Is Vitamin C Supplementation in Patients withβ-Thalassemia Major Beneficial or Detrimental?

Abstract

Globally, β-thalassemia major (β-TM) is one of the most common hereditary disorders. Multiple blood transfusions, that are a life-saving therapy in patients with β-TM, is a major source of iron overload. Iron overload can lead to significant morbidity and mortality. Research evidence indicates that oxidative stress induced by iron overload, is one of the major precipitating causes of vitamin C deficiency in β-TM patients. It has previously been shown that patients with β-TM have significantly lower levels of vitamin C as compared to healthy individuals. It is believed that vitamin C can reduce both ferric (Fe(3+)) and ferrous (Fe(2+)) ions, and also facilitate the accessibility of iron to chelators through increase of iron release from the reticuloendothelial system. Despite the potential benefits of vitamin C in patients with β-TM, several areas of concern exist that should be addressed by high quality research designs. Some recommendations have been provided through this study.

Exploring Iranian β-Thalassemia major patients’ perception of barriers and facilitators of adherence to treatment: A qualitative study

“…β-Thalassemia Major (β-TM) is the most commonly inherited hemolytic anemia. Patients with β-TM require lifelong adherence to distressing treatment regimen, including regular blood transfusions, daily chelation therapy and frequent medical supervision ( 1 , 2 ). Like other chronic diseases requiring long-life treatment, adherence to treatment is a major concern for β-TM patients ( 3 ).…”

“…Patients with β-TM require lifelong adherence to distressing treatment regimen, including regular blood transfusions, daily chelation therapy and frequent medical supervision ( 1 , 2 ). Like other chronic diseases requiring long-life treatment, adherence to treatment is a major concern for β-TM patients ( 3 ). It has been revealed that poor adherence to therapeutic regimen in β-TM patients is associated with poor clinical outcomes including increased risk of complications, highest risk of mortality and morbidity and, consequently, increased healthcare costs.…”

“…This gives a special emphasis to maintain optimal adherence to treatment in these patients. Despite the clinical importance of adherence to treatment in β-TM patients, poor adherence remains a prevalent and persistent problem in these patients, with the reported rates ranging from 30 to 80 percent ( 3 , 5 , 6 ). Although many variables have been studied to determine the predictability of patients’ adherence to treatment, none of them are capable of predicting it precisely ( 7 , 8 ).…”

Vitamin and Mineral Supplementation for β-Thalassemia During COVID-19 Pandemic

“…Interestingly, in the absence of an iron chelator, vitamin C can increase iron absorption by reducing Fe 3+ to Fe 2+ , contributes to hydroxyl radical formation and further increases oxidation. Therefore, with its prooxidant activity, vitamin C supplementation in iron-rich conditions is still debatable [ 33 ]. Despite its prooxidant activity, vitamin C increases the efficiency of chelation agents by promoting Fe 2+ and increases the iron release from reticuloendothelial system.…”

Pain in β-thalassemia major patients: an important yet neglected issue

“…Patients with β-TM require lifelong adherence to distressing treatment regimens, including regular blood transfusions and daily chelation therapy. Therefore, maintaining hemoglobin target levels as well as appropriate chelation therapy has been particularly emphasized in the care of patients with β-TM [ 1 2 ]. In keeping with improvement in life expectancy of β-TM patients, some previously unrecognized health issues have been identified in these patients that affect their quality of life as well as their overall ability to adhere to treatment [ 3 ].…”