Questions From the Fall 2004 Support Group Meeting
Respondents:
Dr. Martin Kazdan
Dr. Yasser Khan
Dr. Stephen Kraft
John Walmsley, PhD.
Questions:
- Does the limited myectomy help with the involuntary facial spasms?
- I understand that the eye would be fine indirectly, right; but the involuntary muscle spasms in the face, is there anything else that can help that?
- But is that not just temporary; the Botox is just temporary?
- This question is for John. I found it very interesting that he would compile a graph on his condition between Botox treatments. ...if you could just brief us a little bit on how you went about doing that graph it would certainly help I think.
- Yes, that's helpful. I did the same thing on a scale of 1 to 10 similar to that. Did you do it a particular time every day?
- It's a great tool when you go to the doctor because I found that it depends on how you feel that day is how the treatment is planned rather than the whole time in between.
- Certainly it's a great thing, and, it must be an advantage to the doctor giving you the Botox because he must get a different story from everybody he's treating in that way.
- Is there any workable graph charts available that John filled out that he can give us to eventually take to a physician and show him?
- Maybe we can get a copy of it?
- I've had blepharospasm about a little over six years... I saw in the latest Support Group Newsletter that Dr. Kazdan had noticed that, for some unknown reason, some of these patients appear to get over the disease for a short period of time. Now I've been in remission about a year and a half. I was just wondered what you meant for a short period of time.
- Well I read somewhere that just 1% go into remission. I guess that's general for dystonia.
- I wear sunglasses a lot during the day. They seem to bother me more even in the sun than if I wear nothing. And I wonder is that unusual with blepharospasm? Also my problem is that when I go out on, let's say an overcast day, I can barely see at all and it doesn't seem to cut the glare. It seems to go right through the glass for me. And, these are supposed to be antiglare and UV and everything, they're a prescription.
- Are there any doctors or optometrists that specialize in helping you with these glasses; like going to them and getting help with them or do you just go to any optometrist?
- A few years back when I was in England I purchased a pair of sunglasses for my grandson. And at that time they were very expensive and apparently the lenses were treated with something that they used to walk on the moon. I wonder if there's any way to research that, if that would help?
- Are they familiar with the coating used on space helmets?
- No, these weren't like that.
- I don't know if this question was asked before but I have Cranial Dystonia (Meige Syndrome) along with the blepharospasm and unfortunately every time I get my injections, I would say 90-95% of the time, I have ptosis and this time it was so bad that I was blind for two weeks in one eye...
- I want to ask one more question. When you start with this, do you start with certain sites to begin with or it's different in the beginning?
- John, I just wondered - I was curious about why you wear the mask to bed?
- My family have seizures, what's it called? Epileptic seizures. Is this related to what I have (BEB)? The seizure is spasms and everything else. Is there anything at all related to that? Is there the possibility for me, because of what I have, to eventually get what they have?
- I find that when I'm wearing glasses or sometimes concentrate while reading there is a lot of spasms around the nose and brow area.
Q. – Does the limited myectomy help with the involuntary facial spasms?
Dr. Khan:It does not per se. Only about 20% of patients have only blepharospasm: only eyelid spasms alone. There is often other spasms going on, but the eyelid spasms are what symptomatically bother the patients the most: from what patients tell us. So basically no, I mean directly it should be no. But indirectly and generally speaking I think it decreases the aggravating stimuli that sort of makes things go out control. So, I think in that sense it indirectly helps but, not directly: no.
Q. – I understand that the eye would be fine indirectly, right; but the involuntary muscle spasms in the face, is there anything else that can help that?
Dr. Khan:It depends where they are, what's causing them. Certainly there are BOTOX® facial injections.
Q. – But is that not just temporary; the Botox is just temporary?
Dr. Khan:Surgically speaking there is not: it depends on where the problem is. Some people have tried but I have no experience with this. The facial plastic surgeons or ENT surgeons do this. It's a Gortex implant or facial sling, done primarily for facial nerve palsy, where you have a tube (implant) that is attached to the cheek bone. But I have no experience with that. And I don't think anybody has really looked at that as helping facial spasms with regards to blepharospasm. So I can't answer that. Perhaps one of the other doctors can answer?
Dr. Kraft:There is a treatment, it wasn't discussed at length today, and, again it's not a permanent treatment, but oral medication can supplement the help that Botox gives to the eyelid and secondarily there are some people who are exquisitely sensitive in a positive way to oral medication. The problem with oral medications and, we have discussed this in the past, and because there are many different subtypes of blepharospasm, have to be a tailored treatment to the individual.
We know now that the eyelid reflex really comes from about four or five different parts of the brain and pathways and they all can be treated with Botox and most people do respond but, there are nuances of other areas of the face that can be involved. With some people, their type of blepharospasm has a biochemistry that is very responsive to oral medication, and usually it is a neurologist that would have to treat them because there are many different drugs available. It's a bit of a trial and error, unfortunately, but sometimes a low dose of one of those medications will suddenly click in and be the one that works for them. And working with the Botox for the face, the oral medication can sometimes be very, very helpful. Again, that's not a permanent fix but it can be of a big help for the lower face. And hopefully with the Botox working, it removes some of the trigger that can cause the facial spasm, and, at the same time it may reduce the oral dose that you need to get that lower facial effect; so the two can work together and be positive.
Q. – This question is for John. I found it very interesting that he would compile a graph on his condition between Botox treatments. I tried to do the same thing myself, I go to Dr. Ostreicher, and I find that it's very difficult to prepare that graph. I did it for two months at one time. And what it would help with, I'm sure, is when you go to the doctor; being able to have some concise way of telling him how long the Botox lasts and the variations over the different time period. So, John, if you could just brief us a little bit on how you went about doing that graph it would certainly help I think.
Dr. John WalmsleyWell just at the end of ever day I sort of thought what kind of day was this? Good or very good or not so good and gave a number to that. And I used just 6, 7, 8, 9, 10 and later I added 9.5 and 9.9 just to get it a bit more refinement at the top. And this can be done by hand. You can use graph paper and plot each day. Or, what I did is use the computer and put the numbers in the computer and how to plot them. So I just used the computer rather than having to do it manually. Does that answer your question?
Q. – Yes, that's helpful. I did the same thing on a scale of 1 to 10 similar to that. Did you do it a particular time every day?
Dr. John WalmsleyJust at the end of the day. Usually I kept a piece of paper in my pocket and wrote it down. After I finished one sheet of paper I entered it into the computer and started another sheet.
Q. – It's a great tool when you go to the doctor because I found that it depends on how you feel that day is how the treatment is planned rather than the whole time in between.
Dr. John WalmsleySometimes it's hard to remember what your response was. It was what your response was like two months ago when you see the doctor every time.
Q. – Certainly it's a great thing, and, it must be an advantage to the doctor giving you the Botox because he must get a different story from everybody he's treating in that way.
Dr. KraftI think that is what I was alluding to earlier and, now you know what I'm talking about. If you have informed patients who can articulate exactly where they are at, it really makes the partnership that much more valuable and useful. I felt like a member of the Canadian Diving Team. I knew John was about to come back and I wondered if I had scored a 6.9. When he was down about 6.5, I said whoa, no gold medals in this round, I can tell you that much. But it was very helpful and you can see just where it sort of puts you. I was glad at least I never got below 6. But we wanted to get him up to the 8's and 9's, and, it was not just a matter of trying to vary the dosage levels but also the sites. I think we changed the sites a little bit here and there. And it's a partnership as I say. Every patient responds differently. You can do well for a while and then having those numbers helps. Also the e-mails came from him too. I'm at about a 7.2, or I'm about down to 6.5, I think it's time. And what do we do next time?
So, if you can put it numerically, and that is a very unusual situation, but if you can do it and do it for yourself you may be surprised at how well you're doing versus comparing you one year to three years down the road. Sometimes it's hard to remember exactly how you are doing unless you keep a day-by-day diary. Another example is that I also do that for my eye muscle patients. I tell the parents, how often does the child's eye turn? Keep a running log and that way they can see if they're improving on treatments or not. For the doctor it is very helpful if you have that. Bob Campbell is actually the first one of my patients who developed a scale for himself, and, it is very helpful when you have that.
Q. – Is there any workable graph charts available that John filled out that he can give us to eventually take to a physician and show him?
Dr. KraftYou just have to take some graph paper and make a chart and that and just do it yourself day by day. You can make it yourself with graft paper. There are no, as far as I know John, there are no commercially available ones for patients to use but you can make one up yourself just on some graft paper as Dr. Walmsley has done.
Q. – Maybe we can get a copy of it?
Dr. John WalmsleyI did mine on the computer but you could go to a stationery store and ask for some graph paper, just spacing a quarter inch or something and just use that to plot it by hand.
Q. – I've had blepharospasm about a little over six years, I guess. I just wanted to go on in this area so I give some of my fellow BEBs a chance to think about hope. Because, I didn't have much hope. But there is hope. If I can go in remission I'm sure anybody else can. Then I saw in the latest Support Group Newsletter that Dr. Kazdan had noticed that, for some unknown reason, some of these patients appear to get over the disease for a short period of time. Now I've been in remission about a year and a half. I was just wondered what you meant for a short period of time.
Dr. KazdanNo I haven't had many who have gone into remission. I find it much more common in hemifacial people and I don't know why it happens that way. I don't know. Do you have a theory?
Q. – Well I read somewhere that just 1% go into remission. I guess that's general for dystonia.
Dr. KazdanI suppose that could happen with any physical problem, in many diseases that seem to be continuous and long lasting. There are some people who are lucky.
Dr. KraftAnd I think it is; Marty and I have been doing Botox injections for about 20 years now. There are people that I have not seen for a long time. Some unfortunately have passed on, which we know, but there's many, many that I have not seen in a long time and I don't know what the reasons may be. They may have moved away, but usually people who move away ask for referrals to peers who are in other centres. I have a large body of patients who have stopped coming and, perhaps, some of those patients have gone into a type of remission or no longer need the treatment. So, among the patients that I have not referred to a neurologist, an oculoplastic surgeon, have not moved, or, passed on, there is a group that I have no trail of where they've gone or what has happened. And it may very well be that some of them have fortunately entered to the same type of situation you have.
And given that I hear about 1% are known to improve to the point that they no longer need treatment and, I guess as fickle as this disease is coming on out of the blue for whatever reason, it's fickle enough to go and, your biochemistry changes, so it is a type that will go away. The hemifacial, for those of you who have the hemifacial spasm, we know is related to a nerve and artery connected together that keeps touching each other. Depending on how the brain may be developing, and, if the artery grows away from the nerve, or the nerve grows away from the artery, the two can separate and one can go into permanent remission as well. In this case, it is much more likely and we can understand what happened. But as far as the blepharospasm is concerned, it is a disease we still do not fully understand and it has multiple different subtypes: so just be glad you're in remission.
Q. – I wear sunglasses a lot during the day. They seem to bother me more even in the sun than if I wear nothing. And I wonder is that unusual with blepharospasm? Also my problem is that when I go out on, let's say an overcast day, I can barely see at all and it doesn't seem to cut the glare. It seems to go right through the glass for me. And, these are supposed to be antiglare and UV and everything, they're a prescription.
Dr. KraftAre they polarized filters? That is the key. There are two things. Yasser did allude to the glasses. There's a certain type; a certain colour and a certain wave length that is very hard to make. I mean you could find out what wave length it is and have it commercially made but there's a company in Utah or Colorado and the Foundation has that information.
Dr. KhanThe other thing is that I find that a lot of patients are happier with glasses that, and I don't know whether yours are the same, sort of cover all light entering. The less light that gets in from up top on the eye and wearing glasses that has guards that block all light getting in I think you'll find a lot more comfortable.
Dr. KraftCheck if they're polarized filters too. It will get rid of the glare off of things. I say there is a certain type called FL41, I believe is the name of these glasses, but it doesn't help everyone and I don't understand myself why it can't be obtained closer to home. I keep hearing about this company that you have to order it from.
Sam MeisterCan I make a remark to this? My wife for a few years was wearing the polarized eyeglass and it helped. And all of a sudden it started fading out and we went to our optometrist, he's very good and very careful. He changed the green to orange and it helped. So somehow it comes out in statistics that for certain people it is not always the green because it's dark. It could be a dark, dark orange, and it helps.
Q. – Are there any doctors or optometrists that specialize in helping you with these glasses; like going to them and getting help with them or do you just go to any optometrist?
Dr. KraftI think it might help to go to people who actually make the glasses for you. The problem is that they are not medical doctors and, they will not understand this condition, but they can certainly help you try to obtain the wave length you may need from their inventory, or, to at least work with the optical part of it to try and get you the most comfortable one that seems to work for you. And they will work with you in terms of getting the right actual lenses made and that's very helpful.
Dr. KhanThe CNIB might be a good source because patients with macular degeneration often get these tinted lenses and the CNIB would have the exact number and things like that. So, that's another source that could help.
Q. – A few years back when I was in England I purchased a pair of sunglasses for my grandson. And at that time they were very expensive and apparently the lenses were treated with something that they used to walk on the moon. I wonder if there's any way to research that, if that would help?
Dr. KraftThat's why I think if you go again to an optometrist they're trained to put glasses together with all the gizmos and things that can be put in there.
Q. – Are they familiar with the coating used on space helmets?
Dr. KraftI would think that they would be. They won't be familiar with the disease but they'll be familiar with the different wave-lengths and tints and the type of things you're describing. I suspect that what you're referring to is a coating on the lens. It's a natural reflective coating. That's when you see them it looks like a silver, you can't see their eyes through their helmet, you see the silver sort of coating. I don't know if that's used on glasses on earth.
Q. – No, these weren't like that.
Dr. KraftThey weren't like that? Then it must be something else. Whether it is put into glasses for here or whether something similar replaces that for use here, I don't' know: but optometrists should know. I can't remember the name of it but I think I know what you're referring to.
Q. – I don't know if this question was asked before but I have Meige syndrome along with the blepharospasm and unfortunately every time I get my injections, I would say 90-95% of the time, I have ptosis and this time it was so bad that I was blind for two weeks in one eye. And, now this has been my sixth week and it's just starting to get normal. So it's very frustrating. In ten weeks I have to get another injection. So it's four whole weeks and what's my answer?
Dr. KhanPtosis or droopy eyelid can be one sort of side effect of Botox®. It depends on where it's injected. If it's injected close to the eyelid muscle that elevates the eye or, nearby, a little bit of the medication can trickle down and affect the muscle and then cause the eyelid droop. So it all depends on where it's being injected is one thing and certainly changing the location can prevent the complication from occurring.
Dr. KraftWell, have you mentioned this to your doctor. When I see patients who have had this type of history, it's just a matter of asking the doctor, who is doing the injections, to compensate, as Dr. Khan was mentioning, by putting the site of the injection a little further to the side. And when I do that it's very rare to get the ptosis.
Q. – I want to ask one more question. When you start with this, do you start with certain sites to begin with or it's different in the beginning?
Dr. KraftOh no, no. This is why anatomy is important. We put it in all those muscles that are known to be effected by the actual blepharospasm. There are specific sites that we know that are the ones that are most helpful. So, it's not hit or miss. Once you treat people and, this is why having a profile of how they responded is very important, because, for instance, Bob Campbell, he's allowed me to discuss his case, not in detail, but basically, I've customized his treatment. He gets a treatment that is radically different from anyone else that I've done simply because he plots exactly which muscles and areas seem to respond and which don't. And, I think that's true of most of the people in this room, you know you customize them after you get a profile, that the usual sites that work for the majority of patients, do or do not work in a given patient. If they do work you continue on. If they don't work then you sit back and say why? Where do they still have the 'action', or, do they have, let's say, apraxia? This means that you have to sort of modify things to the apraxia or consider the eyelid surgery.
So, you get a profile of how patients respond and change the sites as you need to or changes the dosages. If you get side effects you can change the concentrations; you can change the sites. Then it becomes a little more trial and error, but with the knowledge of what happened before it's not so much trial and error; it's more just adjusting it a little bit and seeing what happens in a controlled fashion. Most people will come back to have a response that is, at least, good enough to let them carry on with their activities. So there are sites that most of us would agree that we would do to start. We don't continue with that by rote or by habit unless we know that it's working. If not we start to change things.
Q. – John, I just wondered - I was curious about why you wear the mask to bed?
Dr. John WalmsleyI guess I forgot to mention, I have partial closure of the eye. My eyes get very dry, especially at night when I'm not blinking or anything. It reduces evaporation, or that's the idea of it anyway.
Q. – My family have seizures, what's it called? Epileptic seizures. Is this related to what I have (BEB)? The seizure is spasms and everything else. Is there anything at all related to that? Is there the possibility for me, because of what I have, to eventually get what they have?
Dr. KraftAs far as we know there isn't any relationship. The only thing I can tell you is that by coincidence a couple of the medications that work for blepharospasm are also medications that are sometimes used for seizures, but it does not mean that what you have is related to or is a seizure. It just happens that the biochemistry that is changed by some of these drugs and the biochemistry causing the blepharospasm is the same type of chemistry that has gone wrong. It doesn't mean that the two diseases are at all related. As far as we know they are independent of one another.
Q. – I find that when I'm wearing glasses or sometimes concentrate while reading there is a lot of spasms around the nose and brow area.
Dr. KraftI have a number of patients whose spasms come from a central focus, that the central part of the face is one of the trigger areas that seem to be predominantly involved. And these areas can be injected. Are you getting Botox injections for it? You can ask the doctor to concentrate a little more in the brow area and just beside the nose here. I have one woman I know that started coming to me a about three years ago who had a very sensible predominant brow ache and nasal kind of twitch right along the central part of the face. And we customized, a little extra site here and in the nose area and she did very well. And, again, you customize it individually. Ask the doctor is treating you: you get judicious injections along here, you have to be very careful because you're near the eyelid so as long as it's kept in the nasal area and the muscles that come down and that Dr. Khan was showing you on the diagram in the centre, you can bias the dosage concentrations towards that and hopefully make your particular problem a little bit better.
BOTOX® is a registered trademark of Allergan Corporation