You’ll probably have a healthcare team that includes a specialist in cystic fibrosis and many other types of caregivers. There is no cure for cystic fibrosis, but your team will help you manage the disease. The major focus of management is keeping your airways clear. The best treatment options for cystic fibrosis may include:
Medications
- Antibiotics to prevent and treat lung infections
- Inhaled bronchodilators to make breathing easier by opening and relaxing your airways.
- Mucolytic agents to thin mucus and make them easier to get rid of
- Chest physical therapy to help clear mucus from the lungs
- Pancreatic enzyme replacement therapy to aid digestion
- Stool softeners to help with constipation
- Vitamin and mineral supplements to address nutritional deficiencies
- Gene therapy and/or small molecule drugs to address the underlying genetic cause of the disease.
Airway clearance techniques.
These can help get rid of mucus. You might try:
- Chest therapy or percussion. This involves tapping or clapping on your chest or back to clear mucus from your lungs. Someone else does this for you.
- Oscillating devices. You breathe into a special device that oscillates, or vibrates, your airways. This loosens mucus and makes it easier to cough up. You can wear an oscillating chest vest instead.
Physical therapy
This includes breathing exercises designed to push air between layers of mucus and your chest wall. They make it easier to cough up the gunk and ease blocked airways. Some common exercises include:
- Autogenic drainage. To do this, you breathe out hard, or huff. This moves mucus from your smaller airways to the central airways and makes it easier to get out.
- Active cycle of breathing. This controls your breath and relaxes the upper chest and shoulders, which can help clear mucus and prevent airway blockages. You breathe in deeply, hold it, and then huff for different lengths of time.
High-calorie, high-fat diet
People with cystic fibrosis have nutritional needs that aren’t the same as the needs of people without CF. People with CF may need 1.5 to 2 times the number of calories as people without CF. You need the extra calories if you have CF because you use more energy than other people to breathe, fight lung infections and maintain your strength.
You also need more calories and fat because cystic fibrosis stops the digestive enzymes made by your pancreas from working completely. This means nutrients and fats from foods aren’t fully absorbed by your intestines.
Surgery
You may need surgery for cystic fibrosis or one of its complications. These might include:
- Nasal and sinus surgery
- Oxygen therapy
- Noninvasive ventilation
- Feeding tube
- Bowel surgery to remove blockages.
- Transplant surgery, including a double lung transplant or a liver transplant.
Home care and monitoring
- Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew.
- Giving plenty of fluids, especially to infants and children in hot weather, when there is diarrhea or loose stools, or during extra physical activity.
- Exercising 2 or 3 times each week. Swimming, jogging, and cycling are good options.
- Clearing or bringing up mucus or secretions from the airways. This must be done 1 to 4 times each day. Patients, families, and caregivers must learn about doing chest percussion and postural drainage to help keep the airways clear.
- No contact with other people with CF is recommended as they can exchange infections (does not apply to family members).