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Intellect

BYU professor, mother of children with cystic fibrosis, researches new approach to treatment

After her son John was born with cystic fibrosis, a terminal genetic disorder that afflicts 30,000 Americans, Brigham Young University political science professor Valerie Hudson put her scholarly training to work in an entirely different field -- medicine.

In the course of exploring widespread treatment avenues within the cystic fibrosis research community, Hudson unearthed what she thinks is a simple approach to alleviating many of the disease's chronic symptoms. Her idea revolves around a substance that occurs naturally in the body called glutathione. Cystic fibrosis patients have low levels of glutathione; that deficiency contributes to lung and immune system problems.

At first, Hudson's efforts to share her insights with the cystic fibrosis research community were met with, at best, indifference. (For more on Hudson's abrupt introduction to medical research, see this previous BYU News release.

Then, in June 2001, Free Radical Biology and Medicine, a cell biology journal, published Hudson's article on her treatment approach. The subsequent regional media attention resulted in $46,000 in donations (much unsolicited) toward a clinical trial of the treatment.

In 2002 Hudson teamed with BYU alumnus Clark Bishop, a pulmonologist and former medical director at 330-bed Utah Valley Regional Medical Center in Provo, Utah, to conduct a pilot study of glutathione treatment. It enrolled 19 children and teens and was funded solely by the private donations; Dr. Bishop and others donated their time and expertise. The study was recently completed, and the results are being submitted to medical journals.

Encouraged by the results, Hudson and Bishop hope that experienced cystic fibrosis researchers with greater resources will conduct a larger, definitive clinical trial.

Since John's birth six years ago, Hudson and her husband David have welcomed two more sons, Tommy, 4, and Jimmy, 1. Both also have cystic fibrosis, with which patients suffer from abnormally thick mucus that clogs their lungs and digestive tract. Their chronically inflamed lungs are plagued by persistent infections that eventually waste away their lung capacity and kill them. Life expectancy for the 30,000 Americans with the disease is about 30 years.

The condition results from a mutation of the gene responsible for manufacturing a protein known as CFTR that normally serves as a channel out of most cells. In cystic fibrosis patients, the channel is blocked and doesn't allow the passage of certain biochemicals. One of them is called glutathione, which performs a number of functions that protect healthy people's lungs.

As teens, cystic fibrosis patients have between 5 and 20 percent of a healthy person's levels of glutathione on the surface of their lungs since the chemical is trapped inside their lung cells. Hudson's journal article showed how this deficiency could cause many of the symptoms of cystic fibrosis.

She cautions that she is not claiming to have understood or solved the entire mechanism with which cystic fibrosis attacks patients. Rather, Hudson says, she has identified a new approach that further research could show to alleviate many symptoms. Hudson and Bishop also caution cystic fibrosis patients to never adjust treatments without their physicians' supervision.

Henry J. Forman, chairman of the Department of Environmental Health Sciences and co-director of the Center for Free Radical Biology at University of Alabama-Birmingham, has published studies on the role of glutathione in cystic fibrosis and is the co-editor of the journal that published Hudson's article.

"(The paper) went through a fairly tough review. It's valid as a hypothesis -- it's testable and the chemistry underlying the potential protective role of glutathione is there," Forman said at the time of the paper's publication.

"In general, most people would agree it's a good idea to restore glutathione to normal," he said of Hudson's assertion. "It's hard to say if this therapy will make a major difference -- it would certainly seem that increasing glutathione in the (lung's) airspace would have an advantageous effect."

Now that she has seen an article published in a medical journal and a pilot trial completed, Hudson recognizes the need for other medical investigators to move the research further.

"It was never my intent to carry the baton forever, but to pass it to the professional community," Hudson said. "I hope the fact that I'm a political scientist and a 'CF mom' doesn't doom this therapy."

Cystic fibrosis patients or family members can learn more by contacting gshtreatment@ihc.com

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