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PDF Data Dictionary (PDF - 4.2 MB)
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PDF Phase 1 Study Overview (PDF - 152.2 KB)
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Phase 2a Forms (HTML )

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Cooperative Study of Sickle Cell Disease (CSSCD)

Study Type: Epidemiology Study
Prepared on October 13, 2008
Study Dates: 1997-1998
Consent: Unrestricted Consent
Commercial Use Restrictions: No
NHLBI Division: DBDR
Collection Type: Open BioLINCC Study - See bottom of this webpage for request information


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 include: 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.


Sickle cell disease is a single-gene defect that results in sickle-shaped red blood cells. Although the manifestations of sickle cell disease have been described, variations in the severity and number of manifestations, as well as interactions with other health events, leads to significant gaps in the understanding of the natural history of the disorder. For example, impairments to renal, cardiac, and pulmonary organ function are known to occur in sickle cell patients; however, the descriptions of these outcomes was generally retrospective in nature and occurred when organ damage was severe. In addition, only limited data existed on the social, economic, educational, vocational, and psychological adjustment of patients and families, and as with any chronic disease, impediments to sickle cell patients achieving their educational and vocational goals needs to be elucidated.


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.


In phase 1, four protocols were developed according to participant age: Newborn to six months, pediatric (6 months to 10 years of age), adolescent (age 10-19 years) and adult (age 20+). Protocols were designed to collect similar data at similar times. Participants underwent a baseline exam for assessment of demographics, prior medical history, lab assessments, and clinical data. Post baseline data included routine follow-up examinations, measures of organ damage, and collection of acute and chronic complications.


Only biospecimens from adult subjects who attended the 2a follow-up study are available for request.