Devastating News

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Offline Lena

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Re: Devastating News
« Reply #150 on: May 05, 2009, 01:50:57 PM »
Dore,

In Greece too, in different transfusion units, there is not the same appliance to chelation scheme.
I think the main reason is there are many doctors who are afraid to press things and keep a steady dose when ferritin is low. But what does really low ferritin mean? when one gets 2 units every 20 or 30 days, a low ferritin can rise and be high in very short time when one does not chelate.
The right protocol is adequate blood to keep  hemoglobin high and adequate chelation to keep ferritin low. Of course, when the patient is too young things change.
When ferritin is low, normal, and your heart and liver are free from iron, then do  you think you must stop chelating? Aren't there any other organs to chelate? I think there are. That is my experience.
Maybe with a lower dose but stop on the whole? No, I do not think this is advisable at all.

Lena.

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Offline Manal

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Re: Devastating News
« Reply #151 on: May 05, 2009, 03:26:58 PM »
Hello Canadian family, and welcome back

Actually i think that Lena has a point to be considered. Why expose the body of little Miss A to an increase in ferritin again during these 3 months and start again chelating from the 1000s level. Can you ask her doctor about his opinion of a maintance dose  like 20mg/kilo

manal

Re: Devastating News
« Reply #152 on: May 05, 2009, 05:04:24 PM »
Hi all,

Great you have raised so many questions. These questions came across my mind as well. Different people react differently to the same drug. Little Miss A reacted so badly to desferal that she showed all the side effects from desferal within two years of usage. There are so many people using desferal for the last 20-30 years, yet they didn't show elevated signs of ear, eye side effects or don't develop osteoporosis.

Little Miss A not only had to stop desferal due to eye and ear problems but she was diagnosed with Osteo as well. Same thing happened when she start exjade, her ferritin dropped very fast and she had severe vomiting, nausea problems that persisted for months (as most of you know from our earlier discussions) than many others who stopped vomiting after few weeks of starting exjade.

We did talk to the nurse and she was concerned for the same (worst side effect of kidney problems, although there is no sign of it now). She was already on the lowest dose possible for exjade (taking 20mg/kg).

Well, I think there need to be a constant watch, i.e. monthly ferritin level check to see if it is going high. So far she is holding her hg pretty good and get transfused every five weeks. So it will be approx two more tarnsfusion between now and when she starts exjade again.

Hope that clears some of the queries. Thank you very much for insightful questions.

Regards
Regards.

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Offline Sharmin

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Re: Devastating News
« Reply #153 on: May 05, 2009, 10:39:38 PM »
Hi Canadian Family,

Yes all of those questions make a lot of sense, however when ferritin levels are very low taking chelators is not a good idea because they can cause many side effects.  Also, it is a good idea to give the body a break from chelators if ferritin levels are conducive.  If chelators don't have iron to chelate they will chelate other useful minerals and eyes, ears and bones can be damaged.  All of the things need to be weighed when balancing iron levels and the use of chelators. 

Perhaps when her iron levels get to about 500-800 it may be a good idea to put her on a low dose and see if you can perhaps maintain her with a tiny dose. 

All the best to our lil miss A,

Sharmin
Sharmin

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Offline Andy Battaglia

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Re: Devastating News
« Reply #154 on: May 05, 2009, 11:56:46 PM »
I do know quite a few people who have been told to take a break when their ferritin got very low. Mostly, this has been with desferal, but some on Ferriprox and Exjade have had the same experience. I think that with little miss A, her iron load has never been high and that regular chelation has reduced it enough to justify the break. With older patients who have had histories of slacking on chelation, their ferritin level may not really reflect the true iron load that still needs work, and taking any break from chelation must be based on both ferritin tests and organ scans. On the other hand, if monitoring is frequent, a good balance can be achieved, as in Lena's case, where the chelator dosage is well matched to the transfusion frequency and no interruption would be justified because she does have such a good balance between chelation and transfusion. Not all patients are monitored this closely and a break from chelation may be needed when the ferritin is quite low, because if any problem such as a negative iron balance occurred, it would not be caught early enough to ensure the safety of the patient. A lot has to do with where you are treated and the specific treatment philosophy of that center.
Andy

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Offline Lena

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Re: Devastating News
« Reply #155 on: May 06, 2009, 05:38:01 AM »
Yes, Andy, you are right. The treatment philosophy of my center is no break at all and in order to be on the safe side they examine our ferritin on a monthly basis and of couse MRI once a year. There have  been times when my ferritin reached 45 and even then I continued chelation. And my transfusion rate is not so high, I get one blood unit every 15 days sustaining my hemoglobin at around 11. It all depends on the treatment you get, and I tend to believe my center is very thorough in it. Consider that we are more or less 60 thals there and about 50% with ferritin around 100. It may sound aggressive to continue chelating with such low ferritin but I think, in the end, it is not. If you are monitored, of course.

Lena.

Re: Devastating News
« Reply #156 on: May 14, 2009, 09:53:27 PM »
Hi Friends,

We finally met with Dr. Sochett today, he is an expert on osteoporosis and bone health in HSC. He provided the following explanation.

1. She was born premature by 6 weeks, (premature babies develop bones slowly than full term babies).
2. There is no real conclusive Z-score for children and its only an approximation.
3. The Z-score of upto -2 in children less than 9 don't normally trigger alarm bells.
4. BMD calculates the mineralization in the last 4 vertabrate down the spine rather than full spine which is true for older patients but not for young children.
5. Little Miss A has a Z-score of -4.3 in the lower lumber spine which supposedly is due to her chronic illness (thalassemia), lack of calcium intake when she was on desferal, premature and genetically thin bone structure.
6. She does not have any fracture, bone pain and passed all the bone markers, therefore he concluded she has no osteoporosis. PHEW!!!!!!
7. Dr. Sochett advised that thalassemia patient have less than normal bone denisty no matter what but its a bigger problem at puberty age.
8. Biphosphnate drugs have side effects on children if they are taken orally (like Fosamax). The 4th generation drugs that are given through IV has less side effects and even they can be managed.

What we do next, keep up with the calcium and diet (he was happy that we were doing all the things right for the past six months, good job group!!). Check her BMD again in October 2009 to see if the scores have improved. Follow up next year.

So yes, Andy you were right she is doing okay so far and as a group we have manged to control the damage further.

If you have any questions, please let me know.
Regards.

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Offline Sharmin

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Re: Devastating News
« Reply #157 on: May 14, 2009, 10:00:45 PM »
Canadian Family,

That appointment went as well as it possibly could have.  I am very glad to hear that things are much better than they initially appeared to be - thank god!  You have done such an amazing job of controlling things and I know that it will all pay off over time. 

All the best to you and lil Ms. A!

Sharmin
Sharmin

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Offline Manal

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Re: Devastating News
« Reply #158 on: May 14, 2009, 11:01:39 PM »
Canadian family, this is indeed so good news and a big relief for all of us. You have really done an excellent job.

Quote
Biphosphnate drugs have side effects on children if they are taken orally (like Fosamax). The 4th generation drugs that are given through IV has less side effects and even they can be managed.

I have heard this too from one of the doctors and this has been done with some of the patients

Good luck. Little Miss A is lucky having such a wonderful and caring parents like you :wink

manal

Re: Devastating News
« Reply #159 on: May 15, 2009, 12:18:34 AM »
Thank you Manal and Sharmin.

We could not have done it without the help of our members.

Actually, Dr. Sochett was quite surprised when we discussed the situation with him, he was wondering where we work, but we told him about our website and the discussions within the group. He said other people should be getting this kind of help too because it helps him in his job as he can explain better.
Regards.

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Offline Andy Battaglia

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Re: Devastating News
« Reply #160 on: May 15, 2009, 03:03:55 AM »
I'm happy to hear the report I was expecting, and I think that the appropriate steps have been taken to get Little Miss A's bone development headed in the right direction. I am not surprised that there is no osteoporosis, as the bone density tests at young ages have little value outside setting baseline measurements for futures tests. Unless a child had been brought up on a diet of Dr Pepper and Skittles, it would seem impossible for a child Miss A's age to have bones as bad as had been suspected. Maintaining a high Hb and non-desferal chelation will play important roles along with the dietary considerations. I would be very slow to even consider biophosphonate drugs at this age. The importance of vitamin D in sufficient quantities cannot be overlooked, as it is probably more important than calcium itself in building bone.

I also want to point out to everyone the importance of vitamin D in this process. Vitamin D is probably mislabeled as a vitamin. It is actually a hormone and acts as a receptor for dozens of essential nutrients. Without adequate vitamin D, it is impossible for these nutrients to be used by the body. Calcium is one of these nutrients and without enough vitamin D, calcium will not do much good. So, everyone should remember that calcium has to be taken in a context that includes vitamin D, magnesium and the essential trace minerals that are required to build bone. A product like Osteocare, takes care of most of the needs. However, added vitamin D is usually necessary. Recent research has shown that an astounding percentage of the human race has low vitamin D levels, and research with thal majors shows that almost all are deficient in vitamin D, which should not be any surprise, as we see this with most vitamins and minerals. The stresses of thalassemia result in a much higher need for most nutrients than what is normally seen in the non-thal population. The best source is direct sunlight. I read just this week that window glass filters out the UV B rays. UV B rays are responsible for producing vitamin D on the skin, so sunlight through a window has a greatly reduced value. It should be direct outdoors sunlight. Unfortunately, this is impossible in northern climates during a great part of the year, so supplementation (or a "safe" tanning bed) is necessary.

The growth and development of this group has been years in the making, dating back to the late 90's, and we have become a significant entity in the international thalassemia community. It is heartening to hear these reports that doctors are pleased that patients are coming to them with a real understanding of what is involved in the various aspects of thalassemia and its treatment. Too often, doctors seem annoyed when patients know a lot and ask questions, so it's very encouraging to hear that our efforts are proving helpful to both patient and doctor. I hope that the doctors are becoming aware of thalpal.com and that there can be real value to internet forums when the dedication and work to succeed are present. The efforts of our community to share experience and to help and teach each other is having tremendous results for many people. It is a true community effort and could well be a lesson to other groups on how to be effective by putting the common purpose first and foremost.

Andy

All we are saying is give thals a chance.

Re: Devastating News
« Reply #161 on: May 16, 2009, 01:08:14 AM »
The importance of vitamin D in sufficient quantities cannot be overlooked, as it is probably more important than calcium itself in building bone.

I also want to point out to everyone the importance of vitamin D in this process. Vitamin D is probably mislabeled as a vitamin. It is actually a hormone and acts as a receptor for dozens of essential nutrients. Without adequate vitamin D, it is impossible for these nutrients to be used by the body. Calcium is one of these nutrients and without enough vitamin D, calcium will not do much good. So, everyone should remember that calcium has to be taken in a context that includes vitamin D, magnesium and the essential trace minerals that are required to build bone. A product like Osteocare, takes care of most of the needs. However, added vitamin D is usually necessary. Recent research has shown that an astounding percentage of the human race has low vitamin D levels, and research with thal majors shows that almost all are deficient in vitamin D, which should not be any surprise, as we see this with most vitamins and minerals. The stresses of thalassemia result in a much higher need for most nutrients than what is normally seen in the non-thal population. The best source is direct sunlight. I read just this week that window glass filters out the UV B rays. UV B rays are responsible for producing vitamin D on the skin, so sunlight through a window has a greatly reduced value. It should be direct outdoors sunlight. Unfortunately, this is impossible in northern climates during a great part of the year, so supplementation (or a "safe" tanning bed) is necessary.


Hi Andy,

You are right above.

Dr Sochett stressed that in northern climate where summer is hardly two-three months, it is important for everyone to have sufficient vitamin D. For people with darker skin living in northern climate it is more important to have vitamin D.

Little Miss A was having 1200 IU of vitamin D and that was enough during winter time. However, Dr Sochett cut the dose of vitamin D to 600IU only and asked to take Little Miss A to the park for an hour during the season for natural source of vitamin D.

He advised to stay away from the contact games, running, jumping, chasing and teasing the brother are recommended.



Regards.

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Offline Dori

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Re: Devastating News
« Reply #162 on: May 16, 2009, 11:27:43 AM »
Recently I reread S. M???  "survival of the sickest" and realized again that I should discuss the connection between caalcium & vit D with my doctor & also should ask for lab work.
Btw like your daughter I was born premature but by 4 weeks. Never heard about this and low bone d.
I am sorry but due my bad health I can not remember the correct spelling of the words.

I am glad the consult went well.

Dore
I am also allergic for desferal. I had to stop because it took my breathe at a Oct evening on 2006. Plus I had a terrible rash with ulcers.....:( have you ever considered L1 (deferiprone/kelfer)?

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Offline nice friend

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Re: Devastating News
« Reply #163 on: May 16, 2009, 01:04:09 PM »
Quote
Btw like your daughter I was born premature but by 4 weeks. Never heard about this and low bone d.
I am sorry but due my bad health I can not remember the correct spelling of the words.

Dore,
i think, you're talking about "Low Bone Density" ...
Best of luck for your health , hope that you will get well soon , :getwell ...

Best Regards
Take Care
Umair
Sometimes , God breaks our spirit to save our soul.
Sometimes , He breaks our heart to make us whole.
Sometimes , He sends us pain so we can be stronger.
Sometimes , He sends us failure so we can be humble.
Sometimes , He sends us illness so we can take better care of our selves.
Sometimes , He takes everything away from us so we can learn the value of everything we have.

===========
Umair

Re: Devastating News
« Reply #164 on: May 16, 2009, 02:54:27 PM »
Hi Dore,

Little Miss A is on Exjade for the last 16 months or so. The underlying Osteo problem was initially diagnosed in November 2008.
Regards.

 

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