Medical Advances Help Cystic Fibrosis Patients
More Money for Research is Still Needed to Find a Cure for CF
It's one of the most insidious of diseases. Young cystic fibrosis patients often look healthy and fit, but inside, their own bodies are slowly killing them.
Forty years ago, most CF patients died by the age of 4 or 5. But since then, more sophisticated treatments have allowed many patients to survive past 18, with some living twice that. Double lung transplants have added even more years to the lives of CF patients, but transplants are not a cure.
What is Cystic Fibrosis?
Cystic fibrosis is an inherited disease that causes the lungs to fill with thick, heavy mucus that hinders normal lung function. A person with CF needs to undergo rigorous physical exercises designed to loosen the mucus and have his or her lungs suctioned free of the deadly gunk twice a day. The mucus often results in a buildup of bacteria that cause repeated lung infections, causing further damage.In most patients, CF also causes thick mucus to block the pancreatic ducts, preventing the release of digestive enzymes. Patients must take 20 or 30 pills every day to enable their bodies to digest and absorb food.
As the patient grows older, lung function deteriorates. Treatment becomes less effective. The patient becomes weaker and weaker, and needs to use oxygen more often. Weight decreases to the point of emaciation. Other problems such as arthritis, diabetes and liver disease can develop. At this stage, only a successful double lung transplant will save the person’s life.
However, even a successful transplant is not a cure. Anti-rejection drugs are taken for life. And, since CF is genetic, the patient still has the disease. Eventually, whether five years or 25 years later, the new lungs may fail, too, from rejection or chronic bacterial infections.
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