Hydroxyurea for treatment of Sickle cell

In Nigeria, 150,000 children are born with Sickle cell every year. Nigeria has the highest rate of sickle cell in the world. There are 20 children born with sickle cell out of 1,000 live births per year.

Sickle cell is a condition that results in sickling of blood cells with resulting health complications. Please read a previous post here to find out more about sickle cell.

Even though the management of sickle cell has improved in high-resource settings, in low-resource settings like Nigeria; sickle cell continues to be a great source of suffering for people affected by this condition.

Study:

In a recent study by Tshilolo et al., they looked at the role of Hydroxyurea in the treatment of sickle cell anemia in children. The study involved 635 children aged from 1 to 10 years who received Hydroxyurea.

Hydroxyurea was first shown to induce fetal hemoglobin production more than 30 years ago. By induction of fetal hemoglobin and other beneficial changes, including mild myelosuppression, hydroxyurea therapy has been shown to have clinical efficacy in reducing the incidence of acute vaso-occlusive events

At the end of the study, they found a significant reduction in of vaso-occlusive pain (98.3 vs. 44.6 events per 100 patient-years; incidence rate ratio, 0.45; 95% confidence interval [CI], 0.37 to 0.56), nonmalaria infection (142.5 vs. 90.0 events per 100 patient-years; incidence rate ratio, 0.62; 95% CI, 0.53 to 0.72), malaria (46.9 vs. 22.9 events per 100 patient-years; incidence rate ratio, 0.49; 95% CI, 0.37 to 0.66), transfusion (43.3 vs. 14.2 events per 100 patient-years; incidence rate ratio, 0.33; 95% CI, 0.23 to 0.47), and death (3.6 vs. 1.1 deaths per 100 patient-years; incidence rate ratio, 0.30; 95% CI, 0.10 to 0.88).

Conclusion:

They concluded that Hydroxyurea treatment was feasible and safe in children with sickle cell anemia living in sub-Saharan Africa.

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Hydroxyurea use reduced the incidence of vaso-occlusive events, infections, malaria, transfusions, and death, which supports the need for wider access to treatment. (Funded by the National Heart, Lung, and Blood Institute and others; REACH ClinicalTrials.gov number, NCT01966731.)

 

Take home points:

Methods to improve outcomes from sickle cell include

  • Early identification of children with sickle cell anemia by means of neonatal screening
  • Comprehensive care
  • Interventions such as penicillin prophylaxis
  • Pneumococcal immunizations
  • Caregiver education
  • Routine transcranial Doppler screening to identify children at risk for stroke
  • Access to safe erythrocyte transfusions
  • Hydroxyurea

 

Reference:
https://www.sciencedirect.com/science/article/pii/S2468124518300251
https://www.nejm.org/doi/full/10.1056/NEJMoa1813598?query=featured_home Retrieved from: DOI: 10.1056/NEJMoa1813598

 

Written by Dr. Ngozi Onuoha.

Photo by Hanna Morris on Unsplash

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