Medical College of Wisconsin
CTSICores SearchResearch InformaticsREDCap

Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood. Pediatr Blood Cancer 2018 09;65(9):e27228

Date

05/26/2018

Pubmed ID

29797644

DOI

10.1002/pbc.27228

Scopus ID

2-s2.0-85050383963   26 Citations

Abstract

BACKGROUND: Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease-modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions. To further advance knowledge of SCD, St. Jude Children's Research Hospital established the Sickle Cell Clinical Research and Intervention Program (SCCRIP), to conduct research in a clinically evaluated cohort of individuals with SCD across their lifetime.

PROCEDURES: Initiated in 2014, the SCCRIP study prospectively recruits patients diagnosed with SCD and includes retrospective and longitudinal collection of clinical, neurocognitive, geospatial, psychosocial, and health outcomes data. Biological samples are banked for future genomics and proteomics studies. The organizational structure of SCCRIP is based upon organ/system-specific working groups and is opened to the research community for partnerships.

RESULTS: As of August 2017, 1,044 (92.3% of eligible) patients with SCD have enrolled in the study (860 children and 184 adults), with 11,915 person-years of observation. Population demographics included mean age at last visit of 11.3 years (range 0.7-30.1), 49.8% females, 57.7% treated with hydroxyurea, 8.5% treated with monthly transfusions, and 62.9% hemoglobin (Hb) SS or HbSB0 -thalassemia, 25.7% HbSC, 8.4% HbsB+ -Thalassemia, 1.7% HbS/HPFH, and 1.2% other.

CONCLUSIONS: The SCCRIP cohort will provide a rich resource for the conduct of high impact multidisciplinary research in SCD.

Author List

Hankins JS, Estepp JH, Hodges JR, Villavicencio MA, Robison LL, Weiss MJ, Kang G, Schreiber JE, Porter JS, Kaste SC, Saving KL, Bryant PC, Deyo JE, Nottage KA, King AA, Brandow AM, Lebensburger JD, Adesina O, Chou ST, Zemel BS, Smeltzer MP, Wang WC, Gurney JG

Author

Amanda Brandow DO Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Adult
Anemia, Sickle Cell
Biological Specimen Banks
Blood Transfusion
Body Fluids
Child
Child, Preschool
Disease Progression
Female
Follow-Up Studies
Genotype
Hemoglobinopathies
Humans
Hydroxyurea
Infant
Informed Consent
Longevity
Longitudinal Studies
Male
Patient Selection
Prospective Studies
Research Design
Sampling Studies
United States