Nutrition Interventions for Children with Special Health Care Needs

Section 3 - Condition-Specific Nutrition Interventions
Table 21-2 describes general biochemical monitoring guidelines for selected
disorders.
Other considerations in management of metabolic disorders include monitoring
(6,7,8):
• Hydration status: Dehydration in children with metabolic disorders often causes
severe metabolic imbalance. Fluid intake and requirements must be carefully
monitored. Constipation is also of medical significance.
• Illness: The “usual childhood illnesses” often cause the child with a metabolic
disorder to lose metabolic balance and become seriously ill. Frequently, children
require hospitalization and the administration of intravenous fluids to prevent
metabolic “crisis”. During infection or illness that results in catabolism, proteincontaining
formula is often refused. Continued administration of some form of
energy and fluids assists in rehabilitation.
• Feeding: Some children who have neurological difficulties develop oral-motor
problems that interfere with the provision of adequate nourishment. A hyperactive
gag reflex is a frequent problem. Some providers use nasogastric or gastrostomy
tubes as a feeding adjunct to prevent metabolic crisis.
The crucial role of nutrition support cannot be disputed in the treatment of these
disorders. Effective treatment requires the expertise of a team, generally comprised
of a geneticist, registered dietitian (RD), genetic counselor, psychologist, and
neurologist. This team of experts is familiar with the nuances of current treatment
for metabolic disorders and will incorporate new treatment innovations as they are
deemed appropriate. However, the complex nutritional and medical management
of these children cannot occur without the follow-up and support of the community
teams. Communication between the team at the tertiary center, the community
teams, and the family is crucial.
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