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Clinical Investigations: TRANSPLANTATION |

Timing of Referral for Lung Transplantation for Cystic Fibrosis*: Overemphasis on FEV1 May Adversely Affect Overall Survival

Carl F. Doershuk, MD; Robert C. Stern, MD
Author and Funding Information

*From the LeRoy W. Matthews Cystic Fibrosis Center, Department of Pediatrics, Rainbow Babies and Children’s Hospital, University Hospitals of Cleveland, and Case Western Reserve University, Cleveland, OH.



Chest. 1999;115(3):782-787. doi:10.1378/chest.115.3.782
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Study objectives: (1) Report our experience with referral for lung transplantation. (2) Review survival in cystic fibrosis (CF) patients without lung transplantation after FEV1 remains < 30% predicted for ≥ 1 years.

Design: Retrospective review.

Setting: A university hospital CF center.

Patients: (1) Forty-five patients referred for lung transplantation evaluation, 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 transplantation was 55% and 45%, respectively. However, among patients without transplants 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% lived > 6 years). (2) Survival after transplantation was not correlated to any of the following: age, sex, genotype, FEV1 percent predicted, insulin-dependent diabetes mellitus, or with waiting time before transplantation, and did not seem to be correlated to serum bicarbonate or percent ideal body weight. Four of five patients already infected with Burkholderia species died 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 transplantation eligibility could lead to decreased overall survival for CF patients. Referral for evaluation and transplantation should also be based on oxygen requirement, rate of deterioration, respiratory microbiology, quality of life, frequency of IV antibiotic therapy, and other considerations. If pulmonary status has unexpectedly improved when the patient is at or near the top of the waiting list, total survival may be improved by “inactivating the patient” until progression is again evident.

Abbreviations: AF = Aspergillus fumigatus; BC = Burkholderia cepacia; BG = Burkholderia gladioli; BSLT = bilateral single lung transplant; CF = cystic fibrosis; %IBW = percent ideal body weight; IDDM = insulin-dependent diabetes mellitus; PA = Pseudomonas aeruginosa; SA = Staphylococcus aureus

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