Discussion Forums > Thalassemia Major

New to Thal Major - lots of questions

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Andy Battaglia:
In reality, many patients do chelate less with desferal as they get older, and this is a big cause of complications in patients in their teens and twenties. It is so easy to brush off chelation when you have an active life. This is why oral chelators give such a huge advantage to patients. It does not disrupt life at all to take something orally once per day. This is actually something that needs a much closer look in many countries where desferal is paid for but Exjade is not due to its higher cost. Better compliance rates will mean fewer complications and lower long term health costs. We have Exjade users in this group with very low ferritin levels, so long term use has been proven to be effective in controlling iron load in patients.

Some patients do use ports but it is a controversial subject. Ports can and will become infected in most patients at some point and often need to be replaced after some years use. A recent thread along with a link to a previous thread about ports can be seen at
http://www.thalassemiapatientsandfriends.com/index.php?topic=1890.0

If oral chelation is chosen, a port will only be necessary for patients whose veins prove unsuitable for long term transfusion therapy. With most patients, veins can be found by good nurses and a port isn't needed. However, like most technology, doctors are very big on ports and sometimes downplay the negatives.

germ79:
Andy,

Thanks again for the information.  Wow!  There is just so much to learn about Thal. 

I get the feeling that you are a big proponent of Exjade.  I've read mixed results from its use.  It seems like maybe it works well for some, but not for others.  Would that be accurate?  Is it just one of those things you have to try and see if it will be able to maintain your levels successfully? 

Also, it seems to me that one of the big things about Exjade is the ease and, therefore, compliance with taking Exjade.  I've read from many people that Desferal is the most effective at chelating.  So, could one use Desferal once a week and Exjade the other days?  Can you mix-n-match? 

J

Andy Battaglia:
There are some doctors in the US who are using Exjade and desferal in combination, even though it hasn't been officially approved. One of these doctors is the eminent Dr Elliot Vichinsky of Oakland Children's Hospital. He is recognized worldwide as one of the top thalassemia experts, both in practice and research.

 I am actually a big proponent of choice in chelation. Unfortunately, the best chelator in terms of lowering cardiac iron and protecting the heart, is not approved in the US and can only be obtained through a special program. This is the drug, deferiprone (L1, kelfer, ferriprox). Since no chelator is ideal for all patients, I believe that all three chelators should be readily available. The reason I do emphasize Exjade is because there is no doubt that an easy to use chelator will result in a much higher compliance rate long term.

As far as Exjade not working for some patients, this is primarily due to patients and doctors not having patience. While, as with all chelators, there are some patients who cannot tolerate it, most patients will find exjade to be an effective chelator if they use it for a long enough period and also use it at the necessary dosage for their iron load.  Exjade can take some time to show results in ferritin tests and often it is a year before patients see a downward trend. Patients may even see a rise in ferritin levels when first starting Exjade. There are two main reasons for this. First, there are side effects in most patients when they first start Exjade, so many patients do not start out on the optimum dose but instead, slowly increase the dosage until it is at the desired level. Second, patients with iron overload will take longer to show good results. While Exjade is reducing tissue iron, this may not be seen in ferritin results initially, but as time passes and tissue iron is reduced, patients do see a reduction in ferritin levels. One person I know took Exjade for a year, only to see her ferritin levels double. All the time, her doctor told her to be patient. Now, after a second year of use, her ferritin has dropped below where it was when she first started Exjade. Doctors should always familiarize themselves with the current dosing recommendations, as can be found in the document at
http://www.thalassemiapatientsandfriends.com/index.php?topic=1928.msg16165#msg16165 Updated Prescribing Information for Exjade

Zaini:
Hi Jeremy and Diana,

Welcome on the forum  :welcomewagon it's very kind of you to consider adopting a child with B Thal major.

I'll try to answer your questions as a parent,it's a bit difficult as english in not my primary language so i feel like i leave so much unsaid.

Normal daily life is not much different for thal majors,if you are taking good care,have knowledge about supplementation,blood you transfuse is properly screened,and if you are compliant to chelation,and if you are maintaining proper pre transfusion hb levels.
These are the necessary precautions you have to take,apart from that i don't think there is any need of hospitalization,unless the child gets very sick or have some problems,it's been 4 years my daughter started transfusing ,and i don't remember going to hospital apart from the transfusion visits.she is Thanks to God doing well,looks and behaves like a healthy and normal child,is a brilliant student,and won the first position in sports in her school last year ( you can see her pics in the gallery ).

I hope this helps,and if you have any other questions in mind,feel free to ask.

ZAINI.

germ79:
Zaini,

Thanks so much for taking the time to respond to my questions.  It was great to hear from someone with a thal child.  Sound like you have quite a wonderful kid on your hands!  How awesome!




I called Dr. Thompson in Chicago today and spoke to her about the little girl we are thinking of adopting.  She was VERY nice and answered all of my questions.  She really made me feel at peace about the whole situation. 

Tomorrow or Friday my wife and I will be receiving the file on the baby girl and will have a lot more information to work with.  We are very excited to review the file and talk to some doctors about what we find. 

I look forward to hearing from more of you and getting to know you as well!

Jeremy (and Diana)

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