Four years have passed since the last time this group of cystic fibrosis (CF) clinicians and researchers last met. They had much to discuss about the changes in the landscape since 2018, beyond even the impact of the COVID-19 pandemic. A far broader range of people diagnosed with CF have seen the approval of more modulator therapies that target their specific cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations, since the first was approved in 2012. These modulators work to control the effects of the mutations, improving lung function and sinus symptoms dramatically, as well as allowing for people with CF to avoid malnourishment and to increase body mass.
More than a hundred attendees from 8 states in the region and 13 medical institutions made the trip to Iowa City from as far away as Oklahoma. Organizers from both Adult and Pediatrics Pulmonary Douglas Hornick, MD; Tahaunty Peña, MD; Brian Wayson, ARNP; Rebecca Weiner, MD; Carrie Huberty, RN; and Kelly Singleton made sure that their travel time was worth it. The first evening offered a social hour and a chance to visit with long-lost friends and colleagues followed by a delicious meal.
After dinner, Hornick welcomed everyone and introduced the evening’s plenary guest, Nicholas Kelly, RD. A registered dietitian and motivational speaker living with CF, Kelly offered inspirational words and a call to action for those assembled. “Use your time wisely this weekend,” Kelly urged. “Let’s make ‘CF’ stand for ‘cure found.'” He also spoke about the disconnect that often occurs between patients and clinicians and shared a personal and moving example of how trust can easily be broken. After Kelly’s presentation and an engaging Q&A, local band The Recliners played a rousing set.
The next morning, attendees were ready to follow Kelly’s advice. Two plenaries in the morning approached the current state of research and therapeutics from two directions. First, JP Clancy, MD, Vice President for Clinical Research with the Cystic Fibrosis Foundation (CFF), gave an overview of why he is optimistic about the search for a cure and where a lot of the basic and translational research is headed, building on modulators’ successes as well as new frontiers the CFF is committed to exploring, like those represented by mRNA and stem cell treatments.
The second plenary offered a more localized and specific form of optimism in the person of Dylan Mortimer, an artist living with CF and recipient of not one but two lung transplants. Over the course of 30 minutes, Mortimer shared examples of his art and its intersection with CF, while also narrating his journey and the insights he has gained. David Stoltz, MD, PhD, Division Director of Pulmonary, Critical Care, and Occupational Medicine revealed in his introduction that one of Mortimer’s works hangs just outside an elevator in the Pappajohn Biodiscovery Building where CF researchers can walk past each day and be reminded of the impact that their work has. Those same researchers signed a print of the Old Capitol as a gift and a thank-you to Mortimer for his contributions.
Over the course of the afternoon, attendees were offered more than a dozen more narrowly focused presentations to attend, some over lunch, others as breakout sessions. At the same time, research center leaders also conferred about ways in which their institutions could foster future collaborations as well as who might host next year’s event.
Conference Sessions
- CFTR & Colorectal Cancer: Implications for Adult CF Care – James Abraham, MD – University of Michigan
- Efficacy of Cyproheptadine for Appetite Stimulation in Pediatric Cystic Fibrosis Patients – Kendall Roessler PharmD; Tara Blesh-Boren PharmD – Children’s Hospital of Omaha
- Effects of CFTR Modulators on CF Disease Progression – Chest CT Findings – Zach Holliday, MD – University of Missouri
- Advanced Lung Disease / Transplant – Tahuanty Peña, MD – University of Iowa
- Are Cystic Fibrosis Carriers at Risk for Cystic Fibrosis-Related Conditions? – Phil Polgreen, MD – University of Iowa
- Transplant Referral – Abby Mack, RN, MNHP; Dani Slaughter, BSN – University of Iowa
- The Lifespan Approach to Mental Health – Leah Colsch LMSW; Erica Gryp LMSW; Emily Mathews LISW, CCTSW-MCS; Jennifer Paternostro PhD; Janet Findlater RN, MA – University of Iowa
- Modulators – Brittany Wright, RPh – University of Iowa
- Impact of Modulators on Pregnant CF Women and Newborn Screening – Rebecca Weiner, MD – University of Iowa
- Nontuberculosis Mycobacteria – Douglas Hornick, MD – University of Iowa
- Impact of Elexacaftor / Tezacaftor / Ivacaftor on Clinical Outcomes in Individuals with CF – Stephanie Duehlmeyer PharmD, BCPPS – Children’s Mercy Kansas City
- Antimicrobial Update: Overcoming Gram Negative Resistance & COVID-19 Antivirals – Heather Bream-Rouwenhorst, PharmD, BCPS – University of Iowa
- Sinusitis Management Consensus – Jarrett Walsh, MD – University of Iowa
- What Does this Staph-positive Culture Mean for My Patient? – Tony Fischer, MD, PhD – University of Iowa
The full group reconvened before the conference’s conclusion to catch James Chmiel, MD, professor and chief of the Division of Pediatric Pulmonary, Allergy, and Sleep Medicine at Indiana University’s School of Medicine. His presentation asked a question that was on the minds of many: “What is the future of anti-inflammatory therapy in the era of highly effective CFTR modulators?” Chmiel addressed in part some of the current challenges of studying a smaller and smaller set of subjects as well as a decreasing lack of interest from pharmaceutical companies and the NIH.
Hornick and Wayson then returned to the stage to thank everyone for attending the long-overdue reunion of friends and colleagues. Hornick expressed his thanks to Wayson and Singleton for their organizational skills in assembling the many parts of the weekend. Wayson offered their assistance to any institution who would like to keep the revived tradition alive for next year.
Institutional affiliation of attendees
Illinois
Lurie Children’s Hospital
OSF Children’s Hospital
Southern Illinois University
Iowa
Blank Children’s CF Center
University of Iowa
Kansas
Children’s Mercy Kansas City
University of Kansas
Minnesota
University of Minnesota
Missouri
University of Missouri
Nebraska
Children’s Hospital of Omaha
Nebraska Medical Center
Oklahoma
University of Oklahoma
South Dakota
Sanford USD Medical Center
Sponsors
Thank you as well to the event sponsors, particularly Vertex Pharmaceuticals, the Platinum Level sponsor for this year.
Abbvie
Alcresta
Chiesi
Genentech
GSK
Hillrom
Insmed
Vertex
Viatris
Vivus