Steven Bachrach, MD
Nemours/A.I. duPont Hospital for Children
Thomas Jefferson University
SUNY-Downstate Medical Center
Pre-Test (detailed explanations at the bottom):
Q1. What is the underlying mechanism of a CP child's nutritional deficiency?
a) Excessive vomiting post prandially
b) Decreased ganglionic motility within the gastrointestinal tract
c) Disordered swallowing mechanisms
d) Apathetic affect
Q2. Which vitamin a CP patient more prone to be deficient in?
a) Vitamin A
b) Vitamin C
c) Vitamin D
d) Vitamin E
Q3. T/F: Children with CP who received a gastrostomy tube for FTT did not gain weight any more rapidly than those with FTT who did not receive a gastrostomy tube.
Q4. If a CP patient has a modified barium swallow test that shows aspiration of all types of foods and liquids, what would be most beneficial for him or her to fulfill daily nutritional requirements?
a) Complete parenteral nutrition
b) Pureed foods
c) Gastrostomy tube
d) Re-feeding via high-output jejunostomy
e) Breast feeding
Q5. Which of the following methods would be most helpful in estimating the amount of calories expended by a CP patient?
a) Clinical observation
b) Indirect calorimetry
c) Fitness assistant
d) Body fat monitor
Q6. Which of the following methods would be most helpful in seeing if a CP patient can safely eat or not?
a) In-transit flow endoscopy
b) Contrast-mediated radiography
d) Video fluoroscopy
Q7. What is a feared complication of oral liquid intake in CP patients who have pseudo-bulbar palsy?
b) Aspiration pneumonia
d) Dental caries
Q8. The most important issue for a CP child with overwhelming infection is matching the infecting organism with the proper antibiotic (T/F)
On completion of this module residents and physicians will be able to:
- Appreciate a causal relationship between cerebral palsy and nutritional deficiency
- Assess the nutritional status of a child with CP
- Similarly assess the special nutritional needs of a child with cerebral palsy
- Respond to the difficulties in meetings these needs
- Use unique diagnostic tools for assessing CP patients
- Develop a team-based, multifaceted treatment strategy for a child with CP.
- Material for this presentation was obtained from Miller F, Bachrach SJ. (2006) Cerebral Palsy-A Complete Guide for Caregiving, Johns Hopkins University Press, 2nd Edition.
This resource will provide the basic knowledge, as well as the diversity of issues involved in caring for a child with Cerebral Palsy.
- Fung EB, Samson-Fang L, Stallings, VA, et al. Feeding dysfunction is associated with poor growth and health status in children with cerebral palsy, Journal of the American Dietetic Association, 2002, 102: 361-368. and
- Evers S, Munoz MA, et. al. Nutritional Rehabilitation of Developmentally disabled residents in a long-term care facility. Journal of the American Dietetic Association, 1991, 91:471-473.
Note that these article appear in the literature of Registered Dieticians. RD's are essential partners in the care of children with CP.
Cerebral Palsy (CP) is a collection of motor disorders resulting from damage to the brain that occurs before, during, or after birth. The damage to the child's brain affects the motor system, and as a result the child has poor coordination, poor balance, or abnormal movement patterns-or a combination of these characteristics. Because CP is considered a static disorder of the brain, the motor capacity should not progressively worsen over time.
There are two overlapping methods for describing Cerebral Palsy; 1) The types of movement the child makes, and 2) The part of body involved
TEACHING CAPTION: CP can be divided based on "type of movement" (1-4) or "body-part-involvement" (5-7).
Children with CP often have superimposed medical problems, including epilepsy, mental retardation, learning disabilities, nutritional deficiencies, and/or orthopedic disabilities. This disorder affects approximately 5 out of every 2,000 infants born. Though there is still no consensus as to what the etiology of CP is, it quite clear that premature infants that do not cry within the first five minutes after delivery, who are on a ventilator for greater than 4 weeks, or have cerebral hemorrhage are at the highest risk for developing CP.
Causes of the nutritional deficiency can vary-from inadequate intake due to underlying neurological problems to pain from gum disease. There may be total caloric deficiencies, protein-calorie deficiency, or specific Vitamin deficiencies-the most common one being Vitamin D. One of the solutions to this problem may be the placement of a gastrostomy tube, in which studies have proven its effectiveness in increasing nutritional intake by CP patients.
Treatment of CP patients will involve multiple issues, including diet, physical therapy, special educational requirements, and parental education and support-group-therapy as well. It will be vital to acknowledge these issues and treat them accordingly to ensure total and proper care of a CP patient.
Angela B. is a 7-year-old girl, cared for at home by her mother, who was diagnosed with spastic quadriplegic cerebral palsy and mental retardation at 6 months of age. She has a seizure disorder that is well controlled with phenobarbital and phenytoin.
Angela lives at home with her mother, father and 8-year-old sister. She does not walk, and her mother gets her around in a manual wheelchair. As a small child, she was in various early intervention programs. Currently, she is in a special school for disabled children. She goes back and forth to school, but otherwise she is not outside a great deal.
With respect to her diet, she is fed by mouth primarily with pureed foods and liquids. The liquids consist of whole milk and juices while solid foods are the family's regular diet put through a blender, including assorted meat, fish, vegetables and fruits. Angela is fed three meals each day. At each meal, her mother spends approximately 1 to 1-1/2 hours feeding. She is fed while sitting in a wheelchair, but because of her strong tongue thrust and tonic bite reflex it is hard give large quantities of food into her at one sitting. Although there is no history of vomiting or spitting up, much of the food ends up on her bib. Though there is no history of aspiration, because of coughing and choking with attempts to feed liquids, her mother now thickens liquids with pureed foods. While she is generally cheerful, she often has periods of irritability, particularly at times when she has to have a bowel movement. Her mother describes hard stools that she passes once a week and often only with the help of an enema or suppository.
Q1. What elements in this history would suggest that Angela is likely to be undernourished? Can you define cause and effect for malnutrition in CP?
Have the residents break into groups of 2 or 3 and see which group can come up with the most pairs. See if they can match the four listed below. Perhaps they will describe others.
TABLE 2: Cause of Malnutrition and Effect
TEACHING CAPTION: Identifying causes of malnutrition in children with CP require a careful evaluation. Often there is an interaction between cause and effect. For example, the effect of malnutrition (difficulty in swallowing foods) becomes a cause.
The case continues
On physical examination Angela's' length is approximately 102 cm, and her weight is 13 kg. Both are far below the 5th percentile for age. Measures weight for height and head circumference are well below the 5th percentile for age. Her BMI is 12.8. This is also well below the 5th percentile. The use of either multiples of SD below the curve (z-score) or percent of standard (50th %ile) are more effective indicators of FTT. [See FTT and Assessing Nutritional Status modules for standards.] Angela's weight scores are a z-score of - 2. Her weight is 82% of standard.
Q2. What other measures of nutritional status (NS) would be helpful?
A2. One should assess NS using the "Waterlow" criteria. [Hyperlink to module on Nutritional Assessment] Angela is clearly both wasted (decreased BMI %ile) and stunted (decreased height for age %ile). (See Pediatric Nutrition Notes for Growth). The height, however, may be inaccurate because of difficulty in accurate measurement. Length may be impossible to obtain as well if the patient has flexion contractures of the legs. Estimated length can be obtained by measuring forearm length (elbow to fingertips) or lower leg length (knee to ankle) and then using tables which give total body length based on these measurements.
Arm anthropometry would be helpful to see if there is appropriate muscle mass. Nutritional status assessment might continue with serum for parietal proteins, a total lymphocyte count and albumin levels for visceral proteins. Nothing is better, however, than observation. The absence of deltoid delineation on the upper arm and wasting at the zygomatic arch are telltale signs of muscle wasting.
The Case continues
Examination of the mouth shows a tonic bite reflex, which makes it hard to examine the throat. There is a strong gag reflex as well. The gums are hypertrophied and bleed easily. Dental caries are obvious once the mouth is opened sufficiently. Her abdomen is very thin and some stool masses can be palpated in both the right and left lower quadrants. A rectal exam shows a small amount of hard stool in the rectal vault. Extremities are extremely wasted and thin with little muscle mass or subcutaneous tissue. There are contractures of all four extremities and passive range of motion is limited. Neurological exam shows spasticity throughout all four extremities, severe mental retardation with no communication skills and no apparent understanding of speech.
X-rays of the pelvis show that the hips remain located well in the acetabulum. However, the lower extremities appear diffusely osteoporotic with apparent decreased bone density throughout. X-rays of the spine and upper extremities show similar findings.
Baseline laboratory studies were obtained and these show: hemoglobin 9.6, hematocrit 28, MCV 65, MCH 25, platelet count 250,000, normal serum electrolytes, BUN and creatinine, calcium 8.6 mg/dl, phosphorous 3.5, magnesium 1.5, alkaline phosphatase 400, albumin 2.5, total protein 4.5. Her serum-ionized calcium is 5.1 mg
Q3. What in the history, physical and laboratory evaluation of Angela suggest the types and causes of the nutritional deficiencies seen in CP patients?
A3. Once again, let the resident groups brainstorm answers. We provide 5. Others are possible.
The case studies provides data suggesting the following forms of malnutrition:
1. Protein-energy malnutrition without edema (marasmus),
2. Iron deficiency anemia,
3. Vitamin D deficiency,
4. Magnesium and
5. Phosphorus deficiency.
The primary cause of this child's nutritional deficiencies is inadequate intake. While the B. family tried to take good care of Angela, the underlying cause of her inadequate intake is the neurological problem, specifically disordered swallowing mechanisms, a tonic bite reflex and tongue thrusting. These make for tremendous difficulties in giving her child adequate food intake, despite many hours of effort on the part of her mother and other caretakers. It results in deficiencies of all nutrients and has resulted in stunting (height less than 5th percentile) and wasting (weight for height below 5th percentile). These swallowing difficulties -- know as pseudo-bulbar palsy -- also make it especially difficult to take adequate fluids resulting in constipation as well.
Another factor causing her nutritional difficulties is the dental decay and gum disease. Phenytoin can cause gingival hypertrophy, and the tonic bite makes it extremely difficult to brush her teeth and gums adequately.
In addition to the overall deficiency in food and nutrient intake, Angela has little exposure to sunlight, thus eliminating a primary source of vitamin D for most children (see Section Nutrition Notes and module on Calcium and Vitamin D). Phenytoin and phenobarbital, both interfere with vitamin D metabolism by causing the active metabolite of vitamin D (25 (OH) vitamin D) to be further metabolized to an inactive one (24-25(OH)2-Vitamin D) in the kidney rather than being converted to the most active form, calcitriol (1-25(OH)2-Vitamin D). Though the overall demineralization suggested on her x ray is consistent with vitamin D deficiency, this condition is also affected by her immobility: inhibiting bone formation and enhancing bone dissolution. Her serum ionized calcium is normal suggesting that she is maintaining her calcium but at the expense of bony mineralization. The total serum calcium is low because of the low serum albumin to which serum calcium is bound.
Other micronutrients , which are commonly found to be deficient, include phosphorous, magnesium and iron, again on the basis of an inadequate intake. If left untreated, patients such as Angela who are non-ambulatory have a significant risk for low-impact (or non-traumatic) fractures, especially of their lower extremities. (Stevenson, RD, Conaway, M, Barrington, JW et al "Fracture rate in Children with cerebral palsy", Pediatric Rehabilitation (2006), 9(4): 396-403. ).
Historically little attention has been paid to the nutrition of the child with severe handicaps. Many assumed that nutritional disorders were part of the natural history of the various conditions and that the neurological damage prevented adequate growth. More recent studies however have shown that when adequately fed by a gastrostomy tube these children can and do grow and are well nourished (Shapiro, BK, Green, P, Krick, J et al: "Growth of Seeverely Impaired Children: Neurological versus Nutritional Factors", Develop Med Child Neurol (1986), 28:729-733).
The case continues
"Angela, her mother says insistently, "is not growing. She is unable to swallow food. What are you going to do about that?"
Q4. Well what are you going to do?
The resident team focus should now be on estimating caloric need and providing it with augmented systems such as a gastrostomy tube
A4. The energy requirements for children with cerebral palsy are often decreased, though in certain cases they can be increased above the norm. For example, children with athetoid cerebral palsy do a great deal of moving of their arms and legs constantly throughout the day. Estimated energy needs are as high as 6,000 calories per day. On the other hand, patients with spastic cerebral palsy, confined to a wheelchair or bed, have energy needs that are much below those of their non-disabled counterparts (our ref). With respect to Angela B., a healthy 13 kg. child would need 1150 calories/day, but Angela's estimated need was for 20% fewer calories -- only 920 calories/day.
Nevertheless, because of her neurological disorder, her ability to take in all nutrients was severely impaired, and the result was a significant protein calorie malnutrition. The diet as offered may have had sufficient calories and did have a reasonable distribution among fat, protein and carbohydrates. However, she was unable to swallow the food, so that her actual intake was inadequate, as was her fluid intake. A three day diet history should have been reviewed, but this would have shown what was offered to the child rather than what she consumed. It is extremely difficult to estimate how much of food offered to handicapped children is actually swallowed. Less than 50% is a reasonable estimate -- far less than the 920 calories/day needed for growth.
Thus, most of the deficiencies described above resulted from inadequate intake by the child, and they could be corrected by the placement of a gastrostomy tube to bypass the failed swallowing mechanism. Once the gastrostomy tube is placed, a diet that is sufficient in macro and micronutrients as well as fluid and fiber could be offered, either via a commercially available enteral product or a mixture of dietary foods that are pureed and made into a more liquid mixture by the addition of milk or other fluids.
The deficiency in vitamin D is partly caused by dietary deficiency, partly by lack of sunlight exposure and partly by her medications. As noted, phenobarbital and phenytoin interfere with the metabolism of vitamin D to its active form (1,25 dihydroxy vitamin D). More sunlight exposure would help Angela; however, even with adequate vitamin D intake, many children with spastic quadriplegia develop osteomalacia, secondary to their non-weight-bearing status (Stevenson et al). Most children with spastic quadriplegia do not walk, and an adequate treatment to prevent osteoporosis in this situation has not been found. Getting patients into standers, walkers and gait trainers does seem to slow the process down, so that the bone density is not as low, but does not entirely prevent the development of osteoporosis and fractures. Treatment with bisphosphonates has been successfully used in this population to improve bone density (Reference: Henderson, RC, lark, RK, Kecskemethy, HH et al: "Bisphosphonates to Treat Osteopenia in children with quadriplegic cerebral palsy: A randomized, placebo-controlled clinical trial" J Pediatrics (2002), 141: 644-651).
The chronic malnutrition and rickets seen in this case complicate the medical and surgical treatment of children with spastic quadriplegia significantly. They often require bone surgeries including spinal fusion for scoliosis or hip osteotomies for dislocated hips. Osteomalacia will complicate these procedures, making it harder to place metal fixation devices into the bone, which is softer and "takes poorly" (ref). In addition, the protein calorie malnutrition makes for poor wound healing and greater complications postoperatively (ref).
Lastly, much of the irritability seen in this child may be improved by relief of the constipation with adequate fiber and fluid intake and by adequate nutrition, taking away their chronic hunger. They may actually become more social and cheerful and make some developmental gains with the relief of their chronic malnutrition.
Diagnostic evaluation begins with the basic measurements of height (or length) and weight, both of which may be difficult to obtain in this type of patient. A special wheelchair scale can be used, with which we would weigh the empty wheelchair, and then weigh the chair with the patient seated in it. As noted above, knee-heel measures can substitute for full height or length.
Special growth charts for CP patients DO now exist. There are growth charts for boys and girls for each of 5 types of CP, from level 5 (the most severely impaired quadriplegics like the case we are describing) to level 1 (mild diplegia). For most handicapped children, growth must be plotted on regular growth charts with close attention to changes in velocity. (Velocity curves are available from Ross Laboratories.) Skinfold thickness and mid arm circumference should also be measured in the child with nutritional deficiency.
Q5. What physical and laboratory findings might be present?
A5. Physical stigmata of chronic malnutrition include severely reduced muscle mass and subcutaneous tissue, and stunted growth. The physical signs of rickets can include widened epiphyses at the wrists and ankles, a "rachitic rosary" (nodules at the costochondral junction) and bowed legs (see Nutrition Notes and Case IIb), but these are rarely seen in a severely malnourished child who isn't growing and laying down new osteoid.
Radiographs are more helpful, as they will show gross evidence of demineralization. The bones appear gracile and less dense and may show unsuspected fractures. Biochemical evaluation in these children would include calcium, phosphorus and alkaline phosphatase. In vitamin D deficiency, the calcium may be normal or low, but the phosphorus is consistently decreased and the alkaline phosphatase elevated. Vitamin D levels (25 (OH) vitamin D and 1,25 (OH)2-Vitamin D) can be obtained to confirm the deficiency and establish predominant cause.
Figure. - Radiograph of Ricketic Epiphysis
Secondary to the chronic malnutrition due to inadequate intake, many micronutrients are decreased, including phosphorus and calcium, but also including magnesium, zinc and iron. Serum albumin and total protein are decreased, though edema is uncommon unless the patient is hydrated by IV prior to or after surgery. A complete blood count (CBC) will show a microcytic, hypochromic anemia, and total lymphocyte count will be decreased.
Several measures of bone density are available that are more accurate than a simple x ray. The most reliable currently available is the dual energy x ray absorptiometry (DEXA). This gives a measure of total body bone density as well as the density of different regions of the body and can be followed so that any benefits resulting from dietary changes can be measured (ref). Indirect calorimetry can be used to measure the amount of calories being expended by a particular patient and may be especially helpful in patients where energy needs do not follow the usual formulas because of decreased activity. This involves breathing into a hood to measure oxygen consumption and carbon monoxide production at a resting state. From this are derived estimated caloric need and basal metabolic rate.
Another diagnostic issue is whether a patient can eat. Feeding under video fluoroscopy will provide information as to swallowing ability with various foods of different consistencies. Videofluroscopy (also called a modified barium swallow) is done with a speech therapist present (but not a nutritionist) present. It involves a mixing of barium into different items in the patient's diet. While being fed either by their parent or as their parent would at home, the barium liquid or barium food mixture is swallowed and followed by fluoroscopy. The images are recorded on videotape so that it can be reviewed by the speech pathologist, nutritionist and the parents. Often patients with pseudobulbar palsy aspirate some or all of their oral intake resulting in chronic congestion, recurrent wheezing or aspiration pneumonia. A good cough reflex will sometimes protect the patient from pneumonia, but some amount of chronic congestion following feeding is often a hint that aspiration is occurring. If the patient is aspirating all foods and liquids, alternatives to continued oral feeding must be recommended.
The case continues
Angela's mother is getting impatient with you. She tells, yells really, enough discussion of the theory. What are you going to do?
Note that nutritional treatment is one part of team delivered care for handicapped children. Speech therapy and nutritionist support are needed. Other professionals including health educators, social workers and psychologists will be helpful in planning a comprehensive rehabilitation program. Issues of cost for care and feeding must be addressed. The following elements, specific to nutrition, should be considered in planning therapy for handicapped children:
- Caloric needs may be considerably lower than expected in these patients because of their CP.
- Vitamin D needs may be significantly increased because of anti convulsant therapy, and lack of sun exposure.
- Micronutrient needs for iron, magnesium and phosphorus would be similar to any child of the same age and weight.
- Treating the gum disease and dental caries in Angela and similar patients may help improve oral intake by making chewing less painful. Sometimes this is sufficient to improve the quantity of intake and the use of oral, high calorie supplements, may be enough to help restore the patient to a better nutritional state.
- If the speech therapist has found that thickened liquids are needed to avoid aspiration, then pureed foods and thickened liquids are recommended (as opposed to straight liquids). In addition high calorie, high protein, commercially available supplements are recommended as these can be taken by mouth. These also provide adequate minerals and vitamins.
- If these dietary measures are insufficient or if the feeding evaluation has shown chronic aspiration of all foods and liquids, then a gastrostomy tube is recommended. This may be used as a supplement to oral feedings if feeding is still safe but inadequate, or as a substitute for oral feedings if oral feedings are totally unsafe. The caloric needs may be as little as 50-60 calories/kg/day. These can be provided with commercial formulas or pureed diet as suggested by the nutritionist.
- Many of the commercially available products also come with fiber, so that adequate fiber and fluids will help overcome the problem of constipation.
- While these formulas will meet many of the micronutrient needs of patients like Angela B, an increased (pharmacologic) dose of vitamin D is needed because of the anti convulsant use. Instead of 400 units per day of vitamin D, she may need from 1,000-4,000 units per day (with monitoring of her serum and urine calcium to adjust her dosage and avoid hypercalcemia and hypercalciuria).
No one case study can represent the full panoply of nutritional consequences associated with permanent handicapping conditions. We suggest that an integrated program providing nutrition support, as a part of comprehensive care will be more effective than a focus on nutrition in isolation.
This case study illustrates the significant nutritional deficiencies that can result from swallowing difficulties as seen in the patient with spastic quadriplegia (cerebral palsy). While neurological difficulties resulting in pseudobulbar palsy may explain much of the difficulties, attention must be paid to tooth decay and gum disease as contributing factors, making chewing and swallowing difficult. In addition these patients are particularly susceptible to vitamin D deficiency rickets because of the lack of adequate sunlight exposure and the often common use of anticonvulsants, which interfere with vitamin D metabolism.
Handicapped children patients may also suffer from significant osteomalacia which is not treatable with vitamin D and which may cause recurrent fractures. Severe constipation resulting from inadequate fluid and fiber is another consequence that can result and cause much discomfort for the patient. Lastly, remember the importance of engaging team in the care of handicapped children.
Annotated Pre-test Answers
A1. The answer is C. Children with CP commonly have swallowing dysfunction that interferes with adequate nutritional intake. While they are also more likely to vomit post prandially than other children, this is less commonly a cause of malnutrition than the swallowing dysfunction. They do not have abnormal smooth muscle function more often than other children. Apathy may occur if the child is not given the love, affection, and attention needed.
A2. The answer is C. There are three reasons for a CP child to not have sufficient Vitamin D. First, they are rarely exposed to sunlight, and second, they may not be given sufficient Vitamin D fortified dairy products. Third, many children with CP have seizures, and some of the anti-convulsants interfere with Vitamin D metabolism. Thus, a source of adequate Vitamin D must be included in the diet and/or as supplement.
A3. The Answer is False. CP children are at high risk for malnourishment, in part because of swallowing difficulties associated with aspiration, as well as tongue thrust and tonic bite that interferes with getting enough food. In many circumstances, a G-tube is essential.
A4. The answer is C for the reasons given above. It is essential for a CP child to maintain positive nitrogen balance. Though large weight gain is inappropriate, no weight gain is associated with protein energy malnutrition and its consequences.
A5. The answer is B. The energy expenditure of a CP child, especially those with the choreo athetoid type, can be higher than expected. Conversely, a child with spastic quadriplegia who is non-ambulatory will have diminished caloric needs. Observation and body fat monitoring would not gain sufficient information until it was too late.
A6. The answer is D. A video fluoroscopy is the easiest and most effective way of assessing ability to eat. The others are indirect, invasive and/or expensive.
A7. The answer is B. Pseudo-bulbar palsy causes the swallowing problems seen in children with CP, and can lead to aspiration. While foods may also be aspirated, liquids are more commonly the cause of aspiration, and can lead to a chemical pneumonitis, with significant morbidity and potential mortality.
A8. The answer is True. During an "overwhelming" infection one should also assess nutrition, as poor nutrition contributed to the infection, but the most important issue at that moment is giving the right antibiotic. Certainly, nutrition needs to be addressed as well, but not before the correct antibiotics are begun.