Sickle cell disease and the incidence and etiology of preterm birth.

BACKGROUND: Medically indicated delivery can be defined as delivery owing to intervention for maternal or fetal well-being-most commonly because of preeclampsia or nonreassuring fetal status. Among the general population of the United States, approximately two-thirds of preterm deliveries are because of spontaneous labor and/or premature rupture of membranes, whereas the remaining one-third are medically indicated. Despite the increased risk of preterm birth among women with sickle cell disease, the specific etiologies have not been described in the medical literature. Without an understanding of the etiologies of preterm birth in women with sickle cell disease, it is difficult to develop preventative strategies. OBJECTIVE: This study aimed to estimate the incidence and etiologies of preterm births (spontaneous vs medically indicated) in women with sickle cell disease. STUDY DESIGN: This was a retrospective, institutional review board-exempt cohort study of deliveries at >20 weeks' gestation in women with sickle cell disease at Duke University Hospital (2013-2020). We screened pregnancy-linked hospitalizations with International Classification of Diseases-9/10 codes for sickle cell disease (n=373). We excluded cases of pregnancy with <20 weeks' gestation, multiple gestation, or unproven sickle cell disease. We limited inclusion to deliveries within Duke (n=66). We compared the proportion of preterm birth cases between the sickle cell disease cohort and the overall Duke population (n=18,365), and the proportion of spontaneous vs medically indicated preterm births between the sickle cell disease cohort and a racially matched US population. RESULTS: Of the 66 pregnancies, 65 occurred in patients who self-described as Black (98.5%). There were 60.6% (n=40) term and 39.4% (n=26) preterm births vs 85.9% term (n=15,771) and 14.1% preterm (n=2594) births in the Duke population as a whole. The sickle cell disease cohort was nearly 3 times more likely to deliver preterm than the Duke cohort (risk ratio, 2.79; 95% confidence interval, 2.06-3.77; P<.001). Among the 26 preterm births in the sickle cell disease cohort, 30.8% (n=8) were spontaneous and 69.2% (n=18) were medically indicated. In the US Black population comparison cohort, 65.4% (n=392,984) of preterm births were spontaneous and 34.6% (n=207,614) were medically indicated. The sickle cell disease cohort had 2 times the risk of medically indicated preterm birth compared with the US population cohort (risk ratio, 2.00; 95% confidence interval, 1.55-2.59; P<.001). CONCLUSION: Maternal sickle cell disease confers nearly triple the risk of preterm birth, which is twice as likely to be medically indicated.

Parental Notification Via Text Messaging for Infant Sickle Screening Programs: Exploration of Feasibility and Acceptability in Uganda.

Sickle cell disease (SCD) in Africa has high prevalence, morbidity, and early mortality. Difficulties in reaching parents following infant SCD screening dampen program effectiveness. Text messaging may support initial postscreening parental notification. We explored SCD awareness, and feasibility and acceptability of text messaging about screening follow-up among convenience samples of caretakers with children under 5 years (n=115) at 3 sites: a SCD family conference or 2 general pediatric clinics in urban or rural Uganda. Two thirds of the conference-based participants and 8% at clinic sites had affected children. At the clinics, 64% of caretakers were aware of SCD. In all, 87% claimed current possession of mobile phones; 89% previously had received messages. A sample text on the availability of screening results and need to bring their child to SCD clinic was at least partially understood by 82%. Overall, 52% preferred communication for initial follow-up by telephone over text message. Concerns about texting included phone access, privacy or cost, and readability of messages. Caretakers identified concerns about distance, cost, or preference for another clinic as additional barriers to SCD follow-up. Findings suggest that text messaging to caretakers may be feasible, but less acceptable compared with a telephone call about initial follow-up from newborn SCD screening.

Family, Community, and Health System Considerations for Reducing the Burden of Pediatric Sickle Cell Disease in Uganda Through Newborn Screening.

Sickle cell disease (SCD) is associated with high mortality for children under 5 years of age in sub-Saharan Africa. Newborn sickle screening program and enhanced capacity for SCD treatment are under development to reduce disease burden in Uganda and elsewhere in the region. Based on an international stakeholder meeting and a family-directed conference on SCD in Kampala in 2015, and interviews with parents, multinational experts, and other key informants, we describe health care, community, and family perspectives in support of these initiatives. Key stakeholder meetings, discussions, and interviews were held to understand perspectives of public health and multinational leadership, patients and families, as well as national progress, resource needs, medical and social barriers to program success, and resources leveraged from HIV/AIDS. Partnering with program leadership, professionals, patients and families, multinational stakeholders, and leveraging resources from existing programs are needed for building successful programs in Uganda and elsewhere in sub-Saharan Africa.