Nutritional Management

Nutritional counseling and education should occur at the time of diagnosis and regularly thereafter. Infants and children under the age of 2 years presenting with growth failure should be evaluated weekly or every other week until normal weight gain is achieved, then every 2 to 3 months. Patients should be seen at least once a year by a registered dietitian, who can make accurate anthropoTable 21-1. Nutritional Risk Factors in Cystic Fibrosis

Increased resting energy expenditure Chronic cough Pulmonary infections Poor and deteriorating lung function

Possibly a genotype-dependent, energy-requiring cellular defect Increased nutrient losses Pancreatic insufficiency Reduced bile acid and bile salt pool Cough-emesis cycle

Poorly controlled blood sugars in CF-related diabetes mellitus Poor energy intake Fatigue Anorexia

Esophagitis from gastroesophageal reflux Depression metric measurements (including arm anthropometrics), analyze dietary intake, help evaluate adequacy ol pancreatic enzyme therapy, and make dietary recommendations.

Table 21-2. Special Aspects of Nutritional Assessment in Cystic Fibrosis

Medical History Pulmonary

Number of pulmonary exacerbations Change in pulmonary function tests Gastrointestinal

History of gastrointestinal disease, meconium ileus, DIOS, intussuception, or gastrointestinal surgery Symptoms of malabsorption, eg, gas and bloating, frequent, bulky, loose stools, floating, fatty, foul-smelling, frothy stools Abdominal pain, vomiting, gastroesophageal reflux Endocrine

Polyuria, polydipsia, steroid use, history of abnormal blood sugars Liver disease

History of biliary cirrhosis, ascites, or esophageal varices


Weight, height, head circumference, midarm circumference, and triceps skinfold thickness, measured every 3 to 6 months Diet History

Total calorie and protein intake

Percent of total calories from fat and/or grams of fat per meal

Food allergies or intolerances

Appetite changes with illness

Use of nutritional supplements or tube feeding

Types and amounts of vitamin supplements

Use of complementary/alternative medicines

Medications Enzymes

Timing and method of pancreatic supplementation Number of enzymes with meals, snacks and/or tube feeding Units of lipase/kg of body weight per day, or per gram of fat Other medications

Antibiotics, acid blockers (H2 antagonists), steroids, alternative medicines

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