The following article was co-written by Nancy Gould, a Systemic Mastocytosis patient who was also a Retired American Nurse. See her helpful mastocytosis and mast cell activation videos on YouTube. The article below has been reviewed by Dr. Cem Akin, one of the well known Mastocytosis physicians in the USA. Nancy Gould granted permission for MSC to share this article as a safe surgery & emergency reference guide for Canadian medical professionals and Mastocytosis patients and caregivers. MSC has made minor edits to reflect Canadian medications and MedicAlert Canada in the text below.
WHAT YOU SHOULD KNOW ABOUT ANESTHESIA . IT COULD SAVE YOUR LIFE
Nancy Gould and Regis (Gigi) Park
INTRODUCTION
Surgery is a stressful experience. For a patient with mast cell disease, that stress is compounded by the possibility of complications including 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 activation.
PLANNING FOR SURGERY SHOULD BEGIN AS SOON AS THE NEED FOR SURGERY ARISES
It is imperative that good communication is established between the patient, referring physician, surgeon, anesthesiologist, nurses, and all others involved in the patient's care before, during, and after surgery. The surgeon must be aware of the patient's mast cell disease and inform themselves of the measures necessary to keep the patient as free of symptoms as possible. Additionally, the patient should contact the anesthesiologist assigned to their care as soon as possible after surgery has been scheduled. Both regional and general anesthetics can cause life-threatening complications. 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 the mast cell disease, it will go a long way toward calming the patient, which in turn may reduce mast cell mediator release.
Symptoms should be as well-controlled as possible in the days prior to surgery. The patient should carefully avoid known triggers of mast cell activity and should take their medications as prescribed. Those medications include H1 and H2 histamine receptor blockers such as Reactine (H1) and Ranitidine (H2). A mast cell stabilizer such as Nalcrom (sodium cromoglycate) or Ketotifen, and medications targeting other mast cell mediators should be taken regularly if they are part of the patient's normal drug regimen.
The patient's complete medication list should be reviewed by the surgical team prior to surgery and any necessary medication changes should be thoroughly discussed with the patient. For example, if the patient regularly takes a medication in the family known as beta blockers for blood pressure or heart rate abnormality, a change to another drug should be considered well before surgery is scheduled. Beta blockers are generally avoided in people with mast cell disease undergoing surgery because they interfere with an important natural control of mast cell activation. These drugs may also interfere with the use of epinephrine, which may be required if the patient has a major release of mast cell mediators resulting in low blood pressure during surgery. Other drugs that may interfere with control of blood pressure during surgery must be carefully reviewed by the patient's physician before the surgery. It may be necessary 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. Resuscitation equipment and drugs including epinephrine must be readily available during the procedure.
PRECAUTIONS TAKEN IN THE HOURS PRIOR TO SURGERY WILL HELP THE PATIENT GO INTO SURGERY IN THE BEST POSSIBLE CONDITION
For pre-operative control of anxiety and the reduction of mast cell activity, drugs in the benzodiazepine family (clonazepam, diazepam, midazolam, lorazepam) are usually effective. Some procedures require the patient not to take anything by mouth including medications after midnight of the night before the surgery. In this case, H1 and H2 blockers should be administered intravenously prior to the surgery. The use of corticosteroids, such as prednisone, has also been suggested although there is no evidence that the short-term use of steroids reduces the ability of mast cells to release the chemicals contained in their granules. However, corticosteroids may reduce the extent of other inflammatory reactions that result from mast cell activation.
A tube may be inserted into an artery and attached to a device allowing the anesthesiologist to vigilantly monitor blood pressure without having to periodically inflate a blood pressure cuff. In addition, a tube is inserted into a vein and securely taped in place, with intravenous (IV) fluids running to keep the patient well-hydrated in all surgeries involving general anesthesia or conscious sedation. This tube will also make it possible to immediately administer any emergency medications that may be needed.
In the operating room, the patient should not be allowed to become either too cold or over-heated. Warm blankets should be used, and all IV fluids should be warmed before they are given. In addition, there should be a minimum of noise and bustle around the patient prior to the administration of the anesthetic in order to reduce the possibility of anxiety-triggered mast cell mediator release.
EMERGENCY SURGERY
An emergency situation may arise in which a person with mast cell disease requires immediate surgery. Wearing a MedicAlert bracelet could be a life-saver if this happens. Inscriptions vary depending upon the patient's specific needs, but generally include the patient's diagnosis and drug sensitivities. Emergency response team members can access more detailed information 24 hours a day, 7 days a week from MedicAlert Canada by dialing the toll-free number on the bracelet.
It may also be helpful for mast cell patients to carry with them at all times emergency information, written on their physician's letterhead, which contains a list of current medications and other instructions for treatment in the event of severe symptoms.
DURING SURGERY
Constant attention from the anesthesiologist is required for the safety of a patient with mast cell disease during surgery, as some of the early symptoms of mast cell mediator release such as flushing, hives, and early signs of obstructed breathing can be masked by the surgical drapes covering much of the patient's skin and by the use of an airway tube during anesthesia.
Should anaphylaxis occur during surgery, the drug thought to be responsible should be discontinued immediately and epinephrine should be administered. Airway support with 100% oxygen, IV replacement fluids to compensate for dilated blood vessels, H1 and H2 antihistamines, bronchodilators, and corticosteroids may also be given. Continuous IV epinephrine and other vasopressor drugs may be necessary to keep blood pressure from falling. However, it is important to keep in mind that not all hypotensive episodes during surgery are due to mast cell degranulation and anaphylaxis. A serum tryptase level obtained during the hypotensive episode and its comparison with pre-surgery or baseline level may be helpful to determine whether the episode is due to mast cell degranulation.
DRUGS
Records from prior surgeries should be examined and drugs tolerated in those procedures should be preferred if possible. Prior to the administration of any drugs associated with surgery, it is important that an IV is running, that epinephrine is available for immediate intramuscular (IM) or IV administration, and that emergency equipment is easily accessible in case of an adverse reaction.
IV preparations without preservative should be used.
Drugs to avoid include ethanol, dextran, and non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and toradol (unless the patient is already successfully taking a drug in this family), polymyxin B, amphoteracin B, quinine, dextromethorphan, beta-adrenergic blockers, and anticholinergic drugs as well as drugs mentioned in specific categories below.
Local anesthetics - True allergic reactions to local anesthetics resulting from mast cell degranulation are thought to be rare. Skin testing and graded challenge protocols have been published and may be administered by an allergist prior to the surgery if questions arise regarding the safe use of a local anesthetic in a patient. If possible, preservative-free local anesthetic should be used in these tests, as the preservative often present in local anesthetics can cause a mast cell reaction. In general, local anesthetics in the ester group should be avoided. This group includes procaine, chloroprocaine, tetracaine, and benzocaine. Anaphylactic reactions to local anesthetics in the amide group are rare. This group includes lidocaine, mepivacaine, prilocaine, bupivacaine, levobupivacaine, and ropivacaine.
Muscle relaxants - Muscle relaxants are the most likely group of the anesthetic drugs to cause anaphylaxis. Succinylcholine, D-tubocurarine, metocurine, doxacurium, atracurium, and mivacurium are more likely to cause a severe reaction than rocuronium or the so-called nondepolarizing muscle relaxants such as pancuronium or vercuronium. Some studies have reported increased numbers of anaphylactic reactions to rocuronium, however, so it may not be appropriate as the first choice for patients with mast cell disease.
Induction drugs - These are medications given to initiate anesthesia. It is rare for mast cell activation to occur in response to the use of propofol, ketamine, or the benzodiazepine drugs such as midazolam.
Inhaled anesthetics - Sevoflurane is an inhibitor of mast cell activation and is less likely to cause liver damage than other inhaled anesthetics in this family.
Opiates and opioids - Oral opioid drugs for pain relief may be tolerated by some mast cell disease patients, but their use should be approached with caution, beginning with very small doses. All drugs in this category are capable of causing mast cell mediator release.
SUMMARY
Surgery for a person with mast cell disease involves exposure to drugs and conditions that may trigger extensive mast cell degranulation. It should be emphasized that it is often impossible to predict or avoid the risk of adverse events which may occur in surgery due to the administration of drugs and the procedure itself. The risk can be reduced, though, if the mast cell disease is brought to the attention of everyone involved in the patient's care and measures are taken to reduce the possibility of mast cell mediator release. Careful planning is important, beginning from the time a need for surgery has been defined and continuing through the immediate pre-operative period, anesthesia, surgery, and recovery. The choice of drugs is of major importance, and the anesthesiologist, the surgeon, the nursing staff, the patient's physician, and the patient should form a working team to ensure that surgery presents the fewest possible dangers for the patient.
Neither Mastocytosis Society Canada nor the authors intend that this information replace medical advice given by the patient's doctor. Patients are encouraged to consult with their doctor regarding medications and procedures related to surgery.
REFERENCES
Hazards in operative management of patients with systemic mastocytosis; HW Scott Jr, WCV Parris, PC Sandidge, JA Oates, LJ Roberts II; Annals of Surgery, May 1983;197(5):507-514
Anesthetic management of systemic mastocytosis: experience with 42 cases; WCV Parris, HW Scott, and BE Smith; Anesthesia and Analgesia (1986)65:S117 (Abstract)
Urticaria Pigmentosa: An anesthetic challenge; Eric P Greenblatt, Linda Chen; Journal of Clinical Anesthesia, March/April 1990;2:108-115
Mastocytosis: Perioperative considerations; VA Goins; AORN Journal December 1991;54(6):1229-1238
Anesthesia in a patient with malignant systemic mastocytosis using a total intravenous anesthetic technique; A Borgeat and YA Ruetsch; Anesthesia and Analgesia (1998);86:442-444
Treatment of systemic mast cell disorders; AS Worobec; Hematology/Oncology Clinics of North America (June 2000);14(3):659-687
Treatment of mastocytosis: pharmacologic basis and current concepts; G Marone, G Spadaro, F Granata, M Triggiani; Leukemia Research, July 2001;25:583-594
Mastocytose: Anesthesie generale par remifentanil et sevoflurane; L Auvray, B Letourneau, M Freysz; Ann Fr Anesth Reanim 2001;20:635-638 (article in French)
Mastocytosis: Current concepts in diagnosis and treatment; L Escribano, C Akin, M Castells, A Orfao, DD Metcalfe; Annuals of Hematology (2002);81:677-690
Anaphylaxis during the perioperative period; DL Hepner and MC Castells; Anesthesia and Analgesia 2003;97:1381-1395
The authors wish to thank Cem Akin MD, PhD, for his review of this article.
The following is general information shared by patients regarding experiences and treatments related to their health emergencies caused by their mast cell disease. It does not constitute medical advice.
The most common types of reported health emergency experienced by mast cell disease patients include syncope, hypotension, full or partial anaphylaxis, persistent and/or escalating pain internally, severe headaches and vertigo. With mast cell disorders, the anaphylaxis signs and symptoms are diverse and unique per patient.
The emergency medical treatment for mast cell disease patients suffering anaphylaxis has been reported by patients as continuous administration of the following: Epinephrine, Benadryl, Ranitidine, Prednisone, and sometimes nausea medication and/or oxygen nebulizer. Dosages are unique per individual. Some patients report tolerating steroids well while others do not. Some patients reported being unable to have epinephrine due to their symptom presentation. Many patients tend to be unique in responding to emergency measures.
The National Institute of Allergy and Infectious Diseases (USA) has a good summary of anaphylaxis symptoms as they pertain to Mastocytosis patients, defined as follows: Anaphylaxis is a severe, systemic, hypersensitivity reaction caused by release of mediators from mast cells and basophils. Signs and symptoms of anaphylaxis may include:
Reference: Blood Factors in Mastocytosis and Unexplained Anaphylaxis and Flushing
In hospitals, anaphylaxis is sometimes undertreated which creates a rebound reaction within hours. Because these are rare disorders and mast cell patients are often unique, it is safest to have an emergency protocol defined in advance by the patient's physician. In addition, medical professionals should be aware of the medications, ingredients and preservatives which may complicate patient care, especially during any medical testing, emergency treatments or surgery.
Physicians diagnosing patients with Mastocytosis, MCAS, or IA have reported two approaches to emergency advice.
The Canadian Society of Allergy and Clinical Immunology (CSACI) has a great publication entitled Anaphylaxis in Schools and Other Settings which is very useful in understanding anaphylaxis, signs, triggers and emergency protocols.
To be written later. Remove from navigation for launch.
From Wendy Busse - MSc, Registered Dietitian
Diet and mast cell disease is a confusing topic. Patients often ask:
In the absence of well researched “facts,” the internet is full of opinions and speculation. When patients look for help, this speculation adds to their confusion and distress.
In my work, I see the devastating effects of false nutrition information. These concerns prompted me to write articles with practical, common-sense nutrition advise for histamine intolerance and mast cell disease. The bottom line is that everyone is individual – and there is not a one-size-fits-all “mast cell diet.” My hope is the articles will empower the reader to make balanced decisions about their diet and health.
More information available at wendybusse.com.
Q: What is the difference between IgE allergies and Masto triggers? Can you have IgE allergies and Mastocytosis? Are there tests to identify these triggers? (written by Wendy Busse)
A: Some people have a genetic tendency to produce IgE (a type of antibody) to normally harmless substances, such as food, pollen, insect stings and medication. The body produces IgE directed against specific substances. For example, one person may produce IgE directed against peanut and birch pollen, and another person produces IgE directed against egg white and dust mites. Once produced, IgE attach to mast cells. If the IgE are exposed to the triggering substance, the mast cells may activate (degranulate). For example, a patient develops peanut IgE which attach to mast cells around the digestive system. When peanut is eaten, the IgE are exposed to the peanut, which causes the mast cells to activate, leading to anaphylactic symptoms. In most cases, IgE mediated food allergies are consistent. The allergic individual reacts every time the food is eaten. The symptoms also occur quickly after the food is eaten. As a result, IgE mediated food allergy is often called immediate food allergy.
Q: It is possible to have both IgE food allergy and masto triggers? (written by Wendy Busse)
A: Your physician may use skin tests and/or food specific IgE blood tests to determine if your body has produced IgE. Each food is tested individually. If the test to a specific food is negative, IgE mediated allergy to that food is very unlikely (although the food could still be a “masto trigger”). If the test is positive, an IgE mediated allergy to that food is possible. However, false positives are very common. In other words, IgE to a specific food, does not necessarily mean you are allergic to that food. If you can eat a food most of the time without symptoms, it is unlikely that you have a true IgE mediated allergy to that food, even if you have a positive skin or blood. A good article on this topic can be found at www.foodallergy.org/diagnosis-and-testing . Read about “false positives” under the skin prick test and blood test sections. It is extremely important to speak with a knowledgeable allergist to sort this out. You should not introduce IgE positive foods without medical supervision and/or guidance.
Mast cell activation can also be triggered by other substances or events, including a variety of food, food additives, inhaled chemicals, medications/supplements, temperature changes, etc. These are commonly called “masto triggers”. The symptoms can be very similar to IgE mediated allergy. However, the reactions tend to be less consistent. A “masto trigger” may be problematic one day, but well tolerated on a different day. Unfortunately, there are no tests to determine “masto triggers”.
Q: Is there a specific diet to be followed for mast cell disease? How can I figure out which foods I cannot tolerate? (written by Wendy Busse)
A: There is no specific diet, however there are basic guidelines. Using an elimination diet can be helpful to determine food triggers. Mast cell disease patients often report symptom improvement with dietary changes, but these changes vary from person to person. Identifying helpful changes takes some experimentation. If you seem to be reacting to all foods, you might be reacting to the act of eating rather than specific foods. It would be best in this case to find a physician to help you with a plan of action to reduce overall mast cell reactions. Once you get your mast cells under control, you can re-evaluate your diet for potential triggers.
Q: What is a low histamine diet? (written by Wendy Busse)
A: A low histamine diet restricts foods that are potentially high in histamine and/or may lead to histamine release. Histamine is one of several inflammatory chemicals released from mast cells (and other immune cells) and is responsible for many of the troubling mast cell disease symptoms. Histamine can be absorbed into the body from food. Food with very high levels of histamine can lead histamine poisoning (a type of food poisoning that is common with improperly handled fish). Some people may have lower levels of the enzymes which break down food histamine, and may be more likely to absorb histamine from food (histamine intolerance). Histamine forms during fermentation and spoilage. It may also be present in certain fruits and vegetables. However, there has been very little research to accurately measure the amount of histamine in food. A second part to the theory is that certain foods may trigger mast cells to release histamine. There are long lists of histamine releasing foods on the internet, but these lists are based on anecdotal reports. Since there is so much individual variation, and very little concrete information, the low histamine diet is a general guideline that requires experimentation, rather than a diet that should be followed exactly. Here’s a link for more information on the low histamine diet.
Q: Will dietary changes completely control my symptoms? (written by Bonnie Nasar)
A: While improving your diet by eliminating triggers and eating wholesome, balanced meals can help to decrease your symptoms, there is no cure-all for mast cell disease.
Q: Should I take nutrition supplements? (written by Bonnie Nasar)
A: A multivitamin and mineral supplement is a good idea. Some people report benefit of specific supplements, but there have not been any studies to confirm this. Vitamin C is the most common one to report. Quercetin, an antioxidant, has been shown in some studies to inhibit mast cell activity. Before taking any supplement, talk to your physician or dietitian. Be sure to check all ingredients for potential triggers. If you decide to try specific nutrient supplements, do so systematically, and only continue if it makes a significant difference.
Q: How do I eat in a restaurant? (written by Bonnie Nasar)
A: Choose restaurants that are allergy-friendly. Call the restaurant in the middle of the afternoon, when the chefs are not busy and can speak on the phone. Explain that you have severe allergic reactions to certain foods. Even though you may not be truly IgE allergic, using the words “severe allergy” gets people to pay attention and realize the risk involved. Ask about cross-contamination prevention. Go over the menu with the chef and figure out which foods are safe for you. This eliminates the need for the server to ask detailed questions while the chefs are busy at mealtimes.
Q: What do I do when I am sending my child to a playdate or birthday party and the cake has an ingredient they don’t tolerate? (written by Bonnie Nasar)
A: Call the child’s parents in advance to find out what they will be serving. You can then send a packed meal similar to what is being offered from your home. Keep safe cupcakes in your freezer and defrost them the day of any party your child will be going to. Explain to the parent who will be in charge that your child has what is comparable to allergic reactions. It is helpful to have a printed paper with the information on it. Always give the parent in charge all rescue medications your child may need, including antihistamines and epinephrine auto injector. For more help in dealing with children and food issues, go to www.foodallergy.org or www.allergyhome.org.
Q: What problems can occur as a result of an overly restricted diet. (written by Wendy Busse)
A: Mast cell disease patients often follow overly restricted diets for several reasons. First, food is a common symptom trigger. Unfortunately, the reactions to food are often inconsistent (a food bothers you one day, but not the next). When patients have increased symptoms, they often pinpoint a food and eliminate it from their diet. Overtime, this can lead to an overly restricted diet. Secondly, there is a lot misinformation on the internet. Something negative is written about almost every food! This can lead to a negative and fearful mindset about food. Additionally, many mast clients receive very long lists of food to avoid after food hypersensitivity testing. Unfortunately, every test gives a different answer, causing confusion and very long food avoidance lists. All of these factors can lead to an overly restricted diet which has several consequences, including:
Here are some tips to minimize dietary restrictions.
It is extremely important for patients to respect their illness, learn their individual symptoms and identify what triggers each symptom for them.
When mast cells degranulate, they dump a host of chemicals at once, triggering the patient's symptoms. These chemicals include histamine, heparin, prostaglandins, neutral proteases, acid hydrolases, chemokines and cytokines, just to name a few.1 So it is vitally important for patients to avoid triggering mast cell degranulation to lessen their suffering and preserve their quality and length of life.
For each patient, each symptom may each have a unique trigger or a cluster of triggers. Some patients may have only a few triggers, some may have many, and some may be susceptible to nearly all known triggers. The best way to identify your symptoms and triggers is to keep a daily record and identify any correlations between trigger and symptom.
When symptoms occur, it is common for patients to have difficulty thinking clearly, restricting their ability to help themselves. Mast cells are in every organ of the body, including the brain. When symptoms occur, many patients experience problems speaking or articulating their needs, understanding what is happening to them, and what they need to do about it.
Listed below are the most common triggers of mast cell degranulation. We recommend keeping this list handy and sharing it with any health care professionals you consult with prior to undergoing any tests or treatments (i.e. family physician, specialists, dentist, optometrist/opthalmologist, nuclear medicine/x-ray technicians, paramedics).
Note: This is not a complete list. Also, many of these triggers may be applicable to one patient but not another.
References: