There is no cure for cystic fibrosis, but treatment can ease symptoms and reduce complications. Close monitoring and early, aggressive intervention is recommended. Managing cystic fibrosis is complex, so consider obtaining treatment at a center staffed by doctors and other staff trained in cystic fibrosis. Doctors may work with a multidisciplinary team of doctors and medical professionals trained in cystic fibrosis to evaluate and treat your condition.
The goals of treatment include:
- Preventing and controlling infections that occur in the lungs
- Removing and loosening mucus from the lungs
- Treating and preventing intestinal blockage
- Providing adequate nutrition
The options may include:
- Antibiotics to treat and prevent lung infections
- Anti-inflammatory medications to lessen swelling in the airways in your lungs
- Mucus-thinning drugs to help you cough up the mucus, which can improve lung function
- Inhaled medications called bronchodilators that can help keep your airways open by relaxing the muscles around your bronchial tubes
- Oral pancreatic enzymes to help your digestive tract absorb nutrients
For those with cystic fibrosis who have certain gene mutations, doctors may recommend a newer medication called ivacaftor (Kalydeco). This medication may improve lung function and weight, and reduce the amount of salt in sweat. It has been approved by the Food and Drug Administration for people with cystic fibrosis who are age 6 and older. The dose depends on your weight and age.
Doctors may conduct liver function tests and eye examinations before prescribing ivacaftor and on a regular basis while you're taking it to check for side effects such as liver function abnormalities and cataracts.
For people with a certain gene mutation who are age 12 and older, another drug (Orkambi) is available that combines ivacaftor with a medication called lumacaftor. The combination of these medications may improve lung function and reduce the risk of exacerbations. However, some people may experience side effects such as chest discomfort and shortness of breath soon after starting the medication. Some people may also have high blood pressure while taking the medication. Doctors may monitor you for any side effects.
Chest physical therapy
Loosening the thick mucus in the lungs makes it easier to cough up. Chest physical therapy helps loosen mucus. It is usually done from one to four times a day. A common technique is clapping with cupped hands on the front and back of the chest. Certain breathing techniques also may be used to help loosen the mucus. Your doctor will instruct you about the type of chest physical therapy he or she recommends for you.
Mechanical devices also can help loosen lung mucus. These include a vibrating vest or a tube or mask you breathe into.
Your doctor may recommend a long-term program that may improve your lung function and overall well-being. Pulmonary rehabilitation is usually done on an outpatient basis and may include:
- Physical exercise that may improve your condition
- Breathing techniques that may help loosen mucus and improve breathing
- Nutritional counseling
- Counseling and support
- Education about your condition
Surgical and other procedures
- Nasal polyp removal. Your doctor may recommend surgery to remove nasal polyps that obstruct breathing.
- Oxygen therapy. If your blood oxygen level declines, your doctor may recommend that you breathe pure oxygen to prevent high blood pressure in the lungs (pulmonary hypertension).
- Endoscopy and lavage. Mucus may be suctioned from obstructed airways through an endoscope.
- Feeding tube. Cystic fibrosis interferes with digestion, so you can't absorb nutrients from food very well. Your doctor may suggest temporarily using a feeding tube to deliver extra nutrition while you sleep. This tube may be inserted in your nose and guided to your stomach, or it may be surgically implanted into the abdomen.
- Bowel surgery. If a blockage develops in your bowel, you may need surgery to remove it. Intussusception, where a section of bowel has folded in on itself, also may require surgical repair.
Lung transplant. If you have severe breathing problems, life-threatening lung complications or increasing resistance to antibiotics used to treat lung infections, lung transplantation may be an option. Because bacteria line the airways in diseases that cause permanent widening of the large airways (bronchiectasis), such as cystic fibrosis, both lungs need to be replaced.
Cystic fibrosis does not recur in transplanted lungs. However, other complications associated with cystic fibrosis — such as sinus infections, diabetes, pancreas problems and osteoporosis — can still occur after a lung transplant.