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hi new here and need some advice

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Emby:
hello,

i am new here and hoping you could advice me on something.  i have 2 children with thal major, one is 16 the other is 12.  they are both really well.  they have regular blood transfusion 4 weekly and both are doing well on chelation with the older one on deferiprone and desferal and the little one on desferal only. 
i would like to know if you could advice me how much blood should be given so that they could  continue to stay healthy.  their specialist previously used to give blood according to this formula
[desired hb, 14/14.5 - actual hb] x weight x3 and they have been well with this however they now have a new Consultant who works it out with the desired hb of 13.  the result of this is that they both now have much less blood than before.  the Consultant said she is doing this for all with thal major because less blood would mean less build up of iron.  both my childrens hb would previously maintain at and above hb of 9.  now pre transfusion hb is 8.8 or lower.  is this healthy ?
i am worried that their bodies would need to 'work' harder with less transfused blood and that it would affect their growth in the long run.  the consultant says she is following the national guidlines for blood trasfusion for thal major uk, can you confirm if this is the process applied nationally and internationally ?
i would be really grateful for your reply.

Emby

Andy Battaglia:
Hi Emby,

The official policy from the Standards of Care Guidelines for Thalassemia is to never allow the Hb to drop below 9. In conferences, I have heard 9.5. I realize we want to minimize iron intake but we also want patients to have enough blood to function normally without fatigue. With good chelation, there really is no risk to maintaining a higher Hb level, and this has changed the acceptable levels over the past 10 years. In the past, it was common to allow the Hb to drop to 8 before transfusions but the patient does not thrive physically at this level, so with chelation advancements, the acceptable pre-transfusion level has been raised. If your sons complain about fatigue before their next transfusion, I would suggest aiming a bit higher. In the long run, this will not require extra blood, as it is simply a matter of starting at a higher level to begin with.

http://www.thalassemia.com/documents/thalhandbook2008.final.pdf

--- Quote ---The amount of blood received on transfusion day is
determined by pre-transfusion hemoglobin levels. The
target is to maintain the pre-transfusion hemoglobin
level between 9 and 10 g/dL. Attempts to maintain pretransfusion
hemoglobin at above 10 g/dL increase transfusion
requirements and the rate of iron loading....post-transfusion hemoglobin should not
exceed 14 g/dL.
--- End quote ---

nice friend:
Hi Emby ,
 :welcome2 , i hope you will find all ther answers/solutions of you concerns/questions ..it ur forum so sont hesitate to share wat-ever you wana share with us and dont hesitate to talk about anything you wana talk here .... feel free to ask any question that you have ...

Best Regards
Umair

Zaini:
Hi Emby,

Welcome on the forum,your concern is right,pre transfusion hb should never go below 9,personally i try to keep my daughter's hb between 9.5 and 10,she also gets her transfusions 4 weekly,especially when kids are at age when they are about to hit puberty,their hb levels should be kept as high as possible and ferritin as low as possible,so that they'll have no issues with fertility in future,i hope you'd be able to convince your doctor :) .

Zaini.

Canadian_Family:

--- Quote from: Zaini on June 03, 2009, 08:37:56 AM ---especially when kids are at age when they are about to hit puberty,their hb levels should be kept as high as possible and ferritin as low as possible,so that they'll have no issues with fertility in future.


--- End quote ---

I think its a fair assumption, however fertility issues will not occur, its not a guarantee. Please read below excerpts from Northern California Comprehensive Thalassemia Center.

Causes of Reduced Fertility
Fertility is influenced by the ability of the female egg or male sperm to mature and be fertilized. In females, it is also influenced by sexual maturation and the ability of the uterus to carry a pregnancy to term. Delayed sexual maturation can preclude the ability to have biological children until puberty is reached and, for girls, menstruation has begun. Some women with beta thalassemia have primary amenorrhea (menstruation has never started). This must be corrected and menstruation initiated in order for such a woman to conceive and carry a pregnancy. The same is true for secondary amenorrhea, in which a woman who previously menstruated no longer does.

Prevention of reduced fertility

Reduced fertility in the individual with thalassemia is mainly attributable to iron overload in one or more of the organs or glands that contributes to egg or sperm development. In females, eggs in the ovary ripen in response to hormones released by the anterior pituitary gland. The pituitary receives signals to release (or stop releasing) these hormones from the hypothalamus, which in turn receives signals from the ovaries. In males, sperm is produced in the testes. Like the ovaries, the testes receive hormonal signals from the pituitary, which receives signals from the hypothalamus. Fertility can be reduced by iron overload in the pituitary gland. The damage that results prevents the release of pituitary hormones in response to signals from the hypothalamus. Iron overload can also occur in the hypothalamus. Infertility may result when iron overload in the ovaries or testes causes damage to the egg or sperm cells.

It seems that the best approach to trying to prevent reduced fertility or infertility is controlling iron levels. The body is unable to get rid of the excess iron it accumulates from chronic blood transfusions. Desferrioxamine (Desferal) helps to remove this excess iron. This medication is usually administered five to seven nights a week through a pump that slowly infuses the desferrioxamine beneath the skin over several hours. Studies suggest that effective use of desferrioxamine can lead to normal sexual maturation. Patients who do best are those that start treatment early, before iron levels become high, as measured by increased ferritin levels. Those that keep their iron levels low throughout treatment also seem to have a better chance at preserving their fertility. However, even those with very high ferritin levels over a long period of time can experience normal sexual maturation, although rarely. This is seemingly counter to the proven positive effects of desferrioxamine therapy. It is important to remember that ferritin values are not an absolute indication of body iron burden, as these values can be affected by other conditions, especially liver disease. The only positive method measuring iron deposition is a liver biopsy.

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