Iron chelation in thalassemia: time to reconsider our comfort zones

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Abstract:

Over the last 20 years, the management of thalassemia major has improved to the point where we predict that the patients'' life expectancy will approach that of the normal population. These outcomes result from safer blood transfusions, the availability of three iron chelators, new imaging techniques that allow organ-specific assessment of the degree of iron overload and improvement in the treatment of hepatitis. The ability to prescribe any of the three chelators, as well as their combinations, has led to a more effective reduction of the total body iron. The ability to determine the amount of iron in the liver and heart by MRI has allowed the prescription of the most appropriate chelation regime for the patient and has allowed the reconsideration of ``the comfort zones''. Thus, normalizing iron stores not only prevents new morbidities but also reverses many complications, such as cardiac failure, hypothyroidism, hypogonadism, impaired glucose tolerance and Type 2 diabetes, therefore improving survival and patients'' quality of life. Furthermore, outcomes should continue to improve in the future. Starting relatively intensive chelation in younger children may prevent short stature and abnormal pubertal maturation, as well as other iron-related morbidities. In addition, further information should become available on the use of other combinations in chelation treatment, some of which have only been used in a very limited fashion so far. New safe oral chelators may also become available that may offer additional ease of use. All these advances in management do require absolute cooperation and understanding on behalf of children''s parents and subsequently the adult themself. Only with such cooperation can normal long-term survival be achieved as it is likely that adherence to treatment is the primary barrier to longevity.

Authors: Berdoukas, Vasilios1; Farmaki, Kallistheni; Wood, John C; Coates, Thomas

Source: Expert Review of Hematology, Volume 4, Number 1, February 2011 , pp. 17-26(10)

Publisher: Expert Reviews

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Offline Andy Battaglia

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Re: Iron chelation in thalassemia: time to reconsider our comfort zones
« Reply #1 on: March 17, 2011, 03:13:01 AM »
I'm glad they're finally catching up and telling people the same thing I've been saying for years. With properly managed treatment, thals can live long, full lives. When I wrote the introduction to this group at the behest of some members and some thoughtful input form Canadian Family, a main focus throughout was to keep a positive tone, but remember that the positive tone had to be based in honesty, and the emerging reality was that thal can be managed when a diligent comprehensive approach to treatment is constant throughout the life of the patient. As usual, we at thalpal have been ahead of the curve.
Andy

All we are saying is give thals a chance.

Re: Iron chelation in thalassemia: time to reconsider our comfort zones
« Reply #2 on: March 17, 2011, 09:24:48 AM »
Why is phytic acid so expensive as an iron chelator ? Is it because a person is buying it from a Health Food store or online ? What I've been able to understand is phytic acid is available through China at under ten cents a gram compared to mimosine which sells for two hundred a gram ? They are both iron chelators except one has been marketed / mimosine and one is not being 'marketed' .. ? Mimosine by the pharmaceutical industry and phytic acid won't even be looked at by the NIH .. ? Someone HAD proposed to the NIH during their request for study of natural substances and phytic acid was refused to be funded. Someone with a purpose could get phytic acid out to anyone who needed it at a MUCH discounted **quantity** out of China .. ?

http://www.made-in-china.com/products-search/hot-china-products/Phytic_Acid.html

 

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