What to know about supportive therapy for cystic fibrosis

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Cystic fibrosis is a genetic condition that affects breathing and digestion. Symptoms result from a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.

The CFTR gene helps make the CFTR protein, which moves water in and out of cells and tissue. When this protein does not work in the typical way, it breaks down too quickly before it can transport water. Consequently, the mucus the body produces becomes very thick and sticky because it only contains a limited amount of water.

Supportive therapies, such as air clearance techniques, help loosen mucus in the lungs so a person can cough it out. Medications, including antibiotics, help prevent lung infections and maintain lung function.

The specific type of supportive therapy required will depend on the person’s symptoms.

According to the Cystic Fibrosis Trust, several airway clearance techniques aim to loosen mucus so a person can breathe better. For example, a physical therapist can show a person how to breathe and cough in a particular way to loosen mucus.

Physical therapy techniques include:

• postural drainage
• manual techniques, including clapping and vibration
• active cycle of breathing techniques, including breath control, thoracic expansion exercises, and the forced exhalation technique
• autogenous drainage
• positive expiratory pressure

Mechanical devices such as vibration vests can also help clear the airways by simulating physical therapy techniques.

According to the Cystic Fibrosis Foundation, a vibration vest inflates and deflates around 20 times per second, putting pressure on the chest. The mechanism is very similar to clapping. Every 5 minutes, a person stops the vest to cough up mucus. Each session lasts 20 to 30 minutes.

Considerations

Physical therapy may not always be possible, especially when a person feels unwell.

If a person’s cough reflex does not improve when using a vibration vest, they may need to purchase additional equipment or a Cough Assist device, which can be costly.

Learn about Medicare coverage for cystic fibrosis.

There are several medications a person with cystic fibrosis takes to manage their symptoms.

Generally, medication aims to:

• maintain and improve lung function
• clear mucus
• improve breathing
• treat infections
• improve CFTR protein function

Options typically include:

• Antibiotics: These can include oral, inhaled, or intravenous forms, all of which aim to prevent and treat infections and improve lung function.
• Anti-inflammatories: These include ibuprofen and corticosteroids. Inflammation is one possible cause of lung disease in people with cystic fibrosis.
• Bronchodilators: When a person inhales this type of medication, their airways relax and open up.
• CFTR modulators: These drugs aim to improve how the CFTR protein works. Different modulators target different CFTR mutations.
• Mucus thinners: A person inhales these medications, which make it easier to clear the airways.

Considerations

CFTR modulators only target specific genetic mutations. This means they are ineffective in people without those mutations.

According to a 2021 study, CFTRs tend to work best in white populations and are least effective in Black and African American populations. The researchers state this is because the mutations CFTR modulators target tend to be more common in white populations.

Read about health inequity.

Treatments that target breathing support aim to increase the amount of oxygen a person inhales. They can also remove carbon dioxide from the body.

These therapies include:

• Oxygen therapy: People receive oxygen therapy through a face mask and tubes placed in their noses.
• Pulmonary rehabilitation: This includes lung and breathing exercises that help with respiration and mucus clearance.
• Ventilator support: A ventilator machine blows moist air into the lungs through a mask or tubes. A healthcare professional sets the rate and amount of air that enters the lungs.
• Extracorporeal membrane oxygenation (ECMO): An ECMO machine pumps a person’s blood through an artificial lung, adding oxygen and removing carbon dioxide.

Considerations

ECMO carries some risks, including:

• infection
• bleeding
• blood clots

People with advanced health conditions alongside cystic fibrosis may need surgery. For example, individuals with severe lung disease and respiratory failure may need a lung transplant.

Additionally, because the symptoms of cystic fibrosis can also affect other parts of the body, people may require a liver transplant for conditions like cirrhosis.

Exercise can help reduce some cystic fibrosis symptoms.

In fact, regular exercise often increases aerobic capacity and can be an airway clearance technique because it helps the body clear mucus.

Cystic fibrosis affects many parts of the digestive system, particularly the pancreas.

For this reason, a person may need to take certain supplements, such as:

• vitamin A
• vitamin D
• vitamin E
• vitamin K

The body cannot effectively absorb these nutrients due to a lack of enzyme production in the pancreas.

Cystic fibrosis requires medical supervision. People with the condition need regular contact with a healthcare team, including respiratory and digestion specialists.

Generally, adults see a doctor every 3 months, but younger people and children may need more frequent supervision.

Usually, regular checkups include:

• education, for example, learning how to take medication and perform other symptom management techniques
• height and weight measurements to calculate body mass index (BMI)
• nutrition advice and meal planning
• respiratory therapy
• physical examinations
• psychological assessment and support for conditions like anxiety and depression
• physical therapy assessments
• colorectal cancer screening

Supportive therapy for cystic fibrosis includes a variety of treatments, ranging from physical therapy to medication. Because there is currently no cure for cystic fibrosis, supportive therapies focus on symptom management and quality of life.

Some cases of the condition may require more treatments than others. For example, a person who can engage in exercise and breathe without difficulty will need fewer supportive treatments than someone who cannot.

People can discuss which supportive therapies may work best for them with a doctor.