Background
The Cystic Fibrosis Foundation’s commitment to leaving no one in the cystic fibrosis community behind is the driving force for the Cystic Fibrosis Lung Transplant Consortium, which started in 2016. In the era of modulator therapies such as Trikafta, some people with CF may still develop advanced lung disease (ALD). For this reason, we are more committed than ever to advance research and care for those with ALD who have undergone or need to undergo a lung transplant.
Goals
The primary goals of the consortium are to:
- Improve lung transplant outcomes through quality improvement, guidelines development, and research focused primarily on chronic lung allograft dysfunction (CLAD). CLAD is a post-transplant complication related to chronic organ rejection, a primary obstacle to successful and lasting lung transplants.
- Promote interaction between CF and lung transplant physicians to enhance the care of people with CF who have advanced lung disease.
- Provide a forum for exchanging ideas and best practices about the science and art of lung transplantation.
- Establish and maintain a database of CF lung transplant recipients and support the collection of lung tissue samples for research.
Consortium Sites
The 14 consortium sites are:
- The Cleveland Clinic
- Duke University
- Johns Hopkins University
- Loyola University
- University of Minnesota
- University of Pennsylvania
- University of Pittsburgh
- University of Toronto
- University of Washington in Seattle
- Washington University in St. Louis
- University of California, Los Angeles
- University of California, San Francisco
- Massachusetts General
- Stanford University
Research
The Cystic Fibrosis Lung Transplant Consortium Biorepository and Registry stores lung tissue samples and clinical data related to transplant care. Tissue samples and data from post-transplant clinic visits are collected as part of a study. Soon, researchers will be able to access these samples and data, as well as others to support research into CLAD. In the future, this data will be linked to other research databases such as the CF Foundation’s Patient Registry. This will allow clinicians and researchers to understand the long-term outcomes of people with CF who have undergone a lung transplant.
The Airway Brush in CLAD (ABC) study is another study that utilizes the Biorepository and Registry infrastructure. ABC, which began enrollment in early 2024, examines whether certain cells in the lung can be used to identify the beginning stages of CLAD and help determine important targets for early intervention.
Clinical
Many of the clinicians involved in the consortium have participated in the development of care guidelines and resources for people with CF who have undergone a lung transplant. Guidelines highlight best clinical practices based on research and clinical outcomes and are used to provide the best clinical care possible to people with CF. Individuals with CF make up a small portion of the total population of lung transplant recipients in the U.S. but providing quality individualized care for specific CF needs is vital. These guidelines help support clinicians so that people with CF and their families can be informed advocates of their own care.
These guidelines and resources include:
- Clinical care consensus statements for the care of CF lung transplant recipients to standardize post-transplant care
- A position paper that outlines two models of post-lung transplant care for optimal CF care after transplant.
- A post-transplant care checklist that outlines the elements of high-quality care for the CF lung transplant recipient. The checklist was based on the two models of care described in the position paper. Clinicians can use the checklist to guide and track post-lung transplant care. This tool can also benefit lung-transplant recipients and their families by increasing their awareness of long-term post-transplant needs.
All consortium sites have been involved in quality improvement efforts between transplant centers and CF care centers. These efforts optimize care of individuals with CF before and after transplant, including transplant referrals, the transplant process, and care after transplant.
Community Involvement
We are honored to have four community members who have undergone one or more lung transplants on the consortium steering committee. These individuals help guide the consortium’s work by sharing their experiences, providing context to the research and clinical work discussed. Community members are encouraged to share their concerns, along with concerns they are hearing from the community that the consortium may not be addressing. The CF lung transplant community and individuals with advanced CF lung disease are at the heart of the consortium’s work.
The consortium engages the CF community at virtual Foundation events such as BreatheCon. At these events, consortium members answer questions from the community and share information that people with CF can use to advocate for themselves in their care before and after transplant.
We are fortunate to have the following community members as the current representatives:
| Marcia Freeman Adult With CF Marcia lives in St. Louis with her husband of 23 years. They have two 20-year-old children who are out in the world on their own. She works at a small office as a billing specialist and project manager. Marcia has had two double-lung transplants — one in 2013 and another in 2016. She volunteers at the local organ procurement organization and her church, and enjoys reading, music, and exercising in her spare time. |
Ian Tisdale Adult With CF Ian Tisdale was diagnosed with CF when he was 3 months old and since then has been treated by the medical team at Johns Hopkins. In 2017, Ian received a double-lung transplant and has never felt better. He enjoys running several times per week and lives with his wonderful wife and amazing 7-year-old son in northern Virginia. | |
| Fanny Vlahos Adult With CF Fanny received a double-lung transplant in 2012. She has been involved with the CF Foundation for many years both at the national and state levels. Fanny earned Volunteer of the Year in 2019 and has accumulated several other awards and commendations for her dedication and focus on patient advocacy. She lives in the Chicago area with her husband, their teenage son, and their rescue pit bull. |
| Willem Wery Willem was diagnosed with CF at age 8 and received a double-lung transplant in 2019. He and his wife, Kathy, have two adult sons, a daughter-in-law, and four grandchildren. Willem has returned to an active lifestyle and is very engaged with his Oregon Health & Science University clinic and the CF community. He brings the perspective of a person with CF, a transplant recipient, a parent, and a working professional. A video about Willem’s lung transplant journey is available on YouTube. |
We want to hear directly from the community. If you have any questions or comments, please email lungtransplant@cff.org.
Annual Report
To learn more about the work of the Advanced Lung Disease and Lung Transplant Program at the CF Foundation, please see the most recent version of the program’s annual report:
