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May 2003 Issue

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Researchers Find Cystic Fibrosis Patients Value Quality of Life and Health

Published May 2003

Researchers at UC found that a group of patients diagnosed with cystic fibrosis place high values on their current health. In the manuscript titled "Health Values of Adolescents with Cystic Fibrosis," published in the February issue of The Journal of Pediatrics, the researchers assessed the health-related quality of life among patients with cystic fibrosis.

"Although adolescents with cystic fibrosis have a shortened life expectancy and may suffer from significant disability, they valued their current state of health highly," said Michael S. Yi, MD, assistant professor of internal medicine and pediatrics at UC and Cincinnati Children's Hospital Medical Center (CCHMC). "Our findings indicate that these young patients appear to have adapted to life with the disease and its negative impact on quality of life."

Two methods were used to measure health-related quality of life: health status measurement and health value or utility assessment. Health status measurements assess functioning in a specific health-related area such as physical functioning, mental health, and pain. Health value or utility assessments measure the degree to which a patient will go to achieve a desirable state of health.

"Before we began the study, there was a question as to whether one could assess health values from adolescents, as the concepts are complicated," said Joel Tsevat, MD, MPH, associate professor and director of UC's Section of Outcomes Research. "This study showed that one can assess their health values. And, although adolescent patients with cystic fibrosis generally value their health highly, meaning that they wouldn't trade much time or take much of a risk to obtain better health, health values are highly individualistic. You can't predict them from clinical or demographic characteristics, so it's important to assess them directly."

The study tested adolescents 12-18 years of age from CCHMC and Dayton Children's Medical Center. During a single interview session, they completed two health status instruments. One of the instruments assessed whether patients would prefer a shorter but healthier life. Another part of that test assessed whether patients would take a risk of death in exchange for perfect health. Dr. Yi said there was a clear association between self-rated general health and health values. For example, patients rating their health as excellent or very good were willing to give up an average of less than 6 months of life expectancy in exchange for perfect health. Those rating their health as fair or poor were willing to give up nearly 1.5 years, given a life expectancy of 15 years. This relationship did not exist when health values were compared with objective markers of disease until end-stage disease was present. This indicates that a threshold may exist with greater willingness to give up life expectancy or take a risk of death in exchange for perfect health only when severe clinical disease was present.

Cystic fibrosis is characterized by reduced chloride ion permeability (defects in how salt and water are transported across cell linings in the airways, pancreas, intestine, sweat glands and other organs). Since water doesn't transport easily, the respiratory and GI tracts become clogged with mucus. This thick build-up of mucus deposits in the lungs leads to increased susceptibility toward pulmonary infections. There is no cure, although recent years have seen great improvement in maintenance therapy, including effective treatment of secondary infections and new ways to remove the destructive build up of mucus in the airways.

In 2001, John Cuppoletti, PhD, professor of molecular and cellular physiology and internal medicine at UC, lead a team of researchers in the development and patenting of two new cystic fibrosis treatments. Dr. Cuppoletti's team presented a paper on the treatments at the Experimental Biology 2001 meeting.

Due to advances in technology, life expectancy of patients with cystic fibrosis has increased to age 40, compared to the life expectancy of less than one year in the 1940s. Despite these improvements in life expectancy, the surviving adults with cystic fibrosis may have infertility problems. "Many men with cystic fibrosis are infertile and women have difficulty conceiving," said Dr. Yi. "Adolescents with the disease lack knowledge about their own fertility. Future work will need to identify where the communication gap is occurring."

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