The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 56

70. What is premedication and when should I do it?

Premedication is taking extra medication in advance of doing something that you expect to trigger your mast cells. The current premedication protocol for mast cell patients is as follows:
Prednisone 50mg orally (20mg for children under 12): 24 hours and 1-2 hours before procedure
• Diphenhydramine 25-50mg orally (12.5 mg for children under 12) OR hydroxyzine 25mg orally, 1 hour before procedure
• Ranitidine 150mg orally (20mg for children under 12) 1 hour before procedure
• Montelukast 10mg orally (5mg for children under 5) 1 hour prior to procedure

This protocol was developed for the Mastocytosis Society by Dr. Mariana Castells and the original can be found here.

This premedication protocol uses medications to interfere with the molecules mast cells release as well as medication to decrease the amount of molecules mast cells make and release. Diphenhydramine (called Benadryl in the US) stops histamine from getting to the H1 histamine receptors on the outsides of many cells. Ranitidine stops histamine from getting to the H2 histamine receptors on the outsides of many cells. In these ways, these medications can help to stop symptoms from histamine released by mast cells.
In a similar way, montelukast stops leukotrienes from getting to receptors on cells. This helps to curb some of the symptoms that occur when leukotrienes are released by mast cells.

Prednisone is a glucocorticoid, commonly called referred to as a “steroid.” This medication suppresses the production and release of inflammatory molecules by mast cells and other immune cells. Importantly, this medication can take hours to achieve maximum effect. This is why the first dose is the day before the event for which you are premedicating. By being dosed again a couple of hours before the event, it can also provide some additional protection for delayed reactions.

It is important to know that this premedication protocol may need to be changed to achieve the most effective protocol for individual patients. These recommendations are general and are not based upon study or clinical trial data.

This procedure is intended to be used for all major and minor medical procedures, including imaging tests like x-rays and MRIs, whether or not they use contrast. However, many patients find some benefit in premedicating for other types of events as well, such as flying, childbirth, and days of planned elevated physical or emotional stress. Patients should discuss what sorts of events are appropriate to premedicate for with a knowledgable provider.

For more detailed reading, please visit the following post:
Premedication and surgical concerns in mast cell patients

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 55

69. What routine monitoring should mast cell patients receive?

There are not yet routine testing recommendations for MCAS patients, but there are some for mastocytosis patients. Many doctors use the mastocytosis recommendations to monitor their MCAS patients in the absence of specific MCAS guidelines.

Mastocytosis patients should monitor tryptase level annually. In mastocytosis patients, tryptase level is often a good marker for how many mast cells are in the body (although this is not always true.) If a patient’s tryptase is increasing over time, the provider will need to check other things to see if their disease is moving to a more serious disease category.

DEXA scans measure bone density. Osteoporosis is a common complication of systemic mastocytosis. Patients should receive regular osteoporosis screening, even if they are young.

Mastocytosis patients usually receive routine bloodwork annually that includes a complete blood count (CBC), which counts the amount of blood cells a person has; and a metabolic panel, which looks at how well the liver and kidneys are working.

Repeat biopsies are usually only done if the result will change treatment in some way. Most patients with systemic mastocytosis are diagnosed based upon bone marrow biopsies. These don’t usually need to be repeated unless tryptase level increases sharply or there are unusual results in routine blood count testing. Increasing tryptase can indicate that the body is making more mast cells much faster, which is sometimes linked to a more serious disease category. Unusual blood cell counts can indicate not just too many abnormal mast cells, but also other bone marrow conditions sometimes seen in mast cell patients, like myelofibrosis and essential thrombocythemia.

Patients with cutaneous mastocytosis are diagnosed by skin biopsy. There is not usually a need to repeat a skin biopsy for patients with CM.

Patients with systemic mastocytosis are usually diagnosed by bone marrow biopsy but can also be diagnosed as a result of a positive biopsy in any organ that is not the skin. A person can be diagnosed with SM via a GI biopsy.

GI biopsies are a little different than bone marrow biopsies in that there are sometimes reasons to repeat them. GI biopsies may be repeated to see if the general inflammation in the GI tract is improved or worsened. The provider may also be interested in whether or not the amount of mast cells in the GI tract has decreased. The result of GI biopsies often change treatment options so it is not unusual to repeat them. However, unlike bone marrow biopsies, repeated GI biopsies do not tell the provider if the mastocytosis is moving toward a more serious disease category or not.

MCAS patients are diagnosed based upon positive tests for molecules that indicate mast cells are overly active, like n-methylhistamine, and D2- or 9a,11b-F2 prostaglandins. Once the patient is diagnosed, there’s not a clear rationale for repeating these tests, although some providers do for their own information. Some providers like to check prostaglandin levels to see if treatment to stop mast cells from making prostaglandins (like use of aspirin or other NSAIDs) is helping.

However, it is important to understand that the level of mast cell mediators is not associated with symptoms. A person who has a normal level of 9a,11b-F2 prostaglandin may have the same symptoms as a person above the normal level, who may have the same symptoms as a person who has three times the normal level. For this reason, many providers consider these mediator tests to be less about the numerical value of the test and more about whether it’s normal or high, period.

For more detailed reading, please visit the following post:
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 5
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 6
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 7
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 8
The Provider Primer Series: Diagnostic criteria of systemic mastocytosis and all sub variants
The Provider Primer Series: Diagnosis and natural history of systemic mastocytosis (ISM, SSM, ASM)
The Provider Primer Series: Mediator testing
The Provider Primer Series: Mast cell activation syndrome (MCAS)

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 54

68. How does mast cell disease affect pregnancy?

One of the things mast cells normally do in the body is regulate the female reproductive cycle. Mast cells in the endometrium, the uterine lining that is shed during menstruation, become activated and release mediators in the days before and during menstruation. Many of the symptoms of premenstrual syndrome (PMS) occur because of mast cell degranulation. These symptoms include things like cramps and bloating.

Because mast cells are involved in controlling the reproductive cycle, they are responsive to the effects of hormones like estrogen and progesterone. In particular, estrogen can directly cause mast cell degranulation.

In some allergic conditions like asthma, patients often have flares right before or during their menstrual period. This is often the case with mast cell patients as well. The change in hormones, the built in mast cell activation, and the bleeding, can all cause mast cell symptoms.

A study on the effects of the pregnancy on mastocytosis found that there was a lot of variability in what patients experienced. 33% of women had symptom improvement during pregnancy. In these women, their symptoms mostly improved beginning in the first trimester and continued throughout their pregnancy. 45% of patients had no change in symptoms during pregnancy. The remainder had worsened symptoms.

Mastocytosis did not seem to affect the outcome of pregnancy compared to the normal population. Premedication was recommended at the start of labor. Many women safely received anesthesia. In women who reacted, 2/3 had not premedicated. Induction of labor with medication was well tolerated. Both vaginal delivery and Caesarean section was performed safely on women with mastocytosis. The frequency of Caesarean section, miscarriage, prematurity and low birth weight were similar to the general population.

In some instances, severe allergic reactions and anaphylaxis can induce early labor, so patients should be aware of this risk.  Histamine can trigger uterine contractions.

An important thing to consider is that mast cell patients may have to change or stop some of their medications while pregnancy to avoid effects upon the fetus. In particular, the use of epinephrine is discouraged in pregnancy because it causes uterine contractions. Mast cell patients should have an alternative plan for anaphylaxis that excludes epinephrine where possible. Any mast cell patient who is pregnant or considering becoming pregnant should have detailed discussions with their providers about it.

For more detailed reading, please visit the following posts:
Pregnancy in mastocytosis
Effects of estrogen and progesterone and the role of mast cells in pregnancy

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 53

66. How long does it take to react to a trigger?

There isn’t a straight answer to this. The time it takes to react to a trigger is hugely variable. It depends upon the trigger; the strength of the reaction it triggers; the patient; the medications they take; their lifestyle; and other activities that may increase or decrease reactivity. As we have discussed before, the reaction you see from a trigger is often the cumulative result of how much histamine you have circulating at the time, which can be affected by many other things. Reactions can happen immediately or several days later. It is not unusual for mast cell patients to react days later, especially to things they have ingested. This logically makes sense to me as a result of the trigger still being in the GI tract but there is still not definitive proof that explains why you can react days later.

67. What physical things trigger mast cells?

A lot of physical things trigger mast cells. The exact reasoning for why some of these things trigger mast cells is still not well understood. However, these triggers are documented in literature, often as triggers for physical urticaria (hives caused by physical triggers) and/or angioedema (swelling). While reactions to these triggers often start in the skin, the mast cell activation can spread to other mast cells elsewhere in the body. Additionally, patients may not have skin symptoms but have reactions to the following triggers.

Heat and cold can both activate mast cells. Hot water and cold water are both common triggers. Water in general is a trigger for some. Emotional stress is activating, as is various forms of physical stress, including exercise, surgery, physical trauma, infection, or increased activity of another disease. Sweat can be a trigger, regardless of whether the patient is sweating from exercise, heat, or something else. Pressure on the body, even mild pressure, can cause mast cells to release chemicals. Sunlight and vibrations are also known triggers. Mast cell patients are recommended to premedicate before any medical procedure, including imaging like ultrasounds, X-rays or MRIs, as patients have reported activation from these things. Changes in barometric pressure, such as from a change in weather or a storm, are often reported by patients to cause symptoms.

For more detailed reading, please visit the following posts:
Chronic urticaria and angioedema: Part 2

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 52

64. Why do I always have dark circles around my eyes?

It is not unusual for people who are having allergic reactions to have “allergic shiners.” Allergic shiners are dark circles around the eyes, especially evident under the eye where they may look like “bags.” There is not a definitive reason for why they occur but it is thought to be the result of poor circulation near the sinuses. In these patients, nasal congestion is common. This interferes with the normal circulation of blood near the sinuses. The blood “backs up” and pools in the blood vessels nearby. These blood vessels expand to accommodate the extra blood in them. Since the skin is very thin around the eyes, when these blood vessels expand, you can see the blood through the skin, giving an appearance of a dark circle under the eye.

65. Does mast cell disease cause hair loss?

Yes, sometimes. Mast cells release huge amount of prostaglandin D2 (PGD2). They release so much PGD2 that testing for it in urine is one of the more common steps in diagnosing mast cell disease. PGD2 has been linked to hair loss, especially in the scalp of men who experience hair loss. Exactly how PGD2 causes hair loss is still heavily researched, but it seems to stop hair follicles from maturing normally.

PGD2 causes an array of far reaching symptoms. For this reason, many mast cell patients take medications or supplements to decrease mast cell release of PGD2. Aspirin and other NSAIDs are often used. These medications interfere with specific molecules called COX-1 and COX-2. Without these molecules, cells are not able to make prostaglandins like PGD2. There are a number of supplements that can also interfere with one or both of the COX molecules. Curcumin or turmeric is sometimes used for this purpose. (Keep in mind that aspirin and NSAIDs are NOT safe for many patients. Patients should never add a medication or supplement without discussing it with a provider that knows their specific health situation.)

Some medications commonly used by mast cell patients can also contribute to hair loss. H2 antihistamines can sometimes cause hair loss. Some NSAIDS may also do this, even though they should help stop hair loss as I mentioned above. In more serious instances of mast cell disease, patients may need immunosuppressants, interferon therapy, or chemotherapy. These can cause varying degrees of hair loss, too. Steroids like prednisone may also decrease hair production.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 51

63. Why do many mast cell patients gain weight? Why can’t they lose it?

The most common question I get about weight is “Why am I gaining weight when I can barely eat?” Weight gain, or failing to lose weight, is not unusual for mast cell patients. There are a lot of reasons why this happens.

One of the big reasons why mast cell patients gain weight is because mast cells release molecules that cause inflammation. Some of these molecules are known to be linked to obesity when there is too much of them in the body. Mast cells release some of these molecules, like TNF, and IL-6.

Leptin is a hormone released by mast cells that can contribute to obesity. Patients with obesity often have higher than normal levels of leptin in their blood. In these patients, it seems like leptin doesn’t work as well as in others, so their bodies need to make more leptin.

Leptin’s job in the body has long been thought to tell your brain that you are not hungry. More recent research suggests that leptin doesn’t exactly tell your brain that you’re not hungry, and instead tells your brain that your body is starving. The body responds to this “starving” signal very strongly by trying to maintain or gain weight, and to maintain or gain fat stores.

Mast cells live in adipose tissue (fat tissue), often in significant numbers. Leptin level somehow controls the amount of mast cells in adipose tissue (fat tissue) but we are not sure how. Leptin is one of the ways that mast cells tell other cells to become inflamed. It tells cells to make more inflammatory molecules like TNF, IL-2 and IL-6. Mast cells in inflamed spaces can also attract cells from other parts of the body to come and make more inflammation.

Leptin also directly opposes another hormone, ghrelin. Ghrelin is the hormone that tells your brain that you are hungry. When leptin is high, ghrelin is low. Importantly, ghrelin curbs inflammation and tells cells to stop making inflammatory molecules. If leptin is high, ghrelin is not around as much to stop inflammation.

Another way mast cell disease can contribute to weight gain is by swelling. When mast cells are activated, they release molecules that make it easier for fluid in the bloodstream to “fall out” of the bloodstream and get stuck in tissues. When this fluid is stuck in the tissue, your body can’t just pull back into the bloodstream. It takes days for your body to be able to get the fluid out of the tissues and back into a place where it can be used.

Some of the medications used to treat mast cell disease can cause weight gain. H1 antihistamines are probably the drugs most commonly used for mast cell disease. They can cause weight gain. Steroids like prednisone and methylprednisolone cause swelling and weight gain.

Mast cell patients often have difficulty maintaining a normal sleep schedule. Sleep at night is often not restful because mast cells are very active at night. Not sleeping well can cause inflammation, contributing to weight gain.

Exercise can be very tricky for mast cell patients as well. Many patients are deconditioned and out of shape so even low impact exercise can be exhausting or impossible. Mast cell patients often have restrictions on what exercises they can do safely so vigorous exercise to help regulate weight might not be an option.

Mast cell patients often have little control over their diet due to food reactions, reacting to the process of eating, or having other GI conditions like gastroparesis. Safe foods may not be “healthy” and can contribute to weight gain. (Potato chips are a huge part of my diet as a food that is always safe for me.)

I personally struggled with my weight for years as a result of mast cell disease. It has been my experience that reducing inflammation overall is the only way to lose weight. Of course, it is very difficult to reduce inflammation when you have mast cell disease. In my case, I found that a reconditioning program helped me immensely. This is not safe for everyone and you should never start an exercise program without discussing it with the provider that manages your care.

 

For more detailed reading, please visit the following posts:

Leptin: the obesity hormone released by mast cells
Exercise and mast cell activity
My exercise program for POTS and deconditioning
Deconditioning, orthostatic intolerance, exercise and chronic illness (Part One)
Deconditioning, orthostatic intolerance, exercise and chronic illness (Part Two)
Deconditioning, orthostatic intolerance, exercise and chronic illness (Part Three)
Deconditioning, orthostatic intolerance, exercise and chronic illness (Part Four)
Deconditioning, orthostatic intolerance, exercise and chronic illness (Part Five)
Deconditioning, orthostatic intolerance, exercise and chronic illness (Part Six)
Deconditioning, orthostatic intolerance, exercise and chronic illness (Part Seven)

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 50

62. Is it possible to become tolerant of a trigger again?

Yes, sometimes.

Desensitization is the term for when your body becomes tolerant of something that it previously reacted to. While it usually means becoming tolerant of a medication, it is a general term so many mast cell patients use “desensitization” to mean becoming tolerant of anything they used to react to, including food or environmental triggers.

Traditionally, desensitizing is done by exposing the body to a small amount of a trigger, then a little more at a later time, and so on until the body accepts a reasonable amount. In the regular allergies, in the US, “allergy shots” are used for this. A patient is injected with a tiny amount of an allergen repeatedly until their immune system stays calm when exposed to the trigger.

There are some newer approaches for desensitization that use certain newer medications. In particular, anti-IgE therapy has been very well described for helping to force a patient to tolerate a trigger. Antihistamines and/or corticosteroids can be used to control the level of allergic response.

In some instances, a rapid desensitization procedure can be used to force tolerance. These procedures are performed in a medical setting and may provide tolerance in a matter of days. They are usually used in situations where the benefit of a drug to which the patient reacts outweighs the risk of anaphylaxis, such as patients who need to use a specific chemotherapy drug to treat an aggressive disease.

Importantly, if a patient becomes desensitized to a substance, they must be regularly exposed to that substance in order to continue tolerating it. Sometimes, a patient must be exposed daily in order to not react to the trigger. This is very patient and substance specific.

Mast cell patients are different from typical allergy patients in a lot of ways, many of which we don’t understand. Patients ask from time to time if “allergy shots” or something similar will help them. Mast cell patients who have an IgE allergy to a substance may get some benefit from allergy shots. Specifically, allergy shots are recommended for mastocytosis patients who have allergies to certain insect stings.

But what if they don’t have an IgE allergy? Will gradually increasing the amount of trigger in a series of exposures allow the body to accept it?  I know plenty of mast cell patients who have used allergy shots or oral immunotherapy to improve trigger tolerance. I can’t think of any reason why this wouldn’t help if you could safely increase the exposures.

For mast cell patients, the issue is that reactions can be so serious that desensitization is difficult to achieve. Patients can sometimes overcome this by using IV Benadryl, IV steroids, or a continuous IV epinephrine infusion. Mast cell patients should never attempt to force tolerance to any trigger without receiving advice from a health care provider that understands their specific health situation.

Food allergies are widely recognized as being different from other kinds of allergies. We are learning about food allergies in real time right now. Food allergies are on the rise and now affect huge numbers of people around the world. This means that there’s tons of research on it, which is great. But it means that we still don’t understand them that well. For this reason, desensitization to food is trickier.

There are a few methods commonly used in mast cell patients to manage food reactions. Sometimes a gradually increasing amount of trigger is eaten while the patient is monitored and given medications to manage any reactions, essentially a rapid desensitization for food. I find this approach is taken more commonly with children, largely because it is the recommended procedure for reintroducing triggers to children with FPIES. Sometimes people find that when they are exposed to a trigger for the first time in a while, they tolerate it until a second exposure. In these scenarios, rotation diets can be helpful. Allergy shots or oral immunotherapy for substances found in food are sometimes given. Results vary.

I have talked a lot before about the fact that mast cell reactions are often the cumulative result of things that activate your mast cells. This means that if you do something that activates your mast cells before eating a trigger, your reaction may be worse. In some instances, you may only react if you do something irritating to your mast cells shortly before eating it. This doesn’t just happen to mast cell patients. There are many mentions in literature of allergy patients who only experience anaphylaxis to trigger foods if they have exercised shortly before eating.

This means that if you are able to control the experience of eating triggers, you may have better success. You may do better if you refrain from doing anything irritating to mast cells like exercising, getting too hot, or being in a stressful situation. Food temperature can play a role. Many patients react to foods that are too hot or too cold. How you time medications can help. If you eat in the window of time when your medications are most active, you might find that a trigger is less activating. Solids are harder to digest and cause more histamine release than liquids (even thick liquids) so what form your food takes can matter, too.

Additionally, if you are able to control your disease and inflammation, you may find yourself more tolerant of triggers overall. Patients who find that their symptoms are better controlled should discuss trials with their health care providers to see if they can try exposures to previous triggers.

I can tell you that I have personally had a lot of success with using an anti-IgE medication to help me regain foods I lost. I have one IgE food allergy (chicken egg whites) and have no plans to ever try to consume them without thorough cooking (I’m tolerant of well cooked egg whites.) However, I do have a spectacular amount of food triggers that cause reactions ranging in severity from flushing to anaphylactic shock. My severe food reactions largely resolved when I started anti-IgE injections a few months ago. I eat all kinds of things I used to react badly to. I can eat cookies. I can eat cherry pie. I can eat bread. I try not to push my luck with things that have are loaded in histamine. I will never try alcohol or anything fermented again.

Prior to taking the anti-IgE medication, I had some success with rotation diets in which I ate gradually increasing amounts of a trigger every four days. It didn’t really make the reactions stop but it did make them less severe, enough that I could reintroduce small amounts of some previous triggers into my diet. This happened after I had GI surgery that decreased my overall level of inflammation and mast cell reactivity.

For more detailed reading, please visit these posts:

Food allergy series: FPIES (Part 1)

Food allergy series: FPIES (Part 2)

Food allergy series: Mast cell food reactions and the low histamine diet

Reintroduction of food to a child with SM

The Devil’s Arithmetic

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 46

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

60. Is anaphylaxis the same as anaphylactic shock?

No. Anaphylaxis can result in anaphylactic shock but it often doesn’t. When talking about anaphylactic shock, people are referring to circulatory shock that was caused by anaphylaxis. Circulatory shock occurs when there is not enough blood to carry oxygen to all the tissues that need it. When the tissues don’t get enough oxygen, your organs stop working correctly.

Circulatory shock is usually caused by low blood pressure. Anaphylaxis commonly causes low blood pressure and that can cause shock. However, anaphylaxis does not always cause low blood pressure, and it does not always cause shock.

61. If a tryptase level over 10.9 ng/mL is high, why is one of the criteria for systemic mastocytosis a tryptase level of 20.0 ng/mL or higher?

Tryptase level is used in two ways in assessing mast cell patients: as a marker for activation, and as a marker for how many mast cells are in the body.

There are two primary methods of using tryptase to indicate mast cell activation.

The first way is to compare a tryptase level when a patient is reacting to a tryptase level when they are not reacting (baseline). Mast cells release more tryptase when they are activated. For mast cell patients, an increase of 20% + 2 ng/mL is considered evidence of mast cell activation. So if a patient has a baseline tryptase of 5 ng/mL when they are not reacting, anything 8 ng/mL (20% of 5 ng/mL is 1 ng/mL, then add 2 ng/mL = 8 ng/mL) or higher is considered evidence of activation.

The second way is to count anything over 10.9 ng/mL as evidence of activation.

When you are using tryptase as a measure of how many mast cells are in the body, the patient should not be reacting beyond their normal day to day symptoms. This is because you don’t want an increase in tryptase from activation to make the baseline level look higher than it is. Tryptase is used to measure how many mast cells are present because mast cells release some tryptase all the time, even when they aren’t activated.

Anything over 10.9 ng/mL is considered an elevation of tryptase. The reason that 20 ng/mL is the cutoff for the SM criterion is that patients are likely to have a positive bone marrow biopsy when the tryptase level is twice normal (21.8 ng/mL). They round the number down to 20 ng/mL because all tests have a margin of error. By rounding down to 20 ng/mL, they catch patients that might not have made the cutoff before because of an error in the test. This means that a patient who has a tryptase level of 20 ng/mL or higher is likely to have a bone marrow biopsy that will be positive for systemic mastocytosis.

For more detailed reading, please visit these posts:

Anaphylaxis and mast cell reactions

The Provider Primer Series: Mediator Testing

Patient questions: Everything you wanted to know about tryptase

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, part 8

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

59. Is systemic mastocytosis a form of cancer? Why do some papers say the life expectancy for systemic mastocytosis patients is much shorter?

Systemic mastocytosis is a term that different people use in different ways, often without defining them for the audience. This can lead to some confusion.

In its broadest sense, systemic mastocytosis is actually a disease category rather than one specific diagnosis. The subtypes of systemic mastocytosis are indolent systemic mastocytosis (ISM), smoldering systemic mastocytosis (SSM), systemic mastocytosis with associated hematologic disease (SM-AHD), aggressive systemic mastocytosis (ASM), and mast cell leukemia (MCL).

When patients talk about systemic mastocytosis without specifying which diagnosis, they almost always mean indolent systemic mastocytosis (ISM), the most common form of SM. ISM is benign and has a normal life expectancy. But when providers and researchers talk about systemic mastocytosis, they usually mean the disease category that includes all of these diagnoses.

I just recently explained in another post what a neoplasm is. It is essentially when the body grows something that doesn’t belong there, like extra cells or a tumor. Cancers are neoplasms but not all neoplasms are cancerous. Indolent systemic mastocytosis is not cancerous. Even without taking drugs to kill off lots of mast cells, the prognosis is excellent with a normal life span. However, aggressive systemic mastocytosis and mast cell leukemia are considered cancerous. Without taking drugs to kill off mast cells, the body would be unable to cope with the huge number of mast cells and the damage they cause. Smoldering systemic mastocytosis is sort of a bridge between ISM, which is benign, and ASM, which is not.

If you are not aware that research papers usually use the term systemic mastocytosis to mean all forms of systemic mastocytosis and not just indolent systemic mastocytosis (ISM), it is easy to get confused and misunderstand what is being said. There was a paper published in 2009 that discussed expected survival for the various forms of systemic mastocytosis. It provides a very jarring statistic for patients who may not understand the context. This study found that many patients with systemic mastocytosis died 3-5 years after diagnosis.

Let’s pull this apart. We know there are five forms of SM: indolent SM, the most common form, which usually has a normal life span; smoldering SM, which usually has a shortened life span; aggressive SM, which can have a very shortened life span; mast cell leukemia, which has a very shortened life span; and SM with an associated hematologic disorder, which may have a shortened life span. When you average the life expectancies for a mixed group of patients with these various diagnoses, it shows that overall, SM patients are more likely to die 3-5 years after diagnosis when compared to healthy people of the same age.

Additionally, a lot of the patients in this study group were older and died of causes unrelated to systemic mastocytosis. However, because they were part of the study, their deaths of unrelated causes were still included in this data.

Let’s recap: in a research paper, the term systemic mastocytosis includes forms of SM that are malignant and can really shorten your life expectancy as well as forms that are benign and do not shorten your life expectancy. When you average the life expectancies of all of these forms together, it looks like patients are more likely to die 3-5 years after diagnosis. A bunch of other papers then used the data from this study in 2009 without explaining the details behind it. However, most patients with SM have normal life spans.

For more detailed information, please visit these posts:

The Provider Primer Series: Diagnosis and natural history of systemic mastocytosis (ISM, SSM, ASM)

The Provider Primer Series: Natural history of SM-AHD, MCL and MCS

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 46

56. Why do I react every time I eat?

When you swallow food, your nervous system sends signals to tell the cells in the stomach that food is on the way. As a result of this neurologic signal, hormones are released to tell your stomach to get ready to digest. These hormones cause histamine to be released by cells in the stomach. The histamine tells your stomach to make acid to digest your food. Solid food is more activating to the stomach in this way than liquids are.

This is a normal function of the body and happens in everyone, not just people with mast cell disease. However, histamine released in the stomach can activate mast cells and cause typical mast cell symptoms. Like everything else in mast cell disease, how much this affects patients varies a lot. But something to keep in mind is that a lot of mast cell patients who are “allergic to everything they eat” are actually reacting to the normal histamine release that contributes to digestion. They are essentially allergic not just to what they are eating, but to the process of eating.

57. Do I have to go to the hospital every time I use an epipen?

Unless you have received very explicit instructions not to do so from a health care provider that is familiar with the particulars of your life and your health, you need to go to the hospital every time you use an epipen. The reason for this is because an epipen is a temporary measure. The purpose of the epipen is to give you time to get to a hospital for more advanced care. Epinephrine is broken down by your body in a matter of minutes so it only provides a small window of protection. While many patients only need one epipen, there is no way to know if you will have another wave of anaphylaxis after the first one. Additionally, many patients require other medications and IV fluids to treat anaphylaxis. These can be provided at a hospital.

The reason you have to go to the hospital is to give you access to more comprehensive care, not because using an epipen is dangerous.