Collaborative Data Project [C-DATA] of the Comprehensive Sickle Cell Centers Program.

The C-Data Project of the NIH-NHLBI funded Comprehensive Sickle Cell Centers Program was designed to establish a large and geographically diverse patient registry of persons with sickle cell disease regularly followed in the 10 comprehensive sickle cell centers. Opened to enrollment in March 2005 the database now contains current demographic, clinical, and health related quality of life information on 1,673 participants. Retrospective patient data are abstracted from the medical record at enrollment and updated from both patient interviews and medical record review at semi-annual contacts. Common sickle cell clinical events are defined in the context of modern diagnostic criteria so that as prospective data collection continues an unparalleled database of the burden of disease sickle cell syndromes may cause will emerge. While data are preliminary, interesting descriptive statistics have been derived. As of June 2006, 69% of participants are ≤17 yrs of age [peds], 63% have HbSS, 50% are female, and over half have been followed for 5 or more years in the same center. At least one episode of acute chest syndrome was reported by 56% of all subjects (51% peds, 76% adults) and of painful crisis by 69% (62% peds, 94% adults). CNS events occurred in 18% including ischemic stroke in 6% of peds and 11% of adults. Cholecystectomy was the most common surgical procedure reported in 19% (10% peds, 53% adults). Splenectomy was reported in 11% (10% peds, 16% adults), tonsillectomy in 12% (10% peds, 18% adults), and permanent central lines had been placed in 7% (5% peds, 15% adults). Adults reported acute and chronic renal failure in 6% and 3% respectively, and proteinuria in 11%. Transfusion during the past year was reported by 32%, and 54% of patients have received at least one transfusion during their lifetime. Iron overload was reported by 7% (5% peds, 14% adults). One or more hospitalizations within the last two years were reported by 62% (60% peds, 71% adults). Admissions were primarily for management of painful crisis in 39%, fever in 16%, and acute chest syndrome in 10%. Within the last year an MRI of the brain was performed in 16% and transcranial doppler (TCD) in 23% of pediatric patients. A cardiac echo was performed within the last year on 33% of adults. Hydroxyurea, the chemotherapy agent recommended for use by severely involved patients, was prescribed for 23% (18% peds, 41% adults). Twice daily prophylactic penicillin use in the last year was reported by 67% peds and 25% adults. At least 19% of patients had participated in at least one other research study. This database will be an invaluable tool for assessment of adherence to recommendations for care, heath services planning, and outcomes assessment. Year 1 data have demonstrated that CDATA will be able to facilitate the planning of multi-center research studies, provide a mechanism for identifying specific individuals within a center who are potentially eligible for participation in intervention studies, and provide the clinical data to support DNA based genotype-phenotype assessments.