In today’s #apaperaday, Prof. Aartsma-Rus reads and comments on the paper titled: Perioperative Care for Patients with Neuromuscular Disorders in the Netherlands – A Questionnaire Study Among Anaesthesiologists, Neurologists and Clinical Geneticists
Today’s pick is from Journal of Neuromuscular Diseases on perioperative care for patients with neuromuscular disorders (NMD) by Bersselaar et al. Duchenne is of course also an NMD doi 10.3233/JND-221512.
NMD patients have an increased risk of complications during surgery due to having heart and/or respiratory problems. Some are treated with drugs that can cause problems (e.g. steroids for Duchenne – which means the body cannot produce its own steroids during stress- like surgery).
NMD patients are at risk of developing malignant hyperthermia. There are hundreds of different NMDs, and each of them are rare. As such, anesthesiologists will have little familiarity with these diseases except in expert centers.
Authors did an inventory of how familiar neurologists and anesthesiologists in the Netherlands were with guidelines for NMD patient surgery in preparation for an ENMC meeting. 83 departments replied, mostly neurology and anesthesiology, mostly from general hospitals.
93% of respondents indicated the guideline for NMD perioperative care was very good, however, it was not available in many hospitals or health care providers were not aware it existed. Authors also investigated the use of an emergency card -surgery is often needed after an emergency.
While planned surgery will often be in an expert center, emergency surgery will be in the hospital that is closest by. Many general hospital health care providers were not familiar with an emergency card. Authors conclude multidisciplinary meetings are needed to improve things.
Awareness needs to be raised of NMD risks & considerations for anesthesia & the fact that emergency cards need to be considered. Authors point out that this was an evaluation in only one country. However, I have been to enough patient meetings to know it is a wider spread issue.
Good effort on the authors and also good they make this problem public. I am not an anesthesiologist obviously, but hope someone who knows more can outline what specific risks and considerations there are for Duchenne with regards to surgery and anesthesia.
Comments from Elizabeth Vroom, World Duchenne Organization Chair:
Thanks to Prof Aartsma-Rus and the authors, this work is really appreciated. As we see here ‘peri-operative’ is often used for pre-operative care (and during the operation) while we as patient community see the serious problems (sometimes even fatal) are happening post-operative.
DMD patients need specialized care post-operative by clinicians and caregivers informed about all the risks for this group. Ketoacidosis being one of them, another is Fat Embolism Syndrome after a fall, need for stress dosis steroids in case of infections, just to mention a few.