OBJECTIVES: I. Determine the effect of growth hormone on height, height velocity, body weight, and lean body mass in patients with cystic fibrosis.

II. Determine the effect of growth hormone on pulmonary function in these patients.

III. Determine the impact of this drug on the quality of life in these patients.

IV. Determine if the clinical response from this drug is sustained in these patients.

PROTOCOL OUTLINE: This is a randomized, multicenter study. Patients are randomized to 1 of 2 treatment arms.

Arm I: Patients receive growth hormone subcutaneously (SC) daily for 1 year during the first year only.

Arm II: Patients receive growth hormone SC daily for 1 year during the second year only.

Quality of life is assessed at baseline and then every 6 months for 2 years.

PROTOCOL ENTRY CRITERIA:

--Disease Characteristics--

• Diagnosis of prepubertal cystic fibrosis
• No colonization by Burkholderia cepacia

--Prior/Concurrent Therapy--

• No prior or concurrent insulin requirement

--Patient Characteristics--

• Hematopoietic: No hematologic disease
• Hepatic: No liver disease
• Renal: No kidney disease
• Pulmonary: Must be able to perform pulmonary function testing
• Other: No history of diabetes Must be less than 25% of normal height and/or weight for age and sex