living with spina bifida multidisciplinairy clinics 1978-2013
Carla Verpoorten, MD Myleen Christiaens Spina Bifida team Child Neurology University Hospitals K.U. Leuven
Living with spina bifida
1978
2011
What did we learn in 33 years of collaboration between patients, parents and professionals ?
Milestones in history of spina bifida •
1956 : Spitz-Holter shunt
•
1971 - 1973 : selectiecriteria ? to treat or not to treat ? 1970 : need for coordinated care for spina bifida children
•
1984 ; need for coordinated care for young adults
•
1991 primary prevention with folic acid
Milestones in history of spina bifida prenatal detection and need for prenatal counseling
Milestones in history of spina bifida 1978……
…..to 2005
Multidisciplinary Clinics
two major goals • •
to prevent the physiologic dysfunction’s from becoming disabilities to prevent the disabilities from becoming handicaps
achieving these goals requires comprehensive coördinated care
multidisciplinary team care
2005
multidisciplinary team care step by step
1978-1988
Growing pains‌
The Spina bifida Association ( parents and patients) made important contributions to the realisation of the "Convention Spina bifida" with the in Belgian Governement in May 2005
2013
2005
2013
Prerequisites for a Convention between the Hospital and the National Health Service • Ensure affordable health insurance • Financial support from National Health Service to the multidisciplinary centres ( 3 in Belgium) • Convention accorded when there is proof of extensive experience (at least 50 patients) • Training a multidisciplinary team and increasing knowledge about spina bifida
Who has access to the convention? • Children : – spina bifida aperta – spina bifida occulta – spinal cord injuries when it occurs in the first 2 years of life • Adults : – adults from the transition of the pediatric care – with spina bifida who were not yet in a multidisciplinair follow-up
What are the commitments of the convention? -for the patientvisit to the spina bifida clinic depending on the age of the child 0-3 years: 3-4 times a year 3-18years: 2 times a year >18year : 1 time a year
What are the commitments of the convention ? -for the multidisciplinary team• Attending the clinics • Attend the weekly meetings • Making a treatment and rehabilitation plan • Be the go between for the care in the home situations • Coordination of care with input of the individual and family • Gain knowledge on spina bifida
Next step in team coordination
Added value of teamwork •
1978-2013 :
Lissening to parents/patients and answering their questions
Recent concepts in rehabilitation
• in all children with spina bifida functional goals should be established • surgery only to correct or to prevent deformity to promote function
Functional goals these goals vary • with the severity of motor and sensory deficit, • with the child’s developmental progress.
Goals of orthopaedic management • to establish a stable posture
• to maximise mobility - mobility implies more than walking - mobility is essential to attain : social maturation educational and vocational goals
Goals of physical therapy
• to minimise contractures and spinal deformity
• to assist the child in normal development
Tools of physical therapy : orthoses and adaptive devices serve : to improve function and to prevent deformities
a wardrobe of devices : a child who does not sit, crawl, stand, or walk should receive an appliance to assist in any of these activities
Goals of occupational therapy • improving the child’s ability to function as independently as possible • to facilitate the child’s performance of self care, work and leisure in the context of daily life • failure to develop social skills may lead to “secondary disability”
For independent living, other skills are more important than walking
• in spite of their disability it is important for them to develop their bodies, their minds and their social abilities as much as possible. • certain ‘adaptive aids’ can be used to help paralysed children go through the same stages of development as able-bodied children, at close to the same age.
For independent living, other skills are more important than walking • often these children are late in learning basic skills for self-care. - this is partly because of the disability. - but it is also because of their parents often overprotect them
• it is important for parents to help these children to do more for themselves
Transition to adulthood starts from birth on! Avoid learned helplessness!
What is the future for a child with spina bifida ? • is he/she going to be able to walk ? • will he/she be able to go to school ? • and what about incontinence ?
Prognosis for cognitive function 3/4 of the children have IQ > 80 2/3 of these are in mainstream education
however, the frequency of learning disabilities is likely to be high
Learning disabilities
What is the future for a child with spina bifida ? • is he/she going to be able to walk ? • will he/she be able to go to school ? • and what about incontinence ?
Neurogenic bladder *occurs in 90 % of children with spina bifida *90 % of newborns with spina bifida have normal kidney function and drainage *if left unattended over the next 5 years, over 50 % will have upper tract deterioration *1/3 of children who develop impaired drainage of the kidneys do so within the first year of live
Neurogenic bladder management
• early detection of the infant at risk • and early adequate treatment : clean intermittent catheterisation (CIC) + oxybutynin, are the cornerstones of the management to achieve renal protection and social continence.
Neurogenic bladder management
A
• • • •
B
C
A = safe and with CIC B = safe and dry with CIC + sling (+ ditropan ) C = safe and dry with ditropan + CIC D = safe and dry with ditropan + CIC + sling
D
Urological management in the newborn
CIC + oxybutynin started shortly after birth reduces high-pressure detrusor hyperactivity reduces the reflux may prevent the secondary bladder changes and safeguard normal renal function and bladder compliance
Training CIC
Bowel management
• to prevent constipation • to achieve continence : “ trial and error “
Added value multidisciplinary teamwork • More attention to non-medical aspects
Added Value Multidisciplinary Clinics • more attention to early transition, for appropriate education, for better job creation
• prevention ‘secundary disability’
Prevention of secundary conditions decubitus ulcer care spontaneous fractures growth and puberty obesity latex allergy
Added value of multidisciplinary teamwork focus of counseling will change when growing  up: from advice to parents to advice to the young patient himself
Recent concepts in rehabilitation
• training of the disabled to adapt to his limitations • intervening in the environment to facilitate social integration
Transition to adolescence
strategies to help the adolescent with self- image and self-efficacy • discussing diagnosis and impact on personal life • allowing more control in therapy • reinforcing behaviours related to - acquisition of competence - tolerance for failure • helping to make realistic expectations
SPINA BIFIDA LIFE COURSE MODEL WEBSITE (sbpreparations.org)
4 time points: Early Childhood School-Age Adolescence Young Adults
3 domains: Health / Self-Management Personal / Social Relationships Education / Employment / Income Support
Transition to adulthood a number of barriers have been identified : • professionals and society have very low expectations for an individual with a disability • patients are seldom involved in early planning for independent living
The changing incidence of myelomeningocele and its impact on pediatric neurosurgery: a review from the Children’s Memorial Hospital Robin M. Bowman & Vanda Boshnjaku , David G. McLone Childs Nerv Syst (2009) 25:801–806 •
.
the patients are quite hesitant to transition to an adult care team Many young adults have difficulty leaving the treatment team they have known throughout their lifetime, and in many situations, have supported them through life-threatening illnesses. The biggest insecurity expressed by patients and their families in transitioning to an adult care team is a lack of
appreciation for the varied symptoms of a shunt malfunction.
The key to maximising developmental outcomes and fulfilling potential
• is to emphasise strengths and to build on them • think ‘abilities’ and not ‘disabilities’, and focus on the strengths rather than the deficits • to put this principle into practice, professionals and families must work together as an effective partnership
• “ what is the key ingredient to respect for the rights of people living with longterm impairment ? The answer is shockingly simple, it is communication…. • “professionals have to see beyond their case notes; see beyond the impairment; recognise disabled people as carers, parents,employers or potential lottery winners..
As we consider spina bifida through life span
1998
2011
how to promote good health, to prevent problems, and how to improve quality of life at different ages ?
Spina Bifida : Health and Development Across the Life Course pediatric.theclinics.com
Planning for services and supports requires input from an interdisciplinary team of professionals, working closely with the child, parents, and family members. With life expectancy stretching well into the fourth decade and beyond, the approach to childhood service delivery needs to be one that considers the entire life course. Principles of child development and the International Classification of Functioning, Disability and Health offer two complementary approaches to guide services and supports.
http://www.researchgate.net/publication/47155554_C
• “In the Life Course Model the milestones, assessments, and interventions • • • •
are different in each age period. Over the life span, the clinicians taking the more active roles in the transition process may change. In many clinics a nurse acts as a case manager, coordinating care among the various disciplines. In other instances a clinician may find that he or she must assist the individual and family even if care coordination is not his or her primary role. Regardless of who may be involved, the care coordination should always include emphasis on teamwork, loose boundaries around clinical roles, interventions that focus on promoting independence and preserving function, and support for caregivers.”
EVIDENCE-BASED PRACTICE IN SPINA BIFIDA: CURRENT CHALLENGES IN QUALITY CARE A few clinical questions highlighted at that symposium are listed to illustrate the scope of the challenge and opportunity for further important research: •What is the optimal treatment of hydrocephalus? • What is the best method for evaluating and monitoring shunt function? • Which patients would benefit most from tethered cord release? • What is the optimal management of hip dislocation? • How to prevent and manage osteoporosis? • How can animal models be developed to explore neuromodulation of bladder function? • What is the optimal management of reproductive issues in women with spina bifida? • What are the pathophysiologic factors that affect skin breakdown? • What are the instructional and developmental interventions that facilitate learning? • How best to promote optimal psychosexual development and sexual adaptation? • How to effectively teach and promote self-care? Children with Spina Bifida: Key Clinical Issues by Adrian D. Sandler Pediatr Clin N Am 57 (2010) xvexvi
Preparations: Becoming an Adult Living Successfully with Spina Bifida Growing up. •It can be scary for any parent to raise a child and be sure you are providing them with the tools for a successful adulthood. But this can be particularly daunting if your child faces challenges from Spina Bifida. •This site can help! Throughout are interventions and resources from professionals and parents who deal with Spina Bifida every day. What is this site? This site was created to give parents and clinicians tips and tools to help children with Spina Bifida develop into successful and happy adults. It also has information for young adults seeking ways to become more independent and lead a fuller, richer life.
Who is it for? This site was designed specifically to be used by parents of children with Spina Bifida and clinicians. It was also designed as a resource for young adults living with Spina Bifida.
How does it work? By answering a series of questions about your child, or yourself, the site will generate a report specific to your child’s (or your) developmental needs. The report will include suggested interventions as well as resources and tips. View our interactive tutorial.
http://www.sbpreparations.org
From the American Academy of Pediatrics Clinical Report Providing a Primary Care Medical Home for Children and Youth With Spina Bifida Robert Burke, MD, MPH, Gregory S. Liptak, MD, MPH, the Council on Children With Disabilities located on the World Wide Web at: http://pediatrics.aappublications.org/content/128/6/e1645.full.html
The pediatric primary care provider in the medical home has a central and unique role in the care of children with spina bifida. The primary care provider addresses not only the typical issues of preventive and acute health care but also the needs specific to these children. Optimal care requires communication and comanagement with pediatric medical and developmental subspecialists, surgical specialists, therapists, and community providers. The medical home provider is essential in supporting the family and advocating for the child from the time of entry into the practice through adolescence, which includes transition and transfer to adult health care. This report reviews aspects of care specific to the infant with spina bifida (particularly myelomeningocele) that will facilitate optimal medical, functional, and developmental outcomes.
Future prospects : sharing knowledge
An ‘information’ approach M.MILES
• The information needed to make a real change in the lives of at least 70% of disabled people is now known in institutions in over 95% of countries • the means now exists to increase the amount of useful information directly reaching people in all countries, about disability and rehabilitation, at a cost far lower than that of any other strategy • information can be shared and recycled endlessly without exhausting the capital ; in fact the stock accumulates in the process, though this needs monitoring and quality control