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Study Description

The Cooperative Study of Sickle Cell Disease was initiated in 1977 to determine the natural history of sickle cell disease (SCD) from birth to death in order to identify those factors contributing to the morbidity and mortality of the disease. Specific objectives included: 1) to study the effect of sickle cell disease on growth and development from birth through adolescence 2) to study the conditions or events that may be related to the onset of painful crises 3) to obtain data on the nature, duration, and outcome of major complications of SCD 4) determine the nature, prevalence, and age- related incidence of organ damage due to SCD, and 5) study the role of SCD and its interaction with selected health events.

Phases 2 and 3 of the study involved followup of the infant cohort. A total of 709 infants (age less than 6 months) were enrolled during Phase 1 of the Cooperative Study of Sickle Cell Disease (CSSCD), and Phases 2 and 3 of the CSSCD was designed to follow these children for an additional 10 years. The study objectives included: 1) define prospectively the natural history of sickle cell disease; 2) determine the relationships between cognitive and academic functioning and brain status as determined by MRI; 3) determine the cognitive or behavioral markers of silent infarct; 4) determine the relationship of family functioning on the Family Environment Scale (FES) to brain status, cognitive functioning, and social and demographic factors; 5) continue studies that will enhance the state of knowledge on the influence of sickle cell disease on the psychosocial adjustment of children and adolescents.

Phase 2A of the study sought to examine the progression of organ damage in the heart, lung, kidney, and liver in adult cohort patients (born before 1/1/56) enrolled in phase 1 of the study between 3/79 and 5/81. A total of 620 patients from 11 centers were eligible for phase 2A.

Instructions for requesting individual-level data are available on BioData Catalyst at https://biodatacatalyst.nhlbi.nih.gov/resources/data/. Apply for data access in dbGaP. Upon approval, users may begin accessing requested data in BioData Catalyst. For questions about availability, you may contact the BioData Catalyst team at https://biodatacatalyst.nhlbi.nih.gov/contact.

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Study Inclusion/Exclusion Criteria

CSSCD was a multicenter, prospective study on the natural history of sickle cell disease and participant enrollment into Phase 1 of the CSSCD began in 1978. Participant entry ended in 1981 for all patients greater than six months of age; however, infants continued to be enrolled until 1988. Both mild and hospital-based sickle cell patients were recruited. A total of 4,085 participants, ranging in age from newborns to adults, were enrolled in Phase 1 from 23 centers across the US. Data collection for phase 1 of the CSSCD ended in 1988.


For Phases 2 and 3, 450 were enrolled in the Phase 2 followup along with 17 children that were between 6 and 10 months of age at the time of Phase 1 enrollment. Phase 2 followup concluded in 1994 and 378 patients continued to be followed in the Phase 3 continuation. Data collection ended in 1998.

A total of 359 adult phase 1 participants were enrolled in phase 2A of the study between September of 1989 and July 1991. Exit visits began approximately 2 years later and were concluded in September of 1993.
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Diseases/Traits Related to Study (MeSH terms)
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Study Attribution
  • Principal Investigator
    • Martin Steinberg. Boston University, Boston, MA, USA.