hey guys need help

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hey guys need help
« on: November 01, 2009, 07:12:49 PM »
hi sharmin,manal,Andy ... how r u guys , doing a great job  realy proud of u guys , i have question if rectic count is in  little bit more then normal range what does that mean
 as doctor suggested to do bone marrow testing to check the retic count, am curious what is it actualy n what kind of role it plays in being major
 once again need u guys help
hope u all will reply in the same high spirit as u all did help out earlier
 regards to all
tk  me waitingg
bye

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Offline Sharmin

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Re: hey guys need help
« Reply #1 on: November 01, 2009, 07:22:54 PM »
Hi Ash,

retics are immature (precursors of) red blood cells.  Thalassemia major patients are often hypertransfused so that their bone marrow does not attempt to make retics, because although the bone marrow attempts to retic it is not able to make viable blood.  Bones and spleens of undertransfused patients often expand in an attempt to make retics.  In response to low blood counts, the bone marrow "thinks" that by increasing its surface area it can make more red blood cells.   By keeping the hg higher - above 90 (now docs think it is better to keep it even higher) - this can be prevented, therefore allowing the bone marrow to remain suppressed, the spleen to remain normal in size and the body to be better nourished with blood and oxygen - for optimal function.  

It is important to know if the patient is beta zero (very serious deletions or mutations in the beta gene) - in which case you want to prevent the bone marrow from "kicking in" because a few negative affects can occur as a result - bone damage, changes in facial features, *enlarged spleen, and the retics can cause clotting in the blood vessels.  For this reason it is important to keep pretransfusion hg higher.

If the patient is not beta zero, and the retics are resulting in viable red blood cells then it is worth considering if the patient is a thal intermedia and if a hg inducer may be worth trying in place of transfusions.

This is my understanding, perhaps one of our other members can provide a more thorough explanation,

Play keep us posted and let us know what the docs say after bone marrow testing,

Best,

Sharmin

*remember also, an enlarged spleen means more hemolysis, increased transfusion requirement, increased iron load and increased requirement for chelation.  It is better to transfuse adequately in the first place.  It is ideal these days to keep the spleen small, and to not have it removed because long term studies indicate that removing the spleen may lead to long term complications.  
« Last Edit: November 01, 2009, 10:51:55 PM by Sharmin »
Sharmin

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Offline Manal

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Re: hey guys need help
« Reply #2 on: November 01, 2009, 11:08:40 PM »
Hi Ash,

I agree with Sharmin.

What is your Hb level before transfusion and how often do you transfuse.

Thanks for your post and keep updating

manal

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Offline Zaini

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Re: hey guys need help
« Reply #3 on: November 02, 2009, 05:43:45 AM »
Hi Guys,

Ash is talking about her infant daughter here,when she was here in Pakistan (she was visiting here) her daughter was diagnosed as thal major and was transfused twice,now that she is back to Abu Dhabi where she actually lives,doctors there are not ready to transfuse her daughter yet,they are doing some tests and Ash is worried coz her daughter's hb last time checked was 8.6,but the doctors are saying that her retic count is in normal range i.e 1.4 % ,so they are going to do a bone marrow test to determine whether she is a major or not,she is just six months old as i remember so they have repeated the electrophoresis too.She messaged me about retic count and i was really confused as to what should i make out of this reading because she is so young and been transfused just twice,so its not like bone marrow has just shut down,so it is producing Reticulocytes,is it good they are in normal range,does it effect her thal status?

Zaini.
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Offline Zaini

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Re: hey guys need help
« Reply #4 on: November 02, 2009, 06:54:20 AM »
One more thing ,her daughter was transfused two months back and her hb is 8.6,it seems like an intermedia to me,what do you guys think? Is it ok to do Bone marrow test on an infant?

Zaini.
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Offline Andy Battaglia

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Re: hey guys need help
« Reply #5 on: November 02, 2009, 04:08:22 PM »
At this age it is often difficult to tell if it is intermedia or major, hence the bone marrow test to analyze what is happening in the bone marrow.
Andy

All we are saying is give thals a chance.

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Offline Zaini

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Re: hey guys need help
« Reply #6 on: November 03, 2009, 06:11:23 AM »
Thanks Andy,

She was really worried about it,now i'll inform her.

Hope you are feeling better. :flowers

Zaini.
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