Cerebral Palsy: Critical Elements of Care

CRITICAL ELEMENTS OF CARE

Produced by The Center for Children with Special Needs Children's Hospital and Regional Medical Center, Seattle, WA

Fourth Edition, Revised 2/2006 The Critical Elements of Care (CEC) consider care issues across the life span of the child. The intent of the document is to educate and support those caring for a child with Cerebral Palsy. The CEC is intended to assist the Primary Care Provider in the recognition of symptoms, diagnosis and care management related to a specific diagnosis. The document provides a framework for a consistent approach to management of these children. These guidelines were developed through a consensus process. The design team was multidisciplinary with state-wide representation involving primary and tertiary care providers, family members, and a represen- tative from a health plan.

Original Consensus Team: Leslie Babbitt, RD, MS Jean Popalisky, RN, MN Barbara Boldrin, RN Diana Sandoval, MS, OTR/L Charles Cowan, MD Pat Trulson, PHN Betsey Denonville, RN Stephanie Underwood, Parent Kathy Mullin, RN William Walker, MD Chris Olson, MD

Technical Assistance: John (Jeff) McLaughlin, MD

Content reviewed and updated 2/06: William Walker, MD

DISCLAIMER: Individual variations in the condition of the patient, status of patient and family, and the response to treatment, as well as other circumstances, mean that the optimal treatment outcome for some patients may be obtained from practices other than those recommended in this document. This consensus based document is not intended to replace sound clinical judgement or individualized consultation with the responsible provider regarding patient care needs.

CEREBRAL PALSY CRITICAL ELEMENTS OF CARE

I. INTRODUCTION

Definition and Diagnosis of Cerebral Palsy○○○○○○○○○○○○○○○○○○○○○○○○○○○ 1

Patterns of Cerebral Palsy○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 1

Causes of Injury○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 1

Associated Risks○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 2

Prognosis○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 2

Management of Cerebral Palsy○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 2 ○○○○○ Characteristics of Optimal Care○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 2

Interventions and Treatment○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 3 ○○○○○○○○○○○○○○○○○○○○○○○○○○○ Management Issues○○○○○○○○○○○○○ 3

II. GUIDELINES FOR CRITICAL ELEMENTS OF CARE○○○○○○○○○○○○○6

III. CRITICAL ELEMENTS OF CARE ○○○○○○○○○○○○○○○○○○○○○○○○○○○○7 (Ages 1 - 21 years)

IV. QUICKCHECK WORKSHEET ○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○17

V. APPENDICES I. References and Resources

Professional Reading List○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 18

Information and Organizations○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 18 ○○○○○ Periodicals○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 18

Reading List for Families○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ 19 I. INTRODUCTION TO CEREBRAL PALSY

Cerebral palsy is an excellent example of a knowledge of normal motor development and its medical condition that requires competent, compre- variants. In all but the mildest cases, the diagnosis hensive, continuous, compassionate and commu- of cerebral palsy can be made by 12-18 months of nity-based care. It is critical that primary care age. A thorough search for etiologies is warranted providers (PCP) be involved and knowledgeable in the young child newly diagnosed with cerebral about the current and future care plan for the best palsy. This includes a thorough history and physical, outcome for each child. A team approach is re- plus follow-up observation of the progression of quired to develop this practice model. The team motor abilities at frequent intervals. Physicians members may include community service providers experienced with this disorder, such as developmen- such as public health departments, early intervention tal pediatricians, neurologists and geneticists, can be programs/schools or private therapy providers consulted to aid in the diagnosis. including occupational, physical and speech thera- To aid in confirming the diagnosis and ruling out pists. There is also a need for regional centers neoplastic or progressive causes for motor disability staffed by health professionals who have experience such as metabolic and neurodegenerative disorders, in the treatment and management of individuals with central nervous system imaging is usually indicated. cerebral palsy. The family is at the center of the Other diagnostic testing might include cultures, care team. immune status, metabolic screening, karyotyping, genetic probes or confirmatory tests for other What is Cerebral Palsy? specific disorders. EEGs, EMGs and skull films are not useful for the diagnosis of cerebral palsy or its Cerebral palsy is a group of disorders of move- etiology. ment and posture resulting from injury to the developing central nervous system. The neurologic How are the Patterns of Cerebral Palsy De- impairment is nonprogressive, although secondary disability can occur. Characteristics of cerebral scribed? palsy change with developmental stages, especially Studies have demonstrated that injuries to certain in the first few years of life. This impairment and areas of the brain result in general patterns of resultant disability are both permanent. impairment. This observation has led to the "topo-

○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ graphical" descriptors of spastic forms of cerebral When is the "Developmental" Period? palsy such as hemiplegia, diplegia or quadriplegia, which are thought to be the result of pyramidal tract The developmental period is usually defined from the injury. Certain patient populations will be predis- time of conception to 12-24 months post-term birth. posed to a particular "type" of cerebral palsy, as in the case of premature infants and spastic diplegia. What is the Incidence of Cerebral Palsy? The extrapyramidal types of cerebral palsy (athetoid, The overall incidence in the past 20 years has dystonic, ataxic) will have some involvement of the remained rather stable at 2.0-2.5 per 1,000 live entire body. Many children have a "mixed" clinical births, resulting in 5,000 new cases of cerebral palsy picture, where both spastic and extrapyramidal in the United States every year. To the great features are present. frustration of parents and physicians, no specific What are the Causes of Injury in Cerebral etiology is found in 20-30 percent of these cases. Palsy? How is Cerebral Palsy Diagnosed? The causes may be prenatal (including genetic), The diagnosis of cerebral palsy is essentially perinatal or postnatal. Currently, the majority of

clinical and is highly dependent on the physician's cases are prenatal, although the precise etiology ○○○○○○○○○○○○○○○○○○○○○○○○○○

Critical Elements of Care: Cerebral Palsy 1 I. Introduction to Cerebral Palsy

often cannot be identified. This is true in 20-30 predictions about prognosis rarely can be absolute percent of cases. Perinatal asphyxia at or near term and outcomes should be carefully assessed. The causes, at most, 10-15 percent of cerebral palsy clinical evaluation of these children must be ongoing. cases. Many children with cerebral palsy appear to A changing clinical picture may be the natural have a "cascade" of harmful events that often begin progression of the primary injury, modified by in utero and continue during and after delivery. maturation of the central nervous system (i.e.,

increasing tone or spasticity), or it may be a "new" ○○○○○○○○○○○ Are There Associated Risks of Other Disabili- finding identified by assessment methods that are ties for the Child with Cerebral Palsy? age-dependent (delayed language or cognitive

○○○○ abilities). The clinical change may also be a result Yes, cerebral palsy is known to have a higher risk of the emergence of other associated deficits. The association with other disabilities. These risks progressive appearance of signs of cerebral palsy, include, but are not limited to: such as changes from hypotonia to spasticity, need

seizures - 35-45% to be distinguishsed from progressive disorders such ○○○○○○○ mental retardation - 40-60% as those caused by metabolic, neoplastic or degen- erative disorders. visual deficits - 20-60% communication deficits, including hearing - 30% ○○○○ How Can the Child with Cerebral Palsy be feeding difficulties Managed? behavioral concerns The strategic goals in the management of cere- In children with cerebral palsy, multiple disabilities bral palsy are to enable the child to grow up in the tend to be the rule rather than the exception; 80 family and community and to achieve optimal percent will have at least one associated handicap, independence in adult life. Comfort and ease of

while 40 percent will have three or more associated ○○○○○○○○○○○ care are additional valued goals. handicaps. Much of the tactical management of cerebral palsy is aimed at preventing cumulative secondary How is the Severity of Cerebral Palsy de- impairment and disability. Follow-up at regular scribed? intervals (directed at assessing motor and developmental progress) is essential in the optimal manage- The recently developed Gross Motor Functional ment of a child with cerebral palsy. Classification System for Cerebral Palsy (GMFCS) defines five levels of motor functionfor each age ○○○○○○○○○○○ The multi-faceted nature of cerebral palsy group (see references). This simple tool allows requires a comprehensive approach. No two cases different clinicians to describe the severity gross with cerebral palsy are alike. Interventions directed motor function with more consistency. Researchers at one aspect of the child's problem must be made and clinicians now use the GMFCS to compare while taking into account the potential impact they groups of children with cerebral palsy. may have on all areas. Prioritization is crucial. A team of experienced professionals is usually needed A functional approach is used most often to at both the primary and consultative level. describe the severity of cerebral palsy: Mild - some impairment of mobility is present, What are Some Valuable Characteristics of but the child is generally able to function indepen- Optimal Care? dently. Athletic ability may be impaired. Moderate - definite difficulties in daily activities, Effective health care systems for children should often with a need for assistive devices or bracing in be family-centered, competent, comprehensive, order to promote age-appropriate independence. compassionate, continuous, community-based and culturally appropriate.. Severe - substantial limitations in everyday activities that limit independence. Family-centered care encompasses an understanding of the child's place in the family and Does the Presenting Picture of Cerebral Palsy the impact of the disability on all family members. Stay the Same? The family is empowered to play a major role in No, cerebral palsy exhibits an evolving clinical decision-making for their child. Through meetings, picture over time, secondary to the maturation of the educational materials and copies of all professional central nervous system, therefore clinical change reports, families are provided the background

should be expected. Because of this "time effect," necessary for making informed decisions. ○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○

Critical Elements of Care: Cerebral Palsy 2 I. Introduction to Cerebral Palsy

An effective health care system is compre- abilities and intervention programs needed to assist hensive source for the patient's routine, as well as, the child and family. specialty care needs. A child's cerebral palsy does ○○○○ not prevent her from contracting ordinary childhood What Are Some of the Specific Management

diseases, such as ear infections and chicken pox, nor ○○○○ Issues That Can Occur? exclude her from anticipatory care needs, such as immunizations and monitoring of physical growth. Spasticity and dystonia can become problematic as the symptoms evolve over time. When this Continuity for these children and their families is essential. Following these children over time happens pharmacologic or surgical interventions are permits monitoring of the natural progression of the available. Community therapists can help monitor for disability and reduces redundancy of evaluations. these concerns. By developing a working relation- Continuity fosters anticipation of medical, educa- ship with these professionals, the PCP will be able to tional and community needs for the child and the intercede appropriately. The PCP can use the family. Continuity of care promotes the prevention regional center in order to develop a plan of care for of further complications through early intervention. these problems. Musculoskeletal changes are often the most Competence requires that professionals have current training, experience, judgment and interest in easily identified consequences of central nervous cerebral palsy. No single provider has the time or system injury in cerebral palsy. Interventions may expertise to address all of the affected functional address the physical changes of contracture, muscle patterns and effectively manage these children while imbalance, joint instability and body malalignment supporting their families. with the goals of correcting deformity, preserving function and relieving pain. Professionals participat- A community-based approach requires ing in the care of these children should consider the appropriate use of community resources in areas of impact any intervention will have on function. special education, family support systems, financial Everyone involved should encourage input from support, respite and child care. other professionals who are working with the A compassionate approach emphasizes the children, therapists and parents. A thorough ortho- child not the disability. Longterm stressors are pedic assessment should include a detailed visual anticipated and countered with appropriate care. inspection (e.g. gait observation), a hands-on inspec- Rules are bent to serve individual needs. Self- tion of joint mobility (e.g. passive range of motion) defined choices for quality of life are respected. and an assessment of joint alignment. Contractures are more likely in children with When are Interventions and Treatments spasticity than others. There have been specific Indicated? musculoskeletal problem areas identified in this ○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ patient population. Spasticity in the hip adductors Treatment for children with cerebral palsy should may result in subluxation of the femoral head. be centered around improved function and indepen- Dislocations of the hips are more common in non- dence for the child and the family - now and into the ambulatory children when compared to ambulatory future. Interventions and therapies should not be children. X-rays of the hips in children with spastic mandated based on the "label" of cerebral palsy. adductors are recommended beginning at age 18-24 The effectiveness of any intervention is optimized by ○○○○○○○○○ months. Subluxation can reach an advanced stage periodic review and modification. Neither the before becoming clinically apparent. The hip needs clinical presentation nor the clinical treatment should to be relocated in the socket by age 4-5 years to be expected to remain static. Anticipating clinical develop properly. changes allows for improved monitoring and planning for necessary interventions. Scoliosis is the appreciable deviation of the normally straight vertical line of the spine. Children ○○○○○○○○○ with cerebral palsy are at risk for scoliosis, espe- Who Can Help in Monitoring the Need for cially during times of rapid growth, such as the Interventions or Treatments? pubertal growth spurt. This may result from a failure of the back muscles to support the spine against Community and regional resources can assist the gravity. In spastic types of cerebral palsy, the PCP and family in making decisions about services. excessive torque from abnormal tone can worsen The community health department, early intervention ○○○○○○○○○ the degree of deviation. In ambulatory patients there centers, schools and a local therapist can monitor the progression of the child's abilities. Regional centers is a six to seven percent incidence of scoliosis,

will provide the PCP with extended assessment usually thoracic, with its greatest frequency seen ○○○○○○

Critical Elements of Care: Cerebral Palsy 3 I. Introduction to Cerebral Palsy

during a growth spurt in the pre-puberty period. In intelligence, while 75 percent of children with non-ambulators, there is a 30-40 percent incidence, spastic quadriplegia have mental retardation. Chil- the scoliosis is more likely to be lumbar and is often dren with cerebral palsy who have a normal intelli- associated with pelvic obliquity. Management gence quotient are often at risk for learning disabili- decisions in the treatment of scoliosis should be ties. There is a close linkage between communica- made considering the degree of curvature, the tion skills and the outcome of cognitive testing. It is proximity to the age of skeletal maturity and the known that a high percentage of the children with linear growth rate of the child. The goals of any severe motor disabilities also have communication orthopedic intervention for scoliosis should be: to difficulties. Therefore it is imperative that the child's maintain balance in both the sitting and walking communication and language skills be optimized prior positions; to reduce pain; to reduce areas of in- to the initiation of cognitive assessments. creased pressure resulting in decubitus; and to Visual involvement has been shown in up to 60 preserve cardiopulmonary functional reserve. It is percent of all children with cerebral palsy. The important that surgical repair of scoliosis be under- types of impairments have included severe deficits taken by an orthopedist who has experience with of visual acuity. Refractive errors and strabismus individuals with cerebral palsy. (esotropias > exotropias) are most common, but Therapy and bracing concerns can be evalu- nystagmus, cortical visual impairment, visual field ated in the community by therapists or orthopedists. defects and complex disorders of visual control may Therapy is more effective if directed by functional all be encountered. goals (such as getting up stairs, versus increased Hearing problems are estimated to be present in ankle dorsiflexion). Referral to regional centers can 10-15 percent of children with cerebral palsy. An be helpful. accurate measure of hearing ability is essential. Adaptive equipment concerns can arise begin- Oral motor deficits can adversely affect speech ning at an early age when the child's need for safety (dysarthria) and feeding abilities. The child may is addressed. The family may need equipment in have a central speech/language deficit such as order to bathe, transport or position their child in a aphasia. safe manner. The community therapists in associa- Feeding/Nutrition and reflux are all areas of tion with early intervention centers/schools can assist possible concern. The risk for these concerns is the PCP in determining these needs. If the child's greater as the degree of disability increases in needs are difficult to meet, it may be necessary to severity. Children with cerebral palsy are at signifi- refer to the regional center for evaluations . cantly increased risk for osteopenia. Risk factors Seizures also require careful evaluation and include lack of ambulation, anticonvulsant use, low follow-up in children with cerebral palsy. Overall, sun exposure and living above 40 degrees latitude, 35-45 percent of these children will have some kind poor calcium intake and general malnutrition. of seizure disorder. Seizures can be of any clinical ○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ Consider supplementation of nonambulatory children type, though grand mal seizures are reported most and others at risk, with the RDA of Vitamin D. frequently. There is a strong correlation between Those with poor calcium intakes should be supple- the clinical type of cerebral palsy and the incidence mented with calcium (500 mg/day). Careful moni- of seizures. There is a higher incidence of seizures toring of the weight and length/height are critical in in children with hemiplegic and quadraplegic (60 determining the proper growth of the child. Good percent) cerebral palsy. Seizures are relatively nutritional status is essential for healing following uncommon in spastic diplegia (15-30 percent), and in surgery. the extrapyramidal forms (<25percent). The onset Emotional support for the family is important of seizures can occur at any time, but usually begins because family stressors are intensified by the care during the first two years of life. needs of the child. All family members are affected Cognitive abilities should be assessed and must when one member has a disability. The PCP can be considered as a comprehensive treatment plan is help the family find support through the Washington developed. Overall, 40-60 percent of children with State Parent to Parent, United Cerebral Palsy cerebral palsy will have an intelligence quotient Association of Washington, or PAVE - Parents Are below 70 within the range defined for mental Vital in Education (see references). The local public retardation. The severity and frequency of involve- health department, early intervention centers/schools ment is related to the clinical type of cerebral palsy. and other agencies may provide social workers or More than 75 percent of children with hemiplegia, psychologists to assist the family. If these services diplegia and athetoid cerebral palsy will have normal are not locally available, then referrals to the re-

○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ gional center may be of assistance.

Critical Elements of Care: Cerebral Palsy 4 I. Introduction to Cerebral Palsy

Critical Elements of Care ○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ The following outline defines functional areas that need to be assessed and the recommended frequency for assessment. It also provides the PCP with suggestions for appropriate consultations with other specialists, including developmental pediatricians, orthopedists, neurologists, physiatrists (rehabilitation specialists), speech therapists, physical and occupational therapists, nutritionists, psychologists, social workers, educators, public health nurses, equipment vendors and others. Along with these specialists, life-long care always involves the child's family and requires their thoughtful involvement in all decision making. These Critical Elements of Care constitute an initial attempt to provide a framework for long-term management of such children and should not be viewed as a comprehensive manual for care. ○○○○○○○○○○○○○○○○○○○○○○○○○

Critical Elements of Care: Cerebral Palsy 5 II. GUIDELINES FOR CRITICAL ELEMENTS OF CARE

HOW TO The Critical Elements of Care for Cerebral Palsy were designed to organize and simplify the child's care plan. They provide information and education regarding the comprehensive needs of an individual with cerebral palsy. The Critical Elements of Care are divided into five age groupings:

1 - 2 years 3 - 5 years 6 - 9 years 10 - 14 years 15 - 21 years

Within each age grouping six functional categories are identified:

Communication Feeding and Nutrition Musculoskeletal Mobility Cognition Sensor Impairment

In addition, there is a section for Family Issues.

Each age grouping has been divided into functional categories. These are subdivided into a range of functional levels and coincide with specific recommendations for intervention possibilities. By reviewing the Critical Elements of Care specific to your patient's age group, you will be able to assess the areas of concern, identify the intervention possibilities and organize and simplify the child's care plan.

WORKSHEET The Quick Check Worksheet was designed to summarize the Critical Elements of Care using a check-off format to identify concerning trends over time. There is room for short notation regarding specific problems, treatment regimes or referral options. The worksheet may be used during each office visit to consolidate the information. Therefore, past and present concerns can be identified quickly, and intervention referrals initiated.

Critical Elements of Care: Cerebral Palsy 6 CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 1 - 2 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION

Communication

VERBAL Assessment of communication (expressive and receptive) for age/developmental levels

NON-VERBAL Formal hearing evaluation ENT/audiologist Review assessment/plan of the early intervention program Early intervention program* Consider simple augmentative communication devices (picture boards, etc.) Musculoskeletal

CONTRACTURE ABSENT Assess ROM every 6 mo. extremities, hips and back

CONTRACTURE Assess ROM every 6 mo. extremities, back; orthopedic evaluation every Orthopedic consult PRESENT 6 mo., if indicated Early intervention program* Hip x-ray at 18 - 24 months Review OT/PT plan (splinting/bracing) Evaluation by a pediatric orthopedist Cognition

AGE APPROPRIATE Assess per well-child practice guidelines

DELAYED Need formal evaluation to make accurate determination of cognitive delay Early intervention program* (once in this age period) Regional Center Consider associated disabilities when choosing appropriate instrument for testing

Feeding & Nutrition Assess per well-child practice guidelines WIC (ages 1-5) ORAL Plot weight, length, OFC Health Dept. Maintain weight-length ratio @ 5 — 50th percentile Early intervention program* Review drug-nutrient interaction Assess feeding, swallowing skills (duration, parent concern) Consider nutrition consult (WIC, Health Dept., regional center) Behavioral component OT consult (early intervention program, regional center,) May need calorie or nutrient modifications (special supplements)

Assess at each visit: WIC (ages 1-5) NON-ORAL Plot weight, length, OFC Health Dept. Maintain weight-length ratio @ 5 — 50th percentile Early intervention program* Review drug nutrient interactions Evaluate feeding, swallowing difficulties Review history of pulmonary problem, recurrent OM and sinusitis Consider delayed gastric emptying and gastroesophageal reflux Consider nutrition consult, OT feeding evaluation, or swallow evaluation (regional center) Consider behavioral component Pediatric surgical consultation, as indicated Assess nasogastric/gastrostomy/jejunostomy tube care site if indicated

Continued CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 1 - 2 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION

Mobility

INDEPENDENT Visits per well-child practice guidelines AMBULATOR

ASSISTED Yearly assessment of gross motor function and plan for recommendations Early intervention program* AMBULATOR Consider PT/OT, adaptive equipment Consult at a regional center with an experienced cerebral palsy management team

NON-AMBULATOR Yearly assessment of gross motor function and plan Early intervention program* Consider PT/OT, adaptive equipment Consult with an experienced cerebral palsy management team

Sensory Impairment

HEARING NORMAL Assess per well-child practice guidelines

HEARING ABNORMAL Refer to ENT/audiology Early intervention program* Consider referral to early intervention program for speech therapy, Regional Center amplification, sign language

VISION NORMAL Assess per well-child practice guidelines check acuity and binocularity

VISION ABNORMAL Referral to opthalmology Early intervention*, visually impaired programs

Family Issues Anticipate family’s needs Acceptance/understanding of diagnosis (across all functional areas), how to explain to siblings/family members Resources: support groups, respite care, information, financial (SSI, CSHCN, DDD), or contact PHN for further resources Establish mutual goals with family and provider Emotional issues: grief, loss (ongoing) Foster care, institutional care options and/or respite care Lifestyle changes for family, transportation (car seats) and safety issues, literature resources, how to care for a child with CP

Specific Issues for child and family:

* Annual renewal of perscriptions for needed therapies. CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 3 - 5 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION Communication

VERBAL Assess expressive/receptive skills for age/developmental levels

NON-VERBAL Check hearing if not previously done ENT/audiologist Consider assessment/review plan with the early intervention/school for Early intervention program* augmentative communication program. Assistive technology specialist at a regional center may be needed. Musculoskeletal

CONTRACTURE ABSENT Assess ROM (extremities, back) every 6-12 months

CONTRACTURE Assess ROM every 6 mo. (extremities, back) Orthopedic consult PRESENT Hip/spine X-ray, as indicated Early intervention program* Review OT/PT plan Orthopedic evaluation every 6 mo., as indicated

Cognition

AGE APPROPRIATE Assess per well-child practice guidelines

IMPAIRED Need formal evaluation to make accurate determination of cognitive delay Early intervention program* (once in this age period). Regional center Consider associated disabilities when choosing appropriate instrument for testing.

Feeding & Nutrition

ORAL Assess per well-child practice guidelines WIC (ages 1-5) Plot weight, length, OFC Health Dept. Maintain weight-length ratio @ 5 — 50th percentile Early intervention program* Review drug-nutrient interaction Assess feeding, swallowing skills (duration, parent concern) Consider: nutrition consult (WIC, Health Dept., Regional Center) Behavior component OT consult (early intervention program, Regional Center) May need calorie or nutrient modifications If nonambulatory then supplement with the recommended daily allowance of vitamin D; consider supplementation with Calcium (500 mg/day)

NON-ORAL Assess at each visit: WIC (ages 1-5) Plot weight, length, OFC Health Dept. Maintain weight-length ratio @ 5 — 50th percentile Early intervention program* Review drug nutrient interactions Evaluate feeding, swallowing difficulties Review history of pulmonary problem, recurrent OM and sinusitis Consider delayed gastric emptying and gastroesophageal reflux Consider nutrition consult, OT feeding evaluation, or swallow evaluation (regional center - cerebral palsy team) Consider behavioral component Pediatric surgical consultation, as indicated Assess gastrostomy/jejunostomy tube care site If nonambulatory then supplement with the recommended daily allowance of vitamin D; consider supplementation with Calcium (500 mg/day)

Continued CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 3 - 5 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION

Mobility INDEPENDENT Yearly assessment to monitor for possible changing needs Early intervention program* AMBULATOR ASSISTED AMBULATOR Yearly assessment of gross motor function and plan for recommendations Early intervention program* Consider PT/OT, adaptive equipment Regional center Consult with an experienced cerebral palsy management team Assess potential for power mobility coordinate with school/regional center

NON-AMBULATOR Yearly assessment of gross motor function and plan Early intervention program* Consider PT/OT, adaptive equipment Regional center Consult with an experienced cerebral palsy management team

Sensory Impairment

HEARING NORMAL Assess per well-child practice guidelines

HEARING ABNORMAL Refer to ENT/audiology (local/regional center) Early intervention program* Coordinate assessment/plan with programs School program* Consider speech therapy, amplification, sign language, refer to regional Parent education center as indicated Regional center

VISION NORMAL Assess per well-child practice guidelines

VISION ABNORMAL Referral to opthalmology (local/regional center) Early intervention program* School program* Parent education Regional center Family Issues Ascertain family acceptance Parent separation issues — independence of child Changes in family lifestyle Getting ready for school issues Encourage age-appropriate activities Beginning peer relations Include child in family responsibilities, e.g. household chores

Specific Issues for child and family:

* Annual renewal of perscriptions for needed therapies CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 6 - 9 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION Communication

VERBAL Assess expressive/receptive skills for age/developmental levels

NON-VERBAL Check hearing if not previously done ENT/audiologist Assess/review school program School program* Consider assessment/review plan with the school for augmetative communication program. Assistive technology specialist at the regional center may be needed. Musculoskeletal

CONTRACTURE Assess ROM yearly (extremities, back) ABSENT CONTRACTURE Assess ROM yearly (extremities, back) School program* PRESENT Hip/spine X-ray, as indicated orthopedic consult Orthopedic evaluation yearly, as indicated (local/regional center)

Cognition

AGE APPROPRIATE Assess skills for age/developmental level

IMPAIRED Review school program School program* Formal evaluation once as indicated during this age period Regional center Consider associated disabilities when choosing appropriate instrument for testing. Feeding & Nutrition

ORAL Assess per well-child practice guidelines Health Dept. Plot weight, length, OFC Regional center Maintain weight-length ratio @ 5 — 50th percentile Review drug-nutrient interaction Assess feeding, swallowing skills (duration, parent concern) Consider nutrition consult (Health Dept., regional center) Behavioral component OT consult (Regional Center, school program) May need calorie or nutrient modifications If nonambulatory then supplement with the recommended daily allowance of vitamin D; consider supplementation with Calcium (500 mg/day)

NON-ORAL Assess at each visit: Health Dept. Plot weight, length, OFC Regional center Maintain weight-length ratio @ 5 — 50th percentile Review drug nutrient interactions Evaluate feeding, swallowing difficulties Review history of pulmonary problem, recurrent OM and sinusitis Consider delayed gastric emptying and gastroesophageal reflux Consider nutrition consult, OT feeding evaluation, or swallow evaluation Consider behavioral component Pediatric surgical consultation, as indicated Assess gastrostomy/jejunostomy tube care site If nonambulatory then supplement with the recommended daily allowance of vitamin D; consider supplementation with Calcium (500 mg/day)

Continued CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 6 - 9 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION

Mobility

INDEPENDENT Yearly assessment to monitor for possible changing needs AMBULATOR

ASSISTED Yearly assessment of gross motor function and plan for recommendations School program* AMBULATOR Consider PT/OT, adaptive equipment Regional center Consult at a regional center with an experienced cerebral palsy management team

NON-AMBULATOR Yearly assessment of gross motor function and plan School program* Consider PT/OT, adaptive equipment Regional center Consult at a regional center with an experienced cerebral palsy management team Assess potential for power mobility — coordinate with school/regional center

Sensory Impairment

HEARING NORMAL Assess per well-child practice guidelines

HEARING New onset/changes: refer to ENT/audiology (local/regional center) ENT/audiologist ABNORMAL Coordinate assessment/plan with school program School program* Consider speech therapy, amplification, augmentative communication Regional center evaluation at regional center

VISION NORMAL Assess per well-child practice guidelines

VISION ABNORMAL New onset/changes: refer to opthalmology (local/regional center) Regional center Coordinate assessment/plan with school program School program*

Family Issues Address child’s concerns: “Why am I different?” Encourage age-appropriate activities: summer camp, Special Olympics, horseback riding Encourage family participation in support groups: parents, child, siblings Include child in family responsibilities, e.g. household chores Specific behavioral concerns

Specific Issues for child and family:

* Annual renewal of prescriptions for needed therapies. CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 10 - 14 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION Communication

VERBAL Assess expressive/receptive skills for age/developmental levels

NON-VERBAL Check hearing if not previously done ENT/audiologist Assess/review school program School program* Consider assessment/review plan with the school for augmentative Regional center communication program. Assistive technology specialist at the regional center. Musculoskeletal

CONTRACTURE Assess ROM yearly (extremities, back) ABSENT

CONTRACTURE Assess ROM yearly (extremities, back) Orthopedic Consult local PRESENT Hip/spine X-ray, as indicated regional center Orthopedic evaluation yearly, as indicated School program*

Cognition

AGE APPROPRIATE Assess skills for age/developmental level

ORAL Assess per well-child practice guidelines Health Dept. Plot weight, length, OFC Regional center Maintain weight-length ratio @ 5 — 50th percentile Review drug-nutrient interaction Assess feeding, swallowing skills (duration, parent concern) Consider nutrition consult (Health Dept., regional center) Behavioral component OT consult (school, regional center) May need calorie or nutrient modifications If nonambulatory then supplement with the recommended daily allowance of vitamin D; consider supplementation with Calcium (500 mg/day)

NON-ORAL Assess at each visit: Health Dept. Plot weight, length, OFC Regional center Maintain weight-length ratio @ 5 — 50th percentile Review drug nutrient interactions Evaluate feeding, swallowing difficulties Review history of pulmonary problem, recurrent OM and sinusitis Consider delayed gastric emptying and gastroesophageal reflux Consider nutrition consult, OT feeding evaluation, or swallow evaluation (regional center) Consider behavioral component Pediatric surgical consultation, as indicated Assess gastrostomy/jejunostomy tube care site If nonambulatory then supplement with the recommended daily allowance of vitamin D; consider supplementation with Calcium (500 mg/day)

Continued CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 10 - 14 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION

Mobility

INDEPENDENT Assess yearly to monitor for possible changing needs AMBULATOR

ASSISTED Yearly assessment of gross motor function and plan for recommendations School program* AMBULATOR Components of plan: PT/OT, adaptive equipment Regional center Consult at a regional center with an experienced cerebral palsy management team

NON-AMBULATOR Yearly assessment of gross motor function and plan School program* Consider PT/OT, adaptive equipment Regional Center Assess potential for power mobility — coordinate with school Consult at a regional center with an experienced cerebral palsy management team

Sensory Impairment

HEARING NORMAL Assess per well-child guidelines

HEARING ABNORMAL New onset/changes: refer to ENT/Audiology (local/regional center) School program* Assess compliance with hearing aids as indicated ENT/audiologist (local/regional center) VISION NORMAL Assess acuity and binocularity Opthalmology Consult Refer to opthalmology as indicated (local/regional center)

VISION ABNORMAL Assess compliance with use of glasses as indicated Opthalmology Consult Refer to opthalmology as indicated (local/regional center) (local/regional center)

Family Issues Evaluate: Family transportation needs (van/lift) Ability to care for child (carrying, bathing, lifting, toileting, etc.) Puberty issues (behavioral, social) Peer relations Social activities (specialty camp, Special Olympics, etc.) Beginning sexual issues: birth control, menstruation, self-exploration (possible GYN consult local/regional center) Review: Care providers/sitters should be age/sex appropriate

Specific Issues for the child and family:

* Annual renewal of prescription for needed therapies. CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 15 - 21 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION

Communication

VERBAL Assess expressive/receptive skills for age/developmental levels

NON-VERBAL Check hearing if not previously done School program* Assess/review school program ENT/audiologist (local Consider assessment/review plan with the school for augmentative regional center) communication program Regional center Musculoskeletal

CONTRACTURE Assess ROM yearly (extremities, back) ABSENT

CONTRACTURE Assess ROM yearly (extremities, back) School program* PRESENT Hip/spine X-ray, as indicated Orthopedic consult Orthopedic evaluation yearly, as indicated (local/Regional center)

Cognition

AGE APPROPRIATE Assess skills for age/developmental level

IMPAIRED Review school program School program* Consider associated disabilities when choosing appropriate instrument for Regional center testing.

Feeding & Nutrition

ORAL Assess per well-child practice guidelines Health Dept. Plot weight, length, OFC Regional center Maintain weight-length ratio @ 5 — 50th percentile Review drug-nutrient interaction Assess feeding, swallowing skills (duration, parent concern) Consider: nutrition consult (Health Dept., regional center) Behavior component OT consult (school, regional center) May need calorie or nutrient modifications If nonambulatory then supplement with the recommended daily allowance of vitamin D; consider supplementation with Calcium (500 mg/day)

Continued CRITICAL ELEMENTS OF CARE: Cerebral Palsy

A G E 15 - 21 AREAS OF CONCERN ASSESSMENT & INTERVENTION SERVICE COORDINATION

Mobility

AMBULATOR Assess/refer for evaluation of driving skills, as indicated

ASSISTED Assess/refer for evaluation of driving skills/adaptive equipment, as indicated School program* AMBULATOR Yearly assessment of gross motor function and plan for recommendations Regional center Components of plan: PT/OT, adaptive equipment Consult at a regional center with an experienced cerebral palsy management team

NON-AMBULATOR Assess/refer for evaluation of driving skills/adaptive equipment as indicated School program* Yearly assessment of gross motor function and plan Regional Center Consider PT/OT, adaptive equipment Consult at a regional center with an experienced cerebral palsy management team

Sensory Impairment

HEARING NORMAL Assess per well-child practice guidelines

HEARING New onset/changes: refer to ENT/audiology (local/regional center) School program* ABNORMAL Coordinate assessment/plan with school programs Consider speech therapy, amplification, augmentative communication evaluation at regional center

VISION NORMAL Assess per well-child practice guidelines

VISION ABNORMAL New onset/changes: refer to opthalmology (local/regional center) Opthalmology Consult Coordinate assessment/plan with school program (local/Regional Center) Family Issues Review: Family transportation needs (van/lift) Ability to care for child (ADLs) Sexual issues: birth control (male and female), exploration, dating, marriage (GYN Consult - local/regional center) Guardianship issues, full or partial, need to be established by 18th birthday Career guidance/vocational Care providers/sitters are to be age/sex appropriate Long-term living arrangements: group homes, adult foster care, aging parents

Specific Issues for the child and family:

* Annual renewal of perscriptions for needed therapies. IV. Quick Check Worksheet

NAME: DOB: MR #:

WNL Delay/Concern* Referral/Tx* WNL Delay/Concern* Referral/Tx* WNL Delay/Concern*Referral/Tx*WNL Delay/Concern*Referral/Tx*WNL Delay/Concern*Referral/Tx*

DATE Nutrition: Wt: Lt %ile OFC %ile *Feeding: Mechanism Problem Pulmonary: Cold/Pneumonia RAD, Otitis/Sinusitis Hearing Vision Milestones: Gross Motor Fine Motor Communication Musculoskeletal: Hips Spine Other joints X-Ray done Safety (age/dev. approp.) Social/Behavior: Child Parent Sibling/s School Program Equipment (w/c, P.w/c, fdg Pump, Suction, Walker, Crutches) Splints/Braces (AFO, Hand, Back) Skin check Bowel/Bladder Medication review Lab check

*Feeding Mechanism: PO/NG/Gt/Jt Problem: Vomit/Gag/Choke/Cough *Referral Options: PHN/ Early Intervention Center/ School/ PT/ OT/ Speech/ Psych/ Specialist MD

Critical Elements of Care: Cerebral Palsy 17 V. Appendix

I. References and Resources ○○○○○○○○○○○○○○○○○○○○○○○○○○ PROFESSIONAL READING LIST: [Review]. Developmental Medicine & Child Neurology, 46, 461-467. Ashwal S., Russman B.S., Blasco P.A., Miller G., Sandler A., Shevell M., Stevenson R., Quality Standards Hankins G.D., & Speer M. (2003) Defining the pathogenesis Subcommittee of the American Academy of Neurology, & and pathophysiology of neonatal encephalopathy and Practice Committee of the Child Neurology Society. (2004) cerebral palsy [Review]. Obstetrics & Gynecology, 102, Practice parameter: diagnostic assessment of the child 628-636. with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology Houlihan C.M., O’Donnell M., Conaway M., & Stevenson and the Practice Committee of the Child Neurology R.D. (2004) Bodily pain and health-related quality of life in Society. Neurology, 62, 851-863. children with cerebral palsy. Developmental Medicine & Child Neurology, 46, 305-310. Bax M., Goldstein M., Rosenbaum P., Leviton A., Paneth N., Dan B., Jacobsson B., Damiano D., & Executive Committee Karol L.A. (2004) Surgical management of the lower extremity for the Definition of Cerebral Palsy. (2005) Proposed in ambulatory children with cerebral palsy [Review]. definition and classification of cerebral palsy, April 2005 Journal of the American Academy of Orthopaedic [Review]. Developmental Medicine & Child Neurology, 47, Surgeons, 12, 196-203. 571-576. ○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○ Kerr Graham H., & Selber P. (2003) Musculoskeletal aspects of Bjornson K.F., McLaughlin J.F., Loeser J.D., Nowak- cerebral palsy [Review]. Journal of Bone & Joint Surgery - Cooperman K.M., Russel M., Bader K.A., & Desmond S,A. British Volume, 85, 157-166. (2003) Oral motor, communication, and nutritional status of children during intrathecal baclofen therapy: a descriptive King S., Teplicky R., King G., & Rosenbaum P. (2004) Family- pilot study. Archives of Physical Medicine & centered service for children with cerebral palsy and their Rehabilitation, 84, 500-6. families: a review of the literature [Review]. Seminars in Pediatric Neurology, 11, 78-86. Campbell, W.M., Ferrel, A., McLaughlin, J.F., Grant, G.A., Loeser, J.D., Graubert, C., & Bjornson, K. (2002). Long- Liptak G.S. (2005) Complementary and alternative therapies for term safety and efficacy of continuous intrathecal cerebral palsy [Review]. Mental Retardation & baclofen infusion in severely disabled children and Developmental Disabilities Research Reviews, 11, 156-163. adolescents with spasticity of cerebral origina. Developmental Medicine and Child Neurology, 44, 660- McKearnan K.A., Kieckhefer G.M., Engel J.M., Jensen M.P., & 665. Labyak S. (2004) Pain in children with cerebral palsy: a review [Review]. Journal of Neuroscience Nursing, 36, 252- Dormans J.P., & Pelligrino, L. (Eds.). (1998). Caring for 259. Children with Cerebral Palsy: A Team Approach. Baltimore: P. H. Brookes. Morris C., & Bartlett D. (2004) Gross Motor Function Classification System: impact and utility [Review]. Gilmartin R., Bruce D., Storrs B.B., Abbott R., Krach L., Ward Developmental Medicine & Child Neurology, 46, 60-65. J., Bloom K., Brooks W.H., Johnson D.L., Madsen J.R., McLaughlin J.F., & Nadell J. (2000). Intraethecal baclofen Nelson K.B. (2002) The epidemiology of cerebral palsy in term for management of spastic cerebral palsy: A multicenter infants [Review]. Mental Retardation & Developmental trial. Journal of Child Neurology, 15, 71-77. Disabilities Research Reviews, 8, 146-150.

Goldstein M. (2004) The treatment of cerebral palsy: What we Nickel, R.E. (2000). Cerebral palsy. In Nickel, R.E & Desch, know, what we don’t know [Review]. Journal of Pediatrics, L.W. (Eds.), The physicians guide to caring for children 145, S42-S46. with disabilities and chronic conditions. Baltimore: P. H. Brookes. Gorter J.W., Rosenbaum P.L., Hanna S.E., Palisano R.J., Bartlett D.J., Russell D.J., Walter S.D., Raina P., Galuppi B.E., & Wood E. (2004) Limb distribution, motor impairment, and functional classification of cerebral palsy Critical Elements of Care: Cerebral Palsy 18 V. Appendix ○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○

Palisano R.J., Snider L.M., & Orlin M.N. (2004). Recent United Cerebral Palsy advances in physical and occupational therapy for www.ucp.org children with cerebral palsy [Review]. Seminars in Pediatric Information, legal issues, current political topics and links Neurology, 11, 66-77. to relevant organizations.

Samson-Fang L., Butler C., O’Donnell M., & American United Cerebral Palsy of Oregon & Southwest Washington Academy for Cerebral Palsy Developmental Medicine. www.ucp.org/ucp_local.cfm/129 (2003). Effects of gastrostomy feeding in children with (503) 777-4166 or Toll free 1-800-473-4581 cerebral palsy: an AACPDM evidence report [Review]. United Cerebral Palsy of South Puget Sound Developmental Medicine & Child Neurology, 45, 415-426. http://www.ucp-sps.org/ Sanger T.D., Delgado M.R., Gaebler-Spira D., Hallett M., Mink (253) 565-1463 or Toll free 1-866-812-8736 J.W., & Task Force on Childhood Motor Disorders. (2003) National Disability Sports Alliance Classification and definition of disorders causing www.ndsaonline.org/ hypertonia in childhood [Review]. Pediatrics, 111, e89-97. Sports training and competition opportunities for persons with cerebral palsy and other disabilities. Stanley, F., Blair, E., & Alberman, E. (2000). Cerebral palsies: Epidemiology and causal pathways. Clinics in Washington Parents Are Vital in Education (PAVE) Developmental Medicine: Mac Keith Press. www.washingtonpave.org [email protected] 1-800-572-7368 Winter S., Autry A., Boyle C., & Yeargin-Allsopp M. (2002). (253) 565-2266 (voice/TDD) Trends in the prevalence of cerebral palsy in a population- Parent directed organization working to increase based study. Pediatrics, 110, 1220-1225. independence, empowerment and future opportunities for consumers with special needs their families and communities. Also provides STOMP, a program serving US Military INFORMATION AND ORGANIZATIONS families who have a child with special needs.

American Academy for Cerebral Palsy and Developmental Medicine www.aacpdm.org PERIODICALS Current articles and books about developmental disabilities for both professionals and families. Exceptional Parent www.eparent.com ARC of Washington State 1-877-372-7368

www.arcwa.org ○○○○○○○○○○○○○○○○○○○○○○○○○○○ (360) 357-5596 or Toll-free 1-888-754-8798 Advocates for the rights of citizens with disabilities.

Adolescent Health Transition Project www.depts.washington.edu/healthtr/ A resource for adolescents with special health care needs, chronic illnesses, physical or developmental disabilities

Center for Children with Special Needs www.cshcn.org (206) 527-5735 Information and resources for families who have children with special needs. Parent to Parent http://www.arcwa.org/parent_to_parent.htm 1-800-821-5927 Find other parents with children with disabilities and support groups in your area.

Critical Elements of Care: Cerebral Palsy 19 V. Appendix ○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○○

READING LIST FOR FAMILIES Meyer, Donald. and Vadody, Patricia. (1996). Living with a brother or sister with special needs. Seattle: University of BOOKS FOR ADULTS Washington Press. ISBN: 0295975474 Batshaw, Mark L. (2002). Children with Disabilities. Baltimore: Pimm, Paul. (1999). Living with Cerebral Palsy. London: Hodder P.H. Brooks. ISBN: 1557665818. Wayland. ISBN: 0750241624

Blank, Joseph. (1996). Nineteen steps up the mountain: The Taylor, Ron. (1991). All By Self. Milwaukee, Wisconsin: Light story of the DeBolt family. Albany, New York: Harrow & on Books. ISBN: 0938991752 Heaton. ISBN: 051506498X.

Finnie, N., et al. (1997). Handling the young cerebral palsied BOOKS FOR CHILDREN, AGES 9 TO 12 child at home. Woburn, Massachusetts: Butterworth- Berman, Thomas. (1991). Going Places: Children Living with Heinemann Medical. ISBN: 0750605790. Cerebral Palsy (Don't Turn Away). Milwaukee, Wisconsin: Gareth Stevens. ISBN: 08636801997 Geralis, Elaine. (1998). Children with cerebral palsy: A parent's guide. Rockville, Maryland: Woodbine House. ISBN: Gould, Marilyn. (1991). Golden Daffodils. Newport Beach, 0933149824 California: Allied Crafts. ISBN: 0201115719

Laws, R. and O'Hanlon, T. (1999). Adoption and financial Nixon, Shelley. (1999). From Where I Sit: Making My Way with assistance: tools for navigation the beauroacracy. Westport, Cerebral Palsy. New York: Scholastic. ISBN: 059039584X Connecticut: Bergin and Garvey. Sanford, Doris E. (1992). Yes I Can! Challenging Cerebral Palsy. BOOKS FOR CHILDREN, AGES 4 TO 8 Sisters, Oregon: Multnomah. ISBN: 0880705108

Anderson, Mary Elizabeth. (2000). Taking Cerebral Palsy to School. Plainview, New York: JayJo Books. ISBN: 1891383086 Debear, Kristen. (2001). Be Quiet, Marina! New York: Star Bright Books. ISBN: 1887734791

Heelan, Jamee. (2000). Rolling Along: The Story of Taylor and His Wheelchair. Atlanta, Georgia: Peachtree. ISBN: 156145219X ○○○○○

Funded by a contract with the Washington State Department of Health Children with Special Health Care Needs Program

Item #001

Critical Elements of Care: Cerebral Palsy 19