Treatment of Cystic Fibrosis

Currently, there is no cure for CF, but there are treatments for the symptoms of CF, such as lung and digestive problems, hepatic (liver) and biliary tract (gallbladder) diseases, and infertility.

Lung Problems
Lung problems (such as bacterial infections, inflammation and airway blockage) can be treated with pharmaceutical and non-pharmaceutical methods. To treat bacterial infections of the lung, doctors may prescribe antibiotics (such as aminoglycosides - tobramycin [Nebcin or Tobrex]), antipseudomonal penicillins, cephalosporins (such as ciprofloxin [Cipro]), antimicrobial drugs (such as rifabutin - Mycobutin), ethambutol (Myambutol), clarithromycin (Biaxin), clofazimine (Lamprene) and aztreonam (Azactam). Antibiotics may be taken orally, by injection or inhaled (via an aerosol form).

For treating inflammation, doctors may prescribe nonsteroidal anti-inflamatory drugs or steroids, such as ibuprofen (Advil or Motrin) or prednisone. Additionally, pentoxifylline (Trental) has been effective in decreasing inflammation.

For the treatment of airway blockage due to mucus buildup, the doctor may prescribe dornase alfa (Dnase [Pulmozyme]) which is used in an inhalant machine, called a nebulizer.

In conjunction with this drug, doctors advise CF patients to perform bronchial drainage or chest physiotherapy daily. Chest physiotherapy (chest PT) consists of bronchial (or postural drainage), done manually or mechanically.

Manual chest PT can be done in two ways. Autogenic drainage involves using controlled breathing techniques lasting for about 30 to 45 minutes. The other method places the patient in a position that allows drainage of the mucus from the lungs, while at the same time, the chest or back is clapped and vibrated to dislodge the mucus and help it move out of the airways. This process is repeated over different parts of the chest and back to loosen the mucus in different areas of the lung.

There are three airway clearance devices available for use in mechanical chest therapy. The first device is positive expiratory pressure treatment, which uses a facemask or mouthpiece attached to a one-way valve with a set resistance of 5 to 20 cm of water. The second device is the Flutter; another airway clearance device approved for chest therapy. The Flutter is a small handheld device that resembles a covered pipe. The bowl of the pipe contains a steel ball, which during expiration (exhaling), produces oscillations of variable breathing resistance. The third option is a high-frequency chest compression, which uses an inflatable vest driven by a pulsed-air delivery system.

Digestive Problems
The digestive problems in CF can be managed by eating a well-balanced, high-caloric diet that is low in fat and high in protein. Pancreatic enzyme and vitamin A, D, E and K supplements are often prescribed to ensure good nutrition.

Liver & Gallbladder Disease
The treatment of liver disease associated with CF is oral dissolution therapy. Oral dissolution therapy consists of taking ursodeoxycholic acid (Actigall) to dissolve formations in the liver.

The treatment for gallbladder disease associated with CF is laparoscopic cholecystectomy. Laparoscopic cholecystectomy involves removing the gallbladder through a tiny incision in the navel.

Infertility
About 90 percent of all men with CF are infertile. Options for infertile men are currently limited to artificial insemination of the partner with donor sperm or adoption.

Women with CF can become pregnant via natural means or intrauterine insemination with their partner's sperm. However, pregnancy may impose a life-threatening burden on a pregnant woman with CF who has severe lung impairment.

If the patient doesn't respond to any of the treatment methods mentioned, and has severely reduced lung function and declining health, the doctor may recommend lung transplantation, the only definitive treatment for cystic fibrosis. The three-year survival rate following transplantation is about 55 percent.