Thalassemia Patients and Friends
Discussion Forums => Thalassemia Major => Topic started by: Emby on September 21, 2009, 09:25:35 PM
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hiya andy,
how are you? hope you are keeping well.
i would like to ask something. my older son recently had his second t2 scan which revealed that the iron around the heart has gone from severe to moderate and the liver from moderate to totally clear of iron. he was on combi of desferal and ferriprox. his ferritin is 147. he has now been told to not have desferal but to increase ferriprox from 9 tablets to 12 to be taken every day. he weighs 60kg.
what is your opinian?
looking forward to hearing from you.
emby
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Hi Emby,
It's a good improvement but there is still work to be done in the heart and Ferriprox is known for its ability to remove iron from the heart. Desferal is known for having a better ability for removing iron from the liver. Now that the liver iron is gone, the need for desferal is not currently there, as long as another chelator is being used. Even if it turns out that he does have to return to desferal later on, it is good to have a break, as all chelators have side effects and desferal's side effects are more of the long term use variety, so taking a break whenever possible is a good thing. The Ferriprox can work on the current problem and desferal isn't really needed, so I do agree to raising the Ferriprox dose and attacking the heart iron. I am sure he will also appreciate the break. Explain to him that it may or may not be a permanent break from the needle, so that if he does have to resume later on, he won't be so disappointed. As long as he doesn't have any problems using Ferriprox, it is preferred.
I have heard many doctors express a belief that no chelator should be depended on for an entire lifetime because of the long term effects, so switching to another chelator is something most patients should consider at some point during their lives. As more chelators are approved, this does become much easier. Since heart failure is the number one cause of death in thals, a chelator that works better in the heart is important and Ferrirpox does provide this. I am hoping we see similar results with long term Exjade use, but right now we can only be certain about Ferriprox.
I would also recommend that you make sure he is getting plenty of antioxidants in his diet and supplements.
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hi andy,
thank you for your reply.
he is not bad with needles as long as i make up the mixture. he is still my baby.
yes it does make sense to use just the ferriprox just now also because his ferritin readings are within normal range and continuing with desferal could have toxic effects.
thank you again..i just needed reassurance.
emby
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I only want to mention what I have experienced:
I stopped desferal for a year long and took only ferriprox. At the end of the year, heart (already clear) was even clearer and liver (clear too) was clear again but less clear than a year before when desferal was used(together with ferriprox, of course).
You can make your own conclusions...
Lena.
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What Lena said is why I say to look at this as a break from desferal but not necessarily a permanent break. Depending on what happens with liver iron levels, desferal could become necessary again at some point in the future.
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That's right Andy. I am on desferal again and do not think of stopping it ever again, just lower it maybe.
I think it is necessary for the liver to keep it clear--that or exjade.
A mistake many thals do, is to over-evaluate things when stopping desferal. They think they get a permanent divorce from it and they get dissappointed when returning to it.
No, combination therapy is what makes things run.
Lena.
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I would like to stress on the importantce of doing T2* and ferriscan ( if available ) cause ferritin levels are not always a reliable mean of measuring iron overload in organs. We can have a normal level of ferritin as in Emby's son and at the same time iro is being loaded in the organs.
manal
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Manal is right,
T2* is necessary and doing it once a year is a must to check out the iron in the organs and reevaluate the chelation therapy.
There are occasions when serum ferritin is high and there is no iron concentration in the organs and the opposite can also happen, though I would say these are mainly exceptions. The rule is high sf leads to high organ iron and the opposite.
That is, at least,what I have seen happening.
Lena.
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Lena,
Do you know at what age children get their first T2* in Greece?
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Andy,
I think one of our "baby" thals of about 8-9 years old has done her first, but let me check it out and I will let you know for sure.
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Andy,
I just got by my doctor the info on MRI for kids. Here is what she says:
As MRI-T2 has no side effects or radiation, it is considered to be safe for kids as much as for adults.
The only thing that matters is the young one's ability to stay calm inside the mri machine and his/her fear when inside. Even a kid of 4 years old =if he/she is capable of staying motionless= can proceed to the MRI, if he/she can tolerate the whole procedure.
I hope everything is clear now.
Lena.
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Hi Lena,
Thanks for the info. Did the doctor say anything about at what age the T2* is first recommended?
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Andy,
MRI, T2* same thing. Since the kid is capable of staying still inside the mri mashine, it is recommended.
Age does not matter.
Lena.
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Hello Andy,
Just for information, I called Cooley's anemia foundation (few months ago) to arrange for T2* for Little Miss A, I think Elieen Scott (Patient Co-ordinator) is the person I talked to, and she said, in US there is no recommended age for T2*. However, the child needs to be atleast 6 years and able to follow the instructions (that is, holding the breath for certain time and exhaling at certain time). She said, every child is different and not many six year olds can follow proper instructions. So, like it depends when your child is ready.
Thanks
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Thanks Lena and CF. I get many emails and one recent one was asking when the child should have the first heart iron evaluation. I think from what everyone has said that probably 6-10 years old is the range. It may vary according to the patient's history of compliance.
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Just reading thru the post...My 3yr old got MRI and T2* done in Children's Boston when she was 2.5 yrs old for baseline heart and liver iron. Since she is too young, she had to be given anesthesia.
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Bostonian_04 i hope your little daughter is well. It is good to have T2* done at a young age for a child especially for a parents peace of heart and mind and efficiency in addressing any issues.
Hoping she would not need anesthetic in future.
Emby
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I want to follow this up with information acquired at the NYC conference. I posed this question to Dr John Wood, who is very cooperative at every conference and he told me that the goal now is to do the first scan one year after transfusions begin. This will usually mean the child has to be sedated, but the benefit of discovering any early iron loading issues far outweighs any concerns over the child's reaction to having to be scanned. This is just one area where there is a constant evolution in the thinking about how to approach various issues in thals and it's great to hear the doctors talk about the progress being made in understanding how to best manage thalassemia treatment. I know everyone is anxiously awaiting a cure, but we do have to deal with what is and having these improvements makes a great difference in the management of thalassemia, which leads to better quality of life for thals.
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Bostonian_04 i hope your little daughter is well. It is good to have T2* done at a young age for a child especially for a parents peace of heart and mind and efficiency in addressing any issues.
Hoping she would not need anesthetic in future.
Emby
Hi Emby,
Thanks, my daughter is doing well ..... she is scheduled for her annual CMR for T2* and liver iron (R2*) this December. Since she is a toddler, she would have to be anesthetized for the procedure this year as well. I guess this will be the case for the next few years as well till the time she grows up enough to lie still during the procedure ....
Bostonian
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Hiya Andy,
Yes the final decision is with the patient.....but then when i am making clear my decision why does my boys Doc look at me as if I'm from another planet !! :biggrin
Hope you are keeping well Andy. I know from some of your posts that you have been not too well......you are a diamond Andy (or are tanzanites worth more!?!) may God have Mercy on you and keep your light shining the brightest!
I need to ask something about our boys. They have both complained about this to their Doc. I would like to know your opinian. This morning my eldest got up with a slightly uncomfortable feeling in his chest. He went off to college as usual leaving us in anticipation of how went his first day of exams. He text us to say it went well except that he had an excruciating back pain. He was relieved to have made it through exam time. The feeling of tightness in his chest travels down to the lower back, causing it to pain and making it difficult to breath.
Their ECG has always come back normal and their cardiologist has said that this was nothing to worry about. Could this be just muscle spasms? what do you think? It always starts that way with discomfort in the chest receding to lower back with difficulty in breathing and pain that could last for anything upto 5 minutes to half an hour.
Would love to know what your opinian is on this.
Emby
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Emby,
Because of the history of low hemoglobin levels, I would suggest asking at the thal center about investigating the possibility that extramedullary hematopoesis has developed in the boys. What happens is that red blood cells grow outside the bone marrow and develop into masses that can press against the organs or the spine, and this can become painful. The best way to prevent this is by not allowing the Hb to drop low. Hydroxyurea is also sometimes used to shrink the masses. Ultrasound can be used to determine if these masses exist in the boys.
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And, I am doing much better. My shoulder and back finally healed and I re-did my work space at work so that I am no longer stretching to reach the keyboard on my laptop.
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Thank you Andy for a quick response,
Regarding extramedullary hematopoesis, would this not have shown up on the MRI scan they recently had? Also their DEXA scan came back as normal, would the masses on the bone not cause weakness to it? This pain thing happens to them only occasionally, if they had this would they be in pain most of the time?.......you know thal doesn't rule their lives, they have too much stuff to be getting on with but me.....I obcess about things briefly, deal with issues then let it go to the back of my head!!
Really happy to hear you are keeping well.
Emby
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It won't be revealed by MRI and the DEXA scan would scan only the boe. These masses grow outside the bone and tend to cause discomfort more in times when the HB is low and they expand. The symptoms fit but it is not the only possibility, but I would request an ultrasound by a technician who understands what to look for.
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Reading up on it, hydroxyurea is an option for treating it but radiation is a better form of treatment other than surgery which may remove the masses for sometime but may grow back again.....God Willing, I'm hoping it is not this......will ask for this to be investigated.
What do you think are the other possibilities?......the Doc thinks i worry too much (ask many questions) but there is a difference between worrying and keeping informed.....still..the Doc feels i worry too much..
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Radiation is usually reserved for severe cases where spinal pressure is painful enough to require action. The hydroxyurea can have the added benefit of boosting the Hb level and has been found to be very safe in the doses used with thalassemics. One other possibility is simply the pain associated with low Hb, as the bone marrow begins to expand to try to produce RBC's. This expansion is felt in the bones and can be quite painful. There are very good reasons to keep the HB above 9-9.5 all the time.
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Thanks Andy,
.......... :tired ...thats me at the moment though not quite flat on my face yet at 2am...then it will be :sandman
Who put together these smileys...some are hilarious...that great big syringe one...haha
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The smiley's were all added by Danielle, our co-administrator. We don't see much of her on the site these days but she still takes care of the technical stuff. One good note was she recently told me on Facebook that her ferritin is really low now that she's been taking Exjade regularly. Her T2* also came out good.
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Danielle has matched human moods and emotions perfectly with the smileys....i have read some of her posts..she has such a strong personality. She has been through so much in her life but carries herself with incredible strength...there are some very insperational people on this site.
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Yes, Danielle has had to deal with many serious health issues that have nothing to do with thalassemia, in addition to osteoporosis, which is thal related. She has also been busy with school as she furthers her career, so she doesn't have a lot of free time.
Facebook becomes a good way to keep up on a lot of people who don't visit here so often.
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Emby,
when did your son last do the MRI?
Lena.
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Andy,
Glad to hear Danielle is doing well,convey our best wishes to her :) .
Zaini.
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Lena....July/August '09.
They have had this sort of pain attack many times before this but when there are beads of sweat coming down their faces its definately something more than a sharp pain.
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Emby,
I also have extramedullary hematopoesis creating small masses with no pain and no side effects. I came to know about them through my MRI. A lot of thals have these masses but they keep them under control when transfused with high hb- above 11.
I am curious though about the nature of your son's pain,Emby...Please keep us posted.
Lena.
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Lena....it's possible to have this and not have any symptons? When exactly did you find out that you had this?.....and because this is common in people with thal, why isn't their Doc aware of this.....it's so good to know you are not effected by it.
I was grocery shopping this afternoon at a local supermarket, with my other half, and this striking woman, very smartly dressed, stopped with her trolley load to let me pass. She had a lovely smile warming her face...but i probs had a bigger smile than her because i had to stop myself from shouting out ''Lena....!!'' She was the spitting image of you!......strange feeling.....although i don't really know anyone here.... i feel i know you all.
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Extramedullary hematopoiesis can actually grow anywhere in the body, including at the cellular level, where it can be mistaken for lymphoma. The most common place it is found is in the abdominal cavity where it may be innocuous or depending on its position, press on organs or the spine. When the latter happens, it causes variable amounts of pain, depending on location and size. If they don't press on anything, one may never even know they're there. They are very common in thalassemia and as Lena mentioned, the best way to keep them from growing is by maintaining an adequate hemoglobin level. Our member Courtenay, who is a thal minor and had one of the most difficult thal minor pregnancies (the boy was born healthy and Courtenay gave me some credit for dong the research that helped save her life during the pregnancy), grew an extramedullary hematopoiesis that presses on her lungs so much that it created an asthma-like condition. Extramedullary hematopoiesis apparently has something in common with real estate because it's all about location. If it's in the wrong location, it can cause extreme pain, especially when it presses on the spine. This can be a dangerous situation and is when radiation or surgery is used to rectify the problem.
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Emby,
Are your boys treated at Whittington?
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Andy,
I am amazed! Is there something you are not aware of?
We all learn from you.
Emby,
I have these masses and I don't have any symptoms. If it weren't for the MRI, I wouldn't have known about them. According to what Andy said, this may happen.
As for my spitting image - I definitely want to thank you for having so beautifully described her!
Lena.
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Andy......we live in the midlands although i know London and then perhaps Birmingham have excellant thal centres, it is unfortunate we don't live there......everything has its reasons and God knows best.
Thank you for the imformation on extramedullary hematopoiesis. Hope Courteny had it treated and is okay now...may God have mercy on you and bless you for all the good that you do Andy.
Lena......perhaps she was you :biggrin
Its so sad what happened in Haaiti...thoughts and prayers with people in Haaiti or indeed anyone in any form of distress around the world...Allah for what You have taken from them give them a better replacement. Aameen.
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Emby,
Would it be possible to take the boys to Whittington for an annual visit. I'm not sure if it works the same in the UK, but in the US many patients travel to one of the Centers of Excellence once a year for an evaluation, which can then be followed by any adjustments that need to be made to the treatment program. It is quite valuable to the patients and the patients who are seen by these centers tend to have fewer problems, as their care is so well managed. One main benefit of these centers is you get to deal with doctors and nurses who work as a team to design treatment programs for each patient and these professionals are thal specialists with great experience working with thals. If going to London once per year is feasible, you should contact Emma Prescott at Whittington and ask her if this would be possible to set up. I think this would be a very positive step, especially as they move into adulthood.
Lena,
I really have exposed myself to a vast range of information about all facets of thalassemia from the medical to the psychological and emotional, and feel that in some ways I have a broader view of thalassemia than most, but then I meet someone like Dr Vichinsky, who has such an incredible grasp of thalassemia in every possible way, that I feel like I hardly know anything, so your words, coming from a very experienced and knowledgeable person such as yourself, mean a lot to me. As far as extramedullary hematopoiesis, I feel this is one of the most under-diagnosed conditions in thalassemia. I believe that many thals have back pain that is attributed to arthritis, when in fact it is these growths pressing on the spine that are causing the debilitating pain. And they may remain under-diagnosed because it takes a trained tech who knows what to look for in a scan to see them, and they have to be looking for them in the first place, which just usually is not done. However, I think if logic was followed, they would be suspected far more often. Basically, when your body does not have enough oxygen, which is caused by an insufficient hemoglobin level, the body will try to compensate by growing red blood cells outside the bone marrow, often within the organs, the body cavity and even within individual cells throughout the body. In those with thalassemia, this only leads to production of the same bad red blood cells that do not carry much if any hemoglobin, rendering the body's effort to boost the hemoglobin level, futile. There has been a general assumption that these are not very common in majors but I would dispute this, as I personally know of many majors, many of whom are well transfused, who do have these extramedullary growths. This tells me that the hemoglobin does not have to be severely below normal for these growths to begin. Majors tend to maintain their hemoglobin in the same range that is seen in the more symptomatic thal minors, yet many of these majors do get these growths. So, is it far fetched to think that low Hb minors may also get them? Courtenay's is large enough to affect her breathing. I really think that if logic was followed, there would have to be an admission that minors can suffer many of the same things found in majors, but most likely, to a lesser degree. They have one bad gene that causes defective red blood cells to be manufactured. My question to those who claim minors have no symptoms is "why wouldn't they, since half of their hemoglobin production is affected by the thal gene?"
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Andy,
I have been dealing with thalassemia my whole life and because I am analytical by nature, I do not leave anything at random. I always search and study things as a third party and there have been many times when I try to stay outside myself and examine things--observe myself and my reactions.
Being a thal, justifies my knowledge - if any - and my tactics concerning my treatment.
I have never,though, met someone like you: So well informed,inspite of not being a thal and not being a doctor. So what I am saying, I trully believe. Only committment can justify the great job you have done with this site and this is something for us to respect. I know I do.
Lena.
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I would say its more than 100% commitment from Andy...it is a whole lifetimes commitment..and that speaks volumes!!.....words alone cannot express what you feel when you come across such a genuine person such as Andy.......thank you for simply just being there...thank you for your precious time....and thank you for your deeply valued knowledge...
Andy....that is something to definately look into.....you say 'as they move into adulthood' what should we look out for?
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Emby,
When I talk about them becoming adults, I am referring to them becoming more and more responsible for their own treatment regimen. The managed structure found with the comprehensive care programs has proven to be extremely important to adult patients who naturally move from full parental involvement to responsibility on their own. In some ways, the comprehensive programs take over for the parent to make sure that all aspects of treatment from transfusions and chelation to the annual tests, scans and evaluations and also the psychological and emotional aspects of treatment are maintained. Without this direction, young adults often slip on compliance and run into problems by age 30. One of the most touching moments at the recent conference in NYC was when two adult women with thalassemia talked about what is necessary for adult patients. The importance of their comprehensive thal centers to these women was well expressed and their request for more adult centers was a real eye opener. Adult thals, if given the choice, will chose adult centers, and the women lamented how little access to adult facilities currently exist. One thing they stressed was how important the thal centers are for the patients and they also pointed out that it isn't just the patients that have to change. It is the centers themselves, that also need to evolve to include both underage and adult patients. With the advancements in thal care, the need for creation of thal centers for adults has become much more apparent and what we are currently seeing is adult patients being treated in adolescent settings. The need for adult centers is obvious to the adult thals of today but their development has lagged. As your sons move towards this age, you should see a correction as we see more adult centers established with more expertise on the problems of aging thals, which are often quite different from the problems of younger thals. All parents are proud to see their children become adults and forge lives of their own. Parents of thals have not had this experience until more recent years, and to achieve this maturity in their children, it is necessary to begin the transition as teens. The comprehensive approach makes it much easier for this to occur as the young adult becomes the person with primary responsibility for his or her own care. In some ways, the center takes over for the parent to make sure the patients stay in full compliance.
There are many issues that confront thals as they grow and giving them the tools to become independent adults is essential to ensure the mental and physical health of those with thal as they become adults. The comprehensive approach to care will ensure that this can happen for every patient.
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i feel the biggest thing for us as parents is to get the boys to be more aware and in control of the thal and to keep themselves well informed especially as they are getting older i would like them to take the thal into their own hands with us supporting them all the way.
[quote from previous post]
I thank God our boys have always been compliant with all forms of their treatment....and I hope, God Willing, they continue this way....
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Hi Emby Sis,
i hope that ur sons are doing fine... wishing them lots of luck n best of everything :) ..
Best Regards
Take Care
Umair
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Salaam Umair,
They are both very well, Alhumdollilah. About these pains they have only once in a while, though it is quite severe when it does happen, I need to ask their Doc, when we next meet, to have it investigated and InshaAllah take it from there.
Praying that you are keeping well yourself brother Umair....may Allah keep you healthy, happy and content..
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Emby Sis,
Ameen ... always praying the same for them :) ... praying that they r not having anything serious ...
Best Regards
Take Care
Umair
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Hello Andy,
In need of your opinion...
Our oldest needs to to use the loo more often than he is used to..[pass water]. He is used to going twice or 3 times a day. Two months ago he was told that he had Water Infection and was prescribed medication which he took over a period of 3 days. He had another urine test a week or two after because he is still going between 6-8 times a day. The test came back as normal.
What do you suppose could be causing this? He does not drink so much throuout the day. It is annoying him as the urge to go is sometimes very sudden and when he does go it is a normal amount. His blood test are within the normal range. He has an appointment with the GP next week.....I told him that I'll ask Andy about this. They all know you by your photo...I have asked him to post any concerns on this forum himself....hoping he will...soon.
Emby
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Hi Emby,
The first thing I have to say is a normal urination schedule of only 2-3 times daily is not adequate. This means he is not drinking enough fluids on a regular basis, and this is something that needs to be corrected. 6-8 times per day is considered normal. I think with thals, fluid intake is critical, as there are many toxins in the body from the regular breakdown of red blood cells and the oxidative stresses that accompany thalassemia. In addition, when you are regularly on medication, especially chelators, plenty of fluids should be taken daily. Exjade does increase both thirst and urination, so this may be a factor.
As far as his current situation, is he experiencing any pain or burning with urination or any abnormal discoloration of the urine? Any pain in the kidney area? If there is no sign of infection in testing, and the doctor may choose to test again, and there is no reasonable explanation for the frequent urination, his sugar level will probably be investigated. However, if this was a problem, he would also be experiencing excessive thirst.
Please review the fluid intake of both boys. It is very important for anyone to get adequate hydration, but with the use of chelators, it becomes even more important.
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Thank you Andy for your reply.
He does not have any of the symptons which you ask about. As for his fluid intake it is about 5-6 mugs full daily....dunno if that is enough....you are probably right...maybe now his body is telling him 'i'm gonna go 6/8 times..and thats that!'. Just reminding you that they are both on ferriprox alone at the moment and he is only taking 3 tabs 3xdaily 5 days per week [ferritin 100].
Thanks for your advice...will encourage them to drink more.
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Emby,
Since deferiprone is excreted by the kidneys, fluid intake is even more important. In fact, fluid intake is essential to ensure that the drug is working to its potential, so keep the fluid intake up, and as long as there is no sign of infection, don't worry about more frequent urination, with the only exceptions being if there is the feeling that there is a need to urinate and little comes out.
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Dear Emby,
Is there any weight loss?? Please make sure to test his blood glucose level (fasten and after eating)
manal
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Thank you Andy.....I probably need to do the same as I'm not in the habit of drinking throughout the day.
Manal...Alhumdolillah, his blood glucose is within normal ranges and he hasn't lost any weight [actually he has gained a bit and doesn't resemble a beanpole anymore so we are glad about that :biggrin]
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Thanks Emby for your reply, since there is no infection and the glucose level is normal, i guess it is something minor or maybe the cold weather sometimes lead to this.
Wishing you all the best :wink
manal
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Thank you Manal....lots of love to your children too..
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hey andy this is pleasance,
i had forgotten my passwrd and so strted anew account today as manit nyways hows u?????
well jst was goin thru thj=ese post so thought of letting u know tht we stay in mumbai and jst aweek back i did the t2 scan for my daughter anit who is 6yrs old and yes 1st she did cry but then was fine..............n yes she did have problems in holding her breath for fewer sec as she was alil nervous but then the technician suggested tht i should stop her breath as in when req according to the instructions by closing her mouth and nose and then evn i was skeptical about the same but ma daughter suggested tht i should do so and she wld co-operate and her reports had come today hwerein it stated...HEART--------33.38-----NONE
LIVER---------1.27--------SEVERE
SEVERE HEPATIC AND ABSENT MYOCARDIAL IRON OVERLOADING IS SEEN.
so i went to dr mb agarwal and he suggested me starting asunra for her tht is 800mg per day
as her iron levels have exceeded to 5200................
pls lemme knowm what is ip6
and yes we have also done her dxa i:e bone density test........so wld be strting her calcium too any ways u pls share ur opinion on this pls..........need help
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Hi Pleasance,
You really do need to start chelation immediately. Right now the heart is safe but this will change without chelation. Please do start Asunra immediately. To minimize reactions to the drug, start out with a low dose, about 1/3 of the required dose, and stay at this dose for 1-2 weeks and then raise the dose. This helps the body get used to the drug without getting the bad rash that occurs when the full dose is taken when starting. Dr Agarwal is an excellent doctor so please follow his guidance.
IP6 is a naturally occurring nutrient that is related to the B family of vitamins. It is also known as phytic acid. It is derived from the bran of grains and is a natural iron chelator, but not strong enough to rely on by itself, and also a powerful antioxidant. Antioxidants are extremely important for thals to help prevent the damage that oxidation does to cells. Tea is another very good antioxidant.
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Dear Andy, how are you? Hope you are keeping well.
I have a question regarding copper. I was told today by my childrens Dr. that they both have some diffiiciency in copper, with the normal levels being 12 our youger ones at 8 and the older one at 10. We were told that the hospital does not give copper supplements.. ???...that they could eat copper rich foods or buy supplements from Holland and Barrets :-\!
So... we will buy it from Holland and Barrets but the Dr. could not tell us what dose to take.
We were told Ferroprox has an effect on copper in the body.
Please can you tell me about copper difficiency (Dr. did tell me but i forget a little except that it could make them tired) and how much do you think they should take?
Thanks soooo much.
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Hi Emby,
They can safely take 500-1000 mcg copper daily, which is .5 to 1 mg daily. A good calcium and mineral supplement that is sold in the UK is Osteocare chewable. You can see this at http://www.vitabiotics.com/Osteocare/chewable_formula.aspx I believe taking a good mineral supplement is a good idea for all thals.
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Thanks Andy,
When LB (little brother) was taking Osteocare tablets, vit d, calcium, magnesium, he needed to urinate alot so he has stopped them for a while. As for the copper defficiency, how could it affect them if it got lower?
What time is it there..did you just wake up...thanks for getting back to me quick :smiley.
By the way BB (big brother) passed his driving test today...
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Hi Emby,
It's afternoon here right now, but I am not sure I've woken up all day. :rotfl
Copper deficiency affects the nervous system and also can result in a lowered white blood cell count. Deficiency in thals is fairly common and can also be caused by taking high amounts of zinc.
And congrats to BB on passing his driving test. Now you may need something for your nerves. Hehe. My 18 year old son recently got his driver's license and will be off to California if his truck ever gets fixed. It's been a bad few weeks for vehicles in my household. I got my exhaust system fixed yesterday, drove one block and the exhaust pipe fell off.
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Ha Ha...yeah I think we should get something like that for him, we're planning to buy him a car that would run for at least 5 whole minutes then I wouldn't have to worry too much.
Many congratulations to your young man. Gives you a warm feeling to see them 'growing up'.
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Dear Andy
i was buy osteocare tablets .
i was show to my doctor and he say this is very low dose for you.
so cane you know some high dose tablets.
like osteocare
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Rehman,
Which Osteocare tablets are you taking and in what dosage daily?
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Andy...would amino acid chelated copper tablets be ok to take?
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Yes, the amino acid chelated iron is supposedly more readily absorbed, so it is fine to take.
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Thanks Andy....the tablets are 2mg..should it be split in half?
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An adult can take up to 3 mg daily, so adjust according to their weight. They may be able to take 2 mg.
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Dear Andy
im taking Osteocare Original,
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Thank you Andy....how do I work out how much tablet they need according to weight?
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Emby,
What is their weight? They could try taking a tab every other day.
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Andy.....its 62 and 63 kg....BB suggested to take it every other day too
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Rehman,
The dose for Osteocare isn't high enough for a thal patient. I would suggest taking 1-2 more tablets daily or look for stronger cal/mag supplement and I would also say a higher dose of vitamin D should be taken separately. Up to 5000 IU D daily.
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Der andy thanks for reply
ok i will start it .
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Hi, I'm seeing my son's hemo on Monday and we are starting a chelation plan for Adel. I'm mentally prepared for Deferal infusion, although the doctor did mention (via email) to start him on ExJade. His ferritin last week was 1200 and he is 3 years old. In my mind, I am thinking maybe I should start Deferal first, then few months later begin with ExJade. What is your opinion?The reason for my doing that is, at least I can observe what's he is like on Desferal before starting him on a combination program. I was told that L1 is seldom used on small children. Is ExJade more effective than L1?
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Hi Syaida,
Exjade is much easier to administer than desferal, so it has become the drug of preference these days. Its actions are fairly equivalent to desferal and it requires no needles, so most doctors are now leaning towards Exjade for early use. I'm not sure I would want to go through the hassle of trying a needle on a young boy. When he does start Exjade, whether now or later, start with a low dose and build up to the required dosage, as his body gets used to the drug. This will eliminate most of the early side effects, like the rash. Get him used to drinking tea also, as this helps inhibit iron absorption and is a natural chelator.
Which chelator is best? Every company will argue for their drug, but studies have shown that Ferrirprox (L1, deferiprone, Kelfer) is best at removing heart iron and also offers a cardio-protective benefit, that the other chelators do not. Why is this most important? Because heart failure is the number one cause of death in thalassemics. In addition, after over 20 years of use, no further side effects for L1 have been found. The drop in white cells in some patients is long established as the main detrimental side effect of L1. Joint pain is the other side effect most often mentioned. Compared to the other chelators, L1 is the superior drug. I would also be a bit reluctant to start a young child on L1, but by age 6, it should be considered, depending on how well the boy is doing with his current chelation regimen.
I also want to mention for all, that most doctors agree that patients should not rely on only one chelator during their lifetime, as switching chelators can help to minimize any long term effects.
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Thanks, Andy I'll bear in mind what you said. First and foremost, you cannot imagine how happy when I stumble upon this network. My caregiver and I are now on this everyday and taking down notes.
I do not know how/where to begin on what me & Adel had gone through these 3 years. This lil guy is indeed very special. His pregnancy was very dramatic, he had plueral effusion on his left lung at 16 weeks. I went through procedure to "tap" his lungs in in utero. On further investigations, at 19 weeks he was found to be B-Thal Major with a big VSD (hole in the heart). He had had 3 heart surgeries (at birth) and thereafter was very sick including jaundiced. He was termed chronic lung disease and has been on O2 ever since his heart surgeries. Blood transfusion started soon after heart surgeries too. We noticed his spleen & liver were rather big at about 3-6 months old. We opted for a splenic embolisation before his 2nd birthday cos he started become sickly and his transfusion frequency was like weekly due to splenomegaly. Thereafter, he was well (started walking, feeding making huge milestones) for the next 9 months, was transfused between 6-4 weekly and the transfusion frequency became 3 weekly last Dec. We gave him a 2nd embolisation this year January. He has been on 4-5 weekly transfusion since.
I know it's very hard to do the needle on a young boy, but this boy has tremendous understanding, it may be difficult for other kids as they are restrained from their movements, but lil guy is quite used to being confined within limited area, reading, writing, playing his puzzle are his favourite pastime. He was even on a 24 hr feeding pump (via gastrostomy) before his embolisation, as his liver & spleen became so big abdomen became distented, we couldn't keep any milk down. We got rid of that pump after he became well after his 2nd birthday. I guess if we can't cope with desferal, we'll switch to ExJade. I'm not sure if you've heard of Ambrotose, Adel has been on it for 1 year I feel it really helps as a natural chelator.
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Desferal may be a better choice. The prior problems with the liver and spleen lead me to think that desferal may be better tolerated at this point. If liver function is currently affected by enlargement, it might be wiser to wait on starting Exjade. The other problems he faces complicate matters, as you don't know what to expect when new drugs are introduced, and desferal is probably the safest chelator to start. With chronic lung disease, I would suggest always watching for any breathing reactions from any new drugs that are introduced, and desferal is known to cause allergic reactions in a small percentage of patients, so watch for any sign of allergic reaction.
A good buddy of mine, who I met through this group, takes products from the same company that makes Ambrotose, and he feels he has done quite well in terms of health and energy. There are many natural products that can help the health in general, which is very beneficial for anyone already dealing with health issues. I would definitely suggest natural vitamin E for your son. Its long term use will prove quite beneficial for heart and lungs.
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Hello Syaida Lee and welcome to the site :biggrin
I am happy that you were able to pass through all these hardships that faced Adel and you and i hope he can cope with desferal with no problems. Hats off ....you are doing a wonderful job. Keep us updated
manal
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what is green tea ??? ???
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Hello Dear Andy
I m on Desferal + Feriprox combination therapy I m taking 6000mg of Feriprox tablets and 3 Grame of Desferal Injections on SUB-Q.
So cane you tell me when I missed my feriprox tabets dose for example I missed my morning dose so cane I take with midday dose .
And please tell me after taking feriprox . cane I take vitamin C with desferal .
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Yes, you can continue to take vitamin C with desferal. Take it when you are starting the desferal. If you miss a dose...
http://www.ferriprox.com/faqs/default_intl.asp
What should I do if I miss a dose of Ferriprox?
If you miss one dose take it as soon as you remember and take your next dose at its regularly scheduled time. If you miss more than one dose do not take the missed tablets, just continue with your normal schedule. Do not change your daily dose without first talking to your doctor.
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Here we take vitamin C with ferriprox,too.
Ferriprox leaflet writes you can take vit c according to your doctor's guidance.
Lena
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Andy and Lena thanks a lot for you are reply.
my docor advise for taking vitamine c with only Desferal.
butt tell me when i take Feripox so after how jmuch time i can take vitamin c with desferal .
or i take vitamin c then after how much time i take feriprox .
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Rehman,
you can take vitamin c preferably when starting desferal or even a little before. Do not worry about Ferriprox at all. Just take vit.c when starting desferal.
Lena.
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thanks a lot .
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Dear Andy,
Regarding copper supplements BB and LB have been taking half of 2mg tablets now every day. I still need to know how much to give them, how is it worked out?
Both brothers as you know are on Ferriprox so should they continue to take Ascorbic Acid 100mg daily when taking Ferriprox?
I would like to know that if, as LB is, low in Vitamin D, could this cause yellowing in the white of the eye? He has recently started taking Vit D solution again.
Hope you are keeping well yourself.
These may be little issues but i thank God you are there for us.
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Hi Emby,
I would suggest keeping them on the current dose of copper until the doctor says it's time to test again and then adjust the dose if needed. Small amounts of vitamin C are safe. It's best if not taken with meals, as to minimize any added iron absorption from food. I think vitamin D is indicated in thal and especially when you see symptoms like yellow eyes. This is a sign of jaundice which is related to the breakdown of red blood cells. You also will usually see a high bilirubin level when the eyes are yellow. Vitamin D (1000-5000 IU daily), B complex 100 and magnesium (500-750 mg daily) may help to slow this breakdown, leading to less bilirubin development. Vitamin D's importance has only recently begun to be realized and its importance in thalassemia has led to doses of 50,000 IU at once, to correct deficiency. Testing for D is simple. A value below 30 needs correction and we may eventually learn that 30 is low.
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Thank you Andy,
LB's bilirubin was quite high last month ans so was his ALT. This month ALT is normal and bilirubin levels are lower but not quite normal. His Vitamin D was 6.1 (!). I would like VIT D checks to be routine here but it isn't. Takes time for results to come and to get med for it. It's such a headache cos we keep having to chase everything up when the Dr.'s are supposed to be on top of it all :(!
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My son Jia has his ferri scan done at 4. Now he is 7 and hasn't had another one done yet. The ferri scan result was very good the last time and the doctor decided not to do another scan in 1 year. Maybe he should go for a scan soon now that it has been so many year's lapse. His ferritin level is now around 1300. The doctor here doesn't want the ferritin level to drop much past 800. She said it can affect growth if over chelated. I don't have many older thalassemia persons to consult here. Only 4 kids in Perth with thalassemia major.
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Hi jia,
I would suggest another Ferriscan by 8 years old to help judge if his current chelation routine is adequate. These scans help in showing if any fine tuning of the chelation program is needed, so even patients with low ferritins should have regular scans.
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Dear Andy,
Please can you send me a good source of information/recent studies regarding Vit D in relation to people with thalasseamia major. I would like to pass it on to a consultant my boys are seeing. Please also send me the same for calcium and magnesium.
Thank you very much....
Emby
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This is a full article that suggests a correlation between vitamin D deficiency and heart dysfunction in thal majors. This is a very significant recent study by Dr John Wood, one of the top thal cardiologists on earth. Please refer the doctor to the entire article.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2892922/
Vitamin D25-OH and D1-25OH levels were compared with cardiac R2[large star] (1/T2[large star]), left ventricular ejection fraction (LVEF), age, ferritin and liver iron in 24 thalassaemia major patients. Vitamin D25-OH levels were reduced in 13/24 patients while vitamin D1-25OH levels were often elevated. Vitamin D25-OH levels decreased with age (r2 = 0·48) and with liver iron (r2 = 0·20). Cardiac R2[large star] was inversely related with the ratio of D25-OH to D1-25OH levels (r2 = 0·42). LVEF was also proportional to the D25-OH/D1-25OH ratio (r2 = 0·49). Vitamin D deficiency may be associated with cardiac iron uptake and ventricular dysfunction in thalassaemia major patients...
These data reinforce prior evidence that serious vitamin D deficiency is common in thalassaemia major patients (Moulas et al, 1997; Napoli et al, 2006). The sharp decline in vitamin D25-OH stores with age probably reflects a combination of decreased intake (dairy products) as well as a shift toward more indoor activities.
We have previously discussed this research at
http://www.thalassemiapatientsandfriends.com/index.php?topic=2909.msg28575#msg28575
Vitamin D, heart dysfunction tied in thalassemia.
By: Lowry, Fran
Publication: Family Practice News
Date: Saturday, March 1 2008
ATLANTA -- Vitamin D deficiency was strongly associated with high cardiac iron and increased ventricular dysfunction in a retrospective review of 24 young thalassemia major patients.
A review of their medical records showed levels of vitamin D(25[OH]D), the predominant circulating form of vitamin D, were "markedly depressed" in 13 patients and borderline depressed in the remaining patients, said Dr. John C. Wood of Children's Hospital Los Angeles and Keck School of Medicine at the University of Southern California, Los Angeles. There were 11 girls and 13 boys; mean age was 15 years.
Vitamin D(25[OH]D) levels less than 20 ng/mL are considered deficient and D(251OH]D) levels 20-30 ng/mL are borderline or insufficient, Dr. Wood said in a presentation at the annual meeting of the American Society of Hematology. In this study, the mean D(25[OH]D) was 17 ng/mL. The vitamin D levels were then com pared with cardiac R2*--a surrogate MRI measure of the amount of iron in the heart--and left ventricular ejection fraction (LVEF) from each patient's most recent cardiac MRI. As vitamin D levels decreased, cardiac R2* increased. Vitamin D(25[OH]D) levels below 13 ng/mL were associated with severe cardiac iron loading. LVEF also decreased as D25OH decreased.
"In our MRI laboratory, an ejection fraction less than 56% is considered abnormal and indicates poor pump function. In these patients, there was a proportional association between vitamin D(25[OH]D) levels and cardiac function. The four patients with the lowest D(25[OH]D) had an LVEF between 50% and 54%," he said.
The population also was moderately iron overloaded, with mean ferritin levels of 2,089 ng/mL, liver iron 14 mg/g dry weight, transferrin saturation 84%, and cardiac R2* 65 Hz. The normal R2* should not exceed 50 Hz. "Vitamin D deficiency ... is extremely common in thalassemia. Twenty-three of the 24 patients in our study had levels that are considered inadequate to ensure optimal calcium absorption and bone mineralization," he said in an interview. Low vitamin D is linked to decreased cardiac function, muscle weakness, glucose insensitivity, and refractory congestive heart failure.
Increased iron in the heart becomes evident in children with thalassemia major around the age of 9 years. Two-thirds of adults with thalassemia have cardiac iron deposition. Iron cardiomyopathy is the leading cause of death in thalassemia. "Our study describes an association between low vitamin D, high cardiac iron, and increased ventricular dysfunction. We cannot prove [cause and effect], but vitamin D might be worsening the cardiac iron overload and the cardiac dysfunction through its modulation of calcium signaling in these patients."
"Vitamin D deficiency is extremely common in thalassemia, and since osteoporosis is ubiquitous in this disease, vitamin D screening and replacement are probably indicated regardless of the heart findings," Dr. Wood said.
He added that low vitamin D produces secondary hyperparathyroidism, which exacerbates heart failure of any etiology. Because of this, thalassemia patients with ventricular dysfunction should have their vitamin D levels assessed, and replacement should be started if these levels are low.
The National Heart, Lung, and Blood Institute, the Centers for Disease Control and Prevention, and Novartis Pharma funded the study. Dr. Wood disclosed he receives research funding and honoraria from Novartis and Apotex, and is a consultant to Novartis.
Regarding vitamin D and bone mass in thal patients.
http://cat.inist.fr/?aModele=afficheN&cpsidt=17899816
Low serum levels of 25-hydroxy vitamin D in adults affected by thalassemia major or intermedia
Auteur(s) / Author(s)
NAPOLI N. (1) ; CARMINA Enrico (1) ; BUCCHIERI Salvatore (1) ; SFERRAZZA C. (1) ; RINI G. B. (1) ; DI FEDE G. (1) ;
Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)
(1) Dipartimento di Medicina Clinica e Delle Patologie Emergenti, Via del Vespro 143, 90127 Palermo, ITALIE
Résumé / Abstract
Adult thalassemic patients have reduced bone mass due to disturbances in several different mechanisms affecting bone turnover. To determine if vitamin D deficiency contributes to the low bone mass of adult thalassemic subjects, we studied serum 25-OH-vitamin D levels in 90 patients (age ranging between 21 and 48 years) affected with thalassemia major (TM) and 35 (age 21-56 years) with thalassemia intermedia (TI). TM patients had been receiving regular transfusions from the age of 2 years and had increased serum ferritin, glutamic oxalacetic transaminase, glutamic piruvic transaminase as well as low bone density (L1-L4 Z score -2.07 ± 0.2). TI patients did not receive transfusions, but their ferritin levels were increased as well (520.3 ± 138,1). 8 TM patients (10.1%) and 4 TI (11.4%) had serum 25-OH-vitamin D less than 10.4 ng/ml and were considered presenting an absolute deficiency of vitamin D. Mean 25-OH-vitamin D was significantly (P < 0.01) lower in both TM and TI patients (20.3 ± 0.7 ng/ml and 20.9 ± 2.3 ng/ml, respectively) than in 100 healthy control subjects of similar age (25.2 ± 1 ng/ml). 1,25-OH-vitamin D levels were in the normal-lower levels (45.15 ± 1.5 mg/dl), while 24 H urinary calcium was below the normal range (15.75 mg/dl). In TM patients, the 25-OH-vitamin D levels correlated negatively with age (P < 0.05) and with serum ferritin (P < 0.05). TM and TI patients with low 25-OH-vitamin D levels (<17.8 ng/ml) presented higher serum ferritin levels (P < 0.01) and higher PTH (P < 0.05) compared to those with normal vitamin D. Moreover, TM patients with low 25-OH-vitamin D levels were significantly older (P < 0.05) and had higher GPT (P < 0.05) than patients with normal vitamin D. In conclusion, calcium metabolism is frequently impaired in adult thalassemic patients. An early and effective medical treatment should be taken in consideration by the clinician in order to improve the bone health in these patients.
Please note the dosages used in this study.
http://www.springerlink.com/content/f48h34542203214r/
Parathyroid and Calcium Status in Patients with Thalassemia
Meenu Goyal, Pankaj Abrol and Harbans Lal
Abstract
Thirty patients with thalassemia major receiving repeated blood transfusion were studied to see their serum parathyroid hormone (PTH) and calcium status. Serum PTH, serum and 24 h urinary calcium, and serum alkaline phosphatase, phosphorus, and albumin-corrected calcium levels were determined. Half of these patients, in addition to transfusion, were also supplemented with vitamin D (60,000 IU for 10d) and calcium (1500 mg/day for 3 months). Serum PTH, and serum and 24 h urinary calcium concentrations of the patients receiving transfusions were found to be significantly reduced while their serum alkaline phosphatase, phosphorus, and albumin-corrected calcium levels were not significantly altered when compared to the respective mean values for the control group. Vitamin D and calcium supplementation significantly increased their serum PTH and calcium levels. Supplementations also increased urinary excretion of calcium. The results thus suggest that patients with thalassemia have hypoparathyroidism and reduced serum calcium concentrations that in turn were improved with vitamin D and calcium supplementation.
A more general article about calcium and magnesium and the necessary ratio between the two. The two should be taken together along with vitamin D. Calcium taken alone can lead to irregular heartbeats and palpitations. Considering the need for magnesium in thals, calcium should not be considered by itself.
http://www.enerex.ca/articles/calcium_to_magnesium_ratio.htm
Calcium to Magnesium Ratio
Dr. K. Sharma (Ph.D.)
CAL-MAG
Both calcium and magnesium are involved in numerous metabolic functions and are absolutely essential for the maintenance of a healthy body.
Calcium is considered the backbone mineral because of its role in the formation of skeleton and teeth. Magnesium is called the natural tranquilizer due to its relaxing action on nerves and muscles. The biological functions and the therapeutic uses of these minerals are shown below:
FUNCTIONS
CALCIUM
MAGNESIUM
* Development and maintenance of bones and teeth (about 99% of body calcium is in bones and teeth)
* Blood clotting
* Muscle contraction and relaxation
* Transmission of nerve impulses
* Enzyme activation for production of gastric juices
* Fat, protein and carbohydrate metabolism
* pH balance
* etc.
* Development of bones (about 70% of body magnesium is in bones)
* Crucial part of many enzymes involved in energy production and respiration
* Transmission of nerve impulses
* Muscle relaxation
* Regulation of body temperature
* pH balance
* Release of nerve tension
* Absorption and utilization of calcium, phosphorus, sodium, potassium, vitamins C, E, & D.
* etc.
THERAPEUTIC USE
* Arthritis, osteoporosis, rheumatism, other bone disorders, dental decay, epilepsy, insomnia, nephritis, pre-menstrual cramps, stress, constipation, muscle pains, high blood cholesterol, regulation of heart beat.
* Arteriosclerosis, heart attacks, Infant death syndrome (SIDS), hypertension, bone fractures, epilepsy, diabetes, alcoholism, kidney stones, leg cramps, nervousness.
Both minerals require each other for their absorption and utilization and must be provided in adequate amounts. Depending upon the physiological environment, there are cases in which the roles of these two minerals are antagonistic to each other. Magnesium is located inside the cell (intracellular) while calcium is predominantly located outside the cell (extracellular). Consequently, the role of magnesium in intracellular metabolic functions, such as energy production, respiration, and muscle contraction-relaxation is antagonistic to calcium.
let us briefly examine the role and relationship of these two minerals in known clinical studies:
REGULATION OF HEART BEAT
The heart is a muscle and its primary function is to pump blood throughout the body. The heart is composed of billions of cells, each of which works as an electrochemical generator, and contains both calcium and magnesium. On the outer surface of the heart cells, thin fibers made of a substance called "actin", continually expand and contract in unison with the heartbeat. The actin fibers are stimulated by calcium, and then relaxed by magnesium. An electrical charge produced by magnesium then pushes the calcium to the opposite side of the cell. Thus, calcium helps to produce the heartbeat, and magnesium regulates it.
MYOCARDICAL INFARCTION (Heart Attack)
Several researchers have shown that a heart failure involves drastic changes in the concentration of cardiac electrolytes (1). During cardiac stress, some of the magnesium is moved out of the cell accompanied by an influx of calcium into the cell. Thus, the cardiac muscle shows a 20% decrease in magnesium and a 4 1/2 fold increase in myocardial calcium (2). The loss of magnesium and an influx of calcium seriously disrupts the energy potential of the affected muscle (3). The situation can be prevented by increasing the level of magnesium. In clinical practice, intravenous or intramuscular administration of magnesium salts has proven very useful and is highly regarded (4). It is known that magnesium therapy is the most effective to protect myocardial integrity during cardiac arrest (4,5). It is interesting to note that in Canadian surveys of post-mortem tissue composition, about 24% less magnesium was found in ischemic hearts than in non-cardiac cases (6).
ATHEROSCLEROSIS (Heart Disease)
A highly dietary intake of magnesium has been attributed to why heart disease is virtually unknown among Bantu tribesman of South Africa while the disease is prevalent among white South Africans. Clinical studies have revealed that the Bantu's serum magnesium level is about 11% higher than in the white South Africans. The Bantu's high dietary intake of magnesium is largely attributable to intake of unrefined cereals such as maize meal, which has a high magnesium content and also has a high fiber content (12). Also, it has been shown that the ability of high-fat diets to induce atherclerosis is prevented by a high magnesium dietary regime (7).
HYPERTENSION (High Blood Pressure)
For many years, hypertension has been associated with sodium. Consequently, the disorder is treated by substituting potassium in the diet. However, most of us do not realize that magnesium is also considered a well-known vasodilator. The anti-hypertensive effect of magnesium is achieved by a direct effect on the vascular wall or is mediated through the central nervous system (8). Magnesium competes with calcium for binding sites and the net result is that magnesium reduces the calcium-induced contractions. It is well established that magnesium infusions can cause vasodilation and reduce hypertension in humans (9).
UROLITHIASIS (Kidney Stones)
Canadians appear to have a very high incident of kidney stones and the occurence is particularly high in Newfoundland (11, 12). In U.S., South Carolina has the highest urolithiasis rate. South Carolina also has the highest U.S. rate for cardivascular deaths (10). Both Newfoundland and South Carolina regions have "very soft" drinking waters with little magnesium (11).
In Canada, calcium urolithiasis accounts for 70 to 80% of the total kidney-stone problems (12). In the U.S., about 67% of all kidney stones are composed of calcium oxalate or calcium hydroxyapatite (11).
Several researchers have used the magnesium/calcium ratio as an index of susceptibility of urine to form kidney-stones in patients (10,13,14). In general, patients with a urinary magnesium/calcium ratio of 0.7 is normal, whereas a value lower than 0.7 may be considered as stone-forming. The ratio is especially low in the Canadian "Kidney Stone Patients", indicating inadequate magnesium intake.
The oral magnesium supplementation has proven very effective in the prevention of kidney-stone formation (14).
INFANT DEATH SYNDROME (Sids of Crib Death)
Magnesium deficiency has a primary role in sudden unexpected infant-death syndrome. The sequence-of-events are as follows:
Magnesium deficiency causes calcium-dependant release of histamine which, in turn, induces increased release of acetylcholine (especially at high calcium/magnesium ratio). The increased amount of acetylcholine leads to symptoms of neuromuscular hyperirritability and convulsions that can lead to reduced heart rate (15).
The sudden-death syndrome is puzzling since no recognizable allergens are involved. The symptoms are acute respiratory distress, and includes bronchospasm, shortness of breath, and eventual circulatory collapse. Hypomagnesemia is observed throughout this syndrome. Therefore, the role of magnesium in the infant-death syndrome is very significant.
NUTRITIONAL STATUS OF MAGNESIUM
The recommended dietary allowance for magnesium is 300 to 450 mg/day. There are several factors including pregnancy, rapid growth, or a high intake of protein, vitamin D, calcium, fat, carbohydrates or alcohol, that will increase the requirement for magnesium.
Surveys of dietary magnesium intake from different countries show a prevalence of lower magnesium intake than the desired levels. In Newfoundland, the intake is only 50% of the recommended amount (16,17). Other reports (40) show that hospital and institutional diets contain only 61 and 68% of the recommended intake, respectively. In other studies (18,19), it was found that the intake for pregnant women was only 45 to 60% of the recommended allowances. There is definite evidence that magnesium intake is suboptimal or marginally inadequate in regions of the Western World (20). The occurence of hypomagnesemia in humans, due to low magnesium intake and due in part to factors such as, prolonged use of diuretics, alcoholism, pregnancy etc., have been shown to be more prevalent that generally believed (21).
CONTRIBUTION OF DRINKING WATER
Drinking water can significantly contribute to magnesium intake and hard waters can supply 9 to 29% of the daily magnesium intake (23). Because of the metabolic antagonism between magnesium and calcium, the ratio between these two minerals in the drinking water is of considerable significance. In a survey of 25 U.S. cities, the lowest death rates from coronary disease were found in areas where the drinking waters supplied more magnesium and less calcium than the U.S. average (24).
Australia has the highest cardiovascular death-rate in the world and also consumes some of the worlds softest drinking waters (60). On the other hand, the Western region of Texas has the hardest drinking waters and the lowest cardiovascular mortality rates in the United States (25).
The relationship between death-rates from coronary heart disease and the dietary calcium/magnesium ratio in several countries is shown in the following figure:
webgraph.GIF (8793 bytes)
Relationship between death-rates from coronary heart disease and the average dietary calcium/magnesium ratio in several countries (26).
The high mortality rate in Finland is associated with a high calcium/magnesium ratio (26), while the low mortality rate in Japan is related to a low calcium/magnesium ratio as well as to the "protective" effect conferred by the alkalinity (carbonate-biocarbonate content) of water.
CALCIUM TO MAGNESIUM RATIO
From the information presented here it is apparent that the ratio between calcium to magnesium is very important in dealing with the causes and prevention of a number of disorders including myocardial infraction or arrhythmia, atherosclerosis, hypertension, urolithiasis, and infant-death syndrome. In all cases, a lower calcium/magnesium ratio or a higher magnesium/calcium ratio is desirable. This need is further underscored by the fact that magnesium intake is generally suboptimal and that hypomagnesmia is more prevalent than generally believed.
The recommended dietary allowance (RDA) for calcium is 800 mg/day, whereas for magnesium it is 400 to 450 mg/day. Only about one-third of magnesium is absorbed from dietary sources. Therefore, a daily magnesium intake of 1200 mg/day has been recommended by some researchers (22). The traditional ratio of approximately 2 parts calcium to 1 part magnesium needs to be upgraded to increase magnesium intake in view of the overwhelming beneficial role of magnesium. The ideal ratio for most people's needs is an equal ratio of calcium and magnesium.
The absorption and metabolism of calcium and magnesium is one of mutual dependence, and therefore, the balance between these two minerals is especially important. If calcium consumption is high, magnesium intake needs to be high also.
VITAMIN D
Vitamin D is necessary to enhance calcium absorption. Vitamin D works with the parathyroid hormone "PTH" to regulate the amount of calcium in the blood. It also stimulates the production of a calcium binding protein (CABP) in the intestinal wall which helps absorption.
References
1. Seelig, M.S.. 1972 Recent Advances in Studies on Cardiac Structure and Metabolism. Vol. 1: Myocardiology. Publ. By University Park Press, London, Baltimore. Pp 615-638
2. Matyushin, I.F. and Samartseva, T.F. 1972. Kardiologiya 12(3): 1963-69.
3. Levin, R.M., Haugaard, N. and Hess, M.E. 1969. Biochem. Pharmacol. 25: 1963-69
4. Petrie, R.H. et al. 1978. Amer. J. Obstetr. Gynecol. 130: 294-299
5. Hearse, D.J., Stewart, D.A. and Braimbridge, M.V. 1978. J. Thoracic Cardiovasc. Surg. 75: 877-885
6. Anderson, T.W., et al. 1975. Canada Med. Association J. 113: 199-203
7. Thrivikraman, K.V. and George, S. 1972. J. Animal Morphol. 19: 196-204.
8. Szelenyi, I. 1973. World Rev. Nutr. Diet. 17: 189-224.
9. Singh, R.B. et al. 1976. Acta Cardiol. 31: 401-409 and 221-226.
10. Mukai, T. and Howard, J.E. 1963. Bull. Johns Hopkins Hosp. 112(5) 279-290.
11. Churchill, D.N. et al. 1978. Annuals Internal Med 88: 513-514.
12. Yendt, E.R. and Cohanim, M. 1978. Canada Medical Association. J. 118: 755-758.
13. Bastian, H.P. and Vahlensieck, W. 1975. Europ. Urol. 1: 235-237.
14. Gershoff, S.N. and Prien, E.L. 1967. Amer. J. Clin. M. Nutr. 20: 393-399.
15. Cadell, J.L. 1972. Lancet, Aug 5, pp 258-262
16. Neri, L.C. and Marier, J.R. 1978. In: Proc Symp June. Bloomington, Minnosota.
17. Fodor, J.G. Pfiffer, G.J. and Papezik, V.S. 1973. by. Canada Medical Association. J. 108: 1369-1373
18. Ash, J.R., Schofield, F.A. and Gram, M.R. 1979 J. Clin. Nutr. 32: 286-291.
19. Seelig, M.S. 1978. Cardiovasc. Med. June. Pp 637-650.
20. Anderson, T.W. 1977. Nova Scotia Med. Bull. Apr. pp 58-61.
21. Becking, G.C. and Morrison, A.B. 1970. Biochem. Pharmacol. 19: 2939.
22. Ashmead, D. Chelated Mineral Nutrition. 1981. Publ. By Institute Publishers, Huntington Beach, Calif pp 162.
23. Hankin, J.H., Margen, S. and Goldsmith, N.F. 1970. J. Amer. Dite. Assoc. 56: 212-224.
24. Schroeder, H.A. 1966. J. Amer. Med. Assoc. 195: 81/125-85/129.
25. Dawson, E.B. et al. 1978. Amer. J. Clin. Nut. 31: 1188-1197.
26. Karpmannen, H., Pennanen, R. and Passinen, L. 1978. Adv. Cardiol. 25: 9-24
www.Enerex.ca
This post will be also posted as new thread in the Diet, Nutrition and Supplements board.
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Andy...thank you very much indeed...will be forwarding on. Having experienced with both my boys I have realised that their vitamin D levels tend to dip significantly after taking the supplement for about 2-3 months. They do need to be taking vitamin D all the time to maintain acceptable level. The vitamin D test were only carried out because I requested it and not because of consultant request. Also I have realised that although the calcium levels as with BB, shows that it is normal, other investigations in testing for a more accurate reading should be done. We were not aware of this because the results were just sitting around in one of their consultants records but BB's bone mineral density was significantly reduced although his calcium readings appeared normal. The Dexa scan was carried out in April (!) but everytime we visited with their consultant nothing was said although we did enquire. Perhaps the results were delayed in getting to them...don't know. I also realise that as parents we always need to be ten steps ahead because some of the professionals just keep dragging.
....Thanks a ton anyways.
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Andy,
Should vitamin D be taken during pregnancy in the same high dosage,if the mother was previously deficient?
Zaini.
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Yes, the need for vitamin D increases during pregnancy and is vital to proper development of the fetus.
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On the topic of vitamin D and how to address deficiency in thals.
From the Standards of Care Guidelines for Thalassemia
Vitamin D supplementation (50,000 IU once a week
until levels normalize) is recommended for patients with
a 25-hydroxy vitamin D less than 20 ng/dL. Calcium
supplementation should be encouraged if dietary intake
is insufficient.
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Hi Andy,
Manit here again ....well this tuesday manit was taken to hospital for transfusions and i dunno y the blood did not go from her left veins.....this had happened earlier too but i asked the doc n he told me it ius because of regular transfusions tht sometimes there is a blockage and it happens.......but i was really upset as mani gets transfused twice in a mnth so if this is the case then how will it effect her in the long run.....does this happen to anyone herre .........
please lemme know if there is something to be done just to try she was pricked thrice on the same arm but in vain....she is jst 7 and she is being transfused since she was 8 mnths old ....i dunno how to takew this feel pity for her..............i want suggestions from all u guys here.........is it because of some deficiency or what?????????????????help me out really worried
would eagerly wait for ur replies
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Hi Manit,
Make sure she drinks plenty of water daily and especially the day before transfusion,it helps with the veins .
Zaini.
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Hi Manit,
My son also has to take a few pricks before they can find a vein. Once the cannula is fixed do they flush the line with saline. If this is not done blood can clot and block the flow. Also the line is flushed again at the start of the transfusion.
maha
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Thanx ,i will make sure that she drinks plenty of water daily and also i would like to let u all know that the other day when the blood was not goin thru her veins the nurse did flush the vein but i dont know y it didnt work and even before when she had this problem they did try but it did not help
so lets hope now tht it works ..................thank u all
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Just something else I thought of in regards to why the blood wouldn't go through. Sometimes the cannula/needle will be resting on one of the valves in the vein and this can prevent the blood flow, or it maybe that the vein slightly collapsed. It happens every now and then and shouldn't be anything to worry about and won't affect the vein next time but obviously is distressing to have to cannulate again. Hope you have a better time at the next transfusion. X
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Manit,
It may also be a good idea to give her L-carnitine. I agree with Zaini, increasing her fluid intake the evening before her transfusion will be helpful as well. Best of luck for her next tx.
Sharmin
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Hi sharmin.....
May i know what is L-carnitine. and also when i spoke to poirot about it he told me that she should go for a test that is they inject a dye in the vein to see if there is any clot.........i didnt understand but do u think that i should go to the doc and get this thing checked.........is there anyone who has ever had this problem or heard azbout it ......dos m really confused cos today i asked the nurse from the same hospital and its been 10-11 years that she is working with thal children and she said tht she never had such a problem before
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Had mailed DR Agarwal and he informed me take transfusions from the right hand ....but i m still in a fix as to even the haemat doesnt have any answer as to y is this happenin.......................but how long will then the right hand co-operate with us when the transfusions are twice a month................... ???........really disturbed
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i login every mornin to tis site in hope of getting replies but when i dont ...........i am upset .........nyways thts me always in hope !!!!!!!!!!!!!!!
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Hi Manit,
Where are they inserting the needle,my daughter used to transfuse through a vein that was on back of her palm first,but then it was getting difficult with time,so they changed the position to the inner side of her elbow,may be you can change the positions on the same hand and see if it works?
Zaini.
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the sister use to transfuse through a vein that was on back of her palm first,but then it was getting difficult with time,so they changed the position to the inner side of her elbow but now either ways it does'nt work...tried on the same hand .......so was getting upset and the main reason is tht when i wrote a mail about this to my haemat even he doesnt have an answer nor any suggestion or atleast he should lemme know the cause for it i even asked if i could come for a check up but he jst said tht no chnge it to right hand .........this is not the answer u expect from a doc ...........................
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Hi Pleasance
I feel its better to rest her left hand for a while and use her right hand for transfusions. Also is Manit taking any kind of vitamin E . Vit E does have anti coagulant properties and probably help with the clots if there are any. Why does Manit have to transfuse every two weeks? Vit E also helps to lengthen the life of the RBC, so if not anything else it will at least help in increasing the transfusion interval. What is Manit's weight and how much blood does she receive every two weeks?
maha
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Hi Maha, kgs
Thanx for replying ..........its such a relief........she is 7 yr old and her weight is 19kgs she doesnt take any vit e pls suggest something as she is very stubborn and doesnt even have any fruits or veggies pls suggest something she gets 1 bottle twice in a mnth that is wat even i was hesitant as children of her age get it done once a mnth the quantity wld be 350ml
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She actually gets 350ml every 2 weeks? This is by far a huge volume. For her weight of 19 kgs she should be getting 285ml - 300ml every four weeks. Pleasance no doctor suggested me vitamin E or L-carnitine or vitamin D or calcium. I learned all this from the other members here and of course Andy.What supplements do you give her? You should start her on vit e. My son is 4 years old and has been taking 100 IU of natural vitamin E for almost two years now. You could start Manit on 200 IU of natural vit E (d-alpha). Do not use any synthetic brand. Also start her on L-carnitine. If her transfusion intervals can be increased while maintaining a good hb, her hands will also will get pricked less often.
take care
maha
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hi maha
like u said that u give so many essential vit and calcium and other things like IP-6 and ginseng honey dailyand B- vitamin daily so can u name all these medicines so that i can buy from a regular medical shop........as i am totally unaware of the same ..........thanx so mch for replying pls lemme know thw doses of these medicines and the names and the time when u give the meds
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I get the vit E and L-carnitine from GNC stores. You should find this in India also. I get ginseng honey from a supermarket here and I order IP-6 online. Both osteocare and a Iron free multivitamin is also available in India.
My son starts his day with asunra. Half hour later he gets his breakfast. I pop in the vitamin E softgel and folic acid in between. He loves to chew it. I give him his L-carnitine soon after breakfast. He comes from school at 1 pm and I give him IP-6 then. Since IP-6 is a capsule I open it and mix it in a little juice as he is still young to swallow a capsule. Half an hour later he takes his lunch. Between 5pm- 6pm ie after he gets up from his nap I give him his second dose of Asunra and half hour later another dose of L-carnitine. I mix the ginseng honey in his milk after dinner. I give him the osteocare and mixavit just before bedtime.
maha
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can u name the vit e capsule and may i know the cost of ip-6 as i gues its a hb inducer and how does ginseng honey help....pls letme know
any ways thnx to give ur time and u mailed me thnk u so mch and may i also know wat is the actual role of L- cartinine
thanx
Maha
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Hi Manit,
IP6 is not hb inducer,its an anti oxidant which also chelates a little bit of iron from the body,you can find it online at www.puritan.com .
Zaini.
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Hi Pleasance,
I get the vitamin E softgel from GNC. It's sold as Natural E 100. Like Zaini said IP-6 is not a hb inducer it simply aids chelation. I give him ginseng honey to increase his immunity, so as he doesn't fall sick as often as he used to do. Just search for L-carnitine. Manal has explained its benefits many times in various threads.
maha
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Hi Maha i went to the medical store and he gave me vit E capsul;es called Evion-400 wherein each cap contains tocopheryl acetate ip......400mg so shall i start wth that and i ve got ostocalcium b12 syrup wth vit d and b12....so shall i start wth that and they dont have L carnitine ....when i asked for it they gave me levocarnitine......i was confused so i didnt take that...pls suggest
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Levocarnitine is L-carnitine. Same thing.
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I don`t know if Evion is synthetic or natural, Zaini would be able to tell you as I remember her mentioning Evion sometime. In any case 400mg would be almost 600 IU, thats probably too much for a 7 year old.
maha
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The Evion available here is synthetic,not natural.
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@ Pleasance:
One of the few times I got transfused, the nurses had a very difficult time starting the IV for the transfusion because, apparently, my veins kept wanting to "collapse". The initial poke went okay, and then when they were going to flush and/or draw some blood before the transfusion, the vein collapsed around the needle and they couldn't proceed any further.
I was told at the time that it wasn't a serious problem - more of an inconvenience than anything else as this meant I would have to get poked more than once - and that having low blood pressure and being dehydrated can make the veins more prone to "collapse".
The reason for the "collapse" which results in either blood being unable to be drawn or the flow in an IV being unable to flow is usually because of the vein's response to pressure against the wall of the vein and for more sensitive/delicate veins, the suctioning pressure of a needle can prompt the vein to "collapse" around the needle.
I have low blood pressure and am easily dehydrated, so when I heard that my vein collapsed on me multiple times during that time I wasn't very surprised. Furthermore, I am considered a "difficult draw" because it has never been easy to draw blood from me or start IVs even without problems related to my veins "collapsing" simply because I have small veins.
A larger vein is more promising when it comes to both venipunctures (blood draws) and IVs (including for transfusions) because in general, they are less sensitive to pressure/changes in pressure when they are being punctured.
Perhaps your daughter (I believe you are talking about your daughter? If not please correct me!) is experiencing similar issues that I did?
If so, and this is just my gut feeling, I don't think you need to worry so much about the problem.
Drink lots of fluids before the transfusion and/or before blood draws and be patient as the nurses/doctors work with you and your daughter to find the best site for the transfusion.
For myself, the inside of my wrist (closest to my thumb) has been the best place for an IV if the inside of my forearm (bend of elbow) will not cooperate. The top of my hand also works well, usually, but the inside of my wrist closest to my thumbs seems to have worked the best.
Also, I have been told by a blood draw specialist that the veins in the dominant arm (the arm that is used for writing - right handed, left handed, etc) are usually "stronger"/less prone to collapse compared to the less dominant arm because the muscles of the veins have had more exercise.
Perhaps this is something to be taken in consideration as well?
All the best!
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so pls can u name any natural vit e that u know i can buy that frm medical store......
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thanx HBh
i was so confused with all this but lemme tell u tht my daughter never had any issues as in finding the veins or getting the blood from the veins .....if so the way u ve explained any dr could also have done that then y didnt he do tht i wrote a mail to him thrice cos as my daughter goes for transfusion twicwe a week and she had the issue thrice so i mailed him but no replies
any ways thanx
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can any body suggest me a good homeopath in india rather mumbai cos even thru the various posts i ve seen tht homeop[athy is a good way to minimise the issues
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Dear Andy I have a question regarding beta thal trait. Could you please tell me if being aneamic with beta thal could this cause a fit? How does this happen and can it be avoided/stopped?
HbH...both my boys are exactly the same as you with the veins but they do not have low blood pressure, theirs is always a little high before they start transfusions :smiley.
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Hi Emby,
By fit I assume you mean seizure. Correct me if I'm wrong. I will refer you to a previous thread on this topic. This is one of those things where we know thal minors have them but there is uncertainty if there is any connection. Please go through the posts in this link and see if anything looks like it could be a possibility. Various things like hydration and medicines are possibly connected, as are sugar levels.
http://www.thalassemiapatientsandfriends.com/index.php?topic=1750.msg14546#msg14546
As it is almost always with thal minor, there is so little research that we can't say for sure. We always need to remember that thals are part of the general population and should be expected to have various conditions at the same rate as the general public, so it makes it difficult to say for sure that some things are the result of thal.
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Thank you Andy....I needed to know because it happened to someone I know. They are beta thal trait, had blood tests done and waiting to have a head scan.
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Dear Andy...would like your opinion..
Our boys are taking vitamin D, the oral solution, and with this their levels pick up quicker. However, for BB once the levels are normal he switches to 1000iu daily but then it falls quite significantly in about 6 weeks- 2 months. LB's level maintains at satisfactory level longer before dropping again then they start with the colecalciferol solution again (10000iu daily).
Would you say this is a normal pattern and is it ok to take the vitamin like this continuously?
Both the boys copper levels tend to drop so they are taking supplements. When the levels pick up they stop taking the supplements and only start taking it when it drops again.
I know it makes sense to take it this way but do you think thay should take the supplements continuously?
BB has also had reaction to blood at the time of transfusion sometimes severly though the last time was about 2 years ago. He always has pirriton and hydrocortosone (!) before tx...I am thinking that perhaps of the hydrocortosone his vitamin D level may drop faster. Is this a possibility?
Do you think he could just have pirriton through the vein instead as the other is a steroid and because he has not had a reaction recently?
Its not fair to ask you so much when you have a painful elbow, hope you feel better soon.
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Hi Emby,
If the vitamin D levels are dropping when switched to a lower level, you should probably raise the daily maintenance dose. Try doubling it. I am taking 5000 IU 5 days per week and my level is finally in an acceptable range after one year at that dose. My doctor said I should continue that dose, but in summer when I get a lot of sun and took no extra D, he told me to take 5000 IU once per week. Prior to that, 2000 IU daily had got my level up to only 19. My doctor wants a minimum of 35. I wouldn't be concerned about getting too much unless their levels get above 80. I also think it would be best if you found a maintenance dose of copper, rather than waiting for it to drop. Every other day of 3 times per week may prove to be adequate.
Vitamin D is depleted by steroids, so it makes sense that BB's D would drop while using hydrocortisone. Vitamin D plays a protective role in the bones when taking steroids, but that results in a vitamin D deficit, so it should be replaced. I can't say if you should stop the steroid. Talk to the doctor about this. Perhaps a trial without the steroid could be tried. In most cases, the antihistamine is enough to prevent the reaction.
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Andy,
Little A's vitamin D was was 93 a few months ago. I stopped the 50,000 IU a week - but continue to give him 3000IU daily. Should I stop this dose for a while because his levels are so high?
Thanks Andy,
Sharmin
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Thank you Andy for helping us to understand better.
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Dear Andy And Friends
I m suffered with Thallassemia major .
i have Osteoporoses and im Under weight cane you give the suggestion about exercise for my losing weight.
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Hey Andy!
I have a question for you since i didn't found an answer on google!
In thallasemia major, does our bodies ( bone narrow ) produce stem cells ?
There is a drug named Stemulin, which acts like a stem cell activator to restore health of damaged organs. I thought it will be good for our spleen, liver, and why not to increase time between transfusions............ but the problem is that it only activates stem cells and multiply them; that's why I need to know if our bodies produce stem cells before i buy this thing!
Thank you!
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Yes, the bodies of every living person produce stem cells constantly.
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Daniel, you mean something as epo? I think so...
I do not know anyone who takes it, but it let me wonder daily of it would help...
Did we met by chance at the conference of Asociatia Romana A Persoanelor cu Talasemie Major (ARPTM?) held in Sinai in November 2010?
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Did we met by chance at the conference of Asociatia Romana A Persoanelor cu Talasemie Major (ARPTM?) held in Sinai in November 2010?
I wasn't able to go to Sinaia then.....I didn't know that you went, but you met my friends there!
Thanx for answers! :)
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Aha, it is still nice. I am sorry you could not made it. We have learnt a lot. I was with a friend who controls the thalassemia department within our organisation. We were the Dutch delegation. Will you be able to join your group to Turkey? I am not sure of they will come btw.
Your health is overall good?
Say hi from me to all! I've learnt a lot. :)
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Hi Andy,
My son, Jayden, is 4 yrs. Old and he has Beta Thal Major. My husband and I are currently getting him treated at John Hopkins. We have chosen LA children's Hospital for his BMT. Do you think we have made the right choice in choosing that center? I also read that Texas Children's Hospital has done a number of BMTs on Thal major's as well. I just want my son to go to the hospital that has the most experience. Thank you for your time.
Lo
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LA Childrens has one of the top thal centers in the US. I have complete confidence in them. They should be able to provide you with recent success rates on request. In recent years, the better centers are well over a 90% success rate. Check with both centers to compare the numbers of BMT's done for thals and the rate of success for each hospital. With that said, I would choose LA.
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Hi Lo,
Good luck with the BMT, :hugfriend keep us posted.
Zaini.
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My son is now 7 and a half years old. He had a ferriscan 3 years ago. The iron level around the organs were considered good at that time. The doctor is not too willing to have another ferriscan for a while. His ferritin level is around 1000. He is taking exjade solely. Never had any other type of chelation before. He started exjade at the age of 3. Should he be doing more checks?
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Annual tests are recommended. At some point, a T2* scan of the heart should also be done, as liver iron levels don't correlate with heart iron levels. Ferritin tests are good for regular monitoring, but organ iron has to be evaluated, also.
http://www.thalassemia.org/updates/R2T2.pdf
How often should these tests be performed?
Ideally, these tests should be performed yearly, as part of a
thalassemia patient’s annual comprehensive care
examination.
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Hi Andy...just a quick update on my younger son's reaction to Exjade. The rash completely disappeared in exactly 1 week. He was covered in the rash from head to toe but continued with Exjade and also took Piriton 3 x daily. when it came it spread quickly all over about after 1 week of starting on the medication with slight swelling on his face and lips and was really itchy. And when it started to disappear, it was a bit weird, it first went away from the face then neck then chest, arms working its way down the whole body.
He was taking 1500mg (split dose) but now he takes 2000mg daily.
.......Now he has started wearing braces.... Is it okay to drink the Exjade solution as normal even though it comes into contact with the metal in the mouth or to use straws?
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Hi Emby,
The original warnings about metal and Exjade were out of concern over a reaction, but it turned out that the concerns were unwarranted. Don't be concerned at all about the brief contact with the braces. I'm glad to hear that the rash cleared up as expected.
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Thank you Andy...he will be happy to bin the straws and just swig it down :biggrin
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Andy...though my younger son has split dose, having read somewhere here that Exjade does not last in the system for longer than 18 hours (one place stated 16 hours and one, 18) and that the effect of it in the body is higher during the first few hours of taking Exjade, it becomes less effective during the hours after. Also taking split dose would mean true chelation for the full 24(plus) hours. So I was thinking that wouldn't this be putting more pressure on the kidneys especially as Exjade can have more of an impact on kidneys as the split dose is overlapping the 24 hour period?. Maybe the 6 hours of not having Exjade in the system is to give the kidneys a rest because of it's effect on the kidneys.
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Emby,
It has been found that the opposite may be true, because when we talk about the effectiveness of exjade we talk about its effect on free iron. The kidneys are involved with removing exjade from the system. Administering lower divided doses decreases the side effects on the body as it is likely more harmful to hit the body with large doses of the drug. It is much easier on the kidneys to deal with smaller quantities of exjade over time rather than one large dose. This is true also for the stomach, the liver as well as the kidneys. The lower likelihood of irregularities in liver function and kidney function provide evidence of this.
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Hello Sharmin...nice to hear from you..hope you are well and especially that special young man you have in your life..hope he is doing well.
I hear what you say but once the body is used to Exjade it shouldn't have major side effects and split dose might still be making the kidneys work harder...and with that we have no long term evidence on impact on kidneys for this. I am asking about this because Desferal can be given full dose and works best given over a period of 12 hours and the full dose for Ferriprox is split 3x daily working best taken at 8 hour intervals covering 24 hours (and not more than 24 hours) and the both of these chelators are hugely successful. I don't think people stick to taking them strictly at these hours so they have a few hours a day the meds are not having the supposed effect in the body yet most still take these meds religously and have been successful managing their iron levels and maintaining healthy organs. The Pharmaceuticals must know this and may have advised taking Exjade as a single dose to work for 18 hours for better balanced management of iron long term and especially with the kidneys as it's working and resting the kidneys for 24 hours without the 24 hours bleeding into the next 24 hours. I'm sorry if I don't make sense.....but I'm just trying to undestand something here :-\
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....maybe good to split then after a while maintain with single dose?..
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The once daily dosing for Exjade has far more to do with marketing than science. Once a day sounds good and it is what Novartis felt patients wanted most. Oakland has endorsed the split dose method. The reports we hear about split dose are universally positive.
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then I guess its just wait and see...
Both of the boys are not that bothered with 3 times a day over once daily....what does matter is effectiveness plus safety :yes.
My younger son had to take Exjade because of liver iron increasing with ferritin already quite low and I'm praying, praying, praying,
God willing inshaAllah, that it works for him.
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then I guess its just wait and see...
Both of the boys are not that bothered with 3 times a day over once daily....what does matter is effectiveness plus safety :yes.
My younger son had to take Exjade because of liver iron increasing with ferritin already quite low and I'm praying, praying, praying,
God willing inshaAllah, that it works for him.
Oh your both boys are thal major? I pray for their good will. :)
-P.
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Pratik...they are 2 incredible blessings sent to us by Allah the Most Merciful. My husband and I were given the best 2 boys ever.
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Hello Andy,
My younger Son is doing well with Exjade....though we don't know what his ferritin reading is like since he started (400 at that time).
He needs to take it to reduce liver iron. I would like to know that with split dose would this make the ferritin reduce more when the point is not to reduce the ferritin then would a single dose help with the liver iron and maintain a good ferritin level?
A thought just came when typing this...if liver iron levels is steadily going up there may be other organs with increased iron that has not been seen yet. May God protect them from harm. Ameen.
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The ultimate goal of chelation is 24/7 coverage. This is because iron is constantly being released from the red blood cells that are being eliminated from the patient's body. When Exjade is taken only once per day, there will always be gaps in the coverage, creating the opportunity for iron to build in tissue. Even at low ferritin levels, the best way to keep the organs clean is by chelating 24 hours a day. Once the ferritin drops below 300, adjustments to the total amount of chelation drug used are made, but the technique does not need to be altered. There is some correlation between liver and pancreatic iron, but the connection to cardiac iron levels is not so clear. The best way to keep all organs clean is by ensuring there is always an active chelator in the body.
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Thank you Andy....would be more clearer after scan. God willing, hoping it helps..
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God Bless you Andy....
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Hi...hope you are well . Andy or anyone please can I bother you for some help.
Four weeks ago my younger son H had very bad leg pains. The pain was felt in the muscles not in the bones.It spread to the stomach then his shoulders and arms. This lasted up to 4 days. That was 4 weeks ago. Yesterday all of a sudden the pain in the muscles of his legs came back, much stronger this time. H couldn't even manage to walk. We took him to A&E. Bloods were done and urine sample taken. The urine sample came back showing sugar and blood and the urea and creatinine levels were elevated. They don't know what the is cause of the pain or why the urine sample has come back as it has as his past history has been so good thank God. they did perform some test for blood sugar but again thank God it was normal. In between now and when he first had the pains he has been having problems with the stomach like burping alot and without having eaten, the feeling of being full. That's how it starts...burping, felling heavy in the stomach then the leg pains start. H has been on Exjade since end of December 2012. His ferritin was around 150 and he takes the tablets split dose, 2000 mg in total. Could this be too much for him? Could the Exjade be effecting the kidneys?
What could be the cause of the muscle pains, stomach cramps and the urine thing?
Its effecting him alot..he is not sleeping..eating properlly and when the pain is severe he can't walk.
Sorry for a lengthy email and hope you can make sense of what I have written.
Emby
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H has been admitted into hospital today. He has been told not to take anymore Exjade at present. They are putting him on the drip to help clear the kidneys and because of him not having drunk too much liquids. The glucose level is not of concern he has been told and should clear up...he has had blood tested a few times already and alhumdolillah was normal.A scan for the abdomen and kidneys has been requested. The doctors will keep him under observation....prayers needed.
Emby
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Exjade can affect the kidneys and that is why the serum creatinine reports are done regularly. Staying well hydrated is essential, but even then, some patients will have toleration problems with the drug. I think we should wait for the further reports before trying to speculate. It may be that Exjade is not suitable for him and another chelation choice will have to be made. The kidney problem can explain all the other problems too. If creatinine clearance is low, muscle pain will occur. I think his problems will be corrected once his kidneys have become irrigated and flushed.
This is a warning to all parents and patients about the side effects of Exjade. Both serum creatinine and liver functions tests must be done at least monthly to ensure that the drug is not causing dangerous side effects. In between testing observation must be constant. Health problems with no apparent cause should be immediately reported to your thal doctors. All chelators require regular testing. Be vigilant and never skip the tests.
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Thank you Andy....I hope it really is the Exjade because they are not sure what it is yet. H did't sleep at all much last night maybe just 45 minutes and he has been vomiting alot even though he cant eat anytging, keeps bringing it back up. Some blood results:
Urea 11.4
Creatine 138
Alt 53
Bilirubin 71
Had Vitamin K to help clotting
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scan done of liver spleen pancreas stomach kidneys.....alhamdolillah all is well. Drs saying they think it is due to exjade. Praying he gets better soon with God wills.
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The creatinine clearance is good, so the kidneys seem safe. I'm not certain about the bilirubin number. Either it's a different scale than what I am familiar with, or he may have a gallbladder issue (gallstones are common in thals). Has he been keeping well hydrated while using Exjade?
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He doesn't have a habit of drinking between meals...maybe just up to 2 1/2 to 3 litres.
He is not allowed food or liquids at the moment. Was not able to keep it down at all.
Allah is taking care of him...
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Andy, H's reading for the pancreas is 300...the urine still shows glucose though the bilirubin and creatinine has reached normal levels. His blood test show that he is dehydrated so he has had 500ml of liquid twice now transfused quickly within 20 minutes and has been on liquids anyway round the clock since Sunday. His blood pressure is 150-162 over 110 and there has been problems with blood clotting and has 2 doses of Vitamin K via the veins. Alhumdolillah he looks well and the leg muscle pains have gone.He is on antibiotics to fight any infections he may have. Do you have any idea what could be happening at all? Can you ask for advice from someone....I am very worried.
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I don't know if it is the entire cause, but I suspect a combination of Exjade and dehydration is at fault. The effects of Exjade on the kidneys are well known. Sufficient hydration is needed for all patients on the drug, so that creatinine can be properly cleared from the body and not build in the muscles, where the noted pain has been observed.
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What does it mean pancreas level of 300??
Is it T2*Score of MRI?
I have heard that pancreatic iron can be assessed by MRI
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Pancreas AMYLASE 0-137 U/L normal
They say the cause of it is Exjade.
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Emby,
Between the muscle pain which shows some evidence of kidney problems and the pancreatic level, it may be necessary to cease Exjade permanently. Raised blood levels of Amylase are mentioned over and over in case studies of patients using Exjade and having issues. Dehydration is also mentioned over and over.
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Yes that is how I feel but H's Dr said we might start him back on the Exjade at alower level...but I have been clear to the doc about what I feel about that....
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If Exjade is to be re-challenged, it has to be with the understanding that he has to stay hydrated all the time. He's going to have to be the one that makes the effort. With that said, why not Ferriprox?
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Emby,
I just saw your posts. My guess is that if all tests done are within normal, you can blame it on exjade.
Really, what about ferriprox? Has your son even taken them and if not, why?
I wish you the best for your son,
Lena
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I am a little upset these days, due to my MRI getting worse:
T2* heart 31 (last year 34,2)
T2* liver 27 (last year 31)
LIC 1,1 (last year 1)
Although this year's rates are still within normal, they are worse than last year's.
I am trying to find out why and I think it is because in my combination therapy I let the desferal rate drop and had it twice weekly together with ferriprox. You see, my ferritin was 37 and we thought we could afford it. But no! things proved that I could not reduce the rate. Something I have posted again and again in this site. Do not slow down. Now I have to double my effort in order to reach my former rates. :wah
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Thank you Lena...... both my boys were happily on Ferriprox. My older one still takes it. H's liver showed that he had moderate level of Iron so his Dr said he should go on Exjade. I was happier just to stay on Ferriprox but the Dr said that because his ferritin was around 150, she didn't want the ferritin to drop any lower and that Exjade was the best option. I was happy with Desferal/Ferriprox combo but she was determined.
I'm sorry cos I know people do take Exjade, and it works for them, but I just have never felt comfortable with Exjade. I had researched it and there is something about the way it was sort of ''given out'' to the masses that irked me. As I said, it does work for lots of people but it didn't work for H....he is getting better and inshAallah there will be no lasting effects.
Thanks Andy....don't want to go back on Exjade. Within 6 months of being on it being at the hospital we only then realisised all the essentials he has been losing from his body, potassium, phosphate, vitamin k, sodium chloride, glucose. Kidneys playing up, liver gone weird, pancreas abnormal, stomach reacting, blood pressure sky high, blood not clotting as normal. Restarting it would be.......
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Thanks for saying all that about exjade, Emby.
I myself was on exjade one year ago and had terrible stomachburns and doctor gave me stomach pills in order to go on. Then, I made some search and decided on my own to stop exjade.
The truth is I was considering again now to start with it, but I am very reluctant. Thanks for all the info you put through.
I think I will stick to desferal/ferriprox (though a harder therapy it is.)
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Lena you really are someone to look up to. And mashaAllah, as God willed, have done really well on the combi....I know what I have written but I also believe that no harm can come to you unless God wishes and no good can come to you unless God wishes and even if harm comes to you and even if good comes to you, it is only through His Eternal Wisdom and He wants the best for us...I believe that.
My internal feeling was not good when H was told to start on it and maybe it was Allahs way of saying something to me, that this would not be good for him and then He allows some people to take it and IT IS good for them....sorry I'm babbling on, it's just what I believe.
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Lena,
I commend you on your constant vigilance. I am curious about your experience at low ferritin levels. Does your diet affect your ferritin level? Do you find that if you eat high iron foods, that there is an effect on your ferritin? I am curious because the official recommendations for thal diet is to not worry about iron rich foods, because the amount of iron is so much less than what is in one unit of blood.
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Hi Lena,
Little surprised and happy to see you on board after a long time. :biggrin :biggrin
Your Fe, scan results are really fantastic..
Hope you will get ur desired results early.
May i know your bt requirement at present?
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With H things were being drawn out of his body along with the Iron while on this chelator, so Lena do you take sipplements or have you had take something major while your ferritin has been so low and have you always felt well physically? Do you think his Dr is right to be concerned about lowering ferritin levels further?
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H has been told to start eating and drinking again alhamdulillah. The amylase is now 36 and slowly other issues coming back to normal. The Dr says it takes a week to flush Exjade out of the system...so slowly nearly there inshaAllah. He might need to carry on with some meds at home. Still at the hospital...may Allah grant him shifa...ameenl
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Hello Andy, Emby, Hanumant Aashrit,
Andy,
I totally agree with the official recommendation that the food iron is inconsiderable and much less than the iron we take with our transfusions. Never have I taken into account the intaken food iron. If you chelate well, there is no matter at all about the iron rich food you take.
My diet has not affected my ferritin level either high or low. Ferritin is always connected to chelation.
Hanumant Aashrit,
thanks for your kind words. By bt you mean blood transfusion? If yes, I get one blood unit every 2 weeks.
Emby,
I am very touched by your trust in God. This is something I have noticed about the Muslims and I respect it. In Western world we have lost this touch. I am sure H wil be o.k since he has stopped exjade and his body comes back to normal. Be patient.
In my Thal Unit we take a lot of supplements, but I think you already know about them as Andy has mentioned them again and again.
No matter how high or low my HB is, I always feel the same. Since I was a kid, I have been feeling the same. That goes with ferritin, too.
As for H's ferritin, the lower the better, always within normal ranges of course. And the MRI is to be done every year. Some doctors say every 2 years if the results are good. I do not agree, because if you do it each year and the results get worse, you can correct it by making stronger efforts while in two years, you would have lost a year and then it is harder to correct it. (My personal opinion, anyway.)
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Thank you Lena... :heartpink...we are told that there is a big reward in practicing on patience and especially so at that very moment when something happens, from the Quran that Allah is with the patient ones and Allah loves the patient ones. But we should not ask Allah for patience because that way we are asking to be inflicted with something cos you can only practice on patience when you ARE inflicted with something instead ask for Aafiyah, safety security and ease, in everything cos that way Allah will bring you out of that thing with safety security and ease..inshaAllah.
May Allah grant you Aafiyah always, ameen.
My boys too feel mostly the same with their Hb up/down though sometimes a little tired but it does not affect their day to day things...I feel more tired than them. And it is right that even at low ferritin
it would be even more important to take supplements but we are not advised that here. Just stay within the normal ranges and that's it. I agree with what you say about the check ups also.....this is all a learning process..take a lesson from it..understand...then move on..xx
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Thanks Lena. The normal ferritin level thalassemic is a new phenomenon. You are at a ferritin level where it would be easy to measure if dietary iron was a factor on ferritin level. It would appear that avoiding iron rich foods is of no value to thals whose Hb levels are maintained at a good level.
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You are right, Andy about the normal ferritin thal. It may be new phenomenon but it is increased little by little. That is why doctors should study this new entry in thal and take into account that there are cases where chelators, when given in same dosage, may be toxic. Desferal has started to give out cases of macula, if I use the right medical term. This is an eye problem which leads to blindness. This is a problem that emerges after long usage of desferal. I mean there are problems that come out when ferritin is low and these have not yet been studied. We have stepped however to a new thalassemia era and this is to be considered.
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Hi Lena,
Thanks for the reply.. :biggrin
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Lena....with H's ferritin at about 300 now, and given what has happened with the Exjade, what do you think would be best for him in terms of chelation? He is in recovery and may take a while so might not take any chelators for a couple of weeks.
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Emby,
we had a seminar in our Thal Unit yesterday and doctors from other Units, too, were invited. Normal ferritin level was said by all to be below 200 and LIC<3 (although our doctor says 1,5), T2* heart and liver to be >20. If you have not reached this level, you are not to slow down at all. Hepatologists said that whether you have Hepatitis C or not, if your liver iron is above normal you are at risk to get liver cancer in a number of years. This is a possibility to be considered. These are the most important statements I heard yesterday.
So, as you realise one is not to relax. My guess for H is to proceed with a combination therapy, ferriprox and desferal together. Has he done an MRI? could you care to write the T2* score for heart and liver as well as the LIC score?
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Sorry..what is LIC?
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Liver iron concentration.
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Thank you Andy
2012: T2* Heart 58ms
T2* Liver 2.4ms (2010 was 2.1ms)
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InshaALLAH will find out LIC score
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Dr has said that H will not given chelation for a couple of months...
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His scores are quite good at present. Hopefully the break in chelation won't be difficult to overcome once chelation resumes. In the mean time, make sure he is drinking tea regularly. Hot tea. Cold tea. Whichever he prefers.
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Emby,
T2*H seems high enough and that's good. T2*L is a moderate iron liver burden. Here are the rates:
Liver T2* >19,1 normal iron burden
9,1-19 successful chelation
4,1-9 mild iron burden
1,6-4 moderate iron burden
<1,6 heavy iron burden
Heart T2* > 22 normal iron burden
14-21 mild iron burden
8-14 moderate iron burden
<8 heavy iron burden
LIC <1,5 normal iron burden
1,5-2,9 successful chelation
3-6,9 mild iron burden
7-13,9 moderate iron burden
>14 heavy iron burden
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Emby,
Was the liver scan a Ferriscan? If so, the readings are adjusted to coincide with the dry LIC, so a low score is better.
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He loves tea!
The T2* for liver was done with MRI. He would be having the Ferriscan done very soon inshaAllah.
He has been discharged and is back home..quiet weak...but thank Allah getting better. It was such a scare and I would say drink, drink and drink even if you think you have drunk enough then still drink with Exjade and if you feel something is not right then get it checked straight away..you know your body better than anyone else and as a parent you know your child better than anyone else.
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Thank you Lena and thank you Andy... :heartpink
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Andy you mentioned that Amylase problem has been reported regarding Exjade so can you give me that information. H's Dr said that there is no connection with Exjade and that she contacted them but they do s not have any reports this. She did say that everything else was caused by Exjade.
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It's not a common side effect bit increased amylase is on the list of observed side effects in the Exjade study that followed sickle cell patients for 5 years. If levels return to normal while not using Exjade, I would suspect Exjade as the cause.
If his Ferriscan shows a high LIC, there may be no choice but to use desferal.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3170481/
Serious AEs were experienced by 131 patients (70·8%) overall. The most common serious AEs, which were reported in ≥10% of all patients, were sickle cell crisis (n =67, 36·2%) and pyrexia (n =34, 18·4%). Deferasirox was permanently discontinued in 12 patients (6·5%) following serious AEs. Nine patients (4·9%) had 17 serious AEs that investigators suspected to be related to deferasirox treatment, including gastrointestinal disorders (n =3), sickle cell crisis (n =2), lymphadenopathy (n =1), tinnitus (n=1), granuloma (n =1), tuberculosis (n =1), spontaneous abortion (n =1), pulmonary thrombosis (n =1), increased alanine aminotransferase (ALT; n =1), increased aspartate aminotransferase (AST; n =1), increased blood alkaline phosphatase (n = 1), increased blood amylase (n =1), increased blood bilirubin (n =1), increased lipase (n =1) and increased transaminases (n =1).
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Yes the amylase reading went up to 900 but now has come down to a normal level,l 36. His Dr said that it wasn't pancreatitis, with such a high reading, because he didn't have the symptom that goes with it... agonising pain in that area. I also think it had to do with the Exjade.
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Lena can you please tell me how much Ferriprox and Desferal you are taking at the moment. Would help me to understand which direction to go in with H.
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Andy, H's Dr wants to keep H off of any chelation for a few months ???. I know that his body needs to recover from the effects of Exjade but I'm worried about iron overload. Shouldn't he be starting back with even a tiny dose of Ferriprox perhaps or to give time for his organs to recover?
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I think he should wait. His organs have been through an ordeal and need time to recuperate. I would suggest natural chelators like drinking lots of tea and IP6 (inositol hexaphosphate) if you can find it. And keep him well hydrated.
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Thank you for the advice :smiley
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Can anyone please advice which is better to take for Vit D, cholecalciferol or ergocalciferol ?
Thank you.
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Vitamin D3 (cholecalciferol) is preferred.
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Hello Andy,
I would like to know if reading for Urea is a bit high and creatinine a bit low for thals, is this okay?
Thank you.
Emby
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HI Emby,
Low creatinine is fine and it's the high end that is problematic when chelating. Low means the kidneys work well. Slightly high urea may just reflect the level of hydration during the test. It's not an issue.
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Thank you Andy :smiley