Testing should also include a pregnancy test for postmenarchal females because hydroxyurea is in US Food and Drug Administration pregnancy risk category X.
Avoid hydroxyurea in lactating women; dose the drug renally in the setting of renal disease. Because hydroxyurea has a high rate of serious adverse effects and drug-drug interactions, it should be offered in conjunction with an individualized care plan.
Hydroxyurea can also be offered to patients with other forms of SCD who have recurrent vaso-occlusive symptoms.
Two newer medicationsimprove oxygen delivery in patients with SCD. Voxelotor, approved in 2019, works to reduce Hb S polymerization by binding to the alpha chain of Hb S and, subsequently, increasing its oxygen affinity. The drug is generally well tolerated and can be used in patients with SCD who are ≥ 12 years.16 Crizanlizumab is a monoclonal antibody directed against P-selectin, an adhesion molecule located on endothelial cells and activated platelets. The efficacy of crizanlizumab was demonstrated in the SUSTAIN trial, in which it reduced vaso-occlusive pain in patients ≥ 16 years.17
All of these medications have a narrow toxic–therapeutic window. They should therefore be administered with the participation of a multidisciplinary care team.
Continue to: The need to coordinated, comprehensive care
