Peripartum considerations in sickle cell disease

Abstract

Purpose of reviewPregnancy exacerbates sickle cell disease (SCD) and is associated with increased frequency and severity of complications resulting in high levels of maternal and fetal morbidity and mortality. We review recent recommendations for managing SCD in pregnancy. Recent findingsAn updated pathobiological model of SCD now attributes the clinical picture to a vicious cycle of four major cellular disturbances. Management decisions should be guided by an understanding of this upgraded model. Red cell transfusions are a key therapeutic intervention used in managing several acute and chronic complications. Transfusion however has significant drawbacks. The American Society of Hematology recently published transfusion guidelines to support care providers. SummaryPatients should be managed by a multidisciplinary and experienced team. The perioperative episode is a recognized period of disease exacerbation and informed anesthetic management can contribute to improved patient outcomes.

Evidence-based management of pregnant women with sickle cell disease in high-income countries

“…Sometimes, either obstet ric related or indeed in countries where malaria is endemic, they could also be due to malaria. 1,18…”

“…The mode of deliv ery depends on obstet ric com pli ca tions; oth er wise, supporting a vag i nal deliv ery or cesar ean incor po rat ing the patient's choice is very per ti nent. 1,14,18 Optimal intrapartum care in the form of good anal ge sia, the avoid ance of dehy dra tion, reg u lar mon i tor ing of oxy gen sat u ra tion, and the avoid ance of protracted labor is recommended. An ante na tal review by the anes the tist to pro vide the appro pri ate plan ning to avoid a gen eral anes thetic is per ti nent to the care of patients with SCD.…”

“…If in an area of low SCD prev a lence, a link to a spe cial ist cen ter or hemo glo bin op a thy coor di nat ing cen ter is per ti nent. 1,14,18,19 It is essen tial to high light the need for welldesigned clin i cal tri als in this area to ascer tain opti mal preg nancy treat ment options (Table 3).…”

A Comprehensive Review of Pregnancy in Sickle Cell Disease

“…SCD is one of the most common inherited genetic and life-threatening disorders affecting more than 6.4 million people worldwide [ 5 ]. Pregnancy exacerbates SCD resulting in severe complications for the mother and fetus and also increases the chances of maternal and fetal death [ 6 ]. There are approximately 6.4 million people living with SCD worldwide (Figure 1 ), among which 0.1 million are in North America, 85,000 in Latin America, more than 5 million in Sub-Saharan Africa, approximately 0.18 million in the Middle East, and more than 1 million in India [ 7 , 8 ].…”