Cystic fibrosis (CF) is an inherited disorder that affects the respiratory (lungs) and digestive system. It ultimately leads to severe damage of the lungs, causes malnutrition without intervention, and can lead to diabetes and bone disease. It is the most common genetic disorder in Caucasians, affecting about 30,000 patients in the United States and 70,000 worldwide. However, it also occurs in Hispanics, African-Americans and some Native Americans. It is typically diagnosed during childhood. Due to advances in screening and care, more adults are now living better with this disease.
Cystic fibrosis is caused by one of many possible genetic mutations (changes) in the CF protein that makes sweat, mucus, and digestive juices (called bile) abnormally thick and sticky. When these lubricants can’t move through passages in the lungs and pancreas, it leads to life-threatening conditions.
Symptoms of cystic fibrosis vary and can go unnoticed until young adulthood (called adult-onset CF). In addition to a chronic, ongoing, cough that brings up mucus, shortness of breath, and nasal congestion, adult symptoms, and complications of cystic fibrosis may include:
- Arthritis: joint swelling and pain, such as cystic fibrosis-related arthropathy (CFA) and hypertrophic pulmonary osteoarthropathy (HPO)
- Bile duct disease: like biliary cirrhosis (irreversible scarring caused by bile blockage)
- Bronchiectasis: permanently enlarged airways that cause excess mucus in the lungs
- Diabetes: problems regulating insulin, or blood sugar
- Frequent lung infections: like bacterial pneumonia
- Gallstones: stones in the gallbladder
- Infertility: specifically in males
- Malnutrition: lack of vital nutrients due to absorption problems (how the pancreas processes vitamins, minerals, and proteins from food)
- Osteoporosis: bone loss or thinning
- Pancreatitis: inflammation of the pancreas
At the University of Miami Health System, our pulmonologists (lung specialists) help patients with cystic fibrosis live life to the fullest by controlling symptoms and slowing disease progression (worsening). We also help children and teens with CF transition into the adult health care system.
Why Choose UHealth?
Multidisciplinary teams work together for you. Your care team is led by pulmonologists and includes a nurse practitioner, respiratory therapist, dietitian, psychologist, social worker, and physical therapist with special training in CF. We also partner with other specialists in gastroenterology, hepatology (liver care), otolaryngology (ear, nose, and throat), and more. You get the best possible care when we’re all working for you.
Part of the CF Foundation’s Therapeutic Development Network (TDN). TDN is a national research collaborative that conducts and shares groundbreaking CF treatments discovered by specialists in different medical fields. This means you have access to the very latest and greatest clinical trials and CF care right here in South Florida.
One of about 100 accredited adult cystic fibrosis programs in the U.S. We’re recognized by the Cystic Fibrosis Foundation for our high-quality care and support for adult patients with CF. Our comprehensive services — including education, counseling, connection to community resources, and much more — help you stay healthy and strong.
Special transition services for teens moving to adult cystic fibrosis care. Along with the online CF R.I.S.E. program, our transition team helps your high-schooler become independent and responsible for his or her health care. We provide facility tours, care team meet-and-greets, and “life after school” counseling, well before pediatric care ends. You can rest easy knowing your teen has seamless, continuous care for a complex condition. Learn more.
Ongoing Clinical Trials
We currently have multiple ongoing clinical trials which change frequently. You can find the most updated list of the clinical trials we are participating at: