Lauren Kutzscher serves as BAPP's Research Coordinator. Please contact Lauren with your questions or for more information about any of these studies.

The registry is a database of Cystic Fibrosis patients around the United States. The registry contains information about the patient including demographics, nutrition and PFT's. This registry allows us to have a centralized location for information about our CF patients. It also allows us to compare our center to those around the nation and compare how our CF patients perform relative to the national averages.

Eligible Candidates: Any individual diagnosed with Cystic Fibrosis

A Multi-Center, Double-Blind, Randomized, Efficacy and Safety Study of Denufosol (INS37217) Inhalation Solution in Patients with Mild to Moderate Cystic Fibrosis Lung Disease.

This study will last for about one year. The patient will either get Denufosol or placebo. A computer will randomly assign the patient to either get study drug or placebo. There are ten clinic visits spread throughout the year as well as some phone contacts.

The investigational drug in this research study, Denufosol Tetrasodium (denufosol), turns on a different salt channel than the one that does not work in CF patients. Investigational drug means that it has not been approved by the Food and Drug Administration (FDA) to be sold to treat your child's condition. Denufosol may let liquid into the airway, unclogging mucus and allowing the cilia to work better. The purpose of this research study is to find out if the drug is safe and effective.

Treatment consists of inhaling study drug/placebo three times a day for fifteen minutes over the duration of the study.

Eligible Candidates:

• Be a diagnosed CF patient 5 years old or older.
• Have a forced expiratory volume at one second (FEV1) = 75% of predicted normal.
• Be able to reproducibly perform spirometry maneuvers.
• Be clinically stable for at least 4 weeks prior to screening with no evidence of acute upper or lower respiratory tract infection or current pulmonary exacerbation.

Compensation: $30 stipend per visit $7.50 Parking $25 for food for visits longer than two hours $50 gas card if you live 50 miles or more from the hospital

This study is attempting to understand what other genes and other factors may be responsible for the difference or similarity of illness between siblings.

The family completes a medical history questionnaire and each CF patient will complete a personal questionnaire. Blood samples are taken from both siblings as well as the parents.

Eligible Candidates:

• Families with 2 or more siblings with CF may join.

The investigators want to find out whether certain DNA mutations affect lung and liver diseases in CF patients. To find out, investigators need to look at the DNA in the blood samples from CF patients.

This is a one-time blood draw requiring 35ml of blood.

Eligible Candidates:

• A CF patient with any combination of the following Two known CFTR Genotype alleles (both CFTR alleles must be identified): DF508, G542X, G551D, R553X, W1282X, N1303K, 3905insT, 1078delT, 621+1G>T, 1717-1G>A, DI507, R560T, S549N, 3659delC, G480C, Any stop mutation (CFTR Genotype ending in "X")
• All 15-28 years old with a consistent baseline FEV1 % predicted value in the upper 20th percentile.

Compensation: $25 Stipend $7.50 Parking

The Summer Research Program at Children's Hospital Oakland Research Institure (CHORI) provides summer research opportunities for undergraduate students, medical students, and health profession students. Our office is happy to participate in this program and we have welcomed our intern, Felicity Harris, to our BAPP team. Based on a structured curriculum, including participation in weekly seminars, research discussions, and a one-day Research Symposium, the CHORI Summer Research Program is designed to provide an unsurpassed opportunity for students to immerse themselves fully in the world of basic and/or clinical research for three months during the summer.

This summer, Felicity will be working on a retrospective research project studying the causes, effects, and treatments of pulmonary hemorrhage cases over the past three decades here at Children's Hospital Oakland. During the study, Felicity will compare the symptoms, labs, treatments used, and outcomes observed for the pulmonary hemorrhage cases. She will then enter the information into a database to be further analyzed.

This is a very important study and Felicity will greatly help to better classify and treat future patients. At the end of the summer, Felicity will present her research to her peers at a symposium, just as a professional researcher would do.