RAPID CORRECTION OF WASTING IN CHILDREN WITH CEREBRAL PALSY

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John Patrick Margaret Boland Deanna Stoski Gordon E. Murray

Despite the serious nutritional problems of children with severe cerebral palsy (cP), very few recent publications have been concerned with their nutritional rehabilitation. Phelps (1 95 1) considered that these children with abnormal movements had higher than normal energy requirements, but in general most studies have not documented very high rates of energy expenditure. Among older and less disabled people with CP, reduced work-load efficiency was the most significant finding (Lundberg 1976). Many studies relied on measurement of dietary intake, but a few used indirect calorimetry (Eddy et al. 1965, Wakoh et al. 1965) or telemetry (Berg 1971).

We have been concerned by the severity of malnutrition which develops in the more severely handicapped children. In the past this has been accepted almost as part of the disability, but we thought that the principles gained from work on primary malnutrition could be applied to cerebral palsy. We modified the methods of treating children with primary protein energy malnutrition (Waterlow et al. 1978) to make them applicable to children with CP. After successful intervention with six children, we designed a random- ised controlled trial of intensive feeding vs. standard feeding for children with wasting and severe CP. We wanted to be

sure that benefit was not produced merely by increased attention.

Children with CP often have deformity and growth abnormality, so wasting cannot be defined in the usual way (reduced weight for height). Consequently there are no appropriate standards for these children relating weight to height, or to age.

Table I shows how abnormal the measurements were if weights were expressed merely in relation to those of normal children of the same age. In theory it should be possible to detect faltering growth by careful plotting of weight, length and head-circumference over time. As an interim measure, however, we used skinfold thickness on the basis that any child with severely deficient subcutaneous fat is by definition short of energy reserves and therefore more vulnerable to inter- current illness, which further reduces energy intake. Although this approach ‘underdiagnoses’ nutritionally determined growth-failure, it does identify one group of undeniably undernourished children.

Patients and method Ten children with severe neurological deficits were selected from those attending a regional child neurology centre, the selection criteria being a skinfold thickness below age-related

standards and failure to gain weight in the previous year. Their weights for age are given in Table I, but it should be noted that these figures are only a crude indication of how serious the over-all growth problem was. Absence of weight- bearing means that statural growth will be reduced, but because of deformities no weight-for-length data could be obtained; in addition, paralysed limbs have much lower muscle-mass to length ratios (i.e. even a normally nourished child would have a low weight for length).

The essential points of the feeding programme are: (1) The initial phase. This aims at re- establishing normal metabolism, without inducing growth. For practical purposes growth is equated with weight gain if there is no evidence of sodium retention. ( 2 ) The second phase. This aims at increasing the energy intake to the maximum tolerated until weight-gain ceases or intolerance of the feed indicates that energy stores are replete. Since the volume tolerated becomes the factor which limits intake, a feed with high energy content is an advantage. Hence enteral feeds like ‘Isocal’ or ‘Ensure’ are more appropriate than infant formulae. For children with severe neurological deficits, maximal rates of ‘catch-up’ growth require energy intakes that can only be met by tube-feeding. The availability of modern soft tubes has made this a more tolerable procedure, and we have found the ‘Entriflex’ tube the most convenient. Continuous pump- assisted feeding was carried out with the ‘Biosearch’ enteral feeding pump. A patient’s intolerance of a previously accepted intake, indicated by vomiting, bloating or no further gain in weight, indicates that energy intake should be returned to maintenance levels and phase three of the programme commenced. It should be noted, however, that infection or metabolic problems will have a similar effect, so these possibilities must be excluded. ( 3 ) Return to normal feeding. One of the main concerns of caregivers is that sustained high-energy feeding suppresses normal eating, and it may be difficult to feed the child normally when tube-feeding is stopped. This fear is justified to some

TABLE I Comparison of initial weights with expected weight for age

Weight (kg) Age Sex Initial Expected % of expected (YW for age weight

2.8 M 10.9 14.2 77 2.8 F 11.1 14.0 79 6 .2 F 13.0 20.0 65 7.2 M 14.3 23.4 61 8.0 M 18.1 25.1 72

10.3 M 21.5 33-0 65 10.9 M 19.4 35.0 55 1 1 . 1 F 17.3 37.5 46 12.5 M 15.0 42.5 35 15.8 F 17.8 55.6 32

extent, so after returning to maintenance levels we withdraw tube-feeding gradually (a 10 to 20 per cent reduction daily is reasonable). The tube can be left in situ while normal feeding is gradually re- established.

Design of randomised controlled trial T o avoid any bias in selection of patients, our standards for entry into the study were skinfolds below the 5th percentile for age and failure to gain weight during the previous year. The patients were then randomised to regimes of either immediate high-energy feeding or control standard feeding. It was explained to parents that if tube-feeding was demon- strated to be valuable the control group also would be fed by tube a few weeks later. This happened within five weeks in all cases. The study was reviewed and passed by the Ethics Committee of the Children’s Hospital of Eastern Ontario. Signed, informed consent was obtained for each patient.

Results Randomisation produced the expected distribution in terms of sex, but not age. However, when the patients in the control group also were tube-fed after a short delay, they also gained weight. Therefore the most appropriate way to compare results was to use each patient as his or her own control, as well as to compare groups.

Total energy intake from formula started at 0.23 to 0.36 MJ ( 5 5 to 87 kcal/kg) per day and reached maximal /

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TABLE I1 Changes in weight

Group ~~

Age Sex Weight (kg) Change (Yrs) lnitiaf Final Kg 'Yo

Immediate high-energy feeding 7.2 10.3 10.9 11.1 15.8

Control

Mean 11 .1 SD 3.1

2.8 2.8 6.2 8.0

12.5 Mean 6.7 SD 4.1

Delayed intervention* 2.8 2.8 6.2 8.0

12.5 Mean 6.7 SD 4.1

M 14.3 18.7 M 21.5 29.2 M 19.4 25.3 F 17.3 21.0 F 17.8 26.0

18.1 24.0 2.7 2.0

M 10.2 10.9 F 11.4 1 1 . 1 F 14.2 13.0 M 18.3 18.1 M 15.1 15.0

13.8 13.6 3.2 3.0

M 10.9 12.9 F 11.1 14.8 F 13.0 15.0 M 18.1 20.0 M 15.0 16.0

13.6 15.7 3.0 2.6

4.4 7.7 5.9 3.7 8.2 6.0

0.7 -0.3 -0.8 -0.2 -0.1 -0.1 0.5

2.0 3.7 2.0 1.9 1.0 2.1 1.0

30.8 35.8 30.4 21.4 46.1 33.1

6.9 -2.6 -5.6 -1.1 -0.6 -0.6

18.3 33.3 15.4 10.5 6.7

13.4

*Control patients subsequently tube-fed.

TABLE I11 Triceps skinfold and mid-arm muscle circumference

Before feeding After feeding* P (N= 10) (N = 6)

Mean SE Mean SE

Triceps skinfold 7.0 0.94 12.8 1 . 5 <0.05

Mid-arm muscle circumference 13.1 0.35 14-9 1-0 <0.05

*Skinfold data inadvertently were not collected for four control-group patients after they had been tube-fed.

Values Of 0.34 to 0.63 MJ (82 to 150 kcal/kg) per day, with up to 10 per cent still derived from spontaneous eating. Weight gain stopped four to five weeks after it started. The changes in weight are given in Table 11, and the changes in mid- arm muscle circumference and triceps skinfold in Table 111.

The changes in weight were highly significant (p < 0.01, Student t test), both when groups were compared and when the patients were used as their own controls. The changes in mid-arm muscle circumference and skinfold before and after feeding suggest that both lean tissue

and fat were increased. However, with such a small sample size it is not the statistical significance which is of prime importance, but rather the biologicallq significant demonstration of increased weight in these children who had failed tc gain weight for some years previously.

Routine biochemistry revealed nc abnormalities other than in iron metab- olism: initial haemoglobin values were 11 lg / l (SD 17), initial serum iron values were 9*1pmol/l (SD 6*4), and total iron- binding capacity was 46*4pmol/l (sc 7.4). These values were at the lower end of the normal range for our laboratorv.

Discussion The extremely low energy reserves of these children result from problems associated with cerebral palsy, and not from neglect. Children with severe CP spill a lot of their food, often in excess of 50 per cent, and their rate of feeding is very slow. Hypothetically, in an institution with a patient to caregiver ratio of 8:1, even if all caretaking time was spent in feeding it would amount to only three hours per day per child. A spillage rate of 50 per cent reduces that to 1% hours of normal feeding. When the slow rate of eating is added to the equation, the time might well be reduced to the equivalent of only 20 minutes normal feeding per day. Hence malnutrition is inevitable, and it was predictable that the orally-fed children in this study would not achieve significant ‘catch-up’ growth. Unlike children with primary mal- nutrition, their hair, skin and mucosal membranes were in good condition, indicating that there were no serious micronutrient deficiencies.

It is clear from the rates of weight gain we obtained that malnutrition is a correctable feature of severe cerebral palsy. Increases of 10 to 46 per cent bodyweight over four to five weeks were achieved with approximately 50 per cent increase in energy intake. The weight increases were welcomed by most parents and caretakers, especially when associated with healing of persistent pressure sores. An additional benefit was the correction of cold and cyanosed extremities. The prolonged circulation time and low cardiac output associated with primary malnutrition have been well documented (Alleyne 1966), but to our knowledge they have not been reported in CP patients. One of the more striking features of our study was the spontaneous rejection of high-energy intake by vomiting, tachy- carida and/or fever when significant energy reserves had been laid down. The physiological basis for this is obscure.

There were comments about improve- ment in the children’s spasticity and affect, but these were subjective and require further investigation. The work of Russell et af. (1983) gives a potential physiological explanation for such observations.

Malnutrition occurring in children with severe CP can be corrected rapidly and effectively. Given the multiplicity of increased risks associated with primary malnutrition (Jackson and Golden 1983)’ it must be basic to their treatment that malnutrition should be corrected. Iron status also should be assessed period- ically. It seems likely that the haema- tological changes are due to a combination of causes-protein energy malnutrition which reduces protein synthesis rates, coupled with blood loss from oesophagitis due to reflux-but this too merits further study. In two children studied so far the placement of soft nasogastric feeding-tubes has been associated with visible oesophageal blood- loss because of pre-existing oesophagitis and gastric-acid reflux, demonstrated radiologically.

The nasogastric tubes and the use of high-energy intake does inhibit normal feeding and there were some difficulties in re-establishing acceptable feeding patterns. Oral stimulation during the period of ‘catch-up’ growth may minimise such problems, but if they persist it is likely that permanent enterostomy will be required to maintain a reasonable nutritional state.

We have followed these children for between one and two years, so the long- term effects are as yet unknown. One child required permanent gastrostomy and corrective surgery for gastric reflux, and his current nutritional status is good. Two died because of respiratory and metabolic problems within two months of the feeding programme. Three of the remainder have slowly gained weight, two have maintained their weight and one has lost weight. This last patient is being considered for a permanent feeding enterostomy. One patient has moved away and is lost to follow-up. It is difficult to evaluate these results, partly because there is no adequate natural history for severe cerebral palsy. It is our impression that death occurs because of recurrent aspiration in the lung, with consequent scarring and stiffening leading to changes in intrathoracic and intra- abdominal pressures and aggravation of gastro-oesophageal reflux, which results in further damage to the lung. Mal-

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nutrition diminishes respiratory muscle strength in other conditions (Mansell et al. 1984), so it may be an important contributing factor.

The next stage in the compassionate care of these children should be more careful documentation of the onset of gastric-acid reflux in order to determine the impact of improved nutritional status and corrective surgery in alleviating this painful complication (Jolley et al. 1985).

National Health Research Development Pro- gramme, Canada, and from Mead-Johnson Ltd., Canada.

A uf hors ’ Appointments *John Patrick, M.B., M.D., M.R.C.P., Associate Professor; Margaret Boland, M.D., F.R.C.P.(C), Medical Research Council (Canada) Research Fellow; Deanna Stoski, R.N., Research Assistant; Departments of Pediatrics and Biochemistry, Ottawa University. Gordon E. Murray, M.A., Ph.D., Research Associate, Department of Biochemistry, Ottawa University.

Accepted for publication 23rd May 1986.

Acknowledgements This work was supported by grants from The Ottawa, Ontario KIH 8M5.

SUMMARY Severe cerebral palsy is often accompanied by wasting. The authors used standard methods for the correction of primary protein energy malnutrition in children with severe cerebral palsy. A randomised controlled trial was used to compare intensive nasogastric tube-feeding with the best oral feeding that could be achieved. Nasogastric feeding led to highly significant increases in weight. The changes in skinfold thickness and mid-arm muscle circumference indicated increases in both lean and fat tissue. This study indicates that wasting associated with cerebral palsy can be quickly corrected, so there is no reason why such patients should be exposed to the increased risks associated with wasting.

*Correspondence to first author at Department of Biochemistry, Ottawa University, 45 1 Smyth Road,

RESUME Correction rapide de la Cachexie chez certains enfants I.M. O.C. Les formes graves d’l.M.0.C. s’accompagnent souvent de cachexie. Les auteurs ont utilist des methodes standard pour corriger la malnutrition Cnergetique et protidique primaire d’enfants I.M.O.C. Un essai contrBle randomis6 a etC effectue pour comparer une alimentation par sonde naso-gastrique avec les meilleures alimentations possibles par voie orale. L’alimentation naso-gastrique a permis un accroissement de poids hautement significatif. Les modifications de l’epaisseur du pli cutant et de la circonference musculaire a mi-bras ont traduit un accroissement portant a la fois sur les tissus nobles et sur les tissus adipeux. Cette etude montre que la cachexie des I.M.O.C. peut Etre rapidement traitie et qu’il n’y a pas de raison de laisser ces. I.M.O.C. exposes aux risques accrus que provoque la cachexie.

ZUSAMMENFASUNG Rasche Hilfe bei Abmagerung bei Kindern mit Cerebralparese Schwere Cerebralparese geht oft mit Abmagerung einher. Die Autoren haben Standardmethoden zum Ausgleich einer primaren Proteinmangelernahrung bei Kindern rnit Cerebralparese angewandt. Es wurde eine randomisierte kontrollierte Untersuchung durchgefuhrt, urn die intensive nasogastrische Sondenernahrung mit der bestmoglichen oralen Ernahrung zu vergleichen. Die Sondenernahrung fiihrte zu einem hochsignifikanten Gewichtsanstieg. Die Veranderung der Hautfaltendicke und des Armmuskelumfanges zeigten eine Zunahme des Muskel- und des Fettgewebes an. Diese Studie belegt, daR eine bei Cerebralparese auftretende Abmagerung schnell behoben werden kann und es besteht kein Grund, diese Patienten dem erhohten Risiko, das mit einer Abmagerung verbunden ist, auszusetzen.

RESUMEN Correccidn rapida del desgaste en niAos con paralisis cerebral La paralisis cerebral se acompafia a menudo de desgaste general. Los autores usaron metodos estandar para la correccion de la desnutricih proteica primaria en nifios con paralisis cerebral. Se utilizo un ensayo realizado al axar para comparar la alimentacion intensiva con sonda naso-gastrica con la mejor alimentacion oral que podia conseguirse. La alimentacion naso-gastrica dio lugar a un aumento de peso altamente significativo. Los cambios en el espesor del plieque cutaneo y en la circunferencia a nivel de la parte media del brazo indicaron un aumento en 10s tejidos grasos y no grasos. Este estudio indica que el desgaste asociado a paralisis cerebral puede ser corregido rapidamente, por lo que no hay razon para que estos pacientes esten expuestos a riesgos intracraneales asociados a1 desgaste general.

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Lundberg, A. (1976) ‘Oxygen consumption in relation to work load in students with cerebral palsy.’ Journal of Applied Physiology, 40,

Mansell, A. L., Andersen, J. C., Muttart, C. R., Ores, C. N., Loeff, D. S., Levy, J. S., Heird, W. C. (1984) ‘Short-term pulmonary effects of total parenteral nutrition in children with cystic fibrosis.’ Journal of Pediatrics, 104, 700-705.

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Waterlow, J. C., Golden, M. M. N., Patrick, J. (1978) ‘Protein-energy malnutrition: treatment.’ I n Dickerson, J. W. T., Lee, H. A. (Eds.) Nutrition in the Clinical Management of Disease. London: Edward Arnold.

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