Cerebral-Palsy-Feeding Executive
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Comparative Effectiveness Review Number 94 Effective Health Care Program Interventions for Feeding and Nutrition in Cerebral Palsy Executive Summary Background Effective Health Care Program Cerebral palsy (CP) is a “group of disorders of the development of The Effective Health Care Program movement and posture, causing was initiated in 2005 to provide activity limitation, that is attributed to valid evidence about the comparative non-progressive disturbances that effectiveness of different medical occurred in the developing fetal or interventions. The object is to help infant brain. The motor disorders of consumers, health care providers, cerebral palsy are often accompanied and others in making informed by disturbances of sensation, cognition, choices among treatment alternatives. communication, perception, and/or Through its Comparative Effectiveness behaviour, and/or by a seizure disorder.”1 Reviews, the program supports This group of syndromes ranges in systematic appraisals of existing severity and is the result of a variety scientific evidence regarding of etiologies occurring in the prenatal, treatments for high-priority health perinatal, or postnatal period. Though conditions. It also promotes and the disorder is nonprogressive, the generates new scientific evidence by clinical manifestations may change over identifying gaps in existing scientific time as the brain develops, with other evidence and supporting new research. neurologic impairments frequently The program puts special emphasis co-occurring.1,2 on translating findings into a variety of useful formats for different More than 100,000 children are estimated stakeholders, including consumers. to be affected with CP in the United States. Due to advances in supportive The full report and this summary are medical care, approximately 90 percent available at www.effectivehealthcare. of children with CP survive into ahrq.gov/reports/final.cfm. adulthood, resulting in an additional estimated 400,000 adults living with CP in the United States.3-8 Lifetime costs are estimated to be nearly $1 million per person.9 Effective Health Care 1 Classification and Spectrum of Disorder feeding process may require considerable time and may be associated with stress and caregiver fatigue16; stress and CP includes a spectrum of disorders of movement, posture, fatigue may in turn affect the feeding process.17 A number and coordination with heterogeneous etiologies. The of feeding and oral-motor intervention strategies have been diversity of the clinical features is reflected in multiple developed to address difficulties with sucking, chewing, classification systems that include reference to type of swallowing, and improve oral-motor skills. Strategies motor dysfunction, body parts affected, severity, and include oral sensorimotor management, positioning, oral functional abilities. Further classification is by severity appliances, food thickeners, specialized formulas, and level (mild, moderate, severe), and gross motor function, neuromuscular stimulation. These interventions address which reflects the functional capabilities of the affected.10,11 different aspects of feeding difficulties, reflecting the range Developed in the late 1990s, the Gross Motor Function in specific problems associated with feeding and nutrition Classification System (GMFCS) outlines a standardized in CP. Sensorimotor techniques seek to strengthen oral- system for classifying motor function based on constructs motor control and counteract abnormal tone and reflexes of disability and functional limitation.12 The GMFCS to improve oral feedings, and typically require months includes levels that reflect abilities ranging from walking of daily application. Positioning techniques address poor without limitations (level I) to severe head and trunk postural alignment and control that exacerbates swallowing control limitations requiring extensive use of assisted difficulties, and include stabilizing the neck and trunk. technology, physical assistance, and a wheelchair Positioning interventions are individualized and often (level V). Table A summarizes criteria used in widely guided by video-fluoroscopy to optimize swallowing. Oral accepted classification systems. appliances have been used to stabilize the jaw, improve The epidemiologic Oxford Feeding Study reported sucking, tongue coordination, lip control, and chewing. significant correlations between severity of motor Multiple approaches may be used in children with growth impairment and feeding problems including choking, failure. For children with moderate to severe aspiration underweight, prolonged feeding times, vomiting, and need or malnutrition related to oral-pharyngeal dysphagia 13 for gastrostomy feeding (p values typically <0.005). and GER, surgical interventions with gastrostomy (tube Although CP is a motor disorder, many children and feeding directly into the stomach) or jejunostomy tubes adults with CP are affected by other developmental (tube feeding into the middle portion of the small intestine, disabilities, including intellectual disability, impaired the jejunum) and antireflux procedures are often deemed vision and hearing, language and behavioral disorders, and necessary to improve nutritional status and reduce risk of 11,14,15 epilepsy. Survival and quality of life vary across the chronic aspiration.16,18 spectrum of CP, but both are associated with severity and functional disabilities, as well as comorbid conditions.15 No uniform decision pathway exists for deciding when a child should move from oral feeding to enteral Feeding Difficulties and Interventions tube feedings, but there is general consensus.19 If oral Individuals with CP frequently have feeding and calorie intake is insufficient to maintain growth, there is swallowing problems that may lead to poor nutritional increased risk or occurrence of aspiration into the lungs, status, growth failure, chronic aspiration, esophagitis, and or the level of work necessary to maintain adequate respiratory infections. Across the cerebral palsy spectrum, caloric intake orally by the individual and the caregiver poor nutritional status is caused by distinct pathways is excessive, then a medical provider may recommend ranging from inadequate intake, oral dysphagia, oral- enteral tube feedings (see Glossary). The method of pharyngeal dysphagia, gastroesophageal reflux, chronic tube feeding is based on the likely time span needed for aspiration, and behavioral etiologies. Some patients with tube supplementation, the availability of an experienced oral-pharyngeal dysphagia and gastroesophageal reflux surgeon, and specific symptoms of the child. For example, (GER), particularly those with severe CP, are also at a child may be considered too medically fragile for risk for recurrent aspiration, which can lead to chronic surgery, so a nasal tube may be used for a time, which pulmonary disease. Patients with feeding difficulties range may be advanced beyond the stomach into the jejunum to from those with self-feeding skills to populations with reduce gastroesophageal reflux, then later replaced with severe disability (GMFCS V) who require extensive use a surgically placed tube. A gastric fundoplication may be of assisted technology and are dependent on others to feed included to reduce GER, if needed in the judgment of the them. Caregiver burden is a significant concern as the surgeon. 2 Table A. CP classification systems used and understood by qualified medical practitioners* Gross Motor Function Severity Level Topographical Distribution Motor Function Classification System • Mild: Child can move without • Monoplegia/monoparesis means only • Spastic: Implies increased muscle The GMFCS uses head control, movement assistance; his or her daily one limb is affected. It is believed tone. Muscles continually contract, transition, walking, and gross motor skills activities are not limited. this may be a form of hemiplegia/ making limbs stiff, rigid, and resistant such as running, jumping, and navigating hemiparesis where one limb is to flexing or relaxing. Reflexes can inclined or uneven surfaces to define a child’s • Moderate: Child will need significantly impaired. be exaggerated, while movements accomplishment level. The goal is to present braces, medications, and tend to be jerky and awkward. Arms an idea of how self-sufficient a child can be adaptive technology to • Diplegia/diparesis usually indicates and legs often affected. Tongue, at home, at school, and at outdoor and indoor accomplish daily activities. the legs are affected more than the mouth, and pharynx can be affected, venues. arms; primarily affects the lower body. • Severe: Child will require as well, impairing speech, eating, • GMFCS Level I: Walks without limitations. a wheelchair and will have • Hemiplegia/hemiparesis indicates the breathing, and swallowing. Spastic CP significant challenges in arm and leg on one side of the body is is hypertonic and accounts for 70% • GMFCS Level II: Walks with limitations. accomplishing daily activities. affected. to 80% of CP cases. The injury to the Limitations include walking long distances and balancing, but not as able as Level I to run • No CP: Child has CP signs, • Paraplegia/paraparesis means the brain occurs in the pyramidal tract and or jump; may require use of mobility devices but the disorder was acquired lower half of the body, including both is referred to as upper motor neuron when first learning to walk, usually prior to after completion of brain legs, are affected. damage. age 4; and