Hi There, I would like to know if anyone is using Idebenone in boys with DMD, and if so, is there positive results, or side effects etc,
Kind regards
Maxine Strydom

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Joshua weighed in at 60lbs. The dosage for his build is 900mg but we treat all minerals, vitamins or supplements as medication so we wean him up on everything he's on.

Is there anybody who put his (her) son on Idebenone and stop Steroids?Is it recommended to be just on Idebenone?

 

When Joshua started Idebenone, the doctor's intention wasn't to go off of steroids, but not increase the dosages.  Joshua is 60lbs and only on 12mg of Deflazacort.

Hi Joshua's mom!  It sounds like your boy is doing really well and that's great to hear.  Is he able to still go up steps?  My son is 6 but was diagnosed only recently.  Unfortunately he has deteriorated dramatically in the last six months.  Almost beyond recognition compared to the healthy able bodied boy he was.  His decline does seem to have slowed a bit since he has been on a high dose of Q10 (400mg).  We are going to change him to Idebenone over the next few weeks and see how he gets on.  He has never taken steroids and the clinic that looks after him is so incompetent that they haven't even offered us steroids yet!  Obviously we could contact them and ask for steroids but we are really unhappy about the osteoporosis and other side effects and have not yet decided on this issue.  Leo takes Q10, Green Tea extract, creatine, hemp protein, and a range of vitamins and minerals.  I'm really interested in finding out what other people give to their boys, and wonder what supplements Joshua takes?  I hope he carries on doing really well.

Joshua's mom said:

When Joshua started Idebenone, the doctor's intention wasn't to go off of steroids, but not increase the dosages.  Joshua is 60lbs and only on 12mg of Deflazacort.

I just increased idebenone to 300 mg/day for my 4 yo (16.5 kg) and decreased the steroids from 12 mg every day to 12 mg one day, 6 mg the next. My intention is to reduce to 12 mg every other day. My hope is that minimizes some of the side effects and allows him to grow a little. I do not see any significant change since making this change. Stairs were and still are very difficult for him and he still uses hands to push the floor when standing up. He was on steroids (12 mg/day) for 8 months and although we noticed some improvement in his jumping, he never improved on stairs or when standing up (always used hands to push the floor before and after starting steroids). It has been 2 weeks since we incresed idebenone and reduced the steroids. I do not plan to completely stop the steroids but would be happy if I can do alternate day w/o him losing strength. I will keep you posted.

Moein said:

Is there anybody who put his (her) son on Idebenone and stop Steroids?Is it recommended to be just on Idebenone?

 

@ Ruth - Joshua's daily regiment is listed on my page if you do want to take a look.

@ Ofelia - Pay really close attention to the little things he does.  When Joshua first started the Idebenone, the changes were really gradual, but now with the increases, we are more attentive and notice more of the "little" changes.

We received a letter from Joshua's neurogeneticist today:

"Joshua is now 8-1/2 years old. He has Duchenne's muscular dystrophy with a known duplication of exon 8-25.  He continues to do exceedingly well since Dr. Skalsky started him on idebenone.  He is tolerating it very well.  He also has been on deflazacort for the past few years and continues to take multiple supplements.  Since I last saw him last year, he actually stopped using a stroller even for long distances.  He does not have any major limitations to his activities.  He continues to use AFOs to prevent heel cord contractures.  He also receives PT/OT through school.  HE is also not having as much trouble with school and he is now at grade three in a great level, although he still needs a little bit of extra help with mathematics.  He is not having any active behavioral problems, although.  He has no feeding issues.  He is currently on deflazacort 12 mg daily, idebenone was increased to 500 mg daily.  He also takes carnitine 330mg t.i.d., omega 3-6-9 supplements, folic acid, ferrous gluconate, melatonin, vitamin D, B12, and calcium supplements.

On examination today, vital signs were stable.  His fundi were normal.  His cranial nerves 2 through 12 were intact.  I could no longer detect any neck flexor weakness like previously.  I did not notice any scoliotic deformities.  His range of motion is essentially full except for ankle extention.  He does have significant pseudohypertophy.  He has normal tone and bulk.  His power was essentially normal except for hip flexion which is so minimally weak that I would consider it 5-/5.  He has no signs of ataxia and has normal tandem gait.  He is able to walk on his heels and toes and his Gower sing was normal. 

Impression: An 8-1/2-year-old boy with Duchenne's muscular dystrophy with duplication of exons 8-25.

Given how well Joshua is doing, I agree with his mother that perhaps idebenone is having a significant improvement on him.  I wonder if this is the some sort of interaction between idebenone and duplication exon 8-25.  Apparently his mother has contacted patients all over the world with similar mutations and they have also started idebenone and it would be interesting if those individuals are also benefiting from the medication."

I'm sorry for the length of the letter, but felt I needed to share.

Naomi

Does someone know if it is possible to combine green tea extracts and Idebenone? I've been giving my 4 years son green tea (egcg) extracts for one year now and I'm very tempted by starting Idebenone. 

Has anyone heard about the Mustem technique (muscular stem cells transplant)? It gives hope: http://www.ncbi.nlm.nih.gov/pubmed/21924229.

Yes Galia,I have bben giving my son this combination for more than 8 months

I did hear somewhere else about the Mustem technique, on a French website i believe, looks good doesnt'it? Stem cell therapies really seem to be one of the most promising for long-term treatment/cure.  Hopefully it won't be too many years until it's available.  I think what I had read was that this particular tests on dogs meant it would now go faster for humans because the dogs md was pretty much the same than humans, as opposed to mice.  All good anyway!

Galia Ben Ishay said:

Does someone know if it is possible to combine green tea extracts and Idebenone? I've been giving my 4 years son green tea (egcg) extracts for one year now and I'm very tempted by starting Idebenone. 

Has anyone heard about the Mustem technique (muscular stem cells transplant)? It gives hope: http://www.ncbi.nlm.nih.gov/pubmed/21924229.

Just to clarify the comment regarding it being specific to the mutation.  He is a very young neurogeneticist and new to the business.  Our previous neurogeneticist had said it is for everyone, yet not everyone may have the exact same results, but will see results.  Everyone's body is different so it will be used where the body needs it most.

Joshua's mom, can you tell me how much of each of the vitamins that Joshua is taking.   also, is it safe to put them on 300 mg tid of carnitine.  I have had my son on just 300 once a day but maybe i should increase it.    My family doctor put him on a multivitamin that has folic acid in it that says it is 250% of the daily allowance.  It also has vit b12 and others.  any suggestions would be great.  my son Alex has been on the idebenone and his pulomonary function improved.  his walking for a 3 minute walk has increased a little bit, but we are going to do another walk in a month or so.  he recently had a little bit of a decline in walking, he is 11 years old, duplication of exon 2, but I think it was because his prescriptoin for steroids was changed by accident but he is now back to where he should be.  Thanks for your input.  good luck.



Joshua's mom said:

@ Ruth - Joshua's daily regiment is listed on my page if you do want to take a look.

@ Ofelia - Pay really close attention to the little things he does.  When Joshua first started the Idebenone, the changes were really gradual, but now with the increases, we are more attentive and notice more of the "little" changes.

We received a letter from Joshua's neurogeneticist today:

"Joshua is now 8-1/2 years old. He has Duchenne's muscular dystrophy with a known duplication of exon 8-25.  He continues to do exceedingly well since Dr. Skalsky started him on idebenone.  He is tolerating it very well.  He also has been on deflazacort for the past few years and continues to take multiple supplements.  Since I last saw him last year, he actually stopped using a stroller even for long distances.  He does not have any major limitations to his activities.  He continues to use AFOs to prevent heel cord contractures.  He also receives PT/OT through school.  HE is also not having as much trouble with school and he is now at grade three in a great level, although he still needs a little bit of extra help with mathematics.  He is not having any active behavioral problems, although.  He has no feeding issues.  He is currently on deflazacort 12 mg daily, idebenone was increased to 500 mg daily.  He also takes carnitine 330mg t.i.d., omega 3-6-9 supplements, folic acid, ferrous gluconate, melatonin, vitamin D, B12, and calcium supplements.

On examination today, vital signs were stable.  His fundi were normal.  His cranial nerves 2 through 12 were intact.  I could no longer detect any neck flexor weakness like previously.  I did not notice any scoliotic deformities.  His range of motion is essentially full except for ankle extention.  He does have significant pseudohypertophy.  He has normal tone and bulk.  His power was essentially normal except for hip flexion which is so minimally weak that I would consider it 5-/5.  He has no signs of ataxia and has normal tandem gait.  He is able to walk on his heels and toes and his Gower sing was normal. 

Impression: An 8-1/2-year-old boy with Duchenne's muscular dystrophy with duplication of exons 8-25.

Given how well Joshua is doing, I agree with his mother that perhaps idebenone is having a significant improvement on him.  I wonder if this is the some sort of interaction between idebenone and duplication exon 8-25.  Apparently his mother has contacted patients all over the world with similar mutations and they have also started idebenone and it would be interesting if those individuals are also benefiting from the medication."

I'm sorry for the length of the letter, but felt I needed to share.

Naomi

The list of Joshua's daily regiment is posted on my wall. This is what he recently takes:

BREAKFAST
Deflazacort (6mg) x 2
Carnitor (333mg)
Idebenone (500mg)
Melatonin (3mg)

LUNCH
Carnitor (333mg)


SUPPER
Omega 3-6-9 (1200mg)
Calcium & Magnesium
Folic Acid (1mg)
Learning Factors Advanced School-Aid
Vitamin B12
Iron - Ferrous Gluconate (300mg)
Vitamin D (1000IU)
Carnitor (333mg)
Melatonin (3mg)

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