Question for Andy...

Hi Syaida,

Exjade is much easier to administer than desferal, so it has become the drug of preference these days. Its actions are fairly equivalent to desferal and it requires no needles, so most doctors are now leaning towards Exjade for early use. I'm not sure I would want to go through the hassle of trying a needle on a young boy. When he does start Exjade, whether now or later, start with a low dose and build up to the required dosage, as his body gets used to the drug. This will eliminate most of the early side effects, like the rash. Get him used to drinking tea also, as this helps inhibit iron absorption and is a natural chelator.

Which chelator is best? Every company will argue for their drug, but studies have shown that Ferrirprox (L1, deferiprone, Kelfer) is best at removing heart iron and also offers a cardio-protective benefit, that the other chelators do not. Why is this most important? Because heart failure is the number one cause of death in thalassemics. In addition, after over 20 years of use, no further side effects for L1 have been found. The drop in white cells in some patients is long established as the main detrimental side effect of L1. Joint pain is the other side effect most often mentioned. Compared to the other chelators, L1 is the superior drug. I would also be a bit reluctant to start a young child on L1, but by age 6, it should be considered, depending on how well the boy is doing with his current chelation regimen.

I also want to mention for all, that most doctors agree that patients should not rely on only one chelator during their lifetime, as switching chelators can help to minimize any long term effects.

Thanks, Andy I'll bear in mind what you said. First and foremost, you cannot imagine how happy when I stumble upon this network. My caregiver and I are now on this everyday and taking down notes.
I do not know how/where to begin on what me & Adel had gone through these 3 years. This lil guy is indeed very special. His pregnancy was very dramatic, he had plueral effusion on his left lung at 16 weeks. I went through procedure to "tap" his lungs in in utero. On further investigations, at 19 weeks he was found to be B-Thal Major with a big VSD (hole in the heart). He had had 3 heart surgeries (at birth) and thereafter was very sick including jaundiced. He was termed chronic lung disease and has been on O2 ever since his heart surgeries. Blood transfusion started soon after heart surgeries too. We noticed his spleen & liver were rather big at about 3-6 months old. We opted for a splenic embolisation before his 2nd birthday cos he started become sickly and his transfusion frequency was like weekly due to splenomegaly. Thereafter, he was well (started walking, feeding making huge milestones) for the next 9 months, was transfused between 6-4 weekly and the transfusion frequency became 3 weekly last Dec. We gave him a 2nd embolisation this year January. He has been on 4-5 weekly transfusion since.
I know it's very hard to do the needle on a young boy, but this boy has tremendous understanding, it may be difficult for other kids as they are restrained from their movements, but lil guy is quite used to being confined within limited area, reading, writing, playing his puzzle are his favourite pastime. He was even on a 24 hr feeding pump (via gastrostomy) before his embolisation, as his liver & spleen became so big abdomen became distented, we couldn't keep any milk down. We got rid of that pump after he became well after his 2nd birthday. I guess if we can't cope with desferal, we'll switch to ExJade. I'm not sure if you've heard of Ambrotose, Adel has been on it for 1 year I feel it really helps as a natural chelator.
     

Desferal may be a better choice. The prior problems with the liver and spleen lead me to think that desferal may be better tolerated at this point. If liver function is currently affected by enlargement, it might be wiser to wait on starting Exjade. The other problems he faces complicate matters, as you don't know what to expect when new drugs are introduced, and desferal is probably the safest chelator to start. With chronic lung disease, I would suggest always watching for any breathing reactions from any new drugs that are introduced, and desferal is known to cause allergic reactions in a small percentage of patients, so watch for any sign of allergic reaction.

A good buddy of mine, who I met through this group, takes products from the same company that makes Ambrotose, and he feels he has done quite well in terms of health and energy. There are many natural products that can help the health in general, which is very beneficial for anyone already dealing with health issues. I would definitely suggest natural vitamin E for your son. Its long term use will prove quite beneficial for heart and lungs.

Hello Syaida Lee and welcome to the site

I am happy that you were able to pass through all these hardships that faced Adel and you and i hope he can cope with desferal with no problems. Hats off ....you are doing a wonderful job. Keep us updated
manal

what is green tea

Hello Dear Andy
I m on Desferal + Feriprox  combination therapy  I m taking 6000mg of  Feriprox tablets  and  3 Grame of Desferal  Injections on  SUB-Q.
So cane you tell me when I missed my feriprox tabets dose for example I missed  my morning dose so cane I take with midday dose    .
And please tell me after  taking  feriprox . cane I take vitamin C with desferal .

Yes, you can continue to take vitamin C with desferal. Take it when you are starting the desferal. If you miss a dose...

http://www.ferriprox.com/faqs/default_intl.asp

What should I do if I miss a dose of Ferriprox?
If you miss one dose take it as soon as you remember and take your next dose at its regularly scheduled time. If you miss more than one dose do not take the missed tablets, just continue with your normal schedule. Do not change your daily dose without first talking to your doctor.

Here we take vitamin C with ferriprox,too.
Ferriprox leaflet writes you can take vit c according to your doctor's guidance.

Lena

Andy and Lena thanks a lot for you are reply.

my docor advise for taking vitamine c with only Desferal.

butt tell me when i take Feripox so after how jmuch  time i can take vitamin c with desferal .

or i take vitamin c  then after how much time i take feriprox .

Rehman,

you can take vitamin c preferably when starting desferal or even a little before. Do not worry about Ferriprox at all. Just take vit.c when starting desferal.

Lena.

thanks a lot .

Dear Andy,

Regarding copper supplements BB and LB have been taking half of 2mg tablets now every day. I still need to know how much to give them, how is it worked out?

Both brothers as you know are on Ferriprox so should they continue to take Ascorbic Acid 100mg daily when taking Ferriprox?

I would like to know that if, as LB is, low in Vitamin D, could this cause yellowing in the white of the eye? He has recently started taking Vit D solution again.

Hope you are keeping well yourself. 
These may be little issues but i thank God you are there for us.

Hi Emby,

I would suggest keeping them on the current dose of copper until the doctor says it's time to test again and then adjust the dose if needed. Small amounts of vitamin C are safe. It's best if not taken with meals, as to minimize any added iron absorption from food. I think vitamin D is indicated in thal and especially when you see symptoms like yellow eyes. This is a sign of jaundice which is related to the breakdown of red blood cells. You also will usually see a high bilirubin level when the eyes are yellow. Vitamin D (1000-5000 IU daily), B complex 100 and magnesium (500-750 mg daily) may help to slow this breakdown, leading to less bilirubin development. Vitamin D's importance has only recently begun to be realized and its importance in thalassemia has led to doses of 50,000 IU at once, to correct deficiency. Testing for D is simple. A value below 30 needs correction and we may eventually learn that 30 is low.

Thank you Andy,

LB's bilirubin was quite high last month ans so was his ALT. This month ALT is normal and bilirubin levels are lower but not quite normal. His Vitamin D was 6.1 (!). I would like VIT D checks to be routine here but it isn't. Takes time for results to come and to get med for it. It's such a headache cos we keep having to chase everything up when the Dr.'s are supposed to be on top of it all  !

My son Jia has his ferri scan done at 4. Now he is 7 and hasn't had another one done yet. The ferri scan result was very good the last time and the doctor decided not to do another scan in 1 year. Maybe he should go for a scan soon now that it has been so many year's lapse. His ferritin level is now around 1300. The doctor here doesn't want the ferritin level to drop much past 800. She said it can affect growth if over chelated. I don't have many older thalassemia persons to consult here. Only 4 kids in Perth with thalassemia major.