Background Nocturnal enuresis is a common symptom in children with sickle cell disease (SCD). Risk factors for development of enuresis are currently unknown. An early manifestation of SCD-associated kidney damage is glomerular hyperfiltration. We test the hypothesis that in a pediatric SCD cohort, individuals with hyperfiltration are more likely to have nocturnal enuresis when compared to children without hyperfiltration. Procedures To assess the relationship between nocturnal enuresis and hyperfiltration, we retrospectively evaluated children with SCD enrolled in the Evaluation of Nocturnal Enuresis and Barriers to Treatment among Pediatric Patients with SCD study (PEESC; NCT01959958) and prospectively identified children who reported nocturnal enuresis and were enrolled in the longitudinal cohort study Sickle Cell Clinical Research and Intervention Program (SCCRIP, NCT02098863). Results Nocturnal enuresis occurred in 46.5% of PEESC participants and was more frequent in participants with HbSS/HbSβ0-thalassemia and in male participants. We did not identify an association between hyperfiltration from three to five years of age with the later development of enuresis. Hyposthenuria was not associated with enuresis. Conclusions Severe SCD genotypes and male sex were associated with nocturnal enuresis after age 5 years. However, we could not identify additional renal or hematologic predictors associated with the diagnosis of nocturnal enuresis. Future studies should incorporate non-renal risk factors into studies that predict development of enuresis.

Background It is important to ensure access to hydroxyurea (HU) for patients with sickle cell anemia (SCA) living in rural areas without easy access to experts in sickle cell management. The UAB Pediatric Sickle Cell program’s satellite clinics reduce the barrier of transportation to the university-based clinic. However, as compared to the university clinic, these satellite clinics do not offer immediate access to HU dosing laboratory results. Therefore, a nurse clinician calls families with HU dose adjustments after the completion of the clinic visit. This study evaluated the impact of telehealth dosing adjustments on HU laboratory and clinical response as compared to university-based patients. Methods A one year retrospective chart reviewed was performed to evaluate HU laboratory and clinical response based on clinic location and socioeconomic status for patients with SCA. We identified the number of clinic and acute care visits for one year and calculated the mean CBC and HbF values for each patient. Results We identified 107 academic center participants with SCA prescribed HU and 65 satellite clinic participants. We identified no difference in HbF, Hb, MCV, or ANC by clinic location. We also identified no difference in hospital admissions based on clinic location. Finally, mean socioeconomic indicators by zip code were lower in satellite clinic patients but not associated with a difference in HbF response. Conclusions The use of telehealth did not negatively impact laboratory response to HU. Future studies should identify novel approaches to improve access to HU among patients with SCA living in rural areas.

Background: Acute chest syndrome (ACS) is the leading cause of death for children with sickle cell disease (SCD). Recurrent ACS has detrimental effects on pulmonary health and healthcare costs. Neighborhood characteristics affect the outcomes of many pediatric chronic diseases, but their role in SCD is not well investigated. In this study, we investigated the effects of area-level socioeconomic deprivation and racial composition on the recurrence of ACS. Study design: We performed a retrospective cross-sectional analysis of clinical data from a large pediatric SCD center. Patients’ residential addresses were geocoded and linked to a composite Area Deprivation Index (ADI) and percent African American population at the level of Census block groups. The association of recurrent ACS with neighborhood characteristics was evaluated using logistic regression analysis. Results: The sample included 709 children with SCD. Residence in a socioeconomically deprived neighborhood was associated with 27% less risk of recurrent ACS, and residence in a predominantly African American neighborhood was associated with 41% less risk of ACS recurrence. The racial composition explained the protective effect of living in a high-deprivation area after adjusting for sociodemographic and clinical covariates. Demographic and clinical factors associated with recurrent ACS included older age, male gender, asthma, hydroxyurea use, and chronic transfusion therapy. Conclusions: This is the first study to report a protective effect of residing in a predominantly African American community for ACS recurrence. Further prospective studies are needed to confirm the association and to understand the mechanisms of such relationship.