Cystic Fibrosis (CF) is a genetic inherited disease that affects many parts of the body. It causes thick, sticky mucus to build up in the lungs, leading to chronic infections and bronchiectasis (lung scarring with dilated airways) and acute worsening of the disease, which are called exacerbations; it also causes digestive tract problems, usually affecting the pancreas and leading to poor absorption of nutrients or recurrent pancreatitis; diabetes, especially in adult patients; chronic polyps and sinus disease; liver disease in some patients; infertility in men. It is the most common genetic disease in people of European (Caucasian) descent that leads to reduced lifespan.
Multidisciplinary and Specialized Care for Cystic Fibrosis
A multidisciplinary approach to the treatment of disease has been utilized since the early 1960s and has led to dramatic improvements in survival and quality of life in patients with cystic fibrosis. Programs include nurses, respiratory and physical therapists, social workers, nutritionists and pharmacists among other specialists and are accredited by the Cystic Fibrosis Foundation every 5 years.
The Cystic Fibrosis Program
Established in early 1990s, our Cystic Fibrosis Program is accredited by the Cystic Fibrosis Foundation and has one of the largest patient populations in the nation. It collaborates closely with the pediatric program at Children's Hospital of Pennsylvania (CHOP) and other pediatric programs for the transition of patients to adult care. Our program is also active in clinical research and is part of the CF Therapeutic and Development Network and participates in many CFF and NIH funded studies too (with a focus in CF related diabetes, fungal infections in CF and women’s health issues). In addition, we closely collaborate with the Penn Lung Transplant Program to offer the life extending procedure to select patients with advanced disease. We also serve as a second opinion program for patients with complicated CF problems or the ones that require lung transplantation as described above
The program also serves as the coordinating center for the care of patients that require specialists with CF expertise in other areas, including Ear/Nose/Throat, Diabetes, Gastroenterology, Hepatology, specialized Gynecologic, Obstetric and Fertility care.
Our goal is to help patients achieve a full and healthy life, by utilizing all available new treatments that modify CFTR function, prevent and treat respiratory infections, improve nutrition and treat all other complications. We partner with the patients in order to achieve these goals.