Cooperative Study of Sickle Cell Disease (CSSCD)

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Accession Number
HLB00380408a

Study Type
Epidemiology Study

Collection Type
Open BioLINCC Study See bottom of this webpage for request information

Study Period
1977-1995

NHLBI Division
DBDR

Dataset(s) Last Updated
January 4, 2019

Primary Publication URLs
N/A

Consent

Commercial Use Data Restrictions No

Data Restrictions Based On Area Of Research No

Commercial Use Specimen Restrictions No

Non-Genetic Use Specimen Restrictions Based On Area Of Use No

Genetic Use Of Specimens Allowed? Yes

Genetic Use Area Of Research Restrictions No

Specific Consent Restrictions
None.

Objectives

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.

Background

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.

Participants

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.

Design

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.

Disclaimer

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

Additional Details

Subjects:

Newborn with control: 76

Newborn without control: 557

Pediatric <2: 292

Pediatric >=2: 928

Adolescent: 1038

Adult: 1194

Age:
 Newborn with ControlNewborn without Control

Pediatric

< 2

Pediatric >= 2AdolescentAdultAll
N%N%N%N%N%N%N%
076100.00557100.0017158.56......80419.68
1....12141.4490.97....1303.18
2-5......47651.29....47611.65
6-10......44347.7411611.18..55913.68
11-15........54952.89..54913.44
16-20........37335.93826.8745511.14
21-25..........37631.493769.20
26-30..........31926.723197.81
31-35..........18115.161814.43
36-40..........968.04962.35
41-45..........544.52541.32
46-50..........332.76330.81
51-55..........201.68200.49
56-60..........242.01240.59
61-65..........70.5970.17
66-70..........10.0810.02
76-80..........10.0810.02
Sex:
 Newborn with ControlNewborn without Control

Pediatric

< 2

Pediatric >= 2AdolescentAdultAll
N%N%N%N%N%N%N%
Female3647.3726747.9413746.9244047.4151749.8169157.87208851.11
Male4052.6329052.0615553.0848852.5952150.1950342.13199748.89
Race:
 Newborn with ControlNewborn without ControlPediatric < 2Pediatric >= 2AdolescentAdultAll
N%N%N%N%N%N%N%
Missing w/Reason......40.4330.2960.50130.32
Missing w/o Reason..10.18........10.02
Black76100.0052594.2528798.2990297.20101697.88116897.82397497.28
Other..315.5751.71222.37191.83201.68972.37

Please note that biospecimen availability is subject to review by the NHLBI, BioLINCC, and the NHLBI Biorepository. Certain biospecimens may not be made available for your request. Section 3 of the BioLINCC handbook describes the components of the review process

Visits (Vials):

24 July 2023

 SerumDNATotal
Visit 18010801
Visit 23610361
Visit 327027
Unknown139782921

 

Visits (Subjects):

24 July 2023
 

 Serum
Total number of subjectsAverage volume (ml) per subject
Visit 13093.17
Visit 21532.75
Visit 3151.62
Unknown622.64
 DNA
Total number of subjectsAverage mass (µg) per subjectAverage vials per subject
Unknown114196.006.86


 

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Resources Available

Specimens and Study Datasets

Materials Available

Study Documents

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