Apoptosis & L-Carnitine

Hi,

From the first day my son was diagnoised, his hema. prescribed him the L-Carnitine oral drops that were given twice daily for the first month and then twice 5 days in the week forever. Actually, i just thought that it boosts all the body muscles and enhanced the activity ,but i read a study (by the way my son's hema participated in this study) that made me understand what it really does.

It was found that L-Carnitine reduces apoptosis in many diseases such as thal.
http://www.medterms.com/script/main/art.asp?articlekey=11287

Apoptosis: A form of cell death in which a programmed sequence of events leads to the elimination of cells without releasing harmful substances into the surrounding area. Apoptosis plays a crucial role in developing and maintaining health by eliminating old cells, unnecessary cells, and unhealthy cells. The human body replaces perhaps a million cells a second. Too little or too much apoptosis plays a role in a great many diseases. When programmed cell death does not work right, cells that should be eliminated may hang around and become immortal. For example, in cancer and leukemia. When apoptosis works overly well, it kills too many cells and inflicts grave tissue damage. This is the case in strokes and neurodegenerative disorders such as Alzheimer, Huntington and Parkinson diseases. Apoptosis is also called programmed cell death or cell suicide. Strictly speaking, the term apoptosis refers only to the structural changes cells go through, and programmed cell death refers to the complete underlying process, but the terms are often used interchangeably

Apoptosis also can cause heart failure
http://www.medicinenet.com/script/main/art.asp?articlekey=565

Researchers in Boston recently examined hearts obtained during heart transplantation. The hearts from patients with cardiomyopathy demonstrated the characteristic features of apoptosis. These results, reported in the New England Journal of Medicine (1996;335:1182-9), support the concept that programmed cell death plays a role in leading to end-stage heart failure.

So the study they made showed that L-Carnitne helps in reducing apoptosis in thal
http://content.karger.com/ProdukteDB/produkte.asp?Doi=87890

 

Conclusion:L-Carnitine seems to be a good modulator of apoptotic processes in thalassemic patients leading to a decreased frequency of programmed erythroblast death and general improvement of the disease condition.

I thought this information could be of any help

Take care
Manal

Thanks for sharing this info Manal,

It is very interesting indeed.

Take care, Peace!

Manal,
     You never stop researching and sharing things that will help your son and others. You are great! Jean

Thanks Manal,

You are the best  .  I hope your kids are well 

Would L-Carnitine be helpful to thal majors as well?  Andy, do you have any thoughts on this?

Sharmin

You are welcomed Sajid, i am happy you found it interesting


Jean, thanks for the compliments . If you find anything good, it is because of you and each member in this forum  :grouphugwho give a lot of inspiration and hope that there will be a cure, so until then we have nothing but to search and search and share whatever we find useful.


Sharmin, you are welcomed and thanks for the compliment. Speaking of the best , i guess it is you because you are one of the great moms who was able to change a really difficult and hard disease( in all terms) like  thalassemia to something ''nice'' and ''smooth'' that your son is ''enjoying'' and coping with. I guess i will be in need of a lot of advices and strategies because Ahmad is really giving me a hard time (not the attitude of crying,but his depression since he is the only one in the family who is always poked with needles) with every needle he gets.

Anyway, back to L-Carnitine, the study was made on thal major children from the age of 6.6 to 12.2 years.

We investigated the effect of oral L-carnitine therapy on apoptosis in thalassemia major patients. Methods: Eighteen thalassemia major patients with a mean age of 12.2 ± 6.6 years were included

Love to you all

Manal

A big part of the problem facing transfusing thals is the rapid deterioration of the transfused RBC's. L-carnitine has been shown to slow the deterioration of RBC's.

From http://grande.nal.usda.gov/ibids/index.php?mode2=detail&origin=ibids_references&therow=800548

After one month supplementation of L-carnitine lipid peroxidation and intracellular calcium concentrations were found to be decreased and RBC deformability was improved, accompanying the significantly increased plasma L-carnitine concentrations. These results suggest that L-carnitine can be used as a supplement in beta-thalassemic patients, to prevent RBC deterioration.

The significance of this would be that L-carnitine could slow the destruction of red blood cells and increase the time between transfusions. It seems it would also be helpful in both intermedia and minor to help protect the normal RBC's their own bodies produce.

How is it supplied? From http://www.pdrhealth.com/drug_info/nmdrugprofiles/nutsupdrugs/lca_0060.shtml

L-carnitine is available in a few forms. Oral L-carnitine is available as a nutritional supplement and as a prescribed treatment for primary and secondary L-carnitine deficiencies. Intravenous L-carnitine is available as a prescription drug for the treatment of primary and secondary L-carnitine deficiencies. Acetyl-L-carnitine is available as a nutritional supplement. Propionyl-L-carnitine is available in Europe but not currently in the United States. DL-carnitine should be avoided. The available salts of L-carnitine are L-carnitine HC1, L-carnitine tartrate and L-carnitine fumarate.

Those who take supplemental L-carnitine for cardiovascular health (and most other possible indications) take 500 milligrams to 2 grams daily. The higher amounts are taken in divided doses. The doses are taken with or without food.

See Physician's Desk Reference for dosage and administration of levocarnitine in the treatment of primary and secondary L-carnitine deficiencies. See Acetyl-L-carnitine for dosage of this supplement.
HOW SUPPLIED

Capsules — 250 mg, 300 mg, 500 mg

Injection — 200 mg/mL

Liquid

Solution — 100 mg/mL

Tablets — 250 mg, 330 mg, 500 mg, 1000 mg

Wafer — 500 mg

From Natural sources  http://en.wikipedia.org/wiki/Carnitine

The highest concentrations of carnitine are found in red meat and dairy products. Other natural sources of carnitine include nuts and seeds (e.g. pumpkin, sunflower, sesame), legumes or pulses (beans, peas, lentils, peanuts), vegetables (artichokes, asparagus, beet greens, broccoli, brussels sprouts, collard greens, garlic, mustard greens, okra, parsley), fruits (apricots, bananas), cereals (buckwheat, corn, millet, oatmeal, rice bran, rye, whole wheat, wheat bran, wheat germ) and other 'health' foods (bee pollen, brewer's yeast, carob, and kale).
Product    Quantity    Carnitine
Beef Steak    3.5 oz    95 mg
Ground Beef    3.5 oz    94 mg
Pork    3.5 oz    27.7 mg
Bacon    3.5 oz    23.3 mg
Cod Fish    3.5 oz    5.6 mg
Chicken Breast    3.5 oz    3.9 mg
American Cheese    3.5 oz    3.7 mg
Ice Cream    3.5 fl oz    3.7 mg
Whole Milk    3.5 fl oz    3.3 mg
Cottage Cheese    3.5 fl oz    1.1 mg
Whole Wheat Bread    3.5 oz    .36 mg
Asparagus    3.5 oz    .195 mg
White Bread    3.5 oz    .147 mg
Macaroni    3.5 oz    .126 mg
Peanut Butter    3.5 oz    .083 mg
Rice (cooked)    3.5 oz    .0449 mg
Eggs    3.5 oz    .0121 mg
Orange Juice    3.5 fl oz    .0019 mg

Dear Manal

I hv just found out about this L Carnitine from your post after harminder mentioned it on this as well. My Son is 8 years old and is on hydrea and wheatgrass, folic acid.This year has been a bit tough on him. he was miantaining his hb avg on 7.5 but due to viral infection this year , he went down to 5. Therefore his Haemotologist is suggesting splelenectomy. I am thinking wether I shud listen to him or wait for some more time.
I would like your advice on L Carnitine, how do u take it. Are there any side effects. Can it be taken with Hydrea. Is your child also on hydrea.

Pls advise.

Tk care

puja

Hi Puja

Yes he is on hydra and he started it last May but i will be stopping it in the next 10 days as i will start a new treatment.
Concerning the spleenectomy, i think it all depends on the size of the spleen. By the way we expierenced the same situation of viral infection this year and Ahmad Hb reached 5.8 but he was able to catch up again without transfusions and i remember that the hemeo at that time increased the dose of vitamin E to help him up.So i guess you should give it more thought.

Concerning L-carnitine, Ahmad is taking it from the first day he was diagnoised. First he took it twice daily for a month then twice daily 6 days/week and then finally he is taking it twice daily 5 days in a week. They are in the form of drops and the dose is one full dropper.
L-carnitine is a natural substance that is originally found in the human cells and it has no side effect at all except that the child can be a little more active . It can be taken with hydrea

Please Puja if you have any more questions feel free to ask

Manal

I got my L-carnitene prescription through my homeopatic doctor, instead of my  heamotologists. In fact I believe that they never heard of it. So I found this topic very interesting. Am I allowed to share this whit to my Homeopatic doctor and my local GP and nurse practioner?

I use L-carnitene 2 a day 5ml I don't know if this is enough. Amongst other physical problems caused by iron-overload I also have a sever heart condition  my ferritin level is 19134 µ/L

greetz from Holland, anae

Hi Anae
Definetly you can share this study with who ever you want and although your ferritin is high but i read ( can not remember where exactly, but i wll try ) about the expierence of a lady who stopped chelating ( thal major ) when she was 21years and after 8 years, her ferritin reached 28,000

But  after suffering from iron overload problems and with a STRONG WILL she was able to lower her ferritin to 5000 in two years and she continuedl owering it down. So what i mean is that though your ferritin is high, you can lower it down. So you just start and we are all here behind you

Manal

Hi Anae,

Manal is right, please do consider this,we are all here to support you,

ZAINI.

 

lady who stopped chelating ( thal major ) when she was 21years and after 8 years, her ferritin reached 28,000
Manal

Back in 1993 my ferritin level was about 30.000 , after insisting an  implant of a Port-Cath so that the Desferal could right be adminsistered trhoug my veins, my ferritin leveles decreased to 300. I switched to an other hospit for it, cause my doctor didn't want to give me a PAC. Instead of that, he sugested to take two pumps and adminster the Desferal subcutaneously .At last I was over-dosed and became color blind, I also suffered from sever intestinal pain and was real sick of the Desferal my ferritin then was about 300 and I stil was kept on a hihg dose. So I quited the Desferal IV and went back to subcutaneous injection which gave me the same side-effects. I decided to gave desferal up intarly, quality of life is more important to me than quantity. I only want to try Exjade to say to myself and my family that I tried everything. But when I read the side-effects of Exjade, it gaves me the shivers, I am afraid that I will react to it the same I did on Desferal.

I used Desferal since 1967 intramuscalair till 1979, I switched to  subcutaneous infusions and since puberty I was on and off of the Desferal, I guess everyone has this during puberty. ;) Quite normal I guess its a lot of fuzz and it's painfull.

thank you for your reaction and that I can share the information. Of course I will give you the credits.

anae

Hi Anae,

I am so sorry to know what you have passed through especially that i think that the desferal was aggressively administred  but i think you are really such a strong person. I don't think that it  is a rule that you will suffer from the same side effects when using exjade especially if you started with al ow dose. Also there are other chelators and there are too combination therapy which can lower the risk of side effects

Please Andy advise Anae of combination therapy or exjade administration as i am not expierenced with chelators yet

Good luck
Manal

Hi Anae,

I am sure you have thought about the consequences of not chelating while making the decision to stop desferal and realize what this will mean in the long term. I will admit that I have a hard time understanding how patients can choose to not chelate, but I am not a thal and cannot walk a mile in any thal's shoes. I have lost quite a few friends because they would not chelate and just last week lost another friend from the Maldives. He was only 20 years old. The amount of thals not chelating regularly in the Maldives is alarming, especially considering that desferal is free there, and the decision to not chelate is solely based on their own view of their lives and whether or not it is worth living. I talk to thals over and over about their failure to chelate and feel it is my duty to encourage them and show them how there are always things about life that make it worth living, but again, I cannot experience life as a thal, so I will never be able to fully appreciate how they feel. Nor can I ever understand what it means to have lived your life, Anae, and why at this point you have made this choice to not chelate.

However, my advice would be to try exjade. Yes, it can have some side effects but most of these are temporary and some can be avoided by starting low dose for a couple weeks. Try 500 mg a day and see if it agrees with your system. Most doctors will tell you to take more than that but patients who have started on low dose have had some success in avoiding the rash and also the stomach discomfort, that many patients have. If after two weeks, you are doing ok, raise the dose to 750 mg per day. If you continue to tolerate it, slowly raise your dose to the maximum level, as it can't be argued that your ferritin is far too high. One other suggestion would be to try the supplement IP6 (phytic acid). It is from the B family of vitamins and is a natural iron chelator. It is a strong anti oxidant and has also been shown to have some positive effect in diabetics and is the topic of much research into cancer prevention because of its ability to inhibit the damage done to cells by iron. There are also the well known steps you can take, drinking tea with meals to help prevent iron absorption from food, and avoiding citrus with meals, as it aids iron absorption.

I hope you will consider exjade. It is helping many people. One more note, I don't know if ferrirpox is available to you but if it is, it should also be considered. We have a member of this group, Ashish Vazirani, who has recently observed his 20th anniversary of using kelfer, which is the same drug as ferrirpox.

I am sorry for your friend's death Andy ,It's very hard to hear someone loosing their lives to thalassemia,when there is chelation available.

ZAINI.