Anemia, Sickle Cell

Tipsuda Junsanto-Bahri, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Hereditary, hemoglobinopathy marked by chronic hemolytic anemia, acute episodes of painful “crises,” and increased susceptibility to infections

  • The heterozygous condition (Hb AS), sic...

DIAGNOSIS

Diagnosis is often made by newborn screening programs. 

HISTORY

  • Often asymptomatic in early months of life due to presence of fetal hemoglobin

  • In those >6 months of age, earliest symptoms ar...

TREATMENT

GENERAL MEASURES

  • Painful crises: hydration, analgesics; oxygen regardless of whether the patient is hypoxic

  • Retinal evaluation starting at school age to detect proliferative sickle retinopathy...

ONGOING CARE

FOLLOW UP RECOMMENDATIONS

Patient Monitoring

  • Treat infections early. Parents/patients: Any temperature of ≥101°F (38.3°C) requires immediate medical attention.

  • Monitor for hepatitis C and he...

REFERENCES

1 Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA.  2014; 312(10):1033–1048. ...

ADDITIONAL READING

  • Brousse V, Makani J, Rees DC. Management of sickle cell disease in the community. BMJ.  2014;348:g1765.  [View Abstract]

  • Costa VM, Viana MB, Aguilar RA. Pregnancy in patients w...

SEE ALSO

Algorithm: Anemia 

CODES

ICD10

  • D57.1 Sickle-cell disease without crisis

  • D57.3 Sickle-cell trait

  • D57.00 Hb-SS disease with crisis, unspecified

  • D57.01 Hb-SS disease with acute chest syndrome

  • D57.20 Sickle-cell/Hb-C disease wit...

CLINICAL PEARLS

  • Over 90,000 Americans have SCA (~1 in 375 African Americans).

  • The preferred maintenance IV fluid is 1/2 NS, as NS may theoretically increase the risk of sickling.

  • Painful crises in bones...

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