Talasemia adalah kecacatan genetik yang paling biasa didapati, dengan kira-kira 250 juta orang, 4.5 peratus daripada penduduk dunia adalah pengidap yang menghadapi ancaman maut. Pada 2010, Malaysia merekodkan 4,768 orang pesakit talasemia yang memerlukan pemindahan darah yang kerap. Manakala 5% daripada penduduknya adalah pembawa.





How many times a day should Deferiprone be taken?
Deferiprone is taken in three divided doses, morning, midday and evening.

Is there an interaction with other medicines?
There have been no reports of drug interactions with other prescribed medicines. There is, however, a potential interaction with aluminium-based based antacids, since Deferiprone binds other metallic ions as well as iron. The concurrent use of Vitamin C has not been studied but it is potentially toxic and should be avoided.

Why is it necessary to do a weekly blood count?
In a proportion of patients there is fall in the number of polymorphonuclear cells, the white blood cells responsible for the defence of the body against bacterial infections. This may be severe fall to below 500/cmm known as agranulocytosis: this occurs in about 1.2% of patients taking Deferiprone and it may lead to a serious infection. A less severe fall in the neutrophil count may also occur, between 500–1500/cmm, known as neutropenia. This may be indicate that agranulocytosis is imminent and may also predispose to infection. It is important to detect this fall in white cell count early since interruption of the drug will restore the neutrophil count and avoid any complications. This is the reason for the weekly blood counts.

Is Deferiprone used in pregnancy and breastfeeding?
In animal studies it was demonstrated that this drug can cause damage to the early embryo (teratogenic). This has not been recorded in humans but because of the experience in animals it is unwise to use the drug in both pregnancy and breastfeeding. In fact it is strongly advised to avoid pregnancy while on this medication.

Does Deferiprone predispose to Yersinia enterocolitica infection?
Experimental evidence from the Pasteur Institute of Paris, both in vitro and in a mouse model, indicate that this drug does not promote Yersinia growth and virulence. However, in a Canadian review of clinical infections in thalassaemia patients over 15years, one patient was receiving Deferiprone, while 2 patients were not on any chelating agent and the rest of their cases (11) were taking Deferoxamine. For this reason it can be concluded that Deferiprone is less likely to predispose to Yersinia. In numerous studies on Deferiprone in thalassaemia, cases of Yersinia infection are not mentioned.

Does Deferiprone protect the heart more than other agents?
Comparisons have been made mainly with Deferoxamine. Recent publications confirm that Deferiprone is superior as a cardio-protective agent than Deferoxamine, probably because as a smaller molecule, it can remove intracellular iron from myocardial cells.

Can Deferiprone be used in children?
Formal trials of safety and efficacy have not been conducted in children under the age of 10 years.

© 2008 Thalassaemia International Federation


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