Thalassemia & antibodies and frustration

Sorry Sharmin ...

May Allah recover your son early...

Always my prayer to your son and your family....

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?

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

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.


 

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.

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.

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

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

Sharmin,
sorry to hear about what you have been through .... may God bless your son with a healthy joyful and succesful long life... ameen

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!

Hi ,
I'm little confused over phenotype & genotype matching .
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) ?

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.