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Updated: Jul 24, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Sickle Cell Crises in Pregnancy: Fetal and Neonatal Implications
Ashley Appiagyei Cole1,2, Stephen M Wagner1,2, Megha Gupta1,2
1Department of Obstetrics and Gynecology, Beth Israel Deaconess Medical Center, Boston, MA.
Insights
Sickle cell disease (SCD) causes red blood cells to sickle, blocking blood flow and damaging organs. Pregnant individuals with SCD face higher risks of complications for both mother and baby.
Area of Science:
- Hematology
- Genetics
- Reproductive Medicine
Background:
- Sickle cell disease (SCD) is a prevalent inherited blood disorder globally.
- It disproportionately affects individuals of African descent, impacting 100,000 births annually in the U.S.
- SCD involves abnormal hemoglobin leading to sickle-shaped red blood cells under deoxygenation.
Purpose of the Study:
- To review the pathophysiology of sickle cell disease.
- To highlight the increased risks associated with pregnancy in individuals with SCD.
- To underscore the implications for maternal, fetal, and neonatal health outcomes.
Main Methods:
- Literature review of SCD pathophysiology.
- Analysis of epidemiological data on SCD prevalence.
- Synthesis of clinical outcomes in pregnant SCD patients.
Main Results:
- Deoxygenated red blood cells in SCD form a sickle shape.
- This sickling causes vaso-occlusion, leading to ischemia and organ damage.
- Pregnancy exacerbates risks, increasing vaso-occlusive crises and adverse maternal-fetal outcomes.
Conclusions:
- SCD poses significant health challenges due to vaso-occlusion and organ dysfunction.
- Pregnancy in SCD patients necessitates heightened medical surveillance.
- Effective management is crucial to mitigate risks of maternal and neonatal morbidity and mortality.
Abstract:
Sickle cell disease (SCD) is the most common inherited condition worldwide. In the United States, SCD affects 100,000 births every year, mostly persons of African descent. In SCD, red blood cells assume a sickle shape when deoxygenated. This leads to the occlusion of small blood vessels and decreased oxygenated blood flow, resulting in ischemic and thrombotic injury to various organs, which further leads to organ dysfunction. In pregnancy, patients with SCD are at an increased risk of vaso-occlusive crises, which further increases the risks of maternal, fetal, and neonatal morbidity and mortality.
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