Growth Hormone Therapy for Wasting in Cystic Fibrosis

Our hypothesis is that Growth Hormone (GH) will not only target the peripheral tissue to stimulate weight and muscle growth which will maximize nutritional potential and improve overall quality of life. We theorize that this will occur through a multitude of factors: increased appetite, more constructive utilization of caloric intake and decreased catabolic signaling. The first aim will address changes in weight and lean body mass following the institution of GH therapy in adults with Cystic Fibrosis (CF) related wasting. The second aim will measure impact on quality of life of these individuals. Additionally, the third aim will monitor effects of GH therapy on diabetes and insulin sensitivity. Finally, the fourth aim will observe changes in the subjects underlying diagnosis of CF, specifically lung function, muscle strength and inflammatory state.

The following is a more detailed description of the aims listed above:

Specific Aim 1: Measure change in weight in adults with CF related wasting following GH therapy.

1.1) Monitor weight gained or loss from baseline. 1.2) Assess changes in fat free mass from baseline by bioelectrical impedence analysis.

Specific Aim 2: Evaluate overall quality of life (QOL) in adults with CF related wasting treated with GH therapy.

2.1) Perform CF disease-specific and general QOL analysis via CF QOL questionnaires.

2.2) Monitor compliance with therapy via subject report.

Specific Aim 3: Monitor impact of GH therapy in relation to CF related diabetes onset or control.

3.1) Measure impact on insulin sensitivity in non-diabetes subjects 3.2) Observe change in exogenous insulin requirements and glycemic control in subjects with diabetes.

Specific Aim 4: Quantify impact of anabolic therapy on manifestations of underlying diagnosis associated with CF.

4.1) Observe changes in lung function from baseline during GH therapy. 4.2) Determine changes in overall muscle strength via hand grip and six minute walk.

4.3) Evaluate changes in serum markers.

Eligibility Criteria:
Inclusion Criteria:

* Ability to provide written informed consent and comply with study assessments for the full duration of the study.
* Age \> 18 years
* Cystic fibrosis, diagnosed by either sweat chloride or genetic testing
* Less than 92% ideal body weight based on body mass index (BMI) of 22 for women and 23 for men
* Moderate or better pulmonary function (Forced Expiratory Volume (FEV1) \>40% of predicted).
* Agree to use an effective method of birth control to prevent pregnancy during the research study.

Women should not nurse (breast feed) a baby while on this study because Nutropin AQ may enter breast milk and possibly harm the child.

Exclusion Criteria:

* Pregnancy (positive pregnancy test) prior enrollment in the study
* Any other condition that the investigator believes would pose a significant hazard to the subject if the investigational therapy were initiated
* Participation in another simultaneous medical investigation or trial
* Pediatric patients
* Active neoplasm
* History of organ transplantation
* Prader Willi Syndrome who are severely obese or have severe respiratory impairment
* Patients with hepatic impairment resulting in abnormal coagulation studies (\>1.5 times normal reference range)
* Poorly controlled diabetes as determined by a Hemoglobin A1c greater than or equal to 9.0%.
* Individuals with electrocardiogram abnormality or cardiac pacing.