Thalassemia Patients and Friends
Discussion Forums => Thalassemia Major => Topic started by: Emby on June 02, 2009, 08:45:13 PM
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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
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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
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.
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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
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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.
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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 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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hi,
Andy, Umair and Zaini thank you for your reply which i found very helpful. i will talk to the consultant about it. i hope you are well yourselves. i feel you have a great thing going on here and am glad to have found you.If i have any more concerns i will contact you again.
emby
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Canadian_family; Thank you for your information
Emby
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Hi Emby, Welcome to the family. :hugfriend
I agree with my fellow thalassaemics in regards to the hb count not going below 9.5
I live in New New Zealand but was given the opportunity to go to Australia and meet fellow thalassaemics and a professor Bowden. While there we talked about how often I should be transfused. I was told that is each Thalassaemic should be judged on each individual case as we are all different but that the hb should not drop below 100 which I think is 10 or 9.5. Frequency should be based individually as our body's requirements are different. I was also told that if we go too long between transfusions and our body needs the blood, our marrow tends to over work trying to make the blood we need. This in turn can lead to abnormal bone expansion.
I'm not saying this would happen but it can be another talking point with your haem to express your concerns and questions. We all deserve better quality of life and sometimes this may require either transfusing week early or having that extra bag sometimes we need to make that choice. At the end of the day the doctors can go home and forget about thalassaemia, where we still have to deal with it.
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hi cherieann,
thank you for replying. i absolutely agree with you. i believe that living with thalaseamia you develop gut feeling about things.....i mean some health professionals want to start different treatment perhaps to put their 'stamp' on it and seem to class thelaseamia in the same place as common aneamia. i think it is absolutely important that the main consultant for whatever sickness is a specialist on that condition so that by them being well informed we could put 100% confidence in the treatment they give.
recently i feel just that, that they just ''go home''. we as parents have had to do alot of ''homework'' and i feel lately we have had to dispute alot of the way my children have been receiving some of their medical treatment.
anyway i put my trust in God that all will work out for my two kids and all of you...
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Hi Emby.
I don't know what country you are in and I don't want to make things difficult for you but if you feel you need to get a specialist involved why haven't you? As a mother you have a right to question and to make informed choices. If you feel you are being informed, why dont you ask the doctor to request an appointment with another haematologist.
They don't normally like that but as a parent you need to have a relationship with your children's doctor so that you can both work together for your children's sake.
I am different in the fact that my relationship with my specialist is an understanding that I can participate in the clinical mangement of Thalassaemia not as a parent but as a patient.
Sometimes doctors do not always listen or hear the fears that you may have and although you are speaking, their ears aren't turned on. You are in a hard place at the moment but I feel that if you could say how you feel and what you would like and maybe together you and the specialist could come to a mutual agreement. I don''t know how your children are feeling but I know that when my transfusion nurse didn't like my mum coming with me to transfusions, I got very upset and resented my nurse alot. Health professions need to understand that although they work with the disease, family and friends still need to be there for the wellbeing of the patient.
I wish you luck and your children a long life of love, health and happiness.
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hi cherieann,
i'm from the uk. i have usually had good understanding with the previous consultants but i must say that this new consultant had arranged my children to have t2 scans which revealed a significant amount of iron around the heart. they always took desferal without missing a single dosage, perhaps only occasionaly, the consultant then arranged for appropriate treatment with their chelation and so for that reason i feel that God led the consultant into my childrens lives at the right time....perhaps the other issues are just ''teething'' problems which one might expect with new consultants. anyway we have a clinic appointment very soon and i'm hoping all will be well. i must add that we are usually always involved in all their treatment but as i have ''grown'' with thalaseamia, i feel i have learnt what to question when previously i had no idea. i think it is alot to do with forums such as this.
i would like to ask that my boys both take ascorbic acid 100mg, should they be taking other supplements as well and for what reasons?
thanks and take care...by the way you look fab in that pic!
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Hi Emby,
Yes thals can take Ascorbic acid if their ferritin is not too high,but let them take it between meals and not with meals,as it helps absorb iron from food.On the other hand it is advised to take Vitamin C half an hour after starting desferal because it will release iron in blood and will help desferal remove more and more iron.
Antioxidants can play a vital role in keeping thalassemics healthy,i would suggest you start giving your sons IP6,which is Phytic Acid or Inositol Hexaphosphate,it a very strong antioxidant and a natural chelator,it is already present in the body and is extracted from rice barn so there would be no toxicity issue.It will prevent any damage done to cells by free iron present in blood.
I hope your sons take tea with meals,if they don't try to make it a habit as tea inhibits iron absorption from food.
Are they taking calcium? Thalassemics are prone to developing osteoporosis,so they should be taking calcium,Magnesium helps strengthen walls of Red blood cells,infact the best supplement i would suggest is Osteocare,it contains calcium,Magnesium,Zinc and Vitamin D.You can order it from www.vitabiotics.com.
May i know what are your son's ferritin levels,because if there is any iron present in their heart you might wanna consider IV Desferal.
Hope this helps :) .
Zaini.
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Emby,
Are your children only using desferal? Has combination chelation using desferal and Ferrirpox been suggested? Ferriprox is far more effective than desferal for removing iron from the heart and the combination with desferal is the best known chelation method. Also, according to the guidelines from Oakland, it is only when you try to maitain an Hb above 10 that there is a problem with more iron being taken in. Maintaining above 9 can be done with the same transfusion frequency once the HB is raised to this point. I don't think it would require more blood in the long term to keep the Hb from dropping below 9.
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hi zaini, andy,
thank you for info. will talk with consultant about it.
my youngest loves his tea. he prefers a nice hot cuppa than somthing fizzy with a chicken burger!
as for their ferritin levels, eldest- 260 and little one 740.
please remember them in your prayers.
andy, my eldest is on combination therapy (desfrl & feriprx) and hoping , God Willing, to start little one with same. they are also expected to have a repeat t2 scan done pretty soon. i pray that the iron levels (heart,liver) will be more acceptable, God Willing.
keep well andy.....and zaini and your little babe.
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Their ferritin look pretty good. Yes. both of them.
I wish them tons of luck with all those treatments.
to them: :hugfriend
Best wishes,
Dore
p.s. There was a time I had contact with dr. Shah. I really like her. As for the T2*: it's not avaibable in the Netherlands!!! (the same for SQUID.
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Emby,
The ferritin readings are good but as you said, there is iron around the heart. Ferrirpox will help clean this out. This is the one problem with relying on just desferal. It is not the best iron chelator regarding the heart. Ferrirpox is superior and this is why it is often used in combination with desferal. I think your sons will both show improvement with their next heart scans.
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Emby,
The numbers are really really good,congrats on keeping them well chelated,still iron around the heart issue is amazing,atleast with your elder one because he is taking ferriprox which is supposed to remove iron from heart,it make me think that thalassemia can be tricky,we keep relying on ferritin readings and in the end that's not enough.
Wishing you luck with T2*,and lots of love for your young men :) .
Zaini.
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by the way you look fab in that pic!
:rotfl that was a good day. I was holiday.
I can see that Zaini has answered your question so I don't need to say anymore.
Just remember we are all just a keyboard away.
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hiya,
dore, thanks. i hope that a t2 scanner will be available for you very soon.
andy, zaini...i feel the best way forward is combination therapy (desferal and ferriprox). i am aware of the risks involved with ferriprox and we continue to monitor this weekly with blood tests. my oldest, thank God, is tolerating it very well........you know..i had a queasy feeling in my stomach that after all those years of desferal....and being really strict about it...their bodies had plans of its own! although there may be more medication to take and as much as i hate all the needle and stuff, i feel this is definately the best form of treatment...and, most importantly, the boys feel the same.
my oldest has been on ferriprox for a lttle over 2 months now and i hope, God Willing, it has made a difference.
i feel everyones body of coarse has its own level of tolerence. there is a 50 year old man in london with thal major who is of normal height and build, is sooo young looking, married with children, a boy and a girl, both completely healthy. he was on and off with desfearal for some time and i think he now takes oral med but i'm sure that he did not have t2 or dexa scan back then but....hey!...hope and inspiration all around!
cherieann....nice to know you're all a keyboard away.....but the time difference thing.....have to wait tiil next day for reply!!
i pray God keep you all in good health
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I know the time difference can be annoying but I am so wrapped to finally have a place to come to.
I can still remember the times before internet where I had no body to confer with and know that it was just me and the haematologist. Back then it was unheard of in New Zealand. My blood was sent around the world to find out what I had. Plus the haematologist reminded me that it would be her taking me as far as her knowledge. :-\ I really was a guinea pig. :rotfl
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Cheriean,
it mean's u can say that your blood visited many countries :biggrin wow Tourist Blood Sample :biggrin :grin
Umair
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Tourist blood sample :rotfl
It probably rode on Air New Zealand then Quantas, then Polynesia Airlines, then American airlines and then returned home on Emirates. :rotfl (only a guess) :grin