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Cerebral palsy: Nutritional Aspects
CEREBRAL PALSY
Nutritional Aspects J Krick and P Miller, Kennedy Krieger Institute, Baltimore, MD, USA r 2013 Elsevier Ltd. All rights reserved.
Glossary Bisphosphonates A class of drugs that prevent the loss of bone mass, used to treat osteoporosis and similar diseases. They are called bisphosphonates because they have two phosphonate (PO3) groups. Cervical auscultation Cervical auscultation with stethoscope or with transducers can be incorporated into the clinical examination to enhance the clinical examiner’s ability to detect aspiration and to determine specialized diet management. Gross Motor Function Classification Scales (GMFCS) The Gross Motor Function Classification
Nutritional issues (e.g., diet, body composition, growth) are integral aspects of medical care for persons with cerebral palsy (CP). This article focuses on CP and its nutritional implications. The first section defines CP and describes its causes, prevalence, and classification types. Associated deficits related to CP are also explored. The topic of nutritional assessment of children with CP includes discussions on growth, body composition, and energy, and protein, fluid, and nutrient needs. Feeding and swallowing problems and the influence of muscle tone on the ability to eat safely are discussed in depth, as are alternative feeding routes. An interdisciplinary approach is emphasized throughout as the ideal model to provide services to people with CP to ensure quality of life in the community.
Definition and Etiology CP refers to a number of nonprogressive disorders of movement and posture that result from an injury to the central nervous system during early brain development (Table 1).
Classification There are several different classifications of CP. The three most common types are pyramidal, extrapyramidal, and mixedtype. The type of CP and the degree of involvement play an important part in nutritional assessment and treatment.
Pyramidal (Spastic) Cerebral Palsy Children with spastic CP have increased muscle tone with a clasped-knife quality. In spastic quadriplegia (30% of cases
Encyclopedia of Human Nutrition, Volume 1
System (GMFCS) is a five-level classification system that describes the gross motor function of children and youth with cerebral palsy on the basis of their self-initiated movement with particular emphasis on sitting, walking, and wheeled mobility. Distinctions between levels are based on functional abilities, the need for assistive technology, including hand-held mobility devices (walkers, crutches, or canes) or wheeled mobility, and to a much lesser extent, quality of movement. Muscle tone Muscle tone refers to the amount of tension or resistance to movement in a muscle.
of pyramidal CP), all four extremities are involved. In spastic diplegia (25%), both lower extremities are spastic with minimal upper extremity involvement. Hemiplegia (45%) implies involvement on only one side of the body, with the upper extremity usually more affected than the lower extremity.
Table 1
Causes of cerebral palsy
Cause
Percentage of cases
Perinatal First trimester Teratogens Genetic syndromes Chromosomal abnormalities Brain malformations Second and third trimesters Intrauterine infections Problems in fetal/placental functioning Labor and delivery Preeclampsia Complications of labor and delivery Perinatal Sepsis/central nervous system infection Asphyxia Prematurity Childhood Meningitis Traumatic brain injury Toxins Not obvious
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Extrapyramidal Cerebral Palsy
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Choreoathetosis involves the presence of abrupt, involuntary movements of the upper and lower extremities. This condition can greatly increase energy expenditure and is further discussed in the section on energy needs.
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Mixed-type Cerebral Palsy
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Mixed-type CP includes characteristics of both the pyramidal and the extrapyramidal types. For example, a child may have rigidity in the upper extremities and spasticity in the lower extremities.
Associated Disabilities/Deficits Associated deficits of CP are important to note because they affect nutritional status. Cognitive impairments are quite common. Intellectual disability occurs in 60% of CP cases, with the remainder at high risk for some type of learning disability. Sensory deficits are prevalent, including those in the visual and auditory modalities. Seizures occur in 20–30% of cases, with the highest proportion in the spastic type. In addition to medical management, the ketogenic diet is often prescribed to treat seizures. It is a high-fat diet with limited nutrients and fluid and requires implementation and monitoring by both a neurologist and a nutritionist trained in the diet. Feeding, behavioral, or emotional problems are also frequently noted. Complementary and alternative therapies should be evidence-based, effective, and practical.
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Age (months) Figure 1 Weight-for-age for girls aged 0–120 months. The solid lines represent girls with quadriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
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The goal for nutritional assessment and intervention is to have healthy, alert, interactive individuals who are able to take advantage of all that the environment has to offer. Each person must be able to participate to his or her capacity in the learning and therapeutic habilitative processes and in social, community, and leisure activities.
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Growth The literature describes children with CP who are shorter and lighter than the reference standard. This may be the result of several factors. Individuals with CP have alterations in muscle tone affecting their limbs and torso, depending on the level of severity and topography. They often exhibit muscle contractures, depending on the type of CP; muscle spasticity may retard bone growth. Limited physical activity may impede growth. Immobilization may be required after orthopedic surgery. Immobilization inhibits bone formation and longitudinal growth and results in suppression of certain growth-stimulating hormones. It has been suggested that dysregulation of growth hormone secretion may be another factor affecting growth. A growth reference for children with spastic quadriplegia has been developed to facilitate uniformity in clinical assessment as well as to simplify comparative interpretation of growth data. These growth curves can be seen in Figures 1–6. It is important to always evaluate growth velocity from
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Figure 2 Length-for-age for girls aged 0–120 months. The solid lines represent girls with quardriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
one measurement to another, to aid clinical management. The rate of growth in children with CP is slower so that as they get older, the difference from the standard becomes greater.
Cerebral Palsy: Nutritional Aspects
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Height (cm) Figure 3 Weight-for-length for girls aged 0–120 months. The solid lines represent girls with quadriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
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Figure 5 Length-for-age for boys aged 0–120 months. The solid lines represent boys with quadriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
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Age (months) Figure 4 Weight-for-age for boys aged 0–120 months. The solid lines represent boys with quadriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
Steven Day published the largest retrospective study in 2007 describing growth according to the five distinct levels of motor function using the Gross Motor Function Classification Scales and feeding dysfunction. It reviewed almost
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Figure 6 Weight-for-length for boys aged 0–120 months. The solid lines represent boys with quadriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
25 000 children aged 0–20 years and growth charts were developed. Both nutritional and nonnutritional factors influence growth in children with CP. Nonnutritional influences that
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have been suggested to impact growth include weight-bearing opportunities and, by extension, interventions using aggressive physical therapy, growth hormones, and electrical stimulation of muscle. In 1995, Stevenson reviewed growth in hemiplegics and noted that there is diminished growth, decreased muscle mass, and decreased fat stores on the affected side, and that the magnitude of the differences increases with age and functional severity. Gender, age, cognitive impairment, and ambulatory status have also been noted to contribute to the slow growth seen in this population. Measurement of length or height for individuals with CP may require techniques and standards using arm span, lower leg length, or segmental measurements because of the difficulties encountered with joint contractures and scoliosis. The use of height age, rather than chronological age, is a common technique and is defined as the projected age at which the current child’s height crosses the 50th percentile on the National Center for Health Statistics chart. The use of z scores for length-for-age, weight-for-age, and weight-for-length promotes an accurate evaluation of discrete changes from one measurement date to another. Percentile tables describe ranges, and consequently detection of movement within the range is difficult to describe. The z score denotes standard deviation units from the median and allows the practicing clinician and investigator to pinpoint precisely any given measurement. For screening purposes, conventional length/height and weight measures can be completed and compared to the Centers for Disease Control and Prevention growth charts. Reference standards for body mass index for children with CP do not exist; therefore, one must use body mass index data in conjunction with body composition data to determine adequacy of growth. Researchers from the multicenter North American Growth in Cerebral Palsy Project suggest that a practical method to assess nutritional status in a child with CP is to measure body fat. This can be done in the form of either the triceps skinfold (TSF) or both the TSF and the subscapular skinfold. However, patient cooperation with the measuring techniques, required for accuracy and safety, may be difficult to obtain or maintain. For some individuals with CP, the process may be difficult, and training is needed to learn the technique for body fat measures and segmental measures mentioned previously. When trying to obtain growth measurements, joint contractures, muscle spasms, and poor cooperation will impact accuracy. Upper extremity (arm) length, tibial length, and knee height are often noted in the literature as valid proxies for length in children with CP up to the age of 18. (See Table 2 for estimation of height using segmental measures.)
Ideal Body Weight The estimate of ideal body weight (IBW) is also in part determined by the severity of the CP. The IBW should be aimed at maintaining adequate fat and muscle stores to endure repeated surgeries or a common virus while facilitating daily physical care and management. Weight-for-length is an indicator of nutritional status, which obscures the issue of chronological age and addresses whether the individual is
Table 2
Estimation of height from segmental measures
Age 0–12 years (4.35 UAL) þ 21.8 (3.26 TL) þ 30.8 (2.68 KH) þ 24.2 Age 6–18 years White male (2.22 KH) þ 40.54 Black male (2.18 KH) þ 39.60 White female (2.15 KH) þ 43.21 Black female (2.02 KH) þ 46.59 UAL, upper arm length; TL, tibia length; KH, knee height.
proportionate. IBW can be expressed as this ratio. Those with CP should attain and maintain an IBW that takes into account their age, level of physical ability, and their independence. Measurement of arm anthropometry will provide a description of body composition and support clinical judgments related to IBW. For example, children with spastic quadriplegia are the most dependent and the 10th percentile weightfor-length would be designated as the IBW. However, this assignment is done in tandem with assessment and monitoring of body composition, and if either the arm fat or the arm muscle area were less than the 5th percentile, then the IBW would be adjusted upward.
Body Composition Since the 1970s, researchers reviewing body composition have noted reduced lean body mass in children with CP. Recent work examining adults with CP and their age-matched controls found no difference in lean body mass or percentage of body fat.
Bone Mineral Density Bone mineral density (BMD) is markedly reduced in nonambulatory children with CP, placing them at risk for nontraumatic fractures. Osteopenia defined as o2 standard deviations below the mean BMD was found in the femur of most nonambulatory children by the age of 10. Decreased BMD results from a combination of factors, including immobilization, antiepileptic therapy, and nutritional deficiencies. Serum levels of calcium, phosphate, alkaline phosphatase, and osteocalcin were not found to be reliable indicators of low BMD when studied by Henderson. The same author noted that fracture rate is fourfold higher following spica casting and more than threefold higher following an initial fracture. Many nonambulatory children require, and are given, fewer calories than recommended for their non-CP counterparts; therefore, the clinician is obliged to review the adequacy of the micronutrients, specifically calcium. Serum vitamin D levels should be evaluated and the nutrition should be supplemented based on the findings. Most likely, their diets will require supplementation to meet 100% of the dietary reference intake (DRI) standards for age and gender. Methods to increase BMD include weight-bearing activities, dietary adequacy, and the use of bisphosphonates. In several
Cerebral Palsy: Nutritional Aspects
studies, bisphosphonate use has demonstrated increased bone density by 20–89% with no obvious adverse effects.
Energy Needs Equations that are frequently used to predict energy requirements were developed using healthy children and adults in usual environmental and physical activity conditions and do not provide an accurate assessment of the needs of those with CP. From a nutritional perspective, wide-ranging studies demonstrate underreporting of energy needs on food records, which at best provide a qualitative measure of intake. Therefore, clinicians have turned to the use of more sophisticated technology, such as doubly labeled water and indirect calorimetry, to assess the energy needs of this population. Additionally, the energy cost of movement, whether it be wheelchair propulsion, crutch ambulation, or the involuntary movements of the individual with athetosis, must be considered. Those with CP may undergo repeated orthopedic surgery that may impair nutritional status, due to increased nutrient and energy demands. It has also been hypothesized that whole body metabolic rate may be related to differences in skeletal muscle fiber proportions and differences in enzymatic activity. People with CP have abnormal variation in the size of muscle fibers and altered distribution of fiber types. Altered energy needs are common among those with CP and differ widely from the norm. Clinicians use a variety of approaches to estimate energy needs, such as the DRIs for chronological age, the recommended daily allowances forheight age, and the World Health Organization equation. When estimating energy needs, information related to muscle tone, activity level, and needs for growth or catch-up growth must be added to the estimate for resting energy expenditure (REE). The equation designed specifically for this population is REE muscle tone factor activity factor þ growth factorðsÞ ¼ kilocalories per day The REE can be determined using indirect calorimetry or can be derived from estimating body surface area standard metabolic rate for 24 h. Body surface area (m2) is calculated from length and weight using the nomogram derived from the formula of DuBois and DuBois, and the standard metabolic rate (kcal m2 h1) is identified using height, age, and sex by applying Fleisch data. The modifying factors applied are as follows:
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Muscle tone factors: Multiply by 10% for high tone (hypertonicity) and decrease by 10% for low tone (hypotonicity); no adjustment for normal tone. Activity factors: Multiply by 15% for bedridden state, 20% for wheelchair, and 30% for ambulation. Growth factors: Add 5 kcal (20.92 kJ) per gram of desired growth, expected growth, and catch-up.
Energy needs must be viewed on an individual basis assimilating the concepts noted previously. The use of any approach is regarded as a guidepost and requires careful monitoring of body weight. Modifications to the diet should be based on clinical observation and measurement. There is a
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subset of individuals with CP who require significantly less kilocalories than anticipated (as few as perhaps 15 kcal kg1). The use of intrathecal Baclofen to treat spasticity can also reduce muscle tone and, therefore, energy needs.
Nutrient and Fluid Needs Nutrient and protein needs are based on DRIs similar to those of the population without CP. Height age is often used in these determinations. Fluid needs are based on body size rather than calorie intake. Table 3 demonstrates how to calculate fluid needs. Constipation is a chronic problem for most children with CP and is related to muscle tone, loss of sensation, limited physical activity, medication side effects, and inadequate dietary fiber intake or fluid intake. Oral motor dysfunction results in diminished intake as well as in food and fluid loss. Modified food and fluid textures result in less free water and fiber in the diet. Discomfort associated with constipation may decrease appetite and increase gastroesophageal reflux (GER). Dietary intervention may therefore be limited and medical management may be necessary.
Assessment of Feeding Skills and Safety Eating skills are acquired in a sequential pattern so that a developmental history will be helpful in evaluating current function and planning treatment options. Factors affecting feeding performance are shown in Table 4.
Oral Motor Evaluation Feeding and swallowing problems are common in the child with CP, depending on the type of muscle tone, the presence of primitive reflexes, movement patterns, and the integrity of the sensory system. Clinical indicators of feeding and swallowing dysfunction are shown in Table 5. Problems often include poor intake, inefficient and lengthy mealtimes, abnormal oral motor patterns, inappropriate progression of feeding skills, and physiological compromise with feeding. Sensory, cognitive, and language deficits may also complicate the feeding process. An interdisciplinary team evaluation is essential for the assessment and development of appropriate
Table 3
Fluid needs based on body weighta
Body weight (kg)
Fluid need (cm3 kg1)
r10 11–20 Z21
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Suggest monitoring urine-specific gravities when available and quantity, color, and odor of urine and adjust for periods of stress and temperature. Example: 28-kg child.
100 ml 10 kg ¼ 1000 ml 50 ml 10 kg ¼ 500 ml 25 ml 8 kg ¼ 200 ml Total need ¼ 1700 ml.
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Table 4
Cerebral Palsy: Nutritional Aspects
General factors affecting feeding performance
Neuromotor performance Perceptual deficits Cognition and communication skills Vision and hearing Behavior/interaction Growth Dietary adequacy GER and other gastrointestinalrelated issues
Table 5
Constipation Amount of physical and verbal assistance required Physiological support Oral motor skills and swallowing status Medications Dental and gum disease Multiple orthopedic procedures Family/psychosocial stressors
Clinical indicators of feeding and swallowing dysfunction
Congestion Noisy ‘wet’ sounds Multiple swallows to clear bolus Unexplained fevers, unexplained irritability Coughing/choking/gagging before, during, or after swallow Food refusal
Difficulty managing secretions History of upper respiratory infections Apnea during feeding Failure to thrive, failure to maintain weight
goals and facilitation of a treatment plan that respects the developmental progression. A clinical assessment of the feeding process should include observance of facial muscle tone, oral reflex activity, functional oral motor skills, structural abnormalities, sensory responses, behavior and interaction during feeding, respiratory and phonatory status, and posture and positioning. Radiographic and ultrasound studies can provide more detailed information about the oral structures and the competency of the oral, pharyngeal, and esophageal phases, including the detection of aspiration. Cervical auscultation can also be helpful in evaluating the pharyngeal phase of swallowing. In addition, these techniques can assist in determining the suitable solid and liquid texture and appropriate head and neck positioning. Hypertonicity leads to abnormal movements of the tongue, lip, and jaw. These abnormal movements can be manifested as tongue retraction, tongue tip elevation, tongue thrust, tonic biting, jaw thrust, jaw instability, lip retraction, and lip/cheek instability. An abnormally strong gag reflex, tactile hypersensitivity in the oral area, and drooling can also complicate feeding.
Dental Increased incidence of cavities and erosion frequently occur due to poor oral hygiene and teeth grinding. Hypersensitivity in the oral area and hyperplasia of the gums from long-term use of phenytoin may also be seen. Malocclusion is a common musculoskeletal problem and contributes to drooling, which can negatively affect daily oral care. Injury to the mouth can be seen following falls, other accidents, or physical abuse.
Aspiration and Gastroesophageal Reflux Clinical signs of aspiration may include coughing, choking, gagging, inability to handle oral secretions, wet upper airway sounds with poor vocal quality, apnea, food refusal, frequent upper respiratory infections, and aspiration pneumonia. Aspiration of food may occur without physical evidence if the protective cough or gag is not functioning, sensory deficits exist, and the swallowing mechanism is dysfunctional. This results in what is termed silent aspiration. Although aspiration from solid food can be detected, the possibility of aspiration from GER may also need to be considered. The regurgitation of gastric contents from the stomach into the esophagus can lead to irritability during or after feeding, arching, esophagitis, and ultimately food refusal. Other symptoms of GER include respiratory compromise, apnea, and drooling. Treatment for GER includes the use of antacids (H2 blockers or proton pump inhibitors), medications to increase gastrointestinal motility, reduction in feeding rate, positioning, thickening of foods or liquids, or surgical intervention. Small, frequent feedings help to decrease the volume in the stomach at one time. Fatigue may occur in the child who is not able to sustain the work involved with feeding and may be expressed by an increase in respiratory rate, diaphoresis, or increased work of breathing. The causes may be muscular, respiratory, or cardiac, and they may increase the risk of aspiration or hypoxia. The work required to eat a meal is accomplished at a higher physiological cost to the child, thereby increasing caloric needs.
Muscle Tone and Positioning It is important to understand the influences of muscle tone and proper positioning on the ability to eat safely and efficiently in this population. Increased or decreased muscle tone contributes to difficulty preserving a patent airway, compromised self-feeding skills, poor rib cage expansion and esophageal motility, and difficulty maintaining a stable supported base for seating. Fluctuating muscle tone leads to involuntary movements and limited postural stability. Despite the type of muscle tone, optimal positioning is crucial for feeding and swallowing. The proper feeding position includes neutral alignment of head and neck, midline orientation, symmetrical trunk position, 901 pelvic/femoral alignment, and symmetrical arm position with neutral shoulders. An example of proper positioning can be seen in Figure 7. Consultations with orthopedists and rehabilitation physicians to address current and potential musculoskeletal problems, physical and occupational therapists for functional assessment, orthotists for deformity management, and durable medical equipment specialists to customize standard wheelchair components are valuable.
Underweight and Overweight Overweight Most children with CP who are overweight or obese have low muscle tone. Their nutritional status impacts sleeping and breathing patterns, mobility, physical care, and peer
Cerebral Palsy: Nutritional Aspects Chin tuck with neck elongation
Arms free and forward for function
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Most people can be treated nonsurgically with gastric aspiration and nasojejunal or gastrojejunal feedings distal to the obstruction. One study also found that turning to the left from a supine position displaces the SMA from the right to the left side of the aorta in scoliosis cases. Thus, positioning can help alleviate symptoms and special considerations may be indicated in light of the limitations imposed by the CP.
Behaviors at Mealtimes Parent–child interactions can also influence feedings. Ineffective communication, lack of bonding, the absence of social interaction or poor interactive skills, family dysfunction, and decreased environmental stimuli can exacerbate feeding difficulties or lead to frustration and anxiety with subsequent food refusal or parental withdrawal. Aversion to oral feeds can also be an outcome of medical complications, such as esophagitis and GER, or lack of feeding experience at critical milestones secondary to prolonged tube feedings. Behavioral treatment should only be undertaken after thorough medical, nutritional, and neurodevelopmental assessments are completed.
Feeding Issues
Erect spine over pelvis perpendicular to support surface Figure 7 Proper seating position.
relationships. It is difficult to attain an IBW because energy needs are significantly reduced and the options for exercise are limited.
Underweight Typically, children with athetosis struggle to maintain weight given their excessive involuntary movements, which significantly increase energy needs. As these children age, the problem becomes more apparent, and many of these children will require enteral supplementation. One evaluation of this population noted that the basal energy requirement was 40% higher than expected.
Superior Mesenteric Artery Syndrome Superior mesenteric artery (SMA) syndrome is a condition in which the third portion of the duodenum is intermittently compressed by the overlying SMA, resulting in gastrointestinal obstruction. Symptoms include recurrent vomiting, abdominal distension, weight loss, and postprandial distress. People with CP are at high risk for several of the reported causes of SMA syndrome, including body cast compression, severe weight loss, prolonged supine positioning, and scoliosis surgery. Consequently, it is important to recognize the symptoms and know the appropriate treatments for this syndrome.
The feeding plan should be safe, promote growth or weight maintenance without excessive energy expenditure to obtain the required calories, and meet the needs of the family. It should reflect their resources in time and skill, and it should address their concerns and expectations. The goals for treatment once feeding and swallowing problems are identified are: to prevent aspiration and thereby respiratory compromise; to provide adequate calories, protein, vitamins, minerals, and fluid; and to educate caregivers regarding nutritional requirements.
Oral Motor Considerations Management strategies for daily mealtime feeding include positioning, modification of the sensory properties of the food, oral motor facilitation techniques, and equipment adaptations. For individuals with increased energy needs, the nutrient density of their meals may need to be maximized. Table 6 lists commonly used calorie boosters. It is important to acknowledge the inability to change the underlying feeding problem while providing a method of circumventing the problem to allow adequate nutrition and growth. For example, facilitative techniques to minimize excessive jaw movement may entail the feeder providing physical jaw control/support; a change in the food consistency, texture, temperature, or taste to improve the ability to propel a bolus through the oropharynx; the careful selection of adaptive feeding equipment to assist with self-feeding or increased intake; and an appropriate seating system. Proper positioning also allows the feeder to use both hands.
Alternative Feeding Routes Many children with CP are not able to meet some or all of their calorie needs by mouth due to one or more of the
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Table 6
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Calorie boosters
Instant breakfast Powdered, evaporated milk Whole milk cheeses Peanut butter Wheat germ Yogurt, pudding, custards Milkshakes, eggnog Commercial supplements
Table 7 Margarine, butter, oils, gravy Sugar, honey, syrup Cream cheese Sour cream Concentrate juices Breading or cracker meal Fruit canned in heavy syrup
following conditions: oral motor dysfunction, excessive energy needs, recurrent infections, illnesses, and orthopedic surgical interventions. Consequently, if the gastrointestinal tract is functioning, supplemental or total tube feedings may be indicated. Early intervention with enteral nutrition may prevent protein–energy malnutrition and its complications. Studies have shown improvements in weight gain (fat mass as opposed to fat-free mass) with supplemental tube feedings, which better enables individuals to endure short-term medical insults. Enteral nutrition may be delivered by nasogastric, nasojejunal, gastrostomy, gastrostomy–jejunal, and jejunostomy tubes. The degree of GER and risk of aspiration determine where the tube is placed, whereas the length of time needed for tube feedings determines whether a nasoenteral or surgically placed tube is required. The decision regarding continuous, intermittent, or combination tube feeds is dependent on the individual needs of the patient. Tube feedings should be considered a tool to improve nutritional status rather than failure of the child’s ability to eat. Based on the medical diagnosis and developmental stage of the child, the prognosis for return to oral feeding varies, and the length of time to achieve this goal is extremely variable. For some children, the goal of returning to full or partial oral feeding is not realistic. In a study evaluating the health of children with CP, Liptak describes those who were tube fed as having the lowest mental age, requiring the most health-care resources, using the most medications, and having the most respiratory problems. These children were characterized as especially frail and required numerous health-related resources and treatments. Oral motor therapy should focus on maintaining existing oral motor skills, encouraging pleasurable oral experiences, and tolerance of oral hygiene practices. Nonnutritive oral stimulation must be performed when tube feedings are employed as the route of nutrition. The benefits of nonnutritive oral stimulation are listed in Table 7. Improvement in nutritional status can result in positive changes in oral feeding. Parenteral nutrition should only be used when the gastrointestinal tract is dysfunctional. When initiating feedings in patients with major weight loss or failure to thrive, whether enteral or parenteral nutrition is used, it is important to be aware of the ‘refeeding syndrome.’ This syndrome refers to phosphorus depletion and alterations in potassium, magnesium, and glucose metabolism, resulting in severe metabolic and physiological complications. It is imperative to increase calorie delivery slowly with close laboratory monitoring.
Benefits of nonnutritive oral stimulation
Maintains oral sensation and tolerance Facilitates saliva production, swallowing, and other oral motor patterns Maintains or develops coordination of respiration and swallowing Facilitates parent–child interactions
Medications Drug–nutrient interactions should be considered for all children receiving long-term medications for seizure disorders, alterations in muscle tone, attentional deficits, gastrointestinal disorders, and other chronic conditions. One drug or the combination of multiple drugs may affect nutrition in many ways, such as causing decreased appetite, interference with absorption of specific nutrients, nausea, and vomiting. Medication treatment options offer challenges to nutrition. For instance, diazepam, often used to decrease spasticity, increases the potential for drooling. This raises concerns of fluid loss/balance as well as loss of the protective effect of saliva on esophageal mucosa. Additionally, attention must be paid to tone reduction in the trunk and oral structures that would compromise safety of feeding skills. Tone-lowering drugs potentially reduce energy expenditure and, as a result, require increased vigilance to avert excessive weight gain.
Repeated Orthopedic Surgeries These are common in children with CP, and each surgery must be preceded by an evaluation of nutritional status and assessment of the child’s ability to physically heal and recover quickly from the trauma. Many children who are marginal oral feeders will decompensate, lose weight, and have a difficult time healing because of a cascade of events including pain, poor positioning for safe feeding, worsening constipation, minimal intake, lethargy, and increased medications for pain that may have a sedative effect. They may require supplemental feedings before surgery or during the postoperative period.
Coordinated Services The provision of nutrition services and prevention of further disabling conditions can be done in a variety of health care, school, vocational, home, and community settings. Participation in physical fitness activities targeting muscle strength and cardiorespiratory fitness should be encouraged to prevent secondary conditions such as chronic pain, fatigue, and osteoporosis. It is the responsibility of the family in concert with the health-care team to promote nutrition care planning in these settings. More than 90% of children with CP live to adulthood; however, their life expectancy is less than that of the general population. Similar to the general population, individuals with CP are susceptible to developing obesity, hypertension, diabetes, and heart disease and, therefore, require awareness and adherence to prudent dietary guidelines. The chronicity of nutrition problems for
Cerebral Palsy: Nutritional Aspects
individuals with CP is recognized and has in part created a need for care coordination and integrated service planning to provide meaningful and cost-effective services.
See also: Energy Expenditure: Doubly Labeled Water; Indirect Calorimetry. Nutritional Support: Adults, Enteral
Further Reading Bell KL, Boyd RN, Tweedy SM, Weir KA, Stevenson RD, and Davies PSW (2010) A prospective, longitudinal study of growth, nutrition and sedentary behaviour in young children with cerebral palsy. BMC Public Health 10: 179. Brooks J, Day S, Shavelle R, and Strauss D (2011) Low weight, morbidity, and mortality in children with cerebral palsy: New clinical growth charts. Pediatrics 128(2): e299–e307. Case-Smith J (ed.) (1993) Pediatric Occupational Therapy and Early Intervention. Stoneham, UK: Butterworth-Heinemann. Cherney L (1994) Clinical Management of Dysphagia in Adults and Children. Gaithersburg, MD: Aspen. Day SM, Strauss DJ, Vachon PJ, Rosenbloom L, Shavelle RM, and Wu YW (2007) Growth patterns in a population of children and adolescents with cerebral palsy. Developmental Medicine and Child Neurology 49: 167–171.
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Eicher PS and Batshaw ML (1993) Cerebral palsy. Pediatric Clinics of North America 40: 537–551. Ekvall SW (ed.) (1993) Pediatric Nutrition in Chronic Diseases and Developmental Disorders: Prevention, Assessment and Treatment. New York: Oxford University Press. Henderson CR, Lark KR, Gurka JM, et al. (2002) Bone density and metabolism in children and adolescents with moderate to severe cerebral palsy. Pediatrics 110: 1. Hogan SE (1999) Knee height as a predictor of recumbent length for individuals with mobility-impaired cerebral palsy. Journal of the American College of Nutrition 18(2): 201–205. Klein M and Delaney T (1994) Feeding and Nutrition for the Child with Special Needs. Tucson, AZ: Therapy Skill Builders. Krick J, Murphy-Miller P, Zeger S, and Wright E (1996) Pattern of growth in children with cerebral palsy. Journal of the American Dietetic Association 96: 680–685. Liptak GS, O’Donnell M, Conaway M, et al. (2001) Healthstatus of children with moderate to severe CP. Developmental Medicine and Child Neurology 43: 364–370. Samson-Fang LJ and Stevenson RD (1998) Linear growth velocity in children with cerebral palsy. Developmental Medicine and Child Neurology 40(10): 689–692. Samson-Fang LJ and Stevenson RD (2000) Identification of malnutrition in children with cerebral palsy: Poor performance of weight-for-height centiles. Developmental Medicine and Child Neurology 42(3): 162–168. Stallings VA, Charney EB, Davies JC, and Cronk CE (1993) Nutrition related growth failure in children with quadriplegic cerebral palsy. Developmental Medicine and Child Neurology 35: 126–138. Stevenson RD (1995) Use of segmental measures to estimate stature in children with cerebral palsy. Archives of Pediatrics & Adolescent Medicine 149(6): 658–662.
Nutritional Aspects J Krick and P Miller, Kennedy Krieger Institute, Baltimore, MD, USA r 2013 Elsevier Ltd. All rights reserved.
Glossary Bisphosphonates A class of drugs that prevent the loss of bone mass, used to treat osteoporosis and similar diseases. They are called bisphosphonates because they have two phosphonate (PO3) groups. Cervical auscultation Cervical auscultation with stethoscope or with transducers can be incorporated into the clinical examination to enhance the clinical examiner’s ability to detect aspiration and to determine specialized diet management. Gross Motor Function Classification Scales (GMFCS) The Gross Motor Function Classification
Nutritional issues (e.g., diet, body composition, growth) are integral aspects of medical care for persons with cerebral palsy (CP). This article focuses on CP and its nutritional implications. The first section defines CP and describes its causes, prevalence, and classification types. Associated deficits related to CP are also explored. The topic of nutritional assessment of children with CP includes discussions on growth, body composition, and energy, and protein, fluid, and nutrient needs. Feeding and swallowing problems and the influence of muscle tone on the ability to eat safely are discussed in depth, as are alternative feeding routes. An interdisciplinary approach is emphasized throughout as the ideal model to provide services to people with CP to ensure quality of life in the community.
Definition and Etiology CP refers to a number of nonprogressive disorders of movement and posture that result from an injury to the central nervous system during early brain development (Table 1).
Classification There are several different classifications of CP. The three most common types are pyramidal, extrapyramidal, and mixedtype. The type of CP and the degree of involvement play an important part in nutritional assessment and treatment.
Pyramidal (Spastic) Cerebral Palsy Children with spastic CP have increased muscle tone with a clasped-knife quality. In spastic quadriplegia (30% of cases
Encyclopedia of Human Nutrition, Volume 1
System (GMFCS) is a five-level classification system that describes the gross motor function of children and youth with cerebral palsy on the basis of their self-initiated movement with particular emphasis on sitting, walking, and wheeled mobility. Distinctions between levels are based on functional abilities, the need for assistive technology, including hand-held mobility devices (walkers, crutches, or canes) or wheeled mobility, and to a much lesser extent, quality of movement. Muscle tone Muscle tone refers to the amount of tension or resistance to movement in a muscle.
of pyramidal CP), all four extremities are involved. In spastic diplegia (25%), both lower extremities are spastic with minimal upper extremity involvement. Hemiplegia (45%) implies involvement on only one side of the body, with the upper extremity usually more affected than the lower extremity.
Table 1
Causes of cerebral palsy
Cause
Percentage of cases
Perinatal First trimester Teratogens Genetic syndromes Chromosomal abnormalities Brain malformations Second and third trimesters Intrauterine infections Problems in fetal/placental functioning Labor and delivery Preeclampsia Complications of labor and delivery Perinatal Sepsis/central nervous system infection Asphyxia Prematurity Childhood Meningitis Traumatic brain injury Toxins Not obvious
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Mixed-type CP includes characteristics of both the pyramidal and the extrapyramidal types. For example, a child may have rigidity in the upper extremities and spasticity in the lower extremities.
Associated Disabilities/Deficits Associated deficits of CP are important to note because they affect nutritional status. Cognitive impairments are quite common. Intellectual disability occurs in 60% of CP cases, with the remainder at high risk for some type of learning disability. Sensory deficits are prevalent, including those in the visual and auditory modalities. Seizures occur in 20–30% of cases, with the highest proportion in the spastic type. In addition to medical management, the ketogenic diet is often prescribed to treat seizures. It is a high-fat diet with limited nutrients and fluid and requires implementation and monitoring by both a neurologist and a nutritionist trained in the diet. Feeding, behavioral, or emotional problems are also frequently noted. Complementary and alternative therapies should be evidence-based, effective, and practical.
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Age (months) Figure 1 Weight-for-age for girls aged 0–120 months. The solid lines represent girls with quadriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
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The goal for nutritional assessment and intervention is to have healthy, alert, interactive individuals who are able to take advantage of all that the environment has to offer. Each person must be able to participate to his or her capacity in the learning and therapeutic habilitative processes and in social, community, and leisure activities.
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Growth The literature describes children with CP who are shorter and lighter than the reference standard. This may be the result of several factors. Individuals with CP have alterations in muscle tone affecting their limbs and torso, depending on the level of severity and topography. They often exhibit muscle contractures, depending on the type of CP; muscle spasticity may retard bone growth. Limited physical activity may impede growth. Immobilization may be required after orthopedic surgery. Immobilization inhibits bone formation and longitudinal growth and results in suppression of certain growth-stimulating hormones. It has been suggested that dysregulation of growth hormone secretion may be another factor affecting growth. A growth reference for children with spastic quadriplegia has been developed to facilitate uniformity in clinical assessment as well as to simplify comparative interpretation of growth data. These growth curves can be seen in Figures 1–6. It is important to always evaluate growth velocity from
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Figure 2 Length-for-age for girls aged 0–120 months. The solid lines represent girls with quardriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
one measurement to another, to aid clinical management. The rate of growth in children with CP is slower so that as they get older, the difference from the standard becomes greater.
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Figure 5 Length-for-age for boys aged 0–120 months. The solid lines represent boys with quadriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
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Steven Day published the largest retrospective study in 2007 describing growth according to the five distinct levels of motor function using the Gross Motor Function Classification Scales and feeding dysfunction. It reviewed almost
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Figure 6 Weight-for-length for boys aged 0–120 months. The solid lines represent boys with quadriplegic cerebral palsy and the dotted lines represent the National Center for Health Statistics standard curves for 10th, 50th, and 90th percentiles.
25 000 children aged 0–20 years and growth charts were developed. Both nutritional and nonnutritional factors influence growth in children with CP. Nonnutritional influences that
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have been suggested to impact growth include weight-bearing opportunities and, by extension, interventions using aggressive physical therapy, growth hormones, and electrical stimulation of muscle. In 1995, Stevenson reviewed growth in hemiplegics and noted that there is diminished growth, decreased muscle mass, and decreased fat stores on the affected side, and that the magnitude of the differences increases with age and functional severity. Gender, age, cognitive impairment, and ambulatory status have also been noted to contribute to the slow growth seen in this population. Measurement of length or height for individuals with CP may require techniques and standards using arm span, lower leg length, or segmental measurements because of the difficulties encountered with joint contractures and scoliosis. The use of height age, rather than chronological age, is a common technique and is defined as the projected age at which the current child’s height crosses the 50th percentile on the National Center for Health Statistics chart. The use of z scores for length-for-age, weight-for-age, and weight-for-length promotes an accurate evaluation of discrete changes from one measurement date to another. Percentile tables describe ranges, and consequently detection of movement within the range is difficult to describe. The z score denotes standard deviation units from the median and allows the practicing clinician and investigator to pinpoint precisely any given measurement. For screening purposes, conventional length/height and weight measures can be completed and compared to the Centers for Disease Control and Prevention growth charts. Reference standards for body mass index for children with CP do not exist; therefore, one must use body mass index data in conjunction with body composition data to determine adequacy of growth. Researchers from the multicenter North American Growth in Cerebral Palsy Project suggest that a practical method to assess nutritional status in a child with CP is to measure body fat. This can be done in the form of either the triceps skinfold (TSF) or both the TSF and the subscapular skinfold. However, patient cooperation with the measuring techniques, required for accuracy and safety, may be difficult to obtain or maintain. For some individuals with CP, the process may be difficult, and training is needed to learn the technique for body fat measures and segmental measures mentioned previously. When trying to obtain growth measurements, joint contractures, muscle spasms, and poor cooperation will impact accuracy. Upper extremity (arm) length, tibial length, and knee height are often noted in the literature as valid proxies for length in children with CP up to the age of 18. (See Table 2 for estimation of height using segmental measures.)
Ideal Body Weight The estimate of ideal body weight (IBW) is also in part determined by the severity of the CP. The IBW should be aimed at maintaining adequate fat and muscle stores to endure repeated surgeries or a common virus while facilitating daily physical care and management. Weight-for-length is an indicator of nutritional status, which obscures the issue of chronological age and addresses whether the individual is
Table 2
Estimation of height from segmental measures
Age 0–12 years (4.35 UAL) þ 21.8 (3.26 TL) þ 30.8 (2.68 KH) þ 24.2 Age 6–18 years White male (2.22 KH) þ 40.54 Black male (2.18 KH) þ 39.60 White female (2.15 KH) þ 43.21 Black female (2.02 KH) þ 46.59 UAL, upper arm length; TL, tibia length; KH, knee height.
proportionate. IBW can be expressed as this ratio. Those with CP should attain and maintain an IBW that takes into account their age, level of physical ability, and their independence. Measurement of arm anthropometry will provide a description of body composition and support clinical judgments related to IBW. For example, children with spastic quadriplegia are the most dependent and the 10th percentile weightfor-length would be designated as the IBW. However, this assignment is done in tandem with assessment and monitoring of body composition, and if either the arm fat or the arm muscle area were less than the 5th percentile, then the IBW would be adjusted upward.
Body Composition Since the 1970s, researchers reviewing body composition have noted reduced lean body mass in children with CP. Recent work examining adults with CP and their age-matched controls found no difference in lean body mass or percentage of body fat.
Bone Mineral Density Bone mineral density (BMD) is markedly reduced in nonambulatory children with CP, placing them at risk for nontraumatic fractures. Osteopenia defined as o2 standard deviations below the mean BMD was found in the femur of most nonambulatory children by the age of 10. Decreased BMD results from a combination of factors, including immobilization, antiepileptic therapy, and nutritional deficiencies. Serum levels of calcium, phosphate, alkaline phosphatase, and osteocalcin were not found to be reliable indicators of low BMD when studied by Henderson. The same author noted that fracture rate is fourfold higher following spica casting and more than threefold higher following an initial fracture. Many nonambulatory children require, and are given, fewer calories than recommended for their non-CP counterparts; therefore, the clinician is obliged to review the adequacy of the micronutrients, specifically calcium. Serum vitamin D levels should be evaluated and the nutrition should be supplemented based on the findings. Most likely, their diets will require supplementation to meet 100% of the dietary reference intake (DRI) standards for age and gender. Methods to increase BMD include weight-bearing activities, dietary adequacy, and the use of bisphosphonates. In several
Cerebral Palsy: Nutritional Aspects
studies, bisphosphonate use has demonstrated increased bone density by 20–89% with no obvious adverse effects.
Energy Needs Equations that are frequently used to predict energy requirements were developed using healthy children and adults in usual environmental and physical activity conditions and do not provide an accurate assessment of the needs of those with CP. From a nutritional perspective, wide-ranging studies demonstrate underreporting of energy needs on food records, which at best provide a qualitative measure of intake. Therefore, clinicians have turned to the use of more sophisticated technology, such as doubly labeled water and indirect calorimetry, to assess the energy needs of this population. Additionally, the energy cost of movement, whether it be wheelchair propulsion, crutch ambulation, or the involuntary movements of the individual with athetosis, must be considered. Those with CP may undergo repeated orthopedic surgery that may impair nutritional status, due to increased nutrient and energy demands. It has also been hypothesized that whole body metabolic rate may be related to differences in skeletal muscle fiber proportions and differences in enzymatic activity. People with CP have abnormal variation in the size of muscle fibers and altered distribution of fiber types. Altered energy needs are common among those with CP and differ widely from the norm. Clinicians use a variety of approaches to estimate energy needs, such as the DRIs for chronological age, the recommended daily allowances forheight age, and the World Health Organization equation. When estimating energy needs, information related to muscle tone, activity level, and needs for growth or catch-up growth must be added to the estimate for resting energy expenditure (REE). The equation designed specifically for this population is REE muscle tone factor activity factor þ growth factorðsÞ ¼ kilocalories per day The REE can be determined using indirect calorimetry or can be derived from estimating body surface area standard metabolic rate for 24 h. Body surface area (m2) is calculated from length and weight using the nomogram derived from the formula of DuBois and DuBois, and the standard metabolic rate (kcal m2 h1) is identified using height, age, and sex by applying Fleisch data. The modifying factors applied are as follows:
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Muscle tone factors: Multiply by 10% for high tone (hypertonicity) and decrease by 10% for low tone (hypotonicity); no adjustment for normal tone. Activity factors: Multiply by 15% for bedridden state, 20% for wheelchair, and 30% for ambulation. Growth factors: Add 5 kcal (20.92 kJ) per gram of desired growth, expected growth, and catch-up.
Energy needs must be viewed on an individual basis assimilating the concepts noted previously. The use of any approach is regarded as a guidepost and requires careful monitoring of body weight. Modifications to the diet should be based on clinical observation and measurement. There is a
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subset of individuals with CP who require significantly less kilocalories than anticipated (as few as perhaps 15 kcal kg1). The use of intrathecal Baclofen to treat spasticity can also reduce muscle tone and, therefore, energy needs.
Nutrient and Fluid Needs Nutrient and protein needs are based on DRIs similar to those of the population without CP. Height age is often used in these determinations. Fluid needs are based on body size rather than calorie intake. Table 3 demonstrates how to calculate fluid needs. Constipation is a chronic problem for most children with CP and is related to muscle tone, loss of sensation, limited physical activity, medication side effects, and inadequate dietary fiber intake or fluid intake. Oral motor dysfunction results in diminished intake as well as in food and fluid loss. Modified food and fluid textures result in less free water and fiber in the diet. Discomfort associated with constipation may decrease appetite and increase gastroesophageal reflux (GER). Dietary intervention may therefore be limited and medical management may be necessary.
Assessment of Feeding Skills and Safety Eating skills are acquired in a sequential pattern so that a developmental history will be helpful in evaluating current function and planning treatment options. Factors affecting feeding performance are shown in Table 4.
Oral Motor Evaluation Feeding and swallowing problems are common in the child with CP, depending on the type of muscle tone, the presence of primitive reflexes, movement patterns, and the integrity of the sensory system. Clinical indicators of feeding and swallowing dysfunction are shown in Table 5. Problems often include poor intake, inefficient and lengthy mealtimes, abnormal oral motor patterns, inappropriate progression of feeding skills, and physiological compromise with feeding. Sensory, cognitive, and language deficits may also complicate the feeding process. An interdisciplinary team evaluation is essential for the assessment and development of appropriate
Table 3
Fluid needs based on body weighta
Body weight (kg)
Fluid need (cm3 kg1)
r10 11–20 Z21
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Suggest monitoring urine-specific gravities when available and quantity, color, and odor of urine and adjust for periods of stress and temperature. Example: 28-kg child.
100 ml 10 kg ¼ 1000 ml 50 ml 10 kg ¼ 500 ml 25 ml 8 kg ¼ 200 ml Total need ¼ 1700 ml.
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Table 4
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General factors affecting feeding performance
Neuromotor performance Perceptual deficits Cognition and communication skills Vision and hearing Behavior/interaction Growth Dietary adequacy GER and other gastrointestinalrelated issues
Table 5
Constipation Amount of physical and verbal assistance required Physiological support Oral motor skills and swallowing status Medications Dental and gum disease Multiple orthopedic procedures Family/psychosocial stressors
Clinical indicators of feeding and swallowing dysfunction
Congestion Noisy ‘wet’ sounds Multiple swallows to clear bolus Unexplained fevers, unexplained irritability Coughing/choking/gagging before, during, or after swallow Food refusal
Difficulty managing secretions History of upper respiratory infections Apnea during feeding Failure to thrive, failure to maintain weight
goals and facilitation of a treatment plan that respects the developmental progression. A clinical assessment of the feeding process should include observance of facial muscle tone, oral reflex activity, functional oral motor skills, structural abnormalities, sensory responses, behavior and interaction during feeding, respiratory and phonatory status, and posture and positioning. Radiographic and ultrasound studies can provide more detailed information about the oral structures and the competency of the oral, pharyngeal, and esophageal phases, including the detection of aspiration. Cervical auscultation can also be helpful in evaluating the pharyngeal phase of swallowing. In addition, these techniques can assist in determining the suitable solid and liquid texture and appropriate head and neck positioning. Hypertonicity leads to abnormal movements of the tongue, lip, and jaw. These abnormal movements can be manifested as tongue retraction, tongue tip elevation, tongue thrust, tonic biting, jaw thrust, jaw instability, lip retraction, and lip/cheek instability. An abnormally strong gag reflex, tactile hypersensitivity in the oral area, and drooling can also complicate feeding.
Dental Increased incidence of cavities and erosion frequently occur due to poor oral hygiene and teeth grinding. Hypersensitivity in the oral area and hyperplasia of the gums from long-term use of phenytoin may also be seen. Malocclusion is a common musculoskeletal problem and contributes to drooling, which can negatively affect daily oral care. Injury to the mouth can be seen following falls, other accidents, or physical abuse.
Aspiration and Gastroesophageal Reflux Clinical signs of aspiration may include coughing, choking, gagging, inability to handle oral secretions, wet upper airway sounds with poor vocal quality, apnea, food refusal, frequent upper respiratory infections, and aspiration pneumonia. Aspiration of food may occur without physical evidence if the protective cough or gag is not functioning, sensory deficits exist, and the swallowing mechanism is dysfunctional. This results in what is termed silent aspiration. Although aspiration from solid food can be detected, the possibility of aspiration from GER may also need to be considered. The regurgitation of gastric contents from the stomach into the esophagus can lead to irritability during or after feeding, arching, esophagitis, and ultimately food refusal. Other symptoms of GER include respiratory compromise, apnea, and drooling. Treatment for GER includes the use of antacids (H2 blockers or proton pump inhibitors), medications to increase gastrointestinal motility, reduction in feeding rate, positioning, thickening of foods or liquids, or surgical intervention. Small, frequent feedings help to decrease the volume in the stomach at one time. Fatigue may occur in the child who is not able to sustain the work involved with feeding and may be expressed by an increase in respiratory rate, diaphoresis, or increased work of breathing. The causes may be muscular, respiratory, or cardiac, and they may increase the risk of aspiration or hypoxia. The work required to eat a meal is accomplished at a higher physiological cost to the child, thereby increasing caloric needs.
Muscle Tone and Positioning It is important to understand the influences of muscle tone and proper positioning on the ability to eat safely and efficiently in this population. Increased or decreased muscle tone contributes to difficulty preserving a patent airway, compromised self-feeding skills, poor rib cage expansion and esophageal motility, and difficulty maintaining a stable supported base for seating. Fluctuating muscle tone leads to involuntary movements and limited postural stability. Despite the type of muscle tone, optimal positioning is crucial for feeding and swallowing. The proper feeding position includes neutral alignment of head and neck, midline orientation, symmetrical trunk position, 901 pelvic/femoral alignment, and symmetrical arm position with neutral shoulders. An example of proper positioning can be seen in Figure 7. Consultations with orthopedists and rehabilitation physicians to address current and potential musculoskeletal problems, physical and occupational therapists for functional assessment, orthotists for deformity management, and durable medical equipment specialists to customize standard wheelchair components are valuable.
Underweight and Overweight Overweight Most children with CP who are overweight or obese have low muscle tone. Their nutritional status impacts sleeping and breathing patterns, mobility, physical care, and peer
Cerebral Palsy: Nutritional Aspects Chin tuck with neck elongation
Arms free and forward for function
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Most people can be treated nonsurgically with gastric aspiration and nasojejunal or gastrojejunal feedings distal to the obstruction. One study also found that turning to the left from a supine position displaces the SMA from the right to the left side of the aorta in scoliosis cases. Thus, positioning can help alleviate symptoms and special considerations may be indicated in light of the limitations imposed by the CP.
Behaviors at Mealtimes Parent–child interactions can also influence feedings. Ineffective communication, lack of bonding, the absence of social interaction or poor interactive skills, family dysfunction, and decreased environmental stimuli can exacerbate feeding difficulties or lead to frustration and anxiety with subsequent food refusal or parental withdrawal. Aversion to oral feeds can also be an outcome of medical complications, such as esophagitis and GER, or lack of feeding experience at critical milestones secondary to prolonged tube feedings. Behavioral treatment should only be undertaken after thorough medical, nutritional, and neurodevelopmental assessments are completed.
Feeding Issues
Erect spine over pelvis perpendicular to support surface Figure 7 Proper seating position.
relationships. It is difficult to attain an IBW because energy needs are significantly reduced and the options for exercise are limited.
Underweight Typically, children with athetosis struggle to maintain weight given their excessive involuntary movements, which significantly increase energy needs. As these children age, the problem becomes more apparent, and many of these children will require enteral supplementation. One evaluation of this population noted that the basal energy requirement was 40% higher than expected.
Superior Mesenteric Artery Syndrome Superior mesenteric artery (SMA) syndrome is a condition in which the third portion of the duodenum is intermittently compressed by the overlying SMA, resulting in gastrointestinal obstruction. Symptoms include recurrent vomiting, abdominal distension, weight loss, and postprandial distress. People with CP are at high risk for several of the reported causes of SMA syndrome, including body cast compression, severe weight loss, prolonged supine positioning, and scoliosis surgery. Consequently, it is important to recognize the symptoms and know the appropriate treatments for this syndrome.
The feeding plan should be safe, promote growth or weight maintenance without excessive energy expenditure to obtain the required calories, and meet the needs of the family. It should reflect their resources in time and skill, and it should address their concerns and expectations. The goals for treatment once feeding and swallowing problems are identified are: to prevent aspiration and thereby respiratory compromise; to provide adequate calories, protein, vitamins, minerals, and fluid; and to educate caregivers regarding nutritional requirements.
Oral Motor Considerations Management strategies for daily mealtime feeding include positioning, modification of the sensory properties of the food, oral motor facilitation techniques, and equipment adaptations. For individuals with increased energy needs, the nutrient density of their meals may need to be maximized. Table 6 lists commonly used calorie boosters. It is important to acknowledge the inability to change the underlying feeding problem while providing a method of circumventing the problem to allow adequate nutrition and growth. For example, facilitative techniques to minimize excessive jaw movement may entail the feeder providing physical jaw control/support; a change in the food consistency, texture, temperature, or taste to improve the ability to propel a bolus through the oropharynx; the careful selection of adaptive feeding equipment to assist with self-feeding or increased intake; and an appropriate seating system. Proper positioning also allows the feeder to use both hands.
Alternative Feeding Routes Many children with CP are not able to meet some or all of their calorie needs by mouth due to one or more of the
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Table 6
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Calorie boosters
Instant breakfast Powdered, evaporated milk Whole milk cheeses Peanut butter Wheat germ Yogurt, pudding, custards Milkshakes, eggnog Commercial supplements
Table 7 Margarine, butter, oils, gravy Sugar, honey, syrup Cream cheese Sour cream Concentrate juices Breading or cracker meal Fruit canned in heavy syrup
following conditions: oral motor dysfunction, excessive energy needs, recurrent infections, illnesses, and orthopedic surgical interventions. Consequently, if the gastrointestinal tract is functioning, supplemental or total tube feedings may be indicated. Early intervention with enteral nutrition may prevent protein–energy malnutrition and its complications. Studies have shown improvements in weight gain (fat mass as opposed to fat-free mass) with supplemental tube feedings, which better enables individuals to endure short-term medical insults. Enteral nutrition may be delivered by nasogastric, nasojejunal, gastrostomy, gastrostomy–jejunal, and jejunostomy tubes. The degree of GER and risk of aspiration determine where the tube is placed, whereas the length of time needed for tube feedings determines whether a nasoenteral or surgically placed tube is required. The decision regarding continuous, intermittent, or combination tube feeds is dependent on the individual needs of the patient. Tube feedings should be considered a tool to improve nutritional status rather than failure of the child’s ability to eat. Based on the medical diagnosis and developmental stage of the child, the prognosis for return to oral feeding varies, and the length of time to achieve this goal is extremely variable. For some children, the goal of returning to full or partial oral feeding is not realistic. In a study evaluating the health of children with CP, Liptak describes those who were tube fed as having the lowest mental age, requiring the most health-care resources, using the most medications, and having the most respiratory problems. These children were characterized as especially frail and required numerous health-related resources and treatments. Oral motor therapy should focus on maintaining existing oral motor skills, encouraging pleasurable oral experiences, and tolerance of oral hygiene practices. Nonnutritive oral stimulation must be performed when tube feedings are employed as the route of nutrition. The benefits of nonnutritive oral stimulation are listed in Table 7. Improvement in nutritional status can result in positive changes in oral feeding. Parenteral nutrition should only be used when the gastrointestinal tract is dysfunctional. When initiating feedings in patients with major weight loss or failure to thrive, whether enteral or parenteral nutrition is used, it is important to be aware of the ‘refeeding syndrome.’ This syndrome refers to phosphorus depletion and alterations in potassium, magnesium, and glucose metabolism, resulting in severe metabolic and physiological complications. It is imperative to increase calorie delivery slowly with close laboratory monitoring.
Benefits of nonnutritive oral stimulation
Maintains oral sensation and tolerance Facilitates saliva production, swallowing, and other oral motor patterns Maintains or develops coordination of respiration and swallowing Facilitates parent–child interactions
Medications Drug–nutrient interactions should be considered for all children receiving long-term medications for seizure disorders, alterations in muscle tone, attentional deficits, gastrointestinal disorders, and other chronic conditions. One drug or the combination of multiple drugs may affect nutrition in many ways, such as causing decreased appetite, interference with absorption of specific nutrients, nausea, and vomiting. Medication treatment options offer challenges to nutrition. For instance, diazepam, often used to decrease spasticity, increases the potential for drooling. This raises concerns of fluid loss/balance as well as loss of the protective effect of saliva on esophageal mucosa. Additionally, attention must be paid to tone reduction in the trunk and oral structures that would compromise safety of feeding skills. Tone-lowering drugs potentially reduce energy expenditure and, as a result, require increased vigilance to avert excessive weight gain.
Repeated Orthopedic Surgeries These are common in children with CP, and each surgery must be preceded by an evaluation of nutritional status and assessment of the child’s ability to physically heal and recover quickly from the trauma. Many children who are marginal oral feeders will decompensate, lose weight, and have a difficult time healing because of a cascade of events including pain, poor positioning for safe feeding, worsening constipation, minimal intake, lethargy, and increased medications for pain that may have a sedative effect. They may require supplemental feedings before surgery or during the postoperative period.
Coordinated Services The provision of nutrition services and prevention of further disabling conditions can be done in a variety of health care, school, vocational, home, and community settings. Participation in physical fitness activities targeting muscle strength and cardiorespiratory fitness should be encouraged to prevent secondary conditions such as chronic pain, fatigue, and osteoporosis. It is the responsibility of the family in concert with the health-care team to promote nutrition care planning in these settings. More than 90% of children with CP live to adulthood; however, their life expectancy is less than that of the general population. Similar to the general population, individuals with CP are susceptible to developing obesity, hypertension, diabetes, and heart disease and, therefore, require awareness and adherence to prudent dietary guidelines. The chronicity of nutrition problems for
Cerebral Palsy: Nutritional Aspects
individuals with CP is recognized and has in part created a need for care coordination and integrated service planning to provide meaningful and cost-effective services.
See also: Energy Expenditure: Doubly Labeled Water; Indirect Calorimetry. Nutritional Support: Adults, Enteral
Further Reading Bell KL, Boyd RN, Tweedy SM, Weir KA, Stevenson RD, and Davies PSW (2010) A prospective, longitudinal study of growth, nutrition and sedentary behaviour in young children with cerebral palsy. BMC Public Health 10: 179. Brooks J, Day S, Shavelle R, and Strauss D (2011) Low weight, morbidity, and mortality in children with cerebral palsy: New clinical growth charts. Pediatrics 128(2): e299–e307. Case-Smith J (ed.) (1993) Pediatric Occupational Therapy and Early Intervention. Stoneham, UK: Butterworth-Heinemann. Cherney L (1994) Clinical Management of Dysphagia in Adults and Children. Gaithersburg, MD: Aspen. Day SM, Strauss DJ, Vachon PJ, Rosenbloom L, Shavelle RM, and Wu YW (2007) Growth patterns in a population of children and adolescents with cerebral palsy. Developmental Medicine and Child Neurology 49: 167–171.
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Eicher PS and Batshaw ML (1993) Cerebral palsy. Pediatric Clinics of North America 40: 537–551. Ekvall SW (ed.) (1993) Pediatric Nutrition in Chronic Diseases and Developmental Disorders: Prevention, Assessment and Treatment. New York: Oxford University Press. Henderson CR, Lark KR, Gurka JM, et al. (2002) Bone density and metabolism in children and adolescents with moderate to severe cerebral palsy. Pediatrics 110: 1. Hogan SE (1999) Knee height as a predictor of recumbent length for individuals with mobility-impaired cerebral palsy. Journal of the American College of Nutrition 18(2): 201–205. Klein M and Delaney T (1994) Feeding and Nutrition for the Child with Special Needs. Tucson, AZ: Therapy Skill Builders. Krick J, Murphy-Miller P, Zeger S, and Wright E (1996) Pattern of growth in children with cerebral palsy. Journal of the American Dietetic Association 96: 680–685. Liptak GS, O’Donnell M, Conaway M, et al. (2001) Healthstatus of children with moderate to severe CP. Developmental Medicine and Child Neurology 43: 364–370. Samson-Fang LJ and Stevenson RD (1998) Linear growth velocity in children with cerebral palsy. Developmental Medicine and Child Neurology 40(10): 689–692. Samson-Fang LJ and Stevenson RD (2000) Identification of malnutrition in children with cerebral palsy: Poor performance of weight-for-height centiles. Developmental Medicine and Child Neurology 42(3): 162–168. Stallings VA, Charney EB, Davies JC, and Cronk CE (1993) Nutrition related growth failure in children with quadriplegic cerebral palsy. Developmental Medicine and Child Neurology 35: 126–138. Stevenson RD (1995) Use of segmental measures to estimate stature in children with cerebral palsy. Archives of Pediatrics & Adolescent Medicine 149(6): 658–662.