Growing Up With Cerebral Palsy: Contemporary Challenges of Healthcare Transition

Abstract

Cerebral palsy is traditionally known as a major cause of chronic disability in childhood. With advances in neonatal care and improved survival, the majority of adolescents with cerebral palsy require ongoing services into adulthood. This paper highlights some of the challenges in their transition from pediatric to adult healthcare and proposes key elements to ensure a smooth transition process.

Full text

Changing trends in the prevalence of cerebral palsy (CP)
Systematic reviews on the prevalence of CP among children
report a median of 2.4 per 1000 children, which has been fairly
stable among various demographics.1In Canada only two
studies have focused on the prevalence of CP in the general
population of children, both using administrative databases
(ICD-9 codes). The first study from Alberta reports on a birth
cohort from 1985 to 1989, with a prevalence of 2.57 per 1000
children eight years of age.2The second study from British
Columbia reports on a birth cohort from 1991 to 1995, with a
prevalence of 2.68 per 1000 live births.3
Studies on the prevalence of CP among premature survivors
have shown mixed results on trends for increased prevalence
over time, coinciding with the improved survival of extreme
premature newborns. There have been four studies in Canada
following prospective cohort of neonatal intensive care unit
survivors in Alberta, Nova Scotia, Quebec and Ontario.4-7 Of
these, two found an increased prevalence4,5 while two found that
the prevalence remained stable despite increased survival in the
post surfactant era6,7. The worldwide literature also shows a mix
of findings, with evidence for decreased prevalence and severity
in very low birth weight infants.8-10 The first ‘special care baby
units’ were established in the 1950s, and surfactant was
introduced in the 1980s, with routine use in most centers in the
late 1980s and antenatal steroids in the early 1990s. These
advances have contributed to improved survival of premature
newborns and the inverse relationship between gestational age
and prevalence of CP has been well established.11 Whether the
prevalence of CP is stable or changing over time, the
characteristics of the population may be changing with different
severities, subtypes and co-morbidities. This highlights the
ABSTRACT: Cerebral palsy is traditionally known as a major cause of chronic disability in childhood.
With advances in neonatal care and improved survival, the majority of adolescents with cerebral palsy
require ongoing services into adulthood. This paper highlights some of the challenges in their transition
from pediatric to adult healthcare and proposes key elements to ensure a smooth transition process.
RÉSUMÉ: Défis actuels de la transition entre les soins de santé pédiatriques et adultes des patients atteints de
paralysie cérébrale. La paralysie cérébrale est connue traditionnellement comme une cause majeure de handicap
chronique chez l’enfant. Avec les progrès des soins en néonatologie et l’amélioration de la survie, la majorité des
adolescents atteints de paralysie cérébrale nécessitent des services à l’âge adulte. Cet article souligne certains des
défis rencontrés au moment de la transition entre les soins pédiatriques et les soins adultes et propose des éléments
clés pour assurer un processus de transition harmonieux.
Can. J. Neurol. Sci. 2012; 39: 23-25
THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES 23
Growing Up With Cerebral Palsy:
Contemporary Challenges of Healthcare
Transition
Maryam Oskoui
From the Department of Pediatrics, Division of Pediatric Neurology, Montreal
Children’s Hospital, Montreal, Quebec, Canada.
RECEIVED JUNE 14, 2011. FINAL REVISIONS SUBMITTED JULY 19, 2011.
Correspondence to: Maryam Oskoui, Department of Pediatrics, Division of Pediatric
Neurology, Montreal Children’s Hospital, 2300 Tupper street, A-512, Montreal,
Quebec, H3H 1P3, Canada.
REVIEW ARTICLE
importance of population-based patient registries in Canada to
better assess the burden of CP in the Canadian population.12
Neonatal graduates of the post surfactant era are only now
becoming adults. Furthermore, increased use of prenatal
neuroprotective magnesium sulfate in Canada with recent
guidelines can potentially decrease the incidence of CP in
premature infants.13
Growing up with CP
Although CP is defined as a static motor disorder, it is
superimposed on the dynamic processes of development and
aging.14 Over 90% of individuals with CP live beyond their 18th
birthday.15 Adults with CP require ongoing health services to
continue to monitor for needs that were present in childhood.
Feeding difficulties, gastroesophagal reflux, constipation,
seizures, progressive scoliosis, contractures and dislocations
secondary to unremitting spasticity continue to be important
medical issues to address. Access to routine care, dental care and
screening are also important, such as cardiovascular health, pap
smears and mammographies. There are, however, a number of
new health issues that emerge in adulthood for this patient

population. Overuse syndromes, chronic pain and fatigue,
osteoarthritis and osteoporosis, and an overall decline in
mobility have all been reported among adults with CP.16
The access to care once individuals are of adult age remains
problematic. A recent study in Ontario on health care use
patterns among 587 teens and 477 young adults showed a greater
rate of outpatient visits among CP patients (x 2.2 in teens, x 1.9
in adults) compared to age-matched peers and a higher rate of
hospital admissions (x 4.3 in teens, x 10.6 in adults). Adults with
CP had significantly more visits to general practitioners and
significantly fewer visits to specialists than teens with CP, with a
trend toward increased number of emergency department
visits.17 In a report on the burden of neurological diseases in
Canada, the Public Health Agency of Canada estimates that total
costs associated with CP in 2000–2001 were $381.8 million,
recognizing that there are insufficient data to assess the full
impact and explicitly calling for more research in this area.18
Looking at the literature on outcomes in adults with CP from
1970 to 2010, a mapping review showed two new important
trends.19Studies reporting on the access to and use of health care
have only emerged since 1997, showing markedly diminished
access after adulthood. There has also been an increased focus on
contextual factors (personal, environmental) over time, with four
studies published in the 1990s and 21 studies published since
2000. Such contextual factors are important determinants of
quality of life in individuals living with chronic disabilities.20
Family functioning, behavioral difficulties, and motivation are
reported as important predictors of social-emotional adaptation.
These factors among others should be targeted in transition
planning.
Transition challenges
Most institutions transfer patients with CP to adult care rather
than transition them. Transfer is the actual responsibility of care
of the patient being moved from a pediatric setting to an adult
care setting. Transition is the “purposeful, planned preparation of
patients, families and caregivers for transfer of a patient from a
pediatric to an adult care setting.”21 Over the past decade there
have been numerous position statements addressing transition as
a priority mandate for those caring for youth with special health
care needs.21-23 The key challenges in transitioning young adults
with CP are common to all individuals living with chronic
disabilities having special health care needs. The individual’s
maturity and cognitive ability, the preparation of the patient and
family, and access to specialists and multidisciplinary care as
adults are but a few of the key issues.
Although most Canadian medical institutions require transfer
of care at 18 years, it is well accepted that age is not the best
indicator of preparedness.24 The individual’s maturity and
cognitive ability are important factors to consider, and preparing
individuals to become increasingly autonomous in their
interaction within their health care environment within a
developmental appropriate process is encouraged. Examples
include making appointments, taking medications, and actively
participating during medical visits. Individuals with significant
cognitive impairment who will continue to be fully dependent as
adults will need additional consideration for proxy decision
making, legal guardianship and estate planning. Preparing
patients and families for eventual transfer to adult care includes
educating them on their medical condition, their medications and
side effects, self-management skills, and learning to navigate the
adult health care system. It is especially important for patients
and other family members to know their medical history when
faced as adults with the reality of aging parents and new health
care professionals who are unfamiliar with them. The transition
process should focus on preparing these patients and families for
a lifecourse, living with a chronic disability and optimizing their
full intrinsic potential.
Although ensuring the continuity of healthcare, with routine
preventive care, dental care and rehabilitation services is a focus
within medical establishments, individuals need to transition in
other spheres that may be just as important. These include
education and employment, finances and benefits, housing,
transportation, leisure activities and relationships. Without
preparation, families, patients and even pediatric health care
professionals can be reluctant to transfer patients with special
healthcare needs to adult care at a critical time when patients are
undergoing many transitions in their life that create multiple
personal and familial challenges. Pediatric and adult health care
environments have traditionally been different in their approach,
with pediatric care being more family-centred and paternalistic.
Parents worry that their concerns will not be addressed in the
adult milieu, that they will no longer be able to advocate
effectively for their adult children or navigate an increasingly
fragmented health care system on their child’s behalf. There is
also a bond with the pediatric health care professional who has
known the family from the time of initial diagnosis. Difficulty
finding a primary care provider is especially a challenge for
these patients who would benefit from having a primary care
provider to coordinate their care. A multidisciplinary specialized
care environment is also needed as a resource, where a
coordinated multifaceted team approach helps avoid multiple
trips and fragmented care. Specialists and multidisciplinary care
clinics for adults with CP are few and far between, and a lack of
knowledge or treatment comfort of adult care providers for CP is
often postulated.25,26 Adults with CP also see their access to
rehabilitation services progressively diminish over time. A
recent study using semi-structured individual interviews with
teens and adults with cerebral palsy, spina bifida, and acquired
brain injuries of childhood, and their parents, asked them to
identify barriers to transition and propose solutions. The top four
barriers were: lack of access to healthcare, lack of professional
knowledge, lack of information provided, and uncertainty
regarding the transition process.27 The two simple solutions
highlighted were more information and more support throughout
the transition process.
Models of transition
Transition of care is a process incorporating the develop-
mentally appropriate preparation of the patient within their
family unit for their eventual transfer to adult care. Effective
transfer of patients necessitates communication between
healthcare workers and sharing of information, going beyond a
simple transfer summary which may not be obtained in time and
lacks details of previous investigations and functional status of
the patient. Patients with CP can be transferred to a number of
different clinical environments: a primary care clinic, a general
adult neurology clinic within the community, a subspecialty
THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES
24

clinic if available, a rehabilitation center, or a joint pediatric and
adult healthcare provider clinic. The latter are increasingly
favored within various subspecialties, where both the pediatric
and adult health care professionals involved see the patients
jointly to ensure a smooth transfer of care. However such clinics
may not be feasible within most busy practices and may be
limited in number and less accessible to those living outside
urban centers. Allowing the patient to return to see the pediatric
care provider after their initial adult care visit may be a
reasonable option, ensuring that there is no gap in care and
providing the family with any needed additional resources within
their community. Several transition checklists are readily
available in the literature and online which can be adapted and
used clinically at various ages.28,29 An evidence-based model and
suggested practice guidelines for the transition of youth with
disabilities has been elaborated in Ontario, and follow-up on
field testing of this model will be of interest.30
The important elements of the transition process are to start
early and plan ahead, prepare for a lifecourse, and involve
families. Benefits of a transition program for individuals with CP
include better continuity of care and information sharing
between pediatric and adult care providers, improved patient
care and education, and potentially reduced long term health care
costs. A transition program also provides physicians and trainees
with an educational opportunity to learn about the process and
evolving care of adults with CP, with the hope of inspiring some
adult care providers to specialize in the care of this evolving and
expanding adult population.
ACKNOWLEDGEMENT
The author thanks NeuroDevNet for the opportunity to
present on this topic during the second Annual Brain
Development Conference, and Dr. Michael Shevell for his
mentorship.
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