New Drug May Help Cystic Fibrosis Patients

Cystic fibrosis is an inherited disease that can be deadly. The scars that form in the lungs are accompanied by a thick, sticky mucus that makes it extremely difficult to breathe. Usually Cystic Fibrosis patients are disabled throughout most of their lives and die at an early age.

I have a friend named Greg who died a few years ago that had a condition that was similar to cystic fibrosis. The scar formation in his case came from a car accident that he was involved in when he was in his late twenties. During the accident he inhaled some glass dust from a pulverized window. The tiny glass fragments caused scar tissue to form in his lungs, just like in CF.

Over the course of the next 20 years, he slowly suffocated to death. At the end he was left bed ridden and gasping for air. He left behind hundreds of friends (He was very active in his community), and a loving wife and five children. His wife is the chiropractor that I go to see now.

Now there may be some relief for those in the early stages of Cystic Fibrosis. According to Medical News Today:

"Cystic fibrosis (CF) patients with normal to mildly impaired lung function may benefit from a new investigational drug designed to help prevent formation of the sticky mucus that is a hallmark of the disease, according to researchers involved in a phase 3 clinical trial of the drug."

The drug is called denufosol and may be of help for those in the early stage of the disease when lung function is not severely impaired. Taking the drug may delay the onset of the more severe symptoms, but not eliminate them. The results of the phase 3 study were published in the American Journal of Respiratory and Critical Care Medicine.

Most CF patients are born with normal lung function. The problems begin in early childhood. As denufosol can be given very early in life, the hope is that it can keep the lung function normal in these children as long as possible.

Denufosol is known as an ion channel regulator. These drugs balance the flow of ions between cell membranes. By doing this, the mechanism that removes the buildup of mucus in the lungs works normally, preventing buildup and mucus formation. The drug also stimulates the movement of the cilia which are tiny hairs that line the lungs and help sweep out foreign particles.

One only wonders if this drug would have help Greg in the early stages of his disease.

Source; http://www.medicalnewstoday.com/articles/211456.php