Document Type
Journal Article
Publication Date
11-5-2013
Journal
PLoS ONE
Volume
Volume 8, Issue 11
Issue
11
Inclusive Pages
Article number e79923
Keywords
Anemia, Sickle Cell--mortality; Anemia, Sickle Cell--physiopathology
Abstract
BACKGROUND: Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort.
METHODS: Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED) visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation.
RESULTS: Higher baseline hematocrit (p = 0.0008), ferritin (p = 0.005), and HDL cholesterol (p = 0.01) were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03). Higher tricuspid regurgitatant jet velocity (TRV) (RR 2.41, 95% CI 1.5-3.9, p < 0.0001), elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001) and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001) were also independent risk factors for mortality.
CONCLUSIONS: Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies.
TRIAL REGISTRATION: ClinicalTrials.gov NCT00011648 http://clinicaltrials.gov/
Creative Commons License
This work is licensed under a Creative Commons Attribution 3.0 License.
APA Citation
Darbari, D.S., Wang, Z., Kwak, M., Hildesheim, M., Nichols, J., Allen, D., Seamon, C., Peters-Lawrence, M., Conrey, A., Hall, M.K., Kato, G.J., Taylor, J.G. (2013). Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. PLoS ONE, 8(11):e79923.
Peer Reviewed
1
Open Access
1
Comments
Reproduced with permission of PLoS ONE.