Exercise Capacity in Pediatric Sickle Cell Anemia

Ann & Robert H Lurie Children's Hospital of Chicago

Status: Completed

Conditions: Sickle Cell Anemia

Study type: Observational

Funder types: Other; NIH

Identifiers

NCT01527799

2009-13659

1K23HL094376 (U.S. NIH Grant/Contract)

Details and patient eligibility

About

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.

Full 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.

Enrollment: 60 patients

Sex: All

Ages: 8 to 21 years old

Volunteers: Accepts Healthy Volunteers

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

Trial design

60 participants in 2 patient groups

Subjects with Sickle Cell Anemia

Description:

Subjects with Sickle Cell Anemia, 10-21 years of age

Healthy controls

Description:

Healthy controls, 10 to 21 years of age