Guest guest Posted December 3, 2004 Report Share Posted December 3, 2004 Jim, Interesting post. I understand that Idebenone has been used much more in Europe than in the US. European physicians who treat mito certainly have more clinical experience with Idebenone than US mito experts. A few years ago Idebenone was not even available in the US and I found no US based internet outlets selling it. Apparently this has changed. Interesting, too, that all but one of the references cited on the link you provided are based on studies outside the US, which again is consistent with the lack of clinical use and availability of Idebenone in the US. I'm not sure what the leading mito experts in the US currently think of Idebenone, but I do know that Dr. Naviaux included it in his list of possible treatments for mito. Maybe those who see Drs Cohen, Shoffner, Haas, Korson, etc can tell us more about how the US mito experts currently view Idebenone. Laurie, , RH, , , Malissa? Take care, Barbara > > Hello all: > > I havn't posted in awhile. I was just wondering if anyone is aware or > if they take Idebenon. It's a CoQ10 analog - a synthetic version of > CoQ10. My 18 year old son, who has mitochondrial disorder, has been > taking 400mg daily of Sam's Club brand CoQ10 and he has always had > difficulty sleeping - chronic fatigue. Usually he stays up all night > and goes to sleep during the day. His doctor, Dr Ambrus, said that > some of his patients tolerate this Idebenon better and allows them to > sleep. My son is to take 2 doses at 45mg per day. > > Any feedback is appreciated - also if you can recommend a place to > buy on line or in the Western New York area. Here is a link to some > information on Idebenon: > > http://www.smart-drugs.net/South-idebenone.htm > > Thanks > jim Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 4, 2004 Report Share Posted December 4, 2004 Hi I went through my conference materials and there was nothing in print about idebenone. It must have just been a comment made during one of the sessions. I did do some looking on line and it is in various research phases (I, II, and III) for diseases other than mito. They are things like Frederick's Ataxia and Pardinson's. I don't know if drug trials are just for drugs which will become prescriptions or not. I thought they were, but now wonder if I am correct. laurie Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 6, 2004 Report Share Posted December 6, 2004 In a message dated 12/3/2004 2:44:16 PM Eastern Standard Time, wheatchild@... writes: Maybe those who see Drs Cohen, Shoffner, Haas, Korson, etc can tell us more about how the US mito experts currently view Idebenone. Laurie, , RH, , , Malissa? HI Barbara, I am not sure what Dr Korson would say about Idebenone, but I am seeing him in two weeks so am going to ask him. I'll report back on what he says. I know it was in trials for Friedrich's Ataxia but haven't heard anything about it specifically for mito. I would love to try it though b/c I am not getting adequate blood levels of CoQ10 even on 2200 mg/day. Maybe Idebenone would work better. Malisa Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 6, 2004 Report Share Posted December 6, 2004 I saw Dr. C in August and he didn't say anything about Idebenone at all. Also, I don't remember hearing anything about it at the conference. Malilibear@... wrote: > >In a message dated 12/3/2004 2:44:16 PM Eastern Standard Time, >wheatchild@... writes: > >Maybe those >who see Drs Cohen, Shoffner, Haas, Korson, etc can tell us more >about how the US mito experts currently view Idebenone. Laurie, >, RH, , , Malissa? > > > > >HI Barbara, >I am not sure what Dr Korson would say about Idebenone, but I am seeing him >in two weeks so am going to ask him. I'll report back on what he says. I know >it was in trials for Friedrich's Ataxia but haven't heard anything about it >specifically for mito. I would love to try it though b/c I am not getting >adequate blood levels of CoQ10 even on 2200 mg/day. Maybe Idebenone would work >better. >Malisa > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 6, 2004 Report Share Posted December 6, 2004 Malisa, That sure sounds like a lot of CoQ10 to take without seeing results in your blood levels. My muscle tissue is very low in CoQ10 but my blood levels are high (after supplementing at 900mg a day). Possibly my " glitch " is getting it from the blood to the muscle. It would be interesting to see if the Idebeonone would improve your blood levels. I don't see Dr. Haas again for several months, but I'll ask his opinion of Idebenone on my next visit. Maggie > > In a message dated 12/3/2004 2:44:16 PM Eastern Standard Time, > wheatchild@n... writes: > > Maybe those > who see Drs Cohen, Shoffner, Haas, Korson, etc can tell us more > about how the US mito experts currently view Idebenone. Laurie, > , RH, , , Malissa? > > > > > HI Barbara, > I am not sure what Dr Korson would say about Idebenone, but I am seeing him > in two weeks so am going to ask him. I'll report back on what he says. I know > it was in trials for Friedrich's Ataxia but haven't heard anything about it > specifically for mito. I would love to try it though b/c I am not getting > adequate blood levels of CoQ10 even on 2200 mg/day. Maybe Idebenone would work > better. > Malisa > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 In a message dated 12/7/2004 11:13:28 AM Eastern Standard Time, wheatchild@... writes: Just curious--is the Q10 administered through your central line or do you take it orally? Just wondered if an absorption issue was involved, given your gastroparesis. Hi Barbara, Currently there isn't an IV form of CoQ10 so I have to take it through my J-tube. Absorption has been a big question mark, but they measured my Vitamin E level on supplementation and found it to be high so figured my CoQ10 is probably absorbed okay too since they are similar in their ways of absorption. My Vitamin B6 level also went through the roof on only 50 mg a day of B6, so I know I am absorbing some things really well. I was on 2200 mg of Tishcon's CoQ10 too, so you would think it would have been sky high, as well, but NOPE. Still low. Malisa Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 In a message dated 12/7/2004 11:06:29 AM Eastern Standard Time, wheatchild@... writes: Not sure if similar technology exists for CO10 and whether it is possible to measure the esterified fraction, but it might be worth asking about. Hi Barbara, Cincinatti Children's tests CoQ10 levels in a fractionated way. They measure total, reduced and oxidized and then calculate the percentage of reduced. The reduced form is the important one b/c it is the part that works as the anti-oxidant. Malisa Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 Maggie, Perhaps a pathway that is crucial in metabolizing CoQ10 is significantly blocked in your case, so the supplements aren't getting far enough in the chain of metabolism to get to muscle. Something similar happens to me re carnitine. Carnitine supplements raise total and free carnitine to normal or above normal, but the esterified fraction (actually being metabolized) remains very low. I'm told this is due to blocked pathways. Not sure if similar technology exists for CO10 and whether it is possible to measure the esterified fraction, but it might be worth asking about. Barbara > > > > In a message dated 12/3/2004 2:44:16 PM Eastern Standard Time, > > wheatchild@n... writes: > > > > Maybe those > > who see Drs Cohen, Shoffner, Haas, Korson, etc can tell us more > > about how the US mito experts currently view Idebenone. Laurie, > > , RH, , , Malissa? > > > > > > > > > > HI Barbara, > > I am not sure what Dr Korson would say about Idebenone, but I am > seeing him > > in two weeks so am going to ask him. I'll report back on what he > says. I know > > it was in trials for Friedrich's Ataxia but haven't heard > anything about it > > specifically for mito. I would love to try it though b/c I am not > getting > > adequate blood levels of CoQ10 even on 2200 mg/day. Maybe > Idebenone would work > > better. > > Malisa > > > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 Thanks, Malissa. I'd be very interested in hearing what Dr. Korson thinks. Just curious--is the Q10 administered through your central line or do you take it orally? Just wondered if an absorption issue was involved, given your gastroparesis. B > > In a message dated 12/3/2004 2:44:16 PM Eastern Standard Time, > wheatchild@n... writes: > > Maybe those > who see Drs Cohen, Shoffner, Haas, Korson, etc can tell us more > about how the US mito experts currently view Idebenone. Laurie, > , RH, , , Malissa? > > > > > HI Barbara, > I am not sure what Dr Korson would say about Idebenone, but I am seeing him > in two weeks so am going to ask him. I'll report back on what he says. I know > it was in trials for Friedrich's Ataxia but haven't heard anything about it > specifically for mito. I would love to try it though b/c I am not getting > adequate blood levels of CoQ10 even on 2200 mg/day. Maybe Idebenone would work > better. > Malisa > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 Interesting, thanks. On my tube-feeding group, there has been some discussion of reduced absorption through a j-tube, so I have wondered about that issue too. Hope you can get some answers. Barbara > > In a message dated 12/7/2004 11:13:28 AM Eastern Standard Time, > wheatchild@n... writes: > > Just curious--is the Q10 administered through your central line or > do you take it orally? Just wondered if an absorption issue was > involved, given your gastroparesis. > > > > > > Hi Barbara, > Currently there isn't an IV form of CoQ10 so I have to take it through my > J-tube. Absorption has been a big question mark, but they measured my Vitamin E > level on supplementation and found it to be high so figured my CoQ10 is > probably absorbed okay too since they are similar in their ways of absorption. My > Vitamin B6 level also went through the roof on only 50 mg a day of B6, so I > know I am absorbing some things really well. > I was on 2200 mg of Tishcon's CoQ10 too, so you would think it would have > been sky high, as well, but NOPE. Still low. > Malisa > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 Ah, very glad to hear this! Thanks. Barbara > > In a message dated 12/7/2004 11:06:29 AM Eastern Standard Time, > wheatchild@n... writes: > > Not sure if similar > technology exists for CO10 and whether it is possible to measure the > esterified fraction, but it might be worth asking about. > > > > > Hi Barbara, > Cincinatti Children's tests CoQ10 levels in a fractionated way. They measure > total, reduced and oxidized and then calculate the percentage of reduced. > The reduced form is the important one b/c it is the part that works as the > anti-oxidant. > Malisa > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 Info for the lab. The neuropharmacology and Clinical Laboratories of the Children's Hospital Medical Center in Cincinnati have recently joined to develop and offer a new test profile for assessing coenzyme Q10 status. This unique profile includes the concentrations of total, oxidized, and reduced forms of this essential cofactor, plus the total Q10 concentration indexed with total cholesterol. Coenzyme Q10 (also called CoQ or ubiquinone) is an endogenous cofactor which is essential for mitochondrial function and cellular energy production. It also serves as an important antioxidant, protecting membranes from oxidative injury, and conserving and regenerating vitamin E. A new method for measuring the reduced (or antioxidant) and oxidized forms of coenzyme Q10 has been developed in the CHMC neuropharmacology laboratory (see Clinical Chemistry 2001;47(2)256-265). Because coenzyme Q10 may be depleted in certain acute and chromic disease states, the ratio of reduced to total Q10 concentration is also a useful biomarker of oxidative stress. Patients who have coenzyme Q10 deficiency also have increased risk of mitochondrial and cellular injury from excess production of free radicals. Certain drugs and malnourished states may also tend to deplete the body's coenzyme Q10 reserves. Measurement of coenzyme Q10 concentrations in plasma can assist clinicians in detecting coenzyme Q10 deficiency states, and serve as a guide for dosing when oral supplementation is indicated. Because significant inter-product variability in the absorption and bioavailability of coenzyme Q10 has been reported with over-the-counter (OTC) Q10 preparations, the Coenzyme Q10 Profile will also assist in verifying the extent of absorption of Q10 from those products. Included in the Coenzyme Q10 Profile are the oxidized, reduced, and total Q10 concentrations. Also the fraction of the reduced Q10 in the total concentration, and the Q10: Total Cholesterol Index (or QCI0) are reported. Reference intervals and comments are also provided with each report. Potential indications for testing coenzyme Q10 status include: - Suspected coenzyme Q10 deficiency secondary to inheritance (rare) or other mitochondrial disease - Cardiomyopathy - Neurodegenerative diseases, e.g. Parkinson's, Alzheimer's, muscular dystrophy, ALS - Metabolic disease - Malnutrition - Presurgical screening for patients who have pre-existing coenzyme Q10 deficiency or who may have increased risk of ischemia-reperfusion injury - Prior treatment with HMG-CoA reductase inhibitors ( " statin " drugs) or other drugs which may lower coenzyme Q10 levels - Gastrointestinal malabsorption - Assessment of coenzyme Q10 dosing adequacy The Coenzyme Q10 Profile requires a minimum of 2 mL of heparinized blood (green to vacutainer tube), which must be placed on ice immediately and sent to the CHMC Clinical Laboratory for next morning delivery. Please call the CHMC Clinical Laboratory Outreach Director at in advance for billing, ordering, shipping, and specimen collection information, or Dr. Miles, Pharm.D., CHMC Division of Child Neurology at . V. Miles, PharmD Prof. Of Clinical Pediatrics and Neurology Director, Clinical Neuropharmacology Laboratory Childrenc's Hospital Medical Center 3333 Burnet Ave. Cincinnati, OH 45229 Phone: 513/636-7871 Fax: 513/636-3980 wheatchild2 wrote: >Ah, very glad to hear this! Thanks. > >Barbara > > > > >> >>In a message dated 12/7/2004 11:06:29 AM Eastern Standard Time, >>wheatchild@n... writes: >> >>Not sure if similar >>technology exists for CO10 and whether it is possible to measure >> >> >the > > >>esterified fraction, but it might be worth asking about. >> >> >> >> >>Hi Barbara, >>Cincinatti Children's tests CoQ10 levels in a fractionated way. >> >> >They measure > > >>total, reduced and oxidized and then calculate the percentage of >> >> >reduced. > > >>The reduced form is the important one b/c it is the part that >> >> >works as the > > >>anti-oxidant. >>Malisa >> >> >> Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 This is great, . Thank you thank you! B > > > > > >> > >>In a message dated 12/7/2004 11:06:29 AM Eastern Standard Time, > >>wheatchild@n... writes: > >> > >>Not sure if similar > >>technology exists for CO10 and whether it is possible to measure > >> > >> > >the > > > > > >>esterified fraction, but it might be worth asking about. > >> > >> > >> > >> > >>Hi Barbara, > >>Cincinatti Children's tests CoQ10 levels in a fractionated way. > >> > >> > >They measure > > > > > >>total, reduced and oxidized and then calculate the percentage of > >> > >> > >reduced. > > > > > >>The reduced form is the important one b/c it is the part that > >> > >> > >works as the > > > > > >>anti-oxidant. > >>Malisa > >> > >> > >> Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 You are welcome Barbara! wheatchild2 wrote: >This is great, . Thank you thank you! > >B > > >>> >>> >>> >>> >>>>In a message dated 12/7/2004 11:06:29 AM Eastern Standard Time, >>>>wheatchild@n... writes: >>>> >>>>Not sure if similar >>>>technology exists for CO10 and whether it is possible to >>>> >>>> >measure > > >>>> >>>> >>>> >>>> >>>the >>> >>> >>> >>> >>>>esterified fraction, but it might be worth asking about. >>>> >>>> >>>> >>>> >>>>Hi Barbara, >>>>Cincinatti Children's tests CoQ10 levels in a fractionated way. >>>> >>>> >>>> >>>> >>>They measure >>> >>> >>> >>> >>>>total, reduced and oxidized and then calculate the percentage >>>> >>>> >of > > >>>> >>>> >>>> >>>> >>>reduced. >>> >>> >>> >>> >>>>The reduced form is the important one b/c it is the part that >>>> >>>> >>>> >>>> >>>works as the >>> >>> >>> >>> >>>>anti-oxidant. >>>>Malisa >>>> >>>> >>>> Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 Hi all, <>Wow, something I can actually add to the conversation!! Ha ha ha! With all you mito experts out there, I feel I have so little to offer most times. But here is my 2 cents worth. I e-mailed our mito doc about this product and would love to get the other experts opinions. Here is her response. <> My ?: I have seen articles and research on Idebenone as an addition to CoQ10 usage and as a replacement for CoQ10. Is this a safe product? <> Answer: pretty safe, but no studies done to assess this My ?: <>Would it be helpful as an addition or replacement to the current cocktail you have prescribed for me? Answer: Cannot get it in the U.S. The best grade is made by a Japanese company and is only available through clinical trials. There is idebenone available over the internet, but it is not the pure product. <> My ?: Is there risk of the CoQ10 creating problems such as free radicals with/or decreased oxygenation. Answer: Not likely <> My ?:<> Is this a product that could be added to my current compound? Answer: not available in the U.S except on clinical trials So apparently the internet products are not a pure form of this supplement and I don't know how safe they would be or how helpful they would be. I think it is great that there are more products being researched to help treat this disease. Hope this helps, AnnMarie L. - Complex IV Married to my best friend Rick, Mom of (15), (12), Cassandra (6) and Joe (5 on Dec 31st !!) - complex IV, lactic acidosis, CP, tethered chord syndrome, dysautonomia, and a smile that never quits :-) Malilibear@... wrote: > > In a message dated 12/3/2004 2:44:16 PM Eastern Standard Time, > wheatchild@... writes: > > Maybe those > who see Drs Cohen, Shoffner, Haas, Korson, etc can tell us more > about how the US mito experts currently view Idebenone. Laurie, > , RH, , , Malissa? > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 7, 2004 Report Share Posted December 7, 2004 Ann-Marie Thanks for asking your doc. and sharing her answers. laurie > > Reply-To: > Date: Tue, 07 Dec 2004 22:39:26 -0500 > To: > Subject: Re: Idebenone > > Hi all, > > <>Wow, something I can actually add to the conversation!! Ha ha ha! > With all you mito experts out there, I feel I have so little to offer most > times. But here is my 2 cents worth. > I e-mailed our mito doc about this product and would love to get > the other experts opinions. > Here is her response. > <> > My ?: I have seen articles and research on Idebenone as an addition to > CoQ10 usage and as a replacement for CoQ10. Is this a safe product? > <> > Answer: pretty safe, but no studies done to assess this > > My ?: <>Would it be helpful as an addition or replacement to the > current cocktail > you have prescribed for me? > > Answer: Cannot get it in the U.S. The best grade is made by a Japanese > company and is only available through clinical trials. There is > idebenone available over the internet, but it is not the pure product. > <> > My ?: Is there risk of the CoQ10 creating problems such as free radicals > with/or decreased oxygenation. > > Answer: Not likely > <> > My ?:<> Is this a product that could be added to my current compound? > > Answer: not available in the U.S except on clinical trials > > So apparently the internet products are not a pure form of > this supplement and I don't know how safe they would be or how > helpful they would be. I think it is great that there are more > products being researched to help treat this disease. > Hope this helps, > > AnnMarie L. - Complex IV > Married to my best friend Rick, > Mom of (15), (12), Cassandra (6) and > Joe (5 on Dec 31st !!) - complex IV, lactic acidosis, CP, > tethered chord syndrome, dysautonomia, and > a smile that never quits :-) > > > Malilibear@... wrote: > >> >> In a message dated 12/3/2004 2:44:16 PM Eastern Standard Time, >> wheatchild@... writes: >> >> Maybe those >> who see Drs Cohen, Shoffner, Haas, Korson, etc can tell us more >> about how the US mito experts currently view Idebenone. Laurie, >> , RH, , , Malissa? >> > > > Quote Link to comment Share on other sites More sharing options...
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