From Sheila Raj
I had a question regarding the recent MH article posted on PAAD. If a family member tells the anesthesiologist about a close relative who has the RYR-1 mutation, and the patient you are treating is an outpatient, how do you go about getting the patient tested? In my scenario, the patient was scheduled to be admitted post-op for obs 2/2 to their primary problem. However, despite my urging of the primary team to send off labs for genetic testing, they discharged the family home the following day without anything being done. Should I reach out to the family again? Their PCP? Both? It’s equally possible that the family refused testing, but it’s unclear.
Henry Rosenberg MD responds
Very pertinent question. There are three things I would recommend. Check with the MH Registry at U of Florida to see if the relative is in the Registry(NAMHR) . See mhaus.org for information on contacting them. Second, get whole exome testing from a reputable company like Prevention Genetics or In Vitae to determine whether there is a causative variant for MH and if it is the same one in the relative. If none is found, you cannot rule out MH. In countries where contracture testing is readily available such as Canada or countries in Europe, that would be the next step. There are only two contracture testing centers in the US. One in Minneapolis, the other at Bowman Gray. The second is to get a geneticist involved in recommending testing and guidance for the patient.
Further research and guidance is needed on MH by anesthesiologists, surgeons and other physicians. There are about 400,000 people in the US with a DNA variant found in MH. Fortunately the penetrance is quite low.
From Monica S. Vavilala, MD, Professor, Anesthesiology and Pediatrics, University of Washington on induction agents in head trauma
Evaluating the effect of induction agents is complex. Proximal effects of hemodynamics are affected by volume status from hydration and or hemorrhage as well as sympathetic tone from the trauma. The pediatric BTF guidelines from 2019 recognizes ketamine as an acceptable induction agent. Previous concerns of high ICP are not reproduced and there may be some advantages to CPP as well.
The other issues are use of hyperventilation which also decreases ICP acutely and may confound the relationship between induction agent and ICP, as well as TBI severity which is heterogeneous even in the category of severe TBI whose GCS spans 3-8 (large). Plus, suspected severe TBI in this study means that some may not have had severe TBI; confounded by sedation. So, the negative finding does not surprise me.
From Elliot J. Krane MD on IV acetaminophen
Loved the recollections of Cadence Pharma, to date the only pharma company that ever set out to study their IND in children — babies no less! — without arm twisting by the FDA or US Congress. I mourn the company’s passing.
However your recollection of what I said is incorrect. What I said in the investigators’ meeting had to do with my prediction of the ease of getting informed consent from parents for the study. I said as nearly as I recall, “…what parent wouldn’t sign a consent? APAP was like mother’s milk, there was no child in America that hadn’t already been given it by their parents.”
Before the company settled on their proprietary name for the product, Ofirmev, I also lobbied hard for what I thought was the best name in the world: “Apap-asap.” Pronounced “ā-pap’-â-sap.” To this day I still think they made a huge mistake. “Ofirmev.” What a terrible name.
And I agree with the remark about capitalism and the drug industry. Martin Shkreli would endorse the industry’s lamentable behavior.
From Dr Rosalind Morley BM FRCA MA - Royal Manchester's Children's Hospital Manchester UK.
On Reading Brian Schloss, MD, Pediatric Anesthesiologist, Chief Medical Officer, OFFOR Health on plastics and waste question about reusing disposable circuits I thought that I should reply with a UK based prospective. The common practice here seems to be changing circuits once a week but always using a suitable size HMEF filter on the patient end. This cuts down waste to minimal without compromising patient safety. This is probably more important in the UK where anaesthesiologists use anaesthetic rooms to put patients off to sleep (away from the surgeons) and the use of the Jackson- Rees T piece on induction and extubation is far more common - swapping to a low flow circle anaesthesia for the remainder of the operation. In small neonates where a filter would be too big a dead space the circuits are then single use only. I would love to say that this is a green initiative that I have witnessed for about 20 years but I am also guessing that the cost saving is also massive.
From Kevin Ng MD on IV vs PO/PR acetaminophen
Although I agree that the efficacy is probably similar, I have one area to bring up. The advantage of IV Paracetamol (PCM) is that you would be sure that the entire dosage will be received by the patient, which is not always the case with oral PCM, given that not all children would readily swallow the medication. The issue with suppositories would be the difficulty in tailoring the dosage, especially for the smaller children where my smallest supp formulary is 250mg.
Without doubt that IV PCM is a more expensive option, but I would make the argument that in children where receiving the full dose of PCM is important, there is a clear advantage to using the IV formulation.
At this moment in my practice, I am also awaiting the approval of an IV NSAID, which again would replace supp diclofenac that we have now, which is limited by its formulation (12.5mg being the smallest dose). So again, IV would allow me to administer a more accurate dose to my patients.
I do like the suggestion that you have made, where children and adults who have no issues taking oral medications should be given PCM at reception, as a routine premed. Pre emptive analgesia always made more sense to me, but I have not found articles to its efficacy. Some preemptive analgesia studies have not shown remarkable results, perhaps something for us all to look at in more detail?
Thank you again for the PAAD articles, love them to spark some discussions and thoughts in my practice.