Exercise Capacity in Pediatric Sickle Cell Anemia

The Physiologic Assessment of Exercise Capacity in Pediatric Sickle Cell Anemia

The purpose of this study is to use comprehensive exercise testing to examine causes of exercise limitation in children and young adults with sickle cell anemia.

Study Overview

• Status: Completed
• Conditions: Sickle Cell Anemia

Detailed Description

Although the burden of sickle cell anemia (SCA) on affected individuals is significant, few studies have examined the influence of having SCA on such measures of physical function as exercise capacity. Moreover, the physiologic basis of poor physical functioning in children with SCA is unknown and has not been studied extensively. The purpose of this proposal is to use cardiopulmonary exercise testing (CPET) to gain a comprehensive understanding of exercise capacity, as a measure of physical function, in children and young adults with SCA. The specific aims of this project are to: 1) Measure peak oxygen consumption (VO2), the reference standard for exercise capacity, in children and young adults with SCA classified by primary pathophysiologic contributor to their decreased exercise capacity, and 2) Examine the acute inflammatory response, measured by an increase in soluble vascular cell adhesion molecule (sVCAM) activity, in subjects undergoing CPET. These aims will be performed in 60 subjects with SCA and 30 matched controls without SCA. In a secondary analysis, we will also study the impact of baseline exercise capacity and the inflammatory response to exercise on short and long-term disease related morbidity. This study is essential because it will address several areas of exercise capacity, including the physiologic contributors to exercise limitation that remain fundamental knowledge gaps in SCA.

• Study Type: Observational
• Enrollment (Actual): 60

Contacts and Locations

Study Locations

• United States
  • Illinois
    • Chicago, Illinois, United States, 60611
      • Ann & Robert H. Lurie Children's Hospital of Chicago

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 8 years to 21 years (Child, Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Sickle cell anemia patients followed at Children's Memorial Hospital. Healthy controls without sickle cell anemia are recruited through flyers posted in Children's Memorial Hospital.

Description

Inclusion Criteria:

1. age 10 to 21 years old; AND
2. Hb SS or S-β0 thalassemia disease, confirmed by hemoglobin analysis

Exclusion Criteria:

1. inability to perform maximal testing due to physical limitation (e.g. stroke or avascular necrosis); OR
2. history of exercise-induced syncope or arrhythmias. Subjects will wait at least 2 weeks following any vaso-occlusive pain episode and 12 weeks following any disease-related complication requiring transfusion support. Individuals on hydroxyurea will be eligible. A total of 30 controls without SCA or sickle cell trait will be matched for age, sex and race and recruited from the siblings, friends or relatives of subjects enrolled on this study

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
Subjects with Sickle Cell Anemia; Subjects with Sickle Cell Anemia, 10-21 years of age
Healthy controls; Healthy controls, 10 to 21 years of age

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
VO2 max on cardiopulmonary exercise test	Baseline

Secondary Outcome Measures

Outcome Measure	Time Frame
Skin fold measurements to detemine percent body fat	Baseline
All patient reported pain episodes	Every 2 months up to 2 years after baseline
Change in secondary biomarkers in response to exercise test	Baseline (Pre-exercise) and Post-exercise
Change in VCAM level in response to exercise testing	Baseline (pre-exercise) and Post-exercise

Collaborators and Investigators

• Sponsor: Ann & Robert H Lurie Children's Hospital of Chicago
• Collaborators: National Heart, Lung, and Blood Institute (NHLBI)
• Investigators: Principal Investigator: Robert I Liem, MD, MS, Ann & Robert H Lurie Children's Hospital of Chicago

Study record dates

Study Major Dates

• Study Start: June 1, 2009
• Primary Completion (Actual): July 1, 2015
• Study Completion (Actual): July 1, 2015

Study Registration Dates

• First Submitted: January 31, 2012
• First Submitted That Met QC Criteria: February 6, 2012
• First Posted (Estimate): February 7, 2012

Study Record Updates

• Last Update Posted (Estimate): February 4, 2016
• Last Update Submitted That Met QC Criteria: February 3, 2016
• Last Verified: February 1, 2016

More Information

Terms related to this study

• Keywords: Exercise testing; Sickle cell anemia; Cardiopulmonary disease
• Additional Relevant MeSH Terms: Hematologic Diseases; Genetic Diseases, Inborn; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia; Anemia, Sickle Cell
• Other Study ID Numbers: 2009-13659; 1K23HL094376 (U.S. NIH Grant/Contract)