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How Is Cystic Fibrosis Treated?

A complex medical condition like cystic fibrosis (CF) requires a multi-pronged approach and dedication to treatment. Medications, vaccinations, enzymes, and airway clearance techniques only play one part in keeping someone with CF as healthy as possible. Lifestyle also matters, with nutrition, exercise, sleep habits, stress management, and organizational ability all playing equally important roles.

Organization and dedication with cystic fibrosis treatments

Making time for all of a person’s CF treatments requires organization and dedication. It varies by individual, but the average amount of time an adult with CF spends on their treatments (medications, enzymes, and airway clearance) is 108 minutes per day, regardless of the severity of their disease. Caregivers of children with CF report 74 minutes per day spent on treatment tasks.1

To maintain health, chunks of time also should be set aside daily for exercise, nutritious meals and snacks, and adequate sleep. These activities must be built into a day filled with school, work, hobbies, taking care of family, and at least quarterly visits to the CF care center.1,2

What medications are used to treat cystic fibrosis?

The medications needed to manage cystic fibrosis are wide-ranging and vary dramatically from person to person. Some people with a mild form of the disease may only need the occasional antibiotic to fight off a lung or sinus infection and daily pancreatic enzymes (see below). Those with more advanced cases may need frequent to daily doses of inhaled and oral antibiotics, bronchodilators, mucus thinners (mucolytics), and CFTR modulators.

Additional medications are often needed to treat other conditions that often coincide with CF, such as acid reflux, CF-related diabetes, and liver disease.

Exacerbations, which are a very serious lung complication, usually require IV antibiotics, along with extra sessions of airway clearance and supplemental oxygen and nutrition.

What are pancreatic enzymes?

Pancreatic enzyme replacement therapy (PERT) is necessary to help a person with CF digest their food. This is because most people with CF have a condition known as pancreatic insufficiency. Enzymes come in capsule form as soon as the person with CF gets old enough to swallow pills. Enzyme capsules are opened and sprinkled onto food for babies and young children too small to swallow the pills. Someone with CF may swallow as many as 20 pills a day before and during meals to help them absorb nutrition.

What are 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 by age and which method the person with CF prefers. Parents must perform ACTs for infants and toddlers, while older children and adults perform their own.

Usually, older children and adults with CF use a combination of the following airway clearance techniques:

  • 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 drainage2

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.

The importance of nutrition

People with CF need extra nutrition to grow as children and maintain a healthy weight as adults. By some estimates, people with CF need double the number of calories as a person without CF at the same age, height, and weight. But these can’t just be empty calories. Due to their gastrointestinal system not digesting food properly, a condition called malabsorption, people with CF need extra nutrition to prevent malnutrition and failure to thrive as children.3

The benefits of exercise

Among its many benefits for people with CF, exercise builds lung capacity, helps with airway clearance, builds strong bones, and strengthens the heart and breathing muscles. Dedication to daily exercise keeps people with CF healthier, longer. The amount of exercise a person can handle may change dramatically between exacerbations and other complications, but it’s still important.

Vaccinations

Immunizations keep people with CF healthier by helping them avoid illnesses that can be avoided or reduced in severity. Some of the diseases prevented by vaccinations can be life-threatening in those with CF. Strict adherence to the recommended vaccine schedule, including flu and pneumonia vaccines, is recommended for anyone with cystic fibrosis and those around them.

Maintaining safe distances

People with cystic fibrosis are especially vulnerable to lung infections carried by other people with CF. That is why it’s important for people with CF to keep a distance of six feet from others with CF.

Stress management

Having or caring for someone with cystic fibrosis can be stressful, and depression and anxiety are common among both patients and caregivers. That’s why it’s important to find ways to manage stress. Yoga, meditation, exercise, hanging out with friends, and hobbies are all popular and effective ways to manage stress, anxiety, and depression.

Lung transplant

When the lungs are beginning to fail, a person with CF may qualify for a lung transplant.

Complementary and alternative medicine

Complementary and alternative medicine (CAM) must be used carefully in people with CF. Many nutritional supplements are known to interfere with how antibiotics and CFTR modulators work. However, some home remedies and supplements are known to be helpful in dealing with the side effects of CF treatment. For instance, air purifiers and humidifiers can help a person with CF breathe easier, while ginger or mint tea helps calm nausea.

Written by: Jessica Johns Pool | Last reviewed: September 2019
  1. Sawicki GS, Sellers DE, Robinson WM. High treatment burden in adults with cystic fibrosis: challenges to disease self-management. J Cyst Fibros. 2008;8(2):91–96. doi:10.1016/j.jcf.2008.09.007.
  2. Cystic Fibrosis Foundation. Introduction to Cystic Fibrosis. Available at: https://www.cff.org/Intro-to-CF.pdf. Accessed 5/16/2019.
  3. Dennett C. MNT in Cystic Fibrosis Care — What RDs Need to Know. Today's Dietitian. April 2017. Vol. 19, No. 4, p. 44.