Lung Transplant Referral Consensus Guidelines for Individuals with Cystic Fibrosis: An Opportunity for Partnerships between CF and Lung Transplant Centers

Purpose Nearly 40% of individuals with cystic fibrosis (CF) and decreased FEV1 die without lung transplantation (LTx) in the United States, with an annual mortality rate of 10%. In addition, a substantial proportion of CF individuals with rapidly declining FEV1 (baseline FEV1 >30% predicted) die without being referred for LTx evaluation. The objective of these guidelines is to provide recommendations to the CF community to enhance identification and timely referral of individuals with CF who could benefit from LTx, as well as to encourage partnerships between CF Centers and LTx Centers to improve the referral process. Methods The CF Foundation invited a multidisciplinary team to participate in development of consensus guidelines. Three work groups performed literature searches and developed recommendations related to: understanding the timing for transplant referral; early referral and modifiable barriers; and transition to transplant. The committee voted on draft recommendations with an a priori voting threshold of 80% consensus. As part of the consensus guidelines, a focus group of seven CF transplant recipients and two spouses provided qualitative data on transplant transition to supplement objective criteria. Draft recommendation statements were released for public comment Sept 19, 2018. Results The committee reached 100% consensus on all draft recommendation statements. Key elements include: 1) Earlier introduction of LTx as a potential therapeutic option; 2) Employing overall clinical trajectory to determine the timing of referral in lieu of reliance on FEV1; 3) Early referral for individuals with potentially reversible barriers to LTx; 4) Referral for 2nd opinions for patients declined for transplant; and 5) Improving communication between CF and LTx Centers. Conclusion These guidelines will provide guidance to CF Programs, LTx Centers, and individuals with CF and their families to ensure that potentially eligible individuals receive timely LTx referral. They emphasize the important role that Transplant Centers play in patient education. While these recommendations will inevitably lead to the referral of some individuals who will be “too early” to list, earlier referral is intended to ensure that everyone with CF who has the potential to benefit is given the opportunity to consider LTx. Nearly 40% of individuals with cystic fibrosis (CF) and decreased FEV1 die without lung transplantation (LTx) in the United States, with an annual mortality rate of 10%. In addition, a substantial proportion of CF individuals with rapidly declining FEV1 (baseline FEV1 >30% predicted) die without being referred for LTx evaluation. The objective of these guidelines is to provide recommendations to the CF community to enhance identification and timely referral of individuals with CF who could benefit from LTx, as well as to encourage partnerships between CF Centers and LTx Centers to improve the referral process. The CF Foundation invited a multidisciplinary team to participate in development of consensus guidelines. Three work groups performed literature searches and developed recommendations related to: understanding the timing for transplant referral; early referral and modifiable barriers; and transition to transplant. The committee voted on draft recommendations with an a priori voting threshold of 80% consensus. As part of the consensus guidelines, a focus group of seven CF transplant recipients and two spouses provided qualitative data on transplant transition to supplement objective criteria. Draft recommendation statements were released for public comment Sept 19, 2018. The committee reached 100% consensus on all draft recommendation statements. Key elements include: 1) Earlier introduction of LTx as a potential therapeutic option; 2) Employing overall clinical trajectory to determine the timing of referral in lieu of reliance on FEV1; 3) Early referral for individuals with potentially reversible barriers to LTx; 4) Referral for 2nd opinions for patients declined for transplant; and 5) Improving communication between CF and LTx Centers. These guidelines will provide guidance to CF Programs, LTx Centers, and individuals with CF and their families to ensure that potentially eligible individuals receive timely LTx referral. They emphasize the important role that Transplant Centers play in patient education. While these recommendations will inevitably lead to the referral of some individuals who will be “too early” to list, earlier referral is intended to ensure that everyone with CF who has the potential to benefit is given the opportunity to consider LTx.