CSSCD Phase 2 and 3
CSSCD Patient Roster
A. Collection Information:
The Roster dataset contains records for 675 patients from 15 CSSCD clinical
centers that participated in the CSSCD Phase 2 infant cohort study. Patients
included in the dataset
1. participated in the CSSCD Phase 1 study (3/79 through 9/88);
2. were enrolled in Phase 1 at < 6 months of age with the exception of 17
patients who were enrolled in Phase 1 between 6 and 10 months of age;
3. were not known to have died prior to the end of Phase1 (9/30/88).
B. Data Collection Period: 09/89-09/98
Eligible patients were enrolled in the CSSCD Phase 2 study between 9/1/89
and 6/30/91. Follow-up information was collected through 3/94. Patients were
enrolled in the Phase 3 study between 4/1/94 and 6/30/95. Follow-up information
was collected through 9/98.
C. Form Versions: Not applicable
D. Files Used to Store Information:
SAS System File: ROST2N3.SD2
Format File: RST.FMT
E. Unique Record Identifiers: ANONID
F. Number of Observations (Patients) in SAS Dataset: 675 (675)
Four hundred sixty-seven patients were enrolled in Phase 2; 378 were enrolled
in Phase 3.
G. Contents of SAS Dataset:

Alphabetical Listing of Variables: See pp. 1-2

Listing of Variables by Position: See p. 3
H. Notes About Selected Variables:

C_HAP5 – is the variable name for S haplotype results based on 5 RFLP sites:
, G, A, , 3’
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CSSCD Phase 2 and 3
CSSCD Patient Roster

C_HAP7 – is the variable name for S haplotype results based on 7 RFLP sites:
, G, A, , 3’, Xmn, and F.
I.
Computed Variables:

AGE_DOE – is the variable name for age at entry into Phase 1. Age was
calculated in days: DOE-BIRTHDAY.

AGE_DOE2 – is the variable name for age at entry into Phase 2. Age was
calculated in years: INT((NERIDOE-BIRTHDAY)/365.25).

AGE_DOE3 – is the variable name for age at entry into Phase 3. Age was
calculated in years: INT((P3DOE-BIRTHDAY)/365.25).

EOF – is the variable name for end of follow-up. For patients enrolled in
Phase3, EOF was computed to be the date of the last required routine physical
exam visit or last special study visit date, whichever was later, or date of death,
if applicable, and death occurred before the last routine visit or special study visit
was due. For patients who were enrolled in Phase 2, but not Phase 3, the end
of follow-up was computed to be 9/30/93 unless
1. the patient died prior to 4/1/94 in which case EOF was computed to be the
date of death, OR
2. the patient was not seen for a routine physical exam or special study after
9/1/92 in which case EOF was computed to be the date the patient was last
seen + 90 days; OR
3. the patient was seen for a routine physical exam or special study between
10/1/93 and 4/1/94 in which case EOF was computed to be the date of the
last visit.

AGE_EOF – is the variable name for age at end of follow-up. Age was
calculated in years: INT((EOF-BIRTHDAY)/365.25).

HEMO – is the variable name for the hemoglobin phenotype diagnosis made by
the CDC during Phase 1. In analyses of HbSS patients, patients with values of
1 (SS) and 5 (SS -thal) should be combined.

HEMO2 – is the variable name for the local hb diagnosis; only patients with a
missing CDC diagnosis and a couple of patients with a CDC diagnosis which
was documented as incorrect by the local institution have a value for this
variable. The value of HEMO2 can be used to “update” the HEMO value to
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CSSCD Phase 2 and 3
CSSCD Patient Roster
reduce the number of patients excluded from analyses because of missing
HEMO information.

F04ALPHA – is the variable name for number of -globin genes. In addition to
S haplotyping, this test was performed by the “CSSCD” Core Laboratory at San
Francisco General Hospital under the direction of Dr. Stephen Embury.
J. Inter-Relationship With Other Datasets:

The dataset PSERA.SD2 includes the following information for all serum
samples collected during Phases 2 and 3: ANONID, SAMPLE_D, SETNO
(sample date sequence #). PSERA.SD2 includes 1133 observations distributed
among 437 patients.
IMPORTANT NOTE: In the frequency codebook that follows, only data for the
467 patients who were enrolled in Phase2 (PHASE2=1) are included in the
tables and summary statistics.
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