Introduction: Lung transplantation is the only treatment modality that provides a survival advantage in pulmonary fibrosis. However, many patients deemed suitable will die awaiting lung transplantation. While donor organ shortage undoubtedly contributes to this, late referral to the transplant centre may also play a role. This study investigates factors influencing the chance of patients with pulmonary fibrosis reaching lung transplantation.
Methods: A single centre, retrospective review of patient demographics, assessment investigations and subsequent clinical outcomes for patients with pulmonary fibrosis assessed for lung transplantation over a 5 year period.
Results: Between March 1999 and March 2004, 129 patients with pulmonary fibrosis underwent formal transplant assessment. Sixty-nine were accepted and listed for lung transplantation. Of these, 17 were transplanted, 37 died while waiting, 4 were removed from the list and 11 were still waiting at the conclusion of the study. Waiting time on the list for those transplanted was median (range) 103 (6-904) days compared with 125 (2-547) days among those dying on the list, P=0.65. There was no significant difference in age, spirometry, total lung capacity, gas transfer measures or 6 minute walk distance between those who died waiting and those transplanted. However, time from onset of symptoms to transplant assessment was significantly less among those who died waiting, median (range) 29 (2-120) months compared with those transplanted 46 (6-204) months, P= 0.037.
Conclusion: Patients with pulmonary fibrosis who died awaiting transplantation had similar disease severity at assessment as those who achieved transplantation. However, the interval between symptom onset and transplant referral was significantly shorter in those who died waiting, suggesting they had more rapidly progressive disease. Rate of disease progression appears a more sensitive indicator for transplantation referral than any single physiological measure of disease severity and should act as an important trigger for early transplant referral.
- Lung Transplantation
- Pulmonary Fibrosis