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A message for all  parents who are thals. Keeping your iron load under control is an absolute obligation to your children.
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Author Topic: Some notes from the Singapore conference  (Read 12102 times)
Manal
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« on: October 30, 2008, 04:28:12 PM »

Here are just some notes from different topics dicussed in the conference, we can then discuss each speperatly in seperate posts.

1- Usage of Antisense Technology to restore correct splicing... this topic was discussed under the gene correction.
Actually i am trying to read in this topic but can not grasp it 100% so i will be great if Andy would shed some light on this. But we can say that in antisense technology, synthetically-produced complementary molecules seek out and bind to messenger RNA (mRNA), blocking the final step of protein production. mRNA is the nucleic acid molecule that carries genetic information from the DNA to the other cellular machinery involved in protein production. By binding to mRNA, the antisense drugs interrupt and inhibit the production of specific disease-related proteins.

This applies mainly to thal mutation that are related to the m(RNA) and not the beta or alpha globin

2- Thalidomide derivatives increases Hb

3- Oxidative stress oxidizes proteins, lipids and DNA leading to organ failure and antioxidants are
       a) Vitamin E is safe upto 600mg
       b) Curcumin up to 500mg
       c) N-Acetyle Cytosine
       d) Fermented Papaya preparation FPP

4- Usage of Nitric Oxide in thalassemia is best studied by Doctor Gladwin in the USA ( actually this was an advice from doctor Rachmilewitz but he couldn't give me the full name Undecided

5- Labile Plasma Iron is the blood test made to see how much free iron radicles are there in the body  ( actually not availale in every country )

6- In answering one of my questions about any available information about iron being overloaded in the brain, Dr. Ali Taher said that there is an ongoing study measuring this through MRI on the brain.

7- Living with low Hb does not affect mental skills or IQ unless the ''normal for the person'' changes then transfusion is then required.

8-  ECG can detect signs of Pulmonary hypertension and should be done every year. Then from the age of 10 years ferriscan and T2* should be made even if the patient does not transfuse.

9- Very important note: Having gaps in chelation is not recommended at all because in the time of this gap all the iron tend to settle in the heart and not the liver so a patient who have lots of time gaps in chelation has to check the heart iron as the liver iron overload will not be a sufficient indicator

10- We can have iron overloaded in the heart and the liver is free of iron and vice versa, that is why T2* is important to do in addition to ferriscan.

11- We can not predict heart failure but there could be some signs
       a) murmurs
       b) JVP
       c) Low Cardiac output (CO )
       d) High BNP
Heart failure could be seen in both transfused and non transfused thal. In majors through ironoverload and in intermedia through increased CO . In certain cases heart failure can be reversible and L-Carnitine is advises tobe given to major and intermedias.

12-  Ca2 ( Calcium 2 ) works as a blocker to iron in the heart

13- Splenectomy is not at all advised or we should keep it as long as we can

14- Partial splenectomy is no more done in the UK as the spleen grows again

15- Post splenectomy sepsis can not be prevented by education but infections can ( A comment that i liked from one of the doctors )

16- Ironoverload distribution usually in Parathyroid, Liver, Pancreas, Thyroid, Skeletal tissue, then the brain ( can not recall where does the heart fit )

17- Role of Hepcidine in iron overload

18- I asked many of the endocrinologist about this fact, the body of the thal is usually insensitive to the growth hormone and can not use it fully though it is there in the normal range '' they said that this is true and when i asked them should we give growth hormones in order to stimulate the body to use it , all of their answers was a clear NO

19- What is monitered in growth is
        a) Height velocity (very important)
        b) Height sitting and standing
        c) Bone Age

20) Studies show that thal girls height tend to be in the normal range while thal boys tend to be short

21) Very important to measure the spinal growth because reduced spinal growth affects height in puberty.

22) Studies found that the usage of desferal before the age of two years of age cause the spinal cord vertebra to flaten leading to a short spine but the limbs are not affected and they grow normally. This problem was not seen in children who used desferal after the age of two. That is why spinal bones measurement is important.

23) Osteoprosis causes are multi factorial and most of them are not known to doctors. Intermedias and Majors both develop Osteoprosis. Though majors have high Hb but they too develop Osteoprosis. That was one of the lectures that had a negative impact on me cause the two doctors who talked about Osteoprosis said that you can not avoid cause the reasons are unknown multi factorials. But from the known causes
       a) Bone expansion
       b) Hypogonadism
       c) Calcium, zinc, vitamin D definciency
       d) Lack of exercises 
So these lectures talked more on medications rather than preventation

24) Role of Glutamate in growth

25) Intermedias are prone to
       a) Leg ulcers (shortage of bloood supply to limbs)
       b) PHT
       c) Thrombosis
Intermedias more than 20 years  should have thrombophilia screening if they have family history or had previous thrombophilia. Intermedias even children should have anticogulante medications like asprin for example. When i asked Dr. Ali Taher that giving baby asprin is not allowed except after the age of 12or 13 years to any child. He said that this is true and the other fact of high risk of thrombophilia is true too so you have to make a balance between the two risks ( the concept of choosing the best of two worset cases )

Causes of high thromophilia rate in intermedia:
a) increased number of platletes
b) the different nature of red blood cells caused by the presence of extra alha in the blood
c) Endothelial cells injury in splenctomy leads toan increase in the production of platletes

Generally speaking most topics concerning intermedias need further studies and investigation

26) Only thing mentioned about thal minors is that they have a high risk of Oligohydramnios in pregnancy

27) Though Hb F does not release oxygen, it was found that an old man around 65 years has a homozygous thal mutation 39 who has 100% of his blood HB F and was never transfused

28) The same mutation was found in two brothers, one of them is transfusion dependant and the other is not though they have the same mutation. This leads us to a big fact that each thal patient is a completely different and independant and you can not compare two patients togather cause ther are many other factors in the body that play a role in the manifestation of the disease even with the same mutation

Hope this hepls and we can choose any of these topics to discuss seperatly

Take care
Manal
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Zaini
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« Reply #1 on: October 30, 2008, 05:12:13 PM »

Manal,

This information is great,each and every point is important,iron over load,osteoporosis,puberty and what not,let's see what Andy has to say about this.

Zaini.
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« Reply #2 on: October 30, 2008, 06:01:14 PM »

Manal,

Thanks for this very important information. 

Sharmin
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« Reply #3 on: October 31, 2008, 03:01:28 AM »

thank you manal for the efforts to inform us about the updated info's on Thal.

What I dont understand is the use of Fermented Papaya. Isnt it act as a supplement that is rich in Vit E? If it is so, than why should you take fermented papaya instead of the Vit E's tables itself?
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« Reply #4 on: October 31, 2008, 05:50:54 AM »

Hi Nur

FPP is relatively a new product and they have researcehd that fermentation (Yeast0)of these genetically reengineered papay'a are good anit oxidants. There was a doctor in the presentation - the guy who spoke about the antioxidants -highly recommended this FPP. It basically tries and controls oxidative stress and aging.  It is said that Japanes have a longest life span and it has been owing to their way of using fermenting vegetables. This FPP is a Japanese product.

How it is more beneficial than vitamin e, is not mentioned anywhere.

Take care

Puja
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Manal
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« Reply #5 on: November 01, 2008, 01:07:29 AM »

Thank you my friends.

Nur

FPP is a seperate product than vitamin E. It is just another anti oxidant and it does not even have Vitamin E
http://www.osato-ori.com/index.php?option=com_content&task=view&id=19&Itemid=37
Quote
Immun’Âge is a food supplement with 100% FPP®, is made by fermentation of a tropical fruit, the carica papaya. But fermentation ends in an exogenous antioxidative papaya properties high decrease due to elimination of all vitamins A, C or E by this FPP process.


Its usage is recommended for the following:
Quote
Immun’Âge properties associated with its Harmlessness (no toxicity, no side effects) allow
recommending it for anybody for:

1.States related to a free radicals excess due to :
[
b]Aging[/b]

Environment factors such as pollution, tobacco, stress….
People are exposed to lifestyle which promotes stress, fatigue, insomnia, neurosis, nervous breakdown…Everyday pressure relative to work, personal problems, chemical, drugs or sun exposure, noise or cigarette smoke pollution are most common factors which lead to a permanent oxidative stress.

Pathologies: Cataract, Cancers, Cardio-vascular diseases, Diabetes, AIDS, Rheumatisms, Cerebral degeneration, Alzheimer, Parkinson, Hepatitis, Alcoholism, Chronic bronchitis, Asthmas, Thalassemia, Infections, Inflammations.

In quite all these pathologies, we found production of large amount of free radicals leading also to oxidative stress and inflammation when our antioxidative capacity is overtaken.

By fighting against free radical excess Immun’Âge allows patient to recover a better quality of life and ease conventional therapies against diseases. Immun’Âge didn’t cure any disease (it’s not an alternative medicine) but could ease (by a physiologic complementary action) normal treatment by managing oxidative stress.

Antioxidant deficit:
Antioxidant deficits of vitamins A, C, E and minerals (Selenium, Zinc, Copper…) by low intake of nutriments (unbalanced nutrition and diets) could lead to oxidative stress by decrease of antioxidative system capacity.


2.States requiring an optimization of organism defenses system

Aging

Hivernal period : cold, sore throat, inflammations, fatigue.

3.Dermatology
Prevention of skin accelerated aging: exposure to the sun, age.
 
 


So to have a summarey, the FPP is responsible for the regulation of our  natural defense by enhancement of both Antioxidative System and Immune System.

Please feel free to ask anything and a big hug for your sweet daughter

manal
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« Reply #6 on: November 01, 2008, 07:43:10 AM »

Thanx Manal for the valuable info. The point that catch my attention was there should be no gaps in chelation. What if u can't take desferal for few days but still are on L1/Kelfer, that should be OK right?
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Manal
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« Reply #7 on: November 01, 2008, 08:46:11 AM »

Thanks Tariq,

To me this would be okay, as long as there is a chelator in the body that binds with iron to prevent overload especially that L1/Kelfer is the known to remove iron from the heart in an effective way.

manal
« Last Edit: November 01, 2008, 10:27:21 AM by Manal » Logged
Zaini
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« Reply #8 on: November 01, 2008, 07:41:41 PM »

Tariq,

Don't quit desferal for long time,i did it once and regretted it,my daughter's ferritin climbed her highest in those days.

Zaini.
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« Reply #9 on: November 02, 2008, 09:48:47 AM »

Thanks Zaini for your concern, But don't worry i don't have any plan t stop desferal. i just asked to just know if i have to give a desferal break due to some illness or something, i should be sure that it would not harm me.
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« Reply #10 on: November 02, 2008, 10:08:42 AM »

Thank you for the information Manal. I don't think all info is useful for a pk-d patient, but it is interesting stuff! I really hope that there comes a time that they can give us more information about pk-d.

no gaps in chealtion  forever engaged with chelation med 
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