Cystic Fibrosis: How We Treat

How is cystic fibrosis treated?

Cystic Fibrosis at Children's Hospital Colorado

The main treatment strategies for people with CF are airway clearance, prevention and treatment of lung infections, and nutritional therapy.

The daily care program varies from child to child, but usually includes airway clearance treatments with nebulized medications that are mixed with air and then inhaled to help loosen and clear mucus from the lungs. Children may need frequent oral or inhaled antibiotics to treat lung infections.

Children with CF need a high-calorie, high-fat and high-protein diet. They often take oral pancreatic enzymes to help them digest food. They also need vitamin supplements (particularly fat-soluble vitamins A, D, E and K) and extra salt.

The CF Foundation recommends that all people with CF be followed in an accredited CF care center with regular visits at least every three months, and more often before age 1.

A new treatment for CF, which was approved by the FDA in 2012, is called Kalydeco™. This oral medication helps the abnormal CF protein (CFTR) to work better, and dramatically improves lung function and weight gain in people with CF. It is the first drug to treat the underlying problem in CF, rather than the symptoms. Unfortunately, because of the way it helps the protein to work, Kalydeco™ only benefits 4% of people with CF, those who have the G551D CF mutation. However, there are other drugs currently in clinical trials that may help many more people with CF, including those with the most common ΔF508 mutation.

Why choose Children's Hospital Colorado for your child's cystic fibrosis?

Children's Colorado is the site of one of the premiere cystic fibrosis (CF) clinical care and research centers in the country. The Mike McMorris Cystic Fibrosis Research and Care Center at Children's Colorado delivers state-of-the-art care to people with CF, provides education for healthcare professionals and families with CF and advances the field through clinical research.

Our center, established in 1990 and accredited by the National Cystic Fibrosis Foundation, is the largest CF clinical care center in the United States. Our pediatric program cares for more than 400 children and adolescents with CF throughout the Rocky Mountain region, and approximately 300 people with CF are seen at the adult program based at National Jewish Health. Our program has outreach clinics in Colorado Springs and oversees an affiliate CF center in Montana. We also serve as a national referral center because of our experience and advanced diagnostic capabilities.

Our program works to be patient-centered in many ways, including:

  • We have established a CF Family Advisory Council, made up of our patients' parents and clinical staff, who work as partners with the CF clinical care team to ensure excellence in the development and delivery of medical care for those with CF and their families in the Rocky Mountain region.
  • Our multidisciplinary CF care team includes board-certified pediatric pulmonologists and gastroenterologists, pediatric endocrinologists, CF clinical nurse specialists, registered dietitians, social workers respiratory therapists and research coordinators, as well as administrative support staff.
  • Our clinics see children with CF five days a week, Monday through Friday.
  • We offer comprehensive inpatient and outpatient multidisciplinary medical care.
  • Our program consistently ranks as one of the top performing CF centers in the country based on lung function data in our children and adolescents.
  • We work to continually improve care at our CF center through quality improvement initiatives and educational programs for health care providers and families.