Thalassemia Patients and Friends
Discussion Forums => Thalassemia Major => Topic started by: Sharmin on September 19, 2008, 10:37:44 PM
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My dear friends, thank god and thanks to your prayers
my son's hg 1 week post tx today was 132 :stars :stars :stars :stars :stars :stars :stars :stars :stars
We could barely get his hg to 132 immediately after a transfusion before - as the antibodies would begin acting up during the tx.
I hope he is able to go 4 weeks between transfusions again! He had an IVIG today and things went well.
Thank you all for being patient with me I have been such a frantik mess this past year :gaah :gaah :gaah :gaah :gaah :gaah
I hope this progress continues, the ferritin keeps going down and the hg stays up! :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray :pray
The doctors from Oakland called us to let us know that they are very happy with the way things are going. Thank god for them!! And thank god that our wonderful doctor is so cooperative.
I owe this progress to Andy and all of my thalpal friends may you all be blessed with joy & good health,
:kiss :kiss :kiss :love :love :love :love :love :love
Love,
Sharmin
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Sharmin,
I'm so glad to read the good news ,I'm happy that our little friend is getting better.I'm also happy for you and your family.
Its good to read that good things is happening.
:hugfriend
Kathy
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Sharmin dear,
I am soooooooooooooooo very happy for you and our brave little soldier.May god bless him with all the happiness and good health.
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Hi Sis :
Its great to hear that the little soldier is back to fight again ....
May God bless him with good healthy wealthy and life full of life & joy....
after a long night of suffering , its a new dawn ... soo live this moment completely ..
may god bless you with more good news ... BEST OF LUCK :goodluck
BEST REGARDS
TAKE CARE
UMAIR
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Hi everyone,
Thank god once again - and thanks to your blessings and prayers my son's hg after 3 weeks is 118!! :stars
It looks like things are good! If the hg is high again next week we may be able to go 5 weeks between transfusions as we did before the antibody issue. This also means that his iron levels will drop further!
Thank you again for your support during our crisis last year! I wish everyone and their families that best of health and happiness!
Love Sharmin :kiss :kiss :grouphug :grouphug :grouphug
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In addition Andy, it seems that my son won't need a tx for another two weeks. The labs indicate that the antibodies are nearly diminished. His pred dose is being decreased to 5mg alternate days - soon to be taken away completely.
I am very glad for this because it will give his body a chance to get rid of the iron. The blood bank is also working on getting him better matched to prevent problems in the future. Andy, my fear remains the risk of antibodies returning once the effects of the rituximab wears off. Is there anything else we can do to prevent this?
Thanks,
Sharmin
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Hi Sharmin ,
:congrats :congrats :congrats
:congrats congrats to you and your little soldier :congrats
:congrats :congrats :congrats
It's nice to hear that Sis .......
I am realy happy for you and your little Soldier ....
Best Regards
Take Care
Umair
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Thats very good news sharmin. :D
Andy, my fear remains the risk of antibodies returning once the effects of the rituximab wears off.
Praying this doesn't happen anytime soon, coz from what i read, the effect can take as long as 6 months to 3 years to wear off.. Praying for the latter. :hugfriend
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Can I ask a question?
These antibodies your talking about. Does he have a reaction when he has a blood transfusion?
If so, well sorry to say that they will be with him all his life... Correct me someone if I am wrong!!!
I have had some major reactions due to blood transfusions and that my body didnt agree with...
I have to have my blood washed and even filtered now so I dont get anther bad reaction too.. Oh I also have hydrocortisone...
Thats how bad my reactions were...
Hope I was helpful, but somone correct me if I am wrong..
Thank you
Take care all..
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Hi Smurfette,
The antibodies you are talking about are called allo antibodies - the body produces them in response to markers on foreign antigens.
In my son's case, as can often occur with other frequently transfused patients, he formed several of these allo antibodies. His body then created an auto antibody which indiscriminately destroys all red blood cells. Although my son has never had a transfusion reaction - he experienced increased hemolysis leading to an increased transfusion requirement. This lead to some iron overload.
Initially, he was treated with prednisone - but prednisone was not effective enough in slowing the hemolysis. He was then treated with Rituximab which shuts down the production of antibodies - including the antibodies that were causing my son to have hemolysis. The process was very very difficult, and we suffered a lot during this therapy. Thankfully, as a result his transfusions are lasting 3 times as long and the antibodies can no longer be detected in his body. He seems to be very healthy now.
At sometime in the future normal antibody production will resume in his body, and our hope is that the trouble making antibodies will not return. Once shut down, these antibodies need not return, he can have complete remission if we are fortunate. One way of preventing this (as per our hematologist and most prominent hemotologists such as Dr. Vichinsky) is by better matching his blood through genotype matching - so that the auto antibodies will not be triggered again.
Therefore, my hope is that the antibodies will not be with him all of his life. I was wondering what else we can do to keep the antibodies from becoming active again.
As for your allo antibodies - the first line of treatment is always hydrocortisone and carefully cross matching - or the use of washed red blood cells. If the problem is very severe then rituximab can be considered. I would also recommend that you look into genotyping - because in some cases multiple allo antibodies can lead to the development of auto antibodies.
Good luck,
Sharmin
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Sharmin,
Genotyping of the blood is the main thing that can be done to prevent recurrence. It is still only poorly understood why autoimmune disorders occur and preventing and treating various autoimmune conditions is the object of much research. Dr Vichinsky has been heavily involved in this research as it concerns autoantibody reactions in thals. He should be able to give you some insight into the direction this is going. Ultimately, there is a hope that genotyping will join phenotyping for blood matching and incidence of autoantibody problems will be greatly reduced. I think you can probably take some hope in how well your son has reacted to the treatment. And of course, with fewer transfusions it does reduce the odds of a reaction just by lowering the amount of blood received and also does allow more time for matched blood to be found.
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A good news indeed :biggrin Give a tight hug to your little soldier from me. :hugfriend
Zaini.
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Thank you my friends :hugfriend :hugfriend
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Hi little solider
ur such a brave fighter and your curage in taking all this pain really motivate me in fighting this disease. :hugfriend :grin i'm ready to kick out this agony and pain forever in my life and cutiepie would always be thankfull to this forum for support it has given us :bat
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:hugfriend Hope everything's is fine from now on....... Godbless and good health for you and your little angel! :hugfriend
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Thank you all so much :hugfriend :hugfriend
It feels so good to be able to breath and relax, things are good right now. Your wishes mean so much.
Wishing you all many many reasons to smile and be happy.
Love,
Sharmin
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Hey guys,
His hemoglobin after 5 weeks is 90 today. He is transfused on Friday, I hope that this continues to be the case - he used to be transfused avery 4 - 5 weeks when he was younger.
I hope that our case helps others in our situation seek the right treatment and get good results. I have had numerous PMs and questions regarding autoimmune hemolytic anemia and people asking how the treatment is working for my son - if I have missed anyone - please note that things seem to be working and I think it is definitely worth trying. Best of luck,
Sharmin
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Sharmin,
Great news :hugfriend sometimes i wonder why intervals between my daughter's transfusions has decreased,she is regularly goi ng on three weeks intervals,last time her hb was 10.2 so doc gave us the appointment for 4 weeks,but let me tell you that this is the last week and she looks pale and gets tired easily,so i am guesseing her hb must be low,
As you know i have tried so many things to increase her hb,to atleast get back to the normal routine of four weeks,because if intervals keep decreasing i fear where we would end up.I wonder what more i can do ???.
Zaini.
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Zaini, did you check if there are any antibodies running in her blood???
manal
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Hi Zaini,
It is common for children to require more transfusions while they are active and growing, and most likely that is why she is needing blood more frequently. Some of the thal majors I know here, who are healthy adults now, also say that their tx requirements increased for periods of time while they were growing. A three week interval at her age is not too much. How much blood does she get per kilo of her body weight?
It is good idea to make sure that she is getting fresh and very well matched blood - for this if it is at all possible you can try to get genotype testing done. Sometimes children can develop minor antibodies, which don't cause major issues but are aggravating enough to cause an increase in tx frequency. The genotype testing would reduce the likelihood of her developing too many of these antibodies.
It would be a good idea to have the blood bank do a thorough investigation of her blood to see if any antibodies are present - if they are any antibodies they can be avoided, and they should be avoided to make sure that auto antibodies are not triggered. But remember, that what happened to my son is quite rare - and even if it occurs it is treatable as it has been for my son. If the first ritux doesn't work - they often repeat it and the second course of treatment often resolves the problem. But most likely, it is nothing like this.
By the way, if her tx requirements have increased, make sure that her chelation is adjusted accordingly. We fell behind on this a little and it didn't show up in the ferritin but it did on the SQUID, and I know you don't have SQUID available in Pakistan.
Take care and keep us updated,
Sharmin
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Hi Manal,
No we never checked for anti bodies,her doctor doesn't think that her transfusion regimen has something wrong,so she never suggested it.I'll ask the ward doctor this time.
Sharmin,
About the blood,sometimes it's fresh,and sometimes it's a week or 15 days old,not more then that,and i don't know if genotype testing is available here and please to my dumb brain explain in easy words what exactly is genotype testing?
When ever i ask any doctor about decreasing transfusion intervals,either her ward doctor or her hemo,they say that as she grows she'll need more blood,right now she gets 450 ml every three weeks approx,which comes in between 15 to 20 ml per kg,as her weight is 26 kg.
Zaini.
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Zaini,
The blood volume you describe is not very much for a growing and active child, 15 to 20cc/kg every 3 to 4 weeks is what is expected for a child that age. Because you are keeping good records, you must have noticed the change in her tx frequency, her pretx hg and her general appearance before a transfusion. For this reason some parents elect to transfuse every 3 weeks during their children's growth spurts. My only concern would be to ensure that her chelation dosages are increased, not only according to her growth but also according to the increased of blood volume(cc/kg) that she is receiving. It is always better to stay on ahead of the iron, it takes iron far less time to accumulate then it takes to remove it.
I am not sure if phenotype or genotype matching is available in Pakistan. Even in North America there are very few labs that do it, the routine is to do the blood test and then to have it sent to a lab in another city. Is it possible for your doctor to send it to the US? My son has had blood samples sent to the US and even to UK from here. Even the phenotype testing for a chronically transfused child requires testing using multimillion dollar equipment which seperates donor cells from host cells - and then finds markers on the host cells. I don't know if this equipment is available in Pakistan either. Again, perhaps it would be a good idea to have the blood test sent abroad. Maybe Andy knows more about this.
Because your daughter is being transfused in Pakistan, she is less likely than my son to develop antibodies. My son is of East Indian decent receiving blood from a pool of predominantly European decent. Although people can develop antibodies even when the donor pool is less foreign, it is less likely to happen.
When my son was needing more transfusions, our doctor was quite particular about only giving him very fresh blood because it is likely to last longer in his body.
I hope that helps,
XOXO - I think Zainab is doing great - and growing like she should :hugfriend
Sharmin
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Thanks Sharmin,
Chelator's are adjusted according to the body weigh if i am right? she is taking desferal thrice a week,1 gram per night,and ferriprox thrice daily,which makes 1500 mg daily,and she weighs 26 kg,is that dose enough?
Zaini.
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Hi Zaini,
I am not familiar with L1, so maybe Andy can help us out with this one. I doubt that her iron levels have climbed yet, but it is just something to watch out for in the future. Our doctor was increasing my son's desferal according to his weight, but he forgot to factor in the increase in blood volume when he was being transfused more. During the two years he was being transfused 20cc/kg every 2 to 3 weeks, but his desferal was only marginally increased (as per his weight over the two years). I drove the doctors crazy asking about the ferritin each time we had a transfusion, but they would just chuckle and say there is nothing to worry about because the ferritin is below 2000. It wasn't until we went to Oakland that we realized that the doctors were not compensating for the increase in blood volume my son was getting and the SQUID test revealed iron overload.
I am learning about Exjade doses, but I'm clueless when it comes to L1,
Sharmin
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HI Zaini,
The standard dosage for Ferriprox tablets is 75 mg/kg per day, taken in 3 doses total. So, at 26 kg, the dose would be 75 x 26=1950 mg daily. Depending on what her ferritin is, it may be time to bump the Ferriprox dose up to 2000 mg daily. Talk this over with your doctor before changing the dose of Ferriprox. I would also like to point out that Apo Pharma has released a Ferrirpox oral solution that has had significantly less gastric side effects than with the tabs. I will find out if this is available in Pakistan yet. Again, based on ferritin levels, an alternative would be to either add one more night of desferal each week, or extend the length of time for each desferal session.
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Andy,
She is already taking desferal for 11 hours,her ferritin will be tested today ,i think then i'll determine what can we change in her dose,i hope it's lower then 1000,so may be we'll be able to start Asunra and get rid of desferal,last time her ferritin was 1232 ng/dl.
Zaini.
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Zaini,
Please do post the results of her ferritin, Andy is right about the L1 dosage and your daughter is on combination therapy, I only concern the frequency of blood transfusion. Three weeks is not what I expect for a 6-7 year old. I don't know if its the quantity or quality of the blood but its frequent. Our hospital, I hardly see a child on a three week frequency, I think there are only few, if any, that are on three week frequency. The average is four weeks.
Good luck with the results.
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Hi C.F,
Thanks for the concern,i am anxiously waiting for her ferritin results,i hope and wish it's below 1000 so that we can stop desferal and start Asunra.Frequency of her transfusions has worried me also,but i think carao is helping her a bit,this time her pre transfusion hb is 9.9 after 4 weeks,while before starting carao her hb was hovering between 9.4 and 9.6 with three weeks intervals,i don't wanna jump the gun and i don't wanna jinx it ,but i think carao is working for her,may be another transfusion or two will help us to be sure.
Zaini.