Malignant Hyperthermia (anesthesia allergy/sensitivity)

[Strawberry]

Okay, I have never even heard of this before, but now am being told I have this! Does anyone else have this? Or even know about this? I haven't found anything directly linking to mast cell disease, but I am so baffled.

I have one family member that died under anesthesia (uncle), and two others that nearly died under anesthesia (dad and brother). I have always told anesthesiologists this before any procedures. I have a surgery coming up soon so had talked to the doctor about it, now they are trying to figure out if they even dare touch me. I have not talked to the anesthesiologist yet, although he is to call me today.

She also wants me to get a medical bracelet ASAP.

So now I am wondering if this is part of MCAS? Does this fit in with ME/CFS and how so many of us can't tolerate anesthesia???

If it was just one procedure, I think I would be fine with it. Problem is that it is a wire guided surgery and I am to have a wire inserted a few hours earlier. I had a biopsy a few weeks ago (negative for cancer at least) and mildly reacted to the lidocaine (epi free). So they want to give me epi free lido, then 4 hours later sedate me with what ever malignant hyperthermia drug. Doctor is nervous. As I have all ready stated, I have not talked to the anesthesiologist.

Hmmm, another thought, this IS the same hospital that my dad and brother nearly died in. Maybe they still have records from that far back. Doubt it. But worth asking anesthesiologist.

Sorry this is so long, my brain is racing now.

 

[pattismith]

all I know is it's about a ryanodine receptor calcium channel mutation, which lead to anaesthetic bad reaction (but the reaction is not obligatory )

It's nice that your doc know it before performing the procedure, he will adapt his protocol, and keep Dantrolene at hand in case (antidote)

I'm not aware of any other effect/symptome of this mutation, but it is worth invastigating!

 

[Gingergrrl]

Okay, I have never even heard of this before, but now am being told I have this! Does anyone else have this? Or even know about this?

I actually remember someone else posting re: malignant hyperthermia w/anesthesia and am tagging two people who it might have been @zzz and/or @anciendaze. Apologies to both if it was someone else!

I have one family member that died under anesthesia (uncle), and two others that nearly died under anesthesia (dad and brother). I have always told anesthesiologists this before any procedures. I have a surgery coming up soon so had talked to the doctor about it, now they are trying to figure out if they even dare touch me. I have not talked to the anesthesiologist yet, although he is to call me today.

If it was me, I would postpone the procedure unless I personally spoke to the anesthesiologist and felt 100% confident that they are knowledgeable about this issue and will have the antidote ready.

So now I am wondering if this is part of MCAS? Does this fit in with ME/CFS and how so many of us can't tolerate anesthesia???

I actually have no idea if this issue relates to MCAS or ME/CFS vs. it is a separate issue in and of itself? In case it helps though, the Mastocytosis Society (TMS) has a list of which anesthesias are generally tolerated in MCAS patients and which to avoid as dangerous. There is some debate about it but I found the list to be helpful. Let me know if you cannot find it on-line.

If it was just one procedure, I think I would be fine with it. Problem is that it is a wire guided surgery and I am to have a wire inserted a few hours earlier. I had a biopsy a few weeks ago (negative for cancer at least) and mildly reacted to the lidocaine (epi free). So they want to give me epi free lido, then 4 hours later sedate me with what ever malignant hyperthermia drug. Doctor is nervous. As I have all ready stated, I have not talked to the anesthesiologist.

What is the procedure (if you are comfortable saying)? I am glad you were negative for cancer, whatever it was! What was your reaction to the Lidocaine?

Hmmm, another thought, this IS the same hospital that my dad and brother nearly died in. Maybe they still have records from that far back. Doubt it. But worth asking anesthesiologist.

You never know and they might still have the records. I think it is worth asking and nothing to lose. The thought of general anesthesia terrifies me b/c in my case, I am not supposed to have one that blocks the calcium channel and I also have MCAS and allergic to contrast dyes and who knows what else? I will definitely be following this thread and wish you the best.

 

[Binkie4]

@Strawberry
Sending you all good wishes for this surgery and especially the anaesthesia. I too would speak to the anaesthetist well before surgery so they are fully prepared. I have ME but not MCAS and had saline before my recent short surgery. It went well. Do you have an MCAS doc who could advise about saline?

 

[Strawberry]

@pattismith @Gingergrrl @Binkie4

Well the surgeon called me back a bit ago to notify me the anesthesiologist wouldn't be calling until Thursday. So I guess I have a few days to get info. I know I can tolerate propofol, but I doubt that is enough for this. I did okay on lidocaine, but did feel "off" enough they are uncertain if I can have that the morning of the procedure. ugh.

In case it helps though, the Mastocytosis Society (TMS) has a list of which anesthesias are generally tolerated in MCAS patients and which to avoid as dangerous. There is some debate about it but I found the list to be helpful. Let me know if you cannot find it on-line.

Thanks for that! I couldn't remember what it was called, now I found it. Neither TMS or Lisa Klimas have anything about hyPERthermia. HyPOthermia, yes, on Lisa's site. So now I am really confused if this has anything to do with MCAS or if this is a new diagnosis that can complicate further. BTW, its a breast radial scar if you want to google it. They found no cancer cells in any of the biopsies, but they fear it is there hiding inside the tissue. I told the surgeon about my low NK function and how it gave me higher risk of cancer, and she agreed. She also was dumbfounded that I even know my function #!

Lidocaine made me a little fuzzy headed, and a teeny bit nauseous, but not bad. But I had difficulty sleeping for a week afterwards.

My brain is overloaded, I am sorry if I missed anything. Thanks for the well wishes!

 

[Strawberry]

Oh, one more comment for stress relieving humor! I found this in the wikipedia page for malignant hyperthermia:

Pig farmers use halothane cones in swine yards to expose piglets to halothane. Those that die were MH-susceptible, thus saving the farmer the expense of raising a pig whose meat he would not be able to market. This also reduced the use of breeding stock carrying the genes for PSS. The condition in swine is also due to a defect in ryanodine receptors.

So I guess my meat is unmarketable!

 

[Binkie4]

@Strawberry
I bet your head is spinning.
Just to say that it might be worth investigating saline and anaesthesia. It is now being recommended for anaesthesia with ME but I do not know about MCAS. My ME doctor has written to say that it should be given before any stress test or anaesthesia, and hospital are taking it very seriously.

EDIT: just read more about this condition and see you are facing a complicated and serious situation. Hope your doctors can reassure you that they have it thoroughly covered.

 

[Gingergrrl]

So I guess my meat is unmarketable!

Nah... you are still hot property on the market in spite of malignant hyperthermia LOL (actually malignant hyperthermia sounds like "dangerously hot" if I change the wording... and you know I am just teasing you and NOT making light of a serious situation).

 

[Strawberry]

LOL @Gingergrrl thanks for the laugh! (would that be tacky to use that line when talking to the surgeon or anesthesiologist? )

to all:
So are the most important points for me to remember when talking to the anesthesiologist tomorrow the Dantrolene and the saline? (and a list of the safe meds)

Also, tagging @Butydoc and @Jonathan Edwards in case either have input. (I will also PM each of them per advice, so if they don't respond here, please no one take offense). I still have not found a link to either ME/CFS or MCAS, but I have a difficult time believing there is none. I therefore am hoping they will post here for others to learn.

 

[anciendaze]

The case I've heard of was connected with a periodic paralysis patient who experienced malignant hyperthermia in response to a standard muscle relaxant. Generally, the disease is described as a defect in an ion channel, and this fits both the paralysis and hyperthermia, though in different ways. It is not a simple one-size-fits-all diagnosis. Each patient tends to have a unique combination of problems.

(Another patient with periodic paralysis responded to a muscle relaxant in the same class with paralysis that stopped breathing, not hyperthermia. Fortunately, a machine for artificial respiration was handy.)

The connective-tissue problems associated with the genes responsible often result in a narrowed trachea, which is typically considered an unrelated problem from a medical standpoint. Had the doctor simply waited for throat muscles to become fatigued and relax naturally the patient would have been fine, but that would have broken the day's schedule. I can't really ask for much more detail because that patient died, and the use of a type of medication he had warned about became a legal issue.

What was frightening is that virtually every doctor would have done the same thing, because the muscle relaxant works safely and well in the vast majority of patients, who do not have a very specific problem. Other problems constrained what the doctor could do, and he may have decided this difficult patient was only being psychologically difficult. If you are only one in 100,000 you have to be careful how you approach the medical profession. It is very likely your doctor will never have seen a single patient with your problem.

I'm still unsure what to say about a number of related diagnoses. There seems to be overlap between periodic paralysis and episodic ataxia. There are also many patients who show symptoms that could be classified as MCAS. If you concentrate on the connective tissue aspects you may decide this is another form of Ehlers-Danlos syndrome. The patients I know all have issues with autoantibodies, and that fits in with MCAS. So what kind of disease do you call this?

Is a particular patient vulnerable to malignant hyperthermia? This is rare, but the only sure diagnosis might involve an autopsy. It is better to avoid even getting close to that frightening condition, because once it sets in it is hard to treat in time.

Doctors badly need to know what is actually going on in the specific patient in front of them.