Wish us luck !!

Zaini,

You are right to consider combination with desferal, not to mention desferal alone- in this phase.
From my experience, when ferritin does not go down and insists somewhere around 1000, next step is to proceed with something more aggressive- desferal. The only question is Z's age--then again so many young thals have grown up using desferal and of course Little Z will be on it just for a while.
My estimate is that Z's ferritin will not come down at least considerably and if it does it will delay in doing so. That's what I think. Sorry if I disappoint you, but truth must be told and right steps must be taken. I have heard of a case with ferritin 1500 (on ferriprox-exjade combination therapy) and she was removed fron that chelation therapy back to ferriprox-desferal - for a while to manage a drop in ferritin and then again back to exjade....

Zaini,

Do what you feel is best--I only suggest things in this site out of my experience and according to my personal opinion, trying to help to the best of my ability.

Also inform your doctor that ferritin optimal levels have been brought down to 100... Why don't you show him/her info from this site? Will be offended,eh?But doctors must not inhibit our health evolution because of their ignorance, must they?

 
Lena.

I typed a whole bunch and then it was gone. So meet you all in the weekend.
Good luck and happy days!!!

Lena,

You never disappoint me i know you are telling the truth and yes,if that doctor doesn't allow Z to use Asunra and desferal together,i might go back to desferal and ferriprox again,painful it may be but if its necessary,we don't have much choice.

Regarding the doctor,i once tried to show her content from this site but she didn't consider it much,but i have high hopes for this other doctor,i am just waiting for Z to get free from school,she'll get a break for a week or so in february i guess,and thats only because she bites my head off if i make her miss school to go to doctor,so considering that its not an emergency,i am not pushing her.

One more thing why i am a bit hesitant in going back,Z is growing up and now unfortunately she has started arguing and questioning about all the medication or supplements she has to take,she'll literally hate going back on desferal,thats why i was hoping so much that Asunra would work,but if not,don't worry i am not giving up,i'll have to convince her anyway,it just makes me feel bad that i always keep convincing people to take supplements and i didn't know that my own daughter was resenting taking them.Go figure ..

Zaini.

Thanks for your vote of confidence, Zaini.

I would suggest that you persuade little Z that she has to switch back on ferriprox-desferal and that it will be only for a while- just temporary - until she manages to lower her ferritin. The reward will be to continue with exjade. She may accept it this way. I fully understand what you have said about children stating their own opinions and standing up to their feet.

Lena.

Zaini,

I do think that before considering a change of chelators, that you should give the split dose twice daily a chance, before trying other chelators again. She may make a bit more progress because she has a better chance of covering a full 24 hours with chelation. Exjade is not metabolized at the same rate by all patients and for some, a daily dose isn't giving the 24 hour chelation that is intended. Don't forget that one dose of Exjade is supposed to give 24 hour chelation, but this is equal to no more than the chelation provided by 12 hours of desferal. At its best, Exjade has been found to be equivalent to desferal. What this means is that any patient who isn't getting a full 24 hours chelation from Exjade, is also not getting the same chelation they would from daily desferal use. I think this realization has had a big impact in this recent revising of the dosing recommendations that doctors are giving. Hopefully, the result will be less nausea, potentially less effect on liver levels and a true 24/7 chelation regimen.

Little Z is only on a maintenance dose of Asunra so you can't expect much decrease in ferritin, but the slow drop may accelerate a bit if she has full time chelation with the split dose. Another consideration to be made is that since she is an intermedia, she should be taking less annual blood volume than a major, so a full dose of Asunra may not be needed, especially if she continues to make progress, even if slowly. If splitting the dose has a positive effect on her liver levels then bumping the dose another 5 mg/kg per day might be possible. I don't think this trial should drag on too long, but if you try the split dose and see improvement, it will be worth it to continue.

Lena,

You are right,i would have to do that once i give split dose a try.

Andy,

You are right,i am aware that Z is not on her full dose,which would be 800 mg,she is on 600 mg basically because of her liver levels being unstable,so i am also hoping that splitting the dose might help us,regarding blood volume,i don't exactly know how much blood she is taking annually,but she is transfused 450 ml in every transfusion,and sometimes she goes on third week and sometimes on fourth,sometimes i do think about giving hydrea a try,with transfusions,but i am just afraid it would add more tablets to her schedule and more side effects to keep an eye on.

Thanks for your input,i'll check her records and will let you know how much blood she got in last year.

Zaini.

I try to put myself in Zainab's shoes and imagine what it is for a young thal to have to deal with a chelation scheme necessary in order to continue his/her life. You see, when I was young, chelation did not exist and I found out all about it when I was 15. That means all my life as a child was spent without having in mind I had to have desferal or ferriprox or exjade and which combination to follow.
Of course, I was lucky to survive, for I had reached the turning point and I hadn't realised that at all.
Nevertheless, up to that point I had to deal with my transfusion dates only and that gave me a bit of a rest.
In spite of having that rest however, if I had to choose between less chelation concern and being chelated from a smaller age, I would have chosen the second.
What I am really trying to say, is that I am jealous of all young thals as they start their lives with every possible medical help and every possible wide variety of their chelation medicines. And an access to gene therapy.

So, you have to choose and decide and this must make you thankful and optimistic.
Read, be aware of the new developments and choose and do not doubt that life will be fruitful despite the difficulties.


Lena.

Lena,

Thats what i told Z,that you are fortunate enough to have these things in ur life,not every one can afford these medicines and supplements,even some parents can't afford transfusing,they go to charity hospital where transfusion depends on availability of blood.And you are living a normal life despite of what you have,Allah Almighty has saved you from any complications,(God Forbid) and i have seen kids with terminal illnesses,the other day i was visiting the website of Make a wish Foundation Pakistan chapter,and i saw a pic of a boy who was in last stages of cancer,next time when we go to hospital,i'll make her meet cancer patients,if they are there on that day,i hope she'll understand the difference between why she has to take these meds or supplements and why they have to.

May be she is a bit young to understand this comparison,but i want her to be strong and confident,from right now when she is just a kid,so that she won't take people's worthless pity to heart when she grows up,and when she grows up she'll be a strong and confident person,who would take thalassemia as a part of life,and not life itself.She'll realize that these meds and supplements are friends and helpers,not obstacles or hindrances in life.

Andy,

I just check her records,she was transfused 14 times last year in 2009,and every time the quantity was 450 ml,is that ok or is that too much?

Zaini.

P.S She took her first half dose today.

Hi Zaini,

I think that both lil Z and lil A are going through this phase.  I wish we could sit them both together and talk to them!  I am realizing something though.  All of these years I have been very matter of fact with lil A - my attitude has been "it is what it is - here are your pills - lets get on with your day". 

Suddenly, in recent months - especially after having the parasite issue - he began complaining about all of the disgusting and unpleasant medications and supplements he has had to take.  At first I took the too bad, you are fortunate approach but I realized that he just needed to express that the situation "sucks".  He just needed me to acknowledge it - when I failed to, he said one day - "yes mom I AM grateful!  I never said that I wasn't - but all of the meds and pills make me want to throw up all of the time - somedays it is just too much - I am not complaining for no reason.  Caroa is too gross to even smell and I have had to take it.  Exjade tastes like chalk dissolved in water and I take it.  The pills are hard to swallow and make me sick for hours after but I take them - the needles and IVs have hurt but I take them - can't you just agree with me that it sucks sometimes?"  "I know I am better off than others, I am glad that I can run and play and be smart - but this STILL sucks - and I wish you would stop pretending that it doesn't.  It could be worse - but at least admit it is hard on me!"

This opened my eyes.  I do have to understand that most of the time I have to act matter of fact, but when he needs to say this I should let him.  I need to let him vent and feel understood.  I shouldn't make him feel guilty when he doesn't feel strong or when he doesn't take these meds with a smile on his face.  I should listen, understand and gently nudge him back in the right direction.

I hope that this makes sense, I have learned it from lil A recently and I am still in the process of understanding.  He didn't mind IVs and needles as much as he minds ingesting so many things. 

Sharmin

Lena,

What I am really trying to say, is that I am jealous of all young thals as they start their lives with every possible medical help and every possible wide variety of their chelation medicines.

Totally agree. I myself started proper DFO chelation at the age of 15 and oral chelation just 4 years ago. Things would have been completely different if I started it all at the initial age.

Sharmin,

You are totally right as always

Tariq and Lena,

I know,i can understand what you guys must have missed,i had a cousin who succumbed to thalassemia at age of 20 or 22 in 1985 or 86,coz chelation wasn't available commonly at that time,i totally agree with you guys,we have started splitting dose,she is taking 300 mg in the morning and 300 mg in evening,lets hope for the best Inshallah.

Zaini.

Things will work better, Zaini.
And if not, God forbid,you have other paths to follow. Do not worry.

Lena.

Zaini,

I really hope that splitting the dose helps. I am sure Zainab would like to give Asunra as much chance as possible to work for her. I think from the psychological perspective, this is best for her. If it still causes problems, she will know it's not the drug for her, but without trying the split dose, it would always leave a question in her mind.

Lena,

You're one of the survivors from your generation and your own strength of will is a big reason why you are here today to share your experience. I so wish that oral chelators had been available all along to you and those from your generation. I think many more would still be with us if this opportunity had existed. One question I have for you. I have seen this with some long term desferal users. The pump can become something of a crutch that isn't always easy to give up. Did you have any problem putting your pump away once you stopped using it?

Sharmin,

Thank you for a beautiful post and some perspective into the minds of the young. I think all too often we see the world through our own eyes and never understand the little things that can make life so much more bearable for our children. Just showing your child you can see things as he or she sees them can help to forge bonds that will last forever.

Andy,

 No problem at all, none whatsoever. In fact, although the pump has never been a special burden for me, there came a day- out of the blue - that I decided to make a turn with my chelation. I decided I wanted to stop the pump, stop the needles and improve my quality of life. Even if exjade does not work, I think I'll stick to Ferriprox - and that's that. I do not suggest, of course, that every thal should skip desferal like this - my last count showed my ferritin was 103. So, to answer your question Andy, although the pump did not make me considerably suffer I happily parted with it after 30 years.I parted it,though. I have not put it away, I haven't locked it in a drawer but I keep my fingers crossed that I will not use it ever again.And I know many friends of mine who do that, too.


Lena.

Lena,

Your attitude towards stopping the pump is what I expected when people first switched to Exjade, but I was surprised at how many people had a psychological in addition to physical dependency to their pumps, as the one thing in life they identified with having the ability to keep them alive.

These little psychological issues people have to deal with should always be recognized by those who wish to truly empathize with those who are living with thal. On the other hand, I think most patients have greatly enjoyed the freedom from the pump, and when an oral chelator does not work out for a patient, and it requires a return to the pump, there is a new set of psychological issues that needs to be taken into account, as it can be a depressing notion to think about returning to the needle. Because of this, among other reasons like better compliance, I do not think oral chelation should be quickly abandoned at the first sign of trouble, as has usually been the case with Ferriprox and we are now also seeing to some extent, with Exjade. Temporarily ceasing the drug and/or developing a new program for taking the drug, such as dose splitting, should always be tried before ceasing the drug altogether.