some ques about thalassemia major

Hi all, i'm Jonathan 21 yrs old from mauritius. I have the thalassemia as from 5 yrs old and i need to make transfusion every 3 months. I wanted to know what i can do to make my haemoglobin keep up and do transfusion more rarely as possible? Normally after a transfusion it's come above 8 to 9, and after 3 month it comes near 6. sencond ques is i just make a blood test and it says that my level of iron in me it's too high and my doctor just inform me that i need to take deferiprone to elimate it but the probis the in mauritius we don't have it. I just wanted to know where i can buy the deferiprone and how much it cost etc.. ??


Thanks for your help

Jonathan.

Hi Jonathan,

Welcome to the group.

Technically you are a thalassemia intermedia because you are able to keep your Hb above 6 by transfusing every few months. Majors have to transfuse more often and can't maintain an Hb above 6 without frequent transfusions. This means your iron accumualtion isn't as fast as it is for majors, but now it is getting high enough to need to chelate. Did your doctor tell you what your ferritin level is?

Iron chelating drugs such as deferiprone are only available by prescription so they are hard to get from another country. Your doctor should contact Apotex or Cipla to see if deferiprone can be imported into Mauritius. Contact information is below. There are two other chelating drugs, desferal and Exjade. Desferal is taken using a needle inserted under the skin for about 12 hours at a time. Exjade is a new oral drug that is quite expensive. It is important to lower your iron as it will damage your organs over time.

Does anyone in Pakistan know if any of the local suppliers of deferiprone export the drug to other countries?


Apotex Zimbabwe Office

Distribution Office:

Zimbabwe

Silchem Trading (Pvt) Ltd.
10 Kenmark Crescent, Bluff Hill Industrial Park, Bluff Hill, PO Box WGT 1273, Westgate, Harare, Zimbabwe

Phone:  263-4-331126 or 263-4-331446
Fax: 263-4-305926 or 263-4-331168




Cipla Ltd.
Mumbai Central
Mumbai 400 008
India
     
Telephone:
   
2308 2891
2309 5521
     
Fax:
   
91 22 2307 0013
91 22 2307 0393

Hi Andy,

Global Pharmaceuticals manufacture it by the brand of "Ferrinill"

Appearantly they are giving it to their own patients of Jamila Sultana Foundation; but there has been a lot of demand for it by the patients of other cities of Pakistan. I don't know if Global has fulfilled their demand or not but when I asked them for Zaini they told me that they are supplying it to Kashif Iqbal Center in Karachi from where she can get it.

I will ask them about their export policy when I go there in a couple of days and you can also send a letter of request to them at the Jamila Sultana address:

Global Pharmaceuticals.
c/o Jamila Sultana Foundation.
D-24, 6^th road, Satellite Town.
Rawalpindi.
Pakistan

In Pakistan ferriprox(defriprone) is supplied by ICI life sciences,

http://www.icipakistan.com/lifesciences.html

You can contact their marketing manager,pharmaceuticals at saboor_ahmed@ici.com.

ZAINI.

Hi Andy, I'm sorry yesterday i didn't say all the details. When i was young i made transfusion every month and after removing the Pancreas i made the transfusion more rarely i.e every 3-4 month and after 3 month sometimes my HB goes under 6 and then i need to do transfusion. Normally every transfusion now i received 3 pint of blood " Packed cell " . And my doc say that i have the thalasemia major. That's why i wanted to know if it's true or not i have the thalassemia major or not ?? Normally i took 1 Folic acid 5mg every day. and i wanted to know if there is any other things i can do to do the transfusion more rarely or make my HB to stay stable?  I wanted to know also normally for every pint of blood, how many hours need to do one transfusion of 1 pint?? Because in the hospital i went to do the transfusion some nurse say 6hrs for 1 pint, some say 3 hrs. I myself i don't don't know who i need to believe:(


Thanks a lot for your help,

Jonathan.

P.S Any comments or suggestions are the most welcome.

When i was young i made transfusion every month and after removing the Pancreas i made the transfusion more rarely i.e every 3-4 month and after 3 month sometimes my HB goes under 6 and then i need to do transfusion.

Jonathan, are you sure they removed the "Pancreas". Normally it is the "Spleen" that is taken out.

Anyway, diagnosing Thal. Intermedia is very tricky (unless done through proper DNA testing) and it is misdiagnosed as Major a lot of times when the patient becomes transfusion dependant. This could have been avoided if they had not transfused you in your early childhood and let you cope with the low Hb. In intermedia the Hb stabilizes at around 6-7g/dl which can be improved a bit and kept at bay with the help of HbF inducers (Which the doctors were probably not aware of in your early childhood). However, once Intermedias are given transfusions they become transfusion dependant and are misdiagnosed as Thal. Major. However, unlike Thal. Major you don't require monthly transfusion after the splenectomy while in Majors the procedure is beneficial in the beginning but eventually they go back to their old schedule.

I'm sory sajid yeah it's the spleen they removed.

Thanks for your help,

Jonathan

Jonathan, are you sure they removed the "Pancreas". Normally it is the "Spleen" that is taken out.

Anyway, diagnosing Thal. Intermedia is very tricky (unless done through proper DNA testing) and it is misdiagnosed as Major a lot of times when the patient becomes transfusion dependant. This could have been avoided if they had not transfused you in your early childhood and let you cope with the low Hb. In intermedia the Hb stabilizes at around 6-7g/dl which can be improved a bit and kept at bay with the help of HbF inducers (Which the doctors were probably not aware of in your early childhood). However, once Intermedias are given transfusions they become transfusion dependant and are misdiagnosed as Thal. Major. However, unlike Thal. Major you don't require monthly transfusion after the splenectomy while in Majors the procedure is beneficial in the beginning but eventually they go back to their old schedule.

Jonathan,

Has the doctor ever mentioned hydroxyurea therapy to you? Many intermedias are able to boost their Hb enough using hydroxyurea to where they do not require transfusions. You might also consider using wheatgrass in some form to try to boost your Hb and keep taking folic acid.

Spleen removal is often done with intermedia to reduce transfusion frequency.

HI Jonathan...
 It doesnt seem that you are Thal Intermedia.. because you have taken tranfusion regularly before your spleen was removed.. it could be E beta Thal.. which is again another form of Thal major. But what happens in E thal is that the manifestation of the Thal symptoms come a bit late.. age 2-3 years.. or even later.. and these kids are able to lead a fairly regular life with a low Hb of 5/6.. but sooner or later they will need regular transfusions.. as it happened in your case.

Now when the spleen is removed, the first couple of years, the transfusion rate reduces drastically, when you might need transfuions every 3/4 months.. as you do.
But see.. how your Hb doesnt get stabilised.. I dont know how old you are.. but a Hb of 6-8 is really low... and because you are delaying for 3 months.. you are being given 3 units of blood .. and even that doesnt oncrease you Hb as it should.

So I think you should switch over to a 4-5 weeks interval beteen transfusions.. and immedietly start a combination therapy for reducing your iron levels with Desferal and L1

At first you will need to boost up youir Hb with 2/3 transfusions at a gap of 10/12 days.. with 2 units each time... and then continue the 4/5 weeks interval.
There are no set patern for transfuions intervals.. an individual just gets used to a certain pattern

An average 250ml packed cells should take 2 hrs if you are using the filter.. or it should go a bit slower.. maybe 3/4 hrs...

I dont know if any of this heps.. but in case you have any more questions.. please ask..

shikha

HI again Jonathan..
I see that you are 21 years old...
What I read about you.. is very very simolar to my daughters case..
So please do boost up your Hb first.. and thne try the 4/5 weeks interval..

But if you able to carry out your normal activities and if your growth etc is fairly good.. then maybe you cud stick to youir 3 months interval.. but  I woukld doubt that.

But do start the chelation therapy soon..( Ask your doc to see if you are Thalassaemia Hb E .. also called E beta Thal)

shikha

Hi Jonathan,

Shikha brought up an interesting possibility. HbE beta thal manifests anywhere from intermedia to major symptoms, so it is possible in your case. However, HbE is prevalent in Indians and eastern Asians. Do you have an Asian ethnic background? Another possibility is beta thalassemia coinciding with alpha thal trait. Alpha thal trait can moderate beta thal, resulting in a less severe condition.

Proper testing can determine your state.