MH precaution for duchene muscular dystrophy patient?
Started by Chloroform4Life
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http://www.mhaus.org/healthcare-professionals/be-prepared/associated-conditions
MD is not on the list
MD is not on the list
The association between DMD and MH although disputable does have some merit and there are case reports of MH in these patients. So I think it should be considered as MH risk and treated with full precautions.
http://www.ncbi.nlm.nih.gov/pubmed/3742323
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Duchenne's is not an absolute contraindication to using triggering agents. The only absolute contraindications that I remember off the tp of my head are Central Core, Multi mini-core, and Kings Densborough.
The issue with Duchenne's is that it can cause MASSIVE RHABDO with both succinylcholine and volatile agents. Volatile agents have been implicated in several severe rhabdo cases without the use of sux. It's called volatile anesthetic induced rhabdo (VAIR). Too busy to paste links, but google it. At the pediatric hospital I'm at we tend to play it safe and run a full TIVA. Also, avoiding opioids is generally considered a goal since they tend to have reduced respiratory function. Case reports have shown successful use of regional/epidurals/blocks.
So essentially, yes it's MH protocol but not specifically for MH
That's what some "forget" is that even VA's can cause rhabdo in DMD. Thus, best avoided.
TIVA.
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Il Destriero
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Il Destriero
That would be my plan. To add to that, though, I can't say I'd flush the machine and any other MH "prep" I'd do for MHS patients.
Like Il destriero- TIVA.
lots of case reports with cardiac arrest secondary to hyperkalemia in these kids after exposure inhalation agents. The timing in these cases to arrest are variable. Lots of cases of children with DMD having uneventful anesthetics with "triggering" agents too...but in this day an age of readily available alternatives, why take the risk? Older kids with cardiomyopathy may be at risk of cardiovascular collapse with propofol too, so no "safe" anesthetic, but less likely for these older DMD kids to mount a hyperkalemic response with such atrophied muscle. The dangerous age is in the DMD kiddos who still have viable muscle (under 8 or 9) and upon exposure to "triggering " agents may mount anything from fairly benign mild rhabdomyolysis (detected by lab or dark urine) to full blown rhabdomyolysis with hyperkalemic arrest. It's certainly an area of controversy, I have served as a moderator of PBLD's on this topic and it's always a lively discussion.
lots of case reports with cardiac arrest secondary to hyperkalemia in these kids after exposure inhalation agents. The timing in these cases to arrest are variable. Lots of cases of children with DMD having uneventful anesthetics with "triggering" agents too...but in this day an age of readily available alternatives, why take the risk? Older kids with cardiomyopathy may be at risk of cardiovascular collapse with propofol too, so no "safe" anesthetic, but less likely for these older DMD kids to mount a hyperkalemic response with such atrophied muscle. The dangerous age is in the DMD kiddos who still have viable muscle (under 8 or 9) and upon exposure to "triggering " agents may mount anything from fairly benign mild rhabdomyolysis (detected by lab or dark urine) to full blown rhabdomyolysis with hyperkalemic arrest. It's certainly an area of controversy, I have served as a moderator of PBLD's on this topic and it's always a lively discussion.
