Nutrition Interventions for Children with Special Health Care Needs

Chapter 17 - Nutrition Interventions for Cystic Fibrosis
referred for a sweat chloride by their pediatrician. Infants with a positive sweat
chloride are sent to a CF Clinic.
All patients should be followed by an interdisciplinary team at a Cystic Fibrosis
Center. The clinical practice guidelines for Cystic Fibrosis, published by the Cystic
Fibrosis Foundation, require a coordinated, interdisciplinary approach for the
diagnosis and management of patients with CF who have diverse needs and complex
treatment plans (4). Typically, the treatment involves professionals in the following
areas:
• medicine
• nursing
• nutrition
• physical therapy
• respiratory therapy
• social service
• psychologist
The nutrient needs of a person with CF are often difficult to meet because of both
increased nutrient requirements and decreased food intake. The energy requirement
is increased because of hypermetabolism intrinsic to the genetic defect, increased
losses of nutrients attributable to pancreatic insufficiency and chronic pulmonary
infection, as well as in some instances, sinusitis. Decreased intake is the result of
emesis due to coughing, increased work of breathing, anorexia from both GI and
pulmonary symptoms and psychosocial issues (2,5).
Pancreatic insufficiency is controlled with the use of pancreatic enzymes containing
lipase, protease, and amylase. There are several brands and doses of products
available. Generic enzymes are not approved for patients with CF. Powdered enzymes
are also no longer recommended. Enzymes are administered via capsules with acid
resistant coated microspheres released in the alkaline pH of the duodenum. It is
recommended that the enzymes be taken prior to meals, snacks and enteral feedings
either in the intact capsule form or with the enteric-coated microspheres mixed with
an acidic food (2,6,7). Recommendations from the Cystic Fibrosis Foundation are to
limit the amount of lipase to 2500 IU lipase per kg per feeding or meal.
Because of the increased median age of survival of patients with CF today, secondary
illnesses are more common. These include CF-related diabetes, liver disease, and
osteoporosis, which require specific interventions as well (2). The effects of the
disease on growth are more noticeable. Adequate health care for these patients
requires close attention to their nutritional needs. The goal of nutrition intervention
is to promote normal growth and development and optimal resistance to infection.
192 Nutrition Interventions for Children With Special Health Care Needs