Thalassemia Patients and Friends
Discussion Forums => Thalassemia Major => Topic started by: Sharmin on October 27, 2012, 11:40:52 PM
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In recent years the treatment and diagnostic procedures for thalassemia patients have improved drastically. At the same time, the understanding of thalassemia and its effects at a microscopic level are much better understood.
With new chelators, it is now possible to have iron levels very near normal - and there is no reason for children to have iron levels greater than 500. Oakland recommends between a range of 200 - 500 for children. Only 10 years ago - it was not recommended for thals to have iron levels below 1000 because of the toxic effects of desferal. It is now recommended that when a child's iron levels approach 200 chelation be decreased and as they appraoch 500 or 600 chelation be gently increased. A split dose - am and pm is safest and most effective.
This lower level of iron load ensures that iron levels are not to high to be contained in the liver - also constantly having a chelator on board binds free iron - free iron is what corrodes and causes damage to organs.
We also understand that free iron can cause damage at a molecular level - therefore having antioxidants on board at all times protects the body. IP6 is very effective at binding free iron. L-carnitine, vitamin B, (small doses of vitamin C), vitamin E and many others listed in the vitamins and supplements section of this board are very helpful.
Antioxidants also serve other very important functions in the body of a thalassemia patient. Antioxidants such as L-carnitine and vitamin e help remove debris in the plasma which can damage blood vessels, the lungs and the heart. Also, antioxidants assist in keeping the arteries elastic and healthy - which protects against Pulmonary hypertension.
Another important role for chelation and antioxidants is endocrine function - keeping iron levels very low in childhood and adolencense is key to healthy development. Protecting the pituitary gland from iron and free radical damage is very important - without hormones children cannot develop into healthy adults. Protecting endocrine glands ensures that the children will grow healthy and their fertility will be protected.
Another important factor is maintaining a healthy hemoglobin. Allowing the hemoglobin to drop too low is taxing on the bones, the heart, as well as all other organ systems in the body. Low hemoglobin levels also decrease sense of well being and quality of life. Personally, our aim is to prevent the hemoglobin from dropping below 10. A healthy hemoglobin also prevents the spleen from becoming enlarged. An enlarged spleen can lead to increased RBC breakdown - a greater requirement for blood and iron overload. It is also important, when possible, to preserve the spleen in children for normal immune function and prevention of dangerous infections and blood clots.
Because thalassemia has become a chronic, manageable disease and our goal has extended beyond survival. We must now strive for normalcy in health, ability, participation in all activities, life span, appearance and quality of life.
Properly treated, a person with thalassemia should not feel any different than anyone else - this is the goal for treatment of thalassemia today.
My best to everyone,
Sharmin
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Nicely said, Sharmin.
-P.
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Sharmin, that was a wonderful post! Every parent and patient should read that post every day. Thank you
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This thread has been "stickied" so that it will remain easily accessible.
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Very good post. Comprehensive details in one post :biggrin
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Very well summarized Sharni....Thank you for your post. We recently came back from Oakland and also given the direction to up the chelation along with splitting the exjade dose when the ferritin is around 500 to 600.
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Thank you sharmin
what a fine presentation !
Well done.
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Thank you for the positive feedback for the post. Hopefully we will continue to learn from each other's experiences. Thalpal continues to be the most important resource in my son's care.
In 2006, Lil A developed an autoantibody which would quickly break down transfused red blood cells. There was no way to screen the blood to prevent this hemolysis because the antibody indiscriminately destroyed all red blood cells in his body. He was at times requiring two transfusions a week. His doctor used prednisone, which is often the first line of treatment but soon that was not enough and iron levels began to build up. His doctor, going by old standards did not see a need for increased chelation because his 'ferritin' levels were below 2000.
Fortunately, at that time Andy recommended that we place Lil A on high doses of antioxidants and urged us to consult Dr. Vichinsky. Dr. Vichinsky immediately enrolled Lil A in a combination chelation trial (exjade and desferal). Within a year his iron levels decreased from 2800 to below 1000 - and eventually Lil A was maintained on exjade alone. His iron levels since that time remain at 400 or below. Two factors protected Lil A during that stressful time, firstly he had been well chelated since 18 months of age therefore the short duration of iron accumulation did not effect him. Secondly and most important, Dr. Vichinsky stressed that he was surprised at how little affect the iron had on him at all - this he said it was due to the high anti oxidant regimine that Lil A was on. Thank god for Andy advising us about antioxidants such as IP6.
Dr. Vichinsky also helped us deal with the antibodies. Lil A is now transfused every 3 weeks and his hemoglobin is kept at 100 (10.0). This is to optimize his growth and energy - to prevent strain on the heart, spleen and bones. I would recommend this for all patients, especially kids.
Another thing I want to stress is the need for constant chelation. Despite what some medical professionals are saying - chelation should never be stopped. It can be decreased to minute amounts spit into small doses throughout the day when iron levels are low but never stopped. Even when iron levels are very low (below 500) free iron can roam in the body and settle in other organs - and it can cause rustic damage.
In 2010 - little A's iron levels were very low so our local doctor took him off of exjade. It was reasonable to think that we would give his stomach a break because he often complained about stomach ache. A month later, an MRI showed that while there was little iron overload in the liver, no iron in the heart or pituitary there was mild accumulaton of iron in the pancreas and lungs. The average child does not have an mri of the entire body on a regular basis - we were lucky to find out immediately so we could rectify the situation. I stress that this was at a time when his iron levels were very low and we only held the chelation for just over a month. Since that time, we have NEVER stopped chelation.
When Lil A's iron levels are very low we decrease his exjade to minute levels (we always give him 1/2 the dose in the am and 1/2 pm) and if the levels seem to be increasing we increase the dose. During times of low iron load, the chelation serves not to remove iron overload - but to bind free iron which is corrosive no matter how much or little iron overload exists in the body. This must become knowledge for everyone. It is not just iron overload that kills - it is also the free iron which is present even when iron loads are low. Antioxidants and chelating agents bind free iron which protect organs from the free radical damage and settling of free iron into other organs. Dr. Vichinsky and the Oakland program endorse this method and we follow it completely. Lil A remains on antioxdants at all times and is chelated at all times. Dr. Vichinsky says that a transfusing thalassemia patient should not go for more than 24 hours with chelation. We ensure that Lil A gets a boost of chelation every 12 hours.
We are happy to let you know that recent endocrinology testing indicates that all of Lil A's hormones and endocrine system are completely normal - he is completely unaffected by the thalassemia. His pituitary volume, shape and function are completely normal. At one time this was unheard of in thals but today it is achievable for all thals. If this can happen for little A - even though he has had trouble with antibodies and at times has increased need for transfusion - other kids can definitely achieve this.
For these reasons I urge everyone to keep their focus on timely transfusion and constant, constant chelation. On top of this antioxidants are absolutely essential for the cardiovascular system, for iron damage prevention and overall well being. Antioxidants are not just a form of 'alternative health' they are part of the mainstream medical treatment of thalassemia.
Thalassemics tend to be deficient - and have a higher need for vitamins such as vitamin E, Vitamin C, vitamin D, zinc, magnesium - and would benefit greatly from supplements. This is stressed at all of the medical conferences regarding thalassemia and needs to be followed by patients. It is not extra protection, it is necessary for thals.
I hope that this is helpful, please talk to your own medical health care providers about what I say before following my advise. If your healthcare providers are not aware of these trends then perhaps you can contact one of the comprehensive care centers for advise. I recommend Oakland but there are many other centers as well. Regardless of what you do, individual patients need individual attention for their unique care and should be followed closely by their own doctors. It is possible for your local doctors to be in contact with larger centers to coordinate your care.
Best of luck to all,
Sharmin
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Sharmin,
You have followed the advice I have given faithfully and now we see that the reward is being told your child is normal. Thals are not usually told this and it's a tribute to a program of well managed care with additional emphasis on what patients can do for themselves, including faithful chelation compliance and utilizing a program high in antioxidants. Antioxidants prevent the damage that iron does and are 100% necessary for thals. I feel that we should be taking a more in depth look at the value of IP6 and its use not only as an antioxidant, but as an important chelation complement, that chelates where other chelators may not be able to reach. IP6 reaches everywhere, as it is already present everywhere in the body.
I am so pleased that little A is doing so well. This is wonderful news!
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Wonderful news, so happy for you, Sharmin!
However, IP6 is also a sort of chelating agent or helps in it?
I've asked for it in local store but couldn't find it, I'll order it online most probably then.
I cannot find it online also, can you brief me the common name or full form of IP6 so I could buy some?
-P.
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IP6 is an iron chelator and was the original reason I brought it to the attention of this group. It is also a powerful antioxidant that inhibits the actions of iron if the cells, preventing the oxidative damage that iron causes. Even in low iron thals, oxidation from hemolysis and unmatched alpha chains cause much damage, so antioxidants remain key even when the iron status is low. With the success of little A in keeping his pituitary normal, I feel we must re-emphasize the importance of high consumption of antioxidants like IP6 and vitamin E.
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Hi Andy,
Thank you.
I found it on seacoast at last & ordered it:
http://www.seacoast.com/ip6-gold-with-inositol/ip6-international/120-caps/p157
The tablet is only 16 USD but shipping costs me another 34$, making a total of 51$, too expensive. The shipping cost is twice as tablets. I'll still try to search here in local stores meanwhile if I find it.
-P.
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Moreover, you're correct. It's an excellent iron chelating supplement.
A very nice video I found explaining IP 6 and it's benefits, worth watching:
http://www.youtube.com/watch?v=zKVE_p1viMI
-P.
Interesting, look out at youtube video timeline on 5:01 where they discuss about defriprone, so I believe this is very much I needed. And I thank you god for making me visit Thalpal and find out more interesting ways to lead a better life.
Moreover it also promises to boost glucose tolerance (curing diabetes), so I think that can do wonders for me as well along with my iron being decreased, so it's a win-win situation. I will laugh at thalassema one day, ha ha ha.
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Moreover, you're correct. It's an excellent iron chelating supplement.
A very nice video I found explaining IP 6 and it's benefits, worth watching:
http://www.youtube.com/watch?v=zKVE_p1viMI
-P.
Interesting, look out at youtube video timeline on 5:01 where they discuss about defriprone, so I believe this is very much I needed. And I thank you god for making me visit Thalpal and find out more interesting ways to lead a better life.
Moreover it also promises to boost glucose tolerance (curing diabetes), so I think that can do wonders for me as well along with my iron being decreased, so it's a win-win situation. I will laugh at thalassema one day, ha ha ha.
Also Andy as I've mentioned they've discussed about Desferal and Defriprone in the video I mentioned above, so this 800-1000 mg tablet also I believe removes a lot of iron along with this chelator without any side effects, I believe?
Also I want to ask if whether 2 tablets/day would be appropriate I think?
-P.
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This is for You Pratik,
http://www.guardiannutrition.in/vitamins-supplements-best-vitamins-for-joints-gnc-inositol-500-100-tabs-p-297.html
I have bought GNC Natural Vitamin E 400 IU from this site. It is Indian site of GNC.
Hope it will help.
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That's just inositoll I believe.
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Yes, inositol is not the same. IP6 is inositol hexophosphate or phytic acid.
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Dear All,
I am a new member, my son Ryan is 2.5 years old and is having Beta Thal Major. He was doing preety good all this time and was maintaining a Hb level of 8.8, but about5 months back he had viral fever and since then his Hb level has fallen down to 7.2 and he is constantly keeping upto that. He is otherwise preety active and his growth is just okay. I want to be guided as to when is the requirement for transfusion?
I live in Kolkata, India and want to know about any care centre in the city where I can contact for the best advice. I want to give a normal life to my son and am ready to do all it takes.
Please guide me.
Regards
Benjamin Dcruze
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Hi Benjamin,
Has your son transfused before?
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Hi Andy,
My son has not been transfused till now, but last few months his Hb level has come down to 7.2, when is the correct time to transfuse.
Regards
Benjamin Dcruze
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Benjamin,
The standard rule to begin transfusions is two consecutive Hb readings below 7. From what you have told us, your son is more likely a thal intermedia than major, but may be an intermedia that will eventually require transfusions.
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Thanks for the support.
Regards
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Hi All,
What are the test which I can do for my son to ensure that his liver, kidney and spleen are funtioning properly. The local doctor here does a check though by touching his stomach.
Regards
Benjamin
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The doctors try to feel the enlarged liver and spleen through physical examination of the abdomen. They press the abdomen and try to get a sense of how large the spleen or the liver has grown. One can feel the spleen when it is a little large. Some expertise is required to do a physical examination. Functioning of Kidney cannot be ascertained by physical examination of abdomen or the back.
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Hi,
I would like to know more about developing antibody. Jia, my son developed an antibody 3 years ago and now has to have blood with Kell group antibody. This has limited his chances of getting blood easily. However, we haven't had any issues yet, thus far.
On another note, Jia is now playing badminton 3 times a week. I find that he is now capable of doing serious exercise. Should there be any limitation to how much sports he can do? He was just told to avoid contact sport as much as possible due to the difficulty in getting blood. Otherwise, the hospital hasn't advised us against sports at all.
Hooi
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The only limit is what the Hb level puts on a patient. Contact sports should be at a minimum to avoid accidents that could cause broken bones. If he has the energy, let him get as much exercise as he can. Exercise helps to optimize the oxygen available, so it is a huge positive for thals.
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I am curious to know how closely most thals follow the reccommendation of no contact sports or similar. I had some discussions with a mother to a young lady- alyssa that was featured on Cooley's anemia foundations website. She mentioned that her daughter lives a completely normal life outside of her monthly transfusions and at age 25- earlier in life she regularly participated in cheerleading, her daugter being one of the girls thrown up in the air. I know that was slightly inspiring to me as I have aspirations of getting my son on a snowboard as soon as he learns to walk. Thoughts?
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I am curious to know how closely most thals follow the reccommendation of no contact sports or similar. I had some discussions with a mother to a young lady- alyssa that was featured on Cooley's anemia foundations website. She mentioned that her daughter lives a completely normal life outside of her monthly transfusions and at age 25- earlier in life she regularly participated in cheerleading, her daugter being one of the girls thrown up in the air. I know that was slightly inspiring to me as I have aspirations of getting my son on a snowboard as soon as he learns to walk. Thoughts?
I too saw that article. She also goes to Gym if I'm not wrong and shes beautiful!
Modern definition of thals has changed. I'll be now starting gymming as well and I lift weights at home since past 2 months or so, and it has only made me stronger and slightly bigger. CAF also stresses the point to let your child indulge in physical activities, and after he grows up enough old (like 16 or so), he/she should also try to lift light weights or do anti-gravity exercises like pushups, pullups which exerts resistance. Doing so, the bone mass will increase and will only make your child grow stronger.
I myself was a badminton player and represented school until my 9th grade.
So don't let your child hold back, let him fly!
-P.
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So good to hear Pratik.... As you can imagine, as father there is so much that i am looking forward to sharing with my son and i do not intend to hold back any more than i have to. I plan to give him the best and most memorable life there ever was. At nine months we already share so much together I can only imagine what the future holds for us!
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Dear Mike,
If a thal major is properly transfused he will not have an enlarged spleen and contact sports should be fine. My 14 year old son plays ice hockey, soccer and basketball - and we have never limited him from anything. He skiis and will begin snowboarding soon as well. He has always been an excellent athlete - his coaches are not aware of his thalassemia.
I hope that this addresses your concern,
Sharmin
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Sharmin,
it is soo good to hear about little A and his achievements. He is such an inspiration for all the parents like me.
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Thanks so much for sharing Sharmin!! Your little one's achievements are incredible. Makes a parent like myself feel so much better!
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Bostonian,
Thank you so much - I hope your princess is doing great - these children are all so inspiring.
Mike, you have every reason to be optimistic :)
BTW Lil has started snow boarding and the instructor was very happy with his progress in the first few lessons. There is nothing that your child should be held back from.
Best,
Sharmin
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hey Sharmin
thats a very good info....but as antioxidents what do u gv him....???
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hey sharmin,
would u plz elaborate on the split dose-am pm. does that mean dividing the total prescribed intake of medicine into two times a day??
as for me, currently i have been taking 1800 mg of exjade as per my bodyweight which is 52 kgs. and my ferritin level has quite recently dropped to 600.. wat would be the ideal thing to do?? should i consider reducing the dose or after it drops to 200-300??
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hey sharmin,
would u plz elaborate on the split dose-am pm. does that mean dividing the total prescribed intake of medicine into two times a day??
as for me, currently i have been taking 1800 mg of exjade as per my bodyweight which is 52 kgs. and my ferritin level has quite recently dropped to 600.. wat would be the ideal thing to do?? should i consider reducing the dose or after it drops to 200-300??
Hello Kanika,
Split dosage can be taken with a gape of 12 hours e.g. 8AM-8PM. It means half dosage in the morning and half dosage in the evening.
Your ferritin level is good.
Have u had ur T2*MRI? It shows true picture of Iron stored in the body and help in setting correct dosage.
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I just have a question regarding how low the Hb should be before transfusion. Over here in Perth, Australia, they would now transfuse my son if he is 10. He's pre transfusion Hb count is usually between 7.8-8.8. I feel that he really suffers when his Hb count drops to 8.0. He gets his transfusion every four weeks, on 375mg exjade and ferritin level is around 1300. My son plays badminton seriously. He can only join tournaments based on his transfusion date. The latest he could play properly (although I feel there is already danger in it) in a tournament was at 3rd week of transfusion. He did come 2nd on local junior tournament for singles, and first for doubles. In singles he loss to his doubles partner. His friend knew he was already low on blood and couldn't push it any further. Even his coach asked if we could bring the transfusion date forward, but we all know there is no such chance. I wasn't at the tournament, but my husband said that he thought my son had stopped breathing momentarily during the finals of the singles match. Is it advisable to let him continue with the sport?
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Pre-transfusion Hb should be 9.5-10. If he is kept above that level, he shouldn't have problems with the sport.
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i would like to ask what is IP6? i m from Greece and i have not heard of this, can we take this (thal major)? and if yes is there a link where i can buy it from online? what exactly does it do?
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Hello Aus,
Can't your son have transfusion every 3 weeks to maintain a Hb of 9.5 and above? Here is US, many hospitals prefer every 3rd week transfusion to make sure Hgb remains above 9.5.
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Hi Katerina,
IP6, Inositol hexaphosphate is from the B vitamin family. It is a mild iron chelator, it helps to protect against damage from iron and it is a powerful antioxidant. It can be purchased in capsule form from vitamin companies. I recommend it as a supplement to chelation drugs.
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thank you Andy :) i will try and find where i can purchase them from here in Greece
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Thanks Sharmin for your nice post.All are manageable but how to manage if transfusion need after every 4 weeks to maintain hb 9.5 to 10?How my baby will suffer it?Its really very hard to accept.
How the hb will raise in future ?All of us need the solution...
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IP6 is an iron chelator and was the original reason I brought it to the attention of this group. It is also a powerful antioxidant that inhibits the actions of iron if the cells, preventing the oxidative damage that iron causes. Even in low iron thals, oxidation from hemolysis and unmatched alpha chains cause much damage, so antioxidants remain key even when the iron status is low. With the success of little A in keeping his pituitary normal, I feel we must re-emphasize the importance of high consumption of antioxidants like IP6 and vitamin E.
Is there any local importer in Bangladesh, Dhaka for this? Since its herbal i would love to try it out.
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Hi Andy
Why don't doctors tell us these things or recommend anti-oxidants to thal patients as a gold standard???!!
I am interested in doing some anti-oxidant so which is the one to go for - IP6, Vit E, Vit C? Which one has the greatest benefit?
What is your take on the benefits of Vit B complex for thal patients?
Thanks
Khwahish
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Doctors learn very little about nutrition in medical school. I have the benefit of a long history of interest in my personal nutrition, so I had a good understanding already in place when I began to learn about thalassemia, and have greatly expanded that knowledge, and much that I learned, I learned from listening to Dr Eliot Vichinsky, who does stress an overall approach to thalassemia treatment, that includes proper nutrients.
Because you have thal major, you have to limit your vitamin C daily intake, as C frees up iron, which helps with chelation, but when you free too much at once, it can reduce heart function. This is also affected by the iron load. If your iron load is high, no more than 250 mg of C should be taken daily. If the iron load is under control, <500 ferritin, higher amounts of C can safely be taken. I do recommend that all thals do get some C, so get some daily. Vitamin E is very important for thals, as it is a powerful antioxidant, but it also has a blood thinning property that is very useful for thals, whose blood contains a lot of "junk", including alpha globin tetramers from the alpha globin unmatched by beta globin, and the results of the ineffective erythropoiesis that takes place in the bone marrow. Vitamins C and E have a synergy, with C being able to restore the antioxidant properties of E, even after it has already been used. IP6 is a good antioxidant and a supplemental iron chelator. It is useful in cleaning iron from everywhere in the body. I would take vitamin E over IP6 as an antioxidant, but would not reject IP6, as its iron removing properties are quite helpful. I especially think that young thals could benefit greatly by taking IP6 to help keep their pituitary gland free of iron.
I think B Complex has an important role, as there is a high turnover of nutrients in thals from the overactive bone marrow activity. I do think folate and B-12 should be supplemented beyond what is found in a B Complex supplement.
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Hello Andy,
My 3 yrs old babies ferrittin is now 1874.She is taking daily 125 mg Vit C.Is it safe or not?Taking other supplements also Vit E, Zinc, B complex and Folic acid.
Please advice
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That amount of vitamin C is safe, but don't give her any higher dose until she is bigger.
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Thanks Andy :)
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Hi everyone,
I am Jia’s mother. In Perth, Western Australia, we have changed from exjade to Jadenu A few months ago. I have a few of exjade pills left. They are mainly 500 g pills. It would be ashamed to throw them out. Is anyone still taking exjade? Can I send them to someone.
Regards
Hooi
hooi@nw.com.au
61 402910884
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If it's only a small amount, it may not be cost effective to mail outside the country. Postal rates have gotten ridiculous.
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Doctors here at a hospital in Pakistan are fine with HB 9 but they do not transfuse at 10. I usually take my kid for transfusion whenever it get slightly below 10.. such as 9.8 or 9.6 and sometimes it get fell as low as 9.2. Is this an optimal transfusion?
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Kaleem,
It's close enough to 10 to be optimal and is a good strategy if they won't transfuse at 10.
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Hello,
Are the IP6 tablets large or easy to swallow? This is for my 11 year old who prefers tablets over the drink.
Thanks!
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Also, what’s the dosage for a 25kg person? Should I start my daughter on 1 capsule a day?
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IP6 caps are typical size for a capsule. I would suggest one capsule per day and see if it is well tolerated.