Thalassemia Patients and Friends
Discussion Forums => Thalassemia-related Issues => Topic started by: Andy Battaglia on January 23, 2008, 03:19:41 AM
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I have posted the full text of this just released article under the Documents section at
http://www.thalassemiapatientsandfriends.com/index.php?topic=1451.msg11816#msg11816
I would highly recommend that every patient makes sure that their treatment centers and doctors are made aware of this most important review. The original article can be found at
http://www.blackwell-synergy.com/doi/full/10.1111/j.1600-0609.2007.01018.x?prevSearch=allfield%3A%28heart+in+transfusion+dependent+homozygous%29
I cannot overemphasize the importance of what is contained in this article. The number one killer of thals is heart failure and taking the essential steps to prevent this will greatly extend the lives of thals. The article states that over 80% of thals are now surviving past the age of 40, whereas during the 1960's 80% of patients died by age 16. This is remarkable progress made possible by iron chelators and for thals to continue this amazing improvement, proper monitoring and management is necessary. Being proactive and supplying your care giver with the most current information is an important way that thals can use to make sure they are getting optimal care. And most importantly, each patient must make the decision to comply with treatment and stick to that decision.
Many may find it surprising to see that 80% of thals are now living into their 40's. If this is not true where you live, make sure that every medical professional involved with thal that you know is made aware of this article. Following these guidelines will have a very positive effect on survival rates among thals.
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Hi Andy,
Thanx for such a great article,i haven't read it completely yet,but questions i wanted to ask anyway were,zainab is taking ferriprox three times a day,it makes 1500 mg a day,according to the article the dose of defriprone should be 75-100mg/kg divided in three doses daily, zainab's weight is 26 kg,now if i calculate the lowest dose i.e 75 mg/kg,it makes 1950mg,should i increase her dose to 4 tabs a day?should i ask her doctor first?another thing is she is taking two tabs of folic acid dailu,5mg each,can i increase this dose also and make it 4 tabs daily,the main reason is zainab's hb used to be 10 on three weeks tranfusion duration,but since last few months it's always lower then 10,like in between 9 and 10,my husband is arguing with me that it has started happening since she started taking IP6,i don't think so,but he is insisting on quitting IP6 for a while to check it,but i don't want to,bcoz it has bring some colour on her cheeks back,after so long time i,ve seen her face pink,i don't know what to do,plz help me out.
ZAINI.
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Hi Zaini,
Always ask your doctor before adjusting the dose of any medicine. Chelating drugs require specific monitoring, with L1 it is the white blood cell count. Your doctor always needs to know the exact dose she is taking.
The most likely reason that Zainab's Hb level has dropped between transfusions is that she is growing and requires a higher blood volume. This is something all parents of thals should understand. The transfusion requirements of a child may change as they grow. Bigger bodies need more blood. This would be a gradual change and eventually she may need either more frequent transfusions or more blood per transfusion. It is also possible that it is a temporary change in Hb, as even changes of season have some effect on the measured Hb level.
Phytic acid (IP6) has been studied extensively, because it was feared that people who eat a lot of grains (whole grains are high in IP6) might suffer from anemia because of it. Tests on rats showed that there was no effect on hemoglobin levels and no effect on the normal absorption of iron.
http://jn.nutrition.org/cgi/reprint/90/4/423.pdf
The hemoglobin values in table 2 show
that, even at the submarginal level of 10
ppm of iron, the rate of hemoglobin re
generation was not affected by the pres
ence of either level of dietary phytate.
When expressed on the basis of milligrams
of iron consumed, there were no signifi
cant differences, at either level of iron,
between control values and those of the
diets containing phytate.
It was concluded from the results of this
work that high levels of phytate have no
effect on iron absorption in the rat. There
fore, the presence of phytic acid in foods
fed to rats should not be expected to de
crease the physiological availability of the
iron in such foods.
IP6 had no effect at all on the Hb level. What has been discovered is that IP6 works where it is needed in the body. It is found in every cell in the body and supplementing with IP6 has shown abilities to reduce the damage that iron does to cells, lower blood glucose levels in diabetics and inhibit the effect of iron in cancer cells. IP6 has also been shown to extend the lives of cells with its antioxidant properties.
One thing I have been seeing mentioned a lot is the supplement L-Carnitine, which is a precursor of nitric oxide, and has been shown to extend the life of red blood cells in thalassemics.
http://grande.nal.usda.gov/ibids/index.php?mode2=detail&origin=ibids_references&therow=800548
L-carnitine is an essential element of intermediary metabolism and also was shown to be effective in maintaining normal red blood cell (RBC) function. This study aimed at investigating plasma free L-carnitine concentrations and effectiveness of L-carnitine supplementation in protecting deterioration of RBC properties in beta-thalassemia major patients. Plasma free L-carnitine concentrations were determined in the blood samples obtained before their regular transfusion (about one month after the previous transfusion). Each patient received 100 mg/kg/day oral L-carnitine supplementation. RBC deformability, lipid peroxidation and intracellular free calcium concentrations were investigated before and after this treatment. Plasma free L-carnitine levels and RBC deformability before the treatment were found to be lower whereas lipid peroxidation and intracellular calcium concentration in RBC were higher compared to those of the control subjects before the L-carnitine treatment. After one month supplementation of L-carnitine lipid peroxidation and intracellular calcium concentrations were found to be decreased and RBC deformability was improved, accompanying the significantly increased plasma L-carnitine concentrations. These results suggest that L-carnitine can be used as a supplement in beta-thalassemic patients, to prevent RBC deterioration.
I have seen studies on L-carnitine many times and am surprised that it is not more widely recommended for thals.
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Ask the Experts
Question:
My child needs a bone marrow transplant , but there are no matched donors in the family. What can be done?
Answer:
There are experimental protocols offering transplant from alternative donors, which are higher risk than transplant from an HLA-matched sibling. The family needs to be fully aware that the risks of these alternative transplants include death, chronic graft vs host disease, graft rejection (go through all the procedure and end up still with sickle cell disease), and reproductive sterility. Usually the eligibility criteria are: stroke or silent brain infarct and chronic transfusion, recurrent acute chest syndrome (3 episodes) or recurrent hospitalizations for pain (3/yr) despite hydroxyurea and other interventions. Some protocols are only for children under 17yrs.
Different protocols have alternative donors such as HLA-matched unrelated cord blood, partially HLA-matched family member, or HLA-matched unrelated donor. Different preparative regimens are used to combine immunosuppression and bone marrow suppression. Medical compliance is essential and the family needs to set aside all other obligations to follow the pre-transplant evaluation and the post-transplant period can involve very intense medical care for 6 - 24 months... this is a level of intensity more similar to cancer care than to sickle cell care. The child will have to take multiple medications for several months, central line for medications and nutrition, and may have 3 clinic visits a week and frequent hospitalizations. Psychosocial stability and family support are extremely important -- this will be a challenge for a family from Savannah to get a transplant someplace else. However, if they are interested, the family should ask the child's hematologist about making a referral for consultation at a pediatric bone marrow transplant center Emory, Duke, and Jacksonville are relatively close to your patient in Savannah. The transplanters at St. Jude in Memphis, TN, and National Institutes of Health (Dr. John Tisdale) also have sickle cell transplant protocols and often conduct consultations for patients from far away.
Transplantation is the only sickle cell treatment offering the possibility of complete cure from sickle cell disease. However, there are real risks involved, and the alternative donor protocols have higher risks than the HLA-matched-sibling donor transplants. For a patient and family to take on these risks requires considerable discussion to make sure that they understand the risks and their responsibility for medical compliance.
Sincerely,
-Lewis Hsu, MD, PhD
Pediatric Hematologist
For more frequently asked questions please see: http://www.scinfo.org/faq.htm
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This make interesting readings enjoy it
Kathy
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Hi Kathy!
Thanks for sharing this. :happyyes
There are always alternates besides the recommended treatment, but like the post said the protocols change and so does the risk factors.
It is always very very very hard to decide to opt for a specific treatment, weighing the risks involved against the success.
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Thanx Andy,
For your generous reply,i never thought IP6 can play any role in zainub's low hb,and i guess you are right ,chnage in hb can be due to various factors,i understand this, but still not knowing the exact reason frustrates me,and there has been a lot of variation in her hb level since last few months,it was like
17/08/07 =10.6
13/09/07 =9.8
06/10/07 =9.7
27/10/07 =10.1
24/11/07 =8.6
15/12/07 =9.3
05/01/08 =9.6
26/01/08 =10.5
I want to keep her hb above 10,but i am unable to make a pattern due to the variation in her hb levels.
I will ask her doctor about the dose of ferriprox ,but what about folic acid,do i have to ask him about that also.I really doubt if L-Carnitine supplements are available here,i'll try to find them.
ZAINI.
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These small changes in Hb level are nothing to be alarmed about and I think most patients could show you similar changes over the months. Many factors affect Hb including hydration level, illness, climate and altitude and unless you see some sudden drop, there is usually no cause for concern.
10 mg folic acid daily is a good dose, so I don't think a higher dose is necessary. Give L-carnitine a try (I take it myself as it can also help burn fat if overweight) and see if her Hb level maintains longer.
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ZAINI,
You might be aware of it already that each time the blood donated by a person might have the Hb levels at different levels and might therefore fall lower or might be higher than you expect.
As Andy has mentioned the levels do NOT seem abnormal, it seems such levels are normal. You are a good example of a mother trying to give your daughter a better life and I think that's setting up an example for others too (Especially in Pakistan - may be other around you can get inspiration from you)
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Hi Zaini
Please check this too on L- Carnitine
http://www.thalassemiapatientsandfriends.com/index.php?topic=892.0
Manal
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Thanx Andy,Narendra and Manal,
This site always gives me hope,the thing is, i think i am passing through my bad days now a days ,at least this is what i think,i feel like i am not doing enough for my daughter,i want her ferritin to be controlled,and hb to be maintained,when her hb goes down she is like really cranky and irritated,and i can't see her like that,i want her to be a happy child,and i personally know how it hurts when you have low hb ,bcoz i,ve spent so many years of my life with low hb, all the bodily pains ,feeling down and dizzy,and even i sometimes feel that low hb makes me depressed,I am an adult,but i just feel like she doesn't deserve it,she is going through her childhood,and childhood is supposed to be the days of fun and good memories,and it hurts me real bad when i think that when she'll be older she'll have the memories of her childhood filled with needles,pump and medicines.Although she is very active,always playing,non stop talking :) she is participating in her annual sports at school on saturday,i hope she'll win .
Sorry for all this crap,but i hope i'll come out of this phase pretty soon,just pray for me.
ZAINI.
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hi Zaini.
Tomorrow will be better. you know you are doing the best you can for your daughter ,and she will grow up knowing that she is loved.children are resillient.
Its never as bad as we think .
go on remember our little mate :tantrum :tantrum :tantrum :tantrum :tantrum :tantrum :tantrum it will do you good have a good laugh and not to worry,
:flowers :flowers :flowers :flowers :flowers :flowers :flowers :flowers :flowers for you
Kathy
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Hi Zaini,
Don't get depressed with little setbacks in life. You know that you are a great mom and are doing the best you can.
So roll up your sleeves and give little Zainab some milk and cookies and get her in training mode. We want a Gold medal!
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Zaini, you are a great parent and i am sure that God will reward with everything you wish for :hugfriend :hugfriend :hugfriend :hugfriend :hugfriend :hugfriend :hugfriend :hugfriend
Manal
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Zaini,
I am reading your posts for quiet some time. I truly believe you are doing the best for your child and take great lengths to do more. I see you don't want any stone unturned to get the best for Zainab. She is very lucky to have a mother like you.
First of all, you have to admit that you are doing best for Zainab (we all do).
Second, Its okay to feel little worried when you see the unexpected results, we all do. I think this time you are worrying too much, Zainab numbers are just fine. In my opinion (may be wrong) you are somewhat frustrated because Zainab results didn't show up to your expectations, you are worried if you are doing enough or the doctor is vigilant enough. JUST RELAX, everything is fine, and you know its a temporary phase and you will come out of it.
Hope this helps.
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Hi Zaini,
I was reading thru your posts and as everyone said before me, you are a wonderful mother and very vigilant about zainab. So, you are doing everything right and so, please relax....everything is fine, everything is under control.
Also, I want to mention from the day I read your motto "Nothing can stop u unless u want to be stopped", I say it aloud when I feel depressed and it gives me immense strength to move forward. So, please say this aloud.....and I am sure you will get back your strength.
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Thanx a lot,
Kathy,Sajid,Manal,Canadian Family and Hope
Your kind words made my day,thanx a lot for being there for me,we will be checking zainub's ferritin with her next transfusion, i hope there would be a positive change we'll see.
LOVE ALWAYS,
ZAINI.
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Hi Everyone, :grouphug
We went for Zainab's transfusion today as per schedule,after three weeks,last time when we went for transfusion after the same duration,her hb was 10.5, and today they sent us back :yahoo :yahoo :yahoo her hb was 11.7,can you believe it.I AM SO DAMN HAPPY.i don't know how it happened,but isn't it a great difference? do any of you can think of a reason for that, my husband was like ,noway, there must be some mistake ,but then we reconfirmed at the lab and they said her hb is 11.7, Now dr told me to come next week but iam thinking that i'll get her hb checked before going for transfusion,may be her hb will last till ten for two more weeks,i can only hope and i am praying for it.Moreover i am waiting for her ferritin results,and i hope it will be as good as her hb.
LOVE YOU ALL :grouphug
ZAINI.
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This is wonderful Zaini! I hope you always receive results that make you celebrate.
Rejoice - be happy - we should celebrate every positive news along the way - one less transfusion - less iron!
Love,
Sharmin
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Hi Zaini,
That's a great news! :stars
I get excited too when they postpone my Transfusion. You get the idea how I felt in December when I was sent back twice and that too when there was no blood left to be transfused. That was a miracle that I will never forget.
So, this means that you are getting a good treatment and blood that is lasting long.
Best wishes.
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Good news Zaini.
Maybe zenab will be like me as she grows older she will be able to produce more mature red cells /
Keep fingers cross , god is great. I'm happy for my little friend :bighug :bighug :bighug
from aunty Kathy to Zenab and my best wishes to you. :cheer :cheer
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Alhamdull lah Zaini, i am soooo happy to hear those wonderful news
Keep it up
Manal
:yahoo :yahoo :yahoo :yahoo :yahoo :clap :clap :yahoo :cheer :cheer :yay :yay
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Thanx all of you,
As i said before,it's a pleasure sharing anything with you,i hope it will last atleast for two more weeks :) anyways i got her ferritin result also and it's 1549, last checked it was 1871,so not much declline in numbers, but still at least a lil bit reduced ALHUMDULILLAH.
ZAINI.
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Zaini,
It is very positive news and I believe it shows that the things you are doing are having some success. Keep up the supplements and let us know how she does in future checkups.
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Thanx Andy :hugfriend
ZAINI.
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Hi Zaini
I am really very happy for Zenab. :congrats
With best wishes & lots of Love for Zenab.
Dimple
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Hi Zaini
I did not know Zainab was having problems with her hb :what. Alls well thats ends well. I am really happy to know whatever problem she had, it has passed.Keep up the good work.
with warm regards
MAHA
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Hi Dimple,
Thanx a lot :hugfriend lots of hugs and kisses for Priya.
Hi Maha,
There was not much problem,just a few points variation is her pre transfusion hb,though Andy told me it's nothing of great concern,the thing that made me happy is that she didn't have to transfuse this month,and as you know one less transfusion means less iron,thanx for your concern :hugfriend,I hope hassan is doing well.
ZAINI.
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:cheer :cheer :cheer
Hoorray..I am soo happy for you and dear Zainab...Please share your secrets....whatever you are doing is working.... :yahoo
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Hi, :biggrin :biggrin
There are no secrets dear,i think it was pure luck,or may be our prayers.
ZAINI.
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Hi,
As i last posted on feb 16 about how we were sent back from hospital on the date of transfusion as Zainab's hb was 11.7,it was 15 feb when her hb test was done,and after a few days on 21st feb i went to a physician near my place,he has that compact machine on which he can run some tests and give you the results immediately,i asked him about the accuracy of the results on that machine and he said that other then the points variation which comes in different labs reports,the results of this machine are pretty accurate,i asked him to check Zainab'z hb ,he pinched Zainab's finger with some small device and took a very small amount of blood in a capillarie (spelling??) and placed a drop of blood on a strip and slid it into that machine,the reading came with in a minute and it startled me,her hb was 11.8.
I know i should be happy that her transfusion was delayed for one more week,but that's freaking me out somehow,i am happy and worried at the same time,how's that possible that after 5 or 6 days her hb was same? no dropping? not even a point?
Waiting for your comments.
ZAINI.
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Hi Zaini,
Better have the Hb checked at the usual lab using the usual way as every machine has it's own calibration.
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Hi Zaini,
While Zainab's Hb has remained high, what has been your observation of her health and physical appearance? Does she seem to be at the level she would be when her Hb is that high after a recent transfusion? Sometimes I think your own observations are as important as tests. If she is doing well, it is a very good sign that the things you are doing for her are helping. I don't want to make too much of this yet but it will be very interesting to see how she does in the coming months in terms of Hb and ferritin level.
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I am happy that Zainab's hg is holding up. Is this possible because she is thalassemia intermedia - not major? Is it possible that she is reacting well to the natural fetal hg inducers? Whatever it is, the fewer transfusions she receives the better it is for her. I wish the little sweetheart all the best.
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Hi Sajid,
Yes i will get her hb tested from Aga khan lab tomorrow.
Andy,
Like you said,I don't want to make too much of this either,but my observation is that Zainab's skin is way lighter then usual,towards fair complexion,plus her tummy was not as flat as any normal kid ofcourse,but what i've noticed is that it's going back to normal,and usually in the last week before her transfusion she used to be cranky,crying most of the time,fussing all the time about this and that,but that's not the case right now, she is behaving normaly,and when i went to that physician he told me to pull her lower eyelids down a little and see what colour do they have from inside,he said if they are dark pink or reddish pink it means her hb is normal,and i check them daily an they are dark pink.I hope her test tomorrow will bring good news.On coming saturday 5 weeks wil be competed since her last transfusion.
Sharmin,
Thanx for your wishes, :hugfriend Zainab is not taking any Hb F inducers,she is only on IP6 and folic acid along with multivitamins.Although i have wheat grass super shots but haven't tried it yet.
ZAINI.
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Congratulations Zaini, i am so optimistic about the hb test and i am sure everything will go as you want. Who knows, may be it will last more and more
:congrats :congrats
Manal
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Zaini,
I am soo happy for you and sweet litltle Zainab. May be the IP6 is working well. After I joined this forum and learnt about IP6, I also started giving IP6 to my (now) 19 month old daughter. In the last one month, I also noticed she is looking fairer than before. In fact many of my friends commented that she is looking fair and I can see the pink in her cheeks. Also, I feel her energy level has improved.
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Thanx Manal and Hope :hugfriend
ZAINI.
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Hi Zaini
I am very happy for Zainab. And I hope that her lab test also comes with the same number.
Lots of love and wishes for you and Zainab. :hugfriend
Dimple
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Thanx Dimple,
I'll know the latest reading of her hb in few hours ,just praying for the best. :pray :pray :pray
ZAINI.
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We're praying with you Zaini - it will be good :)
:pray :pray :pray