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

doi:10.1111/ejh.13140

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

Pediatrics

Research output: Contribution to journal › Article › peer-review

10 Scopus citations

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 language	English (US)
Pages (from-to)	532-541
Number of pages	10
Journal	European Journal of Haematology
Volume	101
Issue number	4
DOIs	https://doi.org/10.1111/ejh.13140
State	Published - Oct 2018

Funding

We thank the National Health, Lung and Blood Institute (NHLBI) Biologic Specimen and Data Repository Information Coordinating Center for providing access to the data from the Cooperative Study of Sickle Cell Disease (CSSCD) Phase 2A. The findings and conclusions in this report are those of the author(s) and do not necessarily represent the official position of the Centers for Disease Control and Prevention. This project was supported by grant number K12HS023011 from the Agency for Healthcare Research and Quality. The content is solely the responsibility of the authors and does not necessarily represent the official views of the Agency for Healthcare Research and Quality.

Keywords

acute chest syndrome
exercise capacity
fitness
mortality
pain
sickle cell disease

ASJC Scopus subject areas

Hematology

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10.1111/ejh.13140

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@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.}",

note = "Funding Information: We thank the National Health, Lung and Blood Institute (NHLBI) Biologic Specimen and Data Repository Information Coordinating Center for providing access to the data from the Cooperative Study of Sickle Cell Disease (CSSCD) Phase 2A. The findings and conclusions in this report are those of the author(s) and do not necessarily represent the official position of the Centers for Disease Control and Prevention. This project was supported by grant number K12HS023011 from the Agency for Healthcare Research and Quality. The content is solely the responsibility of the authors and does not necessarily represent the official views of the Agency for Healthcare Research and Quality. Publisher Copyright: {\textcopyright} 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd",

year = "2018",

month = oct,

doi = "10.1111/ejh.13140",

language = "English (US)",

volume = "101",

pages = "532--541",

journal = "European Journal of Haematology",

issn = "0902-4441",

publisher = "John Wiley and Sons Inc.",

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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.

N1 - Funding Information: We thank the National Health, Lung and Blood Institute (NHLBI) Biologic Specimen and Data Repository Information Coordinating Center for providing access to the data from the Cooperative Study of Sickle Cell Disease (CSSCD) Phase 2A. The findings and conclusions in this report are those of the author(s) and do not necessarily represent the official position of the Centers for Disease Control and Prevention. This project was supported by grant number K12HS023011 from the Agency for Healthcare Research and Quality. The content is solely the responsibility of the authors and does not necessarily represent the official views of the Agency for Healthcare Research and Quality. Publisher Copyright: © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd

PY - 2018/10

Y1 - 2018/10

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

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