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Sickle Cell Disease in Pregnancy

Sickle Cell Disease in Pregnancy is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Hemoglobin has four heme molecules composed of two α and two β polypeptide chains (1).
  • Sickle cell anemia (SCA) involves formation of abnormal hemoglobin S (Hb S) (1).
  • Sickle cell disease (SCD) is associated with increased maternal and fetal morbidity/mortality related to maternal hemolytic anemia and vaso-occlusive multiorgan dysfunction. Risks include: (1)
    • Spontaneous abortion and intrauterine fetal death (IUFD)
    • Preterm labor and delivery
    • Premature rupture of membranes
    • Need for antepartum hospitalization
    • Intrauterine growth restriction
    • Low birth weight
    • Maternal infection
    • Maternal vaso-occlusive crisis (Acute chest syndrome is most severe.)

Epidemiology

  • SCD is most common in people of African origin but may be seen in Greeks, Italians (Sicilian), Turks, Arabs, Southern Aranians, and Asian Indians in high frequency as well (1).
  • Sickle cell trait is found in 1 in 12 African Americans (1).
  • SCA is found in up to 1 in 600 African Americans (1).
  • Some form of SCD is identified in 1 in every 300 African American newborns in the United States (1).

Etiology and Pathophysiology

  • RBCs with Hb S are more prone to distortion (sickling) with decreased oxygenation.
  • Abnormal RBCs aggregate within organs and small blood vessels.
  • Abnormal RBCs result in increased viscosity, hemolysis, and shorter half-life.

Genetics
  • A single nucleotide substitution of thymine for adenine in the β-globin gene results in a substitution of valine for glutamic acid in the number 6 position of the β-globin polypeptide (1).
  • Carrier has sickle cell trait with heterozygous Hb S, whereas homozygous Hb SS has SCA (1).
  • SCA is inherited in an autosomal recessive form (1).

Risk Factors

Cold temperature, physical exertion, dehydration, and low oxygen tension (high altitude or scuba diving) may precipitate greater sickling (1).

Commonly Associated Conditions

  • Growth and development (1)
    • Retarded growth
    • Skeletal changes
    • Decreased lifespan
  • Sickle cell crisis (1)
    • Painful vaso-occlusive episodes of bones, abdomen, chest, and back
  • Cardiovascular manifestation of hyperdynamic circulation
    • Cardiomegaly
    • Systolic murmurs
    • Failure
  • Pulmonary signs
    • Infection: pneumococcus, mycoplasma, Haemophilus, Salmonella
    • Vascular occlusion
  • Abdominal involvement
    • Painful vaso-occlusive episodes
    • Hepatomegaly
    • Hepatitis
    • Cholecystitis
    • Splenic infarction
  • Bone and joint changes
    • Bone marrow infarction
    • Osteomyelitis: Salmonella
    • Arthritis
  • Genitourinary signs
    • Hyposthenuria
    • Hematuria
    • Pyelonephritis
  • Neurologic manifestations
    • Vascular occlusion
    • Convulsions
    • Hemorrhage
    • Visual disturbances
  • Ocular manifestation
    • Conjunctival vessel change
    • Vitreous hemorrhage
  • In pregnancy, also related to various forms of gestational hypertension and fetal growth restriction (2)

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Citation

Stephens, Mark B., et al., editors. "Sickle Cell Disease in Pregnancy." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688209/all/Sickle_Cell_Disease_in_Pregnancy.
Sickle Cell Disease in Pregnancy. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688209/all/Sickle_Cell_Disease_in_Pregnancy. Accessed April 24, 2019.
Sickle Cell Disease in Pregnancy. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688209/all/Sickle_Cell_Disease_in_Pregnancy
Sickle Cell Disease in Pregnancy [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 24]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688209/all/Sickle_Cell_Disease_in_Pregnancy.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Sickle Cell Disease in Pregnancy ID - 1688209 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688209/all/Sickle_Cell_Disease_in_Pregnancy PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -