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Background High rates of unplanned pregnancy and low rates of contraception use are reported among women with sickle cell disease (SCD). Pregnancy in women with SCD is high risk and unintended pregnancies limit opportunities to provide indicated preconception care and counseling. Contraceptive use in women with SCD is complicated by a lack of disease-specific data about treatment risks and benefits. The purpose of this study was to describe, for the first time in the U.S. context, contraceptive use, knowledge and preferences in adult cohort of women with SCD. Materials and Methods A single-center survey study of women with SCD of reproductive age from our Center's adult and pediatric sickle cell centers. Results Seventy-eight women ages 28-65 years (median 33.5 years, IQR 16) completed surveys. Seventy-three percent of respondents had an average of 2.5 pregnancies (S.D. 1.22) and of these, 58% reported being pregnant when they did not want to be pregnant at least once. The most common forms of contraception used were condoms (87%), birth control pills (46%), medroxyprogesterone (44%) and withdrawal (44%). Twenty-two percent of subjects reported using a long-acting reversible form of contraception and 21% reported a tubal ligation or partner vasectomy. Respondents demonstrated low knowledge of the efficacy of contraceptive options and over-estimated the risk of pregnancy with the IUD, implant. Contraceptive priorities included pregnancy prevention, decreasing HIV transmission and effects on SCD symptoms. Conclusions Women with SCD have high rates of unintended pregnancy, low knowledge of contraceptive efficacy and low use of long-acting reversible contraception.

We determine the association between use of specific cephalosporins and macrolides and hospital length of stay in patients with sickle cell disease (SCD) who are admitted with acute chest syndrome, and determine treatment risk factors for acute chest syndrome-related 30-day readmission.Patients admitted to 48 US hospitals within the Pediatric Health Information System between January 2008 and December 2016 with associated International Classification of Diseases, Ninth Revision (ICD-9) or ICD-10 diagnoses of SCD and acute chest syndrome were included. Primary outcomes were hospital length of stay and acute chest syndrome-related and all-cause 30-day readmission. Data were analyzed with t tests, ANOVA, and bivariable and multivariable linear and logistic regressions.In 21,126 visits (representing 8,856 patients), median age was 11.2 years (interquartile range 6.1 to 16.5 years), 53.5% were male patients, and 77.2% had hemoglobin SS genotype. Median length of stay was 4 days (interquartile range 2 to 6 days; mean 4.76 days [SD 4.62 days]). Ceftriaxone alone (length of stay 4.75 days [SD 4.66 days]; P.001) or the combination of ceftriaxone and azithromycin (length of stay 4.84 days [SD 4.74 days]; P.001) was associated with the shortest length of stay and a reduced risk of acute chest syndrome-related readmission (ceftriaxone odds ratio [OR] 0.31; 95% confidence interval [CI] 0.27 to 0.35; ceftriaxone+azithromycin OR 0.20; 95% CI 0.17 to 0.24). Albuterol (OR 0.97; 95% CI 0.96 to 0.98) and RBC transfusion (OR 0.60; 95% CI 0.43 to 0.83) were also associated with decreased rates of acute chest syndrome-related 30-day readmission. All-cause 30-day readmission rate was 16.7% (95% CI 16.2% to 17.3%).Guideline-compliant therapy for acute chest syndrome could preferentially include ceftriaxone and azithromycin. All-cause 30-day readmission for acute chest syndrome is lower than that reported for all-cause readmissions for SCD and more consistent with rates of readmission for pneumonia in the general population.

Background: The creatinine-based CKD EPI equation is the most widely used method to estimate glomerular filtration rate (eGFRcr) in clinical practice. The eGFRcr adjusts for sex, age, and race (Black vs non-Black) and was derived and validated in aggregated datasets from US-based research studies in which GFR was measured directly. However, in prior work, we found eGFRcr performed worse in people living with HIV (PLWH) compared to HIV negative counterparts which may be due to differences in muscle mass, the major source of creatinine. Here, we focus on African American (AA) participants to determine whether the race eGFRcr calibration factor contributes to poor accuracy and bias in AAs living with HIV. Methods: Beginning in 2010, we enrolled and followed an AA cohort of PLWH and HIV-negative participants in Baltimore Maryland. Annually, we measured GFR by iohexol disappearance from plasma (iGFR) and serum concentrations of creatinine and cystatin C. This analysis was restricted to AA participants. We calculated eGFRcr and the creatinine-cystatin C combination equation (eGFRcr-cys) with and without race adjustment. Accuracy was defined as the fraction of eGFR values within +/- 30% of iGFR. Bias was defined as eGFR minus iGFR, with positive and negative values representing overestimation and underestimation of iGFR, respectively. We used multilevel mixed models to account for the within-visit linked structure of the multiple GFR measures, further nested within repeated observations for individuals. We examined the association between lean mass, HIV status, and eGFRcr bias in a subset with body composition measures. Findings: 207 HIV-positive and 107 HIV-negative AA participants contributed 781 and 376 study visits, respectively, with valid measures of iGFR, creatinine, and cystatin C. Among PLWH, omitting the race adjustment (compared with retaining it) changed average eGFRcr bias from 9.1 to -3.9 ml/min/1.73 m2. Moreover, estimation accuracy improved significantly when race adjustment was omitted rather than retained: 86% vs. 78% for eGFRcr (P