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Sickle Cell Disease, Pediatric

Tiffany F. Lin, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

Sickle cell disease (SCD) is a disease caused by a homozygous inheritance of a one base pair change leading to an amino acid change in the beta globin gene at the sixth position: glu...

DIAGNOSIS

HISTORY

  • Diagnosis:

    • In the United States, diagnosed on newborn screening with hemoglobin electrophoresis showing FSA or FS findings

    • Often asymptomatic in early infancy (<6 months) due to prot...

TREATMENT

GENERAL MEASURES

  • Teaching regarding fever precautions (for functional asplenia in SCD patients), signs of acute chest, splenic sequestration monitoring, aplastic crises, stroke identification...

ONGOING CARE

PROGNOSIS

The prognosis for children with SCD has improved dramatically due to hydroxyurea therapy, increased use of blood transfusion with adequate chelation, and screening for stroke ris...

ADDITIONAL READING

  • Adams RJ, Brambilla D; for Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) Trial Investigators. Discontinuing prophylactic transfusions used to prevent stroke in...

CODES

ICD9

  • 282.60 Sickle-cell disease, unspecified

  • 282.5 Sickle-cell trait

  • 282.41 Sickle-cell thalassemia without crisis

  • 282.61 Hb-SS disease without crisis

ICD10

  • D57.1 Sickle-cell disease without crisis

  • D57...

FAQ

  • Q: When can I stop penicillin prophylaxis?

  • A: Most morbidity and mortality from infections in sickle cell anemia occur in the first 5 years of life. The risk of pneumococcal sepsis decreases with a...

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