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Mortality, Asthma, Smoking and Acute Chest Syndrome in Young Adults with Sickle Cell Disease

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Abstract

Purpose

Sickle cell disease (SCD) patients with asthma have an increased risk of death. Acute chest syndrome (ACS) is a major cause of mortality in patients with SCD, and ACS may be more common in SCD patients who smoke. The purpose of this study was to test the hypothesis that mortality in young adults with SCD would be greater than that of controls during a 10-year period and to determine whether asthma, reduced lung function, ACS episodes, and/or smoking predicted mortality during the follow-up period.

Methods

The outcomes during a 10-year period were ascertained of SCD patients and race-matched controls who had taken part in a pulmonary function study when they were between age 19 and 27 years. Smoking and asthma status and whether they had had ACS episodes were determined, and lung function was measured at the initial assessment.

Results

Seventy-five subjects with SCD were followed for 683 patient years. There were 11 deaths with a mortality rate of 1.6 deaths per 100 patient years, which was higher than that of the controls; one death in 47 controls was observed for 469 patient years with a mortality rate of 0.2 per 100 patient years (p = 0.03). There were no significant associations of body mass index, recurrent episodes of acute chest, steady state haemoglobin, or gender with mortality. Adjusting for baseline lung function in SCD patients, “current” asthma [hazard ratio (HR) 11.2; 95 % confidence interval (CI) 2.5–50.6; p = 0.002] and smoking [HR 2.7; (95 % CI 1.3–5.5); p = 0.006] were significantly associated with mortality during the 10-year period.

Conclusions

Our results indicate that young adults with SCD should be discouraged from smoking and their asthma aggressively treated.

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Acknowledgments

We acknowledge the contribution to this work of Miss Norma Lewis, Research Nurse, who died before the project was completed. We acknowledge the Caribbean Health Research Council and The Principal, Mona Campus, University of the West Indies who funded this work. Dr. Knight-Madden was supported by an MRC Fellowship and the follow-up study was supported by the Caribbean Health Research Council and a New Initiative Grant, University of the West Indies.

Conflict of interest

None.

Author information

Authors and Affiliations

  1. Sickle Cell Unit, Tropical Medicine Research Institute, University of the West Indies, Mona, Kingston 7, Jamaica, West Indies

    Jennifer M. Knight-Madden & Marvin Reid

  2. UWI School of Nursing, University of the West Indies, Mona, Kingston 7, Jamaica, West Indies

    Antoinette Barton-Gooden & Steve R. Weaver

  3. Division of Asthma, Allergy and Lung Biology, King’s College London, London, SE5 9RS, UK

    Anne Greenough

Authors
  1. Jennifer M. Knight-Madden
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  2. Antoinette Barton-Gooden
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  3. Steve R. Weaver
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  4. Marvin Reid
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  5. Anne Greenough
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Corresponding author

Correspondence to Jennifer M. Knight-Madden.

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Knight-Madden, J.M., Barton-Gooden, A., Weaver, S.R. et al. Mortality, Asthma, Smoking and Acute Chest Syndrome in Young Adults with Sickle Cell Disease. Lung 191, 95–100 (2013). https://doi.org/10.1007/s00408-012-9435-3

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  • DOI: https://doi.org/10.1007/s00408-012-9435-3

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