I'm due to have some reconstructive surgery on my left foot that is going to take between 4-6 hours to complete. This will not be the first such procedure I've had but I am aware of the complications posed by MC, hypothermia being one of them and intubation being another.

The last couple of occasions when I've had surgery they tried an epidural, even though I have Spina Bifida, which failed. I was given a general anaesthetic without any problems.

The next time they tried a drug that induced what I can only describe as an halucenogenic; it was horrible!

I was wondering if anyone else has had any experience of anaesthetics and any adive for my up and coming surgery?

Well, up until July 23rd I had many routine surgeries under general anesthetic and never suffered complications. I have had 2 strabismus surgeries, tonsils, gallbladder, a prior c-section and never triggered. I don't know what to tell you now. I have talked to some specialists at UCLA and they seem really interested in finding out more about my case. Good luck with your surgery!

Pete - I think the main thing is to make sure they know what your condition is, and that you print out the MC letter and the anesthesia article, and give a copy to both your surgeon and the anesthesiologist, just to be sure they understand your situation.

Some doctors really don't take it terribly seriously, but if you, as the patient, are proactive, and know what you are talking about, then after they get over their shock, they will usually appreciate that you care enough and are informed enough about your own body that you want to have input.

Additionally, by providing them with this info, they also clearly will know that they can't take the easy way out, because you and your family will know. Do you have a Medic Alert bracelet, or a listing of the medications and chemical agents we are to avoid?

Many thanks Lois. The lead surgeon is someone who has known me for many years and is aware of the MC, indeed I believe that this was one of the reasons why they attempted the epidural the last time he operated on me.

I don’t have a Medic Alert bracelet but my medical file has “At Risk” stamped on it in big red letters and I always seem to meet the anaesthetist before the procedure. That said, I will take copies of your letters with me but a listing of the chemical agents may prove useful also.

Pete - I know that Jan has some links to articles about anesthesia, and there are multiple threads about MH and the meds which are contraindicated... for example: (which isn't really complete)...

http://pub14.bravenet.com/forum/static/show.php?usernum=1129569505&frmid=140&msgid=649502&cmd=show

Off the top of my head, I would say that doctors should know that, for folks with MC, medications to be avoided (or used with extreme care and caution) include epinephrine (adrenalin); anectine (suxamethonium or succinylcholine), IV potassium - "depolarizing agents"; beta-adrenergic agonists and beta-antagonists, propranolol (Inderal)- a beta-blocker; some inhaled anesthetic agents - basically, an anesthesiologist needs to do his homework before a surgery on a myotonic patient.

One of the reasons that anesthesiologists always (except in emergency situations, which is why the Medic Alert bracelet or necklace is so important) meet with patients is because they do need to know about allergies, sensitivities, co-morbidities (other conditions you have which may impact what he uses, when and how), and enough about you to make educated split-second judgments during the surgery. This is for you protection, and also for theirs!

Thanks for the reply Lois.

I intend to ask about the anaesthesia when I meet with my surgeon. As I said above, the last time he operated on me he tried using an epidural even though these are not recommended for people with Spina Bifida, which probably explains why it didn't work even after 8 attempts!

The last time I had surgery the anaesthetist came to see me just a couple of hours before the surgery and clearly knew nothing about MC. He gave me something that led to me having an hallucinogenic experience; it wasn't very pleasant. I hope to avoid that again.

Our anesthesiologists use Fentanyl, Propofol and Versed for IV sedation and Bupivacaine for locals or epidural. Gas inhalation anesthesia should never be used.

Jan

Hi, I just discovered this forum and can't believe all the people with similar experiences. I too have had epidurals tried on me without success and wondered if it was myotonia related. Maybe. I also have a problem getting anesthetics to take effect when having skin cancers removed. Evidently it takes much longer for it to take effect on me than on most patients and I have a hard time convincing the doctor of that. Many times I have had the growth sliced off and cauterized before the anesthetic has taken effect I have since switched doctors. It must have something to do with the myotonia.

Type of Myotonia: acetazolamide-responsive myotonia congenita

Country: usa

Welcome Gail!

Epidurals almost always use an anesthetic called bupivacaine which is safe for us and should work if it is done correctly. However having watching many given to women in labor, the majority that I saw had at least some patches that weren't numbed and some didn't work at all. This is not uncommon in the general population.

On the other hand spinals, where the anesthetic is injected directly into the spinal fluid, always work but there is a risk of a slow leak which can cause a migraine but is fairly easily patched. I have seen a case of meningitis from a spinal and that is also a risk that has to be considered.

In Pete's case (previous posts) he has spina bifida which alters the anatomy of the spine and frequently causes failed epidurals because of the pressure on the dura, the space where the epidural is placed.

If you don't get numb quickly or at all from local anesthetics at the dentist, then you may have a connective tissue disorder as well as the MC. I have Ehlers-Danlos Syndrome which is notorious for causing resistance to anesthesia. Also if you take vitamin C, you need to stop for a few days before getting dental work since that will make the cell membrane resistant to the effects of the anesthetic.

The local anesthetic I recommend is called Marcaine which is the same amide that is used in the epidurals. Always ask for no vasoconstrictors in any anesthetic since the epinephrine will make myotonia worse. It doesn't last as long but is safer for us. Lidocaine (xylocaine) is not a good idea since it alters potassium levels.

Jan

Type of Myotonia: Thomsen's MC

Country: US

Hi Gail:

I'm not a doctor or expert in the field of biophysiology, just someone with myotonia who reads a lot, so please take this with a grain of salt.

I'm not sure what they used on you. Local anaesthetic is often lidocaine though.

As far as I know, acetazolamide-responsive myotonia is usually a sodium channel mutation on the SCN4A gene.

Mutations on this gene may also cause/be allelic with paramyotonia congenita, hyperkalemic periodic paralysis, hypokalemic periodic paralysis, a few other things, and malignant hyperthermia. You should wear a med-alert bracelet for the malignant hyperthermia and know what triggers to avoid, and that dantrolene is used to treat it when it occurs.

Lidocaine and it's derivatives have been shown to be beneficial in treating myotonia caused by chloride channel mutations, which means Thomsen and Becker myotonia congenita.

In sodium channel mutations, the affects of lidocaine are thought to be more varied. Lidocaine might have beneficial affects in individuals with certain mutations but not others. It affects different sodium channel mutations in different ways.

In my reading I had come across an interesting condition called hypokalemic sensory overstimulation. This is a form of ADD caused by a drop in potassium levels, and the study noted lidocaine insensitivity in these individuals. The study went on to discuss similarities between hypokalemic sensory overstimulation and hypokalemic periodic paralysis, which can occur on the SCN4A gene but can also occur on a calcium channel gene and sometimes a potassium channel gene. I think more studies would need to be done before any strong conclusions could be made. I do not believe the condition at hand had been linked to any gene at the time of the study.

It's very well known that sodium channels are affected by though lidocaine.

So the answer to your question is, maybe. But then again, maybe not.

There have been times at the dentist where they could not get me numb with lidocaine, but I have a chloride channel mutation, so it's probably unrelated.

At the dentist, I have a lot more luck with septocaine or mepivacaine.

I also felt it when they snipped the muscle during my muscle biopsy. Not pain, but the electric jolt. Makes me shudder to this day!

Type of Myotonia: Becker

Jenna,

My concern about Lidocaine is primarily for two reasons. First of all, many people with myotonia are taking Mexitil (mexiletine) or Diamox (acetazolamide). These both affect potassium levels and when Lidocaine is injected, infused or even used orally for these people (tocainide is a Lidocaine analog) it can actually trigger cardiac problems and reach toxic levels very quickly.

My second concern is that I have seen so many reactions to Lidocaine and when the reaction is severe it can happen so quickly it can be fatal. I've observed this with topical (for numbing the throat) and injectable. When I first started working in labor and delivery many years ago lidocaine was in epidurals. Over time I think many anesthesiologists changed the mix to primarily bupivacaine and fentanyl. I personally saw the reactions drop dramatically. I went into shock from a sudden reaction myself when it was given locally during childbirth.

When I was on the mailing list for periodic paralysis many people reported reactions to Lidocaine, especially those with hypokalemia. While different ion channels are affected in different ways I still give a strong caution just because I feel that other anesthetics are much safer. There was a short period probably in the 80s as I recall where tocainide was prescribed for myotonia after some studies showed that it improved both MC and myotonic dystrophy symptoms. My cousin took it for a while (Tonacard) but I don't believe he saw much benefit. It has some pretty ominous potential side effects and now is only recommended for very specific issues like severe ventricular arrhythmias.

There was some debate over whether it could trigger malignant hyperthermia but I think most anesthetists agree that it is not a trigger. Lidocaine injectable with epinephrine does contain sodium metabisulfite and I have wondered if that is sometimes responsible for reactions. People with sulfite allergies could easily go into shock from the exposure.

I have also done okay with mepivacaine/Carbocaine but it is considered an ototoxic drug so my first recommendation is always Marcaine with no vasoconstrictors. I've had oral surgery where Carbocaine worked great, and another time when it didn't work at all and I basically had a tooth extracted with no anesthesia. The surgeon said he'd given me enough to knock out a horse, so I think sometimes with dental anesthesia it's a matter of technique and the dentist getting it in the right place to create a successful nerve block. Lidocaine does create topical anesthesia whereas Carbocaine does not. (I can't even tolerate a topical swab on my gums with Lidocaine before an injection - my dentists have indicated that this is not unusual).

Jan

Type of Myotonia: Thomsen's MC

Country: US

Hi Jan:

Thanks for the info. It's always good to know these things!

I didn't even think to look into the affect of local anaesthetics on myotonia involving disorders until after I had a strange "reaction" at the dentist.

That is actually one of the things that prompted me to get my mutations typed.

As you mentioned about the drops in potassium, the epinephrine in the lidocaine can precipitate a hypokalemic periodic paralysis attack. I've read it can even cause hypokalemia, in high enough dosages, in those who do not have a disorder.

It has been suggested that the latter event happened with me, since it's now known I have a CLCN1 mutation and not SCN4A, but the hole in the theory is, I metabolize the epinephrine quickly and this happened a good 30 minutes after all signs of the jitters passed.

Another theory suggested to me, in a very respectful manner, is that I was stressed to the point of near fainting and it was an anxiety type attack. I'm not adverse to such suggestions. As we all known, anxiety can cause very real physical problems for us because we can't respond to the stress hormones properly. I might even have been able to convince myself that I was stressed in some subconscious way even though I felt perfectly relaxed at the time. But the whole thing started with my leg muscles cramping up, and my first thought was "Gee...that's weird," because outside of the myotonia, I really don't get any other types of cramps in my legs. It really just hit me out of the blue and I can't say I have ever experienced a similar situation before. I know I've gone on about this a bit before but it really was unusual.

I never figured out what it was from but they've stopped using the epinephrine on me at my request.

Another thing I'm wondering about is if it was the lidocaine affecting the ion channels in an over-compensatory way.

Type of Myotonia: Becker

Jenna, when I went into shock after getting a large dose of Lidocaine I remember I was terribly weak for hours, very much like a hypokalemic attack. I finally had the presence of mind to ask for orange juice. It really helped and I kept drinking it until the weakness let up. I wish they had tested my potassium levels but no one had a clue. Later I did have a kind of a rebound effect and was very stiff. But I'll take that over the weakness any day.

I think the anxiety explanation can have some validity, but if you were anxious enough to cause that kind of reaction your heart would have been racing, you would have been sweating, etc. I had a malignant hyperthermia type reaction once while getting an MRI. I felt a huge electrical shock (like touching a very high voltage electric fence), my jaw clamped shut, my temperature started rising and I felt like a truck had hit me for several days afterward. The technicians blew it off as a panic attack, but I had been dozing and wasn't the least bit anxious when it started. When I talked to the manufacturer, GE, about it they said that some people who have electrolyte disorders can get shocked and react quite badly to MRIs because their cells can't repolarize rapidly enough to keep up with the shift in the magnetic fields. Static builds up and it eventually discharges. I have never heard of this happening with anyone else with MC so I don't think it's a common occurrence, but it is possible. Maybe they found a way to ground the person to discharge any static buildup.

Jan

Type of Myotonia: Thomsen's MC

Country: US

Hi Jan,
Last week I went to the dentist for a crown and had the same reaction I had the time before that when I had a crown done.....I was weak and tired for days. I will check to see if the dentist used Lidocaine to numb my gum before injecting Novocaine. Could the Lidocaine have caused the weak reaction in me, or could it just have been the trauma to my teeth?
Mary

Type of Myotonia: Thomsens

Country: USA

Mary,

Novacaine is the trade name for Procaine which works by blocking sodium channels, but it can cause toxicity fairly easily and is seldom used in dentistry any more. Usually they use Lidocaine/Xylocaine or Citanest which is Prilocaine. The two I usually recommend are Marcaine and Carbocaine with no vasoconstrictors.

If you are low in omega 6 fatty acids you may have trouble metabolizing the anesthesia and it could have a toxic effect at a lower level than most people would experience. Also being low in cholinesterase enzymes (which we tend to be) can cause toxicity sooner, too.

However there are many other things you could be reacting to including the cement for the crown. They usually contain potassium nitrate which would definitely affect you for a while. You might want to call the office and have them read you the ingredients in the crown cement. If they had to drill any silver fillings in the process you could also be reacting some mercury that would be released.

Jan

Type of Myotonia: Thomsen's MC

Country: US