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Clinical Aspects & Treatment of SCD

Not surprisingly, the major risk factor for early SCD fatality is a high intracellular level of Hb S (and low levels of Hb F)[44]. Hence, the general protocol for managing SCD in patients is to reduce the Hb S level and treat the hypoxemia with oxygen therapy[27]. Standard treatments include periodic blood "exchanges" and transfusions to treat the anemia (low Hb) and transiently replace Hb S with normal adult Hb[27,125]; transfusions are supplemented with iron-chelation therapy to prevent the accumulation of body iron[39].

The more successful treatments of SCD include pharmacological induction of Hb F using butyrate and hydroxyurea[33,46,126]. However, side effects and poor drug efficacy with some of these agents remains as an obstacle for some patients[27]. 

 

Bone Marrow Transplantation

Currently, allogenic bone marrow transplantation (BMT)[127,128] is the only curative regimen for SCD, but is limited to young patients with compatible HLA donors who show limited early organ damage. Still, treatment for SCD is often expensive due to the cost of medication in addition to frequent and extended hospital stays. This places an enormous social burden on both patients and their caretakers[129].  Moreover, notwithstanding BMT, the mean lifespan of treated SCD patients is reduced to ~ 40 years of age[44], and no specific therapy is currently available to treat (or cure) SCD in all  patients.