UPDATED Emergency and Surgery Protocol for MCAS and ME

I am reposting this post because I’ve updated my protocol to include some info from my doctor, David Kaufman. There will be another version once I finish my EDS addendum (although there is some EDS info in this protocol, like cervical spine precautions during intubation and bleeding risks, I wanted to separate the bulk of EDS info because it is not something that weighs heavily in my particular case).

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I started writing an emergency protocol back in 2015 when my mast cell reactions were scaring me with their unpredictability. I wanted something comprehensive, in writing, for anesthesia teams in the case of a planned surgery, but also something that my husband could hand to paramedics or emergency room doctors, if I couldn’t speak for myself. It was a massive undertaking because I tracked down every link and reference I could find about medication and surgery precautions for patients with mast cell diseases and ME. I wanted to gather all the information that was pertinent to me — my particular case — and edit it down to something manageable. I put together something passable and then moved it to the back burner for the last 4 years.

Last week I saw a new GI doctor who was emphatic that I get a colonoscopy and endoscopy at the same time and with anesthesia. I have been completely enema-dependent for years and, honestly, it’s exhausting. My previous GI doctor told me it was due to anatomical abnormalities (an MRI found pelvic floor dysfunction with cystocele, rectocele, sigmoidocele) and that I’d likely need enemas for the rest of my life, but it feels like the issues are getting worse and the new doctor didn’t want to throw medications at the problem without knowing exactly what she’s dealing with.

I cannot imagine voluntarily going under anesthesia. All of my worst reactions in the past 7 years have been to medications and my fear of trying new ones — especially intravenous medications — is so pronounced that I vowed only to agree to anesthesia if I was in a life-threatening situation (or couldn’t speak for myself). How could I be lying on a gurney with a peripheral IV, knowing they are about to inject multiple anesthetic drugs and not jump up and run out of the room? I wouldn’t be able to advocate for myself… I could die for a colonoscopy! So, I left the appointment with a sense of doom that only deepened when I started to feel a new ache in my lower abdomen. It got progressively worse over 3 days, the ache turned to pain and, what I thought of as run-of-the-mill bowel inflammation started to seem like something else. Gallstones? Bladder infection? I got out my emergency protocol notes and spent about 20 hours over the next few days rewriting everything, feeling like I might be working against the clock if this was something like appendicitis. Then I woke up last Sunday to such severe lower abdominal pain that I couldn’t move, could barely breathe or speak. I was shaking all over, in a cold sweat, nauseous and felt like I was on the brink of passing out. My husband wanted to call an ambulance, but I said no, hoping it was some sort of spasm that would pass. And it did… but not entirely. The ache and twinging remained for a few more days. It’s gone now and I think it was my dastardly bowels, after all, but it was bad and it scared me. It’s like the gods heard me say, “no way am I getting a colonoscopy” and decided to stab and twist their Elizabeth voodoo doll to make sure I got the point that there’s a problem I can’t continue to ignore.

The upshot of all this is, I finished the emergency protocol and I wanted to share it here, in case it could be useful to anyone else. There are a few important points about it, though:

  1. When I started, it was for personal use and I didn’t keep track of references. I will go back and gather all the links and add them to this article, but I have no idea how long it will take me and I wanted to share this sooner, rather than later. If you see your own information here without credit, please understand I will add a link to your article/blog/website! If you want me to do it asap, feel free to leave a comment.
  2. This protocol concentrates heavily on mast cell precautions because MCAS has caused my life-threatening reactions such as anaphylaxis and profound hypotension. It does not mention ME or CFS, although I researched and included ME resources, such as Dr. Lapp’s recommendations (Appendix E of the Primer for Clinical Practicioners).
  3. I have an EDS diagnosis (Ehlers-Danlos Syndrome — a connective tissue disorder), which can cause serious surgical complications. There are a lot of guidelines out there for EDS patients and, as yet, I have not researched any of them. It wasn’t until recently that I started to take this diagnosis more seriously and I still haven’t had the gumption to jump down the research rabbit hole, but, once I do, I will be updating my surgery protocol with any additional EDS precautions that are pertinent to my situation.
  4. It bears repeating: This is not medical advice of any kind. This is my personal protocol, for my personal situation. You may be more or less reactive than I am, you may have normal or high blood pressure or you may be far more disabled and need many more accommodations… But, I hope it can be of use as a jumping-off point. Please consult with your doctor.
  5. The link below is a printable pdf file, which is formatted properly, but if you need it in a different format so that you can copy and paste certain parts into your own protocol, don’t hesitate to leave a comment or email me at akaemilo@gmail.com, and I will send you a Word doc or Google doc version.

 

Updated Elizabeth Milo Emergency and Surgery Protocol

 

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Surgery and ME/CFS

After two weeks of wonderful, solid sleep with few awakenings, I was beginning to see the light. Over the long weekend I started to feel more normal than I have felt in months ~ maybe a year. I could do so much more during the day and I was still feeling alright before bed. I didn’t have any periods of utter exhaustion or flu-type feelings and my brain was firing on more than one cylinder (not all cylinders, but obviously more than usual). My physical therapist and I always talk about movies, tv shows, music and books and I can never come up with names or titles: “Oh, you should read… I can’t think of the name… it’s written by what’shisname… you know, the American guy that was living in England…” I get so frustrated. There have been many times I wish I could text him after I get home and can look up the ten things I was reaching for. Yesterday, my brain was a smooth operation. It truly felt like somebody had lubricated the synapses. There was a sense of physical spaciousness. It was a well-oiled, humming machine, almost like my healthy days. “Edward Norton was great in Primal Fear. I loved him in American History X… Yes, I adore Kevin Spacey. He was so good in American Beauty. And Seven! Oh, and the David Mamet play… Glengarry Glen Ross ~ so good! And Swimming with Sharks. I’d love to be able to see him in Iceman Cometh on stage…” All those names! They just came to me! No searching, no hard grinding mental gears, no giving up in frustration. It just illustrated the limits with which I’ve been living.

Anyway, last night I didn’t sleep and I feel dreadful today and my head hurts and my brain hurts and I fear the worst for a downhill turn. I got 4 very broken, very uncomfortable hours of sleep because, in the middle of the night, I woke up with a terrible pain in my abdomen. It is a recurrent sharp stabbing in the upper right quadrant, which has me grimacing and sucking in air every few minutes, trying not to gasp or moan so my dog doesn’t panic (which he does when I’m in pain). Throughout the night, I did everything I could think of: walked, sat, breathing exercises, massaged, drank water… Nothing helped and it is still with me now, nine hours later and definitely has me worried. I assumed it is a issue in my bowel because that is where all my problems lie, but I spent the night lying in the dark quiet, worrying that is my appendix (also, I had a bowel movement and nothing changed). I’m not vomiting and I don’t think I have a fever, so I’m not jumping to see a doctor. But the pain hasn’t dulled at all and I am so, so tired. If it continues into tonight, I won’t get any sleep again.

As I lay there last night, I was tormented by the thought that it would suddenly get more serious and I would need surgery for something. I thought about all the info that I would want doctors and anesthesiologists to know in an emergency situation and decided I had to get up and write a doc that my husband could produce if I were incapacitated. Below is what I put together and I thought it might help someone out there. I wrote my own list and, afterwards, I read Dr. Cheney’s and Dr. Lapp’s advice online (to make my list more thorough) and it is incredible how closely I fit the ME/CFS mold. After two years it still amazes me when my health history PRE-ME fits all the symptoms and idiosyncrasies. For example, vasodilators are problematic to ME patients and I already knew this was a problem for me before becoming sick because of my history with idiopathic anaphylaxis and alcohol causing collapse. Also, the doctors mention sensitivity to epinephrine and I have always told my dentists not to use epinephrine in my shots ~ it has been a nusance for them because they have to give me injections over and over again as my body metabolizes the anesthesia so quickly without the epi. And I had low blood pressure and experienced vasovagal syncope decades before I came down with ME, so reading that Dr. Lapp says “Up to 97% of persons with CFS demonstrate vasovagal syncope” amazes me … still.

I would love to know if anyone has any more information for safe surgeries and/or hospital stays. I’m hoping preparing for emergencies can mitigate long-term crashes.

Here is Dr. Cheney’s advice for surgery and here is Dr. Lapp’s (they’re very similar). I also took Sue Jackson’s advice and made the first sentence: “The most important considerations are…”

The most important considerations are IV fluids, avoiding vasodilators and histamine-releasing agents, and my hyper-sensitivity to medications.

I have a history of hypoglycemia, idiopathic anaphylaxis, autoimmune urticaria and angioedema, Hashimoto’s, vasovagal syncope.

I am allergic to NSAIDS and CODEINE/HYDROCODONE and have other presumed allergies which may have caused tongue swelling (see attached list).

I have orthostatic intolerance (OI) and vasovagal syncope: low blood volume, low blood pressure, high heart rate when standing/moving. Please give me extra saline IVs. Care should be taken to give me adequate hydration prior to surgery and avoid drugs that stimulate neurogenic syncope or lower blood pressure. Syncope may be precipitated by cathecholamines (epinephrine), sympathomimetics (isoproterenol), and vasodilators (nitric oxide, nitroglycerin, a-blockers, and hypotensive agents).

I am extremely sensitive to drugs, usually taking ¼ doses or children’s doses. Please use all drugs sparingly until my reaction can be assessed and do not over-medicate me.

Vasodilators, such as nitrous oxide, should not be used because of my history with autoimmune angioedema, anaphylaxis and orthostatic intolerance.

Use anesthesia that does not release histamine: Histamine-releasing anesthetic agents (such as sodium pentothal) and muscle relaxants (Curare, Tracrium, and Mevacurium) are best avoided because of my history of idiopathic anaphylaxis and allergies.

Use a non-hepatic anesthesia: Potentially hepatotoxic anesthetic gases should not be used, such as Halothane.

BEFORE SURGERY: Serum electrolytes, magnesium and potassium levels should be checked preoperatively and these minerals replenished if borderline or low. Intracellular magnesium or potassium depletion could potentially lead to cardiac arrhythmias under anesthesia. A liver panel and a random serum cortisol should be checked prior to any general anesthesia. 24-hour urine cortisol is recommended before and after surgery.

I have a sensitivity to Epinephrine. For local anesthesia, perhaps use Lidocaine with no epinephrine.

I have a cervical spine injury. Please be careful and gentle when intubating!

It would be wise to keep me on oxygen the entire time I am in the hospital.

Prescription and over the counter medicines and supplements: Please see attached list.