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Advanced treatment improves life for cystic fibrosis patients - WCAX.COM Local Vermont News, Weather and Sports-

Advanced treatment improves life for cystic fibrosis patients

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BURLINGTON, Vt. -

In the 1950s, most children with cystic fibrosis died before the age of 5. But now, major therapeutic advancements in treatment have improved the length and the quality of life for patients dramatically.

Thursday was clinic day at the Cystic Fibrosis Center at Fletcher Allen Health Care in Burlington. And for patients like 10-year-old Bridget Mallo, a height check and weigh-in are routine on these visits made several times a year.

Bridget's cystic fibrosis makes it difficult for her to breathe. Thick mucus clogs her lungs and she needs to do airway clearance and cough it up several times a day. Like most CF patients, the genetic disease also affects her pancreas, so digestive issues are common and so, too, are frequent infections.

Doctors say the treatment is complex with dozens of medications and vitamins required each day.

"There are so many different therapies that are available. We have people that can inhale antibiotics at home. There are medications that help thin down the mucus in their lungs. There is anti-inflammatory medications that we use and all these different antibiotics when people do get some symptoms you try to treat them, and then with all of the other nutritional support that they get," said Dr. Thomas Lahiri of Fletcher Allen Health Care.

But it's all those therapies and a mandated newborn screening test that are now keeping patients alive longer. Decades ago, children died before reaching school age. Today, they're living decades longer with new drugs and early intervention.

"The change in life expectancy is due to different therapies that have come along. First, the enzyme replacement therapies that were available in the late 50s, early 60s and then antibiotics had a huge impact," said Lahiri. "This is no longer a pediatric disease. This used to be a pediatric disease, taking care of by pediatricians and now, I think this year, I think half of the CF patients in the United States are adults."

That's half of an estimated 30,000 nationwide. Stephanie McKeel, 17, is one of them.

"Yeah, people live up until their 60s now than they used to live. Before elementary school they would die, but I guess I have a good outcome,' said McKeel.

And she's living a full life as a high school athlete, a good student and a mentor to others. She's a cystic fibrosis patient expected to live longer because of major medical advancements.

The Vermont Cystic Fibrosis Center currently has about 150 patients. It is one of 110 certified programs in the country with a national study placing in the top ranks for screening, nutrition and lung function.

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