ABSTRACT
Transfusion-transmitted hepatitis C is a major concern among thalassemia patients. Our aim is to estimate the prevalence of Hepatitis C infection among thalassemia patients and to assess the treatment response, adverse effects of Peg-interferon based regimen and the new direct-acting antiviral drugs. Patients with thalassemia receiving regular blood transfusions with positive anti HCV antibodies during a period from January 2012 to June 2017 were analyzed. Serial HCV viral load and genotype and liver function tests were performed. Peg interferon and Ribavirin were used in patients diagnosed before January 2016 and patients diagnosed after January 2016 were started on the combination of Ledipasvir/Sofosbuvir. Thirty-two patients aged between 2 and 28 years were analyzed. Genotype 1 was the predominant type. Twenty-one patients were initiated on Peg Interferon with Ribavirin, and 14 achieved sustained virological response. All of them had increased blood transfusion requirements with significant compliance issues. All eleven patients started on Ledipasvir and Sofosbuvir including 4 undergoing hematopoietic stem cell transplantation and 7 interferon failures showed sustained viral clearance with good compliance. Ledipasvir/Sofosbuvir combination can be safely used in thalassemia patients and in young children. The cost of therapy is less compared to peg interferon based regimen with good compliance and superior efficacy.
ABSTRACT
OBJECTIVE: To compare quality of life of children with thalassemia major who have undergone stem cell transplantation with those on regular transfusion. METHODS: The study included 40 children who underwent transplantation and 40 children and 20 adults on regular transfusion and iron chelation therapy. The quality of life assessment was done using the Pediatric Quality of Life Inventory 4.0 Generic Core Scale. RESULTS: The mean total summary score, psychosocial summary score and physical score was 92, 91 and 92.8, respectively in transplant group and 83, 82.7 and 83.6, respectively in children in transfusion group. The adult group on transfusion showed overall poorer scores of 74.9, 76 and 73.9, respectively. The average scores in all domains were significantly (P<0.05) lower and drop steeply in second decade in transfusion group. CONCLUSIONS: Allogeneic stem cell transplantation improves quality of life in thalassemia major.
