Therapies

Clearing the Airways

People with cystic fibrosis do airway clearance techniques (ACT) to loosen and get rid of the mucus from the lungs. Clearing mucus helps to reduce the severity of lung infections and improve lung function.

Some airway clearance techniques require help from family members, friends or therapists. Adults with cystic fibrosis can do many airway clearance techniques themselves.

One basic technique is called “postural drainage and percussion” (PD&P). People with cystic fibrosis sit, stand or lie in a position that will help free up mucus as their chest and back are pounded or clapped. Sometimes a patient will use an inflatable vest that is attached to a machine that vibrates it at high frequency. The vest vibrates the chest to loosen and thin mucus.

Equipment such as electrical and nonelectrical palm percussors – which give repeated vibrations to break up mucus—may be helpful and can be purchased from medical equipment stores. Older children and adults may find percussors useful when performing their own PD&P, but because proper placement and use is essential for a percussor to be effective, a doctor or respiratory therapist should be consulted for training.

Another airway clearance technique is “oscillating positive expiratory pressure” (oscillating PEP), where a person blows all the way out many times through a device. These devices include the FlutterTM, AcapellaTM, CornetTM and Intrapulmonary Percussive Ventilation (IPV). Breathing with these devices vibrates the large and small airways, and this vibration thins, dislodges and moves mucus.

Inhaled Medications

Inhaled drugs are commonly used in cystic fibrosis care because they reach the airways quickly and easily. Inhaled treatments can be given by aerosol—a mist made from liquid medicines. The medicines go into a cup (nebulizer) that is attached to a small air compressor. The compressor blows air through the cup and makes a mist. People with cystic fibrosis breathe the mist in through a mouthpiece or mask for several minutes. Some medicines can also be given as metered dose inhalers (MDI), which deliver one dose of medicine at a time.

There are several kinds of inhaled medications used to treat CF symptoms:

• Mucolytics like Pulmozyme® to thin mucus so people can cough it out easier.

• Antibiotics to treat infections. Inhaled TOBI®  (tobramycin solution for inhalation) is a widely used antibiotic treatment. TOBI can be effective against the most common source of chronic lung infections, a bacterium called Pseudomonas aeruginosa. Cayston (aztreonam for inhalation solution) also is used to improve respiratory symptoms in people with CF who have Pseudomonas aeruginosa.

• Hypertonic saline to draw more water into the airways and make it easier to cough out the mucus.

Some CF patients also use a “bronchodilator,” such as albuterol, to relax the muscles in the airways of the lungs, which in turn enlarges the airways themselves and makes it easier to breathe.

In addition, inhaled corticosteroids can reduce inflammation in the lungs of CF patients. Ongoing research is determining the specific benefits and potential harmful side effects, but these medicines, such as Pulmicort , currently are being recommended for people who have significant shortness of breath and wheezing, or an infection caused by a fungus.

Antibiotics

Antibiotics are used to fight infection-causing bacteria. Infections are common in the lungs of people with cystic fibrosis, so antibiotics are an important part of regular care.

The antibiotic drug, the dosage, and the length of time to take the drug, all vary from person to person. The infection-causing bacteria can become resistant to some drugs.

Antibiotics come in three different forms:

1. Oral antibiotics – liquids, tablets or capsules that must be swallowed.
2. Intravenous (IV) antibiotics – liquid medicine that goes directly into the blood through an IV catheter. An IV may require a hospital stay, but can also be done at home.
3. Inhaled antibiotics – an aerosol or mist that can reach the airways directly.

Other Drugs

Research has led to other respiratory treatments that are now considered “standard therapies” for many people battling cystic fibrosis.

For example, ibuprofen, which is an anti-inflammatory, was found to benefit people with cystic fibrosis. It can slow the rate at which lung function usually declines for some people with CF. However, patients need to be closely monitored by their CF physician if taking this drug for CF.

Azithromycin, a common antibiotic, was shown to have special benefits for some people with cystic fibrosis. A Phase 3 clinical trial found that, in some cases, the drug helped to preserve and improve lung function. The drug also reduced the number of hospital stays for many people who participated in the clinical trials.

Implanted Devices

Implanted devices that allow repeated and long-term access to the bloodstream for frequent or regular administration of drugs as an alternative to shots are called ports and PICCs.

Nutrition

Nutrition needs change with age—especially for people with cystic fibrosis. Children and teens with cystic fibrosis need extra calories to grow and develop. Everyone with CF, no matter their age, needs good nutrition to stay strong against lung infections and other challenges. Dietitians at CF care centers offer specially tailored dietary programs for each stage of life.

The mucus that builds up in people with CF can block tubes, or ducts, in the digestive system. The pancreatic duct is especially vulnerable; when it is blocked, enzymes that help digest food cannot reach the intestines, and the body cannot properly absorb nutrition from food. The resulting malnutrition compromises the body’s immune system and can lead to diarrhea, poor growth and weight loss.

Most people with CF take enzyme replacements before each meal so their food is properly digested. They also may take supplemental vitamins and maintain a diet that maximizes healthy weight gain. Occasionally, patients need to take nutrition through a tube to provide extra calories that help the body grow and stay strong.

Almost half of all people with cystic fibrosis in the United States today are adults, and many face diseases most often associated with the middle-aged or elderly. These diseases include CF-related diabetes and brittle bones (osteoporosis or osteopenia). By working closely with CF care centers, good nutrition can help to prevent or lessen the impact of these and other health problems.

Sources:Cystic Fibrosis Foundation; Cystic Fibrosis Research Inc.; WebMD