In sickle cell patients with continued pain crises despite hydroxyurea, how do we sequence the use of newer agents?

As the data supporting the approval of crizanlizumab in NEJM by Ataga et al demonstrated improvement in pain, that is our usual next agent based on the question of pain crises. The approval data around voxelotor related to an increase in hemoglobin of about 1gm/dl, so it may have a different role an...

Hydroxyurea reduces the incidence of acute vaso-occlusive episodes and hemolytic anemia in most adult patients with sickle cell anemia. Nevertheless, the majority will continue to be symptomatic and anemic. This implies the need for additional treatment. Three FDA-approved options are available. Vox...

For younger children in this situation (who typically will not be able to receive crizanlizumab as it isn’t approved for younger patients), l-glutamine would probably be a reasonable next step given the data cited below by Dr. @Dr. First Last.