Slow rate transfusion and warm antibody

Thank you so much Andy, 
his hg today was 101.  His prednisone dose has been cut back to 25mg for a few days - and then will be reduced further.  Our pediatrician suggested that we discuss ritux once we get to Oakland.  It would be great to have a break from all of the steroids and frequent transfusions.  His ferr right now is 1800 - if his tx requirement is reduced we can bring it back down. 

I understand that some patients had a complete recovery after two cycles of rituxmab.  With my son also being at the age he is (almost 10) the ritux along with changes in his body over the next few years may take care of this problem.  Thank you for the information Andy - I'll bring back as much more information as I can - I know a few of our members can use it. 

Andy, would it be helpful if we provide information on the website for newly diagnosed patients to ensure that they have phenotype matching or genotype matching so that they don't end up in our situation? Should other parents have this done for their children even if they have been transfusing for a while? We simply were not aware when we began transfusing our son.  Hopefully other parents can avoid this complication. 

I'll let you all know how our transfusion goes tomorrow - thanks again everyone  - and thank you dear Sajid for your constant support and good wishes:)  Your not just an awesome pastry chef - you're just plain awesome:) 

Sharmin

Hi Sharmin,

That's a good level all things considered.

I do think in general we should be pushing for genotype matching of blood along with phenotype matching. It seems practical enough to do and would save patients many problems. Again, I would like to know Vichinsky's take on this. I feel I am getting closer to making a decision about Singapore soon (my worker's uncertain state of health has put me in a holding pattern but hopefully he will have some info later this week)  and if I go, I will definitely try to corner Vichinsky again for a few minutes. He is always in such a hurry that he's hard to pin down for long. I am really glad you are seeing him soon.

Singapore is such a long distance from here and quite a costly place, but the outpouring of support I have seen here makes me want to try very hard to get there. I hope I am soon in position to make a decision.

Sharmin, i am so happy for the good news, wish you all the best
manal

Thanks Andy - I'm getting pretty eager to talk to the folks in Oakland soon. 
I hope I can offer some positive info and hope for everyone here. 

I am eager to reduce the dose of pred soon here too - it's time to begin deflating his ballooning cheeks!  He looks like he's storing something in them cheeks. 
Seriously, when I see the effect on his face - it makes me wonder if he is having other side effects too. 

Thank you so much Manal - I hope things continue to get better.  I hope that the new treatment works!  I hope all of our little children have bright & healthy futures!

My heart goes out for our little soldier,Sharmin,what did doc say about swelling on his face? I'll pray that everything goes back to normal,wish you the best.

ZAINI.

Hi everyone,
the transfusion went well - the doctor thinks we can do the ritux when we return - he is hopeful that it will be a good thing and we can get off of the prednisone if he administers the new treatment - of course he is consulting with Oakland. 

Zaini,  thank you so much for your prayers.  The swelling is a temporary side effect of the prednisone - he has had it twice before when he is on a high dose of pred and it usually goes down quickly once the dose is reduced.  It's just hard looking at his face looking so different.  Chances are that over the next few weeks he will return to normal. 

Thank you everyone - I'll keep you all posted as we prepare for our trip over the next week. 

love,
Sharmin

The little bug goes in for a blood test tomorrow and tx on Thursday. 

Andy, he was transfused last Tuesday and this Monday (6 days later) his hg was 104 - the tx he received on Tuesday was small - 10 cc/kg - as he has been getting for a while now.  He is slowly being tapered off of the pred he is now on 25mg of pred alternate days and this dose will be given 7 times before it is reduced to 15mg alternate days and then 10mg alternate days.  And if Dr. Vichinsky agrees we will try Rituximab on our return so we can lay off of the pred.

I am wondering if it would be best for him to receive a bigger tx, the normal 20cc/kg on Thursday because we will be gone for 10 days and his pred dose is being reduced.   

Since he will be in California for a while in the heat running around, my fear is that his hg may drop too quickly - I hope that giving him more blood and the 25mg alternate day dose will hold him for the 10 days until his next tx.  I guess worst case they could tx him in Oakland. The problem is that our appointment in Oakland is not till the end of our trip.  What is your opinion Andy?  Should we go for a bigger tx?  I am a little scared going away for such a long time. 

Thanks again Andy,
and thank you everyone else for your prayers:)

Love,
Sharmin

Hi Sharmin,

It is quite common for thals to take an extra bag of blood before leaving on a holiday. I would say definitely, yes, do load him up so you he can enjoy the vacation.

Thanks Andy,

His hg this morning was 103.  His doctor has decided to give him the two bags.  My hope was to split it up between two days so that we aren't giving him a lot at once and getting the antibody all frazzled and excited.  I hope that the pred will keep things in check.    Transfusion tomorrow and Disneyland the next day    I don't know who is more excited me or the kids!! 

I am excited about our trip and very excited about meeting Dr. Vichinsky.  I hope I can bring back info that will help all of our thal patients and friends. 

Sharmin

Hi Sharmin

I am sure that everything will be just fine. Please do enjoy the trip to the fulliest, you really need it Wishing you and the family all the best.

manal

P.s : Sharmin , don't forget the photos

Thanks Manal my friend,

We look forward to this vacation - and hopefully some relaxation.  I will take lots of pictures - you know my obsession with pictures   

We'll be thinking of all of you   

Good to hear that

Enjoy your trip.

Looking forward to see great snaps

Hi all,

the transfusion went well - we got a little extra. 
Thank god - and thanks to all of your prayers - all of the tests that were done for my son - including blood tests, bone age, bone density, ecg, echocardiogram came back completely normal.  His vitamin D was slightly low but the doctor will supplement him for that.  I am very very thankful for these results.  Other than the thal and the antibody he is a very healthy boy.  The doctor was not surprised, he told me that he did not expect anything to be wrong as he sees a very healthy boy before him.   I hope we'll find a way to alleviate the antibody issue in Oakland. 

We're off to Disneyland in the morning!  Miss you all very much!!!!!  I wish you all loads of happiness and good health!!! Thank you for everything!!!   

Love,
Sharmin