Thalassemia Patients and Friends
Discussion Forums => Living with Thalassemia => Topic started by: Sharmin on March 13, 2016, 12:10:57 AM
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As many of you know, my son has thalassemia major which he has dealt with very well. Because a phenotype was not done before his transfusions - he was given blood that wasn't well matched for many years. This lead to production of various antibodies. For this reason I urge everyone - before allowing a child to have the first transfusion have at the very least phenotype testing done - and whenever possible do geneotype testing to avoid this issue.
During the first 4 or 5 years of his life he produced several antibodies, and eventually this lead to him developing autoimmune hemolytic anemia - an antibody that destroys all red blood cells indiscriminately. This lead to several years of prednisone, multiple transfusions and even iron overload for a short while. Every 6 to 10 months he is given medication to control his antibodies. It takes several weeks for the medication to become effective.
When the antibody is active, he will require multiple transfusions each week - sometimes twice in one day. His body hemolysis very quickly during these times leaving him exhausted and pale. Recently he has grown quite a bit, the transfusion volume he is getting isn't enough for him - yet he is not big enough for 3 bags at one time. This week he dropped very fast, I warned the hospital but they wanted to wait until next week to transfuse. Yesterday we found out that his hemoglobin is very low and he needs an immediate transfusion. Because of all of his antibodies he is very very difficult to match and we have been waiting since yesterday for a match. He is becoming very anemic, he is pale and is having terrible headaches from the anemia. At this time, I would appreciate any prayers for him. He is struggling and as his mother I cannot stand to watch this. I am praying for a cure for these damned antibodies and for him to get blood very soon.
It has been an ongoing battle with me and the nurses to listen to me when I feel this is about to happen...but they fail me every time.
We are in need of your prayers... :pray
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Assalamualaikum Wa Rahmatullah.
I'm also a father several time facing different kind of problem. But my personal opinion says that-due to we live outside of our country an atomic things also heated our heart about our child.& there is nobody to share it us Except ALLAH SUBHANAHU TA'AlA. i'm not a reguler member of this side. Every year I try to send my family member to my own country & only that time I got a chance to share something with this side. However, last month I went Bangladesh, i meet a sister who has the same antibody problem, Now a days she takes blood with some of fixed donner & also used lucocyte filter.
I think you live in Canada. I request you whenever you will go for transfussion that time try to an investigation about that RBC Bag. From where & who was the donner of that blood bag. If this oppourtunity you get, after a few months may be it is become easyer to you for collecting the blood. But it is very difficult in there. Please try one time & you also asked the doctor about lucocyte filter. Is it helpfull for antibody when tranfused the RBC?
Pray for my child Tanzim & all Thall Patient.
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Sharmin,
I am sad to hear about this latest development. Your son has been doing so well and looks so healthy. It's difficult dealing with staff that know less about your son than you and refuse to do what needs to be done. The arrogance of authority permeates our society.
Praying for A.
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Thank you Tanzim. He has always had a filter. Unfortunately not all antigens can be filtered. I appreciate your prayers and wish the best for your child too.
Thank you so much Andy. They finally got one unit this morning. It's not enough as he's quite low but its better than nothing and may alleviate his horrible headaches. I warned them that he's difficult to match and we shouldn't let him drop do much that it becomes urgent. On Friday after telling me how low his hemoglobin was the nurse refused to contact his doctor. I had to plead with her to finally have our Dr paged. He immediately started the search. I've been begging for a transfusion since last Monday as I saw this coming. I'm glad he's getting blood now - but the fight with staff becomes as difficult to deal with as the disorder.
At some time almost all parents will find themselves struggling with authority. I have had calls from mothers who had to stand their ground because the particular hospitals weren't following the recommendations. These battles can really break parents down. It's horrible to say the least. But we have to stay strong for our kids and continue to do what is best. I hope they find more blood soon.
Thank you
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Hi Sharmin,
I am sorry to hear about your son`s situation. Over years you have taken excellent care of him. This situation was unduly created and could have been avoided. The secrecy of hospital admin is an issue in Canada, we are very litigious society and hospitals fears for lawsuits, hence they cover themselves.
I know you will stand tall and deal with the situation like you have demonstrated over the last so many years.
Well wishes and prayer for your son.
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The solution to this question is Thalidomide.
The first case report published in journal was of a girl having severe antibodies problem.
Thalidomide is having good success over this issue.
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Very sorry to hear about your son Sharmin...thoughts and prayers...think positive...
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Thank you kindly JV. Thank you also Dharmesh.
Andy have you heard of thalidomide and its use in AIHA?
Many thanks to you all for your prayers. I'm hoping for more blood today so that his hemoglobin will be at an acceptable level
Thank you again for your thoughts and prayers,
Sharmin
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Thank you for all of your prayers. Two more units were found and lil is getting his blood. Already looking and feeling stronger. It's possible that the drop was related to a slight cold and him growing quickly. Hopefully he'll do well after this transfusion.
Once again thank you all for your prayers. Hoping you are all doing well.
Sharmin
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Sharmin,
I haven't seen much about the use of thalidomide for AIHA, but did find that there was a recent study at NIH. I do not believe results have been published. We do have several members of this group currently taking thalidomide as part of Dr Ramana's therapy to raise Hb. I have not heard of any serious side effects here or on Facebook. If the opportunity arises, it may be worth trying. Dr Ramanan will say it won't do enough for the Hb of beta zero patient to try and free him from transfusion with the drug, but since it may have use in AIHA, I think it would be worth a trial if offered.
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Dear Sharmin, nothing is difficult for the Creator of your son, of us and all our children. I pray that He makes the difficult easy for Little A and for every member here. Aameen.
Please don't be worried about 'making noise' when it comes to your child. Better still for him to be the one to tell them how it is. With all my heart, I get how you feel.
Here is a saying of Prophet Muhammad, peace and blessings be upon him, if you don't mind me sharing;
''When you visit a sick person, (minor sickness or a major sickness), then tell him to make dua (supplications) for you because his duas are accepted readily like the duas of the Angels.''
Our children are extremely precious and they carry Golden Prayers on their lips and in their hearts...readily accepted.
Prayers...and the voice...powerful tools...they will just have to listen.
Little A knows his body best. They can't argue with that.
I pray for the best of health and a very long and happy life for A.
:heartred
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Sharmin good to know that the hospital has managed to get more blood units & hope your son is feeling better now. Will pray he always stays in best of health.
On the topic of Phenotype and Genotype testing of blood I am a bit confused and I am not sure I understand this fully. I did try asking the hospital staff if they do the phenotype testing of the blood long back and they did assure me that cross match that they do after taking the blood from the donor is nothing but phenotype match to see if the blood would be compliant for my son. I have a fixed list of donors from whom we take blood donation for my son and till date the hospital has not rejected any donor we got. After 9 transfusions I have also not seen any abnormal things with his health after transfusion . Is there anything more I should enquire with the hospital on this topic or is there any test that would tell me my son has developed any antibodies.
As of today my son gets B+ (His blood group) , Cross matched , Prestorage leucodepleted , Irradiated RBC for his transfusion.
Any guidance on this topic would be of great help for me to understand this process better.
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Thank you Andy, I will discuss it with our doctor.
Thank you for your wishes Mukta and Emby. You are both correct, it is very important to be the advocates for our children's health. I wish you all the best as well.
Little A is back to annoying his sister and he has resumed his intense fitness regimine. He is a difficult person to match, the hospital seems to realize why it is important to transfuse him before he drops so low next time.
Best,
Sharmin
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Seeing your posts now Sharni...I am so glad that all is well now and Little A is back to being himself.
We all have learnt to deal with the transfusions, chelations but sometimes the hospitals, nurses and doctors make it hard for us even though we know what is best for our children.
All the best to Little A and to us parents who will never stop fighting for what is right for our children :smiley
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Sharmin ,
Hope your son is quite well now. May almighty God bless him.
Before reading your post I thought that it is the problem in developing countries where clinical condition is not so good and there is not also enough support from government level.
But I can't believe though you are from a modern country then you are also facing difficulties with hospitals.
In our district all the hospitals supply whole human blood to thals. Only one hospital produce pack cell by limited amount. People are also unaware about it.
I have faced this type of problems one time when my Hb was very low but after repeated transfusion there is also no further increase of Hb.
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Sorry Sharmin ...
May Allah recover your son early...
Always my prayer to your son and your family.... :hugfriend
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Thank you Bostonian, Abyaya Jyoty and Lokkhi maa - I wish the very best for you as well.
Little A's levels are good now - and we thank you for your prayers.
My issue right now is that the hospital nurses complained to our doctor about us calling to ask for his hemoglobin. The doctor forbid us from asking for his hemoglobin anymore. This is very sad and difficult because if the doctor leaves without checking - which he often does - we have no way of knowing what his level is. He is very difficult to match, it sometimes takes 3 days to find a match. If his levels are low we now have no way to know that a cross match is required. When we are concerned that he has suddenly gotten low we often send him in for a blood test. If the staff finds it convenient they will book him a transfusion but usually they are short staffed and they only book him in when it is convenient for them.
Right now I am in a state of panic because often his hemoglobin can drop very low with no warning. The nurses do not feel obligated to tell the doctor - and until he checks we will have no way of knowing. I am beyond frustrated with this archaic and selfish system. Where I don't have access to or decision making power over my son's health care. Basically I have been informed that the doctor will decide if and when he will receive a transfusion and if his hemoglobin drops to 5 every time then that's too bad.
Please excuse me for my rant but I am at a loss here :(
Canadian Family, have you encountered this in Eastern Canada?
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If possible buy a hemoglobin meter.
Knowing weakness of US healthcare system is making me stressed.
There are some traditional methods to check hb level by checking eyes, nails and tongue but you need to develop expertise to figure out it exactly. It also can be co-related with physical symptoms
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Sometimes, whatever is agreed between the main consultant and the patient in term of treatment, at times it is not noted on their records so this does not get fed back to the nurses on the ward where our children receive their treatment. Nurses work according to what is written for them to work by. Full treatment plan set out by the consultant needs to be regularly checked and updated as patient needs may not always be the same. This will help greatly towards better and personalised care with treatment...ideally.
It's very difficult too when all thal patients are boxed in 'one box' and the treatment plan is one fits all.
May God grant ease.
:heartred
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Dear Sharmin,
I just read your message.
Have you taken this higher....above your consultant. The manager, chairman....?
This is a concern when it affects quality of health.
Are they cutting costs...trying to save money? At what costs...Human health..more visits to hospital...more costs..
It doesnt make sense why they wouldn't be giving Little A the proper treatment for him.
Your rant is asolutely excused and i feel your hurt.
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Canadian Family, have you encountered this in Eastern Canada?
Hi Sharmin,
Sorry I did not got back to you sooner. I am still recovering with a bad case of shingles. Its not fun and pain is unbearable sometimes.
With respect to your question. I must say we have had no particular issues at sickkids in Toronto. However, we have to be political about getting the information. We are informed about the hg every single visit. The nurses are usually under lot of stress and we sometimes leave them alone or simply ask, everything alright. In your case, I would simply ask why are we forbidden to ask about Little A hg. To me, this is bullying, yes parent can be little anxious sometimes, but isn`t it these nurses are trained to handle things. I pray for Little A and hope you would have a chance to have a nice chat with doctor. Please keep us posted.
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Hi Canadian_Family,
I'm glad that things are better in Toronto. Quite honestly, things were great here for many years - but the emergence of this antibody has caused problems for us and perhaps the hospital staff doesn't understand why we need to be aware of his numbers all of the time. The doctors in Oakland have given me instructions on what to watch for - they are very open about information and have advised me to make sure that he receives a transfusion at certain levels and to adjust chelation depending on his levels. Nurses her are under the impression that I don't need to know anything - and if the doctor notices anything that should be done he will do it himself.
At present little A's ferritin levels have remained above 1,500 for almost 1.5 years because he requires additional transfusion due to the antibodies. I spoke with the doctors at Oakland, and they have advised the addition of desferal for a few months to decrease his iron levels quickly. We are getting set up to do the combination therapy until his ferritin levels are below 1000 - and then we will chelate a little more aggressively until his ferritin levels are below 400. He can have lower ferritin now because he will be 18 later this year.
I hope you are feeling much better, give our best to little Ms. A.
Sharmin
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Dear Mukta,
My apology, in all that was going on I overlooked your question:
Sharmin good to know that the hospital has managed to get more blood units & hope your son is feeling better now. Will pray he always stays in best of health.
On the topic of Phenotype and Genotype testing of blood I am a bit confused and I am not sure I understand this fully. I did try asking the hospital staff if they do the phenotype testing of the blood long back and they did assure me that cross match that they do after taking the blood from the donor is nothing but phenotype match to see if the blood would be compliant for my son. I have a fixed list of donors from whom we take blood donation for my son and till date the hospital has not rejected any donor we got. After 9 transfusions I have also not seen any abnormal things with his health after transfusion . Is there anything more I should enquire with the hospital on this topic or is there any test that would tell me my son has developed any antibodies.
As of today my son gets B+ (His blood group) , Cross matched , Prestorage leucodepleted , Irradiated RBC for his transfusion.
Any guidance on this topic would be of great help for me to understand this process better
Phenotype testing can only be obtained before transfusions are started. This looks at characteristics of your child's own blood (other than the ABO type or rh type) to ensure that blood that he or she receives is as compatible as possible. Giving mismatched blood over time can cause the body to form antibodies which could result in reactions or hemolysis. The gold standard of testing is genotyping - which ensures a much greater analysis of the blood and ensures well matched blood for your child.
For my son we were not able to obtain a sample for phenotyping before transfusions were started - and when we attempted it at 8 months of age - he had already been transfused several times, and a clear sample could not be obtained. He received mismatched blood for many years. At age 10 he finally had genotyping done. Since then he has not produced any more allo antibodies.
I hope this answer is helpful to you. Thank you again for your wishes for lil A. Sending good wishes your way as well.
Sharmin
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Sharmin,
sorry to hear about what you have been through .... may God bless your son with a healthy joyful and succesful long life... ameen
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Dear Sharmin,
I am so sorry to hear about your recent problems. I really hope there comes a breakthrough and a fresh view very soon. And I cannot bellieve little A is about to turn 18!!! What happens with the time? Will his current doctor transfer him soon to the adult ward? I know you know this but be sure to make with them a good treatment plan so things work out fine in this new department.
I am shocked to hear his hb dropped to 5 gl/dl. No wonder you were scared and you needed to rant.
Sending good wishes!
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Hi ,
I'm little confused over phenotype & genotype matching :huh.
Attaching a lab report . Earlier only anti- E &anti-K antibodies were present, recently developed anti-c antibody. What are the chances of developing more antibodies ? and how do we protect ourselves from developing more and more antibodies (should we test phenotype before each transfusion ?)
Age =25 and thal major . Testing for genotype will do any help now (already transfused for so many years) ?
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If genotype matching was done before the first transfusion, antibody reactions can be reduced about 90%. Unfortunately, it is not generally done (Shame on the thalassemia medical establishment for not pushing this the way it should be pushed). Phenotype matching should be done before each transfusion, if at all possible.