Abstract
BackgroundBeta thalassemia is the most frequent genetic disorder of haemoglobin synthesis in Pakistan. Recurrent transfusions lead to iron-overload manifested by increased serum Ferritin levels, for which chelation therapy is required.FindingsThe study was conducted in the Pediatric Emergency unit of Civil Hospital Karachi after ethical approval by the Institutional Review Board of Dow University of Health Sciences. Seventy nine cases of beta thalassemia major were included after a written consent. The care takers were interviewed for the socio-demographic variables and the use of Desferrioxamine therapy, after which a blood sample was drawn to assess the serum Ferritin level. SPSS 15.0 was employed for data entry and analysis.Of the seventy-nine patients included in the study, 46 (58.2%) were males while 33 (41.8%) were females. The mean age was 10.8 (± 4.5) years with the dominant age group (46.2%) being 10 to 14 years. In 62 (78.8%) cases, the care taker education was below the tenth grade. The mean serum Ferritin level in our study were 4236.5 ng/ml and showed a directly proportional relationship with age. Desferrioxamine was used by patients in 46 (58.2%) cases with monthly house hold income significant factor to the use of therapy.ConclusionsThe mean serum Ferritin levels are approximately ten times higher than the normal recommended levels for normal individuals, with two-fifths of the patients not receiving iron chelation therapy at all. Use of iron chelation therapy and titrating the dose according to the need can significantly lower the iron load reducing the risk of iron-overload related complications leading to a better quality of life and improving survival in Pakistani beta thalassemia major patients.Conflicts of Interest: None
Highlights
Beta thalassemia is the most frequent genetic disorder of haemoglobin synthesis in Pakistan
Use of iron chelation therapy and titrating the dose according to the need can significantly lower the iron load reducing the risk of iron-overload related complications leading to a better quality of life and improving survival in Pakistani beta thalassemia major patients
Beta thalassemia ranks first amongst the genetic disorders associated with haemoglobin synthesis in terms of prevalence and it is the result of an inherited defect in the synthesis of the beta chain of the adult haemoglobin
Summary
Beta thalassemia is the most frequent genetic disorder of haemoglobin synthesis in Pakistan. Considering the 5 to 7% carrier rate in Pakistan, the number of carriers is expected to approach 10 million [2] This grades thalassemia as one of the most common inherited disorders in Pakistan [3]. Due to repeated blood transfusions, all thalassemic patients require Iron Chelation Therapy (ICT) in order to reduce the increased iron load that if not eliminated properly can result in life threatening complications, including severe cardiac toxicity in the second decade of life, producing a significant decrease in the total life expectancy of these patients [6]. The practice of repeated parenteral ICT therapy imposes various economic burdens in those who cannot afford such a therapy Oral chelators such as Deferasirox are much more expensive than the traditional ICT’s which makes access difficult for the majority of socio-economically underprivileged patients [7]. This study highlighted the dismal situation of ICT having a negative impact on health related quality of life (HRQOL), and that in most of the cases compliance with ICT being suboptimal, signifying non-satisfactory provision of chelation therapy with respect to economic, clinical and quality of life related outcome
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