Hi,
I'm new to this board, but not new to CPEO. I'm not really up to date on what's going on with CPEO research, but I'm guessing nothing.
I have the eyelid ptosis in my right eye only, but the paralysis is in both eyes and getting worse all the time.
I had the frontalis sling surgery in 2007 and have been depressed about my situation ever since. The surgery totally screwed up my eyelid. The skin got all shiny and thick and never returned to normal. The worst part is that the doctors don't seem to care. I really need to get back to the eye clinic because the sling needs to be adjusted (lifted further), but after my last experience I am afraid to go back. I just want to look semi-normal. I am very frustrated.
Has anybody had this experience? Any ideas? I have wondered if my eyelid is just a mass of scar tissue and whether I should try some of that old-scar-healing stuff they advertise. Then again, I'm not sure I want to put that stuff so near my eyes.
I haven't read many of the other posts yet. Can anybody give me a brief synopsis of where the medical community is on treating this?
Thanks.
Sorry to hear about your messed-up lift. I too have more severe ptosis in the right eye. As to what to do and who to see I'm at a lost. I think I'm not going back to my neuro-ophthalmologist in March. I'm sure he's great for other problems, but not mine. I'm getting a lot more light in my right eye, so I think something internal is going on. I told him about it and he wasn't interested. I asked about a little hit of botulism in my right forehead just the relax the muscle, make it look normal, and he was totally against. Then the next year he suggested a little hit of botulism in my right eye lid to kept it closed to stop the double vision. Can you imagine how that would look!!! Don't they think we exist in a world. The next year I told him what he suggested both years and he gave a shy grin, he knew it was just silly. He's getting close to retirement. Maybe I should see a young eager neuro-opht, but they can be dangerous too. Two weeks before Christmas I saw a new young dermatologist. I had chapped lips, she directed her resident to take a little shave. I say, I had a few get togethers, not a problem a little red for a couple of days, that's all. Well, let me tell you, a chunk of the lip was gone, very unsightly and sore. I hope the PDT takes care of the scar.
On another note I have been assessed by two muscle biopsies, actually three but one doesn't count, with a positive mtDNA deletion/duplication, complex 1 deficiency, ragged red fibers and so on. But sporadic inheritance didn't address my autosomal dominant family's brain-muscle disorder. I have more then mild peripheral neuropathy, I have something central going on affecting voice, breathing, teeth, pupil and so on. I'm looking into one of the ad inherited ataxia disorders now. My father has CMT with ataxia. The SCAs also have CPEO, ptosis, neuropathy, and most of the other things found in my family. So many other diseases are finding positive mito mutations, including cancers, that it's not a final diagnose, at least not for me. In a multi-symptom disorder it's hard to decided what specialist to go to. I asked my neurologist about it and he suggested another type of neurologist. So, I'm on my way.
diane-I will try to find the number for the support group, today, I'm snowed in.
Bye,
Sandy
Last edited by moderator2; 01-28-2009 at 07:11 AM.
Reason: disallowed use
MissyFaye- Sorry to hear that you are having such problems with the sling surgery. There have been a few people on this message board who have had/having problems with theirs as well. I guess I'm one of the successes. There is only so much the surgery can do though and I think sometime people may be dissapointed with the results because their expectations were too high.
Our ever changing health condition along with the progression of the CPEO may cause some problems down the road. All the doctors I went to were very careful to list both the pros and cons.. Because there were cons that's why I liked the frontalis sling surgery. I could reverse the procedure if it didn't agree with my own particular set of health issues.
I investigated for a full year prior to the surgery. I got three opinions and would have continued if I wasn't certain of what or who to go with. I urge everyone in the group to be their own health advocate. Question the doctors and if you don't get a helpful informative response then move on to the next. I think you also need to think of smart thought out questions to ask.
Do you have exterior scar tissue? The frontalis surgery is for the most part unseen. The only scars you should see should be near you eye brow? I would also talk to your doctor about your tear production... Is there a reason they set your lid at it's current level? The doctor should take your tear production into consideration when even performing this procedute. I have one eye slightly lower than the other. Not happy with it but my doctor forwarned me going in that he was doing that because my eye was weaker than the other in all aspects. To lift the lid any higher would cause problems. I still went ahead with the surgery and I hate to think of how I'd look without it. Anyway, maybe this will help you troubleshoot.
Sandy- you sound like you've got a lot of other medical problems besides the CPEO.. you wrote that you asked about botox inj for your forehead. Think about this.....Do you think any kind of botox injection would be wise when the problem with CPEO is muscle paralysis? I am glad your doctor was totally against it. You have the sling? So the muscle probably looks likes it's tight because it is in fact lifting your eyelid up. It's the brow muscle that the sling is attached to lift your lid and it's doing it's job. I'm amazed that this doctor even joked about botox in the eyelid. I hope he was joking... If not... please seek another doctor who will take every complaint seriously.
It’s been a while since I’ve been here. I’m really worried at the moment because our youngest daughter will be tested starting next week. I will join her in hospital. We have to stay there a week.
She will have emg, mri, eeg, lactate, and blood dna tests. For the moment fortunately no biopsy yet.
It will be very difficult, because she has the understanding of a 2year old(she’s 5) she is also verbally behind.
I am rather handicapped (wheelchair, low vision, a.s.o.)and no language wonder of the polish language either. So we’ll be a special mother and daughter team!
As far as I’m concerned, they found out that not only my vision is diminished because of the optic atrophy but also the width of my vision( I don’t know the word in English). On top of the ptosis and difficulty of moving my eyes.
I never realized so many things could go wrong with your eyes. My youngest daughter already has a slight ptosis.
Some years ago I was examined by a prof in Holland. He has now written me that he thinks I may have kss after all. I was never tested for kss, because this normally has a much earlier onset.
It’s been a while since I’ve been here. I’m really worried at the moment because our youngest daughter will be tested starting next week. I will join her in hospital. We have to stay there a week.
She will have emg, mri, eeg, lactate, and blood dna tests. For the moment fortunately no biopsy yet.
It will be very difficult, because she has the understanding of a 2year old(she’s 5) she is also verbally behind.
I am rather handicapped (wheelchair, low vision, a.s.o.)and no language wonder of the polish language either. So we’ll be a special mother and daughter team!
As far as I’m concerned, they found out that not only my vision is diminished because of the optic atrophy but also the width of my vision( I don’t know the word in English). On top of the ptosis and difficulty of moving my eyes.
I never realized so many things could go wrong with your eyes. My youngest daughter already has a slight ptosis.
Some years ago I was examined by a prof in Holland. He has now written me that he thinks I may have kss after all. I was never tested for kss, because this normally has a much earlier onset.
Bye, Zofia
Zofia,
You definitely are one brave mother/daughter team! Hope that things turn out better for your kids- hoping that the medical community finds some form of treatment/ cure for this horrible disease. You mentioned that you might have kss- have u had your heart checked out? Many people with mitochondrial myopathies can have conduction defects in their hearts- which can be easily corrected with a pacemaker- so it might be something u may want to discuss with your docs. Let us know how everything turns out.
i just started taking some coenzymeq10 for my cpeo. I can't really say that it has helped any- but i just keep taking it probably more for psychological reasons.
Was wondering if there was any other supplement that anyone was taking (besides the basic vitamins and such) that has helped at all.
My new neuroglogist prescribed a mitochondrial cocktail: CoQ10 200mg, one tablet 3x a day (600 mg.day); Carnitine 330 mg up to 3 tablets 3x a day, 990 to 2700 mg/day; creatine 5g poweder, Take daily with juice; Mastinon tablets 60 mg, one tablet 3x a day(180 mg/day). He suggested that I start with one of these nutraceuticals so I started taking Coq10 in December. I have just started to feel some results. I have a lot more energy. I am careful not to take it late in the evening because it causes insomnia for me. I am not sure if it translates into anything related to my CPEO but I do feel better overall. I was taking over the counter CoQ 10 but after reading a posting to this site I asked my doctor to write the prescription for Cyto-Q,ubiquinol,the nano liposomal liquid form. I am to start that this week, and was told that my insurance (Medicare and Tricare for life) does cover it. I am not sure if the Carnitine, Creatine and Mestinon is covered by insurance and I am not sure if I will start on any of them in the future.. Many thanks for the helpful input.
Sorry I didn’t write sooner, but we just got out yesterday. Our bad dream has come true: they found our sweet little girl has a brain damage caused by insufficient oxygen and she also has a too high lactate level.
We expected bad news, but still it was like a bomb dropped on us. Next week hopefully we’ll get more information from the Prof. they also are considering the possibility of k.s.s in our daughter.
By the way, I have had 2 heartblocks half a year ago and have had a pacemaker implanted already..
My new neuroglogist prescribed a mitochondrial cocktail: CoQ10 200mg, one tablet 3x a day (600 mg.day); Carnitine 330 mg up to 3 tablets 3x a day, 990 to 2700 mg/day; creatine 5g poweder, Take daily with juice; Mastinon tablets 60 mg, one tablet 3x a day(180 mg/day).
Hello Diana,
That’s interesting that your doctor subscribes mestinon. At first my doctors thought I had a myasthenia.
They subscribed at first mestinon, then ubretid en then I had to take injections with nivalin.
These medicines are all acetylcholine stimulating medicine. I had the idea that it was (at least for I while) beneficial for me.
When they quitted the idea of myasthenia, they stopped the subscription of these medicine too.
How do they explain the benefit from mestinon to you?
I had the same question for u Diana as Zofia. I have never tried mestinon but do u think it provides any symptomatic relief- specifically with the ptosis? Is there any harm to using it?
Zofia: I am sorry to hear about your daughter. Hopefully the doctors can figure out some intervention to prevent further damage- but sadly as you know there is little consolation for this disease. But your daughter will have you as a support system and can know that u will understand what she goes thru.
Quote:
Originally Posted by zofia
Hello Diana,
That’s interesting that your doctor subscribes mestinon. At first my doctors thought I had a myasthenia.
They subscribed at first mestinon, then ubretid en then I had to take injections with nivalin.
These medicines are all acetylcholine stimulating medicine. I had the idea that it was (at least for I while) beneficial for me.
When they quitted the idea of myasthenia, they stopped the subscription of these medicine too.
How do they explain the benefit from mestinon to you?
Di 88 and Zofia, I was not given any significant information about the mitochondrial cocktail components, just that this was what was being recommended in the literature. I did a bit of a literature search myself and could only find documentation for the use of CoQ 10, and I found out about Co-Quil. The doctor had told me to start off with just one of the nutraceuticals, so I opted to start with over the counter CoQ10 which was costing me between $30 and $90/mo. When I read on this thread that the specific formula CoQuil might be covered by insurance, I asked the doctor to prescribe that. It took a few months, but he did send me the prescription. It took my drug store almost two weeks to get it, but I started on that nano, liposomal, more biologically available liquid formula today, 80 mg 3xday. The cost is $ 210/month but my insurance is covering it so thankfully my cost is only the $3.00 co-pay for a months supply.I do not plan to take the other neutraceuticals until I know more about their side effects and cost. The OTC CoQ10 has given me more energy, but it took several months for me to feel that. I am not sure of any effect on my ptosis at this point. I was taking 200 mg 3 x a day.
Regarding the "Mito Coctail" or what some would call "neutroceuticals" mix is explained to some extent are the following website: [url]http://www.mitoaction.org/blog/mito-cocktail[/url]
UMDF also has additional information on the mito coctail at [url]www.umdf.org[/url]
Regarding Mesitnon - I looked into this a few years ago. Sounded good to me even though it was for MG because it's supposed to increase tear production and better eye movement. Unfortunately it has a lot of side effects not good for those of us with Mito problems, first and foremost being muscle cramps and muscle weakness. I could not locate any studies that this drug was proven effective in CPEO or with anyone suffering from Mito disorders. Seemed like there were far more negatives that would outweigh any possible positive.
I've been using mestinon for some months and had lots of cramps and myoclonic jerks. also very bad diarrea.
With ubretid and later nivalin this was better, but i also felt that the effect was getting less after a while.
I thought it helped me because of a possible strong placebo effect. But maybe it helped a bit with the polyneuropathy I also have apart from the musscle weakness.
My neurologist wants me to get a muscle biopsy to confirm my diagnosis. But I feel that there is a large chance that the muscle biopsy (from my arm) wont' show anything.
Did any of your muscle biopsy results change your treatment plans? Like did it show some major vitamin/ co-factor deficiency or something that could be repleted. Or did it show another problem that could be corrected? Or did it just confirm the diagnosis.
I don't want to undergo another unnecessary procedure at this point if it is not going to change anything.
I had a muscle biopsy a while ago and it merely confirmed that I had CPEO.... kind of wish that I didn't have it done (it's a little scar on my arm that's kind of noticeable - I had knee surgery when I was 16 and have a big scar on my leg that I am SO self-conscious about, didn't want yet another scar too!) but at the same time, it was I guess nice to have a definitive diagnosis...
I had a muscle biopsy a while ago and it merely confirmed that I had CPEO.... kind of wish that I didn't have it done (it's a little scar on my arm that's kind of noticeable - I had knee surgery when I was 16 and have a big scar on my leg that I am SO self-conscious about, didn't want yet another scar too!) but at the same time, it was I guess nice to have a definitive diagnosis...
Hi Jaylite,
thanks for your response. Did you have any symptoms in your arm when you had the biopsy? My symptoms are mostly in my eyes and I feel that even though it would be nice to have the diagnosis confirmed, there is a large chance that the arm biopsy will be a false negative. And just to go thru another procedure that won't change anything is not so palatable at this time. Thanks for your input again!
I’ve had a muscle biopsy from my thigh. It only showed swollen mitochondria, but then they also messed up. They only did the staining things. Never looked if there were ragged red fibres, never looked at my atp levels and they finally lost the specimen..
They suggested our daughter could have a biopsy too, but that is one of the things I won’t have her go through for the moment. So we decided I’ll have another biopsy this autumn.
Yes, surprisingly they were able to confirm a diagnosis from the ragged red fibers that appeared in the muscle from my upper arm (bicep) and I feel like my symptoms are most apparent in my eyes/facial muscles. I think that once we have this disease, ALL the mitochondria in our bodies are affected, just to lesser degrees. A doctor told me once that it is only more apparent in our facial muscles because those muscles are thinner than the others in our bodies - specifically those in our outer limbs (arms, legs, etc) So even if we don't really "feel" or notice any weakness in those muscles, they are also affected. I think that's where the "exercise intolerance" bit comes into play. I feel fatigued VERY quickly every time I run or exercise or dance. And surprisingly, when I do work out or exercise, my muscles respond very quickly and get toned right away, so I guess that's a good thing. Though I can't think of ANYTHING good about CPEO. It stinks. And I hate it!! Anyway - good luck! :-)
thanks for your response. Did you have any symptoms in your arm when you had the biopsy? My symptoms are mostly in my eyes and I feel that even though it would be nice to have the diagnosis confirmed, there is a large chance that the arm biopsy will be a false negative. And just to go thru another procedure that won't change anything is not so palatable at this time. Thanks for your input again!
Hi Di,
Yes, surprisingly they were able to confirm a diagnosis from the ragged red fibers that appeared in the muscle from my upper arm (bicep) and I feel like my symptoms are most apparent in my eyes/facial muscles. I think that once we have this disease, ALL the mitochondria in our bodies are affected, just to lesser degrees. A doctor told me once that it is only more apparent in our facial muscles because those muscles are thinner than the others in our bodies - specifically those in our outer limbs (arms, legs, etc) So even if we don't really "feel" or notice any weakness in those muscles, they are also affected. I think that's where the "exercise intolerance" bit comes into play. I feel fatigued VERY quickly every time I run or exercise or dance. And surprisingly, when I do work out or exercise, my muscles respond very quickly and get toned right away, so I guess that's a good thing. Though I can't think of ANYTHING good about CPEO. It stinks. And I hate it!! Anyway - good luck! :-)
