Cystic fibrosis
- Article
- 2021-02-01
Mucus is normally smooth and protects the lining of the airways, digestive tract and other organs and tissues. People with cystic fibrosis make thick, sticky mucus that can build up and lead to blockages, damage, or infections in the affected organs. Inflammation also damages organs such as the lungs and pancreas.
Some people with cystic fibrosis have few or no signs or symptoms, while others experience severe symptoms or life-threatening complications. Symptoms of cystic fibrosis depend on which organs are affected and the severity of the condition. The most serious and common complications of cystic fibrosis are problems with the lungs, also known as lung or breathing problems, which may include serious lung infections. People with cystic fibrosis also often have problems maintaining a good diet, as they have difficulty absorbing the nutrients from food. This is a problem that can slow growth.
Your doctor can recommend treatments to improve lung function and prevent or control complications. Early treatment can improve your quality of life and help you live longer.
Symptoms
Symptoms of cystic fibrosis depend on which organs are affected and the severity of the condition. Most cystic fibrosis patients have noticeable symptoms. Some patients have few or no signs or symptoms, while others experience severe symptoms or life-threatening complications. Symptoms can also change over time. The most common complications of cystic fibrosis affect the lungs and pancreas.
Therapy
While there is no cure for cystic fibrosis yet, advances in treatment are helping people live longer, healthier lives. To better manage your condition, you or your child will work with cystic fibrosis specialists. In newborns with a positive screening result, treatment can begin while the diagnosis is confirmed. Treatment of cystic fibrosis focuses on clearing the airways, drugs to improve the function of the defective CFTR protein and prevent complications, and surgery, if necessary.
Your healthcare team
Your health care team will likely include a cystic fibrosis specialist. This is a doctor who is familiar with the complex nature of cystic fibrosis. Your doctor can work with a medical team specializing in cystic fibrosis, often in large medical centers. The United States has more than 100 CF care centers, with medical teams consisting of:
- Doctors specializing in the lungs, diabetes, and digestive system
- Genetic counselors
- Nurses
- Nutritionists and dietitians
- Pharmacists
- Physiotherapists
- Psychologists
- Respiratory therapists
- Social workers
Airway clearance techniques
Airway clearance techniques help loosen the lung mucus so it can be removed, reducing infections and improving breathing. The techniques include special ways of breathing and coughing, devices used by mouth and therapy vests that use vibrations to loosen mucus, and physical therapy of the chest. These techniques are often used in conjunction with medications such as bronchodilators and mucus thinners.
Medicines
Drugs to treat cystic fibrosis include drugs used to maintain and improve lung function, fight infections, clear mucus and help breathing, and work on the faulty CFTR protein. Your doctor may prescribe some of the following drugs to treat cystic fibrosis:
- Antibiotics to prevent or treat lung infections and improve lung function. Your doctor can prescribe oral, inhaled, or intravenous (IV) antibiotics.
- Anti-inflammatory drugs, such as ibuprofen or corticosteroids, to reduce inflammation. Inflammation causes many of the changes in cystic fibrosis, such as lung disease. Ibuprofen is vooral beneficial for children, but side effects can include kidney and stomach problems. Corticosteroids can cause bone thinning and increased blood sugar and blood pressure.
- Bronchodilators to relax and open the airways. These treatments are performed by inhaling them.
- CFTR modulators that improve the function of the defective CFTR protein. They improve lung function and help prevent lung problems and other complications. Examples include ivacaftor and lumacaftor and a triple combination drug (elexacaftor-tezacaftor-ivacaftor). The combination drug is the first approved treatment that can help up to 90% of people with cystic fibrosis. It is currently approved for use in adults and children over 12 years old.
- Mucus thinners to help clear mucus from your airways. These treatments are performed by inhaling them.
Surgery
Surgery can be an option for people with advanced conditions.
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