Combating Pediatric Cystic Fibrosis on All Fronts

For a condition that impacts as much anatomy as cystic fibrosis does, nothing short of an aggressive, multidisciplinary treatment approach makes much sense.

“Cystic fibrosis affects multiple body systems, but most critically the lungs, gastrointestinal tract and pancreatic functions,” says Nathan Kraynack, MD, a pediatric pulmonologist who directs Cleveland Clinic Children’s comprehensive cystic fibrosis care offerings out of the Center for Pediatric Pulmonary Medicine. “One of the leading advantages here is that we have experts in every subspecialty needed to care for these complex patients.”

All about integration

The multidisciplinary team, which has a combined 140+ years of experience in CF clinical care, includes pediatric subspecialists in pulmonology, endocrinology, gastroenterology, infectious diseases, adolescent medicine and rheumatology.

Patients can also benefit from specialized pediatric supporting services including nutrition, respiratory therapy and physical therapy. Center for Pediatric Pulmonary Medicine staff help patients and families navigate the network of necessary providers, arranging same-day appointments with any required specialists.

“The integrated nature of Cleveland Clinic Children’s sets us apart,” Dr. Kraynack notes. “All our specialists can easily talk to each other by simply walking down the hall or using our integrated healthcare record, which does not exist in all healthcare organizations.”

Tapping adult-care colleagues as a resource

Patients also benefit from the Center for Pediatric Pulmonary Medicine’s close ties with Cleveland Clinic’s Respiratory Institute, which treats adult cystic fibrosis patients.

Elliot Dasenbrook, MD, was recently recruited from Cleveland’s University Hospitals to direct the Respiratory Institute’s adult cystic fibrosis program. Both he and Dr. Kraynack, who previously headed the Cystic Fibrosis Center at Akron Children’s Hospital, are leaders in their field. They will work closely to foster collaboration between the pediatric and adult programs.

“We can reach out to the Respiratory Institute and take advantage of its resources and expertise if something seems atypical in our patients,” Dr. Kraynack says. “The relationship also allows us to offer pediatric patients seamless transition to the adult cystic fibrosis program.”

Other notable Cleveland Clinic offerings to pediatric cystic fibrosis patients include:

Standardized protocols for care delivery that have been proven to improve outcomes

• Specialized services, including interventional bronchoscopy
• An active, experienced pediatric and adult lung transplant program
• A robust research program, including use of cystic fibrosis lung explants to conduct basic-science research on the condition

Advice on screening and referral

All 50 states now offer newborn screening for cystic fibrosis, and about 75 percent of U.S. patients are diagnosed through newborn screening.

“Newborns with an abnormal screen can be referred to Cleveland Clinic Children’s,” says Dr. Kraynack. “They can come on a same-day visit, get their diagnostic sweat test, see a cystic fibrosis clinician and meet with a geneticist for genetic counseling.”

He cautions, however, that screening is not perfect. “Some newborns with cystic fibrosis can be missed,” he says. “If you have a patient who seems to be struggling to gain weight, you need to consider cystic fibrosis as a possibility and refer them for assessment.”

Referral is also in order when older children with cystic fibrosis are not responding to treatment. “If you are concerned that one of your patients isn’t doing as well as expected or you want a second opinion, consider reaching out for a consult,” Dr. Kraynack advises.