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Cystic Fibrosis Center at Women & Children’s Hospital Ranked #8 Among Top Centers in the U.S. for Pediatric Lung Function

Updated: 7/12/2016

The Cystic Fibrosis Center at Women & Children’s Hospital of Buffalo (WCHOB) has ranked among the top Cystic Fibrosis centers in the U.S. for 2015 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 38 years, but is increasing due to newborn screening, care guidelines and new therapies.

According to center specific statistics release by the CF Foundation (the most recent data released), in 2015 at Women & Children’s Hospital, the lung function (FEV1% 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. Lung function in adults at our center is above the national average.

The Center’s nutritional outcomes are improving over time. Both weight and weight for length for children less than 2 years were approaching the top 10 in the country. Body Mass Index (BMI) is also above the national average for both children over 2 years and adults.

“The Cystic Fibrosis Center strives to provide exemplary care to our patients with our team of physicians, nurse practitioners, dietician, respiratory therapist and social worker,” said Danielle Goetz, MD, Director of the CF Center at WCHOB. “We are proud of our accomplishments but continue to strive for improvement in the health and quality of life of our patients. In the coming year, we will continue our emphasis on the maintenance of optimal lung function and nutrition through our clinical care, research and quality improvement initiatives. We will continue to focus on mental health as patients with chronic disease have more depression and anxiety. We will also emphasize quality improvement in the transition of care from pediatrics to adulthood, as more than half of people with CF are now adults.”

Certified by the national Cystic Fibrosis Foundation as a care, teaching and research center, the CF center at WCHOB includes both pediatric and adult programs for the care of patients with non-acute respiratory diseases (care is provided to adults by internal medicine-certified specialists).

In terms of research, the WCHOB CF Center 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, focusing on inhaled antibiotics, mucoactive drugs and CFTR potentiators and correctors.

The CF Center in the Division of Pulmonology (Lung Center) is part of UBMD Pediatrics based out of WCHOB. For more information, please visit www.wchob.org/lung or call (716) 878-7524.