Thalassemia: A Community Challenge

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Presentation transcript:

Thalassemia: A Community Challenge

Thalassemia: A Community Challenge What is Thalassemia? (it is a genetic hemoglobin problem) Explain to Dr. Chris how hemoglobin works.

Normal Hemoglobin

Who has Thalassemia patients in their community? How do you know they have Thalassemia? How old are they? What problems are these patients having?

The gene frequencies of alpha-thalassemia reach 30-40% in Northern Thailand and Lao PDR, of beta-thalassemia vary between 1-9%, and HbE has a frequency of 50-60% at the junction of Thailand, Lao PDR, and Cambodia (Fucharoen and Winichagoon, 1992).

29 cases of Alpha thalassemia. The hospital data from Pediatrics wards of 3 central hospitals in Vientiane were obtained from 2005 -2011. 51 cases of beta-thalassemia major, 224 cases of Beta-thal/Hb E disease, 78 cases of Hb E trait 29 cases of Alpha thalassemia. 37 cases of splenectomy, 177 cases of iron overload, 82 cases of iron chelation. Incidence of hydrops fetalis is 0.3%. Sourideth Sengchanh MD, Department of Pediatrics, Alongkone Phengsavanh MD, Department of Obstetrics and Gynecology, Faculty of Medicine, University of Health Sciences, Lao PDR, Assoc. Prof. Dr. Khampe Phongsavath, Sethathirath Hospital

They develop chronic anemia, the stigmata of profound hemolysis, and suffer the noxious effects of massive ineffective erythropoiesis upon the body. Cannot carry and deliver oxygen. Cannot make proper rbc = ineffective erythropoiesis. Damaged rbc fragment, jaundice. The body therefore tries harder. Epo rises. Marrow expansion results in thin bones, expansion of bones, recruitment of fetal erythropoietic spaces liver and spleen. Intravascular hemolysis Transfusional iron overload.