Chest
Clinical Investigations: Cystic FibrosisRisk of Death in Cystic Fibrosis Patients With Severely Compromised Lung Function
Section snippets
MATERIALS AND METHODS
Spirometry results, microbiological results, and demographic data from all the patients with CF followed up at our center since 1975 (which is the earliest data in the database) were obtained from the Minnesota Cystic Fibrosis Database.11 Spirometry is routinely performed at our center on all patients at every clinic visit and during hospitalizations. Spirometry is performed following previously described procedures12 and standardized to the predicted norm for sex and height.13 Only patients on
RESULTS
From 1975, we have followed up at our center 635 patients who were able to perform valid spirometry testing and on whom consistent follow-up data were available to the time of their death, lung transplantation, or the time of this analysis. Of this group, 57 patients had died from pulmonary complications of the disease at the time of the analysis.
In this group of 57 deceased patients, and close to the time of their death, 49 (86%) had an FEV1 <30% predicted.
For the 49 patients who had an FEV1
DISCUSSION
Prediction of prognosis and survival in patients with chronic obstructive lung disease, as CF is, has often proved to be difficult because of the variable course of the disease in the individual patient. Many studies have agreed in that physiologic variables such as FEV1 can be important predictors of the risk of death and are strongly related to survival.15, 16, 17, 18, 19 However, specific cutoff points of excess risk found in a population study do not necessarily apply to the individual
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Cited by (154)
The Lung Transplant Candidate, Indications, Timing, and Selection Criteria
2023, Clinics in Chest MedicineConsensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation
2021, Journal of Heart and Lung TransplantationCitation Excerpt :Although mechanistically anti-fibrotic medications could affect wound healing, recent case series have shown no impaired wound or anastomotic healing and no increase in bleeding risk in patients on these medications.240,241 FEV1 has been the best individual predictor of mortality in CF, with studies from the 1990s demonstrating a median survival of 2-4 years after reaching an FEV1 < 30% predicted.242-244 More recent studies have shown improved outcomes in advanced CF lung disease, including an analysis demonstrating a median survival of 6.6 years in patients in the U.S. with FEV1 < 30% predicted.245
Lung Transplantation: Recipient Selection
2021, Encyclopedia of Respiratory Medicine, Second EditionLung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines
2019, Journal of Cystic FibrosisCitation Excerpt :While many studies have demonstrated an association between FEV1 and mortality [12–18], FEV1 is an imperfect marker of disease severity. Survival with low lung function is improving, and some individuals with CF live for many years with severely reduced lung function while others die quickly following a decline in FEV1 [1,12,19,20]. Determining which FEV1 (best, worst, “baseline”, or during an exacerbation) should prompt action is challenging.
Does current reporting of lung function by the UK cystic fibrosis registry allow a fair comparison of adult centres?
2017, Journal of Cystic FibrosisCitation Excerpt :An alternative strategy is to assess rate of decline in lung function for individual patients within a centre. Increased rate of decline in FEV1 is associated with increased mortality [16,21] and rate of decline of FEV1 has been shown to be a better predictor of death than a single value of FEV1 [22]. Hence we chose the longitudinal measure of rate of decline of FEV1% over the course of the study as our alternative outcome measure.
Supported in part by the Annalisa Marzotto Chair in Cystic Fibrosis Care and a Cystic Fibrosis Foundation Center Grant. Presented in part, in abstract form, at the 1994 American Thoracic Society International Conference, Seattle.