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.
university hospital CF center.
Forty-five patients referred for lung transplantation evaluation, and
(2) 178 patients without Burkholderia sp infection, with
the above FEV1 criterion.
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