Cystic fibrosis is a hereditary disease that causes certain glands to produce abnormal secretions, resulting in tissue and organ damage, especially in the lungs and the digestive tract.
- Cystic fibrosis is caused by inherited genetic mutations that cause thick, sticky secretions to clog the lungs and other organs.
- Typical symptoms include vomiting and abdominal bloating, loose stools, and poor weight gain in newborns, as well as coughing, wheezing, and frequent respiratory tract infections throughout life.
- The diagnosis is based on sweat test results and/or genetic testing.
- Half of the people with this disease live to their early 40s.
- Treatments include antibiotics, bronchodilators, drugs to thin lung secretions, airway clearance treatments for respiratory problems, supplements of pancreatic enzymes and vitamins for digestive problems, and a drug to improve cystic fibrosis protein activity in people with certain mutations.
- Some people benefit from lung transplantation.
Cystic fibrosis is the most common inherited disease leading to a shortened life span among Caucasian people. In the United States, it occurs in about 1 of 3,300 white infants and in 1 of 15,300 african american infants. It is rare in Asians. Because improvements in treatment have extended life expectancy for people with cystic fibrosis, about 50% of people in the United States with this disease are adults. Cystic fibrosis is equally common among boys and girls.
- Routine immunizations
- Antibiotics, inhaled drugs to thin airway secretions, and airway clearance techniques
- Drugs that help prevent airways from narrowing (bronchodilators) and sometimes corticosteroids
- Pancreatic enzyme supplements
- High-calorie diet
- In people with specific mutations, ivacaftor or an ivacaftor/ lumacaftor combination