Management of Cystic Fibrosis (CF)

How is cystic fibrosis diagnosed?
How is cystic fibrosis best managed?
What is the prognosis of treatment?

How is cystic fibrosis diagnosed?

There are a number of ways to diagnose CF.  Both parents must carry the gene.  If you have a sibling, aunt, uncle, cousin or other relative who has CF, you may also carry the gene.  A blood test, a scrape of tissue from inside the mouth, or a sweat chloride test can be used to detect CF.

Unfortunately, many people only discover that they are carriers when they have a child born with CF.  Several states have mandatory cystic fibrosis screening for newborns.

How is cystic fibrosis best managed?

Treatment for CF is individualized, since no two patients are alike.  Yet, many therapies, including inhaled antibiotics, and enzymes to replace those produced by the pancreas are available.  Gene therapy (transplanting or replacing genes) – while not available yet - is at the forefront of research.

The only commonality among all the treatments is that the more active patients can be, the better their lungs function.  Even if they are on oxygen or a night ventilator, people with CF should do mildly vigorous aerobic exercise.  This may require supervised activity, during which oxygen saturation is measured and heart rate is monitored.  Some patients may also need supplemental oxygen while exercising.

For those whose condition is not very severe, aerobic exercise such as swimming, running and biking is beneficial.

What is the prognosis of treatment?

CF has a remarkably variable clinical course, depending on the severity of the genetic mutation, the types of infections acquired, and the organs other then the lung that is affected.  For all patients with CF, the median survival has risen steadily over the past four decades.

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This information has been approved by Jerry Nick, M.D. (March, 2006).