Pediatrics: Pulmonary and Sleep Medicine

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Cystic Fibrosis Program

What is cystic fibrosis?

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

  • Clogs the lungs and leads to life-threatening lung infections
  • 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. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

Symptoms of cystic fibrosis

People with CF can have a variety of symptoms, including:

  • Very salty-tasting skin
  • Persistent coughing, at times with phlegm
  • Frequent lung infections
  • Wheezing or shortness of breath
  • Poor growth/weight gain in spite of a good appetite
  • Frequent greasy, bulky stools or difficulty in bowel movements

The Cystic Fibrosis Center at Children’s Hospital of Wisconsin is accredited for care, teaching and research by the Cystic Fibrosis Foundation. Accreditation requires that our CF clinicians provide care based on the following guidelines: a recommendation for a minimum of four visits to a certified CF center annually, two pulmonary function tests each year, sputum cultures frequency and laboratory testing.

The clinic offers multidisciplinary care for newborns, children, and adolescents. The CF team includes specialists in pediatric and pulmonary medicine, nutrition, social work, genetics, nursing, and respiratory care. Children and young adults receive their care at Children’s Hospital of Wisconsin. Adults patients are cared for in the CF Clinic at Froedtert Hospital.

Based on the most recent data provided by the Cystic Fibrosis Foundation Patient Data Registry, children managed at our center have lung function above the national average. More specifically, these outcomes place our center among the top 10 centers in the country for pulmonary outcomes in children.  The Registry also shows a strong association between a higher body mass index percentile and better lung function in children with CF.  Our goal is for children with CF to grow and develop like children without CF.

Outpatient services include:           

  • Pulmonary function testing
  • Instruction in airway clearance
  • Nutrition counseling
  • Sweat testing and genetic counseling for newborns and families identified through the Wisconsin Newborn Screening Program
  • Genetic counseling for individuals and families already diagnosed with CF
  • Referral to other specialty services

Inpatient services include:

  • Hospitalization for aggressive airway clearance and intravenous antibiotics.

Additional services:

  • Research focused on improved treatments
  • Educational program for families and health professionals
© 2015 Medical College of Wisconsin
Page Updated 12/01/2014