Tuesday, April 15, 2008

Outlook for people with cystic fibrosis improving

The Baltimore Sun reported April 13 on advancements that have led to many more people with cystic fibrosis living to adulthood.

Baltimore is home to the 12-year-old adult cystic fibrosis clinic run by Johns Hopkins Hospital. "Affecting 30,000 people in the United States alone," The Sun reports, "cystic fibrosis is an inherited disease that causes the body to produce thick, sticky mucus that can clog the lungs and damage the pancreas, interfering with the body's ability to absorb nutrients. As a result, victims are prone to dangerous respiratory infections and struggle to gain weight. In the early 1960s, the median survival age was 10 years.

"A steady progression of drugs, medical devices and improvements in disease management has made it possible for patients not only to survive longer, but also to attend college, have careers and raise families," The Sun reports "By the early 1980s, median survival had risen to 21 years. Today, it is 37."

Even though treatments and therapies have vastly improved the treatment of its symptoms, medical researchers are now working to develop medications that will make the protein that causes cystic fibrosis work correctly.

"These are interesting and good times to be in cystic fibrosis research," said Dr. Craig Gerard, who treats patients at Boston Children's Hospital. "We now have drugs in development that directly target the nature of the disease."

People with cystic fibrosis are now faced with traditional life decisions once thought impossible -- whether to get married, have children, pursue long-term career opportunities, etc.

James Albright, 44, of Virginia, has cystic fibrosis and wears an IV to deliver the medications he needs; he said in the article that he was very honest with his future wife about his uncertain longevity before they married 20 years ago.

"I was pretty up-front about it," Albright said. "If we could get five good years out of it, it would be great. I couldn't promise anything beyond that. But married couples tend to live longer - my goal is 70s."

The couple now has a family of five, having adopted three boys, including one with cystic fibrosis.