Discussion Forums > Thalassemia Major
some ques about thalassemia major
§ãJ¡Ð ساجد:
--- Quote from: Nathan899 on December 31, 2007, 02:32:21 PM ---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.
--- End quote ---
Jonathan, are you sure they removed the "Pancreas". Normally it is the "Spleen" that is taken out. :biggrin
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.
Nathan899:
I'm sory sajid yeah it's the spleen they removed.
Thanks for your help,
Jonathan
--- Quote from: §ãJ¡Ð ساجد on December 31, 2007, 03:18:57 PM ---Jonathan, are you sure they removed the "Pancreas". Normally it is the "Spleen" that is taken out. :biggrin
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.
--- End quote ---
Andy Battaglia:
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.
Shikha Mitra:
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
Shikha Mitra:
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
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