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The objective of this study is to investigate the impact of hormones on lung disease in Cystic Fibrosis (CF) patients. Due to improved therapies, CF patients are living longer and healthier lives than they did 20 years ago. However, females have been shown to have a survival disadvantage. The median life expectancy is 33 in women and 37 in men with CF. The hypothesis is that estrogen and/or progesterone negatively impact lung health in CF. Therefore, understanding the impact of sex hormones (including the use of birth control pills) on the disease process is increasingly important. The purpose of this study is to determine if lung function, respiratory symptoms, or various markers of lung health change during different phases of the natural ovulatory cycle in order to understand if estrogen or progesterone hormones are impacting the disease relative to fluctuations in men with stable testosterone levels. The research objectives of this project are to:
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Main study | No Intervention | The main study is an observational study. All women will be followed for one menstrual cycle (or approximately one month) observationally off of any hormone supplementation. They will have 3 study visits corresponding to their menstrual cycle phases (menses, ovulation, and luteal). Women participating in the main study may participate in the optional interventional sub-study. Men participating in this study will be followed for 1 month observationally. They will have 3 study visits that correlate with the female arm of this study. | |
| Loestrin Optional Substudy | Experimental | Women participating in the main study may participate in the optional sub-study. Following a negative urine pregnancy test, women will be started on once daily oral Loestrin (1.5 mg norethindrone + 0.03 mg ethyl estradiol). They will be followed for two months on this agent and have 2 additional study visits. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Loestrin (norethindrone acetate and ethinyl estradiol) | Drug | This is an optional substudy that females participating in the main study can choose to participate in. Loestrin, an oral contraceptive or birth control pill, will be prescribed and taken daily for approximately 2 months. |
| Measure | Description | Time Frame |
|---|---|---|
| Change in percent predicted forced expiratory volume in 1 second (FEV1) | Through Visit 5, if applicable (or approximately 3 months) |
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Inclusion Criteria:
Exclusion Criteria for Main Study:
Exclusion Criteria for Substudy:
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| Name | Affiliation | Role |
|---|---|---|
| Raksha Jain, MD, MSCI | University of Texas Southwestern Medical Center | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Medical Center | Dallas | Texas | 75207 | United States | ||
| UT Southwestern Medical Center |
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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| ID | Term |
|---|---|
| C020416 | norethindrone acetate, ethinyl estradiol, ferrous fumarate drug combination |
| D000077563 | Norethindrone Acetate |
| D004997 | Ethinyl Estradiol |
| ID | Term |
|---|---|
| D009640 | Norethindrone |
| D009652 | Norpregnenes |
| D009650 | Norpregnanes |
| D009654 | Norsteroids |
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|
| Dallas |
| Texas |
| 75390 |
| United States |
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |
| D013256 |
| Steroids |
| D000072473 | Fused-Ring Compounds |
| D011083 | Polycyclic Compounds |
| D009651 | Norpregnatrienes |
| D042782 | Estrogenic Steroids, Alkylated |
| D045166 | Estradiol Congeners |
| D012739 | Gonadal Steroid Hormones |
| D042341 | Gonadal Hormones |
| D006728 | Hormones |
| D006730 | Hormones, Hormone Substitutes, and Hormone Antagonists |