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Children's Health

Understanding Cystic Fibrosis


Author:

Karen Barrow

Medically Reviewed On: November 02, 2005

How would a doctor go about diagnosing the disease?
The way to diagnose CF is a sweat test, the "gold standard" for CF diagnosis. The simple test is noninvasive and relatively inexpensive. A small electrode is placed on the arm to stimulate a sweat gland, and the sweat is collected. An abnormally high concentration of salt in the sweat indicates that a person has CF. Infants may be tested as early as one week if enough sweat can be collected. It is strongly recommended that the sweat test be performed in one of the CF Foundation's accredited care centers, which have guidelines in place to ensure the test's accuracy.

The other means of diagnosing CF is to do a genetic analysis looking for mutations in the CF gene. A diagnosis of CF requires the identification of a mutation in each of the two CF genes in an individual. This information complements the sweat test for most individuals, and for some with a borderline or normal sweat test, it's the only way to make the diagnosis

What type of treatment helps to improve the lung function of someone with cystic fibrosis?
The ongoing treatment of CF depends upon the stage of the disease and which organs are involved. Most patients with CF need airway clearance. One form of airway clearance, chest physical therapy, requires vigorous pounding with cupped hands on the back and chest to dislodge the thick mucus from the lungs. Other forms of airway clearance can be done with the help of mechanical devices used to stimulate mucus clearance. Antibiotics are often used to treat lung infections and are administered intravenously, via pills or with medicated vapors, which are inhaled.

Mucus-thinning drugs such as Pulmozyme are also available. And many patients take Tobramycin Inhalation Solution (TOBI) to treat chronic lung infections. This drug can help reduce the number of hospital stays a patient must endure. Azithromycin, a type of antibiotic, has been shown to be effective in improving the lung function of people with CF who are chronically infected with the bacteria Pseudomonas aeruginosa. It is also used to treat common bacterial infections, such as pneumonia and ear infections.

When should a patient consider a double lung transplant?
Deciding to have a double lung transplant is a very complex issue that revolves around how long the patient is expected to live without transplant versus the predicted outcome following lung transplant. Lung transplants have the worst outcomes of all solid organ transplants. There is approximately a 60 percent five-year survival rate for patients post-transplant.

The decision to have the transplant and the timing of the transplant is an individual decision that involves the patient, family and the CF care team at the transplant center.

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