Devastating News

Wishing you the best C.F .

Zaini.

You are in my prayers

manal

HI CF,
praying  for you ...

Best Regards
Take Care
Umair

ANDY i have been giveing aysam fish oli and may other stuff te doctors keep telling me that his results arent to bad his liver mmm is it from exjade or his iron knowne knows

proffecer ali taher saidfrom his iron overload he said get his iron our fast and exjade will work perfectly you know andy you say that his liver test not that bad but he is 2 years and 7 months how much can he handle ieen maybe liver test are not going to kill him now but what d we know what is happeing inside him he is to young to have theses reading high low somethig is happeing or something is dameged i feel alreday to late
they said if e liver test goes to 600 then we stop exjade mmmm well its 300 isnt is alreday high i dont know today im going for blood test for him hopefully he will have normal results i will imform you guys of his liver test and hb his hb i always going down 9.3 never higher so lets see

Hi Mariam,

If Aysam can lie still, he can have a Ferriscan MRI done. It is usually not done on such young kids because it is hard to get them to sit still for the required time. At his age, I don't even know if it is necessary. Keep chelating and working to get his iron down. If you do move to Lebanon, perhaps something can be arranged to import Osveral from Iran. The cost is very low compared to Exjade and it is the same drug. This may also be of interest to Dr Taher. He may have met the rep for Osveral in Singapore. This would greatly help patients in many countries and should be explored.

Osveral ? whats this i never heard about it so andy really you think his iron is to high still

Mariam,

His iron is still high but it has come down from the peak. Is he taking the proper dose of Exjade yet? 30 mg/kg daily. At his age, many problems can be averted if you can get his iron levels down. Exjade will work, given time and taken in the proper dose.

Osveral is the same drug as Exjade, but made by an Iranian company. You can read about it in our thread at
http://www.thalassemiapatientsandfriends.com/index.php?topic=2271.msg20044#msg20044

Osveral = Exjade = Asunra

Deferasirox is the name of the substance. The other names are only given to the drug by those compagnies.

How high is his iron?

Btw I met an aunt from my mother today. She got primary iron overload (hemochromatose). She told me her ferritin must be under 50. My doc want to stop with Exjade when it comes under 1000. May I laugh? I will never stop at that level. duuhh!

Dore,

Exjade=Asunra=Osveral=Desirox.

Andy,

I tried contacting Osvah Pharma,through email,but there was no response  .Do you know what price they are offering?

Zaini.

Dear Canadian Family

I wish to clarify a few things and hope these would help you.

First of all, I would like to confirm if your child truly has osteoporosis as BMD is never accurate at that young age. Does your child had any fracture at all? If she is so osteoporotic at her age she would be having fracture every where like the brittle bone disease. Doctors should treat patients as a person and not on results of investigations. If she doesnt have any symptoms, so not to worry yet just start your prevention regime as mention by your doctors and the best would be excercise. Most doctors would like to be overcautious and warn you of the worst scenario and most of the time the disease is not that bad. It is one way to make sure, patients follow the doctors instructions. So dont worry too much.

Secondly if she truly has osteoporosis, i would like to find out if other underlying causes of osteoporosis has been ruled out, as it is uncommon to have osteoporosis at this young age in thalassemia. Furthermore, BMD or Dexa scan cannot really diffenrentiate between the bone quality and bone density. Poor bone Quality is like Brittle Bone Disease? osteodysthrophy? Athrogryposis? and poor bone density is like Osteoporosis.

In the treatment of osteoporosis the best would be - adequate calcium suppliment, Vit D, sunshine and light exercise. The most important of all would be EXERCISE. Building bone is like building a house, you need the bricks (calcium) but you need a builder too. The builder are the stimulus that exercise can provide. I would not suggest bisphosphontes, or forteo or strontium for the treatment in young children as there are no studies to show its effectiveness and I personally feel there is a risk that this medication would stunt the growth of the bone. Weight bearing exercise such as light jogging would be the best. Zinc is a good suppliment and there is an ongoong studies to prove its effectiveness.

Osteoporosis is symptomless, it only present with pain when there is micro fracture or obvious fracture. So avoidance of fall or trauma is a another important and most neglected way to prevent fracture in the treatment of osteoporosis. One of the best way is to use protective gear and avoid contact sports. Aquatic exercise is one of the best form of safe resistance exercise and kids just love it.

Since if she is asymptomatic, you should not be too worry. Protect her but not too overprotective as this would affect her mentally as she grows older. So try not to think of her as a fragile child but a special child with a special needs and care.

Dear MikeKew,

You posted a very valuable and comprehensive post, I loved reading it.

Yes you are right that there is no standard BMD scores for children under age 8. However, the DEXA 'tries' to translate the scores for bone density results on an age appropriate basis for children(you need special software for translation and not many DEXA machines have it). The results can be seriuosly flawed. Our hospital claims to have such software.....

My daughter has no fractures, no bone pain and she regularly goes and enjoys her gym classes. We are on prevention regimen as you mentioned (i.e. calcium, vitamin D and food supplements) since Novemebr 21, 2008. I agree with your observation that doctors do 'over exagerate' the situation to get patients or caregiver worried for more compliance.

I am not looking forward to the biphosphanates myself and have raised this issue with the hospital. We are still waiting for the appintment from bone clinic. Our recent conversation with the hospital was that there may be a cocktail of calcium, zinc, vitamin D etc. be provided, for her to take and repeat the test in six months but we will see and wait.

You explained the bone building procedures accurately.

Take care

MikeKew, thanks for such an informative post and welcome to the site

manal

Hi Canadian Family and Manal

Hi guys, Thanks for your pleasant welcome. With regards to your child, the thing that I would like to advice is the exessive use of DEXA scan to diagnose and to assess oseteoporosis. I do think that DEXA scan is overated investigation with not much practical use. In my opinion, it actually does more harm than good especially in children.

My reasons are,

1) DEXA scan is Dual Energy X-ray Absorbmetry, it is not important to know how it works but what is important is that it is an investigative tool that useses X-rays. X-rays means irradiations. Irradiations means risk of cell or genetic mutation. Imagine what it would do to the already immature sexual gonads of thalassemic child. Irradiation would also cause damage to the bone marrow function which is important in thalassemic child who needs good bone marrow function to produce new blood. DEXA has much higher irrradiation level than a normal x-ray.

2) Inaccurate comparative results for children and young adults.There are no accurate refferances for DEXA scan in these age group which is the common age group for thallasemic to have osteoporosis. You can get high rate of false positive or false negative.

3) Does the results of the DEXA scan alter the management of Osteoporosis in the Thalassemic child? No, it doesnt at all. Would you stop your osteoporotic preventive regime if the results comes back as normal? No, you still continue the preventive meassures despite the results. Would you increase the dose of the calcium or other medication if the results is low? No, the treatment of osteoporosis is not dose-related as in hypertension or diabetics. You still take the same dose calcium, zinc, forteo, bisphosphonates and still do exercsie.

So why in the world do we do DEXA scan? You see, doctors are thought to to treat patient on "evidence based medicine". It is the right way to treat but not in all diseases. Doctors lost the holistic approach in treating patients. I find the usage of DEXA scan is to treat the doctors and parents, not treating the child. If the results is back to normal, it makes the parents and the doctors happy and if comes back with worse results it adds anxiety to the parents and the doctors but at the end how does the results affect the management and the well being of the child.... NONE. Basically DEXA scan is to treat the parents so that they can sleep better knowing that their child is not osteoporotic but fact remains Thalassemia will have osteoporosis at a much younger age.

As I have said treat your child as a human being not as just figures of a DEXA Scan. If you child is symptomless and happy, why subject them to this harmful irradiation, which serve no practical advantange or change in the management of osteoporosis.Doing once in few years time is alright.

However DEXA scan is a very useful tool to assess the results of a treatment in the experiments of new drugs or in studies of osteoporosis.

Lastly, the main objective in the treatment in all disease is to give them a happy and productive life. Ask yourself if you are overprotective would they be happy? On the other hand you need to caution them to be extra careful. So let them be as happy as they possibly can.

Cheers.

P.S. I have posted this in other thread too.

Hello all,

I just found this info on DEXA and think it may be useful for all of us.

http://www.radiologyinfo.org/en/info.cfm?pg=DEXA

Thank you for the link Canadian Family,

I also called Oakland to address these concerns.  They have also told me that risk of exposure to radiation is less than that for a chest xray and probably less than it would be for an x ray taken for a broken bone.  Oakland routinely conducts DEXA scans on thal children. 

This is probably a silly question, but is it the hip that will be scanned during the DEXA scan for thalassemia?  In that case, I may have some concern about the proximity to gonads being exposed to radiation......

Andy, what do you think of this procedure?

I guess it is always good to ask questions,

Sharmin