Some good news!!!

Thank you all so much 

It feels so good to be able to breath and relax, things are good right now.  Your wishes mean so much.

Wishing you all many many reasons to smile and be happy. 

Love,

Sharmin 

Hey guys,

His hemoglobin after 5 weeks is 90 today.  He is transfused on Friday, I hope that this continues to be the case - he used to be transfused avery 4 - 5 weeks when he was younger. 

I hope that our case helps others in our situation seek the right treatment and get good results.  I have had numerous PMs and questions regarding autoimmune hemolytic anemia and people asking how the treatment is working for my son - if I have missed anyone - please note that things seem to be working and I think it is definitely worth trying.  Best of luck,

Sharmin

Sharmin,

Great news  sometimes i wonder why intervals between my daughter's transfusions has decreased,she is regularly goi ng on three weeks intervals,last time her hb was 10.2 so doc gave us the appointment for 4 weeks,but let me tell you that this is the last week and she looks pale and gets tired easily,so i am guesseing her hb must be low,
As you know i have tried so many things to increase her hb,to atleast get back to the normal routine of four weeks,because if intervals keep decreasing i fear where we would end up.I wonder what more i can do  .

Zaini.

Zaini, did you check if there are any antibodies running in her blood???

manal

Hi Zaini,

It is common for children to require more transfusions while they are active and growing, and most likely that is why she is needing blood more frequently.  Some of the thal majors I know here, who are healthy adults now, also say that their tx requirements increased for periods of time while they were growing.  A three week interval at her age is not too much.  How much blood does she get per kilo of her body weight?

It is good idea to make sure that she is getting fresh and very well matched blood - for this if it is at all possible you can try to get genotype testing done.  Sometimes children can develop minor antibodies, which don't cause major issues but are aggravating enough to cause an increase in tx frequency.  The genotype testing would reduce the likelihood of her developing too many of these antibodies. 

It would be a good idea to have the blood bank do a thorough investigation of her blood to see if any antibodies are present - if they are any antibodies they can be avoided, and they should be avoided to make sure that auto antibodies are not triggered.  But remember, that what happened to my son is quite rare - and even if it occurs it is treatable as it has been for my son.  If the first ritux doesn't work - they often repeat it and the second course of treatment often resolves the problem.  But most likely, it is nothing like this. 

By the way, if her tx requirements have increased, make sure that her chelation is adjusted accordingly.  We fell behind on this a little and it didn't show up in the ferritin but it did on the SQUID, and I know you don't have SQUID available in Pakistan. 

Take care and keep us updated,

Sharmin

Hi Manal,

No we never checked for anti bodies,her doctor doesn't think that her transfusion regimen has something wrong,so she never suggested it.I'll ask the ward doctor this time.

Sharmin,

About the blood,sometimes it's fresh,and sometimes it's a week or 15 days old,not more then that,and i don't know if genotype testing is available here and please to my dumb brain explain in easy words what exactly is genotype testing?

When ever i ask any doctor about decreasing transfusion intervals,either her ward doctor or her hemo,they say that as she grows she'll need more blood,right now she gets 450 ml every three weeks approx,which comes in between 15 to 20 ml per kg,as her weight is 26 kg.

Zaini.

Zaini,

The blood volume you describe is not very much for a growing and active child, 15 to 20cc/kg every 3 to 4 weeks is what is expected for a child that age.  Because you are keeping good records, you must have noticed the change in her tx frequency, her pretx hg and her general appearance before a transfusion.  For this reason some parents elect to transfuse every 3 weeks during their children's growth spurts.  My only concern would be to ensure that her chelation dosages are increased, not only according to her growth but also according to the increased of blood volume(cc/kg) that she is receiving.  It is always better to stay on ahead of the iron, it takes iron far less time to accumulate then it takes to remove it. 

I am not sure if phenotype or genotype matching is available in Pakistan.  Even in North America there are very few labs that do it, the routine is to do the blood test and then to have it sent to a lab in another city.  Is it possible for your doctor to send it to the US?  My son has had blood samples sent to the US and even to UK from here.  Even the phenotype testing for a chronically transfused child requires testing using multimillion dollar equipment which seperates donor cells from host cells - and then finds markers on the host cells.  I don't know if this equipment is available in Pakistan either.  Again, perhaps it would be a good idea to have the blood test sent abroad.  Maybe Andy knows more about this. 

Because your daughter is being transfused in Pakistan, she is less likely than my son to develop antibodies.  My son is of East Indian decent receiving blood from a pool of predominantly European decent.  Although people can develop antibodies even when the donor pool is less foreign, it is less likely to happen.     

When my son was needing more transfusions, our doctor was quite particular about only giving him very fresh blood because it is likely to last longer in his body.

I hope that helps,

XOXO - I think Zainab is doing great - and growing like she should 

Sharmin

Thanks Sharmin,

Chelator's are adjusted according to the body weigh if i am right? she is taking desferal thrice a week,1 gram per night,and ferriprox thrice daily,which makes 1500 mg daily,and she weighs 26 kg,is that dose enough?

Zaini.

Hi Zaini,

I am not familiar with L1, so maybe Andy can help us out with this one.  I doubt that her iron levels have climbed yet, but it is just something to watch out for in the future.  Our doctor was increasing my son's desferal according to his weight, but he forgot to factor in the increase in blood volume when he was being transfused more.  During the two years he was being transfused 20cc/kg every 2 to 3 weeks, but his desferal was only marginally increased (as per his weight over the two years).   I drove the doctors crazy asking about the ferritin each time we had a transfusion, but they would just chuckle and say there is nothing to worry about because the ferritin is below 2000.  It wasn't until we went to Oakland that we realized that the doctors were not compensating for the increase in blood volume my son was getting and the SQUID test revealed iron overload. 

I am learning about Exjade doses, but I'm clueless when it comes to L1, 

Sharmin

HI Zaini,

The standard dosage for Ferriprox tablets is 75 mg/kg per day, taken in 3 doses total. So, at 26 kg, the dose would be 75 x 26=1950 mg daily. Depending on what her ferritin is, it may be time to bump the Ferriprox dose up to 2000 mg daily. Talk this over with your doctor before changing the dose of Ferriprox.  I would also like to point out that Apo Pharma has released a Ferrirpox oral solution that has had significantly less gastric side effects than with the tabs. I will find out if this is available in Pakistan yet. Again, based on ferritin levels, an alternative would be to either add one more night of desferal each week, or extend the length of time for each desferal session. 

Andy,

She is already taking desferal for 11 hours,her ferritin will be tested today ,i think then i'll determine what can we change in her dose,i hope it's lower then 1000,so may be we'll be able to start Asunra and get rid of desferal,last time her ferritin was 1232 ng/dl.

Zaini.

Zaini,

Please do post the results of her ferritin, Andy is right about the L1 dosage and your daughter is on combination therapy, I only concern the frequency of blood transfusion. Three weeks is not what I expect for a 6-7 year old. I don't know if its the quantity or quality of the blood but its frequent. Our hospital, I hardly see a child on a three week frequency, I think there are only few, if any, that are on three week frequency. The average is four weeks.

Good luck with the results.

Hi C.F,

Thanks for the concern,i am anxiously waiting for her ferritin results,i hope and wish it's below 1000 so that we can stop desferal and start Asunra.Frequency of her transfusions has worried me also,but i think carao is helping her a bit,this time her pre transfusion hb is 9.9 after 4 weeks,while before starting carao her hb was hovering between 9.4 and 9.6 with three weeks intervals,i don't wanna jump the gun and i don't wanna jinx it ,but i think carao is working for her,may be another transfusion or two will help us to be sure.

Zaini.