Cystic fibrosis (CF) treatment typically involves procedures and medications that help erase the airways.
Severe cases may require a lung transplant.
Self-Care and Lifestyle
CF treatment guidelines generally focus on self-care.
Verywell / Emily Roberts
They may be performed several times daily depending on the severity of your condition.
Fitness testing may be performed in advance to establish your baseline level of training.
Diet
Cystic fibrosis affects digestion by clogging the ducts in thepancreasthat produce digestive enzymes.
Get our printable guide for your next healthcare provider’s appointment to help you ask the right questions.
Without these enzymes, the intestines are less able to break down and absorb nutrients from food.
Coughing and fighting infection can also take their toll, burning calories and leaving you drained and fatigued.
A healthcare provider will determine what you or your childs weight should be.
To aid in the absorption of nutrients, your healthcare provider may prescribe a pancreatic enzyme supplement.
These come in capsule form and are swallowed whole after a meal or snack.
Pancreatic enzymes can also be prescribed for children, when appropriate.
Your healthcare provider may also recommend vitamin or mineral supplements if blood tests reveal any significant deficiencies.
Get our printable guide for your next healthcare provider’s appointment to help you ask the right questions.
Prescriptions
Prescription drug therapies are used to manage symptoms and slow the decline of organ damage.
Bronchodilators
Bronchodilators are drugs that relax constricted airway passages and allow more air into the lungs.
Drugs options include albuterol and Xopenex (levalbuterol).
Bronchodilators are inhaled 15 to 30 minutes before starting airway clearance.
There are two types commonly used in CF therapy:
Antibiotics
Antibiotics are drugs that kill bacteria.
With cystic fibrosis, the accumulation of mucus in the lungs provides bacteria the perfect breeding ground for infection.
Because of this, recurrent lungs infections are common in people.
The more infections you have, the more damage your lungs will sustain.
They are delivered either orally or with a nebulizer or DPI.
Serious infections may require intravenous treatment.
Cystic fibrosis carriers have a normal life expectancy but may be at increased risk of chronic bronchitis.
This may involve oxygen therapy and enteral nutrition.
Healthcare providers, however, generally agree that oxygen therapy has its place in the short-term treatment of CF.
People with CF who have significant lung damage will invariably begin to experiencehypoxemia(low blood oxygen saturation).
You are taught how to perform the feedings at home, usually, with the same liquid supplements.
It is meant to supplement eating, not replace it.
Enteral feeding can take several forms.
Alung transplantcan improve your quality of life and help you live longer.
If eligible, you will be placed on a national lung transplantwaiting list.
Children on the list are offered lungs on a first-come, first-served basis.
Those with a higher LAS will be given priority.
Complementary Medicine (CAM)
Complementary therapies may help improve breathing and enhance appetite and nutrition.
Why is there no cure for cystic fibrosis?
Pancreatic enzymes and other supplements can help support your nutrition.
Prescription treatments like bronchodilators and newer drugs called CFTR modulators can also help manage the condition.
Some people may need supportive therapies such as oxygen or a feeding tube.
Lung transplant may be considered in some cases.
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Ottawa (ON):Canadian Agency for Drugs and Technologies in Health; 2018.
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