Access to patient-centered medical homes in children with sickle cell disease.

To determine the proportion of children with sickle cell disease (SCD) followed in a subspecialty clinic with access to a primary care provider (PCP) exhibiting practice-level qualities of a patient-centered medical home (PCMH). We surveyed 200 parents/guardians of children with SCD using a 44-item tool addressing PCP access, caregiver attitudes toward PCPs, barriers to healthcare utilization, perceived disease severity, and satisfaction with care received in the PCP versus SCD clinic settings. Individual PCMH criteria measured were a personal provider relationship and medical care characterized as accessible, comprehensive and coordinated. Although 94 % of respondents reported a PCP for their child, there was greater variation in the proportion of PCPs who met other individual PCMH criteria. A higher proportion of PCPs met criteria for coordinated care when compared to accessible or comprehensive care. In multivariate models, transportation availability, lower ER visit frequency and greater PCP visit frequency were associated favorably with having a PCP meeting criteria for accessible and coordinated care. Child and respondent demographics and disease severity had no impact on PCMH designation. Average respondent satisfaction scores for the SCD clinic was higher, when compared to satisfaction scores for the PCP. For children with SCD, access to a PCP is not synonymous with access to a medical home. While specific factors associated with PCMH access may be identified in children with SCD, their cause and effect relationships need further study.

Alloimmunization in sickle cell anemia in the era of extended red cell typing.

BACKGROUND: Red blood cell (RBC) transfusion remains an essential part of the management of patients with sickle cell disease (SCD). Alloimmunization is a major complication of transfusions. Extended RBC typing is advocated as a means to reduce alloimmunization in SCD. Our goal was to assess alloimmunization among individuals with SCD at our center since implementing extended RBC typing. MATERIALS AND METHODS: We reviewed electronic medical records of all patients with SCD (N = 641) in our comprehensive SCD Program to determine transfusion histories. Cross-referencing with our blood bank database, we extracted data such as antibodies identified, detection date and genotyping in specific cases. Transfusion sources were determined for those with C, E, and Kell antibodies. RESULTS: Of 180 patients transfused from 2002 to 2011, 26 developed at least one new antibody. The majority of alloimmunized patients (14/26) received episodic transfusions only. The most common antibodies formed were against C and E antigens. Of the 16 patients who developed C, E, Kell antibodies, nine had one or more documented transfusions at an outside hospital. Five patients had Rh variants undetectable on routine phenotyping including two novel e alleles related to ceAR and ce(S)(733G). CONCLUSION: Despite extended RBC typing, alloimmunization may still occur due to RBC variants that are not detected on routine screening and transfusions at institutions where extended RBC typing is not done. Extended RBC typing should be the standard of care for patients with SCD. Prospective genotyping may reduce allosensitization to rare variants not detected on routine screening.