Emergency and Surgery Protocol for MCAS and ME

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 I also wrote the protocol in its entirety on this page so others could copy and paste anything they wanted into their own protocols.

Elizabeth Milo Emergency and Surgery Protocol

IMG_20150105_132432-1

The most important considerations in emergency situations and for surgery are:

  • My reactivity and hyper-sensitivity to medications, which necessitates very low dose and slow administration (see attached allergy listI have reacted to Morphine and Morphine derivatives with breathing difficulties).
  • I HAVE NEVER HAD ANESTHESIA and cannot predict how I will respond to medications.
  • Maintaining my very low blood pressure.
  • My history of idiopathic and atypical anaphylaxis with syncope.
  • Avoiding vasodilators and histamine-releasing agents.
  • I have cervical spine instability and care must be taken not to damage or hyperextend my neck.

For a patient with mast cell disease, the stress of surgery is compounded by the possibility of complications such as anaphylaxis, cardiovascular collapse, increased bleeding and even death. Therefore, general anesthesia is considered a high-risk procedure in patients with mast cell disease. It is critical that all members of the patient’s operating team take proper precautions before, during, and after surgery to protect against potentially life-threatening mast cell reactions.

An experienced anesthesiologist is aware of medications known to cause mast cell degranulation and medications that stabilize mast cells. If the patient is satisfied that their anesthesiologist fully understands the importance of planning around mast cell disease, it will go a long way toward calming the patient, which in turn may reduce mast cell mediator release. Bear in mind that some of the most common mast cell triggers are medications, pain, stress, hormones, temperature extremes, molds, perfumes, cleaning products and detergents.

BEFORE SURGERY:

Review medication and supplement list with the surgical team. I will stop as many medications and supplements as possible at least a week in advance, especially ones that lower blood pressure (vasodilators such as aspirin, nitroglycerin, vitamin E).

Discuss premedications. Some procedures require the patient not to take anything by mouth including medications after midnight the night before surgery. In this case, discuss administering H1- and H2-blockers intravenously prior to the surgery (note: I have never had IV antihistamines, so this is a concern). Premedication with corticosteroids may also be indicated. Ask anesthesia team and surgeon if I am allowed to drink clear fluids before surgery and be sure to be adequately hydrated.

Discuss medications that will be used during surgery. I have mast cell activation syndrome and a history of idiopathic anaphylaxis. Anaphylactic episodes are generally treated with intravenous H1- and H2-blockers and IM epinephrine, if there is hemodynamic instability or respiratory distress. In addition, IV fluids and other support and resuscitation measures should be undertaken.

I am unusually reactive to small amounts of medications. All medications should only be used if absolutely necessary and then used sparingly and in small doses until my response can be assessed. For example, I have had reactions to every benzodiazepine I’ve tried (tongue swelling, respiratory distress, migraine) and am very sensitive to even antihistamines. I have autonomic nervous system dysfunction. Due to this, anesthetics can cause gastro-esophageal reflux, tachyarrhythmias and difficulty maintaining blood pressure. It may be prudent to perform a graded challenge procedure in the hospital under the supervision of an allergist and an anesthesiologist for certain medications if there is no history of exposure to that medication. This procedure usually starts with scratching the skin with a small amount of medication followed by injection of increasing amounts with careful monitoring after each injection. 

I have very low blood pressure and probable depleted blood volume. Intraoperative hypotension is one of the most encountered factors associated with death related to anesthesia. Consider an intra-arterial catheter for continuous blood pressure monitoring during surgery (as opposed to a cuff). Discuss how hypotension will be treated during surgery (print study).

Controlling the depth of anesthesia limits the hypotensive effect of anesthetic drugs. Deep hypnosis (BIS <45) has been associated with postoperative complications and mortality. To limit hypotension induced by intravenous drugs, anesthesia should be titrated to clinical response. To guide induction anesthesia and identify the depth of anesthesia, consider bispectral index (BIS) monitoring.

Discuss continuous IV hydration to maintain blood pressure, however, please note that I have had reactions to IV fluids in the past due to running them too quickly or the saline being too cold. I typically run 1 liter of B. Braun Excel normal saline over a maximum of 4 hours. Fluids should be warmed in a 35-40 degree celsius water bath.

Discuss pain control: I have had reactions to Morphine and its derivatives and NSAIDS.

I have low cortisol and take daily low-dose hydrocortisone. Consider a 24-hour urine free cortisol level before and after surgery. A liver panel and serum cortisol should also be checked prior to any general anesthesia. I will double or triple my hydrocortisone dose before and after surgery.

Check serum electrolytes, in particular red blood cell magnesium (an intracellular test) and serum potassium and replenish if borderline or low. Low magnesium or potassium depletion could potentially lead to cardiac arrhythmias under anesthesia. Consider potassium 10 mEq, 1 tablet BID, and magnesium sulphate 50% solution, 2cc. IM 24 hours prior to surgery.

I have sleep apnea and asthma. Discuss the use of oxygen throughout procedure (use a disposable mask, not a reusable one in case of sensitivity to disinfectants). A preoperative lung vital capacity could be helpful. (The marker for real difficulty is thought to be a VC <1.0 liter. Such a patient needs good pulmonary preparation before surgery and a plan for postoperative ventilatory support.)

Discuss possible airway difficulties (review C-spine flexion-extension MRIs, if needed), such as: neck pain, cervical spine subluxation, limitation of cervical spine movement, nerve root impingement and/or spinal cord compression. I have a diagnosis of Ehlers-Danlos syndrome (EDS), TMJ disorder, cervical spinal stenosis, degenerative disc disease in my cervical spine and a previous neck injury. Care must be taken to keep my head and neck in a neutral position during surgery. Discuss wearing a soft neck collar to reduce neck hyperextension; this might help minimize inadvertent stretching of my neck muscles during intubation or use of an endoscope. To avoid neurological damage, patients with cervical spine instability should generally be intubated and positioned awake before surgery. This can also help assess the possibility of arytenoid involvement and determine the size of the glottic opening. The TMJs must be examined to ensure that mouth opening and anterior subluxation of the mandible will permit direct laryngoscopy/intubation. Also, be aware that the larynx may be displaced from its usual location by erosion and generalized collapse of the cervical vertebrae.

Pressure dressings are indicated even for minor procedures because excessive bleeding is common in mast cell disorders, even when blood counts are normal (this is related in part to the release of heparin during mast cell degranulation). For major procedures, a history of mast cell disorder should be included in bleeding risk assessment and may require special protocols. (I have included this in During Surgery discussion, also.)

The surgical team should be aware that I should not be exposed to perfume or scents at any time before, during or after the procedure.

Discuss post-operative recovery accommodations in the hospital, if needed — see After Surgery section. 

DURING SURGERY:

Constant attention from the anesthesiologist is required for the safety of a patient with mast cell disease, as some symptoms of mast cell mediator release such as flushing, hives, and early signs of obstructed breathing can be masked by surgical drapes and airway tubes.

Prior to the administration of any drugs associated with surgery, it is important that an IV is running, epinephrine is available for immediate intramuscular (IM) or IV administration, and emergency resuscitation equipment is easily accessible in case of an adverse reaction.

Be sure the patient does not become either too cold or overheated. I have subnormal body temperature (typically ~97.7). Warm blankets should be used and all IV fluids should be warmed (not over 40 degrees Celsius) before they are given. In addition, there should be a minimum of noise and bustle prior to the administration of the anesthetic in order to reduce the possibility of anxiety-triggered mast cell mediator release.

Intravenous (IV) fluids should be continuously running to keep the patient well-hydrated in all surgeries involving general anesthesia or conscious sedation. Hypovolemia resulting from blood loss or dehydration is a frequent cause of hypotension in the perioperative setting. IV access will also make it possible to immediately administer any emergency medications that may be needed. Hypotension episodes should be promptly treated by intravenous vasopressors — and/or antihistamines/epinephrine, in the case of anaphylaxis. 

Although the diagnosis of anaphylaxis usually depends on the involvement of 2 organ systems, even if it presents with 1 organ system, such as the skin, epinephrine administration may be indicated. Anaphylaxis may present as an acute cardiac or respiratory event, with hypotension as the only manifestation. Should anaphylaxis occur during surgery, the drug thought to be responsible should be discontinued immediately and rescue medications should be administered (H1 and H2 antihistamines, corticosteroids, airway support with 100% oxygen, IV replacement fluids to compensate for dilated blood vessels, bronchodilators). Because of the risk of potentially lethal arrhythmias, IV epinephrine should only be administered in profoundly hypotensive patients or patients in cardio/respiratory arrest who have failed to respond to IV volume replacement and several IM doses of epinephrine. *I am extremely sensitive to epinephrine, so even IM epinephrine should only be used if completely necessary. I am also extremely sensitive to antihistamines, so care must be taken to use the lowest dose possible and push very slowly. Vigilantly monitor me for reactions to rescue medications, also.*

*See anaphylaxis protocol below*

It is important to keep in mind that not all hypotensive episodes during surgery are due to mast cell degranulation/anaphylaxis. It would be ideal to get blood tests during a reaction, especially tryptase (see recommended tests below).

One of the most important considerations during anesthesia is maintaining a neutral neck position. A hyperextended neck can increase spinal cord compression and this, coupled with hypotension, which decreases blood flow to the spinal cord, can have a severe effect. Due to EDS and osteoporosis, my head, neck, pelvis and limbs should be moved carefully (limbs kept close to my body). 

Excessive bleeding is common in mast cell disorders, even when blood counts are normal. Pressure dressings are indicated even for minor procedures.

AFTER SURGERY:

If the patient is being admitted to the hospital, discuss what is needed for a safe recovery:

  • Request a private room or a sound-proof door (versus an open curtain) and placement on a quiet ward.
  • Explain any sound, light, or chemical/fragrance sensitivities and the need for scent-free nurses (a sign next to the patient’s door can help remind nurses changing shifts).
  • Discuss any food sensitivities.
  • Discuss temperature sensitivities and the need for extra blankets or adjustment of the thermostat.
  • Ask whether it’s possible to minimize nighttime disruptions (for example, middle of the night blood pressure monitoring).
  • Discuss the potential need for extra recovery time in hospital or for a reclining wheelchair or gurney during discharge from the hospital.
  • Bring your own soap, hand sanitizer and bedding.

______________________________________________________________

MEDICATIONS:

Consider using (but administer with extreme caution):

  • Diprivan (propofol) as the induction agent. (However, Propofol, used alone at induction of anesthesia, promotes a significant decrease in arterial blood pressure compared with thiopentone or etomidate, even with reduced doses.)
  • Versed (midazolam)
  • Fentanyl (a short-acting narcotic; may require adjunct treatment with Zofran)
  • Droperidol (an anti-nausea agent)
  • Tylenol
  • Tramadol

Second choice is ketamine or benzodiazepines, such as midazolam (however, I have had reactions to benzos in the past).

Atarax (Hydroxyzine) should only be used in an emergency. It can cause heart palpitations and arrhythmias. Doxepin should only be used at very low doses (<5mg — I have never taken it).

A small amount of Diazamuls, a preservative-free Valium emulsion, could be considered for long surgeries where the possibility of “awareness” is a concern. This drug can also be used pre- and post-operatively for sedation, and for cramping in certain types of procedures.

Use IV preparations without preservatives or alcohol. Use B. Braun Excel normal saline. Do not use lactated ringers, if possible.

Oxygen should be used throughout procedure (possibly at 5 liters pre and post-operatively). 

Do not use epinephrine except in life-saving situation. But Epinephrine must be on hand during the procedure.

Do not use medications that contain preservatives, artificial colorings, dyes, flavorings or alcohol (topical alcohol, as in alcohol prep pads, is fine, as is povidone iodine). Single-use vials are usually preservative-free. 

Do not use medications that stimulate neurogenic syncope or lower blood pressure: 

  • No cathecholamines (Epinephrine)
  • No sympathomimetics (Isoproterenol)
  • No vasodilators (nitric oxide, nitroglycerin, alpha-blockers, and hypotensive agents)

I have orthostatic hypotension (OI), chronically low blood pressure and a history of vasovagal syncope. Many people with dysautonomia have low plasma volumes, low RBC mass, and venous pooling and the above drugs may cause or exacerbate these conditions. 

Do not use histamine-releasing anesthetic agents:

  • No Sodium Pentothal or other thiobarbituates (Thiamylal, Thiobarbital etc.)

Do not use histamine-releasing muscle relaxants:

  • No muscle relaxants in the Curare family, such as Tracrium and Mevacurium.
  • No Succinylcholine ((Anectine) often causes severe, generalized muscle pain, post-op.)
  • No D-tubocurarine
  • No Metocurine
  • No Doxacurium
  • No Atracurium
  • No Mivacurium
  • No Rocuronium

Muscle relaxants are the most likely group of the anesthetic drugs to cause anaphylaxis. If muscle relaxants must be used, the current recommendation is to start with a quarter to half the usual dose of whatever you’re using. Intraoperative neuromuscular monitoring helps prevent overdose. Consider using “nondepolarizing” muscle relaxants, such as pancuronium or vercuronium. 

Do not use anesthetic gasses:

  • No Halothane
  • No Enflurane
  • No Isoflurane
  • No Desflurane

These can cause problems for patients with chemical sensitivities and are highly hepatoxic. An alternative might be continuous IV infusion of a short acting anesthetic by injection or a continuous flow pump. If necessary, consider Sevoflurane.

Do not use:

  • No Morphine, codeine or morphine derivatives
  • No Opioids
  • No Vancomycin
  • No Ethanol
  • No Dextran
  • No Non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and Toradol
  • No Polymyxin B
  • No Amphoteracin B
  • No Quinine
  • No Dextromethorphan
  • No Alpha-adrenergic blockers
  • No Beta-adrenergic blockers
  • No Anticholinergic drugs, if possible, such as Hydroxyzine and Cimetidine
  • No decongestants, cold or flu medications
  • No SSRIs

Do not use local anesthetics, if possible. If needed, do not use ones with preservatives or epinephrine:

  • No Procaine
  • No Chloroprocaine
  • No Tetracaine
  • No Benzocaine

Consider using:

  • Lidocaine
  • Mepivacaine
  • Prilocaine
  • Bupivacaine
  • Levobupivacaine
  • Ropivacaine

Antibiotics are not well tolerated by mast cell patients, however, these are some that have been reported as safest by patients (use with caution):

  • Clindamycin
  • Cephamandole
  • Azithromycin (Z-pak)/Zithromycin/Zithromax
  • Keflex
  • Omnicef
  • Beconase AQ
  • Biaxin (eg. Clarithromycin)
  • Rhinocort

_________________________________________________________

MEDICAL EMERGENCY RESPONSE PLAN for Mast Cell Activation and Anaphylaxis:

If the patient presents with flushing, rash, hives, swelling, abdominal pain, nausea, vomiting, shortness of breath, wheezing or hypotension, respond with the following: 

Administer:

  • Epinephrine (preservative-free) 0.3 cc of 1:1000 (1mg/1ml) solution = 0.2mg–0.3mg IM (Vastus Lateralis muscle) and repeat 3x at 5–15 minute intervals if BP < 90 systolic (my BP averages 86/55).
  • Benadryl (diphenhydramine) 25mg (max 50mg) orally, IM or very slow IV push, diluted in normal saline over 2-5 minutes. Can repeat every 2–4 hours.
  • Solu-Medrol (methylprednisolone) 120mg (40mg for children under 12) IV/IM (I have never had Solumedrol, so monitor closely. My usual steroid is oral Prednisone 6mg)
  • H2 antagonist, such as Famotidine IV, should also be given (I have never had this, either — my usual H2 blocker is oral Zantac 150mg). 
  • Oxygen 100% by mask or nasal canula.
  • Albuterol nebulizatio,n if wheezing present.
  • IV fluids (warmed in 35–40 degree celsius bath and run as slowly as possible for the situation).

*All mast cell patients must be monitored for biphasic (rebound) reactions.*

Pre-medication for major and minor procedures:

  • Prednisone 50mg orally 24 hours and 1–2 hours prior to surgery (I’ve never had more than 7mg)
  • Benadryl (diphenhydramine) 25–50mg orally, 1 hour prior to surgery
  • Zantac (ranitidine) 150mg orally, 1 hour prior to surgery
  • Singulair (montelukast) 10mg orally (5mg for children under 12), 1 hour prior to surgery (I’ve never had Singulair)

*Note: my current premedication protocol is 25mg oral diphenhydramine (Benadryl brand dye-free capsules only), 150mg oral ranitidine (Zantac brand), 6mg oral Prednisone and 325mg acetaminophen (Tylenol brand).**

Drugs to be avoided:

  • Aspirin and non-steroidal anti-inflammatory (NSAIDS) medications
  • Morphine, codeine derivatives
  • Vancomycin

_______________________________________________________

LABORATORY TESTS TO RUN IN THE EMERGENCY ROOM DURING AN ACUTE MAST CELL REACTION OR ANAPHYLAXIS:

  1. Serum Tryptase — upon arrival in the ER and three hours later.
  2. 24 hour urines for:
    1. n-methyl histamine
    2. prostaglandin D2 ( PGD2)
    3. 11-beta prostaglandin F2 alpha (BPG-F2A)
  3. CMP
  4. CBC with differential

Also consider:

  1. Chilled plasma histamine
  2. Chilled plasma prostaglandin D2 (PGD2)
  3. Chilled plasma heparin
  4. Serum chromogranin A
  5. Serum magnesium

If possible, hourly determinations of serum tryptase, plasma PGD2 and histamine, and spot urinary PGD2 and N-methylhistamine should be pursued at baseline and over the next 2–3 hours as a reaction evolves.

SPECIMEN HANDLING ISSUES:

The short half-lives and thermolability of many mast cell (MC) mediators require continuous specimen chilling throughout collection, storage, and transport. Particularly with regard to 24-h urine collections for MC mediator testing, patients should be carefully educated to pre-chill the collection container overnight before beginning the collection and then to keep the container continuously chilled while following an otherwise standard 24-h urine collection protocol; the container should be removed from the refrigerator or ice chest only when imminently needed and should be returned to chilling as soon as possible. Patients should also be cautioned to maintain the container in a chilled environment throughout transport. We recommend the container be placed in a bag filled with ice and sealed, with the bag then placed in an ice chest filled with ice and sealed for transport to the accessioning lab, whereupon the bag can be removed from the chest and provided to the technician with a reminder of the criticality of keeping the specimen chilled. Laboratory staff often are unfamiliar with MC mediator testing, previously a rarely undertaken endeavor. Consideration should be given by the diagnostician to sharing with laboratory personnel the ultimate clinical goal of such testing and the importance of maintaining thermal integrity of these specimens at all times, including at the time of initial accessioning as well as when packing specimens for transport to reference laboratories that may be thousands of miles distant, transits that may involve long periods sitting in unventilated cargo containers on hot tarmacs. Use of well-insulated containers, and liberal placement of cold packs in the insulated container, should be de rigueur when packing such specimens for long-distance transport.

____________________________________________________

You MUST have your allergist or primary care provider sign the bottom of this form stating that

he or she will be responsible for the follow-up on the 24 hour urine collections. Otherwise, the ER physicians will be reluctant to order them since they cannot be sure of follow-up care. Remember to contact your physician for follow-up after discharge.

***********************************************

I agree to provide follow-up care for my patient,____________________________________, and will obtain the results of the 24-hour urine collections that were initiated in the emergency room setting and will provide appropriate care based on the results.

__________________________________________________________

Printed Name of Physician

__________________________________________________________

Signature of Physician Date

Contact Address_________________________________________

__________________________________________________________

Phone Number__________________________________________

Fax Number_____________________________________________

____________________________________________________

KOUNIS SYNDROME IN MAST CELL PATIENTS

Acute coronary syndromes can occur in allergic and anaphylactic reactions. One example, called Kounis Syndrome, is highly likely in patients with a wide variety of mast cell activation disorders and can affect patients of any age. The main triggers of Kounis syndrome are drugs, environmental exposures, and various pre-existing conditions. When patients are on a protocol exposing them to many medications, the cascade leading to anaphylaxis and Kounis syndrome can be very rapid, with the heart and coronary arteries as the primary target. Multiple mast cell mediators have direct action on coronary vessels and together result in hyperresponsiveness of mast cells, which can result in the Kounis syndrome cascade. Please note: Coronary artery spasm induced by mast cell mediators may initiate Takotsubo Syndrome or stress-induced cardiomyopathy during anaphylactic reactions.

Type 1: Normal coronary arteries, no coronary disease, no predisposing conditions; acute allergic attacks resulting in coronary vasospasms without elevations in cardiac enzymes OR coronary vasospasm with myocardial infarction with elevation of cardiac enzymes and troponins.

Treatment of the allergic episode can terminate the type 1 variant

  • Corticosteroids
  • H1 and H2 blockers
  • Vasodilators such as calcium channel blockers and nitrates can decrease hypersensitivity induced vasospasms

Type 2: Quiescent pre-existing atheromatous disease in whom acute allergic attacks can induce either vasospastic angina or plaque erosion, or rupture manifesting acute myocardial infarction.

Treatment of acute coronary event comes first, then treat allergic attack:

  • acute coronary event protocol
  • Corticosteroids
  • H1 and H2 blockers

Type 3: Stent thrombosis with eosinophils and mast cells identified on pathology (Giemsa, hematoxylin-eosin stain).

Treatment of stent thrombosis with allergic attack:

  • Corticosteroids
  • H1 and H2 blockers
  • Mast cell stabilizers
  • Biopsy of thrombus stained for mast cells and eosinophils. Nitroglycerin causes decreased blood pressure and increased heart rate. 

Fentanyl is the opiate with the best profile for mast cell patients; administer with extreme caution.

_______________________________________________________

 

Nonimmunologic Histamine Releasers and Other Degranulation Triggers

Foods and misc Chemicals & Drugs Physical Agents
Polymers
Dextran
Compound 48/80
Calcium ionophore A23187
Bacterial Toxins
Snake venoms
Biologic polypeptides
Ascaris species
Jellyfish
Portuguese man-of-war
Spines & hairs of Caterpillars
Nettles
MothsFoods high in Histamine or Tyramine, Gluten, Soy, Salicylates (eg: fish, strawberries, egg whites, tomato)
Foods containing preservatives, artificial colorings or flavorings.
Aspirin
Alcohol
Opiates (Codeine, Morphine, Percocet, Oxy etc)
NSAIDs (Advil, Motrin etc)
Polymyxin B sulfate
Colistin sulfate
Thiamine hydrochloride
Tyramine
Trimethaphan and Trimetaphan
Tubocurarine chloride
Quinine sulfate
Papaverine
Dipyridamole (Persantine)
Iodine-based radiographic dyes
Scopolamine hydrochloride
Neuromuscular blocking agents (eg: Dexamethonium, Gallamine triethiodide etc)
Dextromethorphan (cough suppressant)
Stilbamadine isethionate
Chlortetracycline bisulfate
Amphetamines
Hydralazine hydrochloride
Tolazoline hydrochloride
Cinnamaldehyde
Cinnamic acid
Benzoic acid
Sodium benzoate
Fungal infection drugs
Local anesthetics: lidocaine etc. (any amino amide-type)
Dermatographia (Darier’s Sign)
Pressure
Cold
Heat
Exercise
Hot baths/showers
Fever
Occlusive dressings
Eating spicy foods
Emotional stress
Solar light waves
Water
Vibration
Insect bites (Wasp, Hornet, Bee, Mosquito)
Infections
Inhalants
Smoke (wood burning, cigarette, marijuana) Scents/Odors/Perfume
Cleaning products and chemicals
Latex
Cosmetics/Creams containing salicylates
Preservatives, Additives, Dyes, Artificial flavours/colouring
Vasculitis
Internal diseases

 

Screenshot 2019-07-13 at 11.45.21 PM

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SIBO Antibiotic Failure in Half a Milliliter.

Referring to my last post:

I am devastated. But allow me to give you some backstory, so you understand my emotional reaction to a failed drug trial. It took me a year to try the SIBO protocol, but, during that year, I wasn’t just sitting around, waiting to get the nerve up — there was so much time, energy and money invested in procuring the safest and smartest medications for me.

Initially, Dr. K wanted me to take the two gut antibiotics without having the SIBO test, but I asked if I could do the test first ($180) because I had been negative for SIBO a few years ago. The preparatory diet was brutal for me this time (when I did it in 2014, I don’t remember it being a big deal). I had to eat only meat, eggs and rice for two full days because of my chronic constipation and it made me very sick and weak, nauseous, hungry and shaky. During the test, I had a massive blood sugar crash, but you’re not meant to eat or drink while collecting breath samples, so I waited too long and got more and more hypoglycemic, finally giving in to apple juice, but the whole experience took a toll. So, there was that.

Then there was the energy involved getting the Rifaximin: Asking my doctor to send in a pre-authorization, getting refused, sending in an appeal, getting refused, a third party appeal and refusal — all of this taking so much time in between each step. Calling around to pharmacies to see if there was anywhere that sold it for less than $1,500. Not wanting to buy the generic for $200 because it has colourings that I avoid. Asking my doctor to send a sample of the tablets, so I could try them before buying them. Waiting on that sample to arrive. Waiting for a good day to try it — a day I felt strong enough with no other conflicting variables like a migraine or a day I was doing my infusion. Calling a pharmacist to see if I could cut the tablet (they said no because it’s enteric coated to stay in tact until it reaches your gut), but cutting it anyway because I have to start with a sliver and the worst that can happen is it’s not effective and who cares? — this is just a test. Taking bigger and bigger slivers over the course of a week. Deciding it’s okay and safe to order from the online pharmacy in Singapore and, because so much time has gone by and it takes another 2-4 weeks for delivery from the time of order, having it sent to our California address.

In the meantime, once I knew I wouldn’t react to the Rifaximin, I started calling around about the Vancomycin (because I’m meant to take them concurrently). I called so many compounding pharmacies, so much time invested, taking notes on brands, ingredients, prices, my options for liquids or capsules. Then, when I had found the cheapest ($200) and most competent-sounding pharmacy, I consulted with the pharmacist over and over about the details: first, about ingredients (no flavourings, no preservatives, compounded only in sterile water). Then about the timeline, explaining that I couldn’t start at full dose, that it would take me a few weeks to titrate up and is there a way to prolong the 2-week shelf life? He said he could freeze it, extending the “discard by” date from 14 to 90 days. Then we brainstormed some more and decided to freeze it in 4 bottles, so I only needed to defrost one at a time, keeping the others preserved. Then he said he should make it at the last minute, to keep it fresh as long as possible. My husband drove across town on the day we were leaving for California to pick it up. I kept it in a cooler with ice packs during our road trip and managed it like a bird on a nest: tending to it, moving it out of the sun, re-freezing the ice packs each night. And then, once we were here, I just waited for the Rifaximin delivery so I could start them both together.

So much goes into this sort of thing, aside from the $580. Not to mention my hopes. For all my fear of repercussions, once I decide to do something, I put nothing but a positive and excited spin on things. Taking antibiotics for the first time could be a game changer — like antivirals have been for so many. I’ve never addressed my gut and I certainly don’t have a strict diet, so there’s hope for positive change there. What if my brain symptoms are better and my sleep is better and I don’t have to do enemas anymore? I am an expert at swallowing something and forgetting about it, so I’m not nervous or over-analyzing my body. Down the hatch and that’s it. Don’t pay attention. But last night the Vanco got my attention.

My prescribed dosage is 30 ml a day. THIRTY. Last night, I took 0.5 ml. HALF A MILLILITER. Soon after, something started happening in my throat on the left-hand side. Then my tongue started swelling on the left. Then a headache on the left. And, finally, heart palpitations. My tongue got bigger and bigger. I was dumbfounded. If I were going to react to anything, I thought it would be the Sunset Yellow generic Singaporean Rifaximin, not the sterile water vanco that Kyle the pharmacist put so much care into!

Dumbfounded and devastated. For me, tongue swelling is as scary as it gets because it is the precursor to full-blown anaphylaxis — especially tongue swelling with head and heart involvement. The mast cell meltdowns that I experience in the night, with sweats and chills and poisoned feelings, are much worse physically, but not as serious as tongue swelling. Not as scary. All of my anaphylaxis ER visits involved tongue swelling. It’s something that can get worse quickly. So, how do I get the nerve up to try the Vanco again? Are all those frozen bottles of medication a loss? That’s what made me start crying. Not the time or money or hopes dashed, but the thought that I can’t try it again. It’s not like my hydrocortisone success story; I can’t push through. Next time, it could be much worse, like your second bee sting. My control is taken away. Even if I wanted to try again tomorrow… I can’t risk anything even akin to anaphylaxis. It’s the trauma I will always carry. If I spontaneously recovered from ME today, I would still carry the fear of anaphylaxis with me for the rest of my life, like a brown recluse spider, hiding in plain sight, threatening sickness and death when you least expect it. Damn.

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Update: A Google search shows me that people take Rifaximin without the second antibiotic. I inferred from my doctor that they had to be taken together, but maybe not. Maybe all is not lost for treatment.

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Another update: One long bath, one meditation and a good conversation with my husband later… Feel much better about the whole thing. He’s so good at saying, “don’t think about the money, let it go” and “it’s just a drop in the bucket of the last 6 years” and “move on to the next thing” and “you’re doing okay, you’re not bedbound, you’ve made improvements without this treatment.” And then I look at the vast desert sky and envision the stars and universe beyond and think about how small I am. And how lucky I am. My tongue swelling resolved with Benadryl last night and today I’m eating ice cream next to my dogs in the sun, listening to a cacophony of birds nearby and coyotes howling in the distance. 

Medication Wars: Treating SIBO and Low Cortisol

13 months after my California doctor wrote the prescriptions for two gut antibiotics to treat SIBO, today is the day I have to face the music. I’ve put it off for this long out of fear: Fear of a mast cell reaction (Rifaximin ingredients: Sunset Yellow FCF, ffs); fear of no reaction, but feeling terrible from die-off (we just arrived in the Cali desert for a month, so it’s really fear of destroying my idyllic get-away); fear of altering my microbiome for the worse, rather than the better (causing more of a candida flare, causing C. diff etc); fear of spending the money, but not not being able to take the medicine (each one was $200!). Also, although the SIBO test was “off the charts” (in my doctor’s words), I don’t have the symptoms, so fear of messing with the gut I know and creating new issues. I haven’t taken an antibiotic in almost a decade–well before I got sick–so, there’s fear there, too.

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But I see my doctor later this month and I’m determined to do the treatment before I see him. I now have both medications in front of me, money is spent, no excuses. One of them is compounded in sterile water and needs to be thrown out in a few weeks, so I’m starting now, with one drop, as soon as I stop typing… which, of course, makes me want to keep typing, keep putting it off, what else can I tell you…?
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Okay, I’ll quickly tell you a good drug story, which will bolster my confidence. The first medication I was ever prescribed after getting sick was hydrocortisone. The pharmacist said, “If it gives you a headache, let me know.” It gave me a whopping headache and, back then, I didn’t understand my reactions and how I have to start at micro-doses–I didn’t even know you could cut a tablet or open a capsule–so, I just stopped taking it after two days. The ND said she presented my case to her colleagues and everyone said, “Yes, hydrocortisone!” but it was my first experience with an ND and perhaps I didn’t fully trust her, but, more so, I didn’t want any worsening symptoms, so I just stopped going to her. That has been my MO thus far: try not to rock the boat, except very gently, over a very long period of time (and, by the way, for the most part, I have improved over the years (knock on wood, toba, toba), which has reinforced my careful tendencies).

Last year, my California MD Rxed hydrocortisone again. I tried an 1/8 of a tablet in August and felt short of breath, so didn’t take it again until 3 months later. Then I was spurred on by a receptionist at a doctor’s office who started crying (!) on the phone to me while talking about her daughter who needs hydrocortisone all day long, so I tried it again. It went okay for a few months. Then one day it made me feel gittery, spacey and short of breath again. Then, a few weeks after that, it hit me like a freight train. I wrote in my calender: “Shaky, drugged, agitated, buzzy muscles, feel like I’m on speed, then possible blood sugar crash (or maybe just still shakes from hydrocortisone). Then, after hours, a dull obvious-reaction headache and stuffed ears.”

This is what used to happen to me with antihistamines: I’d handle them for days and then, without warning, the same dose would send me into a scary cascade of anticholinergic symptoms (I still mourn the loss of Unisom, which helped a lot with sleep for a while).

But, I persevered with the hydrocortisone (yay, me!) and, last month, something clicked, I could feel it help my body. I can feel the uptick in energy and the decrease in brain symptoms. I give hydrocortisone full credit for getting me through the weeks of packing for this trip and those back-to-back high-step count days. Each morning, I marveled: I’m not bedbound, I think I can do it again. I have no side effects now and I might even try more than a 1/4 tablet. 😉

Grab that cash with both hands and make a stash.

This is a hard one to talk about. It’ll be more of a rant. And I’d like to preface it with all of the obvious about how grateful I am that we were in the economic position we were in when this illness started, how grateful I am that I had a few years of good earnings and decent savings, how grateful I am that my family is healthy and has never been bankrupted by health woes. I am, I truly am, and I think about–probably too much–what must happen to others with a severe chronic illness (that has no knowledgeable doctors and no decent treatment) who are in worse financial shape than we are, without our resources, who are in countries steeped in poverty, refugees fleeing wars… on and on… I do know how lucky I am. But, I’m scared. Mostly because I don’t know what treatment to spend money on and what to reject.

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Recently, after 6 months of immunoglobulin infusions, I got a slew of bills that I didn’t anticipate. It turns out that for my itsy bitsy dose of 5 grams each week, I pay $164 after insurance. Out-of-pocket. That’s $655 per month. If I had known this before starting, would I have done it? I don’t know. But now that this is the only treatment that has helped me, how can I stop? And I’m in this tricky spot. I have private health insurance because I was too scared Medicare wouldn’t cover these treatments (also because Medicare won’t cover acupuncture, nutritionists or physical/myofascial/craniosacral/massage therapy — some of the only things that have made a difference in my pain levels). After being told IVIG was not an option by so many doctors, knowing that I didn’t have a history of bacterial infections and a vaccine challenge is usually required for approval, and having Coram (the infusion service) tell me that Medicare hardly ever covers treatment, I was just too scared to give up the private health insurance that had already approved my treatment for the whole year. Of course, I have since heard from others that Medicare covers their IVIG or SCIG at 100%, but … how could I risk changing coverage now when my IgG levels have come up and that alone might disqualify me from continued treatment? I’ll have to revisit this next January when I’m eligible for Medicare enrollment again, but, if I’m still improving with my infusions, I don’t know how I’d take that leap of faith.

An aside for those in other countries or for those that don’t know this fucked up aspect of our healthcare system here in the U.S.: Medicare (government health coverage) isn’t free. You pay each month just as you do with private health insurance. It’s usually cheaper, but not always. It can range from $105-$771 a month, depending on your situation (the higher end is reserved for people who have not worked enough in their lifetimes to qualify. So, if you are struck down with a chronic illness as a young adult and you haven’t worked the requisite 30 quarters in a tax-paying job, you’re not married and you undoubtedly have little savings, then you get to pay the highest premium for our national health coverage– oh, but only if you’re lucky enough to be granted full disability, which very few ME/CFS/Lyme sufferers are). And don’t think that Medicare actually covers your healthcare in full, though. You will still have a deductible each year and co-insurance (the patient pays 20%, typically), you’re prescription medications aren’t paid for unless you get extra coverage and hospital stays can still leave you in horrendous debt. You can stay in a hospital for a few months for the low, low price of $1,260 (although skilled nursing facilities will be more because that price doesn’t cover people to care for you), but let’s take a pretty terrible example: 150 days in the hospital. In 2015, that would have cost you $47,565 out-of-pocket. If you had to stay any longer, all additional costs are your problem. The government washes its hands of you. But wait, there’s more! If you choose not to enroll in Medicare when you become eligible, your monthly payment when you do enroll will be higher–forever–usually 10% higher for each year you could have signed up but didn’t. In my case, if I’m covered by Medicare next year, I will be paying an extra $300/year because I didn’t enroll when I first became eligible. If I wait until 2018, I’ll pay a penalty of at least $440 that year, plus more each year as the premiums continue to rise over my lifetime. Lovely.

SO… Last month I finished up weeks of financial slog for our 2015 taxes and was happy to see our (and by our, I mean my because my husband’s medical expenses are only about a quarter of our total and that is solely health insurance premiums because he never needs a doctor, knock on wood, toba toba) out-of-pocket medical costs had come down slightly.

2012: $14,480
2013: $19,032
2014: $19,564
2015: $17,912

That doesn’t allay the fear, however. After utilities (sewer, water, garbage, recycling, gas, electricity) and mortgage payments, we’re left with about $20K a year to live on and medical expenses have been almost $20K a year since I got sick. That means most everything else–food, clothes, toiletries, dogs, phone, internet, gas for cars– comes out of our savings. I’m trying to be healthier, place fewer burdens on my system and subdue my chemical sensitivities by eating organic food, pastured meat and buying less toxic products. All of these things are more expensive. For the last year and a half, I’ve been paying $200/month for compounded medications instead of the cheap, generic, filler-filled ones. It hardly costs anything to get sick, but the system is rigged to bankrupt those that are.

I feel very fortunate that we had saved money before this happened, but it will run out eventually and I don’t want to make all the wrong decisions now because I’m frozen in fear of the future. Our day-to-day living is all-encompassing, so time slips by in survival mode and the big decisions never get discussed. I’m happy that we didn’t sell the house when I first got sick because we’re finally not under water and it might actually be worth what we owe again. But when do we sell? And do I switch back to generic meds? Do I stop supplements (around $100/month)? Do I stop seeing my doctor who doesn’t take health insurance? Do I not try human growth hormone or hyperbaric oxygen or nutritional IVs? Do I stop my immunoglobulin infusions?? Last year, I thought a time would come when we just moved somewhere very small and affordable, maybe a foreign country, and I stopped all medical visits and we tried to exist on pittance and make our money last as long as possible… But now that I’ve found a treatment that helps my functioning, I have renewed hope. Maybe I’ll be able to earn a living again if I keep making progress. How can I give up on that? Or should I accept the fact that this is as good as it’s going to get, income-wise? My husband will get older, he’ll be able to work his manual-labour job less and less and I won’t ever recover to the point of being able to hold a job… I think that’s the reality. I know a lot of people with this illness and many have made improvements, but I’m not sure if I know any that have gone back to full-time work.

So, we beat on, boats against the current, cut costs where we can, shop the deals online, grow some veggies, sell some stuff, and pray that in ten years time, the tides have changed for the better.

Title Credit

Finally getting the first immunoglobulin infusion…

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The IgG infusion didn’t happen. They called me and said they were missing some small tube or something that was needed for my pediatric dose. It’s a little frustrating since they had literally months notice, but they have been excellent through this entire process–communicative, understanding, informative–so, I’m not annoyed. They asked if I’d like to do last Thursday instead, but I didn’t want to risk any reaction with my period, which came early last month. All I know for sure about anaphylaxis and angioedema is that they happen during a perfect storm of triggers (food, mood, hormones, hydration, pain) that is very hard to predict or control, but almost always involves my menstrual cycle and that is the one thing I can avoid. In the end, my period did come early, so I’m glad I made the choice to wait on the SCIG.

Today is the day. The nurse gets here in a few hours. I’m not out of bed yet. I’m in a lot of pain today and I was awake in the night with terrible vertigo. Every time I turned my head and changed position, the room lurched and woke me. I think it’s probably from the full dose of Zyrtec and Zantac I took yesterday, which I’ve never done before, but it could very well be payback from the 4+ hour journey to the dysautonomia specialist two days ago (more on that anon).

Anyway, the ball is rolling, the die has been cast, the airplane doors are closed (that’s what I used to tell myself when I was nervous about flying–once the doors are closed, it’s out of your hands, so no point in fretting anymore), so it’s happening and I am focusing all my attention on how incredible it will be to have a treatment that might help me feel better. Honestly, I’m dreading the premedications (Benadryl, Prednisone, Zantac, Tylenol) more than the IgG. I already know they do a number on my sensitive, unable-to-detox body.

Please wish me luck and send good juju this way. It makes a difference, I know it does. Thank you for holding me up. As Clarence said, “Remember, no [wo]man is a failure who has friends. Thanks for the wings.” ❤

Addendum: it just occurred to me that I didn’t specifically tell the infusion company to tell the nurse not to wear perfume, so I called her and she said, “Oh, I do wear body spray because I hit menopause and I can smell myself.” OMG. Body spray? She kindly said she would stop by her house and take a quick shower.
“I buy whatever shampoo is on sale, so I hope it’s not too smelly,” she said.
Gulp.
“No, I’m sure it’ll be fine.”
She then said, “I understand about sensitive patients. Remind me to tell you about the lady who was severely allergic to cats.”
Oh, for fuck’s sake.
“I’m very allergic to cats,” I told her.
“Oh, I have cats, so I’ll change my clothes, too.”
This is a nightmare.
She ended with (I kid you not): “I’ll tell you some horror stories when I get there.”

I’m vacillating between guilt at putting someone out (she was SO nice and sweet), frustration at my ridiculous body and total disbelief that a home-care nurse would wear body spray to visit patients and that the office didn’t explain my sensitives to her (they also didn’t tell her about my history of idiopathic anaphylaxis or that I have two big dogs. She said she just got a name and address). Please please please let this go okay.

Second addendum: the nurse is incredibly nice and lives very close to me, so the shower wasn’t a big deal and she doesn’t have a heavy smell at all. We’re half way through the IgG and the saline fluids. All good so far. 💪

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That's my abdomen--just one site for such a small dose.