How Is Cystic Fibrosis Treated?

A complex medical condition like cystic fibrosis (CF) requires a complex approach to treatment. Medicines, vaccinations, enzymes, and airway clearance techniques all play a part in keeping people with CF as healthy as possible. Lifestyle also matters. Nutrition, exercise, sleep, stress management, and staying organized are all important for successful treatment.^1,2

Organization and dedication to cystic fibrosis treatments

Making time for all of a person’s CF treatments requires organization and dedication. Treatment methods vary by person, but many people need drugs, enzymes, and airway clearance to manage their disease. Caregivers caring for adults or children with rare diseases report that caregiving can take 40 hours per week.^1,3

People with CF must make time for exercise, nutritious meals and snacks, and good sleep every day. They have to work in these activities around school, work, family obligations, and visits to a CF care center.¹

What drugs are used to treat cystic fibrosis?

The medicines needed to manage CF vary greatly from person to person. People with a mild form of CF may need only daily pancreatic enzymes and the occasional antibiotic (to fight off a lung infection). Others may need frequent to daily doses of:^1,4

• Inhaled and oral antibiotics
• Bronchodilators
• Mucus thinners
• Cystic fibrosis transmembrane conductance regulator (CFTR) modulators

More prescriptions may be needed to treat other conditions that often occur with CF. These conditions include acid reflux, CF-related diabetes, and liver disease.⁵

Serious lung complications called exacerbations usually require IV antibiotics plus extra airway clearance, supplemental oxygen, and nutrition.⁶

Pancreatic enzymes for CF

Pancreatic enzyme replacement therapy (PERT) helps people with CF digest their food. This is because most people with CF have a condition known as pancreatic insufficiency.^2,5,7

Enzymes come in capsule form and are taken as soon as the person with CF gets old enough to swallow pills. For babies and children too young to swallow pills, enzyme capsules are opened and sprinkled onto food. Someone with CF may take dozens of pills a day before and during meals to help them absorb nutrition.^5,7

Airway clearance techniques

Airway clearance helps loosen the thick, sticky mucus that tends to clog the lungs of people with CF. The type of airway clearance technique (ACT) used varies with age and preference. Parents must perform ACTs for infants and toddlers. Older children and adults perform their own.^2,7

Usually, older children and adults with CF use a combination of airway clearance techniques. These may include:^5,8

• Coughing/huffing
• Postural drainage and percussion (chest PT)
• High-frequency chest wall oscillation (the vest)
• Positive expiratory pressure therapy (PEP)
• Oscillating positive expiratory pressure
• Active cycle of breathing techniques
• Autogenic drainage

Manual percussion or chest PT is recommended for infants and toddlers. Once children with CF get old enough, airway clearance is usually replaced with a vest.⁸

Complementary treatments for cystic fibrosis

Nutrition

People with CF need extra nutrition to grow as children and maintain a healthy weight as adults. People with CF may need twice the number of calories people without CF need at the same age, height, and weight. But they can’t be empty calories. Because their bodies do not digest food properly, people with CF need extra nutrition to prevent malnutrition and becoming underweight.^4,5

Exercise

Dedication to daily exercise keeps people with CF healthier for longer. Exercise has many benefits:⁵

• Builds lung capacity
• Helps with airway clearance
• Builds strong bones
• Strengthens the heart and breathing muscles

Vaccinations

Vaccinations keep people with CF healthier by helping them avoid illnesses or reducing the severity of illness. Some diseases prevented by vaccinations can be life-threatening in those with CF. Sticking to the vaccines their doctor recommends is important for anyone with CF as well as for those around them.^5,9

The 6-foot rule

People with CF are vulnerable to lung infections carried by other people with CF. So, it is important for people with CF to stay 6 feet away from others with CF from a different household or from anyone who is sick.¹⁰

Stress management

Having CF or caring for someone with it can be stressful. Managing the stress of illness and caregiving can help. Popular and effective ways to manage stress include:³

• Yoga
• Meditation
• Exercise
• Hobbies

Lung transplant

When their lungs are beginning to fail, a person with CF may qualify for a lung transplant. A lung transplant is a difficult, expensive surgery. You have to be sick enough to need a transplant but well enough to recover from the surgery. Among those who do receive a transplant, about half are still alive about 10 years after the surgery.^5,11

Supplements, herbs, and home remedies

People with CF often need extra vitamins to support healthy growth and body functions. Supplements people with CF may need include:¹²

• Fat-soluble vitamins A, D, E, and K
• Salt
• Omega 3 fatty acids
• Fluoride
• Zinc

Extra fiber also may be recommended.¹²

Herbs, home remedies, and other supplements must be used carefully in people with CF. Many nutritional supplements interfere with how well antibiotics and CFTR modulators work. Talk to your doctor about which supplements might be right for you.^12,13