Thalassemia Patients and Friends
Discussion Forums => Thalassemia Major => Topic started by: SIL on March 13, 2009, 10:49:12 AM
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Hi everyone! My brother-in-law(bil) has Thalassemia and has to go in for regular transfusions. His HB is barely 8 and it drops to as low as 4.5-6. We, his family are pained to see him suffer so. He is only 24 yrs old and is cheated out of a "normal" life. He hardly ever goes out and has few friends who are all working abroad.
He is almost always working in their family owned laboratory, where he helps out. Even the doctors in our city are not very aware of Thalassemia and everytime he falls sick, we are at a loss as to what his treatment should be. We dont even know what his diet should be! I learnt about iron chelation only on this site. What kind of medicine should he take?
He has undergone a spleenectomy in the hopes that his hb levels will not fall, but to no avail!
He normally refuses to take medicines and only takes folic acid tabs irregularly. It seems to me as though he is counting his days.
I'm soooooo glad I found this site. Glad and elated to see so many wonderful people fighting it out and willing to share their experiences and hopes. I adore u all for it! :hugfriend
What are we to do? :huh
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HI SIL
Welcome aboard. You will find valuable information here. Do you mean your brother in law gets regular transfusion but does not get chelated?
If this is so then you should have him go for a complete blood test, more specifically ferritin and start chelating accordingly.
Where are you from? The doctors have never deemed it necessary to have him chelate?
You should read the different posts where you will find a wealth of information.
I believe first and foremost you should have him go for the ferritin test and if the values are too high(which I believe will be very high if he was regularly transfused and had never been chelated) start chelating immediately.
After that you can look at the supplements to be provided.
Take care
Jade
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Hi SIL
:welcome2
Umair
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Hi! Welcome!!!!!!! :welcome I believe you have come to the right place! I really hope learn more about thalassemia here......
As Jade said chelation is very important, your brother in law should discuss this with his hematologist.Having the disorder doesn't mean we can't have normal life, we just have to be strong and brave in facing it, to handle it really well. Compliance is important, along with patience, one could suceed in making his/ her life "normal". But yes many doctors are still unaware with this disorder and mistakenly as just plain and simple anemia and prescribes the very poison to our condition which is Iron, and that makes it worst.I do hope you keep us updated. :hugfriend
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Dear Sil,
Welcome to the board. You are doing a great thing getting information for your brother in law. I have a few questions for you which may will make it easier for us to guide you.
Has your brother in law been transfusing all of his life? Does he have antibodies which cause his hemoglobin to remain so low? Ideally he should receive enough blood so that his hemoglobin is above 90, 4 weeks after a transfusion. At each transfusion he should be getting 14 - 16 cc of blood for every kg of his body weight. Also if you can tell us what country you live in we can recommend a thalassemia center for your brother in law.
There are several chelators available. Desferal, exjade and L1 - are any of these available where you are? Please keep posting and we will do our best to guide you.
Welcome to our board and wishing you the best,
Sharmin
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:welcome
I have the some questions as the previous posters. :)
Take care, Dore
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Hi Jade, Umair, Lyanne, Sharmin and Dore!
First, tanx a lot 4 ur replies. I'm overwhelmed! :hugfriend
I'm from Coimbatore in South India. I have asked my brother-in-law to check out this site and he is now a member-Ray. Hope u guys will guide him correctly. He is of the notion that chelation drugs have to be taken only when the levels go too high.
He refuses to take any drugs, which I feel is foolhardy as these will help him lead a better life. I will ask him to post his stats so that u all can advice him.
I heave a sigh of relief knowing that I have led him to the right place, into ur safe and knowing hands. God bless u all!
:biggrin
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Hello Sil,
Thats great to know that he has joined the forum,if he has been transfusing for years,and regularly ,his ferritin must be high,how high ? only he can tell us.
Ray,
I hope you would post soon :) .
Zaini.
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Sil,
You have done a great thing. Ray we look forward to your posts.
Sharmin
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hi to everyone here,
thank you for responding to my sister in law's questions, I am Ray the one she was talking about. I was diagnosed as Thallasemia intermedia at the age of 1 1/2 yrs and have been receiving transfusions every six months when I was a child and subsequently once a year as i grew up, my haemoglobin so far has been between 5.5 to 8.0 and whenever I fall ill due to fever or any infection it reduces and thats when I go for a transfusion. since my spleen was removed my hb level has been around 6.0 to 6.5 for most of the time and when it drops we transfuse with 2 units.
The haematologist suggested a ferritin test in 2007 which was 1400 at that time and more recently I had a transfusion of 2 units of blood in october 2008 which I have every year but it reduced because of fever and dropped to below 5.2 so we transfused in february 2009 before the transfusion the ferrittin level was 2109,bak in 2007 i was prescribed an iron chelating drug to be taken regularly with the doctor mentioning there would be symptoms of vomiting and some pain but the vomiting was too much and I was becoming weaker and more sick so I stopped taking it, I read in a medical website that ferrittin levels in excess of 4000 only cause problems, correct me if I am wrong. at present i take folic acid to maintain my hb levels. I appreciate your replies and welcome your suggestions. I am confident about myself and try to enjoy whatever I do but my loved ones are concerned about my situation, I hope you can encourage SIL to feel relaxed about this issue. thank you and God bless you all.
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Hello Ray,
I am truly glad you posted,welcome on the forum.
Which chelator did your doctor prescribe? There are variety of chelators available.
I don't know where you read it but its not true that only ferritin of above 4000 can cause problems,ferritin above normal levels is harmful,and if its persistent it will start loading in your organs which in turn will damage your organs,i am not trying to scare you,but you need to start chelation right away,my daughter transfuses every four weeks but her ferritin level is lower then yours,and we have so many thal majors here who transfuse regularly ,some have managed their iron levels very nicely,others are fighting to bring them down.
When you'll start chelating regularly and your ferritin will be lower,i think it will also help your hb levels to stay more stable,as free iron in the blood does damage red cells,and for this purpose all thals should be taking antioxidants such as Vitamin E ( natural ),IP6,green tea etc.
One more thing i'd like to suggest is Carao,You can read about it in these threads.
http://www.thalassemiapatientsandfriends.com/index.php?topic=1259.0
http://www.thalassemiapatientsandfriends.com/index.php?topic=1791.0
You should avoid taking high iron food in your diet as when hb is constantly low,body starts absorbing more and more iron from food,so that will just add to your iron overload.Once you start chelation and stay compliant there is no reason you can't bring your ferritin levels down.
Good luck with chelating and feel free to ask if you have any questions in mind.:)
Zaini.
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Hi Ray ,
:welcome2 , Its nice to see you posting , stay in-touch with us and keep posting ....
Best Regards
Take Care
Umair
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Ray,
You need to be chelating regularly. Ferritin levels above 1000 will cause damage over time. In addition, it has been found that ferritin tests are not necessarily accurate in intermedias who do not transfuse regularly. Intermedias can have secondary iron overload caused by absorption of iron from food, and this iron overload often does not show in ferritin tests, so it would be best if you could get a liver MRI to assess the iron load. There are two oral chelation meds Exjade (Asunra, desirox, Osveral) and Ferriprox (kelfer, deferiprone, L1) and the injected chelator, desferal. You need to get a proper assessment of your iron load and then chelate accordingly.
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Welcome Ray.
You should avoid taking high iron food in your diet as when hb is constantly low,body starts absorbing more and more iron from food,so that will just add to your iron overload.
To add to the above comment, I would say it would be a good idea to drink tea with meals to decrease Iron absorption
Check this NIH trial - http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1727318
Background—Black tea is known to be a potent inhibitor of intestinal absorption of non-haem iron at least in healthy subjects.
Aims—To investigate this effect in patients with genetic haemochromatosis, and, more importantly, the effect of regular tea drinking on the accumulation of storage iron in these patients over one year.
Patients—Investigations were carried out on 18 patients with clinically proven genetic haemochromatosis. For the study of storage iron accumulation, they were separated into a group instructed to drink a particularly tannin rich tea regularly with meals and a control group.
Methods—Intestinal iron absorption from a test meal was measured using whole body counting. Body iron stores were evaluated quantitatively by exhaustive phlebotomy, using haemoglobin, saturation of serum iron binding capacity, and serum ferritin for the assessment of body iron status.
Results—A significant reduction in iron absorption was observed when the test meal was accompanied by drinks of tea instead of water. In the tea drinking group, the increase in storage iron was reduced by about one third compared with that of the control group.
Conclusions—Regular tea drinking with meals reduces the frequency of phlebotomies required in the management of patients with haemochromatosis.
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Hello Ray
I would like to stress so much on Andy's point about the inaccuracy of ferritin readings in intermedias. Thsi was thoroughly discussed in the last thalassemia conference.
Also the role of supplements can not be ignored as it can contribute to the the overall health in addition to increasing the life span of the red blood cells. Please read the section of supplemetation carefully and good luck
manal
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Hi Ray,
Welcome aboard! I just joined the group last weekend myself. I'm so glad I did, and I can assure you that you'll also benefit from the great friends on this forum!
Your hemoglobin levels seem to run low. I'd suggest you get a thorough evaluation of both your blood transfusion regimen and iron overload by a hematologist, and yes, you definitely need to get on a chelation therapy. If you didn't like one chelator, try another one (under an approval and direction of your hematologist). It's no reason to give up.
Take care,
SF
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Thanks Zaini, Andy, Nice friend, Narendra, Manal, Sf,
thanks for the replies and suggestions,
I think the name of the chelating drug which I used previously was desferoximine,
the haematologist has suggested another one I am not sure but will find out.
I do drink a lot of tea. not green tea but regular tea with milk so I guess thats helpful too,
thanks a lot again for welcoming me and giving your suggestions, I ll keep posting, God Bless you all.
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Hello Ray,
Desferoximine is generally known as desferal,how were you using it? its administered throught a needle placed under your skin for about 10 to 12 hours.
You can ask your doctor about a new oral chelator called Asunra.
Zaini.
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hi Zaini,
I was given these capsules which had to be taken once in 6 hours orally, the haematologist has suggested a new one i think its desirox, i am not sure but will find out and let you know, have a nice day....God bless you
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Ray,
I think you are talking about Defriprone/L1/Ferriprox/Kelfer.But these are supposed to be taken along with Deferoximine.So if you want oral chelation only ,yes Desirox is much better option,it will take time but i hope it works,Have you started taking it yet ?
Zaini.
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Although deferiprone is an excellent choice for combination chelation with desferal, it can be and is used alone with great success. I would point to Poirot as one who has used only kelfer for many years and has kept his ferritin well under control. Any of the three available chelators can be used by themselves. With Ray's ferritin level hovering just over 2000, any one chelator would be sufficient, unless a liver scan showed a high iron load. Kelfer alone, would probably be sufficient.
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With ferritin around 100, I used only ferriprox for one year and then proceed to an MRI.
It showed heart and liver iron within normal range, no iron at all. However liver - due to my taking only ferriprox for the whole year - had accumulated a little iron, still within normal range. Of course I cannot say that this goes for everyone, it might happen only to me.
Lena.
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hi Guys,
sorry that I Did not know the name correctly, I had been taking Kelfer in 2007 and stopped, so if desirox is good I could try that, thanks, God bless you
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Hi evrybody!
I'm so glad Ray has joined the forum and tanx to all of u for guiding him. He is usually the advice-giver in our family, now its good to see him on the receiving end :biggrin :clap!!!!
Hope he finds the right chelation drug. I would also like to know what his diet should be like. Doctors adviced him not to include greens in his diet, is it right? What about chicken(his fav), fish and meat? Any fruits or veggies he should avoid taking?
His urea levels are also high.
:heartpink
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Hello SIL,
Thals should avoid red meat,chicken is fine,green veggies like spinach does contain iron,but iron in red meat is more readily and easily absorbed by the body,while iron in veggies,which is non-heme iron is not that readily absorbed,but for me,if some one is trying to lower their ferritin,they should avoid iron in diet,as in thal intermedia body automatically absorbs more iron from the food.
Zaini.
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SIL ,
:agree with zaini , ...
here is a quote from our expert Mr.Andy's post
Some green vegetables that are high in iron should be eaten only in moderation, but please keep in mind that vegetables contain non-heme iron and it is not as readily absorbed as the heme iron in meats, so as long as you eat meat, I wouldn't worry much about the non-heme iron in veggies. The iron in meats is very highly absorbed so it makes more sense to watch how much and what type of meat you eat, if you want to cut back your dietary intake of iron. Red meat contains the highest quantities of iron.
As confounded as researchers have been about this subject, it is becoming more clear. Iron stores in the body are not the primary source of iron for building red blood cells. Iron from the gut is. Regardless of your high iron stores, your body will look to the gut for the iron it needs to produce RBC's. At some point, the researchers will finally say that the reason hepcidin levels don't increase in iron overloaded people, is because the body is actually trying to absorb even more iron because it is working extra to produce RBC's, so it cuts production of hepcidin. The best way to remedy this is by keeping a higher Hb level, so the bone marrow activity is not increased. It's a vicious cycle and for transfusing patients, keeping the Hb higher is the best way to stop excess iron absorption from food.
Best REgards
Umair