Little Ari

Hey AUS,
Sharmin is the right person to ask about. Her son and she has been through the same, if I recall that correct.
Stay strong and keep in touch with us. We are going to explain this do you.Where are you from?

Hello all,

Just wanted to update because we were really excited today.  For the first time since transfusions began, Ari's interval for this month is going to be at 5 weeks. We checked today at week 4 and his hg was at 10.7. 

I'm not sure why as he did have a cold earlier this month and was on antibiotics.  They didn't give him more blood than usual at the the last transfusion either. He was at 9 at the time and that transfusion had taken place.

We were thrilled because the last one (where he was at 9) took place within a 13 day interval as he was really sick.    He has been getting  177ml per transfusion. 

Transfusions usually occur every 4 weeks lately but it has always been in the low 9's or high 8's when we have him checked.  There was also a month where he was completely fine - no colds nothing and his hg had gone down to 8.5. 

OF course, that is always discouraging as we would love to prolong the intervals but I started to think that maybe we need to get to a 3 week intervals rather than 4.    I hope he can keep this up for a while.  We will be doing his FerraScan in August and I believe they will start exjade when he turns 2. 
 

Cari,

I am glad that Ari's transfusion lasted longer this time.  It is very common for changes to take place over time - especially in that age group.  I think the vitamin supplements will help his transfusions last longer too.  Ari looks like he's grown quite and bit in the last little while, which can increase transfusion requirement.  Also, when the immune system is more active transfused blood is broken down faster.  You should see him settle down into a routine of roughly 4 weeks soon. 
 

Sharmin

Cari,
Our little SG(Supergirl) has also been going through the same phase....last month it fell to 7.5 and this time it was 8.5.  Well, so it seems that Little Ari and SG being the same age are following a similar pattern, which somewhere is a little comforting, not that we would  want them to go through any of this.

Anyways, so I wanted to share with you what Dr. Vichinsky recommended for SG. We are following his suggestion and are comfortable with it, as it gives us a routine.
He suggested that th hemoglobin be kept  >9.5. AND that THE TRANSFUSION DATES BE FIXED IN ADVANCE. THE AMOUNT OF BLOOD GIVEN AT  EACH TRANSFUSION BE ADJUSTED RATHER THAN THE TRANSFUSION INTERVAL.

Our SG has gone through 15 transfusions so far. First 6 months we were adjusting the transfusion interval, but since then have been adjusting the amount of blood. Today I have dates for the next 4 transfusion schedules, and that just takes away a bit of uncertainity from our lives and helps me plan better.

Hope all this makes sense.

Hugs
SK

I really like this transfusion regimen that Dr Vichinsky is recommending. It makes total sense and will result in a relatively stable Hb level.

Thats interesting,we should see how it works in the long run.

Thank you for sharing

We have done this with little A in the past - increasing his transfusion volume from 15cc/kg to 20cc/kg - this works well in most cases.  In our case eventually, because of the antibody, we came to a limit where giving him more blood volume would put a load on his heart but in most cases it is a great idea. 

How's little Ari doing?

how is Ari doing? It looks like Ari turned into 2. Any plans doing stem cell transplant?

Little ari is doing good, we just had another little one last week and he is adjusting to his new baby brother beautifully! ari is growing up fast and currently transfused every 3 to 4.weeks. We have not discussed treatment for him at this time as we would like the options out to mature   to putting him through any. Things are not always smooth but he is as normal as any child and makes me proud every day. how old is your tdaughter van?

Congratulations!!
My daughter is 18 months older.

Congratiulations with your new son and Ari's little brother. <3

Hi everyone, hope you have had a very happy holiday and that you are all doing well!

I wanted to touch base and see if you could help me with some issues we r having. Ari has been on exjade since september and they informed us in october that his liver enzymes were elevated. They took him off exjade for a month in november and after two weeks of being on it his levels were back up...

 Alkaline phosphate 242

Alaline amino trans..620.

Asperate amino trans. 97

I think the last two are ALT / AST

They have him in exjade until thursday and they will check again. Any one experience something like this where exjade wasn't fitting? I am concerned because the doctor hadn't heard of any other oral chelator.

Yes İ have had this too. as long as it is not like 5x higher you should not worry too much. i would advise an other try out and maybe with a lower dose. one's body need to adjust to it. Also it is recommend to drink a lot of water. whrn that happend to me İ stopped for three weeks. Maybe this helps?