Exercise capacity and clinical outcomes in adults followed in the Cooperative Study of Sickle Cell Disease (CSSCD)

Sherif M Badawy*, Amanda B. Payne, Mark J. Rodeghier, Robert I Liem

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

Objectives: To determine the factors associated with exercise capacity in adults with sickle cell disease (SCD) and its relationship to hospitalizations and mortality. Methods: A total of 223 participants in the Cooperative Study of Sickle Cell Disease (CSSCD) (64% female, 70% hemoglobin SS/Sβ0 thalassemia, mean age 43.3 ± 7.5 years) underwent maximal exercise testing using a treadmill protocol with a mean duration of 11.6 ± 5.2 minutes. Results: Female sex (β = −3.34, 95% CI [−1.80, −4.88], P < 0.001), older age (β = −0.14, 95% CI [−0.24, −0.04], P = 0.005), higher body mass index (β = −0.23, 95% CI [−0.37, −0.10]; P = 0.001), and lower hemoglobin (β = 0.56, 95% CI [0.08, 1.04], P = 0.02) were independently associated with lower fitness, while there was a trend with abnormal pulmonary function testing (β = −1.42, 95% CI [−2.92, 0.07]; P = 0.06). Lower percent-predicted forced expiratory volume in 1 second (FEV1) was independently associated with lower fitness (β = 0.08, 95% CI [0.03, 0.13], P = 0.001). Genotype and hospitalization rates for pain and acute chest syndrome (ACS) prior to testing were not associated with exercise capacity. Baseline exercise capacity predicted neither future pain or ACS nor survival in our cohort. Adults with SCD tolerated maximal exercise testing. Conclusions: Prospective studies are needed to further evaluate the impact of regular exercise and improved fitness on clinical outcomes and mortality in SCD.

Original languageEnglish (US)
Pages (from-to)532-541
Number of pages10
JournalEuropean Journal of Haematology
Volume101
Issue number4
DOIs
StatePublished - Oct 1 2018

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Sickle Cell Anemia
Acute Chest Syndrome
Hospitalization
Sickle Hemoglobin
Pain
Thalassemia
Mortality
Forced Expiratory Volume
Hemoglobins
Body Mass Index
Genotype
Prospective Studies
Lung

Keywords

  • acute chest syndrome
  • exercise capacity
  • fitness
  • mortality
  • pain
  • sickle cell disease

ASJC Scopus subject areas

  • Hematology

Cite this

@article{157ad52fae244f78884aded395daa51d,
title = "Exercise capacity and clinical outcomes in adults followed in the Cooperative Study of Sickle Cell Disease (CSSCD)",
abstract = "Objectives: To determine the factors associated with exercise capacity in adults with sickle cell disease (SCD) and its relationship to hospitalizations and mortality. Methods: A total of 223 participants in the Cooperative Study of Sickle Cell Disease (CSSCD) (64{\%} female, 70{\%} hemoglobin SS/Sβ0 thalassemia, mean age 43.3 ± 7.5 years) underwent maximal exercise testing using a treadmill protocol with a mean duration of 11.6 ± 5.2 minutes. Results: Female sex (β = −3.34, 95{\%} CI [−1.80, −4.88], P < 0.001), older age (β = −0.14, 95{\%} CI [−0.24, −0.04], P = 0.005), higher body mass index (β = −0.23, 95{\%} CI [−0.37, −0.10]; P = 0.001), and lower hemoglobin (β = 0.56, 95{\%} CI [0.08, 1.04], P = 0.02) were independently associated with lower fitness, while there was a trend with abnormal pulmonary function testing (β = −1.42, 95{\%} CI [−2.92, 0.07]; P = 0.06). Lower percent-predicted forced expiratory volume in 1 second (FEV1) was independently associated with lower fitness (β = 0.08, 95{\%} CI [0.03, 0.13], P = 0.001). Genotype and hospitalization rates for pain and acute chest syndrome (ACS) prior to testing were not associated with exercise capacity. Baseline exercise capacity predicted neither future pain or ACS nor survival in our cohort. Adults with SCD tolerated maximal exercise testing. Conclusions: Prospective studies are needed to further evaluate the impact of regular exercise and improved fitness on clinical outcomes and mortality in SCD.",
keywords = "acute chest syndrome, exercise capacity, fitness, mortality, pain, sickle cell disease",
author = "Badawy, {Sherif M} and Payne, {Amanda B.} and Rodeghier, {Mark J.} and Liem, {Robert I}",
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}

Exercise capacity and clinical outcomes in adults followed in the Cooperative Study of Sickle Cell Disease (CSSCD). / Badawy, Sherif M; Payne, Amanda B.; Rodeghier, Mark J.; Liem, Robert I.

In: European Journal of Haematology, Vol. 101, No. 4, 01.10.2018, p. 532-541.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Exercise capacity and clinical outcomes in adults followed in the Cooperative Study of Sickle Cell Disease (CSSCD)

AU - Badawy, Sherif M

AU - Payne, Amanda B.

AU - Rodeghier, Mark J.

AU - Liem, Robert I

PY - 2018/10/1

Y1 - 2018/10/1

N2 - Objectives: To determine the factors associated with exercise capacity in adults with sickle cell disease (SCD) and its relationship to hospitalizations and mortality. Methods: A total of 223 participants in the Cooperative Study of Sickle Cell Disease (CSSCD) (64% female, 70% hemoglobin SS/Sβ0 thalassemia, mean age 43.3 ± 7.5 years) underwent maximal exercise testing using a treadmill protocol with a mean duration of 11.6 ± 5.2 minutes. Results: Female sex (β = −3.34, 95% CI [−1.80, −4.88], P < 0.001), older age (β = −0.14, 95% CI [−0.24, −0.04], P = 0.005), higher body mass index (β = −0.23, 95% CI [−0.37, −0.10]; P = 0.001), and lower hemoglobin (β = 0.56, 95% CI [0.08, 1.04], P = 0.02) were independently associated with lower fitness, while there was a trend with abnormal pulmonary function testing (β = −1.42, 95% CI [−2.92, 0.07]; P = 0.06). Lower percent-predicted forced expiratory volume in 1 second (FEV1) was independently associated with lower fitness (β = 0.08, 95% CI [0.03, 0.13], P = 0.001). Genotype and hospitalization rates for pain and acute chest syndrome (ACS) prior to testing were not associated with exercise capacity. Baseline exercise capacity predicted neither future pain or ACS nor survival in our cohort. Adults with SCD tolerated maximal exercise testing. Conclusions: Prospective studies are needed to further evaluate the impact of regular exercise and improved fitness on clinical outcomes and mortality in SCD.

AB - Objectives: To determine the factors associated with exercise capacity in adults with sickle cell disease (SCD) and its relationship to hospitalizations and mortality. Methods: A total of 223 participants in the Cooperative Study of Sickle Cell Disease (CSSCD) (64% female, 70% hemoglobin SS/Sβ0 thalassemia, mean age 43.3 ± 7.5 years) underwent maximal exercise testing using a treadmill protocol with a mean duration of 11.6 ± 5.2 minutes. Results: Female sex (β = −3.34, 95% CI [−1.80, −4.88], P < 0.001), older age (β = −0.14, 95% CI [−0.24, −0.04], P = 0.005), higher body mass index (β = −0.23, 95% CI [−0.37, −0.10]; P = 0.001), and lower hemoglobin (β = 0.56, 95% CI [0.08, 1.04], P = 0.02) were independently associated with lower fitness, while there was a trend with abnormal pulmonary function testing (β = −1.42, 95% CI [−2.92, 0.07]; P = 0.06). Lower percent-predicted forced expiratory volume in 1 second (FEV1) was independently associated with lower fitness (β = 0.08, 95% CI [0.03, 0.13], P = 0.001). Genotype and hospitalization rates for pain and acute chest syndrome (ACS) prior to testing were not associated with exercise capacity. Baseline exercise capacity predicted neither future pain or ACS nor survival in our cohort. Adults with SCD tolerated maximal exercise testing. Conclusions: Prospective studies are needed to further evaluate the impact of regular exercise and improved fitness on clinical outcomes and mortality in SCD.

KW - acute chest syndrome

KW - exercise capacity

KW - fitness

KW - mortality

KW - pain

KW - sickle cell disease

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