Timing of referral for lung transplantation for cystic fibrosis - Overemphasis on FEV1 may adversely affect overall survival

Study objectives: (1) Report our experience with referral for lung transpla ntation. (2) Review survival in cystic fibrosis (CF) patients without lung transplantation after FEV1 remains < 30% predicted for greater than or equa l to 1 years. Design: Retrospective review. Setting: A university hospital CF center. Patients: (1) Forty-five patients referred for lung transplantation evaluat ion, and (2) 178 patients without Burkholderia sp infection, with the above FEV1 criterion. Main outcome measure: Survival. Measurements and results: (1) One- and 2-year survival after transplantatio n was 55% and 45%, respectively. However, among patients without transplant s with FEV1 < 30% predicted, median survival, 1986 to 1990, ie, before the transplant era, was 4.6 years with 25% living > 9 years (before 1986, 25% l ived > 6 years). (2) Survival after transplantation was not correlated to a ny of the following: age, sea, genotype, FEV1 percent predicted, insulin-de pendent diabetes mellitus, or with waiting time before transplantation, and did not seem to be correlated to serum bicarbonate or percent ideal body w eight. Four of five patients already infected with Burkholderia species die d within 5 months of transplantation; the fifth died at 17 months. All five died of pulmonary or extrapulmonary infection with Burkholderia species Conclusions: Use of FEV1 < 30% predicted to automatically establish transpl antation eligibility could lead to decreased overall survival for CF patien ts. Referral for evaluation and transplantation should also be based on oxy gen requirement, rate of deterioration, respiratory microbiology, quality o f life, frequency of IV antibiotic therapy, and other considerations. If pu lmonary status has unexpectedly improved when the patient is at or near the top of the waiting list, total survival may be improved by "inactivating t he patient" until progression is again evident.