Cystic fibrosis (CF) is the most common lethal genetic disease in the white population. The pulmonary infections and pancreatic insufficiency make CF a medically challenging disease. Although the importance of nutrition in the CF patient is known, approximately 50% of CF patients are in less than the 10th percentile for weight and height as reported by the 1991 CF Foundation Registry of 114 CF Centers in the United States. This paper addresses the nutritional status of 10 pediatric CF patients who underwent double lung transplant at Children's Hospital of Pittsburgh between August 1991 and May 1993. Patients who survived beyond 1 year gained a significant amount of weight sooner after transplant than those who survived less than 1 year. Gastrostomy tube feedings were more effective than oral intake for weight gain after transplant. CF patients with pancreatic insufficiency have more difficulty with adjustment of doses of immunosuppressive agents for reasons that are not clearly understood.
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