HY 11/06: Coping With Cystic Fibrosis | Lehigh Valley Hospital

For children with this chronic disease, staying healthy is a constant challenge

If your child catches a cold, you know she’ll probably be healthy in a week or so. If 4-year-old Bree catches a cold, it can be life-threatening. Bree has cystic fibrosis (CF). “It’s a constant balancing act, shielding her from infection and letting her enjoy childhood,” says Bree’s mother, Liza Gould.

Bree needs to take 30 tightly scheduled medications a day—enzymes to aid digestion, vitamins to boost nutrition, and drugs for sinus and lung problems. Twice a day she has a therapy vest treatment to vibrate her chest and loosen mucus so she can cough it out. Twice a day she inhales antibiotics, and at night she gets extra nourishment through a feeding tube.

Despite all this, Bree is a happy little girl, her mother says. “She loves swimming, fishing and playing in the park with other children, although she needs to avoid contact with people who are sick. She understands that she has CF, but together we try each day to make sure her whole life is not consumed by this illness.”

Cystic fibrosis is a genetic disease in which the defective gene—inherited from both parents—affects the regulation of fluids and mucus in the lungs and digestive organs. The mucus becomes thick, trapping bacteria in the lungs and blocking the action of digestive enzymes. Like Bree, some 85 percent of children with CF need enzyme medication to help them absorb nutrients from their food.

“This is a complex illness with more than 1,000 genetic variations,” says Robert Miller, M.D., pediatric pulmonologist at Lehigh Valley Hospital and Health Network. “Its effects can vary from severe to mild.

Some children struggle constantly against infection, while others live moderately healthy lives. Many children with CF are able to attend public school and even take part in sports and other activities.”

Though there is no cure for CF, researchers have made progress understanding and detecting the disease. Of the two main tests that help in diagnosis, Miller says, the gold standard is the sweat chloride test measuring the amount of salt in the child’s perspiration. “Many children with CF,” he says, “are identified at a very young age through supplemental newborn screening tests. This early identification means we can begin treatment before problems become more severe.”

Researchers have been working to develop new therapies to manage CF symptoms. In the past 10 years, the median life expectancy of CF survivors in the United States has risen from late teens to 36.8 years. “Our goal,” Miller says, “is to help the child and family lead as healthy a life as possible.”

Want to Know More about the expanding cystic fibrosis program at Lehigh Valley Hospital’s Pediatric Specialty Care Center? Click here.