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Anemia, Sickle Cell

Joseph P Wiedemer, MD and Smriti Gupta, MD Reviewed 06/2022
 


BASICS

DESCRIPTION

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

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

DIAGNOSIS

HISTORY

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

  • In those >6 months of age, earliest symptoms are irritability and painful swelling of the hands and fe...

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
Onimoe, Grace and Rotz, Seth. Sickle cell disease: A primary care update. Cleveland Clinic Journal of Medicine. 2020. vol87(1): 19-27.

SEE ALSO

Algorithm: Anemia 

CODES

ICD10

  • D57.212 Sickle-cell/Hb-C disease with splenic sequestration

  • D57.211 Sickle-cell/Hb-C disease with acute chest syndrome

  • D57.21 Sickle-cell/Hb-C disease with crisis

  • D57.01 Hb-SS disease with acu...

CLINICAL PEARLS

  • >100,000 Americans have SCA (~1 in 365 African Americans). The majority of newborns with SCD are born in Nigeria, the Republic of Congo, and India.

  • The preferred maintenance IV fluid...

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