News from the Lung, Cystic Fibrosis & Assisted Breathing Center

Released: 8/19/2013

Women & Children’s Hospital of Buffalo, UBMD Pediatrics, and the University at Buffalo School of Medicine and Biomedical Sciences welcomes Danielle Goetz, MD, who is joining the Division of Pediatric Pulmonology and the Women & Children’s Hospital of Buffalo Lung Center.  Dr. Goetz is Board Certified by the American Board of Pediatrics in both Pediatrics and Pediatric Pulmonology. 

We also welcome Barbara Surace, RN, MS, CPNP-PC, as the new Pediatric Cystic Fibrosis Center Coordinator.  Barbara was Director of School Health Services for the LockportCitySchool District.

Michael Bye, MD, continues to serve as Chief Editor and contributor for Pulmonology for e-Medicine Pediatrics published by WebMD.  A recent review article on tracheomalacia was revised along with Emily Concepcion, MD, who is back in Buffalo to complete a one year Sleep Medicine Fellowship.

In May, Jane O’Donnell, PNP, and Marc Leaderstorf, RRT, presented “Discharge Management of Neonates with Bronchopulmonary Dysplasia” at the NICU Perinatal Updates Conference in Buffalo.    

With the assistance of the Cystic Fibrosis research coordinators, Nadine Caci, RNC, CCRC, and Jameelah Ali, RN, CF Therapeutics Development Center, has initiated several new studies, including the Phase III trial of ivacaftor plus lumacaftor, two drugs that act directly on the CF chloride channel in patients who are homozygous for the most common CF mutation and a phase II study of these drugs in patients who are heterozygous for this mutation.  If we demonstrate efficacy and safety, the results of these studies will lead to mutation-specific treatment for over 75 percent of patients with CF. 

Daniel Gelfond, MD,
 (GI) and Drucy Borowitz, MD, are collaborating with the CF Center in Toronto to extend ground-breaking measurements of the extra-pulmonary effects of ivacaftor and continue to serve as national principal investigators for the NIH/CFF-sponsored Baby Observational Study.  Drs. Gelfond and Borowitz have co-authored three published articles this year on gastrointestinal complications of cystic fibrosis.  Dr. Borowitz was invited to lead a symposium and roundtable at the recent European CF Conference in Lisbon, PT.  Dr. Beth Smith (Psychiatry) was invited to participate in an international effort to establish guidelines for the screening and treatment of depression and anxiety in CF. 

Carla Frederick, MD, the CF Adult Program Director, is an editor of the CF Foundation’s on-line resource “For Adults with CF” (www.cff.org) and was selected to present a case at Physicians Grand Rounds at the upcoming North American CF Conference in Salt Lake City.  Support from Loughran’s Restaurant & Grill enables our team to attend this international conference.

The AssistedBreathingCenter continues to work to advance care regionally and nationally for individuals with neuromuscular disease.  In June, Sandy Prentice, RT, Lisa Flattery-Walsh, RT, and Daniel Sheehan, MD, PhD, presented “Inspiring Wellness: Respiratory Care Options in Duchenne Muscular Dystrophy (DMD)” at the Parent Project Muscular Dystrophy (PPMD) Roundtable in Rochester.  Sandy Prentice attended the 2013 Families of Spinal Muscle Atrophy Conference in Anaheim, CA as an invited guest of the local FSMA chapter. 

In July, Dr. Sheehan presented a similar lecture at the 2013 Nemaline Myopathy Forum in St. Catherine’s, Ontario.  As an invited guest of PPMD, Dr. Sheehan participated in a pre-conference meeting on Transition with representatives from the Muscular Dystrophy Association, PPMD, and Muscular Dystrophy Surveillance and Tracking Network (MD STARnet) as well as families and young men with DMD.  With MD STARnet collaborators, Dr. Sheehan co-authored a published article on oral corticosteroids and delayed onset of cardiomyopathy in DMD.

As an American Board of Pediatrics recognized sub-specialty, the Division of Pulmonology is part of UBMD Pediatrics and affiliated with the University at Buffalo School of Medicine and Biomedical Sciences.  Certified by the national Cystic Fibrosis Center as a care, teaching and research center, the CF center of WNY includes both pediatric and adult programs for patients with non-acute respiratory diseases (care is provided to adults by internal medicine-certified specialists). The Center has the only New York State designated Apnea Program in Western New York, providing management for children who are discharged home on oxygen, apnea and bradycardia monitors.

The Cystic Fibrosis Therapeutic Development 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.