Shine the light on Sickle cell- Hydroxyurea in Sickle cell disease.

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 June 19 is observed as world Sickle cell disease  and celebrated globally to raise awareness about Sickle cell disease.



The theme  is “Shine the light on Sickle cell”.

In this post let me shine some light on “Hydroxyurea in Sickle cell disease”. 

Properties

Hydroxyurea

 

Drug class

Antimetabolite 

Mechanism of action 

Inhibition of ribonucleotide reductase (RR) 

Increases HbF production

Pharmacology 

Half-life :4 hours 

Potential benefits 

·      Improved survival & increased life expectancy

·      Decreased venoocclusive pain 

·      Decreased Acute coronary syndrome episodes 

·      Decreased transfusions

·      Decreased priapism episodes 

·      Preserve splenic function in infants 

·      Improved quality of life and daily functioning 

 

Beneficial 

In HbSS or HBs/ Beta0 thalassemia genotypes

Standard indications –

Can be given at any age  from 6 month old infants to adults

·       Frequent pain crises

·       Acute chest syndrome

·       Severe or symptomatic anemia (Hb < 7 g/dL)

·       Hemolytic alloantibodies/autoantibodies.

·       Severe complications without benefit from standard therapy (priapism, leg ulceration).

Contraindications

·      Severe bone marrow suppression 

·      Pregnancy

·      Attempted conception

·      Breast feeding 

Discontinued

3 months before attempted conception and during pregnancy


References:

Rohit Kumar Agarwal et al. Hydroxyurea in Sickle cell disease: Drug Review. Indian J Hematol Blood Transfus. 30(2):91-6.

                                                                                                    By Priyavadhana B

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