Blood pressure thresholds for the diagnosis of hypertensive disorders of pregnancy in sickle cell disease.

In this retrospective cohort study of singleton pregnancies in people with sickle cell disease (SCD) delivered at two academic centres between 1990 and 2021, we collected demographic and SCD-related data, pregnancy outcomes, and the highest systolic and diastolic blood pressure (SBP and DBP) at seven time periods. We compared the characteristics of subjects with new or worsening proteinuria (NWP) during pregnancy to those without. We then constructed receiver operating characteristic (ROC) curves to determine the blood pressure (BP) that best identifies those with NWP. The SBP or DBP thresholds which maximized sensitivity and specificity were 120 mmHg SBP (sensitivity: 55.2%, specificity: 73.5%) and 70 mmHg DBP (sensitivity: 27.6%, specificity: 67.7%). The existing BP threshold of 140/90 mmHg lacked sensitivity in both genotype groups (HbSS/HbSß0 : SBP = 21% sensitive, DBP = 5.3% sensitive; HbSS/HbSß+ : SBP = 10% sensitive, DBP = 0% sensitive). Finally, percent change in SBP, DBP and MAP were all poor tests for identifying NWP. Existing BP thresholds used to diagnose hypertensive disorders of pregnancy (HDP) are not sensitive for pregnant people with SCD. For this population, lowering the BP threshold that defines HDP may improve identification of those who need increased observation, consideration of early delivery and eclampsia prophylaxis.

Natural history of blood pressure in sickle cell disease pregnancy.

In this retrospective cohort study of singleton pregnancies in people with sickle cell disease (SCD) delivered at two academic centres between 1990 and 2021, we collected demographic and SCD-related data, pregnancy outcomes, and the highest systolic and diastolic blood pressure (SBP and DBP) at seven time periods. We compared blood pressure values and trajectories in the composite cohort and in each genotype group to control values in a non-SCD pregnancy dataset. There were 290 pregnancies among 197 patients with SCD. Sixteen per cent (n = 47) of pregnancies had a hypertensive disorder of pregnancy (HDP); the rates did not differ by genotype. The mean SBP and DBP were lower in the HbSS/HbSß0 group than in the non-SCD control group at all timepoints. Mean SBP and DBP trajectories were similar between the HbSS/HbSß0 group and non-SCD controls, whereas the mean SBP and DBP in the HbSC/HbSß+ group decreased between the first and second trimesters and plateaued between the second and third trimesters. There were no differences in blood pressure trajectory by haemoglobin >/< 10 gm/dL or by chronic transfusion status. Overall, pregnant people with SCD have lower blood pressure than unaffected pregnant people, raising the possibility that HDP are underdiagnosed, particularly in people with HbSS/HbSß0 .

Family planning needs of young adults with sickle cell disease.

Sexual and reproductive healthcare standards for adolescents and young adults with sickle cell disease (SCD) are not established. A total of 50 young adults entering adult SCD care completed a Family Planning Survey assessing sexual and reproductive health needs from March 2019 to July 2020. Clinical data were abstracted from respondents' electronic medical records. Linear and logistic regression was applied to explore associations between clinical characteristics and survey results. Few respondents (8%) wished to be pregnant in the coming year, and 46% answered yes to at least one of four needs assessment questions. Those who were not employed full time were more likely to endorse needing help with getting sickle cell trait testing for a partner (ORadj = 9.59, p-value = 0.05). Contraceptive use was associated with having an obstetrician-gynecologist (OR = 6.8, p-value = 0.01). Young adults with SCD entering adult care have diverse reproductive health needs, highlighting opportunities to provide multidisciplinary, SCD-specific reproductive healthcare.

Severe Maternal Morbidity and Mortality in Sickle Cell Disease in the National Inpatient Sample, 2012-2018.

Importance: Pregnancy outcomes are historically poor among people with sickle cell disease (SCD) in the US, most of whom have Black race. Whether outcomes have improved is unknown. Objective: To tabulate adverse pregnancy outcomes among patients with SCD, comparing outcomes of deliveries among Black people with SCD with those of Black people without SCD and a control non-Black population, and to measure the association of racial disparities with adverse outcomes in SCD pregnancies. Design, Setting, and Participants: This cross-sectional study was a secondary analysis involving data from National Inpatient Sample, a nationally representative sample of 20% of acute hospital admissions in the US, between 2012 and 2018. The data set included all admissions with codes for delivery of a pregnancy among people aged 11 to 55 years. Data were analyzed from September 2021 to August 2022. Exposures: SCD, racial disparities. Main Outcomes and Measures: Severe maternal morbidity (SMM) as measured by the US Centers for Disease Control and Prevention's index alongside other outcomes; multiple logistic regression was used to compare the odds for adverse pregnancy outcomes. Results: The sample included 5 401 899 deliveries, including 3901 deliveries among people with SCD and 742 164 deliveries among people with Black race. Compared with the non-Black control group, patients with SCD and Black patients were younger (mean [SD] age: SCD, 27.2 [5.9] years; Black, 27.1 [6.1] years vs 28.7 [5.9] years) and more likely to have public insurance (SCD, 2609 deliveries [67.3%]; Black, 496 828 deliveries [65.4%] vs 1 880 198 deliveries [40.8%]). The maternal mortality rate in deliveries among people with SCD was 26 times greater than in the non-Black control group and more than 10 times greater than among Black pregnant people without SCD (Per 10 000 deliveries: SCD 13.3; 95% CI, 5.7-31.2; Black race, 1.2; 95% CI, 1.0-1.5; non-Black control 0.5; 95% CI, 0.5-0.6). Compared with the control group, SCD deliveries had higher odds of SMM (adjusted odds ratio [aOR], 7.22; 95% CI, 6.25-8.34; P < .001), especially cerebrovascular events (aOR, 22.00; 95% CI, 15.25-31.72; P < .001) and thromboembolism (aOR, 17.34; 95% CI, 11.55-26.03; P < .001). Racial disparities explained a median (IQR) 28.9% (21.2%-33.1%) of the increased risk in deliveries to people with SCD and between 40% and 50% of the increased risk for acute kidney failure (excess risk [ER], 56.9%; 95% CI, 54.3%-59.3%), intrauterine fetal demise (ER, 47.8%; 95% CI, 46.6%-49.1%), and eclampsia (ER, 42.1%; 95% CI, 37.9%-46.1%). Conclusions and Relevance: In this large cross-sectional study of pregnancy outcomes in people with SCD, the risk for SMM was higher compared with deliveries among people without SCD, especially for thrombotic events, organ failure, and death. Racial disparities were associated with adverse outcomes. Our findings compel scientific, clinical, and political effort to improve outcomes for pregnant people with SCD.

Comparisons of Severe Maternal Morbidity and Other Adverse Pregnancy Outcomes in Pregnant People With Sickle Cell Disease vs Anemia.

Importance: Pregnancy in sickle cell disease (SCD) is high risk, but whether prenatal anemia, which is treatable with red blood cell transfusions, is a mediator associated with adverse pregnancy outcomes (APOs) is not known. Objective: To compare rates and odds of severe maternal morbidity (SMM) and other APOs in pregnancies among individuals with SCD vs those without SCD but with prenatal anemia. Design, Setting, and Participants: This cross-sectional study was conducted using data from 2012 to 2018 from the National Inpatient Sample, a nationally representative sample of 20% of acute hospital admissions in the United States. All admissions with codes for delivery of a pregnancy among people aged 11 to 55 years were included. Only admissions coded with Black race were included. Data were analyzed from September 2021 through August 2022. Exposures: Prenatal anemia and SCD. Main Outcomes and Measures: SMM was tabulated per the Center for Disease Control and Prevention SMM Index alongside other APOs. Multiple logistic regression was used to compare the odds for APOs and risk ratios (RRs) to compare rates of APOs. Results: Among 764 455 total delivery admissions among patients identified as Black (mean [SD] age at delivery, 27.00 [6.08] years), 3200 deliveries were coded with maternal SCD, 34 808 deliveries were coded with maternal anemia, and 726 447 deliveries were control. Most patients were publicly insured (499 060 [65.4%]). For most outcomes, including SMM and mortality per 10 000 deliveries, the SCD group had higher rates (SMM: 5.9%; 95% CI, 5.1%-6.8%; maternal mortality: 13.0 deaths; 95% CI, 4.9 to 35.0 deaths) than anemia (SMM: 2.1%; 95% CI, 2.0%-2.3%; maternal mortality: 0.9 deaths; 95% CI, 0.3 to 2.8 deaths) or control groups (SMM: 1.1%; 95% CI, 1.0%-1.1%; maternal mortality: 1.2 deaths; 95% CI, 1.0 to 1.5 deaths). SCD (adjusted odds ratio [aOR], 5.51; 95% CI, 4.71-6.45) and anemia groups (aOR, 2.00; 95% CI, 1.84-2.17) had higher adjusted odds of SMM compared with the control group. However, for many complications associated with ischemia or abnormal placentation, CIs of aORs for SCD and anemia groups overlapped (eg, eclampsia: aOR, 2.74; 95% CI, 1.51-4.96 vs aOR, 1.40; 95% CI, 1.08-1.81). For these complications, RRs for SCD vs anemia were between 1.0 and 2.1 (eg, eclampsia: 1.76; 95% CI, 0.93-3.32). For complications associated with thrombosis or SCD-specific pathologies, rates and aORs were greater for the SCD vs anemia group. For these complications, RRs were between 3.70 and 10.90. For example, rates of acute respiratory distress syndrome, including acute chest syndrome, were 56 of 3144 deliveries (1.8%) vs 122 of 34 686 deliveries (0.4%), and the RR was 4.99 (95% CI, 3.65-6.84). Conclusions and Relevance: This study found that risks associated with prenatal anemia and SCD were similar for many APOs, especially those associated with ischemia and abnormal placentation, suggesting that prenatal anemia may be a mediator associated with pregnancy risk in individuals with SCD.

The Montreal cognitive assessment as a cognitive screening tool in sickle cell disease: Associations with clinically significant cognitive domains.

Adults with sickle cell disease (SCD) are at risk for cognitive impairment, which causes significant morbidity. Guidelines support routine cognitive screening, but no screening test is validated in this population. We explored the Montreal Cognitive Assessment (MoCA) as a possible screening test in SCD. We administered the MoCA; a literacy test, the Wide Range Achievement Test, fourth edition (WRAT-4); and a health literacy test, the Shortened Test of Functional Health Literacy in Adults (S-TOFHLA) to adults with SCD and gathered clinical variables through chart review. Spearman's rho, Mann-Whitney, and Kruskal-Wallis tests and quantile regression models were used. Among our sample of 49 adults with SCD, the median MoCA score was 25.0 [interquartile range (IQR) 22.0-28.0]. Higher educational attainment was associated with MoCA scores (p = 0.001). In multivariable models, MoCA scores were associated with S-TOFHLA (p = 0.001) and WRAT-4 Reading (p = 0.002) scores, and overt stroke (p = 0.03) at the median. This pilot study adds to the limited literature of cognitive screening tests in adults with SCD and demonstrates a relationship between MoCA scores and measures of literacy and health literacy. The MoCA is a promising option for briefly screening for cognitive impairment in adults with SCD, though further study is needed to confirm its validity.

Acceptable, hopeful, and useful: development and mixed-method evaluation of an educational tool about reproductive options for people with sickle cell disease or trait.

PURPOSE: People with sickle cell disease (SCD) or trait have many reproductive options, some of which decrease the chance of passing SCD to children, including in vitro fertilization with preimplantation genetic testing (IVF + PGT). Few are aware of these options, and educational materials are needed. This study aimed to develop an accessible, non-directive patient education material about reproductive options for those with SCD or trait via a process that incorporated stakeholders from the SCD community. METHODS: Multidisciplinary stakeholders guided development and revision of a novel pamphlet. Researchers applied health literacy scales to measure pamphlet understandability. We interviewed nine patients with SCD and six multidisciplinary clinicians to evaluate the pamphlet. Interviews were recorded, transcribed, and coded by a five-member team who developed a codebook and proposed themes that were revised by all research team members. Feedback was incorporated into a revised pamphlet. RESULTS: A two-page pamphlet describing reproductive options for people with SCD including IVF + PGT was acceptable to key stakeholders, including people with SCD. Material about this complex topic met health literacy standards, including being written at a 5th grade level. Patients reported feeling hopeful after reviewing the pamphlet, and participants considered the pamphlet useful, clear, and appropriate for distribution in clinics and online. CONCLUSIONS: Though awareness of reproductive options for those with SCD or trait is low, patients and providers find a novel pamphlet about this topic acceptable and useful. Educational materials about complex topics including IVF + PGT can be written at a level understandable to the average American.

Literacy assessment of preimplantation genetic patient education materials exceed national reading levels.

PURPOSE: In vitro fertilization with preimplantation genetic testing (IVF+PGT-M) reduces the risk of having a child affected by a heritable condition, yet only one-third of eligible patients are aware of this reproductive option. Access to education materials written at appropriate literacy levels could raise patients' awareness, but there is a mismatch between patient reading ability and the literacy demand of most materials. This study aimed to systematically identify written education materials on IVF+PGT-M and evaluate their literacy levels. We hypothesized that materials would fail to meet standards set by the Joint Commission and Centers for Disease Control and Prevention (CDC). METHODS: To identify patient education materials about IVF+PGT-M from academic databases and public-facing sources, an environmental scan was performed. Materials were analyzed using three validated scales: Simple Measure of Gobbledygook, Patient Education Materials Assessment Tool, and Clear Communication Index. RESULTS: Seventeen patient education materials about IVF+PGT-M were identified from patient education databases, a consumer search engine, and professional organizations. The median reading level was 14.5 grade, median understandability was 74.2%, and median comprehensibility was 73.3%. CONCLUSIONS: For most American adults, materials about IVF+PGT-M are not readable, understandable, or clear. The Joint Commission requires patient education materials be written at or below 5th grade reading level and the CDC recommends a 90% minimum score for comprehensibility. No evaluated material met these guidelines. Patient education materials that exceed average American literacy skills may perpetuate disparities in the utilization of IVF+PGT-M. Materials that communicate this complicated subject at an understandable level are needed.