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Sickle Cell Disease in Pregnancy

Angelique S. Forrester, MPH, MD, Sarah L. Carroll, DO and Roselyn Jan W. Clemente-Fuentes, MD, FAAFP Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Sickle cell disease (SCD) is an inherited hematologic disorder that results in the deformation of red blood cells (RBCs) into distorted (sickled), rigid shapes that can block or dama...

DIAGNOSIS

HISTORY

  • It is critical to identify signs and symptoms related to hemolysis, vaso-occlusive disease, or infection early to avoid rapid decompensation.

  • Threshold for hospitalization with close m...

TREATMENT

  • Preconception counseling:

    • Women with SCD should seek preconception care to confirm diagnosis and receive counseling on possible pregnancy complications secondary to SCD (3)[C].

    • Offer testing...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Progesterone only and barrier methods recommended for contraception

  • Do not restart hydroxyurea until after breastfeeding completed.

Patient Monitoring

  • Regular screen...

REFERENCES

1
Malinowski AK, Shehata N, D’Souza R, et al. Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis. Blood.  2015;126(21):2424–2437...

ADDITIONAL READING

  • Alayed N, Kezouh A, Oddy L, et al. Sickle cell disease and pregnancy outcomes: population-based study on 8.8 million births. J Perinat Med.  2014;42(4):487–492. [View Abstract ...

CODES

ICD10

  • O99.019 Anemia complicating pregnancy, unspecified trimester

  • D57.1 Sickle-cell disease without crisis

  • D57.3 Sickle-cell trait

ICD9

  • 648.20 Anemia of mother, unspecified as to episode of care or ...

CLINICAL PEARLS

  • Preconception counseling for women with SCD is important.

  • Once pregnant, patient will require close antepartum monitoring.

  • SCD is associated with increased maternal and fetal morbidity/m...

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