Discussion Forums > Thalassemia Major

AP Biology Student Looking for Insight

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Manal:
You are welcome faltdavid. Actually I don't use chelators till the moment so I will leave the detailed description for any of the members to explain it, but in brief deferral, is administered by putting a needle in the tummy for around twelve hours. This is so harsh on children especially that those twelve hours will definitely be during their sleep time so I guess it makes sleeping uncomfortable. Also being uncomfortable leads to uncompliance and therefore leads to iron overload with all it's side effects.

There is two other chelators, one is so expensive that you have to be insured to use it and the other is oral but it won't suit anyone
Manal

faitdavid:
Thank you all,

Zaini- I think the only way it would be possible not to need transfusions would be if we could modify DNA in the RBC's so that they could produce the beta protein again. I know they have found the gene loci of the mutation, but I don't believe we are able to do that on a large scale yet so that all RBC's would have that gene... It's possible that some iron could be removed from blood before it is transfused, but it may reduce the efeectiveness. Oh yes... I wasn't sure where the pump went, I didn't know it was in the tummy, that would certainly be uncomfortable and make it a bit harder to sleep.
Thanks!

Cari- The post about your son was the first one I saw on this site, actually! It's good that he is having a pretty normal life so far. Yes, babies begin to produce adult hemoglobin normally at around 6 months, give or take a month or two. In thal majors, the beta protein is not manufactured correctly, and then a beta chain can't be formed as part of the normal adult hemoglobin. How much blood does he receive each transfusion?
And thank you for the reference!

Manal- I can see why noncompliance would be a problem, yes. To me, I think the benefits would outweigh the discomfort, but that comes from someone who hasn't experienced it.

David

Poirot:
Hi David,

Welcome to the site!

I *had* to reply to your message, when I saw it due to a funny coincidence - my daughter, who also has Biology in her high school and has to do a school project for the year, also selected Thalassaemia for her school project - mainly, I think, because she can get all her dope from me, sitting at home, while not having to do much research! Also, thanks to all the good reviews being received by Vampires lately, she probably thinks it is cool to have a Vampire Dad!

So, to your question - what is a normal day for a Thal?

In most cases, very much like yours I suspect, when I was your age - go to school/college, see if you can cut classes, hang out in the cafetaria, break as many rules as you can safely get away with, play video games, read some books, and do the least amount of studies that could potentially get you an A grade!  The difference were the days when one went to the transfusion centre, when the whole thal thing sits on your head  and sick days, when you generally get sicker with the common bugs/parasites than your friends would. 

The other overhang is the whole iron thing - transfusions are a double edged sword - they keep your HB high, saving your life for now, while injecting a lethal, slow poison into you in the form of iron, that WILL eventually kill you, if untreated. Therefore, you endure all the fuss about chelation. With Desferal, it is not really the needle in the stomach or the thigh that is the real problem, but all the bumps and painful lumps that you get post infusion - that used to be my bane. Until L1/Kelfer came along - I think that saved my life.

Cheers

Poirot

faitdavid:
Thanks!

Oh! That's funny. Yes, I get the feeling that it's become very normal, and that's good.  I think it's as if the extra iron is like a second disorder or something. Oh, yes. Kelfer is another oral chelator, right?

David

Zaini:
Yes David,

Kelfer/Defriprone/ferriprox/L1,different names for the same drug,and it is the first ever oral chelator.

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