Treatment
Cystic Fibrosis

Clearing the Lungs
Fighting the Bacteria
Improving Nutrition
Future Treatments

There is no cure for CF, and the disease invariably shortens lifespan. But improvements in treatment have increased the average CF patient's lifespan from less than 8 years to more than 35. Prompt, aggressive treatment of CF and the problems it causes is the key to lengthening life. The Cystic Fibrosis Foundation accredits more than 100 centers nationwide for their advanced ability to provide such intensive monitoring and therapy.

The goals of CF treatment are to clear the lungs of mucus, kill harmful bacteria living there, and improve nutrition.

Clearing the Lungs

A number of treatments have been devised to help CF patients clear mucus from their lungs. The classic therapy is called chest physical therapy (CPT). This consists of a regimen of thumps on the patient's chest, chest vibration, deep breathing exercises, and coughing that is designed to break up the mucus and make it easier to cough up. Postural drainage -- lowering the patient's head below the lungs -- is also a part of CPT.

Newer technologies for clearing the lungs use high-frequency vibrations to help break up mucus. Alternative oscillatory positive expiratory pressure devices use a special valve that causes rapid vibrations in the air as a patient exhales. Studies suggest that these devices may be three times as effective as traditional CPT.

Another approach to clearing the lungs uses an inhaled version of a protein that eats up stray DNA. This method helps by destroying the sticky DNA that is present in the CF mucus in the lungs. This DNA comes from white blood cells that come to the lungs to fight bacteria and then die there. This drug-based approach can reduce severe infection episodes and improve lung function slightly.

 

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Fighting the Bacteria

Antibiotics are the method of choice for killing dangerous bacteria in the lungs in CF. Originally these drugs had to be given through a vein. Doctors had to administer large doses to ensure enough entered the lungs where it was needed. These high doses carried a risk of kidney and hearing damage.

A newer generation of inhaled antibiotics has revolutionized CF treatment. Necessary in far lower doses, they cause far fewer side effects than the old-generation antibiotics. These new drugs may be administered via nebulizer or in pill or liquid form.

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Improving Nutrition

Because CF mucus often blocks digestive enzymes getting from the pancreas to the stomach and intestines, it can cause people to lose weight even when they have an appetite and are eating. Part of CF treatment consists of nutritional counseling to help patients eat a high-calorie diet that is nevertheless healthy and balanced.

In addition to taking in more calories, CF patients may also need to take nutritional supplements to help them digest it. Vitamins and minerals are a part of a CF dietary plan. So also are pancreatic enzymes to replace those lost to the disease. These enzymes require a skilled nutritionist and a CF-experienced treatment team to administer properly, as they can cause serious side effects if taken at too high a dose.

Occasionally, a CF patient may require feeding through a nose tube or intravenous nutrition. CF nutritional care is designed to try to prevent those methods from becoming necessary.

 

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Future Treatments

In addition to developing new antibiotic therapies and methods for improving airway drainage and nutrition, researchers are exploring a number of avenues for treating, and maybe someday curing, CF.

Perhaps the most exciting possibility for the future of CF treatment lies in gene therapy -- the use of DNA or other genetic material to repair or replace the function of a mutated gene. Unfortunately, gene therapy is far from ready for prime time. While trials in patients have begun, current methods seem some way off from being able to correct or replace enough CFTR function to reverse the disease, particularly over the long term.

Gene therapy may well be the wave of the future for CF treatment, and the chance for a cure is attractive to many well-informed patients in clinical trials of the method. Unfortunately, no one can yet say when this future -- and the cure -- will arrive.




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