and their families, members of the surgical team find that they have to continually adapt their approach. That’s not to mention the difficulty in conversing with children who are too young to communicate themselves, to tell the surgical teams when or where they are experiencing pain.

“Nearly every day, you are constantly varying between looking after very small babies or big children in the middle of the age spectrum, which extends from birth to 17 years of age,” says Dr Liversedge. “That’s part of the challenge – you’re always tailoring what you do. When you meet the patient, you speak to them in an age-appropriate manner, which sounds easy, but when

“Working in paediatrics makes all the simple things that we do a bit more challenging.”

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you go from a toddler, to a 16-year-old, to a 12-year-old with autism, you are constantly altering how you present yourself and how you communicate.”

to deliver the best patient care. But when those individuals work together, it allows the surgical staff to overcome tough challenges from the outset.

“Sometimes you are putting the parents at ease as much as you are the child,” continues Dr Liversedge. “Anaesthetists are there the whole time during surgery, so we are involved from before surgery to after surgery, as a patient advocate, looking after the patient, making sure they are safe all the time, keeping them asleep and pain-free, and responding to any kind of emergency. We are there as a vigilant body the whole time.”

“We have a tremendous team ethic here,” says Professor Cuckow. “By the time we get to surgery, we have a pretty clear picture of what is going on with every patient, but that is not without a significant amount of help. A simple solution to a simple problem requires fewer people, but the more complex things that we deal with involve multiple people, and it’s the way those people work together that enables us to deliver the care. If the patients we’ve treated are doing well, then it is really a testament to our ability to work together as a team.”

The bonds formed between patients, families and staff are incredibly important.

It’s easy to forget that not every patient at the hospital can be treated with one simple operation. Some patients have conditions so complex that they will come to the hospital for several operations, so establishing those relationships early on is vital.

“There is a great focus on the child and family here at the hospital,” says Professor Cuckow. “Having a sick child can put a lot of pressure on the family unit, and that is something that the hospital understands and is always looking to improve. We see many of these patients a number of times over the course of their lives. We support the families and we become, in a way, part of the family.”

Teamwork is at the forefront of the hospital’s approach to the patient experience. It’s the responsibility of each individual, from the theatre porter to the scrub nurse, 16 Pioneer

Take for instance an orchidopexy, a common surgical procedure performed by urologists on boys who have an undescended testicle. More than 30 years ago, the patient would spend three days in hospital to have surgery and recover.

Today, the process has been refined so that, following pre-admission and assessment, the patient may only spend six hours in the hospital, including admission, surgery and post-operative care.

“We are always refining what we do as surgical and anaesthetic teams,” says Dr Liversedge. “We do this in a similar manner to Formula One teams or the Great Britain cycling team: improvement through a ‘cumulative effect of marginal gains’, as David Brailsford [British Olympic cycling coach] said. That means trying to improve in every little area of patient care and focusing on patient safety. Even if these improvements or refinements seem only small, the effect of all of these changes together can be large.”

“Every advance brings a need to advance further,” says Professor Cuckow. “If you cure cancer, you are still going to ask the question why it ultimately arose in the first place, and you are still going to want to stop it from even happening.

With bladder exstrophy, where the bladder is exposed on the outside of the abdomen, more than 50 per cent of patients are known to us before birth.

We could look at putting things back together the way they should have been before the patient is born, so that much earlier in the development of the child, the bladder is in the right place and the muscles are together.”

“Sometimes you are putting the parents at ease as much as you are the child.”

The surgical teams at Great Ormond Street Hospital are always looking to make improvements. When it comes to the more routine operations, where surgical practices have remained at a standard for a length of time, it can be difficult to show a headline-grabbing leap forward. But even in those high-volume cases, the teams have been able to reduce the amount of time a patient spends in hospital, making the process more effective, efficient and, above all, safer for the patient.

This, of course, raises a number of ethical questions surrounding surgery, questions that the surgical teams face on a daily basis: when is the best time to intervene and operate on a child? Should a child with a limited lifespan be prioritised for a transplant when they have been on the waiting list longer than a healthy patient?

And just because you can do it, should you do it?

As you read this, a team of highly skilled and dedicated surgical staff members, who have asked themselves questions like these, are carrying out an operation, focused on improving the life of the tiny patient that lies in front of them on the operating table. Their life and thousands of others couldn’t be in better hands.

Far right: Victor Tsang, Paediatric Cardiothoracic Surgeon

Speaking from the heart “In a small baby with cardiac problems, one or two millimetres can be the difference between life or death.”

Tsang. “We believe in the philosophy that the earlier we can normalise a circulation, the better.

This is the reality that Victor Tsang has to face every day in his role as Paediatric Cardiothoracic Surgeon at Great Ormond Street Hospital. During his time as a cardiac surgeon, Mr Tsang has witnessed a change – not necessarily in the complexity of the heart defects, but in the landscape of the patients he operates on.

“The risks of operating on very small babies are high, but we happen to be very good at it. We deliver highquality services and the outcomes are spectacular.”

“We are dealing with an increasing number of very small babies, with a lot of complex heart defects, and we are operating on them earlier,” says Mr Tsang. “In a newborn or premature baby, the organ tissue is quite fragile and the organs can still be maturing.

The fact that the patient is becoming so unwell means that they have very unstable circulation. That would often require ventilation and intensive care, which carry risks like infection or brain damage.

“The whole package can cascade out of proportion to the weight of the baby: the smaller the baby the bigger the challenge.”

The ability to perform surgery on such a small patient, and weighing as little as 1.9kg, requires an enormous amount of skill, understanding and support. “In a two-kilo baby, can you imagine the size of the heart? We’re talking the size of the baby’s fist, a walnut,” says Mr

With more than two decades of experience in cardiothoracic surgery, you have to wonder whether someone in Mr Tsang’s position would still get nervous when embarking on an operation. When asked by a visiting surgeon how he was able to make an operation look so simple, Mr Tsang replied: “It took me 20 years.”

“It is quite a privilege to be able to do this sort of work; it is a very highpressured job. Mentally, one has to be very strong. There are not that many professions I can ever imagine that would need to deal with life and death within a very short time. It is a major responsibility to look after such a small baby, and in the back of our minds, we always think of the parents.

They carry the baby to the operating theatre, and hand over their most precious offspring to strangers. The parents have to trust those strangers to deliver the outcome we would all like to see.”

Unfortunately, there are those rare occasions when the surgical teams are not able to arrive at a positive outcome.

“In a two-kilo baby, can you imagine the size of the heart?

We’re talking the size of the baby’s fist, a walnut.”

How does Mr Tsang move forward when faced with that experience?

“When a patient passes away, it’s very difficult. Surgeons are self-critical by nature, so we do think about things greatly. In private, I’ll think ‘could I have done things differently? Could I have operated on a different day? Did we miss something?’ It can be very emotionally draining to face fatality, especially with children. But we need to learn each time so that the next time, hopefully, we’ll be able to improve.”

The resolve of surgeons like Mr Tsang to constantly improve means that those cases where patients pass away are very rare. Last year, the cardiac team performed 700 heart surgery cases, with an early survival rate between 98 and 99 percent. “We always need to remember that the cardiac unit here offers the last hope for families with very difficult problems,” says Mr Tsang.

“With that in mind, we always try to help.”

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The team:

Leah Moffat Senior Practitioner Social Work since 2011 • M y role involves managing social workers and family support officers, as well as working directly with patients and families. We receive referrals from the medical teams and then assess families needs and help manage risk.

Psychosocial and Family Services The Psychosocial and Family Services department supports children, families and staff to cope with the illness, treatment and, in the worst case, loss of a child. The department works closely with medical teams throughout the hospital and comprises a wide range of highly skilled professionals, including social workers, paediatric psychologists, the Chaplaincy and Spiritual Care team and Bereavement Services

Clinical psychologists need to understand the medical specialty involved, so the likely impact of diagnosis and treatment can help us to anticipate how a patient will react

Mandy Bryon Consultant Clinical Psychologist since 1990 • W ith Daniela (overleaf), I jointly head the Paediatric Psychology team, which specialises in caring for the psychological and emotional wellbeing of children and families who have a medical condition.

• S ometimes providing psychological support has a subtle impact – a lot of what we do is preventative. Clinical psychologists need to understand the medical specialty involved, so the likely impact of diagnosis and treatment can help us to anticipate how a patient will react, rather than waiting for problems to occur.

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• A unique challenge for our team can be dealing with high levels of distress, conflict and emotion. We need to take those high levels and bring them down to something that is containable, so that the children and families can carry on with their lives.

• I relocated to London from Wales due to my husband’s work. I came kicking and screaming because I liked Wales and my previous job. Clearly, 24 years later, I love London and particularly love working here.

• C hildren often come here with very complex medical issues. The support we offer means that medical concerns and social implications are considered to ensure a positive outcome for the child. We also react to any concerns the hospital might have regarding safeguarding children.

• W e have some children who have been in hospital for long periods of time, which creates challenges for the family. I help bridge the gap between the medical teams, community resources and families. It’s a very positive experience when a patient leaves the hospital and you have helped with their safe discharge home.

• S taff and patients may not know that I perform a mean Haka!

Adam Fearon-Stanley Social Worker since 2012 • I ’m part of a team of social workers that are allocated to specific specialties. I came to Great Ormond Street Hospital because I’m interested in this multidisciplinary team working, particularly for children with complex needs.

• W e are vital to the hospital because families can be especially vulnerable during their child’s treatment and the experience of being in hospital has a massive impact on them emotionally, practically and financially. Many children visit the hospital from all over the country and world, which can be difficult for them, so it’s important that these

challenges are recognised and that we support every family.

• T he best thing about working at Great Ormond Street Hospital is getting positive outcomes for children. Sometimes families aren’t getting the support they should because they haven’t been assessed by local services, or they don’t know where to go for help. We can help signpost families to the right place to help them resolve issues like housing difficulties, financial obstacles or simply receiving lunch vouchers.

Penny Sherrington Anglican (Church of England) Chaplain since 2003 • T he Chaplaincy team provide pastoral, spiritual and sensitive care to patients, families and staff. We are here for all religious faiths and we also help people who don’t have a faith, offering whatever support is appropriate for them at the time.

• T he most important part of my role is to listen and simply be there. We try to really hear the person’s story because often there aren’t any answers. Our support extends to pastoral care and I’m still in touch with a number of families who have since left the hospital. We also support all hospital staff and teach

student nurses about bereavement and how to cope with grief.

• W e’re a very busy team but we support one another. Being on call for the entire hospital can be challenging, especially when you walk into an unknown situation, but we approach every family sensitively and lovingly.

• O ne of my fondest memories was leading a baptism in Wales for a patient who was seriously ill. I went there as a surprise to the family and that was incredibly special.

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Gurpreet Kalyan

he best thing about working here is T that everything we do is child-focused and child-friendly

Psychosocial Liaison Worker since 2010 • I provide emotional and practical support for children and families by helping them cope with diagnosis and treatment, both in the hospital and at home.

• It’s important for our team to be approachable to both families and medical teams. We need to be understanding and help children feel empowered and confident in an unfamiliar hospital environment.

• T he best part of my job is working closely with some amazing children and pulling together as a team to put their best interests first. Children are so brave and always manage to put a smile on my face.

Rachel Cooke Bereavement Service Manager and Child Death Helpline National Manager since 2010

• O utside of work I go to yoga classes, which are great for relaxation.

Daniela Hearst Consultant Clinical Psychologist since 1989 • O ur service helps children and families adjust to diagnosis, treatment and everyday family life. In essence, we help ordinary families deal with extraordinary circumstances, before, during and after their time in hospital.

• C hildren can stay at the hospital for relatively short periods of time, so we then have to work quickly to ensure longer-term support is in place – either by us, local services or schools.

• A s part of the multidisciplinary team, we play an important role in decisions regarding the timing and delivery of treatment, as well as how to help a child prepare for it. Where treatment

Romana Kazmi

• T he Chaplaincy is a non-clinical area, where everybody can come and have space away from the medical environment. Some children stay at the hospital for a very long time and families 20 Pioneer

• I will always remember seeing a terrified boy who needed a lumbar puncture, which can be a particularly difficult and painful procedure. I had not met his family before and had no time to prepare, but we worked together to overcome his fears. After his procedure, the child was very proud of his achievement, as was I. It was a magical moment.

We provide a connection to their family network and acknowledge their special festivals

Muslim Chaplain since 2012 • G reat Ormond Street Hospital has a large number of Muslim patients who I work with, but I also support multi-faith families as well. Many people come here under a lot of stress and their situation can constantly change, so we are here to support them on their journey.

is elective, for example a patient with a craniofacial condition who might be offered appearance-changing surgery, we help families decide what, how and when those changes are made.

can be in isolation, especially if they come from overseas and language may be a barrier. We provide a connection to their family network and acknowledge their special festivals, which can provide a lot of comfort, so the hospital becomes like a second home.

• I feel proud to work at Great Ormond Street Hospital. We have a fantastic network of staff who respect and understand one another.

• G reat Ormond Street Hospital holds a very special place in my heart. I did my training here in the 1980s and came back to work in the Bereavement team three years ago, following my career as a nurse.

• W e help families for as long as they require, offering face-to-face, written and telephone support. We can also complete memory photo albums or boxes to help keep the memory of their child alive.

• M y role involves working closely with local authorities, the psychosocial teams, the police and other health professionals when a child dies. I am also involved in updating policies and procedures around bereavement services for the whole hospital and ensuring that parents have all the information they need.

• T he best thing about working here is that everything we do is child-focused and child-friendly. They are at the heart of it and I’m a big kid myself, which helps!

Jamie Johnstone Bereavement Service Support Worker since 2012 • I help support families and staff around the loss of a child. I also work with bereaved parents who are volunteers for the Child Death Helpline, a freephone national charity that works in partnership with the hospital to offer telephone support to anybody affected by the death of a child. We have over 70 volunteers across two cities who answer calls to the helpline and provide this much-needed service on a national level.

• A s part of my role I help train student nurses in end-of-life care and it is always moving to hear nurses say they feel their “duty of care continues” after a child has died.

• I felt especially proud when I was able to help a mother be closer to her young daughter after her son died. We played activities together and they found they were able to express themselves and support each other.

I help train student nurses in end-of-life care and it is always moving to hear nurses say they feel their “duty of care continues” after a child has died

• I think the slogan ‘the child first and always’ captures the ethos and principal value of the hospital. Seeing my colleagues’ compassion inform their professionalism, no matter what their role is, makes me feel privileged.

It’s inspiring to see people work at their very best.

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Milk – guiding the way Cow’s milk allergy (CMA) is a common food allergy among children in the UK. Great Ormond Street Hospital Consultant Paediatric Gastroenterologist Dr Neil Shah was part of a team to develop national guidelines to help quickly recognise and treat the allergy, improving the lives of young children and their families

E

stimated to affect two to five per cent of children in the UK, CMA usually develops within the first six months of a child’s life. A reaction to cow’s milk can appear within minutes of feeding (severe) or after a few days (mild to moderate). The severity of the allergy determines how a child is diagnosed.

“Cow’s milk allergy can significantly affect a family’s everyday life. Watching your child in discomfort and not knowing why is distressing for parents. Going out to a restaurant or a playgroup can quickly become a stressful experience.

The important thing for parents to know is that if it is diagnosed quickly, children can go on to lead normal healthy lives,” says Dr Shah.

“Children with a severe reaction to cow’s milk will immediately begin to show symptoms, such as rashes or swelling, and experience breathing difficulties. This type of milk allergy is life-threatening and can usually be confirmed using a quick and simple skin prick or blood test,” explains Dr Shah.

In 2011, the National Institute for Health and Clinical Excellent (NICE) published clinical guidelines for GPs, dietitians, nurses and other community-based healthcare professionals on how to diagnose food allergies in young children.

What was clear from the guidelines was a need to extend this into more practical guidance for CMA.

The milder, but more prevalent, form of CMA that affects the gut can be more difficult to diagnose. Children can experience anything from vomiting, reflux, diarrhoea and constipation to skin rashes, persistent crying or colic.

Given that these symptoms are common in a whole host of health conditions in infants, along with the delayed nature of the reaction, often as late as 72 hours after feeding, mild or moderate CMA is more complex to identify.

At the same time, recent evidence indicated significant under-diagnosis, delayed diagnosis and incorrect diagnosis of CMA. “If a child first shows symptoms at three months but diagnosis takes place three-and-a-half months later, you fall into the window of when a child is being weaned. This is a fundamental time in a child’s life and has huge implications if the allergy is not picked up quickly. It was clear there was significant room for improvement

22 Pioneer

Dr Neil Shah, Consultant Paediatric Gastroenterologist

in the identification and management of CMA in the UK,” says Dr Shah.

Keen to tackle this problem, Dr Shah joined healthcare professionals and other doctors from across the UK. The group consisted of an allergy specialist dietitian, a consultant paediatric allergist, a general paediatrician and a GP. “Working as a multidisciplinary team was essential in pooling everyone’s skill and expertise,” emphasises Dr Shah.

The group set about developing practical guidelines for mild CMA to complement

the NICE food allergy guidelines. The aim was to develop a complete package that aimed to improve diagnosis, provide better management for the allergy and give clear guidance on how to re-introduce foods into a child’s diet.

The group undertook a careful review of all current milk allergy guidelines published in the last five years, as well as the NICE guidelines. They also drew on their own experiences of children under their care. Their efforts culminated in the publication of The Milk Allergy in Primary Care Guidelines in 2013.

“The guidelines are carefully designed to help GPs and other primary care providers recognise all forms of CMA and to put in place the most effective management for children with mild forms of the allergy. They take into consideration the busy nature of GP and health visitor practices and have been developed to be used in under five minutes,” explains Dr Shah. The importance of the guidelines led to them being featured on the front cover of the BMJ (formerly the British Medical Journal) and in the British Journal of General Practitioners, vitally raising awareness among professionals who will use the guidelines.

Harms of screening for pre-dementia Urinary incontinence in women Atrial myxoma: a patient’s journey Economic recession and suicide 347:1-40 No 7925 ISSN 1759-2151

21 September 2013 | bmj.com

Cows’ milk allergy in Children

Dr Shah is quick to highlight the importance of the post-publication effort. He adds: “The publication of the guidelines is just the first stage. The key now is to translate this into real-life practice and improve the diagnosis and management of children with CMA.” To increase awareness of the guidelines, the team are actively disseminating them through various channels. Dr Trevor Brown, a Consultant Paediatric Allergist, continues to work closely with GPs in the Northern Ireland group, while Dr Shah is running national teaching courses through the Academy of Paediatric Gastroenterology. At the same time, the team continue to keep in mind the time constraints of those

“Cow’s milk allergy can significantly affect a family’s everyday life. Watching your child in discomfort and not knowing why is distressing for parents...The important thing for parents to know is that if it is diagnosed quickly, children can go on to lead normal healthy lives.”

In May and June 2013, the guidelines were trialled across Northern Ireland by a group of 1,500 GPs who used them to treat babies and children suspected with CMA. “Within the next year we will know the impact these guidelines are having, but we already have early evidence showing a significant reduction in prescribing unnecessary medication and inappropriate formulas. For example, there has been a dramatic reduction in prescribing colic relief formulas, such as Infacol, as people begin to correctly recognise CMA,” says Dr Shah.

The acid test, though, lies in measuring improvements in the quality of life for children and their families. “This is the first set of CMA guidelines that is truly aiming to assess impact. We would like to look at long-term impacts on growth and wellbeing of children because of earlier diagnosis. At the same time we will also assess improvements in aspects of everyday family life.”

involved in primary care and so are using online tools such as YouTube as another quick and readily deliverable portal for publicising the guidelines.

Reflecting on the wider impact of the team’s efforts, Dr Shah adds: “If we can prove these guidelines allow children and their families with CMA to lead fulfilling lives, we will be in a better position to implement this across the UK.”

“Within the next year we will know the impact these guidelines are having, but we already have early evidence showing a significant reduction in prescribing unnecessary medication and inappropriate formulas.”

Pioneer 23

A family’s story of life with a gastro condition

Working out Pioneer sees how a successful collaboration between the Cystic Fibrosis Unit at Great Ormond Street Hospital and private sector gyms is benefitting patients

treatment of children with CF typically consists of a combination of regular airway clearance and inhalation therapies, and exercise. Occasionally, when required, children may be admitted to hospital for intensive intravenous antibiotic treatment.

Brother and sister Samuel, 11 and Heather, six, have been coming to Great Ormond Street Hospital with eosinophilic gastroenterocolitis, an allergic gut that causes inflammation of the large and small intenstine, since they were born. While Sam and Heather have wider problems than cow’s milk allergy, the delay in recognising that they were allergic made the early months more difficult.

“I had a normal pregnancy and birth with Samuel,” says Amanda, their mum. “However, he had cradle cap, was difficult to breast feed and the more we introduced his bottle the worse he was getting. We were back and forth with our GP who thought it was infantile eczema and prescribed different feeds and creams. He really was very ill with eczema, chronic diarrhoea, back arching and was constantly being sick and crying.

The first seven months were incredibly difficult as we didn’t know what was wrong and he wouldn’t sleep. Seeing other parents who had children at the same time and whose babies had started to settle was very difficult. It only served to confirm how different Samuel was.

“After seven months of countless doctor appointments Samuel was failing to thrive, armed with some basic information from the internet, 24 Pioneer

I went to my locum consultant and said I felt Samuel needed to see a gastroenterologist. On cue, Samuel was sick on the floor on the consultant’s shoes! It was at this point that we were referred to Great Ormond Street Hospital.

The first thing Dr Elawad said after our consultation was ‘you’re right, something is wrong’. As bizarre as this sounds it was a huge relief! After seven difficult months, someone was agreeing with us as parents that Samuel’s behaviour wasn’t normal.

We were sent home with a specialised formula, Neocate. As a result, we could now get nutrition into Samuel. It was only the start of a very big mountain for us but it was a huge relief to finally be recognised.

“Five years after Samuel was born, we had his sister Heather. We didn’t know that Heather would have the same problems as her brother and for the first six months I breast-fed her while I was on a restricted diet and she was quite well. However, after six months, the telltale signs appeared and she was also diagnosed with eosinophillic colitis.

“It’s been life impacting over the 10 years since Samuel was diagnosed, living with the constant ongoing challenges and symptoms of allergies, chronic multiple intolerance, seasonal infections, behavioural and joint problems. Samuel

Samuel has much wider problems than Cow’s Milk Allergy which makes things more difficult. However, without doubt if his problems with milk had been diagnosed earlier using the guidelines now available then it would have made things easier in the early months. has much wider problems than cow’s milk allergy which makes things more difficult. However, without doubt, if his problems with milk had been diagnosed earlier using the guidelines now available then it would have made things easier in the early months. The first six months with any baby can be hard but one that cries constantly and is ill is very tough.

Hopefully the guidelines will help local healthcare services that naturally don’t have the same level of specialisation and ensure other families don’t go through the same thing as us.”

Dion and Sean working out

I

f you don’t ask, then you don’t get,” says Sean Ledger, Lead Paediatric Respiratory Research Physiotherapist of INSPIRE-CF, a research programme investigating the potential benefits of exercise in children with cystic fibrosis (CF). He is referring to the 45 requests he’s made of private sector gyms in London and surrounding counties to provide free gym access for children with CF. “We’ve had a 98 per cent success rate from our requests. Generally, centre managers have only said no if they have very stringent corporate rules about under 16s exercising in their gyms.”

CF is an autosomal recessive inherited disease, such that if each parent has one copy of the recessive gene, then the child has a one in four chance of having CF. The disease affects multiple organs, especially the lungs and digestive system, and is characterised by chronic lung infection and inflammation and digestive problems. Physiotherapy

Sean and Dr Eleanor Main, Senior Lecturer in Physiotherapy at the UCL Institute of Child Health, are leading research into an alternative model of physiotherapy care based on a previous successful pilot study, the Frequent Flyer Programme (FFP), which involved encouraging children with CF to regularly engage in high intensity exercise, and more regular monitoring of their nutritional intake.

The results were impressive. “With the pilot we purposefully chose the sickest group of children as they were spending considerable periods of time in hospital.

These children were spending many days off school and away from their homes.

We were looking for a way to reduce the amount of inpatient care they required.”

“In the FFP I personally trained the 16 children on a weekly basis. By using the gyms I’d arranged to support the venture, we were able to build up the children’s overall fitness. The results of gold standard exercise testing showed that we’d significantly improved global fitness by more than 50 per cent in some patients. The subsequent improvement in the children’s health was fantastic, with children telling us they were able to exercise at the same level or higher than their peers. They also spent less time in hospital, which is so important for helping them maintain a normal lifestyle.”

With the success of the FFP assured and having been published in the Journal of Cystic Fibrosis, Sean launched INSPIRE-CF, funded by Great Ormond Street Hospital

Children’s Charity. “This study involves 70 children and we’ve now got four specialist trainers working with the children. We started the project in May 2012 and it’ll run for three years in total. It’s slightly different from the pilot in that we’ve opened the programme up to children with a wider range of disease severity.

While the FFP successfully showed the difference we can make to the most ill children, if we’re to roll this out on a much larger scale we need to prove it can work for most children with CF. Great Ormond Street Hospital’s CF Unit is currently the only Unit that offers this innovative programme, but if INSPIRE-CF can be proven to make a difference to these children, then we’d hope that this model be adopted as standard clinical practice.”

Good for children, good for the NHS As the health of children involved in the first pilot improved, it reduced the amount of time they spent in hospital, with financial benefits for the NHS. The smaller pilot alone saved the NHS over £220,000 (without startup costs). Hopes are high that the current project INSPIRECF, may show similar savings: Cost per patient: Pre study

Post study

£60,244 £46,472

Benefit per patient £13,772 Start-up costs £100,000

Pioneer 25

A parent’s perspective:

R

which they believed was growing on top of his spinal cord. Due to the awkward position of the tumour, surgery had to be performed with Rudy sitting upright and the surgeon standing behind him.

udy was five years old when he suddenly started being sick for no apparent reason.

Whatever food we gave him he would throw back up, so we took him to our local GP who suspected there might be a problem with his stomach.

For the next five months, Rudy didn’t eat very much and had no energy. The spells of sickness became more frequent and lasted for longer. On particularly bad days Rudy would throw up six times a day. This got progressively worse until Rudy couldn’t attend school anymore, which was really hard because he missed his friends. As well as nausea, we noticed that Rudy had started rolling his head and slapping his feet as he walked – he looked like he was in a lot of discomfort. Rudy had lost the cheeky and charming character that we knew and loved.

After a particularly harrowing holiday in Cornwall, in which Rudy didn’t eat for three days, we sat down and decided that enough was enough. We saw Dr Warren Hyer, a private consultant paediatrician, who asked us to diarise Rudy’s symptoms and suggested a possible diagnosis called cyclical vomiting syndrome. However, after watching video footage of Rudy’s unbalanced walking, the consultant instructed us to pack a bag and go straight to the A&E department at Chelsea and Westminster Hospital, where they wanted to do a scan of Rudy’s brain. As his dad it was so scary. We’d always been concerned about Rudy’s stomach and now they were talking about his brain. I didn’t understand where this was coming from, but with Rudy getting increasingly worse I was happy that we were at least moving forward.

My heart sank as they explained that Rudy had a sizable tumour and that this was stopping the flow of cerebral spinal fluid to his brain. They said we needed to go straight to Great Ormond Street Hospital for an operation to release the

26 Pioneer

in rude health

The operation took a nerve-wracking sixand-a-half hours. Afterwards, there were a few complications and Rudy had to stay in recovery for quite a while. His condition worsened and Rudy was unable to feel the right side of his body. A scan revealed a blood clot had formed in his brain, which was almost the same size as the tumour and, during surgery, a lot of air had entered his brain. Mr Aquilina operated once again to release the air and remove the blood clot. pressure building up in his brain. I’d always associated the hospital with really sick children, so I felt incredibly scared.

We arrived at Great Ormond Street Hospital and went to Koala Ward, the neurosciences ward, where we met the most reassuring and calm team of staff. It felt like one of their jobs was to look after the parents as much as it was to look after the children.

Our surgeon, Mr Kristian Aquilina, spent time explaining what was going to happen next. He understood what a shock it was to hear all this new information.

Rudy had his first operation the next day.

The doctors released the pressure in his brain and took a sample of the tumour for examination. Tests revealed that Rudy’s eyes were in a very bad way and Consultant Ophthalmologist, Mr Richard Bowman, explained that if the tumour had been left any longer, Rudy’s vision would have been compromised. The seriousness of his condition was just setting in.

Finally, after 10 days, we were told that Rudy had a rare kind of benign brain tumour called pilocytic astrocytoma.

Previously, chemotherapy had been mentioned as a treatment option, but now we were told that Rudy needed to have a risky operation to remove the tumour,

The following days remained worrying as Rudy’s recovery was slow and variable.

One week later though we had our first good day with no headache, temperature or vomiting – Rudy was on the up at last.

During Rudy’s recovery, the nurses on Koala Ward were fantastic. They all fell in love with Rudy and looked after him as if he was their own. I’d frequently find the nurses dancing around Rudy’s bed and making him laugh. Whether it was the volunteers that played with Rudy so I had time to talk to the consultants, Mr Aquilina popping in on his weekend off or Syed, the cleaner, who helped me change Rudy’s sheets, everybody worked together to make Rudy better. I’ve counted over 75 people who helped Rudy in one way or another, and I can’t thank each of them enough.

The future looks good for Rudy. Our consultant recently told us that we don’t need to go back to hospital for another six months, which is a great boost.

Children are the centre of everything Great Ormond Street Hospital does. The best is normal for them, and yet they still want to do better. It’s the most humbling place I have ever been in my entire life. Without them Rudy wouldn’t be here today.

Rudy and his dad, Sam

Pioneer 27

Great Ormond Street Hospital Children’s Charity 40 Bernard Street London WC1N 1LE 020 7239 3000 www.gosh.org Great Ormond Street Hospital Children’s Charity.

Registered charity no. 235825.

1 Pioneer