Thalassemia Patients and Friends
Discussion Forums => The Spotlight => Topic started by: Jos.- on April 26, 2009, 10:19:39 AM
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Hi,
My name is Jos.-. On April 17, 2009 I was diagnosed having Beta Thalassemia with HbE-disease. So I'm rather new to this idea itself and this 'part of the world'. Looking all over the internet for information. My specialist for internal diseases told me that I'm heterozygote and there were not many bad effects to be expected. But he prescribed me Exjade to get rid of my too high serum ferritine.
I didn't start yet with Exjade. Looking around on the internet I'm not very happy with all the stories about the side effects of this medicine. Is there anyone who can tell me some more about Exjade? Are there other parts of this forum to look at or ....
Kind regards,
Jos.-
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Hi Jos
HbE-beta thal is usually less severe and most patients may or may not require hypertransfusions. How old are you and how was your thal diagnosed ? Since your doc has prescribed you exjade he probably must have conducted your liver and kidney function tests and found it ok. My three year old has been on this drug for the last 6 months without any side effects, so you don`t have to worry much about it. Do check out the posts in the iron chelation corner, you will probably find all the answers to your queries and if you don`t just question.
welcome to the site
maha
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Hi Jos and welcome to the site
How much is your serum ferritin???
manal
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Hi Jos
Has your serum ferritin been checked and have you had any liver or heart scans to to give an accurate measurement of your iron load? Even without transfusions, your iron can get high because your gut will absorb too much iron from food. There are some things you can try to reduce this absorption and there are also natural supplements that can help reduce the iron load, but an analysis of your true iron levels is a must.
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Thanks maha, Manal and Andy for your kind replies. Let me give you some answers to your questions.
* First of all some personal data: I'm 60 years old, live in the so called Green Heart of The Netherlands, work at the student affairs department of an university. I'm married and we have two kids studying at two other universities in The Netherlands
* Last January I decided to take part in a health check organized by my university. 'Let's see how healthy I am!' ;D
I didn't had any complaints till then
* Results: serum ferritine 1255 ug/l, plus too much glucose and too much cholesterol. 'Of course' my blood pressure was too high too.
* the medical examiner referred my to my GP who in turn referred me to an internist. The first diagnosis of all three doctors was hemochromatosis
* In last February in hospital I had several extensive blood tests resulting in: no hemochromatosis, we have to look further. On April 17, 2009 I got the word: heterozygous beta thalassemia and heterozygous HbE-disease. So I'm a carrier and don't need blood transfusions. Because there was no hereditary hemochromatosis I got medicines for the diabetes mellitus type 2 (metformine) and for the cholesterolemia (atorvastatine).
* Last February I also had an ECG and an echography. All was fine except for a too fat liver (don't know the correct word in English).
* On April 17 my blood data regarding cholesterolemia, diabetes and blood pressure were really good because besides using the drugs I live like a monk with respect to my diet. My serum ferritine which was at the start 1255 came down thru 1003 to 800, but went up to 920 ug/l, probably as a result of atorvastatine. So my doc prescribed me Exjade (deferasirox) to get that serum ferritine down and referred me to one of the sickle cell centers in The Netherlands where all patients with sickle cell anemia and thalassemia have to be treated/examined.
So that's the story to my rather simple question 'are there any positive results about Exjade and it's side effects to tell?'
Thanks in advance for another round of replies.
Jos.-
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Jos,
Thousands of patients around the world use Exjade daily with few if any side effects. Most side effects are temporary and can be minimized by using lower doses to start and working your way up to the effective dose. With that said, it is only honest to mention that there have been deaths in patients using Exjade and most of these have occurred in older patients, but of great importance is that most patients who have died had a long history of kidney and/or liver problems. Because of your fairly low ferritin level, you may be able to take a lower dose, as you don't appear to be dealing with an ongoing iron buildup, although an MRI scan would help verify this.
Your ferritin is high but not dangerously so. If you are interested in trying a natural approach, there are several things you can do to lower your iron level. Observing a low iron diet is the first step. Using some simple changes to eating habits will also affect how much iron is absorbed from your food. Eating dairy and drinking tea with meals will lower the iron absorption in your gut. Vitamin C increases iron absorption from food so citrus fruits and juices should be taken only between meals. There are also some commonly used supplements that are known iron chelators and also strong antioxidants which can help protect your body from the damage excess iron does to the cells. IP6 (Phytic Acid or inositol hexophosphate) and green tea extract are both in this group. Many members of this group have seen a change in skin tone within weeks of starting to use IP6 and this shows a reduction in iron. Because your ferritin is not terribly high, and if you're in no hurry to use a drug to remove iron, you may want to investigate a natural approach first.
HbE and beta thal carrier together can actually result in a transfusing major, depending on the particular beta mutation. Some HbE beta thals manifest in more of an intermedia condition, where transfusions aren't needed but hemoglobin levels are significantly lower than normal. One effect seen in some with non-transfusing intermedia is excess iron absorption and the effect is sometimes much higher in organs than ferritin tests would indicate. Because diabetes can be caused by iron overload, this should be investigated. Again, if you have not had any scan of your liver or heart for iron load, this should be done. Do you know what your hemoglobin level is and do you have a history of a low hemoglobin level?
Also, your children should be tested to see if they are carriers of either gene, as this is important information to know when they choose to have children of their own.
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Hi Jos,
Welcome on the forum :urwelcome About your exjade, personally i don't think your serum ferritin is that high that you should be using exjade,exjade has a few side effects like any other medicine,and people who already have liver problems should not be using it,because it can be fatal as it can result in hepato failure.IF you already have problems in liver,other wise there are quite a few members here,including my 8 years old daughter who are using exjade,but the problem with these patients is that they keep recieving huge amounts of iron through regular transfusions,but thats not your case,so i think you should first try some natural ways to lower your ferritin.
First of all,don't take too much iron rich food,red meat,i assume your doctor must have asked you to either stop or cut down due to your cholestrol? Its good because red meat is the main source of iron.Between do you know what is your hb level? Start taking tea with meals as tea inhibits iron absorption from food.
One thing you should look into are anti oxidants like IP6 ( Inositol Hexaphosphate) Its a powerful anti oxidant and a mild natural chelator too,It will help avoiding damage caused by extra iron in the blood and will help you chelate naturally,of course its not powerful like exjade but its natural and has no side effects.
I hope this helps,if you have any other questions in mind,please feel free to ask :) .
Zaini.
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Andy, Zaini great minds think alike ''at the exact same time'' :rotfl :rotfl :rotfl
manal
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Manal, :wub
I didn't see Andy's post first otherwise i wouldn't have posted the same thing,and its a coincidence that we posted by the difference of few minutes :biggrin.
Zaini.
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Thanks Andy and Zaini for your responses. I'm going to look at them right away. And of course to the Iron Chelation section of this forum as Manal suggested earlier on.
A quick respons @ Zaini: my Hb is 8,2 during all of the four times they examined my blood the last few months.
Jos.-
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Hi Jos,
If its 8.2 gm/dl then its quite low,that makes sense that your body is absorbing more and more iron,when hb is chronically low,body tends to absorb more and more iron through the gut,so i think avoiding iron rich food,tea after meals,no citrus fruit with the meals and IP6 will hopefully solve your high iron problem,it may take a little bit time to do it the natural way,but i'd say its better then taking exjade.
For your low hb,please take folic acid,and if you can find wheat grass it may help you,even if it won't raise your hb you will feel a big difference in your energy levels.
Hope it helps :) .
Zaini.
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Hi Jos,
Have you ever been told you are anemic? Hb of 8 is classified as thalassemia intermedia. It's high enough to avoid transfusion, but low enough to have an impact on many quality of life and health issues. In light of your low Hb, I think it is imperative that you do have a liver MRI or heart scan (T2* is the most accurate measuring tool for cardiac iron load) to determine if your iron load is higher than your ferritin would indicate. It is quite common for intermedias to have ferritin levels that are high but don't look dangerous, but actually have a high iron load in their organs. This really needs to be determined to decide the course of your treatment. If it is a simple matter of high ferritin levels, this will be easy to address with short term chelation and possibly at a low dose. If the organs are involved, you will have to chelate accordingly. If your organ iron is high, it means that there is an ongoing problem of excess iron being absorbed in your gut. Regular chelation would be needed if this is an ongoing problem.
The dietary tips given previously are very important when thals have low hemoglobin levels. Do all you can to minimize your iron intake and avoid iron cookware. The body will absorb more iron than it needs when anemic, regardless of the cause of the anemia and in thalassemia, low iron is not the problem, so be conscious of your iron intake and try to take tea with your meals.
I find it amazing that this was never diagnosed before in your life.
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Hi Zaini and Andy,
Thanks again for your responses, although it's not fun reading them, because it underlines the possible seriousness of my current health problem.
To be as clear as possible, I'm afraid we use different measures for describing Hb. In the Netherlands mmol/l is used as a measure for Hb. The normal levels for men are 8,5 - 11 mmol/l. According to Wikipedia 1 g/dL equals about 0.6206 mmol/L. My arithmetic isn't that good this morning to be able to calculate how much 8,2 mmol/l is expressed in g/dl. Let me say it is (just) a little below the minimum normal level for a grown up man.
So no one ever told me that I'm anemic. And in my opinion I never had any reason for thinking of having for example an energy problem or what ever problem with quality of life or health issues. Because of that my blood wasn't examined for a long long time.
As I understand out of your reaction Andy an MRI is really necessary, the echography plus ECG I had are not enough to know the real situation of my organs and to decide the course of my treatment. Does this still stands now our confusion of tongues is taken away? By the way what does T2* mean?
I will try to follow the tips regarding the diet.
Thanks once again. Great site, great people.
Kind regards,
Jos.-
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I remembered something our member Dore, also from the Netherlands told us about hemoglobin measurement, so we need to clarify the unit of measurement in which your hemoglobin level is expressed. Is your Hb listed as g/dL or mol/L? this makes a huge difference. If it is the latter, your Hb level would actually be normal.
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Jos,
Thats why i wrote "IF its 8.2 g/dl" because i had a hunch that you were not using this unit,as our member Dore is from Netherlands and once i got confused with her readings too.
God its confusing,it took me an hour to convert this :rotfl your Hb is 13.2 g/dl which is quite normal.:)
Zaini.
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It sounds strange to me to have a HB of 13.2 with a HB E and a thal minor mutation??? Andy what are your commets????
manal
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Thanks for converting it Zaini. I couldn't do it myself :wink And yes Andy, my Hb measurement is made in mmol/l. I'm sorry provoking this misunderstanding by not mentioning the kind of measurement at once.
So if my Hb is rather normal what about the necessity of having MRI scans of my liver and heart?
Jos.-
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What i am unable to understand is that if your hb is normal,then why is your ferritin high ? :huh Were you ever transfused at any time in your life? For any reason?
I think we all have to wait for Andy's response.
Zaini.
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Hi Zaini,
No, I've never had a transfusion. All my internist could tell me was that the origin of my high serum ferritin is the thalassemia.
Yes, I'm looking forward for Andy's reaction too.
Regards,
Jos.-
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Hi Jos
The necessity of MRI does not related to the level of HB but rather related to the elevated serum ferritin.
Having a high ferritn may indicate having iron overloaded in organs such as liver, heart, pancreas,.. so an MRI is important to know if these organ have iron in them or not.
One very important note, is that in intermeidas (which is supposed to be the case according to your mutation, but it is not according to your HB level :huh :huh) serum ferritin is not a reliable measure to indicate iron overload in organ.
The level of ferritin could be the same in two patients (one is major and the other is intermedias),but when you do an MRI, you can find that there is an iron overload in the organs of the intermedia patient.
So to su up, serum ferritin is not a reliable indication for iron overload in organs in intermedia mutations, that is why an MRI is essential for you.
T2* is the name of the scan made on the heart to show if there is iron deposits in heart or not. It is exactly like an MRI on the heart
manal
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Jos,
Do you have access to your blood reports? I would very much like to see results of CBC (complete blood count) and hemoglobin electrophoresis. Also, have iron studies been done? Serum ferritin can be raised for various reasons and it does not always indicate iron overload. Iron studies would clarify this.
With an Hb over 13, it is unlikely that you have a serious iron load unless somehow you actually do have hemochromatosis.
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Serum ferritin can be raised for various reasons and it does not always indicate iron overload
Andy,
Do you mean that it could be a reaction from the body to a another chronic disease or inflamation?? If so, is it a possibility that the ferritin will cross the 1000s levels???
manal
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Hi Andy and others,
Here are as attachment my blood reports of April 10 and March 2, 2009. They printed the unit of measurement after each examined item. I hope this and the used terms are understandable to you. I also included the report of February 9, 2009, the first extensive report I've got.
On January 26, 2009 there was a less extensive examination. The result then were:
Hb 10 mmol/l
Hct 47.7 %
MCV 74
MCH 1.6
FE 26
TYBC 58
TF% 45
Ferritin 1255
Cholesterol 6.9
HDL 0.9
LDL 4.1
Triglyceriden 4.25
Glucose 10
ASAT 50
ALAT 66
TSH 1.58
Kreatin 83
To round it up: on March 3, 2009 I started taking drugs against diabetes and cholesterolemia, on March 24, 2009 I visited a dietician, but immediately after the very first blood results I started eating a look more consciously, kicked eating candy and so on. Lost already a bit more than 10 kilogramme of weight.
Thanks in advance for having a look at the blood reports.
Thanks manal for your explanation about MRI en T2*.
Greetings,
Jos.-
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Jos,
I see nothing in your reports that indicates any need for chelation. Yes, your serum ferritin is high but this does not mean you have iron overload and your other iron results are all within normal range. Determining iron deficiency or iron overload has to be done using several factors and using the ferritin test alone is a mistake. If anything, your results are indicative of diabetes and you are already addressing this.
http://www.med.umich.edu/1libr/aha/aha_ironstud_crs.htm
Results of these iron studies tests may mean the following:
* A low serum iron level and low serum ferritin level may be caused by iron deficiency anemia.
* A high TIBC and low serum iron level may be caused by iron deficiency anemia, pregnancy, and chronic blood loss.
* A high serum iron level may be caused by too much iron in your diet, vitamin B6 therapy, or some anemias caused by an inability to use iron.
* A high ferritin level and a normal serum iron level might indicate liver disease from infection or alcoholism, chronic inflammatory disease (such as arthritis or asthma), hypothyroidism, and type 2 diabetes.
* A high ferritin level combined with a high serum iron level may be a sign of hemosiderosis (an accumulation of iron in some of your tissues).
* A low TIBC and high serum iron may be a sign of sideroblastic anemia (a condition that prevents your red blood cells from using iron).
* A high serum ferritin level, high serum iron, and low TIBC may be caused by hemochromatosis.
I think you should be taking IP6 daily as this will help control the ferritin and studies have also suggested that IP6 (inositol hexophosphate or phytic acid) may play a role in controlling HbA1C levels, which will help moderate the diabetes.
I do wish I understood some of the reports better, especially the notes, but I cannot see why you would be told to take Exjade. I see no sign at all that you have iron overload and it is far more likely that your high ferritin is related to diabetes. Your hemoglobin level is fairly normal so there is almost no possibility that you would be absorbing excess iron from your diet unless you also have hemochromatosis, and that would be reflected in high serum iron also, which you do not have.
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Hi Andy,
Thank you very much for taking the time examining my blood reports. It's great you do this.
A few reactions:
1. I started taking IP6 on Wednesday April 29, so that's a good start. Thanks for the advice last week.
2. As I get it from the quoted text from the University of Michigan my diabetes type 2 could be the cause of the high serum ferritin. My internist told me that the high serum ferritin was caused by the HbE-Beta-thal. Besides that he told me that the distorted liver values - AST (SGOT) or ASAT used in the Netherlands and ALT (SGPT) or ALAT the expression used in my country - were most probably caused by the drug I take for the cholesterolemia.
3. Regarding the notes written on the reports. On the report of February 9 my wife wrote the normal range for all checked items and besides that the meaning of MCV and the type of indicator GGT is. So nothing very special I think.
On the reports of March 2 and April 10 I wrote down the results of February 2 for AST and ALT and for cholesterol (below). In the upper part I wrote behind 'HbA2 4,1 %' what my internist said about that value. He said that this indicates that there is too much alpha-globulin, so I'm having a problem with beta-globulin -> Beta-thal.
4. Are there any other items you want to know regarding the blood reports? I would be happy to tell you.
5. All in all I'm glad I didn't start taking Exjade yet. On May 11 there will be the the start of the next blood examination. I'll get the results on Friday May 15. I'm afraid my internist - a new one - will not be very happy with me not taking the Exjade yet, but after all it's my body and my life.
Once again, thanks a lot Andy!
Jos.-
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Jos,
Ik zal je wel een berichtje sturen een dezer dagen. Ik heb nog lang niet alles gelezen, maar heb je ook gehoord van (primaire) hemochromatose?
Groetjes, Dore
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Again me,
At 1 September I started my fourth try of Exjade and I succeed.
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Good for you Door! I'm happy this drugs works fine for you.
Jos.-
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Hi Jos,
The HbA2 is almost always elevated in thal minor and is not considered to be any problem, as it does add to the total hemoglobin available. The determination of iron overload has to be made through the iron studies. The other info is not really relevant to measuring iron load. If a doctor demands that you chelate, I would suggest you demand an MRI of your liver to establish that there is any iron load, before chelating. I do not think this is necessary, because your serum iron is not high at all, even though the serum ferritin is high. All the iron readings have to be considered together to determine iron load. A visit to a hematologist would probably be more enlightening than what you will learn from most internists. Frankly, most doctors are not familiar with blood disorders and are also not familiar with what treatment, if any is indicated. Also, without and history of chronic anemia, there is no reason to believe your body would be absorbing high amounts of iron.
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On August 20, a long long time away, I'm going to have my first appointment at a so called Sickle Cell Center with all kinds of experts; in my case with a hematologist-internist.
Thanks a lot Andy for your explanation. It surely will be of use on May 15.
Jos.-
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I haven't time to answer your message. I should be very busy with study at the moment, but i am now here...
Dutch:
Je moet mmol/l x 1.6 (of 1.8) vermenigvuldigen en dan krijg je gl/dl. Desondanks blijft het lastig om steeds terug te rekenen. Vermenigvuldigen of delen met 2 is natuurlijk iets makkelijker :)
Aha, Sickle Cell Center in Rotterdam. You really should contact the organisation for Sickle Cell and Research, because they are great. I am a member too (this is a Dutch organisation!) and at the moment they are busy with the websites. Hmm, deze werkt misschien nog wel http://www.thalassemie.nl/index.htm (http://www.thalassemie.nl/index.htm) Je kunt ze bellen, maar ik je anders ook wel een emailadres geven.
Succes, Dore
P.S. I still haven't read all message, though I must warn you: not all the tests are aviable in the Netherlands. This makes me feel sad too, but more angry. But I have heard that MRI (it has a name, help me) will be ready this summer in the Netherlands. Though I am not sure, because OSCAR hasn't heard anything of it....
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Hi Dore,
Thanks for your reaction. I'm a member of OSCAR too, only just recently. Have a problem paying digitally my membership fee. :huh
You know the Sickle Cell Center of Erasmus MC in Rotterdam? Have you been there? Do I get it right that for example a MRI is not possible yet? What kind of other tests are not available yet in The Netherlands?
Well, don't let me distract you any longer from your study duties. Give it a try, good luck with it!
Cheers,
Jos.-
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How went your consult by the Doc?
I hope you aren't too dissapointed. I am sometimes, because all the things they talk about here. Just read that someone said the usually yearly MRI scan.... I don't think so!! I am pretty lucky to say as Dutchy that I had my last in jan 2002. Organized by my doc in that time by a private research institute... No joke! :p Desalniettemin: Have a nice weekend!!
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Hi Dore,
How went your consult by the Doc?
Thanks for asking! My experiences last Friday with my new doctor are here to cut it across http://www.thalassemiapatientsandfriends.com/index.php?topic=2855.new;topicseen#new. I wasn't disappointed at all as you can see.
But let's wait for my visit to the Sickle Cell Center next August before getting overoptimistic. My new doc is acquainted with, did research and wrote articles together with Anita Rijneveld, the hematologist-internist I'm going to meet then. Sounds good to start with.
Your last MRI in January 2002? Unbelievable. Is the management of blood diseases in The Netherlands that far behind compared to foreign countries?
The same goes for you: have a nice weekend!
Jos.-
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It is. So far as I know I am the only one who had a MRI. Btw the ferriscan is located in Hamburg. European law (can) give us the permission to go to a foreign country to have test done who aren't available in country of origin.
You got the " europese overkoepelende patientenorganisaties' . I will ask my father; i can't remember where my notes are from the 'zeldzame ziekten DAG'.
I should have write this in Dutch!!!
edit at monday 18 May
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As I said: unbelievable.
Thanks beforehand for the information about the Éuropese overkoepelende patiëntenorganisaties'.
Jos.-
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Hi Alex
:welcome2 , i hope you'll find this forum more useful , keep visiting the forum and keep posting n stay in-touch with us
best rgerds
Umair
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Thanks for sharing infor here
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Hi Alex nice to meet you.I hope u will find new friends here.
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Hello Jos :hugfriend
Im Mariam from Kuwait
Im glad You found this group and joined Us you have no idea HOW HAPPY I AM TO SEE ALL THALS HERE :biggrin
well about exjade: I tried it for a year but one of the side effects for me was vometing (sorry to mension that)
I will post some pictures for you in this group of exjade and its Kit
for me it didnt work out because it made me too sick... plus there are major side effects like liver damage and if u ask me id say id rather have more iron than a damaged liver in the future...
Im using desferal now and my iron level is good thank god
there is also a tablet called ferriprox some thals in kuwait combine it with desferal
but the problem with ferriprox is that it decrease your white blood cell counts so u have to be carefull every thing has side effects for sure id say stic with desferal so far so good and safe :wink
WISH U THE BEST,
MARIAM
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Thanks Mariam for your reply/information.
Jos.-
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You are most welcome Jos :biggrin