Update and questions

My son's summer has been busy with the rituximab, ivig and transfusions.  His rituximab was completed on aug 7th.  He received a single unit tx  (approx 300cc) on aug 8 (pre tx hg 91).  His pretx hg today was 82.  I know that antibodies have a half life of several weeks - maybe a couple months - but is this drop too much at this stage of his treatment Andy? 

He had an ivig today and is having a tx tomorrow, he was in 3 days last week.  Frankly, he just wants to play as his summer holidays are coming to an end.  I am so worried about him getting depressed if we have to have so many frequent visits.  Does anyone have any ideas on how to prevent this?  I am emotionally exhausted myself, but I am doing everything I can for my son. 

Also, there are different opinions on whether or not to continue the ivig treatments - what do you think Andy? 

Thank you so much everyone for your support,

Sharmin

Hi Sharmin,

In a study of young patients using rituximab which followed 15 patients ( http://bloodjournal.hematologylibrary.org/cgi/reprint/101/10/3857 ) the response time to rituximab was quite varied, ranging from as little as 5 days to a high of 72 days. If there is a recurrence, the rituximab is often repeated. Treatment with IVIG also has a greatly varied response and is directly related to how long the autoantibody problem has existed in the patient. The longer the problem has existed prior to commencing treatment, the longer before the effect of IVIG is seen.

It's too early to determine the effects of the treatment, and because of the long interval between the appearance of the autoantibody and the beginning of treatment with IVIG, it may have to be continued for awhile to see if it will have an effect. Your doctor should have some time frame in mind for determining what is working and also some possible adjustments to the therapy if no results are seen.

Your doctors should be able to give you some idea about how long this may take to see results. Hopefully this next transfusion will keep his Hb up a little longer. Both traveling and the summer heat can lower Hb so this may be something that should be factored into his current Hb level. I'm happy to hear he wants to spend his time playing.

Hi Andy,

Our doctor has told us that with the Rituximab - although new antibody production should be suppressed - the old antibodies can survive for up to 5 months. Blood tests show that antibodies are still present, but both Oakland and our doctor had told us that they would be around for a while.   Of course during this time they are expected to decrease in numbers and cause less trouble.  It is after this time that full effects of the rituximab are expected to be seen.  We hope that his transfusions begin lasting longer and longer....

The ivig in our case is being used to compensate for the loss of immunity resulting from rituximab - to prevent infections.  Different clinics have different opinions regarding the need for the ivig because they are not sure whether it is necessary and whether the immuity is actually suppressed that much with rituximab.     A second benefit ivig can have is to decrease hemolysis by the antibodies that remain.  We hope this effect will help him until the antibodies dissappear.  I am sure that the travelling and summer heat have contributed to his decrease in hg.  We are still on a low does of prednisone for a little while 10/mg alternate days.  We hope we can get off of this soon. 

His ferritin is 2138 - we hope that this number begins to decrease soon.  Although not a perfect measure of liver iron, it will give us a general idea of how things are working.  We have been on desferal/exjade combination therapy for 3 weeks now.

He is wanting to play a lot more.  I think he is dissappointed with how much of his summer has been spent in the hospital this year. 

Our doctor did put things in perspective for us though - while I was telling him that the reaction with the ivig was 'the sickest our son has ever been' - he smiled at us and said ' yes, i've known him all of his life and that IS the sickest he has ever been - in fact i don't ever remember him being sick before that at all" .  I guess I should be thankful that for a child with a serious disorder he has never really been very sick before.  It is also a reminder that with proper management a person with thalassemia can be very healthy. 

I hope that this treatment works the way we hope for it to.  I will keep you posted with our numbers in the future - hg, ferr etc. 

Thanks again Andy:) 

Sharmin 

You are in my prayers

manal

Be strong and positive Sharmin. We know you are doing the best anyone can do.

It's good to hear that he is active even with all the medical treatment around him.

I hope he gets completely well soon.

Sharmin,

We hope your son gets well soon, you have come a long way and things have started to look better. We certainly hope the old antibodies will suppress with each coming day. Best of luck.

 

Andy, Manal, Sajid and Canadian Family - your support means the world 

Sharmin

Hi everyone,

It has now been 2 weeks post tx for my son.  At one week tx his hg was 127.  He looks well at this time, his color is quite pink.  I will probably test him next week to see if he needs a transfusion.  This is the longest he has gone without a transfusion in over 2 years (on such a low dose of prednisone).  I am hopeful that the antibodies are diminishing. 

He began school this week and hockey and his other activities begin in a few days.  He seems very energetic and enthusiastic, even at the end of the day.  I hope this means that the treatment this summer has worked!  My hope is that he will eventually be on a once a month tx schedule again very soon (even once every 3 weeks would be good).  I will update you with his hg and ferritin levels next week. 

Anyone else concerned about antibodies, I highly encourage you to speak with your medical caregivers about genotype matching, rituximab and IVIG.  I hope it continues to work well for my son and I hope that others who have antibodies will have their iron levels checked.  The iron accumulates very quickly!

Please feel free to ask me questions,

Sharmin

Andy,

How soon would it be okay for me to begin giving him supplements such as IP6 again?  He has not had any supplements (other than vitamin C) all summer. 

Thanks,

Sharmin

That's a great news

I'm so happy that he is going well without transfusions!

It is very satisfying news and we hope it continues to get better.

Thanks God he is doing fine,

Last few months were for sure a rollar coaster ride for you,what with all these treatments ,now just lay back and relax  .

Zaini.

Hi Sharmin,

You should probably check with Vichinsky's office about resuming the supplements but I think that there would be no problem starting up again, especially IP6. The only one that they might be cautious about would be vitamin E because of its blood thinning properties but with the therapy over for now, this probably is no longer an issue.

Thank you my dear friends - your support has kept me sane during the past few months.  I am cautiously optimistic at this point - because some antibodies may be around for a while - but I hope they are diminishing to very low levels.  

Canadian Family - how are your daughter's hg levels holding up?  I am sure the drop was due to the procedure the hospital was using to store the blood.  Please keep us posted.  You would be surprised what a difference such things make.  We were told that the reason for the harsh reaction our son had to the ivig was due to the particular brand of ivig they used.  Apparently all kids reacted to that brand - and my little one was given 4 times the amount to prepare him for his treatment.  YIKES!     I pray that your daughter's hg holds up.  

I love you all and pray for all of you:)  

Sharmin
 

Thanks Andy,

I think I will begin IP6 soon.  I should be in touch with Oakland next week for his Exjade follow up so I can discuss his other supplements then.  I put him back on vitamin C last week. 

During our first visit Dr. Vichinsky emphasized using zinc.  I will ask them if it is okay for us to begin that now.  I hope everyone else here is taking zinc.  I think I mentioned that he was suggesting that all thals take centrum silver - or some equivalent. 

Thanks again Andy,

Sharmin