New drugs and technologies have helped improve the lives of cystic fibrosis patients in recent years, but one advancement is a return to basics: More and more, doctors are encouraging exercise.
CF is a life-threatening genetic disorder that creates a build-up of mucus inside the body. It affects about 30,000 people nationally, and about 70,000 worldwide.
Many organs are affected by CF, but the lungs most severely, where that build-up of mucus can make breathing difficult and foster infection. The most common cause of death in CF patients is respiratory failure, and the median life expectancy now hovers at about 40.
It’s that build-up of mucus inside the lungs that exercise targets directly.
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“In recent years, there is an increased recognition that exercise — pretty much any form of exercise, but especially cardiovascular exercise — is helping to clear the lungs,” said Dr. Matthias Salathe, the director of the Cystic Fibrosis Center and chief of the Division of Pulmonary, Allergy, Critical Care and Sleep Medicine at the University of Miami Miller School of Medicine.
The movement and vibrations involved in exercise help to break up and loosen the mucus, as does the deeper breathing, according to Salathe. Muscle strengthening and building exercises are also typically recommended, he said, because they’re linked to a stronger immune system.
With research suggesting that the salty mist helps liquefy the mucus, swimming or surfing in the ocean is also recommended.
There haven’t been any long-term studies done with controls, which would determine how effective exercise is in improving the lives of CF patients, Salathe said. But the studies that have been done, he said, concur with growing anecdotal evidence from the CF community and its health workers that exercise dramatically helps.
Michele Diaz, a clinical nurse manager at Regional Memorial Hospital who has worked with CF patients for nearly 20 years, agrees. In her experience, she said, patients who exercise “seem to have a much better quality of life and to live a lot longer compared to certain individuals that come in and do not exercise.”
Exercise helps maintain bone mass, which CF patients more easily lose, and also helps manage diabetes, which often develops in CF patients.
Both UM and Memorial now incorporate tailored exercises into their treatment plans for CF patients.
Brian Callanan believes strongly in encouraging exercise in CF patients — in fact, in 2003, he founded a non-profit to help kids and adults living with the disease help pay for the costs associated with living an active lifestyle.
But for Callanan, who has CF himself, exercise isn’t just about “avoiding getting sick.” It’s also about motivation and fulfillment.
“The paradigm of CF has always been the poor sick child that has a death sentence and may not live a full long life,” he said. “We’re hoping to transform that into CF as a reason to be active. To have this as a driving force.”
Finally, for Callanan, it’s about building friendships and finding support —something that’s particularly important for CF patients, who can’t interact with other CF patients for fear of potentially life-threatening cross-contamination.
Skiing, hiking and cycling have helped him tremendously, he said, but it was the friendships he developed doing those activities that helped “in dealing with a lot of the social and psychological challenges that come along with a pretty involved and complicated disease.”
In that vein, the Cystic Fibrosis Lifestyle Foundation doesn’t just award grants directly to CF patients; in some cases the foundation will pay for a grant recipient’s friend to join them in their activity, or help train a CF patient’s chosen mentor in the management of the disease.
To find out more about the Cystic Fibrosis Lifestyle Foundation, whether to volunteer, donate or apply for a grant, visit the website at http://www.cflf.org/ or call 802-310-5983.