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Cystic Fibrosis Center Ranks Among Top Centers in the U.S. for Pediatric Lung Function

Updated: 8/15/2017

The Cystic Fibrosis Center at Women & Children’s Hospital of Buffalo (WCHOB) has ranked #8 among the top Cystic Fibrosis centers in the U.S. for 2016 for pediatric lung function.

Cystic Fibrosis (CF) is a genetic disease that affects approximately 30,000 people in the country. With CF, a defective gene called CFTR causes the body to produce abnormally thick and sticky mucus. The mucus clogs the lungs and obstructs the pancreas, making breathing and food absorption difficult, as well as impacting other organs. According to the Cystic Fibrosis Foundation’s National Patient Registry, the median age of survival for a person with the disease is approximately 40 years, but is increasing due to tremendous breakthroughs in research, newborn screening, care guidelines and new therapies.

According to center specific statistics released by the CF Foundation (the most recent data released), in 2016 at Women & Children’s Hospital, the lung function (FEV1 percent predicted) for children ages 6-17 years was ranked #8 in the country out of approximately 120 care centers. Pulmonary function is still the most important outcome in CF across the lifespan.

The Center’s nutritional outcomes are improving over time as well, ranking #9 nationally in weight for length, a major marker of nutrition in infants less than 24 months old. The median Body Mass Index (BMI) is above the goal (60.8% vs. goal of 50%) for ages 2-19 years, which is in the top third of all centers nationally.

“The Cystic Fibrosis Center of Western NY is committed to providing the best care possible to the nearly 200 people living with CF in our area. We have a team of physicians, nurses, nurse practitioners, physician assistant, social worker, respiratory therapist, and nutritionist that work together for the care of our patients,” said Danielle Goetz, MD, director of the CF Center of Western NY. “We are proud of our accomplishments but continue to strive for improvement to CF care through quality improvement and research initiatives.”

Certified by the national Cystic Fibrosis Foundation as a care, teaching and research center, the CF center of Western NY includes both pediatric and adult programs (care is provided to adults by internal medicine-certified specialists).

In terms of research, the CF Center of WNY is one of the 82 centers involved in the Cystic Fibrosis Foundation Therapeutic Development Network, through which multicenter research trials are performed to help develop new therapies for CF. The center has participated in more than 40 clinical trials and has helped bring several drugs through development to clinical use for patients with CF, including KalydecoTM (Ivacaftor) and OrkambiTM (Ivacaftor/Lumacaftor), which are drugs that correct the CFTR protein. Research with Next Generation compounds is underway: initial clinical data have shown their potential to dramatically improve lung function and survival, when combined with ivacaftor and tezacaftor (

The CF Center in the Division of Pulmonology (Lung Center) is part of UBMD Pediatrics.

For more information, please visit or call (716) 878-7524. The phone number will change to(716) 323-0110 after August 28, 2017.