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Does my thalassaemic daughter need regular blood transfusions?

Q: My daughter was diagnosed as beta thalassaemic when she was 4 years old. She has been transfused blood about 10 times. Her haemoglobin (Hb) is always around 8.5 to 9.0. Tests have concluded that she is Thalassaemia Intermedia and her doctor says Hydroxyurea may not be good for her, which she has been taking for the last 6 years. Her TSH is 9.7 and is also taking Thyronorm 50mg and 75 mg alternately. Her bone age by left hand and wrist is above 11 years. All other tests are in normal range except MCV, MCH. If she is Intermedia thalassaemia and with minor thyroid problem then will she require blood transfusion intermediately? Will the control of thyroid increase her Hb? What is the best available treatment to control her thalassaemia? How can her growth become better as she is 13.5 years old. Her puberty is yet to start. Please advise.

A:Traditionally, the clinical presentation of thalassaemia can be of 3 types - Thalassaemia major (profound anaemia which is transfusion dependant), Thalassaemia intermedia (moderate to severe anaemia which requires only occasional blood transfusion) and Thalassaemia trait (clinically asymptomatic presenting as only a mild anaemia). This clinical severity depends on several factors like the degree of reduction in â globin chain synthesis, co-inheritance of á thalassaemia or of genetic determinants which increases ã chain synthesis and other genetic factors that modify secondary complications of disease. Clinically, thal intermedia covers a broad spectrum, ranging from a relatively severe anaemia that requires intermittent blood transfusions to an asymptomatic condition diagnosed via a chance blood test. The disease presents late compared with transfusion-dependent forms of the disease and the most common symptoms are anaemia and jaundice with some degree of splenomegaly (spleen enlargement). Bone changes are variable and range from none to the severe skeletal deformities characteristic of transfusion-dependent â thalassaemia. Due to the uncertainties of its clinical course and pathophysiology, there are no set guidelines for the optimal management of thal intermedia. Close monitoring of growth and iron status is mandatory. Patients are given folic acid supplements, blood transfusion, iron chelation and splenectomy (removal of spleen) as indicated. Sometimes small doses of radiation or hydroxyurea or transfusion may be required for extra-medullary masses of haematopoiesis. Blood transfusion is given if there is evidence of impaired growth & development during growth spurts, infection, and pregnancy and to relieve complications like leg ulcers or extra-medullary haematopoiesis. Please be guided by your haematologist as he would take into account the entire clinical picture.


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