October 2017

Introducing Mikal

We are in the process of making some changes here at MastAttack, including building a team to help manage the site, the group, and access to me, in general. We recently have begun the process of digging through the huge backlog of email and FB questions for me. This will help to prioritize things that need my attention and allow me to focus more on my health, my job and my family.

I wanted to introduce everyone to Mikal Mowdy, who has come aboard to help with MastAttack. Many of you likely remember her daughter, Yzzy, who was cured of systemic mastocytosis and hemophagocytic lymphohistiocytosis when she received a bone marrow transplant in January. She has a wealth of mast cell knowledge and will help me to respond to questions.

Please join me in welcoming Mikal!

Lisa

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 63

77. Can you have anaphylaxis with high blood pressure?

  • Yes.
  • The misconception that a person with high blood pressure cannot be experiencing anaphylaxis is enduring and dangerous.
  • Author’s note: Thanks to the intrepid reader who caught a big typo right here. When I published the post, it said, “The misconception that a person with high blood pressure can be experiencing anaphylaxis is enduring and dangerous.” This is a whopper mistake. It should say,  “The misconception that a person with high blood pressure canNOT be experiencing anaphylaxis is enduring and dangerous.” You CAN have high blood pressure and anaphylaxis at the same time. Thanks again!
  • Lots of providers (and patients) think that high blood pressure rules out anaphylaxis. This is not true.
  • This misunderstanding comes from confusing two closely related but distinct concepts: anaphylaxis and anaphylactic shock.
  • Anaphylaxis is a severe allergic reaction affecting multiple organ systems.
  • Anaphylactic shock is when anaphylaxis causes such poor blood circulation that the heart cannot pump out enough blood to the body.
  • Anaphylactic shock is a form of circulatory shock, which means exactly what I just described: oxygenated blood is not being pumped out of the heart and through the blood vessels to the tissues that need it.
  • Anaphylactic shock is defined as blood pressure 30% below the patient’s baseline or a systolic blood pressure below 90 mm Hg. The systolic blood pressure is the top number when you get your blood pressure checked. If that top number is below 90 mm Hg, and that is the result of anaphylaxis, you are in anaphylactic shock.
  • Anaphylactic shock is the most serious potential complication of anaphylaxis. Anaphylactic shock happens when the chemicals released by mast cells cause a lot of the fluid in the bloodstream to “fall out” of the bloodstream and get stuck in the tissues.
  • When this happens, that fluid loss causes the blood pressure to drop. In response, the heart beats faster to try and use the blood it still has left to get oxygen to the body. However, at a certain point, even beating really fast is not enough to get enough blood to the tissues. At this point, shock sets in.
  • Anaphylactic shock occurs specifically as a result of low blood pressure. Because of this, providers strongly associate low blood pressure with anaphylaxis. They may not realize that while a person with high blood pressure cannot be having anaphylactic shock, they can be having anaphylaxis.
  • Part of the confusion is that anaphylaxis has been defined lots of different ways by many different groups. I have written a very detailed post about this (see the link below). Even today, exactly what constitutes anaphylaxis not agreed upon by everybody.
  • The most widely used criteria in the US are the criteria published in 2006 by the World Allergy Organization journal. These criteria explicitly state that a person does not need to have low blood pressure to be having anaphylaxis. A person can meet these criteria based upon a variety of combinations of symptom and vital signs that do not include low blood pressure.

2006 WAO Anaphylaxis Criteria

For additional information, please visit the following posts:

The definition of anaphylaxis
Anaphylaxis and mast cell reactions

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 49

 

 

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 62

76. Is it true that allergic reactions can cause heart attacks?

  • Yes.
  • Kounis Syndrome is an acute coronary syndrome caused by activated mast cells releasing chemicals. It is sometimes referred to as “allergic heart attack.” In acute coronary syndrome, there is not enough blood being pumped into the heart. It is named for two of the large blood vessels supplying oxygen to the heart, the coronary arteries. When not enough blood is getting to the heart via the coronary arteries, it can damage heart muscle, sometimes permanently. Heart attack and angina are examples of acute coronary syndromes.
  • In Kounis Syndrome, mast cells become activated, releasing lots of chemicals. These chemicals can irritate the coronary artery, causing it to spasm. This spasm reduces the amount of blood getting to the heart. Sometimes, mast cell activation can trigger the formation of a clot. A clot can be the reason not enough blood is passing through the artery.
  • Several of the molecules released by mast cells can affect the cardiovascular system and contribute to causing Kounis Syndrome. Histamine and leukotrienes can cause the coronary artery to narrow. It can also activate platelets, helping a clot to form. Both tryptase and chymase can cause clots formed elsewhere to break off and get stuck in the coronary artery.
  • Mast cells also help regulate an important molecule called angiotensin II. Angiotensin II is a powerful regulator of blood pressure and can cause the coronary artery to narrow and tighten up.
  • People with Kounis Syndrome may have a history of coronary artery disease. Some patients have a stent in the coronary artery from a previous coronary issue. A stent is a tube that helps keep the blood vessel the right size so that the heart gets the blood it needs. However, many patients with Kounis Syndrome do not have any history of problems with their heart or blood vessels.
  • The symptoms of Kounis Syndrome sometimes look just like the symptoms of any other mast cell reaction or anaphylaxis, making it hard to know that a person is having Kounis Syndrome. Chest pain, irregular heart beat, the heart beating too fast or too slow, and palpitations are all common symptoms of Kounis Syndrome.
  • Another tricky thing about Kounis Syndrome is that it doesn’t always show up on the tests we use to look for heart attack or coronary issues. Because of this, doctors don’t always realize what is happening. Some people do have positive results to these tests, things like EKG, echocardiogram, chest x-ray, and bloodwork to look at levels at cardiac enzymes or troponin. Cardiac enzymes and troponins are often high in a person who is having a heart attack but are sometimes normal for patients with Kounis Syndrome.
  • In order to manage Kounis Syndrome, patients may need treatment for both the allergic reaction and the coronary syndrome.
  • Treatment for the allergic reaction is similar to anaphylaxis treatment: an H1 antihistamine like Benadryl, an H2 antihistamine like famotidine, a corticosteroid like methylprednisolone, IV fluids, and sometimes epinephrine, if that’s appropriate. Please note that epinephrine is not always appropriate for patients who have Kounis Syndrome because epinephrine can actually also cause the coronary artery to narrow.
  • Treatment for the cardiovascular aspect of Kounis Syndrome is very dependent upon symptoms and test results. Calcium channel blockers like verapamil, aspirin, and nitroglycerin are commonly used. Importantly, some of the common medications used to manage coronary syndrome are not safe for mast cell patients. These medications include beta blockers like metoprolol or atenolol, and, to a lesser extent, ACE inhibitors like lisinophil. These medications can interfere with epinephrine so epinephrine may not work if a patient needs it for anaphylaxis.
  • Anything that triggers mast cell activation can cause Kounis Syndrome, including emotional stress.

For additional information, please visit the following posts:
Kounis Syndrome: Subtypes and effects of mast cell mediators (Part 1 of 4)
Kounis Syndrome: Diagnosis (Part 2 of 4)
Kounis Syndrome: Treatment (Part 3 of 4)
Kounis Syndrome: Stress (Part 4 of 4)
Beta blockers and epinephrine
Cardiovascular manifestations of mast cell disease: Part 1 of 5
Cardiovascular manifestations of mast cell disease: Part 2 of 5
Cardiovascular manifestations of mast cell disease: Part 3 of 5
Cardiovascular manifestations of mast cell disease: Part 4 of 5
Cardiovascular manifestations of mast cell disease: Part 5 of 5
The Provider Primers Series: Medications that impact mast cell degranulation and anaphylaxis

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 61

75. What other diseases and disorders are commonly associated with mast cell disease?

I often joke that it would be easier to list what conditions are not commonly associated with mast cell disease because so many conditions occur alongside it. However, there are some conditions that you see a lot in the mast cell population relative to others. In every instance, mast cell disease has the potential to irritate the other condition and vice verse.

Clonal hematologic disorders. Systemic mastocytosis is so frequently accompanied by other blood disorders that it has a diagnosis specifically for this phenomenon: systemic mastocytosis with associated hematologic disorder (SM-AHD). It is estimated that up to 40% of patients with SM eventually develop another clonal hematologic disorder. A clonal hematologic disorder is a condition in which your bone marrow makes too many blood cells. Examples include chronic myelogenous leukemia, acute myeloid leukemia, polycythemia vera, myelofibrosis, and essential thrombocythemia.

Unlike mastocytosis, MCAS can occur secondarily to lots of conditions. In some instances, it’s not clear if the MCAS is secondary to a condition or the condition is secondary to MCAS or neither.

Heritable connective tissue diseases. Ehlers Danlos Syndrome (EDS), is the most common connective tissue disease in the mast cell population. There are multiple types of EDS. While hypermobility type EDS (formerly called Type III) is the most common in MCAS patients, other forms occur also. Other connective tissue diseases seen in mast cell patients include Marfan Syndrome and Loeys-Dietz Syndrome.

Dysautonomia. Dysautonomia is a condition in which your body’s autonomic nervous system doesn’t regulate essential bodily functions correctly. POTS is the most common form of dysautonomia found in mast cell patients but other forms occur, too.

Mast cell patients commonly have MCAS, EDS and POTS together. They cooccur so commonly that some experts think that that this presentation is actually one overarching disease rather than three separate ones affecting mast cell patients.

Eosinophilic GI disease. Mast cells are closely related to eosinophils. They activate eosinophils and eosinophils activate them. Mast cell patients sometimes have eosinophil GI disease where eosinophils activate to lots of triggers and damage the GI tract.

Immunodeficiency. Conditions that specifically impair a person’s immunity, especially those that affect T or B cells, like SCID or CVID, are not unusual in mast cell patients.

Gastrointestinal disease. Mast cells normally live in the GI tract so they are very sensitive to GI inflammation. MCAS can occur secondarily to lots of GI diseases. Crohn’s, ulcerative colitis, inflammatory bowel disease, and irritable bowel syndrome are examples. GI disorders that specifically affect motility are also seen in mast cell disease, like gastroparesis and chronic intestinal pseudoobstruction.

Allergies. Some mast cell patients have true IgE allergies or other allergic disorders like atopic dermatitis.

Autoimmune disease. Autoimmune disease is more common in MCAS patients than in SM patients. The specific disorder could be virtually any autoimmune condition, including rheumatoid arthritis, lupus, Hashimoto’s thyroiditis, autoimmune urticaria, and many others.

Adrenal insufficiency. The body’s mechanisms for produce stress hormones like cortisol can become dysregulated in mast cell patients. This results in a situation in which the body does not make enough steroids of its own to take care of the body during periods of stress. Patients with adrenal insufficiency are dependent upon daily steroids to stay safe.

Chiari malformation. This condition affects the space around a person’s brainstem, causing a wide array of symptoms. Some patients have surgery for this condition.

Asthma. It is difficult to draw an exact line where mast cell disease ends and asthma begins in mast cell patients as the symptoms can be virtually identical.

This list is not exhaustive. Many other conditions sometimes occur in mast cell patients.

For additional reading, please visit the following posts:
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 31
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 32

Somebodies

I’ve been trying to get this post out for a few days. I feel like it’s not finished and sharp in the wrong places but I feel like this needs to be said so I’m saying it now.

When I was first diagnosed with mast cell disease, I was pretty relieved. I had been sick a long time and was so tired of being abused by doctors and called a liar. I could have been diagnosed with anything. I could have been diagnosed with weekly limb falling off disease where every week one of my limbs fell off until I had no limbs left. I needed something to hold onto and a diagnosis had that. (I am grateful to announce that I do not have limb falling off disease.)

I do, however, have mast cell disease. It was a few months before it occurred to me that having mast cell disease might be scary. There wasn’t a lot of information available on it and I didn’t have great journal access then, so I wasn’t able to validate those fears. But I was still afraid. Just a little, at first. And then another several months past and I started having major organ involvement. And I started being afraid for real. This time, my fears were validated.

One of the more common questions I get is whether or not people can die from mast cell disease. I get it a lot from people who are newly diagnosed but I get it from people who have been diagnosed a while. I realized recently that people who have been diagnosed a while only ever ask me this question in private message or email. I’ve been thinking about why that is.

The answer is simple: people are afraid to ask if they can die from mast cell disease in a public forum because, overwhelmingly, the responses are not kind. I am guilty of this, too. Those of us who have been in this community a long time have learned to stratify mast cell patients by level of hematologic malignancy – that is, to separate mast cell patients into those who have malignant forms of mast cell disease (aggressive systemic mastocytosis, mast cell leukemia, and mast cell sarcoma) and those who don’t. Because typically the people who lose their lives to mast cell disease are those with those malignant forms, and those who don’t have them don’t die from mast cell disease. The medical institution views malignant mast cell disease as dangerous and the other forms as not dangerous. Specifically, the establishment touts to everyone who will listen that you don’t die from mast cell disease if it’s not malignant.

But the truth is that’s not really the case, if you think about disease and what it does to a person and all the ways it kills them. It’s true that a person with ASM is not likely to die in the same way as an MCAS patient. A patient with ASM will die from mast cell disease if the thousands of extra mast cells burrow into their organ tissue and destroy that tissue so much that the organ stops working. That’s not what happens in a patient with MCAS. But a patient with MCAS can die in other ways. They can die from anaphylaxis and complications of huge steroid doses and side effects from chemo and sepsis and not being able to afford their health care costs and not having insurance and not being able to face one more minute of the humiliation and desperation that is begging for care from people who don’t want to provide it. All of those things can kill a person, too.

Defining death from mast cell disease along by delineating along the lines of organ failure is disrespectful, unfair and missing the point. All of us who have spent years living inside the data of this disease have done it, including me, and we should be sorry. I am. It has never been my intention to characterize MCAS as less serious than other forms of mast cell disease but I think I did anyway, and whether or not I wanted to do it doesn’t change that. I am sorry for doing this. It is not okay. I am committed to doing better in the future.

Fall is a difficult time for mast cell patients. It’s a lot of change at once. It’s new routines and major environmental upheaval. It’s triggers on crack. Season changes are always hard for us but autumn is harder, I’m not sure exactly why. But in the same way that I associate September with ports, I associate fall with mast cell patients crashing and dying. This year has been no different.

In the last several weeks, we have lost a number of mast cell patients across a variety of diagnoses, to the tune of six in six weeks. It’s painful to even type that. One of them was my friend, an SM patient who died of complications of anaphylaxis. Another was a touchstone in the MCAS community, a young woman who did a great deal to comfort others, and who undoubtedly died of complications of MCAS. Still another died of suicide. These last few weeks have been so, so crushing.

There is a very, VERY good chance that you will live a full life with a normal lifespan as a mast cell patient. But it’s not enough to say that people don’t die from non-malignant forms of mast cell disease because “almost nobody does.” Those “almost nobodies” are somebodies, and they are people with lives and dreams and futures they don’t get to experience. They do not deserve to be lost in the data, digits rounded down to zero.

We owe it to them to remember that they were real and that they were here and that they mattered.

I owe it to them. So let’s do that.

Additional posts on prognosis and disease progression:

Progression of mast cell diseases (Part One)

Progression of mast cell diseases (Part Two)

Progression of mast cell diseases (Part Three)

Progression of mast cell diseases (Part Four)

Progression of mast cell diseases (Part Five)

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 15

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 48

On prognosis and dying from mast cell disease

I am not there. I do not sleep.