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