Classification and Spectrum of Disorder

CP includes a spectrum of disorders of movement, posture, and coordination with heterogeneous etiologies. The diversity of the clinical features is reflected in multiple classification systems that include reference to type of motor dysfunction, body parts affected, severity, and functional abilities (see Table 1).

Table 1

CP classification systems used and understood by qualified medical practitioners.

Of note, when classifying on motor function, spastic CP accounts for 70 to 80 percent of all cases of CP, with dyskinetic accounting for 10 to 15 percent and ataxic 15 percent, though combinations of clinical manifestations are common. Further classification is by severity level (mild, moderate, severe), and gross motor function, which reflects the functional capabilities of the affected.^19,20 Developed in the late-1990s, the Gross Motor Function Classification System (GMFCS) outlines a standardized system for classifying motor function based on constructs of disability and functional limitation.²¹ GMFCS includes levels that reflect abilities ranging from walking without limitations (level I) to severe head and trunk control limitations requiring extensive use of assisted technology, physical assistance, and wheelchair (level V).

The epidemiologic Oxford Feeding Study reported significant correlations between severity of motor impairment and feeding problems including choking, underweight, prolong feeding times, vomiting, and need for gastrostomy feeding (p values typically <0.005).²² Although CP is a motor disorder, many children and adults with CP are affected by other developmental disabilities, including intellectual disability, impaired vision and hearing, language and behavioral disorders, and epilepsy. Population-based studies have reported the proportion of children with CP who have intellectual disability ranges from 31 to 65 percent and 20 to 46 percent for epilepsy of children with CP.^20,23,24 Intellectual disability varies with subtype of CP and level of impairment.^13,24-26 Survival and quality of life vary across the spectrum of CP, but are associated with severity and functional disabilities, as well as comorbid conditions.²⁴

Feeding Difficulties and Interventions

Individuals with CP frequently have feeding and swallowing problems that may lead to poor nutritional status, growth failure, chronic aspiration, esophagitis, and respiratory infections. Across the cerebral palsy spectrum, poor nutritional status is caused by distinct pathways ranging from inadequate intake, oral dysphagia, oral-pharyngeal dysphagia, gastroesophageal reflux (GER), chronic aspiration, and behavioral etiologies. Some patients with oral-pharyngeal dysphagia and GER, particularly those with severe CP, are also at risk for recurrent aspiration which can lead to chronic pulmonary disease. Patients with feeding difficulties range from those with self-feeding skills to populations with severe disability (GMFCS V) who require extensive use of assisted technology and are dependent on others to feed them. Chronic pulmonary disease related to aspiration is a leading cause of death among patients with severe CP.^27-30

Data suggest that during the first year of life, 57 percent of children with CP have sucking problems, 38 percent have swallowing problems, 80 percent have been fed nonorally on at least one occasion, and more that 90 percent have clinically significant oral motor dysfunction.³¹ More severe motor impairment is associated with greater difficulty with swallowing.³² Among children with spastic quadriplegia, one third has been reported to require assisted feeding.³³ Individuals with severe functional limitations (GMFCS level IV or V) commonly need assisted feeding. Caregiver burden is also a significant concern, as the feeding process may require considerable time and may be associated with stress and caregiver fatigue³⁴; stress and fatigue may in turn affect the feeding process.³⁵

A number of feeding and oral-motor intervention strategies have been developed to address difficulties with sucking, chewing, swallowing, and improve oral-motor skills, including oral sensorimotor management, positioning, oral appliances, food thickeners, specialized formulas, and neuromuscular stimulation. These interventions address different aspects of feeding difficulties, reflecting the range in specific problems associated with feeding and nutrition in CP. Sensorimotor techniques seek to strengthen oral-motor control and counteract abnormal tone and reflexes to improve oral feedings, and typically require months of daily application. Positioning techniques address poor postural alignment and control that exacerbates swallowing difficulties, and include stabilizing the neck and trunk. Positioning interventions are individualized and often guided by video-fluoroscopy to optimize swallowing. Oral appliances have been used to stabilize the jaw, improve sucking, tongue coordination, lip control, and chewing. Multiple approaches may be used in children with growth failure, including sensorimotor stimulation, positioning, food thickeners, and caloric supplementation. For children with moderate to severe aspiration or malnutrition related to oral-pharyngeal dysphagia and GER, surgical interventions with gastrostomy (tube feeding directly into the stomach) or jejunostomy tubes (tube feeding into the middle portion of the small intestine, the jejunum) and antireflux procedures may be necessary to improve nutritional status and reduce risk of chronic aspiration.^34,36

No uniform decision pathway for deciding when a child should move from oral feeding to enteral tube feedings exists, but there is general consensus.³⁷ If oral calorie intake is insufficient to maintain growth, there is increased risk or occurrence of aspiration into the lungs, or the level of work necessary to maintain adequate caloric intake orally by the individual and the caregiver is excessive, then a medical provider may recommend enteral tube feedings (see Glossary). The method of tube feeding is based on the likely time span needed for tube supplementation, the availability of an experienced surgeon, and specific symptoms of the child. For example, a child may be considered too medically fragile for surgery, so a nasal tube may be used for a time, which may be advanced beyond the stomach into the jejunum to reduce gastroesophageal reflux, and then later replaced with a surgically placed tube. A gastric fundoplication may be included to reduce GER, if needed in the judgment of the surgeon.

Clinical Uncertainties

The goal for management of CP is to improve the quality of life for both the child and family, through interventions that maximize independence in activities of daily living, mobility, and nutrition. Guidelines have been published by the American Academy of Neurology on the use of pharmacologic treatment of spasticity in children and adolescents with CP.³⁸ However, there is a limited evidence-base for the majority of interventions in CP, including those that address nutrition and growth.³⁹ Despite a range of potential feeding interventions for patients with CP, synthesis is lacking on the efficacy, safety and applicability of these interventions. Limited information is available on the impact on health outcomes, including quality of life. Existing reviews are limited in scope, and clinicians and families will benefit from consolidation of data for making clinical decisions.

Goals of treatment and measures of effectiveness may differ by type of CP (spastic or nonspastic), location of motor involvement (e.g., diplegia, quadriplegia), functional status, including ability to walk or sit, and degree of head and trunk control. Comorbid conditions, particularly intellectual disability (related to ability to monitor and maintain appropriate nutrient intake) as well as concurrent medications that potentially have gastrointestinal side effects may influence treatment outcomes. Different feeding interventions may perform differently across the spectrum of CP. For example, oral-motor interventions may be highly effective in populations with oral dysphagia with malnutrition. However, these same interventions could have less value in less mobile populations that are experiencing pharyngeal dysphagia with aspiration. Gastrostomy feeding may reduce aspiration during swallowing, but does not address aspiration of oral secretions, and could exacerbate GER. Additional interventions, such as positioning and caloric supplementation may still be needed. To examine the overall effectiveness of interventions intended to improve feeding and nutrition outcomes in CP, adequate characterization of the patient populations is essential. Additionally, the need for management into later life has increased, and the optimal interventions for adults with feeding difficulties are unknown.^17,40

Potential harms associated with feeding interventions include surgical complications, new or worsening GER, risk of aspiration, and mortality. Gastrostomy feeding has been associated with excess weight gain.⁴¹ The impact of antireflux procedures in addition to gastrostomy is relatively unknown. Finally, there is a need to understand the potential impact of feeding interventions on families and caregivers as substantial caregiver time and training may be required.

Importance of This Review

Families of children with cerebral palsy face significant challenges in providing the best care for their children. The known high rates of morbidity, including aspiration and pneumonia, associated with feeding difficulties, cause substantial stress and have significant health implications. Collecting what data exist in one location, and assessing the studies objectively, will provide families and clinicians with an overview of potential interventions and what they might expect with them.

Uses of This Report

We anticipate that the report will be of value to clinicians who treat individuals with CP, including pediatricians, occupational and physical therapists, speech and language pathologists, nurses, nutritionists, and other health professionals who provide care for individuals with CP. The report itself is not a guideline. It is a review of evidence that other groups and individuals can use in developing guidelines or treatment decisions, but we assume that those decisions would be made with other considerations as well, including an individual's diagnosis, severity of CP, concomitant conditions, and familial context.

In addition, this review will be of use to the National Institutes of Health, Centers for Medicare & Medicaid Services, and the Health Resources and Services Administration–all of which have offices or bureaus devoted to developmental issues. This report can bring practitioners up to date about the current state of evidence, and it provides an assessment of the quality of studies that aim to determine the outcomes of therapeutic options for the management feeding- and nutrition-related difficulties in CP. It will be of interest to individuals affected by CP and their families because of the significant personal costs associated with it and the recurring need for individuals with CP, their families, and their health care providers to make the best possible decisions among numerous options.

Researchers can obtain a concise analysis of the current state of knowledge in this field. They will be poised to pursue further investigations that are needed to understand best approaches to interventions for feeding and nutrition in CP.