The Care of Adults

with Pediatric Onset

DisabilitiesMichael Green, DO

Division of Physical Medicine and Rehabilitation at the University of Utah

Attending Physician at Primary Children’s Hospital and in the Adult

Transitions’ Clinic at the University of Utah

01/29/2015

Cerebral Palsy:

Transitions and Adult Care

Objectives

• Discuss Cerebral Palsy and develop a better

understanding of the issues encountered by

individuals with Cerebral Palsy.

• Discuss the care of these individuals when they

become adults.

• Discuss the process of transitioning a child to adult

care medicine.

Cerebral Palsy (CP)

• Cerebral Palsy is a non-progressive neurological disorder

which occurs before, during or after birth in the first

few years of development

• Cerebral Palsy is the most common major disabling

motor disorder of children

• Cerebral Palsy affects movement and posture, causing

activity limitations

Epidemiology

• In the US there are 3.6 cases per 1,000 among 8-year-

old children1

• Data from the 2004 National Health Interview Survey

indicate that ~1.4 million children with some kind of

activity limitation were in the 14-17 year old age

group, a critical period for transition planning2

• Over 90% of individuals with CP live beyond their 18th

birthday5

Comorbidities

• The degrees of motor function vary greatly among

children and adolescents with CP

• Children with CP, in addition to physical

impairments, may also have associated impairments7:

• learning disabilities

• communication difficulties

• perception and sensation disturbances

• epilepsy

Gross Motor Function

Classification Scale (GMFCS)

Level I move freely in almost all situations.

Level II can walk, run and climb in most

settings but may need held mobility

equipment for safety and wheeled mobility

for travelling long distances.

Level III can walk using assistance and can

self-propel manual or powered wheelchairs.

Level IV can walk short distances with

assistance but use wheeled mobility in most

settings and need physical assistance for

transfer.

Level V require extensive support in all

situations.7

Types of Cerebral Palsy

• Spastic: most common type of CP; reflexes are exaggerated, weakness and discoordination, as well as muscle movement is stiff

• Dyskinetic:Athetotic: Involuntary, slow, writhing movements Dystonic: Trunk movements are more affected than limb muscles,

resulting in a twisted position

• Ataxic: Voluntary muscle movements are not well coordinated

• Hypotonic: Muscle tone is decreased or floppy

• Mixed: Combinations of the symptoms listed above

Distribution of Limbs Affected

Quadriplegia Both arms and legs are affected. The muscles of the

trunk, face and mouth are often also affected

Diplegia Both legs are affected.

Hemiplegia One side of the body (one arm and one leg) are affected

Transitions From

Pediatric Care to Adult Care

• Transitions involve a significant change or set of changes in a person’s life circumstances during a relatively limited time period rather than over the life span.2

• People with CP often achieve fewer developmental milestones, face more restrictions in social participation compared to healthy peers, and may lag behind in education and work, independent living, and relationships.3

TransitioningCritical Steps to Successful Transition from Child-Oriented to Adult-Oriented Health

Care for Youth with Special Health Care Needs.

1. Ensure that an identified clinician assumes responsibility for health care and

planning for youth with special health care needs during the transition period so as

to provide uninterrupted, comprehensive care.

2. Incorporate into physician training and certification the core knowledge and skills

to provide developmentally appropriate transition services to young with special

healthcare needs.

3. Maintain a portable, accessible, up-to-date medical summary to facilitate

collaboration during the transition.

4. Write a transition plan by age 14 years with the patient and family that outlines

services, providers, and payment. Review and update the plan annually and at the

time of transfer

5. Adhere to standard guidelines for primary and preventive care, recognizing that

youth with special healthcare needs may require more resources to optimize their

health.

6. Ensure the continuation of affordable health insurance that provides appropriate

compensation for planning and coordination for youth with special healthcare

needs.

What to address in a Transitioning

Process?

Issues to address during health care

transition

Changes in health insurance

Identification of an adult-oriented

primary care provider

Identification of an adult-oriented

subspecialist

Need for disability-based services

Capability for self-management

Skills in health care advocacy

Health promotion and

disease/injury prevention

The top four barriers

identified:

Lack of access to healthcare

Lack of professional

knowledge

Lack of information provided

Uncertainty regarding the

transition process.5

Adult Providers

• Few adult medicine practitioners have been trained in the care of the patient with CP, and may therefore be uncomfortable providing care.1

• General practitioners, gynecologists and medical students have all been shown to have limited knowledge about CP, which promotes misunderstanding, negative attitudes and stereotyping of patients.14

• Few medical facilities are prepared to treat adults with developmental disabilities.1

• Treating adults with complicated musculoskeletal and neuromuscular impairments is challenging.

Adults with Cerebral Palsy

• Adults with CP require ongoing health services to continue to monitor for needs that were present in childhood.

• Feeding difficulties, gastroesophagel reflux, constipation, seizures, progressive scoliosis, contractures and joint dislocations secondary to unremitting spasticity continue to be important medical issues to address.5

• Adults with the CP often develop musculoskeletal and neurological symptoms, such as severe pain, chronic fatigue, and a premature decline in mobility and function, as they age. 1

Pain in Adults with

Cerebral Palsy

• Twenty-seven per cent of the adults with CP had chronic pain, compared with 15% in the general population.

• However pain did not increase with age, which is different from the general population.

• The most frequent site was back pain, both in adults with CP and in the general population.15

• There is a high prevalence of neck and shoulder pain in persons with dyskinesia.15

• Overuse syndromes, chronic pain and fatigue, osteoarthritis and osteoporosis, and an overall decline in mobility have all been reported among adults with CP.5

Musculoskeletal Issues in Adults

with Cerebral Palsy• Studies of adults with CP have reported pain in 67 to 82%. Back, hip and

lower extremities (knee) were the most common pain locations in many

studies.1

• Numerous musculoskeletal impairments, including patella alta, hip

displacement, spondylolysis, cervical stenosis, scoliosis, foot deformities,

and disuse osteoporosis.1

• Patella alta, most common in individuals with CP of the spastic diplegic type, can cause

anterior knee pain and is complicated by stress fractures, tendonitis/bursitis, and

subluxation/dislocations.

• Hip subluxation/dislocation occurs in approximately 1% of individuals with spastic

hemiplegia, 5% of those with diplegia, and up to 59% of those with quadriplegia.1

• Spondylolysis in weight-bearing adults with CP is reported to be up to 21 to 30%.

• Cervical stenosis, most likely an overuse injury, has been found to be more common in

individuals with CP than in the general population.1

Osteoarthritis was reported as a cause of pain in several

studies; one study found clinical evidence of arthritis in 27%

of young adults participates with CP compared with only 4%

of the general population.1

• Early osteoarthritis is a significant issue in many adults with CP.1

• Joint destruction is believed to occur disproportionately in

persons with CP, particularly of the hip1

.

The epidemiology of osteoporosis and fractures in children

and adults with CP is not well characterized.1

Osteoarthritis in Adults with

Cerebral Palsy

Psychological Issues in Adults with

Cerebral Palsy

It has also been shown that adults with CP tend to present with

higher levels of depressive and stress symptoms when compared

with the general population.15

Physical pain was noted to occupy the thoughts of individuals with CP

and took a lot on their energy level, which reduced their opportunities to

participate in leisure activities, and this resulted in sadness and feeling of

hopelessness.7

Psychosocial issues associated with ageing with CP include the need

for social support, self-acceptance and acceptance by others.12

Fatigue in Adults with

Cerebral Palsy

• Adults with CP reported significantly more physical but not mental fatigue than the general population. In a Norwegian study, 30% of adults with CP versus 22% of the reference population reported substantial fatigue.15

• In a study of individuals with CP, an overwhelming amount of the complaints focused on fatigue and emphasized that fatigue occurred with walking and prolonged activity. Self-awareness of the individuals’ own bodies and adapting activity to continue to participate in various aspects of their lives emerged as the most important theme.8

• Fatigue was most prevalent in persons at Gross Motor Function Classification System levels II and III.15

Medical Care in Adults with

Cerebral Palsy

• Research suggests that adults with CP use preventative care

services less than do persons without disabilities.

• Adults with CP use specialty health-care and rehabilitation services

less, and emergency room cares more, than their non-CP peers.

• Less than half of women with CP have Pap smears, and there have

been no studies addressing the impact of menopause on bone health

in adult women with CP.1

• In Ontario’s healthcare, patterns have shown a greater rate of

hospital admissions among people with CP compared to age-

matched peers.5

Functional Outcomes in Adults with

Cerebral Palsy

• CP has frequently caused a cycle of deconditioning, in which physical function deteriorates, followed by a further decrease in physical activity, and a cascade of functional decline.1

• About a third of adults with CP reported modest-to-significant decreases in walking or self-care tasks, especially in dressing and walking.1

• The reasons for these early changes have similarly not been fully worked out; it is known, however, that the natural history of mobility in CP is one of early decline.1

• Children with CP are less active than their non-disabled peers and have a lower maximum oxygen consumption, muscular endurance, and peak anaerobic power.1

The Workplace

• It has been shown that individuals with physical

impairments tend to be more loyal employees.

• Workplace accommodations may be needed to

enhance continued employment for adults aging with

CP.12

Conclusions

• The diagnosis of CP is often thought of as a pediatric condition; it

must be understood as a life-long disorder.

• Gaps in transition to the adult health system result in poor health

outcomes and diminished opportunities to participate as productive

members of the community.11

• The goal of the Transitions process: Fostering autonomy and

preparing adolescents for autonomous medical decision making. To

maximize “lifelong functioning and potential through the provision

of high-quality, developmentally appropriate (and technically

sophisticated) health care services that continue uninterrupted as

the individual moves from adolescence to adulthood.”2a

More Conclusions

• Exercise and activity are effective means of pain

management.1

• Ensuring that persons with CP remain as active as

possible is of critical importance.1

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