Executive Summary: Providing a Primary Care Medical Home for Children and Youth With Cerebral Palsy

Cerebral palsy (CP) is the most common motor disorder of childhood, with prevalence estimates ranging from 1.5 to 4/1000 live births. This executive summary is a companion for the full clinical report “Providing a Primary Care Medical Home for Children With Cerebral Palsy” and specifically seeks to provide primary care physicians (PCPs) with guidance to detect CP in children; collaborate with specialists treating patients with CP; manage associated medical, developmental, and behavioral problems; and provide primary medical care to their patients with CP.

The current consensus definition of 2006 states that “cerebral palsy describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems.”

The term CP describes “a group of disorders,” or what may be considered a “phenotype.” Different brain disturbances can result in a common clinical picture of motor disability. These include intrauterine and perinatal infections, intraventricular hemorrhage, hypoxic-ischemic encephalopathy, stroke, cerebral malformations, genetic disorders, and others. These etiologies are “permanent” and “nonprogressive” in that the brain disturbance is not ongoing nor worsening; however, the physical manifestations of movement and posture problems can change over time.

• All physicians who provide care for children must be familiar with the definition, manifestations, and management of CP. Pediatricians, neonatologists, and other specialists who care for hospitalized newborn infants should recognize infants who have a greater likelihood of CP, use available tools to diagnose CP as early as possible, and promptly refer for therapy.

There is increasing recognition of the importance of detecting CP and other neuromotor disorders as early as possible. For CP, early identification and initiation of evidence-based motor therapies can improve outcomes by taking advantage of the neuroplasticity in the infant brain.

Some children will be identified as “at risk for cerebral palsy” or other developmental problems because of factors from their medical history, such as preterm birth and other pre- or perinatal problems. For these patients, PCPs can work with neonatal follow-up programs and therapists to further evaluate these children using validated tools, which require specialized training.

• Primary care pediatricians should implement formal developmental surveillance and screening in their practice so that children with possible motor delays such as CP can be identified as early as possible; these children should be referred promptly and simultaneously for a diagnostic assessment by a specialist and to therapy.

The American Academy of Pediatrics (AAP) recommends that standardized developmental screens be administered at 9, 18, and 30 months. Screening for motor disorders in particular is recommended at these ages and should include a neuromotor examination with particular attention to acquisition of motor milestones and assessment of muscle tone. Specifics on screening and tools used for early diagnosis of CP can be found in the Early Detection of CP Care Pathway from the American Academy for Cerebral Palsy and Developmental Medicine (https://www.aacpdm.org/publications/care-pathways/early-detection-of-cerebral-palsy).

Signs that should alert the PCP to the possibility of CP include motor asymmetry such as early hand preference (not seen in typically developing children until 18 months of age); “thumb-in-fist” posture beyond 7 months of age; inability to sit independently by 9 months of age; and altered muscle tone, such as scissoring of the legs.

When a child is identified as having a possible neuromotor disorder, the AAP recommends that the PCP take 3 actions simultaneously: initiate a diagnostic workup based on the suspected disorder; refer to a medical specialist to complete the diagnostic evaluation; and refer for treatment to Early Intervention programs and therapists.

• When a child is identified to have CP, the PCP should engage with the child’s family and specialists to establish a patient- and family-centered care team that encourages shared decision-making, coordinated care, and interventions that promote the goals of the family.

Children with CP benefit from a multidisciplinary approach to care with teams typically consisting of the child and family, PCP, medical specialists, and other health professionals such as physical, occupational, and speech therapists. Other team members may include professionals from Early Intervention or school services, community health workers, family support groups and other community and state resources, psychologists, home health care agencies, medical equipment vendors, and adaptive recreation programs. A key role of the PCP is to integrate care across multiple specialists so that that all of the child’s medical needs can be addressed.

Pediatricians can guide families in developing a treatment plan starting with a discussion of the family’s and child’s goals. Interventions for CP aim to optimize functional independence, promote participation in activities, enhance quality of life, and minimize secondary musculoskeletal deformity. Fundamental aspects of patient- and family-centered care for children with CP include emphasizing child and family strengths, facilitating family choice and control, and collaborating with the child and family in setting individualized goals.

• Pediatricians should advise families who have a child or adolescent with a diagnosis of CP about available medical, social, and educational services such as Early Intervention, the State’s Title V program, family support groups, special education services through the public school system, and Social Security, as applicable.

Even before the diagnosis of CP is established, the PCP can turn his or her attention to maximizing the patient’s potential and caring for the medical, psychosocial, and educational needs of the youth and family. In addition to referral to Early Intervention programs, a young child should be referred to state resources for children with special needs. Some children and adolescents with CP may be eligible for income support through Supplemental Security Income, secondary insurance through Medicaid, and Home and Community Based Services through Medicaid. Families can benefit from peer support groups with other caregivers of children and adolescents with developmental disabilities. These groups can help reduce a family’s social isolation, improve well-being, and increase parental knowledge.

Pediatricians play an important role in assisting their patients and families when it comes to therapies, school services, and class placement. Pediatricians should periodically review a patient’s academic and therapy evaluations with the patient and family to promote services that are appropriate and provided in the least restrictive environment.

• Pediatricians should be aware that cerebral palsy is more prevalent in Black children and children who come from families with lower socioeconomic status. Recognition of these disparities should prompt physicians to identify implicit biases and barriers to screening, identification, treatment, and familial support for children with CP whose lives are impacted by social determinants of health.

Because race is a social construct, these disparities may be attributable to systemic racism within the medical system as well as other systems providing care for women before and during pregnancy. Efforts to combat racism and eliminating barriers to culturally sensitive prenatal, perinatal, and later pediatric care may help to improve outcomes for all children with CP.

Further research is needed to better understand and address these disparities, and changes in social and economic policies at the societal level are needed to reduce them.

• In keeping with the recommendations of Bright Futures, children and adolescents with CP should receive standard primary care visits and interventions to promote health, well-being, and optimal growth and development. This care includes receiving vaccinations according to the recommended childhood and adolescent immunization schedule, with additional vaccines for those with chronic pulmonary or other conditions.

Patients with CP need the same approach to primary care as all other children. For children and adolescents with special needs such as CP, some of these practices, such as vaccination and hearing and vision screening, are particularly important.

Preparation for puberty, menstruation, and healthy, safe sexual relationships needs to be part of primary health care for all youth, including those with CP. Adolescent care for youth with CP includes screening and performing assessments for sexual activity, substance use, and mental health problems and performing appropriate testing (if indicated), as is standard for all youth. The approach for each patient needs to be individualized and geared toward the patient’s capacity.

Children and adolescents with disabilities and special health care needs account for a disproportionate percentage of cases of child maltreatment, with increased risk of physical, sexual, and emotional abuse and neglect. Pediatricians can help reduce the occurrence of maltreatment by educating caregivers regarding prevention and indicators of abuse and by identifying family stressors and making referrals for support services.

• Primary care pediatricians should encourage their patients with CP and their families to participate in social, recreational, and community activities based on the child and teen’s interests and assist with locating opportunities for adaptive sports and recreation when indicated.

Participation in sports and other recreational activities has multiple benefits for children, including physical fitness, social opportunities, reduced challenging behaviors, and the development of independence and teamwork. In addition to occupational, physical, and speech therapies, other therapies and services can help optimize participation and provide opportunities for fitness and fun. A number of adaptive sports programs, such as wheelchair basketball and sled hockey, are available for individuals with physical disabilities, and children with CP are often eligible to receive adapted physical education at school as part of their Individualized Education Program. Pediatric providers can encourage participation by talking to children about what they like to do for fun, helping families explore local opportunities for participation in adaptive sports and other activities, and providing guidance on activities appropriate for the child’s condition.

• Primary care pediatricians should monitor for problems that commonly cooccur with CP. An increased frequency of visits should be considered with referral to specialists as concern for secondary complications arise.

Although CP is a motor disorder caused by an injury to the brain, there are often problems in other areas of the nervous system as well as nonneurologic comorbidities affecting almost every organ system.

• - Cognition and learning: Almost 50% of children and adolescents with CP also have intellectual disability and more have specific learning difficulties. However, not all individuals with CP have cognitive challenges, and it is important not to assume cognitive impairment on the basis of an individual’s motor impairment. Given the risk of challenges in this area, the PCP should have a low threshold to refer for formal testing if there are concerns about learning or cognitive abilities.

• - Communication, speech, and language delay: Approximately 60% to 80% of children and adolescents with CP have communication difficulties, and approximately 25% are nonverbal. Pediatricians should be aware of ways of efficiently communicating with their patients, maximizing the use of augmentative and alternative communication devices and involving experts in speech and language pathology.

• - Behavior and mental health: Behavioral and mental health symptoms and disorders are prevalent among children and adolescents with CP with rates consistently higher than the general population. Commonly reported symptoms include inattention, hyperactivity and impulsivity, disruptive behavior, anxiety, and depression. Because behaviors can be a means of communicating a need or distress, primary care pediatricians play a key role in evaluating for medical conditions when a child or adolescent with CP presents with behavioral concerns. It is also important to keep in mind that mental health conditions can manifest as behavioral outbursts or changes in eating and sleeping patterns in children with communication challenges.

• - Epilepsy: Epilepsy is present in 35% to 60% of children and adolescents with CP.

• - Sensory impairments: Children and adolescents with CP have a greater likelihood of sensory impairment, including cortical visual impairment, ophthalmologic conditions, and sensorineural hearing loss. Starting in early childhood, the PCP should screen children with CP regularly because addressing correctable problems or providing accommodations can improve learning, school performance, and social participation. Some children will require formal ophthalmology and/or audiology evaluations if they are unable to participate in office screening or have specific risk factors for vision or hearing loss.

• - Pulmonary problems: Pulmonary problems are the leading cause of hospitalization and death for children and adolescents with CP. Aspiration from dysphagia with oral feeding or gastroesophageal reflux can lead to chronic lung inflammation and infection. The ability to clear secretions from the airways may be impaired. PCPs can prevent or minimize pulmonary complications through immunization against preventable respiratory diseases and screening for signs and symptoms of aspiration and sleep-disordered breathing.

• - Sleep: Difficulties falling asleep and staying asleep are common in children and adolescents with CP. Optimizing management of medical comorbidities such as sleep-disordered breathing, gastroesophageal reflux disease, and pain that may be interfering with sleep is an important component of addressing sleep problems.

• - Nutrition, growth, and gastrointestinal problems: Many individuals with CP have dysphagia or other difficulties with feeding and growth, and approximately 1 in 15 receive nutrition through a feeding tube. Dysphagia may be associated with aspiration or prolonged feeding times. Other common issues include oral feeding aversions, drooling, gastroesophageal reflux, and constipation. The pediatrician should screen for feeding difficulties and use a multimodal approach to assess the appropriateness of growth and nutritional status.

• - Bone health: Low bone mass can lead to fractures, particularly in patients who are not ambulatory.

• - Orthopedic complications: Orthopedic complications are major morbidities for patients with CP and can include joint contractures, scoliosis, and hip dysplasia. Early detection and intervention for hip dysplasia reduces the likelihood of progression to subluxation and dislocation. Key roles for the PCP include obtaining screening hip radiographs, identifying when a referral to orthopedics is necessary, and ensuring that the patient is in the best possible medical health before surgery.

• When new symptoms or functional declines are seen in patients with CP, these should be investigated fully as new medical problems without assuming that they are related to the underlying CP.

The brain injuries that cause CP are permanent and nonprogressive; however, the physical manifestations of movement and posture problems can change over time. CP should not be considered “static” in that sense. For example, changes in gait, pain, or posture may be related to the underlying CP as a result of worsening contractures, poorly controlled tone, or progressive orthopedic deformities, etc. Symptoms should not be assumed to be related to CP without proper investigation into other causes.

Challenging behaviors may be a means of expressing physical discomfort, fatigue related to poor sleep, or distress related to a change in routine in an individual with limited verbal skills. The PCP plays an important role in assessing for sources of physical discomfort (eg, constipation, gastroesophageal reflux disease, musculoskeletal pain, dental pain, ear or sinus infection, etc) that may be contributing to the behavioral presentation.

• Pain is underrecognized in children and adolescents with CP. All youth with CP should be screened for pain. Pain in patients with limited verbal ability may be difficult to detect and may manifest as a change in behavior.

The frequency of pain in children and adolescents with CP varies, but pain can be present in as many as 75% of patients. Because of the high frequency and impairment to functioning and family life, pain should be discussed at every routine health care maintenance visit, not only after a surgery or when concern is raised by the patient or family. Assessing the level of pain is an important part of evaluating and monitoring treatment. In addition to assessing the patient’s report of level of pain, parent reports and understanding of baseline behaviors is essential in understanding changes that may represent pain in a child with CP. Targeting the source of the pain (if known) and using a stepwise approach to pain medication as well as nonpharmacologic therapies should be the standard approach to pain treatment.

• A health care transition process for shifting from pediatric care to adult care should be implemented for adolescents with CP, with planning beginning by age 12 to 14 years.

Late adolescence and early adulthood are vulnerable times for those with special health care needs. If proper preparation and handoffs to adult physicians do not occur, there is an increased risk of morbidity, medical complications, unnecessary emergency department visits, hospitalizations, and procedures. Studies of young adults with CP show that they do not feel well prepared for this transition. Starting the process early and using a structured process is recommended for all patients. Patients have smoother transitions if they are more involved in making decisions and managing their health. Youth with CP who are cognitively able should be given opportunities throughout adolescence to develop understanding of their medical condition, decision making, and self-management skills. Pediatricians can take an active role in recommending maximal independence (and discouraging overprotection) in children with CP.

Before transfer to an adult provider, the PCP should write a comprehensive medical summary with the input of the patient, parent/guardian, and pediatric subspecialists. This medical summary should include information regarding the patient’s level of functioning, current and past medical problems, efforts at tone management, history of surgical interventions, and complications of medications or procedures. In addition, for patients who have chronic pain, the summary should include a description of the pain level, location, and methods used to alleviate pain (and those methods that were unsuccessful). Medical comorbidities and information on the use of adaptive equipment and assistive technology should also be included. Guidance in the structured process of preparation, including tools for transition, can be found in the AAP report “Supporting the Health Care Transition From Adolescence to Adulthood in the Medical Home,” at Got Transition (www.gottransition.org) or from the Child Neurology Foundation (www.childneurologyfoundation.org/transitions/).

Evaluation of decision-making capacities should be performed and monitored throughout adolescence. If legal supports are indicated (for example, guardianship, health care proxy), they should be put into place when the adolescent turns 18 years old. Pediatricians can facilitate this discussion. Consultation with a lawyer who specializes in disabilities may be helpful, keeping in mind that the least restrictive support is preferable depending on the individual’s capabilities. Pediatricians should assist in identifying an adult clinician. Medical, legal, and psychosocial information should be communicated to the adult PCP at the time of transfer.

• Pediatricians can advocate for their patients on an individual level and with Medicaid and other payers at a state and federal level to ensure that resources are adequate to achieve best outcomes for children with CP.

The care of children with CP can be extremely complex and costly, both for the family and for medical providers. Children and families may be eligible for a range of services that are, in reality, difficult to access. Payment for physician services is often inadequate in comparison with the amount of work actually provided by the medical home to provide expert, coordinated care for children. The breadth of the pediatrician’s care, as illustrated in this clinical report, is difficult to sustain solely through payment for intermittent office visits. Pediatricians can advocate for systemic change to help children with CP, their families, and their medical homes.