How Cystic Fibrosis Is Treated

Cystic fibrosis (CF) treatment typically involves procedures and medications that help clear the airways. Other treatments may include antibiotics, pancreatic enzymes, a high-calorie diet, and newer-generation drugs known as CFTR modulators. Severe cases may require a lung transplant.

There is no cure for cystic fibrosis (CF), but advances in treatment have extended both the life expectancy and quality of life of people living with the disease.

Self-Care and Lifestyle

CF treatment guidelines generally focus on self-care. This involves airway clearance techniques to remove mucus from the lungs, exercise to maintain lung capacity and strength, and dietary interventions to improve the absorption of fats and nutrients.

Airway Clearance Techniques

Airway clearance techniques (ACTs) commonly used by people with chronic obstructive pulmonary disease (COPD) are just as effective in treating CF lung disease.

These techniques aim to dislodge mucus from the lung's air sacs so you can cough it out. They may be performed several times daily depending on the severity of your condition. Some of these include the following:

• Huff coughing involves taking deep, controlled breaths so that enough air can get behind the mucus in your lungs to dislodge it. As a result, you don't need to exert as much energy to expel it. Inhale deeply, hold your breath, and exhale forcefully to dislodge the mucus.
• Chest percussion, also known as postural percussion and drainage, is performed with a partner who rhythmically claps your back and chest with cupped hands as you alter positions. Once the mucus is loosened, you can expel it with huff coughing.
• Chest wall oscillation works similarly to postural drainage but employs a handheld, non-electrical device that vibrates and loosens mucus. Some of the devices can be connected to a nebulizer to combine oscillation with the delivery of inhaled medications.
• High-frequency chest oscillation involves an inflatable vest attached to an air pulse generator. The machine mechanically vibrates the chest at high frequencies to loosen and release mucus.

Exercise

You can't avoid exercise if you have CF. Exercise helps maintain lung function and reduces your risk of CF-related complications such as diabetes, heart disease, and osteoporosis.

Exercise programs need to be individualized based on your age and health status, and ideally designed with your physical therapist or medical care team. Fitness testing may be performed in advance to establish your baseline level of training.

Fitness plans should include stretching exercises (to promote flexibility), aerobic training (to improve endurance and cardiorespiratory health), and resistance training (to build strength and muscle mass).

When first starting out, consider aiming for five- to 10-minute sessions, performed three or more days per week, and gradually build to 20- to 30-minute sessions.

Diet

Cystic fibrosis affects digestion by clogging the ducts in the pancreas that produce digestive enzymes. 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.

To compensate for this loss and maintain a healthy weight, you need to eat a high-fat, high-calorie diet to give you the energy reserves to better fight infection and stay healthy.

A healthcare provider will determine what you or your child’s weight should be. A specialist dietitian can help design a diet with the right balance of protein, carbohydrates, and fats based on your weight values, age, fitness level, and overall health.

The Cystic Fibrosis Foundation recommends the following daily caloric intake for females, males, toddlers, children, and teenagers, depending on weight goals:

Enzymes and Other Supplements

Cystic fibrosis is associated with chronic inflammation due to increased stress placed on the lungs and pancreas by the accumulated mucus. The inflammation causes as much damage to the lungs as recurrent infection and can also lead to impairment of the pancreas, kidneys, liver, and other vital organs.

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.

While available over the counter, they need to be dose-adjusted by your healthcare provider based on your weight and condition. Pancreatic enzymes can also be prescribed for children, when appropriate.

Cystic Fibrosis Healthcare Provider Discussion Guide

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Prescriptions

Prescription drug therapies are used to manage symptoms and slow the decline of organ damage. Depending on the medication, these drugs may be delivered either orally, by injection, intravenously (into a blood vein), or inhaled.

Bronchodilators

Bronchodilators are drugs that relax constricted airway passages and allow more air into the lungs. They are most commonly delivered with an MDI, which includes an aerosolized canister and a mouthpiece called a spacer. Drugs options include albuterol and Xopenex (levalbuterol).

Bronchodilators are inhaled 15 to 30 minutes before starting airway clearance. They not only increase the amount of mucus you can cough up, they help you inhale other medications, such as mucolytics and antibiotics, deeper into the lungs.

Mucolytics

Mucolytics, also known as mucus thinners, are inhaled medications that thin the mucus in your lungs so that you can cough them up more easily. There are two types commonly used in CF therapy:

• Hypertonic saline, a sterile salt solution, can be inhaled with a nebulizer after you’ve taken a bronchodilator. The salt content draws water from surrounding tissue and, by doing so, thins the mucus in the lungs.
• Pulmozyme (dornase alfa) is a purified enzyme that both thins accumulated mucus and increases the viscosity (slipperiness) in the lungs.

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.

Antibiotics can be used to treat acute CF symptoms (called exacerbation) or prescribed prophylactically to prevent infections from occurring. They are delivered either orally or with a nebulizer or DPI. Serious infections may require intravenous treatment.

CFTR Modifiers

The cystic fibrosis transmembrane receptor (CTFR) gene produces the CFTR protein, which regulates the movement of water and salt in and out of cells. If the CTFR gene is mutated, as is the case with this disease, the protein it produces will be flawed and cause mucus to thicken abnormally throughout the body.

In recent years, scientists have developed drugs called CFTR modulators that can improve CFTR function in people with specific mutations.

Some of the CFTR modulators approved for use by the U.S. Food and Drug Administration (FDA) include the following: 

• Kalydeco (ivacaftor) is a drug that binds to the defective CFTR protein and "holds the gate open" so that water and salt can flow in and out of cells. Kalydeco can be used in adults and children ages 2 and over.
• Orkambi (lumacaftor + ivacaftor) can only be used in people with two copies of the deltaF508 mutation. Having two deltaF508 copies causes the severe deformity of the protein. Orkambi works by correcting the shape of the protein and restoring its intracellular function. Orkambi can be used in adults and children six and over.
• Symdeko (tezacaftor + ivacaftor) is also a corrector drug designed for people with two deltaF508 mutations. It is used in people who cannot tolerate Orkambi. It may also improve CFTR function associated with 26 other common CFTR mutations. Symdeko can be used in adults and children 12 and over.
• Trikafta (elexacaftor/ivacaftor/tezacaftor) is a triple combination therapy available for people with the most common type of CFTR mutation.
• Alyftrek (vanzacaftor/tezacaftor/deutivacaftor) is a newer CF treatment approved for people ages 6 and over who are eligible to be treated with Trikafta or have one of 31 rare mutations that can't be treated with any other available CFTR modulator.

Supportive Therapies

During severe exacerbations or in cases of chronic disease, support measures may be needed to assist with breathing or nutrition. This may involve oxygen therapy and enteral nutrition.

Oxygen Therapy

Oxygen therapy involves the use of a portable oxygen tank with either a mask or nasal prongs to deliver concentrated oxygen to the lungs.

At present, there are no guidelines on the appropriate use of long-term oxygen therapy (LTOT) in people with CF and little evidence as to its benefit one way or the other. 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 experience hypoxemia (low blood oxygen saturation). It is a condition associated with poor sleep quality, reduced exercise tolerance, and the loss of muscle mass.

Given the degenerative nature of cystic fibrosis, LTOT may become necessary if the loss of lung function is causing disability and a low quality of life.

Enteral Nutrition

Enteral feeding (tube feeding) involves the placement or surgical implantation of a feeding tube through which liquid food is delivered. 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. 

Tube feeding is generally considered if you are losing weight despite eating a high-calorie diet, are unable to tolerate food, or are trying to gain weight prior to a lung transplant. 

Enteral feeding can take several forms. Among them:

• Nasogastric feeding is the least invasive form of enteral feeding in which an NG tube is placed in your nostril, down your throat, and into your stomach.
• Gastrostomy is a more permanent option in which a G-tube is inserted into your stomach through an incision in your belly.
• Jejunostomy is a procedure in which a J-tube is inserted through the abdomen into a part of the small intestine called the jejunum. This is most often used if you cannot tolerate feeding into the stomach.

Lung Transplantation

Transplant is typically only considered if the result of a pulmonary function test, called the forced expiratory volume in one second (FEV1), drops below 40% and your lung functions have decreased to a point where mechanical ventilation is required to perform even basic tasks.

A lung transplant can improve your quality of life and help you live longer. After the transplant, you will no longer have cystic fibrosis symptoms in your lungs, but CF can still affect other areas of your body, and you may have to continue treatments.

Complementary Medicine (CAM)

Complementary therapies may help improve breathing and enhance appetite and nutrition. If you decide to pursue any form of complementary or alternative medicine (CAM), speak with your healthcare provider first to ensure it neither conflicts with your therapy nor causes harm.

CAMs are not regulated in the same way as pharmaceutical drugs or medical devices and, as such, are not usually endorsed as effective means of treatment.

• Buteyko breathing is a breathing technique that involves conscious control of your breathing rate and/or volume with diaphragmatic breathing and nasal breathing. It is thought by some to improve mucus clearance without coughing. While evidence of its benefits is poorly supported, it is not considered harmful and may help reduce stress, anxiety, and sleep problems.
• Ginseng is used in traditional Chinese medicine but doesn't have much research to support it. Older research found that the oral use of a ginseng solution in mice was shown to disrupt the protective biofilm of the Pseudomonas auruginosa bacteria commonly associated with CF lung infections, but the same result cannot be guaranteed in humans. Another older study suggests that ginseng may impede bacterial colonization and support antibiotics in controlling infections.
• Turmeric contains a powerful anti-inflammatory compound called curcumin that works much in the same way as COX inhibitor medications. It is unclear whether it can reduce the inflammatory effects of CF since it is so poorly absorbed in the intestines and unlikely to reach therapeutic levels.
• Medical marijuana, while inappropriate for children and teens, is known to be a powerful appetite stimulant for people with CF or treatment-associated anorexia. However, it is unclear what effect smoking marijuana may have on lungs already heavily damaged by CF. Some research indicates that oral drugs containing marijuana's active ingredient, tetrahydrocannabinol (THC), may help achieve weight gain but improve FEV1 in people with CF.

Summary

There is no cure for cystic fibrosis, but with proper management and treatment, people can live for much longer with the condition. The mainstays of CF treatment include using airway clearance techniques, following a high-calorie diet, and getting plenty of exercise. 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.