Advances in cystic fibrosis treatment transforming patients' lives

WEST LEBANON, N.H. (WCAX) Dramatic advances are being made into cystic fibrosis, a genetic disorder that affects thousands of people across the country.

Just looking at 31-year-old Daniel Benner, you would never know that he has been battling a deadly disease his entire life. Benner was diagnose with cystic fibrosis at age three.

"I played outside all the time. I rode my bike. I did my medications of course, but that was done in the morning, just like brushing my teeth or any other normal activity," Benner said.

When he rolls up his sleeve, there is evidence of the CF. An IV dock that, when needed, provides regular doses of antibiotics.

"It plugs up the pancreas so patients don't secrete the normal pancreatic enzymes, therefore they don't digest food well -- particularly fats -- and they have thick, sticky mucus in the airways that causes blockage and is a good medium for growing bacteria," said Dr. Margaret Guill, a pediatric pulmonology specialist at Dartmouth-Hitchcock Medical Center.

Cystic fibrosis occurs in 1 of 2,500 to 3,500 white newborns, according to the government statistics. "It is considered to be the most common, life-shortening disease in the caucasian population," Guill said.

"My dad always told me that if there was a piano hanging over your head, and it was going to drop someday, eventually you would stop thinking about it. So, that is the same approach I really take with this," Benner said.

And there is reason for him to keep his head up. Doctors say big gains are being made treating the disease. For one, babies are now being screened at birth, before symptoms begin. Like most illnesses, catching it early is important. Also, there are new drugs that are literal game changers. "New oral medication that actually change the expression of the gene and therefore decrease the manifestations of the illness," Guill said.

Years ago, CF patients weren't expected to live past their 20s. But now, while there is still no cure, doctors say it is no longer a death sentence. "Patients born now born with cystic fibrosis should expect to have a normal lifetime. I tell my patients I expect them to pay my social security," Guill said.

That's a bill Benner says is worth fighting for. "I think as long as you try to be as preventive and take care of it and stay on top of the medication, I really think the future is looking bright," he said.