Combination Chelation and Survival

This week I posted the review from the European Journal of Haematology, The heart in transfusion dependent homozygous thalassaemia today. The review strongly supports combination chelation using desferal and deferiprone (L1) for reducing iron overload and especially for use in patients with iron overload in their hearts. It has been shown to be the best way to quickly reduce iron overload and remove thal patients from immediate danger.

A real eye opener in this review is that iron overload in the heart cannot be accurately predicted by testing serum ferritin or liver iron. This is major news concerning liver iron, as it was always thought that if liver iron is low, heart iron will also be low. This was not what this review found. Heart iron needs to be directly tested.

What has really made me think this week is learning that the person who sent me this review is now on combination chelation because she found out she is suffering from iron overload in the heart. She has had excellent care and was one of the first people I knew to be on an Exjade trial. She has complied with chelation, yet still has iron overload in her heart. She and I are both optimistic that the combination therapy will quickly reduce this iron load. But the questions remain. If a patient who has excellent care and monitoring can find herself with iron overload in the heart, shouldn't all patients have their hearts tested for iron overload, regardless of their perceived health and measured iron loads? I realize these tests are not readily available to many patients but shouldn't one of the goals of the international organization be to make these tests available? Patients need to know and the international thal community needs to do more to make sure patients know their true status and also have ready access to chelators. (I find it disgraceful that L1 is not easily available to patients in the US and Canada).

Once again, the link to the review is at
http://www.thalassemiapatientsandfriends.com/index.php?topic=1451.msg11816#msg11816

Hi Andy,

What tests would you think will help in determining wether there is iron overload in  heart or not ? Is Echo sufficient for this purpose,the thing is that in the third world countries like ours,every advance test and technology is not available,and sometimes if something is available it's not necessarily affordable,i hope you know how the picture is here in Pakistan.

And by the way what is T2?

ZAINI.

Echo can show heart function but not iron overload. T2 testing using MRI imaging can give an accurate picture of the iron load in the heart. You can read about it at   http://www.cooleysanemia.org/sections.php?sec=2&tab=10&sub=204

 

T2* is a time measurement that reflects iron content in tissue. The T2* measurement is obtained from images taken on a standard Magnetic Resonance Image (MRI) machine. The MRI machine uses a strong magnet and radio waves to image body issues. The MRI machine detects differences in magnetic properties of the body and converts these differences into pictures that physicians can use to diagnose various diseases. Iron overload causes changes in the magnetic properties of tissues, which causes the T2* values to become smaller. The T2* measurement of the heart is important because it has been found that patients with a cardiac T2* value less than 20 milliseconds (a millisecond is 1/1000 of a second) are at higher risk for heart problems related to iron overload than patients with T2* value greater than 20 milliseconds.

I don't know if T2 is available to you but please try contacting the Journal Of Pakistan Medical Association.

http://jpma.org.pk/Static/ContactUs.aspx

P.M.A. House, Aga Khan III Road,
Karachi- 74400 (Pakistan)

Phone/Fax No. +92(21)222-6443
E.mail: jpma_jpma@hotmail.com

Hi Andy,

MRI is available here,but as far as i know it's really very expensive.i'll try to get more info.

ZAINI.

Zaini,

Below link will explains the basics of T2 test and how it is done.

http://www.cooleysanemia.org/sections.php?sec=2&tab=10&sub=204&node=204

Thanks