Hormonal Influences on the Airways

Summary

This study will include cystic fibrosis patients followed at UTSW or Children[Right Quote]s Medical Center who are greater or equal to 16 years of age. A total of 20 subjects may be consented at UTSW or Children[Right Quote]s Medical Center.

We will follow CF women prospectively to assess lung indices during various ovulatory phases, and on and off hormone modulating treatment.

The core study will follow 1 month of natural ovulatory cycles with a visit in each of the three main phases (menses, follicular, luteal phase). Subsequently, subjects may opt to participate in the sub-study. In the sub-study, following their menses, women will be given Leuprorelin intramuscular injection (Lupron 11.25 mg IM), which lasts 3 months to inhibit Luteinizing hormone (LH) and Follicle Stimulating Hormone (FSH) secretion and ultimately other sex hormones. While hormones are suppressed, subjects will be given oral estrogen (Estrace 2 mg BID) for 2 weeks, estrogen with progesterone (Estrace 2 mg BID and Prometrium 200 mg BID) for 2 weeks, and progesterone (Prometrium 200 mg BID) alone for 2 weeks. If subjects participate in the sub-study they will undergo 8 visits over approximately 90 days and serve as their own controls. At each visit, spirometry, Respiratory and Systemic Symptoms Questionnaire (RSSQ), vitals, a nasal mucocilliary clearance (saccharin) test, and blood and sputum specimens will be collected.

Subjects will be required to stop any hormone contraceptive or oral or inhaled steroid 1 month prior to start of the study. Urine pregnancy tests will be performed at screening and at every visit. All women will receive a urine pregnancy test before beginning the study and at visits 4, 5, 6, 7, and 8.

The estimated time for completion of this project is 4 years. It will take 2-3 years to identify, consent subjects, and to collect pertinent data. It will take one more year to analyze the data and write appropriate manuscripts.

Participant Eligibility

• Female CF patients followed at Children’s Medical Center and U.T. Southwestern.
• Must be greater than or equal to 16 years of age.
• All subjects must understand and sign the informed consent.
• Subjects 16 or 17 years old must have at least one parent sign consent and give assent.
• Subjects must have the ability to read and write in English.
• Confirmation of CF by sweat test or genetic test.
• Stable health status (lack of exacerbation in the prior 2 months).
• History of regular menstrual cycles (26-34 days) for the previous 2 months.