On Thursday I'm going back into hospital to have my Ilizarov frame removed from my left foot and leg, 5 months since it was fitted. This will be a surgical procedure so I'm going to be knocked out again.

I've revised my fact sheet to mention Succinylcholine specifically as a chemical to be avoided so hopefully the anaesthetist will have an idea what to replace it with before they drag me down there.

Being able to educate the physiotherapists has proven to be a big step in the right direction. I've met people who have been wearing their Ilizarov frames for over a year so to be back up on my feet and walking (with crutches) after only 5 months whilst suffering with MC too is a big achievement. The fact that they were able to tailor my physiotherapy to my abilities has helped enormously, the only time I injured myself was when I pushed it a bit too much!

Anyway, I've said it before but it bears repeating, this site has given me not only a lot of useful information but also the impetus to confront the medical fraternity with their own ignorance regarding MC, in a nice way of course.

Many thanks to Jan and Lois!

Type of Myotonia: Becker's

Country: England

Pete,

I hope your surgery goes well. I'm sure it will be nice to get the frame removed.

It is possible to do surgery on the legs with a spinal instead of general anesthesia which would be much safer from the myotonia standpoint and it's a much easier recovery since your system doesn't have all those drugs to metabolize. You might want to ask the anesthesiologist about it.

I'll paste in below a basic protocol that I've shared before. It's based on recommendations of anesthesiologists I've worked with over several years.

Jan

Anesthesia protocol for surgery:

Check pseudocholinesterase levels before surgery to determine risk from anesthetics and depolarizing agents - if low use extreme caution

Make sure filter medium and tubes have not been contaminated with inhalation anesthetics; evacuate operating room air thoroughly before patient enters

Warm IV fluids and operating table if possible. Keep warm blankets on patient during the surgery to avoid triggering myotonia from chilling.

Anesthetics/Analgesics: Propofol, Fentanyl and Versed

Paralyzing Agent: Vecuronium (NO succinylcholine)

Local - Bupivacaine, no vasoconstrictors (no Lidocaine); epinephrine will trigger myotonia

IV saline only - no potassium added. Glucose/dextrose slow drip to avoid blood sugar elevation

Monitor potassium levels throughout surgery - cardiac arrest from hyperkalemia is a bigger risk than malignant hyperthermia

Type of Myotonia: Thomsen's

Country: US

Thanks Jan, I've already used your protocol for my fact sheet but I'm sure others might find it useful for future reference.

As well as MC I also have Spina Bifida Occulta and although the anaesthetist was of the opinion that an epidural was suitable I didn't agree. I was under the impression that epidurals were not suitable for people with Spina Bifida, that's what it says on the approrpiate boards, and a previous experience, which failed and left me in pain, doesn't encourage me to use them.

The Ilizarov frame is being used for a second attempt at correcting the deformity in my foot that has resulted from the Spina Bifida. At the moment it looks wonderful but who knows how it is going to appear after they remove the frame that is holding it in place?!

Type of Myotonia: Becker's

Country: England

Pete,

The literature from anesthesia research indicates that epidurals and spinals are safe for spina bifida (they are used routinely for women in delivery), but I think the decision would depend on where the level of the malformation is and what an MRI shows. Sometimes there is no dura so an epidural is not even an option. But spinal anesthesia is injected directly into the spinal fluid rather than the space surrounding it.

Spina bifida occulta is fairly common in the general population and many people don't even know they have it because there are no outward signs. If you had a bad experience with either type of spinal anesthesia before then I cam understand why you wouldn't want to try again. But in general, it's always a better choice over general anesthesia for surgeries in the lower pelvis and legs that will last less than a few hours.

Jan

Type of Myotonia: Thomsen's

Country: US

The epidural is a great idea but when I was researching my Spina Bifida I came across several references that did not advocate it for people who suffer the more severe symptoms and unfortunately I am one of the 10% who does.

I have a persistent and degenerative deformity of both my feet that the surgeons believe is caused by a muscle imbalance arising from the Spina Bifida. It wasn't dealt with in childhood, despite being obvious, and I didn't have surgery on my spine until I was in my mid-30's, which doesn't seem to have worked.

When they did try to give me an epidural they made at least 8 attempts, I actually lost count, but they all failed and left me in considerable pain until the general anaesthetic took effect. I am not keen to repeat that experience unless absolutely necessary, the surgery itself is painful enough!

Anyway, everything went well and the anaesthetist assigned to my surgery already knew which chemicals to avoid. He was very good to me and didn't seem to mind that I had educated myself on my condition. I recovered very quickly without any other discomfort other than a sore throat from intubation, but that always happens.

I hope anyone else who is facing surgery will use your fact sheet too, it really does give you the sense that you have a say in your own medical care.

Thanks again!

Type of Myotonia: Becker's

Country: England

Pete,

Will you be wearing a brace after the frame is removed? I've had a couple of friends with similar issues and they did seem to improve. It's a shame they didn't catch it early but spinal surgery is pretty scary for kids (and hard to keep them still afterward!). I hope you see some encouraging results when the frame is off.

I don't want to beat the topic to death, but I do want to explain that epidurals and spinals are two different things. Epidurals are called spinal anesthesia because they are in the space around the spine called the dura. The anesthesia never mixes with the spinal fluid (unless the doctor punctures through which does happen). A spinal is one shot of local anesthetic put directly into the spinal canal, whereas an epidural is a drip fed by a catheter into the dura.

I have seen many epidurals fail in normal, healthy women when I worked in labor and delivery. I don't think I would ever personally trust them for surgery like a C-section, knee replacement, etc. There can be patches that don't get anesthetized properly. Like you mentioned, some people are much harder to get anesthetized than others depending on the specific structure around their spine and even the condition of the paraspinal muscles - if they are well developed it can be difficult to insert the needle and feed the catheter. And unfortunately some anesthesiologists are just plain incompetent. At our hospitals they generally get two tries to do the epidural and if that doesn't work they have to call in another doctor. The only exception I saw was a military hospital and the guy was totally inept...I had to leave the room because it made me so sick to see how he bungled the insertion over and over.

Spinals definitely have a bit more risk but they always work. You are completely numb from the level of the injection down for hours. The main risk is infection or a slow leak of spinal fluid in the injection spot which can cause a headache, but is easy patched by using a few drops of your own blood. I had a spinal years ago with no side effects at all.

After your experience it makes sense to go with the general anesthesia, but I just wanted you to know that there is an option other than an epidural if you ever need the regional anesthesia instead.

Jan

Type of Myotonia: Thomsen's

Country: US

jan , this whole conversation is on anesthesia is very unnerving for me. this is kind of embarrassing but i had a Colonoscopy back 10 years ago and i'm due for another one sometime in the near future. when i had my first one i tried to tell them i might have to be careful with anesthesia , i even made them call my neurologist to make sure it's ok. he told them not to worry that it was ok. but i'm finding out that anesthesia is dangerous?

Type of Myotonia: becker

Country: USA the best !!

Ron,

Unfortunately many neurologists and other doctors who treat MC are totally unaware of the potential problems. We have to be our own acvocates in this area - the anesthesiologists take it very seriously.

A colonoscopy generally uses IV sedation anesthesia like Versed, Fentanyl, and/or Propofol so you should be fine. You are actually conscious and can respond to questions but won't remember it afterward.

It's the inhalation anesthesia like Halothane that can cause the problems as far as sedation. By far the most dangerous drug is a paralyzing agent called succinylcholine. If you are having actual surgery this is given to keep you from moving. Your breathing will become paralyzed, too, so you are on a respirator during that time.

With any type of procedure it's always good to mention to the doctors the potential for reactions with MC, and you need to tell the anesthesiologist both at the pre-op and again just before the surgery (since you often see a different doctor for pre-op and they may not convey it properly in the notes).

I once refused to let my mother get colon surgery until I could speak to the actual anesthesiologist who would would be monitoring her during the surgery. He came in just as they were getting ready to wheel her off to the OR and everyone acted put out that I was so insistent and holding up the schedule.

Even though I had given them extensive information during pre-op and they knew that my mother had suffered cardiac arrest from the MC during a previous surgery, the doctor on duty did not read through all the notes and would have used the same triggering agents that almost killed her before. You can't be too careful!

Jan

Type of Myotonia: Thomsen's

Country: US

I don't think that you are overdoing this topic Jan, it deserves to be discussed extensively. I was interested in your comments regarding the difference between an epidural and a spinal anaesthesia. When I had the frame fitted in June he definitely suggested an epidural to me and that brought back the bad memory, perhaps a more skilful doctor could apply one successfully but I remember all too clearly sitting on the side of the trolley and gripping a nurses forearm; I thought that it was to stop me from falling not to help me deal with the pain about to be inflicted!

I presume that in America patients have a right to discuss their treatment just like we do here in Britain? It certainly helps to do so as it made me feel more like an active participant in the decision making process than just a lump of meat on the operating table and I would encourage every MC sufferer to get involved in their own medical care when talking to the 'experts'.

It should be remembered, however, that everyone faces a risk when going under a general anaesthetic, no such procedure is 100% safe, but the risk is very small even for us MC patients as long as our susceptability to other problems is taken into account.

On the Spina Bifida, the protocal here is to operate on the spine as soon as it is diagnosed and surgeons can now do this on unborn babies. For some reason this was not done in my case even though the protocol existed when I was born, hence the failed spinal surgery when I was much older.

My leg and foot are in a plaster cast at the moment to allow the pin site wounds to heal and keep the bones supported. I'm going to be measured for a leg splint and I may have to wear it for the rest of my life but at least I should be able to walk and stand without the pain I used to suffer.

Next year we're doing the right leg!!!

Type of Myotonia: Becker's

Country: England

Yep, I too cant sing the praises of this site loud enough... also great 'community' spirit

Type of Myotonia: Beckers

Country: UK

I was given a spinal for Fibula/ankle surgery about 11 years ago, and no complications. I remember wondering if myotonia would be a problem. A framed leg you must of really had a bad accident, best of luck with recovery.

Type of Myotonia: Thomsens

Country: USA

Pete,

How are you doing with your post-surgery recovery? I hope the unusual cold there isn't making your myotonia a lot worse!

Jan

Type of Myotonia: Thomsen's

Country: US

Morning...well it is here!

Post op-recovery has gone very well thanks. I'm still stuck with the cast while I wait for a leg splint and boot to be made for me. I'm off crutches and down to a walking stick now, which is how I started!

The cold doesn't really seem to bother my MC, I know it does for other sufferers but for some reason it's never been a great concern for me. The worst part is ice and frozen snow for obvious reasons.

I hope everyone got through that awful weather in North America okay?

Type of Myotonia: Becker's

Country: England