Anemia, Sickle Cell

Tipsuda Junsanto-Bahri, MD Reviewed 06/2017
 


BASICS

DESCRIPTION

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

  • The heterozygous condition (Hb...

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 1[A]

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

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

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

  • For patients who receive chron...

REFERENCES

Brousse  V, Makani  J, Rees  DC. Management of sickle cell disease in the community. BMJ.  2014;348:g1765. [View Abstract on OvidInsights]
Yawn  BP, Buchanan  GR, Afenyi-Annan  AN, et...

ADDITIONAL READING

  • Aragona E, Kelly MJ. Hyperhemolysis in sickle cell disease. J Pedriatr Hematol Oncol.  2014;36(1):354–356.

  • Cober MP, Phelps SJ. Penicillin prophylaxis in children with sickle ...

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).

  • Painful crises in bones, joints, abdomen, back, and viscera account for 90% of all hospital admissions.

  • Acute chest syndrome...

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