Chest
Volume 113, Issue 5, May 1998, Pages 1230-1234
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Clinical Investigations: Cystic Fibrosis
Risk of Death in Cystic Fibrosis Patients With Severely Compromised Lung Function

https://doi.org/10.1378/chest.113.5.1230Get rights and content

Background

Lung disease accounts for most of the mortality in patients with cystic fibrosis (CF). Lung transplantation is an option for patients severely impaired, being recommended when life expectancy is estimated to be <2 years. Our objectives were to evaluate in our patient population the validity of currently accepted criteria for low life expectancy and to identify other potentially useful criteria.

Methods

Data were retrieved from CF patients followed up at our center who reached and kept an FEV1 <30% predicted. A life table was created and stratified according to characteristics believed to be of importance. In addition, the rate of decline in percent predicted FEV1 was analyzed. These characteristics were evaluated as predictors of risk of death.

Results

The median survival was 3.9 years (95% confidence interval, 2.88 to 4.12 years), with no significant differences according to gender, nutritional status, presence of diabetes, or decade in which the patient was cared for. Only by age was there a significant difference in the median survival (p<0.05). By proportional hazards regression, only the rate of decline in percent predicted FEV1 was a significant predictor of the risk of death, with a borderline effect from younger age (p=0.06).

Conclusion

In our patient population, a cutoff value of FEV1 of <30% predicted is not a reliable predictor of high risk of death within 2 years. The yearly rate of decline of percent predicted FEX1 is a better parameter to identify those patients at high risk for death.

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

REFERENCES (21)

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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.

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