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Thalassaemic minor

Q: I and my nine year old son are both beta thalassaemic minor, detected a year back while investigating loss in Hb even after administration of Iron tabs. We both were prescribed Folvite - one tab daily since October 2000. My sons Hb raised from ~7.5 to 9.4 in six months. He did not have any sickness during this period. But I had problems of loose motion for the last three months and Hb has come down to 7.4 now. Our physician has prescribed 2 Tabs of Folvite a day and opined that both I and my son should hv to continue this medicine life long. Please advise (1) how long should we have to continue Folvite and is there any adverse effect? (2)What precautions to be taken to arrest fall in Hb? (3)How frequently do we have to get our Hb checked? (4) What are the potential emergencies? (5) What is the alarming low level of Hb and what action to be taken then? Date of birth: 03/10/1964 Sex: : female Weight: 48 Height: 160 About the current problem:

A:In beta thalassaemia minor (also referred to as beta thalassaemia trait), the Hb concentration is generally 10g/dl or more. When the Hb is lower than that one should exclude other causes of fall in Hb lavel. The rise in Hb following administration of Folvite (which is folic acid) suggests that the son of the inquirer, had folate deficiency. Folate requirements go up in thalassaemia trait. Therefore, its deficiency in this disorder is not infrequent. Diahorrea is uncommon in folate deficiency but can cause folate deficiency. 1. To answer these questions one has to know the details of tests which led to the diagnosis of thalassaemia minor. As a fall in Hb level to less than 9g/dl is unusual the diagnosis should be on firm grounds. Associated disorders should be excluded. 2. Folic acid ('Folvite') administration for long durations is generally safe. 3. It may no be necessary to take it life long. It will depend on response to therapy. Generally a dose of 1 mg a day taken orally is enough. I do not think that folic acid in this dose is readily available. Most tablets contain 5mg of folic acid. 4. If there is a fall in Hb it is desirable to seek consultation and find out its cause. If it is due to folic deficiency one has to find a cause for that. Hb of 7-8 g/ is considered severe anaemia and should be treated. 5. If Hb is below 8 g/dl frequent checks are required. 6. Some individuals with thalassaemia trait also develop cocomitant iron deficiency. This may be unlikely as the son of the 'inquirer' did not show a rise in Hb level following iron therapy. It will, however, be a good idea to exclude iron deficiency by appropriate tests such as serum iron, TIBC and serum ferritin. The blood should be taken when the patient is fasting and is not on iron therapy. 7. If the spouse has also thalassaemia trait, the individuals with thalassaemia trait should consult a physician to understand the risk of having a child with thalassaemia major . Thalassaemia major is a serious disease and its prevetion is considered important.

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