Factors associated with survival in a contemporary adult sickle cell disease cohort

Hany Elmariah, Melanie E. Garrett, Laura M. De Castro, Jude C. Jonassaint, Kenneth Ataga, James R. Eckman, Allison E. Ashley-Koch, Marilyn J. Telen

Research output: Contribution to journalArticle

95 Citations (Scopus)

Abstract

In this study, the relationship of clinical differences among patients with sickle cell disease (SCD) was examined to understand the major contributors to early mortality in a contemporary cohort. Survival data were obtained for 542 adult subjects who were enrolled since 2002 at three university hospitals in the southeast United States. Subjects were followed up for a median of 9.3 years. At enrollment, clinical parameters were collected, including hemoglobin (Hb) genotype, baseline laboratory values, comorbidities, and medication usage. Levels of soluble adhesion molecules were measured for a subset of 87 subjects. The relationship of clinical characteristics to survival was determined using regression analysis. Median age at enrollment was 32 years. Median survival was 61 years for all subjects. Median survival for Hb SS and Sβ0 was 58 years and for Hb SC and Sβ+ was 66 years. Elevated white blood count, lower estimated glomerular filtration rate, proteinuria, frequency of pain crises, pulmonary hypertension, cerebrovascular events, seizures, stroke, sVCAM-1, and short-acting narcotics use were significantly associated with decreased survival. Forty-two percent of subjects were on hydroxyurea therapy, which was not associated with survival. SCD continues to reduce life expectancy for affected individuals, particularly those with Hb Sβ0 and SS. Not only were comorbidities individually associated with decreased survival but also an additive effect was observed, thus, those with a greater number of negative endpoints had worse survival (P<0.0001). The association of higher sVCAM-1 levels with decreased survival suggests that targeted therapies to reduce endothelial damage and inflammation may also be beneficial.

Original languageEnglish (US)
Pages (from-to)530-535
Number of pages6
JournalAmerican Journal of Hematology
Volume89
Issue number5
DOIs
StatePublished - Jan 1 2014
Externally publishedYes

Fingerprint

Sickle Cell Anemia
Survival
Sickle Hemoglobin
Comorbidity
Hydroxyurea
Narcotics
Life Expectancy
Glomerular Filtration Rate
Proteinuria
Pulmonary Hypertension
Hemoglobins
Seizures
Stroke
Genotype
Regression Analysis
Inflammation
Pain
Mortality
Therapeutics

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Elmariah, H., Garrett, M. E., De Castro, L. M., Jonassaint, J. C., Ataga, K., Eckman, J. R., ... Telen, M. J. (2014). Factors associated with survival in a contemporary adult sickle cell disease cohort. American Journal of Hematology, 89(5), 530-535. https://doi.org/10.1002/ajh.23683

Factors associated with survival in a contemporary adult sickle cell disease cohort. / Elmariah, Hany; Garrett, Melanie E.; De Castro, Laura M.; Jonassaint, Jude C.; Ataga, Kenneth; Eckman, James R.; Ashley-Koch, Allison E.; Telen, Marilyn J.

In: American Journal of Hematology, Vol. 89, No. 5, 01.01.2014, p. 530-535.

Research output: Contribution to journalArticle

Elmariah, H, Garrett, ME, De Castro, LM, Jonassaint, JC, Ataga, K, Eckman, JR, Ashley-Koch, AE & Telen, MJ 2014, 'Factors associated with survival in a contemporary adult sickle cell disease cohort', American Journal of Hematology, vol. 89, no. 5, pp. 530-535. https://doi.org/10.1002/ajh.23683
Elmariah, Hany ; Garrett, Melanie E. ; De Castro, Laura M. ; Jonassaint, Jude C. ; Ataga, Kenneth ; Eckman, James R. ; Ashley-Koch, Allison E. ; Telen, Marilyn J. / Factors associated with survival in a contemporary adult sickle cell disease cohort. In: American Journal of Hematology. 2014 ; Vol. 89, No. 5. pp. 530-535.
@article{7c520724a769422e98115d0974bb1daf,
title = "Factors associated with survival in a contemporary adult sickle cell disease cohort",
abstract = "In this study, the relationship of clinical differences among patients with sickle cell disease (SCD) was examined to understand the major contributors to early mortality in a contemporary cohort. Survival data were obtained for 542 adult subjects who were enrolled since 2002 at three university hospitals in the southeast United States. Subjects were followed up for a median of 9.3 years. At enrollment, clinical parameters were collected, including hemoglobin (Hb) genotype, baseline laboratory values, comorbidities, and medication usage. Levels of soluble adhesion molecules were measured for a subset of 87 subjects. The relationship of clinical characteristics to survival was determined using regression analysis. Median age at enrollment was 32 years. Median survival was 61 years for all subjects. Median survival for Hb SS and Sβ0 was 58 years and for Hb SC and Sβ+ was 66 years. Elevated white blood count, lower estimated glomerular filtration rate, proteinuria, frequency of pain crises, pulmonary hypertension, cerebrovascular events, seizures, stroke, sVCAM-1, and short-acting narcotics use were significantly associated with decreased survival. Forty-two percent of subjects were on hydroxyurea therapy, which was not associated with survival. SCD continues to reduce life expectancy for affected individuals, particularly those with Hb Sβ0 and SS. Not only were comorbidities individually associated with decreased survival but also an additive effect was observed, thus, those with a greater number of negative endpoints had worse survival (P<0.0001). The association of higher sVCAM-1 levels with decreased survival suggests that targeted therapies to reduce endothelial damage and inflammation may also be beneficial.",
author = "Hany Elmariah and Garrett, {Melanie E.} and {De Castro}, {Laura M.} and Jonassaint, {Jude C.} and Kenneth Ataga and Eckman, {James R.} and Ashley-Koch, {Allison E.} and Telen, {Marilyn J.}",
year = "2014",
month = "1",
day = "1",
doi = "10.1002/ajh.23683",
language = "English (US)",
volume = "89",
pages = "530--535",
journal = "American Journal of Hematology",
issn = "0361-8609",
publisher = "Wiley-Liss Inc.",
number = "5",

}

TY - JOUR

T1 - Factors associated with survival in a contemporary adult sickle cell disease cohort

AU - Elmariah, Hany

AU - Garrett, Melanie E.

AU - De Castro, Laura M.

AU - Jonassaint, Jude C.

AU - Ataga, Kenneth

AU - Eckman, James R.

AU - Ashley-Koch, Allison E.

AU - Telen, Marilyn J.

PY - 2014/1/1

Y1 - 2014/1/1

N2 - In this study, the relationship of clinical differences among patients with sickle cell disease (SCD) was examined to understand the major contributors to early mortality in a contemporary cohort. Survival data were obtained for 542 adult subjects who were enrolled since 2002 at three university hospitals in the southeast United States. Subjects were followed up for a median of 9.3 years. At enrollment, clinical parameters were collected, including hemoglobin (Hb) genotype, baseline laboratory values, comorbidities, and medication usage. Levels of soluble adhesion molecules were measured for a subset of 87 subjects. The relationship of clinical characteristics to survival was determined using regression analysis. Median age at enrollment was 32 years. Median survival was 61 years for all subjects. Median survival for Hb SS and Sβ0 was 58 years and for Hb SC and Sβ+ was 66 years. Elevated white blood count, lower estimated glomerular filtration rate, proteinuria, frequency of pain crises, pulmonary hypertension, cerebrovascular events, seizures, stroke, sVCAM-1, and short-acting narcotics use were significantly associated with decreased survival. Forty-two percent of subjects were on hydroxyurea therapy, which was not associated with survival. SCD continues to reduce life expectancy for affected individuals, particularly those with Hb Sβ0 and SS. Not only were comorbidities individually associated with decreased survival but also an additive effect was observed, thus, those with a greater number of negative endpoints had worse survival (P<0.0001). The association of higher sVCAM-1 levels with decreased survival suggests that targeted therapies to reduce endothelial damage and inflammation may also be beneficial.

AB - In this study, the relationship of clinical differences among patients with sickle cell disease (SCD) was examined to understand the major contributors to early mortality in a contemporary cohort. Survival data were obtained for 542 adult subjects who were enrolled since 2002 at three university hospitals in the southeast United States. Subjects were followed up for a median of 9.3 years. At enrollment, clinical parameters were collected, including hemoglobin (Hb) genotype, baseline laboratory values, comorbidities, and medication usage. Levels of soluble adhesion molecules were measured for a subset of 87 subjects. The relationship of clinical characteristics to survival was determined using regression analysis. Median age at enrollment was 32 years. Median survival was 61 years for all subjects. Median survival for Hb SS and Sβ0 was 58 years and for Hb SC and Sβ+ was 66 years. Elevated white blood count, lower estimated glomerular filtration rate, proteinuria, frequency of pain crises, pulmonary hypertension, cerebrovascular events, seizures, stroke, sVCAM-1, and short-acting narcotics use were significantly associated with decreased survival. Forty-two percent of subjects were on hydroxyurea therapy, which was not associated with survival. SCD continues to reduce life expectancy for affected individuals, particularly those with Hb Sβ0 and SS. Not only were comorbidities individually associated with decreased survival but also an additive effect was observed, thus, those with a greater number of negative endpoints had worse survival (P<0.0001). The association of higher sVCAM-1 levels with decreased survival suggests that targeted therapies to reduce endothelial damage and inflammation may also be beneficial.

UR - http://www.scopus.com/inward/record.url?scp=84898429927&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84898429927&partnerID=8YFLogxK

U2 - 10.1002/ajh.23683

DO - 10.1002/ajh.23683

M3 - Article

VL - 89

SP - 530

EP - 535

JO - American Journal of Hematology

JF - American Journal of Hematology

SN - 0361-8609

IS - 5

ER -