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Skeletal changes by low hbg

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Dori:
Dear forum,

I think a few of you know that I don't have thal., but an other are hemolytic anemia called pk (d).
We have English forum and I need your help.

Parents of a young boy are asking about skeletal changes caused by a low hbg. Their son get an transfusion by 6. A lot lower than the level of you all keep. In general I see that people don't undestand why you should stay at higher level (by our disease). Our treatment is the same as you, but a lot of people are uninformered. I am happy to have a doc (since 1,5 year) who treat me as a patient with thal.

I know what a low hgb does with your bones. Your bone marrow has to work harder. The bone marrow is in your bones, so the bones are growing. Right? Your english is probably better than me and that's why I ask for help. My jaw looks a lil weird, but I am probably the only one who have noticed that ;).

Thanking you in advance,

Dore

Manal:
Dear Dore

When the Hb is low, the bone marrow inside the cavity of our bones starts working very hard in attempt to produce more blood to increase the Hb. Working hard, makes the bone marrow increase in size. When the size increases, the cavity of the bones become small for the big size the marrow which starts to make pressure on the bones making them brittle ( that is why having calcium daily is very important) and also causing them to chnage resulting in bone deformity.

Also sometimes this increased in size marrow comes out of the bones causing something called extramedullary hematopoesis which cause pressure on the organ it is created around. Usually this mass is decrerased or controlled in size when regular transfusion starts and when Hb is always kept at high levels, not less than 10. Sometimes medicines like hydroxyurea are used to supress the bone marrow, leading to a decrease in the  extramedullary hematopoesis and if it does not work radioactivity is used to decrease or control this mass, butusually regular blood transfusion solves the problem.

Children should be monitered for growth and  bone deformity to avoid bone complications. Also bone age should be done too.

But i too have a question: Is bone deformity reversible when transfusion starts or it depends on the place that the deformity has occured???

Thanks

Manal

Zaini:
Manal,

I don't think bone deformity is reversible,i have seen so many patients who started chronic transfusions but their boes didn't change shape.let's see what Andy has to say.

Zaini.

Dori:
Thank you Manal and Zaini!
I copied your message and post it on the pkd forum. I hope it's ok. You are personal translator :)

Manal:
You are more than welcomed Dore :hugfriend

manal

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