Cystic Fibrosis (CF) is a common genetic disease that affects nearly 30,000 children and adults in the United States. It typically presents in the first year or two of life, and more commonly now during newborn genetic screening. CF occurs when a defect in a single chloride channel called the Chloride Transmembrane Conductance Regulator (CFTR) affects the body’s salt and water balance. As a result, the mucus in the lungs gets dehydrated, causing thick, sticky mucus build up, chronic airway infections and a form of permanent airway damage called bronchiectasis. CF also affects the GI tract, sinuses, liver and reproductive tract. Thanks to aggressive medical care and new cutting-edge treatments, the average life expectancy continues to advance at a rapid pace.
A Comprehensive Team Approach to Care
California Pacific Medical Center’s (CPMC) Adult Cystic Fibrosis Program has been accredited by the Cystic Fibrosis Foundation since 2006 and is now affiliated with Stanford University. We also work closely with CPMC’s Pediatric Cystic Fibrosis Center.
Our experienced and caring multidisciplinary team, including physicians, nurse coordinators, social workers, dieticians and respiratory and physical therapists, focus on providing expert, quality care tailored to the specific needs of people with CF. Treatment often includes airway clearance, inhaled medicines and pancreatic enzyme supplements. But because the type and severity of CF symptoms can differ widely from person to person, we tailor your treatment plans to your unique circumstances.
If you need inpatient care, we are directly connected to the CPMC Pacific Campus. Experienced specialists there offer a broad range of related services, including interventional radiology, psychiatry, cardiothoracic surgery, otolaryngology and endocrinology.
New Therapies
There are now two FDA-approved therapies that aim to correct the basic gene defect in people with specific types of mutations in the CFTR gene. These are exciting breakthroughs, but more work needs to be done so that we can develop therapies for every person with CF. Several other medications are in various stages of development, and it is our hope that in the near future, we can treat 100 percent of CF patients with medications aimed at restoring CFTR function.
The Adult Cystic Fibrosis Program at CPMC is committed to staying at the leading edge of this revolution and bringing you the latest in personalized CF care. We also offer you the chance to participate in research studies of new CF therapies through the CPMC Research Institute.