The 45th Kit Taylor Memorial Lecture was a mix of the old and the new.
The old: continuing the tradition of bringing a nationally recognized expert to increase our understanding of CF care. This year’s guest lecturer was Dr. Michael Narkewicz, Professor of Pediatrics at the University of Colorado School of Medicine. He is recognized for his expertise in pediatric hepatology, liver transplantation and cystic fibrosis liver disease. Dr. Narkewicz has been an important leader in advancing GI care for people with CF and has been pivotal in providing mentorship for other CF care center providers around the world.
The new: adjusting the format of the lecture to encourage greater involvement. The community event, held at Sager-Stoneyard Pub, mixed a brief presentation by Dr. Narkewicz with plenty of time for casual conversations with Dr. Narkewicz, CF Center staff and community members. Dr. Narkewicz gave a more detailed presentation, “Cystic Fibrosis Liver Disease,” at Pediatric Grand Rounds which was made available to community members through Zoom.
The information shared through this year’s presentations lived up to the high standards expected from the Kit Taylor lectureship. A recording of the Pediatric Grand Rounds presentation has been made available by URMC. A few highlights from both sessions are presented below.
In opening the evening session, Dr. Narkewicz explained the importance of using the term “liver involvement” for the effects of CF on liver function before the symptoms elevate to “liver disease.” This led to noting the current effort to update the guidelines for liver care in the CF population including improvements in screening and monitoring. Dr. Narkewicz is a key player in advancing scientific knowledge regarding the etiology of liver involvement and developing predictive metrics to help stratify risk.
Historically liver involvement was thought to begin early on, with most cases identified in preteens. Until recently, people living with CF who did not have liver involvement as a child were not likely to develop liver complications later in life. However, with the aging of the CF population, liver involvement is appearing in older individuals.
The CFTR protein is present in the biliary ducts and CF mutations interfere with proper bile flow which can result in liver injury. Research to date indicates that CFTR modulators do not produce improvements in liver function; however, studies will be continued to learn if extended use of modulators has an impact. Research also continues to develop other therapies to address the effects of liver involvement, such as medicine to reduce scarring of the liver. The interactions with other parts of the gastrointestinal system are also being studied.
With the successes in treating lung disease, greater attention is being given to other organs including the liver. The Cystic Fibrosis Foundation has created the Developing Innovative Gastroenterology Specialty Training (DIGEST) Program. Through this 3-year program, Dr. Narkewicz is one of the physicians mentoring GI clinicians to increase the understanding of gastrointestinal and liver needs unique to the CF care community. This year’s Kit Taylor Lecture is also part of that effort. Dr. Gabel, a pediatric care team member, participated in the DIGEST program from 2016-2019 and continues to provide care and education locally.
Much more was discussed at both Kit Taylor events. We encourage you to check out the recording of the Pediatric Grand Rounds.