Sickle cell disease (SCD) has long been considered a major risk in pregnant women, and in recent decades there has been an abundance of medical literature on how to manage it efficiently. Obstetricians have a well-earned reputation of leaving nothing to chance, so at-risk pregnant women are usually tightly monitored. 

In this article, we will review some of the latest guidelines on the management of SCD in pregnant women. 

Preconception Care 

A team of British researchers has compiled a number of guidelines on managing SCD in pregnancy based on recommendations made by the British Society of Hematology (BSH). In their guidelines, Oteng-Ntim and colleagues made their own series of recommendations based on the best and latest clinical evidence. 

In this care framework, healthcare for women with SCD begins even before conception. This is a broader view of pregnancy care than some physicians are accustomed to taking, but having this framework in mind primes physicians to educate their nonpregnant patients with SCD on what to expect when pregnant. 

They wrote, “Discussion of pregnancy and conception should be embedded into routine care for women with SCD and be part of the comprehensive annual review (at least) from young adulthood onwards and this should begin in pediatric clinics where appropriate.” 

Read more about SCD epidemiology 

In routine check-ups prior to pregnancy, physicians should aim to build rapport and trust so that their patients feel comfortable discussing anything related to their illness, including the desire to become pregnant. Physicians should then offer advice on the kind of support that is available to them. 

This can, for example, include partner testing prior to embarking on pregnancy. This is because a woman with SCD with a partner who is a carrier of the beta-globin variant will have an up to 50% risk of giving birth to a child with a sickling disorder. In addition, high-risk couples should be counseled on their reproductive options. This may include prenatal diagnosis, preimplantation genetic diagnosis, or nonintervention. In addition, at the preconception stage, patients can also discuss with their physicians how to optimize their health prior to conception, such as the taking of folic acid supplements.

Antenatal Care 

One of the most important aspects of antenatal care in pregnant women with SCD is the avoidance of sickle cell crises at all costs. This means avoiding dehydration, overextension, and exposure to extreme temperatures. Sickle cell crises are painful in normal circumstances, but in pregnancy, they may pose an additional risk to the fetus. Also, the evidence on pain management during sickle cell crises in pregnancy is inconclusive. 

Oteng-Ntim and colleagues wrote, “There are no randomized controlled trials examining the management of painful crisis in pregnant women with SCD, so treatment of acute pain in pregnant women should follow national recommendations applicable to non-pregnant women with modifications to account for evidence of safety of specific analgesics for the fetus.” 

There is also debate on the role of prophylactic transfusions during pregnancy. Jain and colleagues, in their published study on SCD and pregnancy, wrote, “Early studies recommended prophylactic transfusion during pregnancy as there was a decrease in maternal morbidity and perinatal mortality among transfused women, but these are not recommended due to risks of alloimmunization, iron overload, transfusion reactions and infections.” 

Read more about SCD treatment 

The American Society of Hematology (ASH) 2020 guidelines for SCD likewise state that there is insufficient evidence to recommend prophylactic blood transfusions as a therapeutic strategy in pregnant women with SCD. However, the group suggests prophylactic transfusions at regular intervals for women with a history of severe SCD-related complications pre-pregnancy and women with additional comorbidities that increase the risk of the pregnancy.

Indeed, the detection of comorbidities that increase the risk of a pregnancy is the main focus of prenatal appointments for women with SCD. Studies indicate that women with SCD are at a higher risk of developing pregnancy-induced hypertension and pre-eclampsia. Hence, BSH guidelines recommend that women with SCD be given aspirin prophylactically unless contraindicated. 

As for the type of blood transfusions during pregnancy, “the ASH guideline panel suggests either red cell exchange with [isovolemic hemodilution (IHD-RCE)] or conventional RCE in patients with SCD (all genotypes) receiving chronic transfusions (conditional recommendation based on very low certainty in the evidence about effects),” the ASH research team wrote. 

Education Is Key 

Pregnant women with SCD should be referred to specialist care, as a variety of scenarios can occur—each requiring emergency care that is evidence-based and highly personalized. 

There is a rather morbid Chinese saying that loosely translates to: “Every pregnancy is a knock on death’s door.” Pregnancy-related deaths are rare in modern medicine, but the number of things that can go wrong (such as pre-eclampsia, infection, fetal problems, and gestational diabetes) is amplified in women with SCD. 

Indeed, care for pregnant women with SCD extends even after childbirth. Oteng-Ntim et al cautioned, “It is important for clinicians to remain vigilant postnatally as the risk of sickle cell crisis remains increased with 21–25% of women having a crisis post-delivery and crisis being more common following general anesthesia.” 

Aside from close monitoring of the woman’s health throughout her pregnancy, the best weapon that obstetricians can offer patients with SCD is education: education on risk factors, comorbidities, warning signs, and the best mode of childbirth. When women with SCD are adequately informed before and during pregnancy, they are best positioned to make sound health decisions for themselves. 

References

Oteng-Ntim E, Pavord S, Howard R, et al. Management of sickle cell disease in pregnancy. A British Society for Haematology Guideline. Br J Haematol. 2021;194(6):980-995. doi:10.1111/bjh.17671

Chou ST, Alsawas M, Fasano RM, et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020;4(2):327-355. doi:10.1182/bloodadvances.2019001143

Jain D, Atmapoojya P, Colah R, Lodha P. Sickle cell disease and pregnancy. Mediterr J Hematol Infect Dis. 2019;11(1):e2019040. doi:10.4084/MJHID.2019.040