Presentation on theme: "Presented by: Dr. Afsana Jahan (PGT) Dr. Aditi Baruah (MD); Dr. P. Bishwanath (MD) Department of Paediatrics Assam Medical College & Hospital, Dibrugarh."— Presentation transcript:
Presented by: Dr. Afsana Jahan (PGT) Dr. Aditi Baruah (MD); Dr. P. Bishwanath (MD) Department of Paediatrics Assam Medical College & Hospital, Dibrugarh
Thalassemia is the commonest chronic haematological disease needing repeated blood transfusion. North-East specially Assam is a reservoir of abnormal haemoglobins & also thalassemia because of ethnic diversity of the inhabitants. Present thalassemia clinic was established in April2005, AMCH, Dibrugarh where regular follow-up of thalassemia pateints are done.
To study the follow-up cases of haemo- globinopathy with special reference to thalassemia in thalassemia clinic
Place of Study:Thalassemia clinic, AMCH Period of Study:May2005 to Oct2007 Study universe:50 diagnosed and registered cases of Haemoglobinopathy Selection criteria:Cases of Haemoglobinopathy diagnosed by HPLC.
SPLEEN SIZE β THALASSEMIA MAJOR HbE – β THALASSEMIA Not palpable13 < 2cm02 2 – 7cm111 >7cm52 Splenectomized46
No. of patients Drug and dose mg/kg/day Length of study S. Ferritin level (μg/L) InitialFinal 16 Deferiprone 50 – 100 1yr32611420 4 Desferoxamine 35 – 55 1yr31901392 3Combined9months52611276
DIAGNOSISCASE NO.MOTHERFATHER β – thalassemia major49β – thalassemia trait Hb E - thalassemia 1β – thalassemia traitHb E trait 2Not doneHb E trait 3 Hb E trait - β – thalassemia trait Hb E trait 6 β – thalassemia trait 7 Hb E trait 8β – thalassemia traitHb E trait 13Hb E traitβ – thalassemia trait 40β – thalassemia traitNot done 48Hb A A
Family study and convincing parents and relatives for necessity of the test. Irregular follow-up visits. Irregular blood transfusion Lack of voluntary blood donation. Cost of filters and chelation therapy limits their use. Cost of different laboratory tests.
Public awareness. Education of health professional. Antenatal diagnosis. Emphasis on conventional treatment in particular chelation in view of its high efficacy. Cure of the disease with increasing possibility of bone marrow transplantation and gene therapy. Concrete support for achieving high standard for management of thalassemia in this part of Country.
Thalassemia clinic is playing an important role in making thalassemia a preventable, manageable and curable disease in prospect of high incidence in this region.