Hemoglobin S (HbS) polymerization is the root cause
of red blood cell (RBC) sickling in sickle cell disease (SCD)1-4

  • HbS molecules have a lower affinity for oxygen
  • When HbS releases oxygen, it can polymerize into long, rigid rods
  • These long, rigid rods deform the RBCs into sickled RBCs which, in turn, may contribute to other complications of SCD

HbS polymerization leads to RBC sickling and may also contribute to other SCD complications, including organ damage due to1,5-7:

  • Anemia
  • Hemolysis
  • Hemolysis-mediated
    endothelial dysfunction
  • Inflammation
  • Adhesion-mediated
    vaso-occlusion
Potential contribution of HbS polymerization and RBC sickling to the complications of SCD. Potential contribution of HbS polymerization and RBC sickling to the complications of SCD.

Anemia, hemolysis, and vaso-occlusive crises are hallmarks of SCD6,8

Every patient with SCD experiences anemia and hemolysis8

Anemia and hemolysis may have an impact on organ damage and morbidity in
patients with SCD1,9,10

Chronic organ damage:

  • Has emerged as a common cause of death in SCD10-12
  • Progresses slowly in SCD and is associated with chronic anemia and hemolysis9

The extent of the association between chronic organ damage, anemia, and hemolysis is unknown.

Lower hemoglobin (Hb) levels and
anemia in SCD are associated with
increased risk of:

  • Cardiovascular events13
  • Renal damage1,9
  • Multiple organ complications1,9
Heart at risk. Kidneys at risk.

Elevated markers of hemolysis in
SCD are associated with increased
risk of:

  • Stroke or silent cerebral infarct10,14
  • Cardiovascular events13
  • Pulmonary hypertension1,15,16
Brain at risk. Lungs at risk.

Oxbryta is designed to specifically address the root cause of RBC sickling in SCD17

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