Energy and nutrient intakes of children with spastic quadriplegia

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Energy and nutrient intakes of children with spastic quadriplegia M a r t i n D. Fried, MD, a n d Paul B. P e n c h a r z , MB,ChB, PhD From the Division of Clinical Nutrition and the Departments of Pediatrics and Nutritional Sciences, University of Toronto, Toronto, Ontario, Canada

In the clinical nutrition outpatient clinic at the Hospital for Sick Children, we observed children with spastic quadriplegia who were gaining weight at normal to above normal rates 1 with energy intakes lower than the recommended dietary allowances. 2 These patients were fed exclusively through a gastrostomy tube with a commercial formula that contained a fixed nutrient/energy ratio. Concerns have been raised about the possibility that specific nutrient deficiencies will cause disease in this type of patient 3, 4 (particularly bone disease4, 5). We therefore retrospectively examined the energy and nutrient intakes of a group of 19 normally growing patients with spastic quadriplegia who were attending our clinic, comparing their nutrient intakes to the RDA. 2 METHODS All patients with spastic quadriplegia who were fed exclusively by gastrostomy tube were eligible for selection, provided they had been followed in our clinic for 1 year and were gaining weight during that year, at or above the 50th percentile for age and genderJ Nineteen patients (12 of them female), ranging in age from 3 to 20 years (9 + 4 years), were identified. They were followed in the clinic every 3 months for a period of 1 year. Weight and formula intake in milliliters per day were recorded at each visit. Gastrostomy feeding varied from 775 to 1700 ml/day (1139 _+ 247 ml/day) of a commercial tube-feeding formula (lsosource; Sandoz Co., Whitby, Ontario, Canada). Parents or guardians were advised to give additional water sufficient to ensure that the patients thoroughly soaked their diapers Supported by research grants from the Research Institute, Hospital for Sick Children, and from Nestle, Inc., Canada (Dr. Fried). Submitted for publication May 8, 1991; accepted July 15, 1991. Reprint requests: Paul B. Pencharz, MB, ChB, PhD, Head, Division of Clinical Nutrition, Hospital for Sick Children, 555 University Ave., Toronto, Ontario M5G 1X8, Canada. 9/22/32412

at least three or four times a day. Weight velocity for each patient was calculated during 1 year of gastrostomy feeding and was compared with the standards of Tanner et al.1 The composition of the formula published by the manufacturer was used to calculate individual nutrient intake for each subject. Energy, mineral, trace element, and vitamin intakes were expressed as a percentage of the R D A for age and gender. Energy requirements may also be expressed by weight, age, and gender, as are protein needs. 2 We therefore also expressed protein and energy intakes as a percentage of the RDA per kilogram of body weight. Results were expressed as mean _+ SD. RESULTS Of the 19 patients, 18 had energy intakes that were ----_80% of RDA for age and gender (Table), with the mean of 61% _+ 15% and a range of 43% to 98%. However, the mean RDA

Recommended dietary allowances

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energy intake per kilogram of body weight for the group was 103% _+ 32% of the RDA. Fourteen patients exceeded 80% of the R D A for energy intake per kilogram of body weight. The remaining five patients, whose energy intake per kilogram of body weight was less than 80% of RDA, still achieved an annual weight velocity greater than the 50th percentile.1 All patients exceeded 100% of the RDA for protein (225% _+ 75%; range 119% to 408%). Similarly, all exceeded 100% of the RDA for vitamins A, E, C, and B12, folic acid, and zinc. As shown in the Table, most patients did not achieve 80% of the RDA for vitamin D (62% _+ 14%), calcium (74% _+ 20%), and phosphorus (64% + 17%). For iron, the average percentage of the R D A was 96% __+25% but was only 67% ___ 5% in girls during their adolescent growth spurt. No fluid or electrolyte abnormalities were seen.

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Clinical and laboratory observations

200

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]

The Journal of Pediatrics December 1991

T

m Adult T

~

Formula PediatricFormula

15O <

100 O 13. "O t---

E E

50

8 CE

0

Vitamin D

Calcium Phosphorus

Iron

Figure. Percentage of RDA for selected nutrients in 19 mentally retarded, nonambulatory patients with spastic quadriplegia who were fed through a gastrostomy tube with a standard adult tube-feeding formula (Isosource; Sandoz Co., Whitby, Ontario, Canada). The pediatric tube-feeding formula (Pediasure; Ross Laboratories, Columbus, Ohio was used for comparison.

T a b l e . Number of children with cerebral palsy receiving low intakes of energy and selected nutrients No. of children (n = 19)

Energy (kcal/day) Energy (kcal/day per kg of body weight) Vitamin D Calcium Phosphorus

> 8 0 % RDA

~<80% RDA

1 14

18 5

3 8 3

16 1l 16

DISCUSSION Several investigators have shown that total daily energy expenditure and resting metabolic rate are lower in nonambulatory subjects with motor dysfunction, presumably because both fat-free body mass and activity are decreased. 6, 7 Determining expenditure is one way of approximating the RDA for energy. The main drawback is that even the double labeled-water technique employed by Bandini et al. 6 reflects only the expenditure during a 1- to 2-week period. On the other hand, the relationship between energy intake and growth employed in the present study provides at least a confirmation of energy needs measured for a prolonged period. Our findings are in accord with the reduced total daily expenditures recently reported by Bandini et al. 6 in nonambulatory quadriplegic patients. Because we recognize that energy expenditure relates to lean body mass, 2 we were not surprised to find that most of our patients' energy needs were within 80% of the RDA expressed per kilogram

of body weight. Our results point to a need for further study of the relationship between body composition and energy expenditure in this group of patients. The nutrient intake of our subjects was also a concern because the nutrient/energy ratio in tube-feeding formulas is fixed. In receiving an amount of formula that provided less than 80% of the total daily energy requirements, 2 these subjects received less than 80% of RDA of certain nutrients. In particular, vitamin D, calcium, and phosphorus intakes were low, placing the patients at risk for bone disease. As suggested by Tollman et alJ a lower nutrient profile, coupled with long-term use of anticonvulsant medication, also places this patient population at risk for rickets and osteopenia. Intakes of vitamin D, calcium, phosphorus, and iron would have been improved, mostly to within the requirement range, if the subjects had been given a tube formulation designed to meet the nutritional needs of young children (Figure). We had considered providing vitamin D and mineral supplementation to patients on low-intake regimens, but we realized that this would involve significant additional expense and inconvenience to care givers. As an alternative, we thought that using a formula with a higher nutrient/energy ratio might solve the problem; in most instances, it does. We conclude that the total energy needs of children with spastic quadriplegia who require gastrostomy feeding are reduced and are best considered on a unit-weight basis. These children, who depend entirely on tube feeding to meet their nutritional needs, should be fed a formula designed for young children, in view of the higher nutrient/energy ratios of such formulations. We thank Ruth Clarke for her assistance with this study. This

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article was prepared with the assistance of Medical Publications, Hospital for Sick Children, Toronto, Ontario, Canada. REFERENCES 1. Tanner JM, Whitehouse RH, Takaishi M. Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965. Part I, part II. Arch Dis Child 1966;41:454-71,613-35. 2. Food and Nutrition Board, Commission on Life Sciences. Recommended dietary allowances. 10th ed. Washington, D.C.: National Academy Press, 1989. 3. Berg K. Nutrition of children with reduced physical activity due to cerebral palsy. Nutr Diet 1973;19:12-20.

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4. American Dietetic Association. Infant and child nutrition: concerns regarding the developmentally disabled. J Am Diet Assoc 1981;78:443-52. 5. Tolman KG, Jubiz W, Sanella J J, et al. Osteomalacia associated with anticonvulsant drug therapy in mentally retarded children. Pediatrics 1975;56:45-51. 6. Bandini LG, Schoeller DA, Fukagawa NK, Wykes L J, Dietz WH. Body composition and energy expenditure in adolescents with cerebral palsy or myelodysplasia. Pediatr Res 1991;29: 70-7. 7. Culley W J, Middleton TO. Caloric requirements of mentally retarded children with and without motor dysfunction. J PEDIATR 1969;75:380-4.

Sickle cell-hemoglobin E disease: Clinical findings and implications Kathy S. Rey, PAC, Carol A. Unger, RN, Sreedhar P. Rao, MD, and Scott T. Miller, MD From the Department of Pediatrics, State University of New York, Children's Medical Center at Brooklyn

Double heterozygosity for hemoglobins S and E (lysine substituted for glutamic acid at position 26 of the/3-chain [t326glu~lY~]) is relatively rare. Clinical expression is variable; some patients have no symptoms, and others have sickle cell-related complications. The disorder was first reported in Eti-Turks by Aksoy and Lehmann, 1 and has not been described in Haitians. We recently saw a patient of Haitian origin who was initially reported to have hemoglobin S-/3+-thalassemia on newborn screening and was subsequently found to have hemoglobin SE disease. With the widespread implementation of newborn screening programs for detection of hemoglobinopathies, it is likely that identification of this disorder may increase. The clinical course and hematologic values of our patient and three affected siblings, a review of the literature, and recommendations regarding clinical care of such patients form the basis of this report. CASE REPORT A 5250 gm black male infant was born without complications or postnatal problems. The father was reported to have sickle cell disease and the mother an undetermined type of anemia. Supported in part by contracts (Nos. NO1-HB-97069 and NO1HB-77035) from the National Institutes of Health. Submitted for publication April 29, 1991; accepted July 9, 1991. Reprint requests: Scott Miller, MD, State University of New York Children's Medical Center of Brooklyn, Box 49,450 Clarkson Ave., Brooklyn, NY 11203.

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The New York State Newborn Screening Laboratory, which screens filter-paper blood specimens by cellulose acetate electrophoresis (pH 8.6) and then performs citrate agar electrophoresis (pH 6.0) on presumptively positive specimens, reported the child's hemoglobin electrophoresis to show hemoglobins F, S, and A, consistent with hemoglobin S-13+-thalassemia. Although the correct diagnosis was not made, the screening laboratory's function was fulfilled by identifying the possible presence of a significant hemoglobinopathy that required further investigation. At l month of age the baby was seen in our clinic for confirmatory testing; hemoglobin electrophoresis was reported to show hemoglobins F and S, with "inability to quantitate [hemoglobin] A2." The reason for the inability to quantitate hemoglobin A2 was presumably due to the presence of an abnormal hemoglobin migrating with hemoglobin A2 (we frequently receive such reports for patients with hemoglobin C), but an abnormal hemoglobin was not specifically mentioned in the report. Hemoglobin electrophoresis of the mother's blood specimen, however, revealed hemoglobin A and an abnormal hemoglobin that migrated on cellulose acetate with hemoglobins C and A2, and migrated on citrate agar with hemoglobin A; the sickle cell preparation was negative. Subsequent analysis by Dr. T. H. J. Huisman (Medical College of Georgia, Augusta) with high-performance liquid chromatography demonstrated the abnormality to be hemoglobin E. Three siblings also were found to have hemoglobin SE disease; another had sickle cell trait. Hematologic values and electrophoretic patterns are presented in the Table. No hospitalizations were reported for these four children, and they had no symptoms suggestive of a sickle cell hcmoglobinopathy. None had abnormalities on physical examination. All had borderline anemia with microcytosis and normal reticulocyte