The Cystic Fibrosis Center at St. Vincent Hospital is dedicated to providing exceptional care to patients and families with cystic fibrosis (CF). We provide ongoing education for health care professionals and participate in clinical research in cystic fibrosis.
Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus which clogs the lungs and leads to life-threatening lung infections while also obstructing the pancreas and stopping natural enzymes from helping the body break down and absorb food.
Cystic fibrosis is a genetic disease, meaning that children who have cystic fibrosis are born with the disease even though they may not show signs of the disease until they are older. Until there is a cure, the person will have cystic fibrosis for his/her entire life.
- About 1,000 new cases of cystic fibrosis are diagnosed each year
- More than 70% of patients are diagnosed by age two
- More than 45% of the CF patient population is age 18 or older
- The predicted median age of survival for a person with CF is more than 37 years
The onset of symptoms can vary. Some infants show symptoms soon after birth. For example, some babies are born with meconium ileus, a blockage in the intestines. Other children do not develop symptoms until they are much older.
People with cystic fibrosis do not have identical symptoms. Some have more digestive involvement while their lungs seem unaffected. Others have respiratory symptoms and normal digestion.
The severity of symptoms also varies from one person to another. Some children develop lung infections that require hospitalization early in life while others remain healthy for many years.
- Very salty-tasting skin
- Persistent coughing, at times with phlegm
- Frequent lung infections
- Wheezing or shortness of breath
- Poor growth/weight gain in spite of a good appetite
- Frequent, greasy, bulky stools or difficulty in bowel movements