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Cystic Fibrosis

About Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease resulting from the amount or function of a cell protein called the cystic fibrosis transmembrane regulator (CFTR). Problems with CFTR alter how the body adjusts salt and water in certain tissues and body fluids. A signature of CF is a high salt concentration in the sweat. Mucus another fluid normally produced in several organ systems becomes thick and sticky. This leads to tissue damage as mucus builds up and interferes with the normal function of those organs. The lungs are primarily affected in most individuals with CF: thick mucus blocks the airways and allows for infection by various types of microbes. Thick mucus also impacts other organ systems including the gastrointestinal tract (pancreas, liver, gallbladder, and intestines), reproductive tract and the upper respiratory tract (sinuses). These result in other complications of CF such as difficulty digesting and absorbing nutrients from food, impaired intestinal motility, increased risk for diabetes, and problems with infertility. While CF is a chronic and usually progressive disease, advances in CF therapies and disease management in recent decades, have improved survival dramatically. Median survival of patients with CF is now in the late 30s, and many patients are living with CF in their 50s and beyond. Sustaining health by following daily self-care regimens and regular visits with an integrated Adult Cystic Fibrosis Care Team has enabled many individuals to grow into adulthood and live full lives.

UCSF Adult Cystic Fibrosis Program

Adults with cystic fibrosis have received care at UCSF for over thirty years. Many cystic fibrosis specialists throughout the State of California were trained by the Pulmonary Division of UCSF Department of Medicine.

The UCSF Adult Cystic Fibrosis Program is accredited by the Cystic Fibrosis Foundation and the State of California Department of Health. The Adult CF Program is led by adult lung specialists in the Division of Pulmonary and Critical Care Medicine (Mary Ellen Kleinhenz, MD, and Yvonne Huang, MD). Individuals who are 18 years of age and older are seen, including many older individuals with CF. A team of experienced providers from nursing, nutrition, social work and respiratory therapy provide the expert care required by adults with CF in the ambulatory and inpatient settings. The Adult CF Care team also works closely with the pediatric CF care team at UCSF to help prepare patients for the transition from pediatric to adult CF Care. This integrated CF care team approach addresses and manages the needs and issues faced by CF patients and their families.

Physicians:

In addition, CF patients who require hospitalization are cared for by adult pulmonologists on the Advanced Lung Disease Service who are experienced in caring for patients with CF.

Clinic Services:

The Adult Cystic Fibrosis Care team sees patients weekly on Wednesday mornings in the Chest Practice (5th Floor) of the UCSF Ambulatory Care Center, 400 Parnassus Avenue. Appointments can be scheduled at
(415) 353-2961.

More Resources on Cystic Firbrosis