Greater life expectancy means cystic fibrosis patients face new challenges
By Ross Tomlin
Now more than ever, cystic fibrosis patients can expect a better quality of life – even though it means facing new challenges as a result, say lung experts at Baylor College of Medicine in Houston.
Marcia Katz, M.D., assistant professor of medicine and director of the Adult Cystic Fibrosis Center at BCM, says that while understanding of and treatment for the genetic lung disorder have come a long way in recent years, a greater life expectancy introduces a new set of obstacles.
Challenges of adulthood
"As our patients are living into adulthood, we're also dealing with cancer, coronary artery disease, high cholesterol and menopause," said Katz. "You can imagine the diagnoses that are part of growing old in our society that – good news/bad news – cystic fibrosis patients are starting to deal with."
Whereas before, patients didn't live long enough to make decisions about their future, advances in research and medical treatment have raised expectations of living into their 30s, 40s or beyond. Thanks to better interventions, 40 percent of cystic fibrosis patients are adults or nearing adulthood. This introduces life choices they didn't have before.
"Cystic fibrosis is no longer a death sentence," said Katz. "It's vital that these patients are counseled in choices of college education, vocation, lifestyle and family planning."
With the advent of assisted reproductive technology, sterility, commonly associated with cystic fibrosis, no longer necessarily precludes cystic fibrosis patients from having children.
Therapies have come a long way
An inherited chronic disease, cystic fibrosis causes a build-up of mucus that clogs the lungs and leads to life-threatening lung infections and obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
In the 1950s, few children with cystic fibrosis lived to attend elementary school, a prognosis extended dramatically over the years. Modern therapies include an extensive drug regimen – consisting of inhaled antibiotics and steroids as well as bronchodilators and numerous medications – and airway clearance methods like wearing a vibrational vest.
Getting treatment buy-in
These methods can take up to an hour or hour-and-a-half and are recommended twice a day. Although shown to be effective, such aggressive treatment interventions invite resistance among some teen patients.
"You couple normal adolescence growth and the need for independence with the need to be aggressive with their treatment regimen," said Katz. "The biggest challenge we face with the young adult population is to get them to buy in that complying with their treatment regimen is necessary. This will buy them years later on down the road."
