Types of Mast Cell Disease
The three major forms of mast cell diseases are: (1) Mast Cell Activation Syndrome (MCAS), (2) Hereditary alpha Tryptasemia (HaT), and (3) Mastocytosis.
Mast cell diseases can cause tremendous suffering and disability due to symptomatology from daily mast cell mediator release, and/or symptoms arising from the infiltration and accumulation of mast cells in major organ systems.
Mast Cells
What are Mast Cells?
Mast cells are immune (white blood) cells found throughout the body that live in the bone marrow and in internal and external body tissues (including connective tissue), such as the gastrointestinal tract, the lining of the airway, and the skin.
Mast cells play many complex and critical roles in keeping us healthy. They protect us from infection, and help our bodies by participating in the inflammatory process. Mast cells are also involved in allergic reactions, from your sniffly nose reacting to pollen to the tiny swelling that appears after a mosquito bite to life-threatening, full-blown anaphylaxis and everything in between.
How Mast Cells Work
Mast cells contain small sacs, or granules, which are surrounded by membranes. The sacs contain many different kinds of substances called mediators, such as histamine, which participate in all the roles mentioned above. The mediators are selectively released when there is an allergic or mast cell-based reaction.
When triggered, mast cells can react explosively, producing and releasing into the tissues and into circulation a wide range of mediators, each of which has a vast array of effects on other cells and tissues to help the body resist and recover from the assault.
This triggering is called "activation," and the release of these mediators is called "degranulation." Mast cells can also be activated by other substances, such as medications, infections, insect or reptile venoms, vibration, fragrances, chemicals, etc.
When Mast Cells Go Wrong
Someone with a mast cell disease has mast cells that may be activated inappropriately in response to triggers that shouldn't cause such a reaction, and these mast cells may also proliferate and accumulate in organ tissue.
Mast Cell Activation Syndrome
Understanding MCAS
Everyone experiences mast cell activation - it is a normal, healthy process. Without it, we'd be in trouble. Mast cell activation becomes problematic and diagnosable as MCAS when the mast cell activation is abnormal and inappropriate.
In other words, when an individual responds with allergic-type of reactions (including anaphylaxis) in response to triggers that shouldn't otherwise cause a reaction, you're a likely candidate for an MCAS diagnosis.
MCAS causes chronic inflammation in multiple organs systems, with or without allergic-type problems and sometimes even abnormal growth and development in various tissues. People can experience acute flares of symptoms.
Recent Recognition
Specialist physicians officially recognized MCAS as a disease in 2010 at an international Mast Cell Disorders Working Conference. This recent recognition explains why so many of us went years - even decades - without explanations for our symptoms.
Hereditary alpha Tryptasemia
What is HaT?
Hereditary alpha Tryptasemia (HaT) is an inherited genetic trait where an individual has at least one extra copy of the alpha tryptase gene (TPSAB1). Tryptase is a chemical (protein) produced by mast cells during an allergic-type reaction, and it can circulate in the bloodstream.
The additional tryptase copies result in increased levels of tryptase detected in the blood, whether a reaction is happening or not. HaT may be a disease-modifying factor, rather than a disease-causing mutation. In other words, HaT may modify an individual's diseases, causing them to present differently (and potentially more extremely) than they typically would.
Personal Connection to Research
Fun fact: my twin sister and I were in the 2015-2016 NIH study that led to the "discovery" of HaT; I have a duplicate of the alpha tryptase gene, she does not. You can learn more about HaT on The Mast Cell Disease Society's website, from a publication authored by one of the lead researchers of the HaT study.
Mastocytosis
Understanding Mastocytosis
Mastocytosis occurs when there is an abnormal accumulation of mast cells in one or more organ systems. There are two main forms of Mastocytosis:
(1) Cutaneous Mastocytosis
Affects only the skin and is more common in children
(2) Systemic Mastocytosis
Affects more than one part of the body and is more common in adults
Mastocytosis is usually caused by variations in the KIT gene. Most cases are caused by somatic genetic changes, meaning they only occur in certain parts of the body and are not inherited or passed on to the next generation. Mastocytosis can, however, affect more than one person in a family. A Mastocytosis diagnosis may be confirmed with a skin biopsy or bone marrow biopsy.
Learn More
I recommend you check out The Mast Cell Disease Society to learn more about Mast Cell Diseases and discover valuable resources.
Mast Cell Reaction Symptoms
Cardiac & Circulatory
- • Hypotension (low blood pressure)
- • Hypertension (high blood pressure)
- • Heart palpitations
- • Fainting
Dermatologic
- • Flushing, pruritis (itching), rashes
- • Urticaria (hives), edema (swelling)
- • Acne-like folliculitis
- • Pregnancy-like purplish lines/bands ("striae")
- • Dermatographism (redness in the track of a scratch)
- • Poor healing, nail issues
Gastrointestinal
- • Abdominal pain, bloating
- • Nausea, diarrhea, vomiting
- • Reflux (GERD), constipation
Neurological
- • Brain fog, cognitive difficulties
- • Anxiety, depression, tremors
- • Headaches, migraines
- • Lightheadedness, dizziness, vertigo
- • Tingliness, numbness, weakness
- • Sleep disruptions
Respiratory
- • Nasal congestion, nasal and throat itching
- • Runny nose, watery eyes
- • Shortness of breath ("I can't catch a deep breath")
- • Wheezing, coughing
- • Bronchitis, obstructive sleep apnea
Emergency Reactions
- • Anaphylaxis: Life-threatening allergic reaction; immediate systemic reaction caused by rapid, IgE-mediated immune release
- • Anaphylactoid reaction: Immediate systemic reaction that mimics anaphylaxis but is not caused by IgE-mediated immune responses
Additional Systems Affected
Endocrine:
- • Weak bones, bone lesions, bone pain
- • Painful/irregular periods
- • Thyroid abnormalities
- • High cholesterol or triglycerides
- • Blood sugar fluctuations
Other Systems:
- • Genital Tract: Decreased libido, inflammation
- • Hematologic: Easy bruising and bleeding
- • Immunologic: Unusual sensitivities/allergies
- • Joint: Migratory pain and swelling
- • Lymphatic: Enlarged lymph nodes
Potential Mast Cell Triggers
Food & Substances
- • Food and alcoholic beverages
- • Preservatives, pesticides, additives
- • Leftovers
- • Drugs (opioids, NSAIDs, local anesthetics)
- • Contrast dyes, excipients
Physical & Environmental
- • Friction, vibration, mechanical irritation
- • Heat, cold, sudden temperature changes
- • Emotional, physical, social, or environmental stress
- • Chemicals and fragrances
- • Environmental factors, allergens (grass, pollen, mold)
Biological Triggers
- • Infections (viral, bacterial, or fungal)
- • Venoms (bee/wasp, jelly fish, snake, spider, fire ant)
Products & Materials
- • Toiletry and beauty products
- • Clothing and upholstery, dyes
Understanding Excipients
What are Excipients?
Excipients are the inactive ingredients that are added to medications and that are known to cause reactivity in patients with mast cell diseases. The same drug can include very different excipients when produced by different manufacturers (e.g. between a brand name version and generic versions).
It's important to learn how to read drug ingredient labels because that gives you the best chance at avoiding triggers whenever possible.
Types of Excipients
Lubricants
Prevent pills from sticking together. Examples: silica and magnesium stearate.
Binders and Fillers
Provide volume to pills and bind ingredients together. Examples: cellulose and polyethylene glycol.
Coatings
Protect pills from damage, make them easier to swallow, and may provide "time-release" functions. Examples: shellac and gelatin.
Dyes & Flavorings
Add color and taste. Examples: FD&C red #5, FD&C blue #2, sucralose, xylitol.
Preservatives
Improve medication shelf life. Examples: citric acid and retinol palmitate.
Tips for Navigating Excipients
- • Try to limit or avoid taking medications and supplements with excipients
- • Research all ingredients using the National Library of Medicine's website
- • Always note the manufacturer on the label of any new drug
- • Ask your pharmacy to special order drugs made by a particular manufacturer
- • Look at the children's section for dye-free medications
- • Consider medications from other countries (like Canada) for cleaner drugs
- • Work with compounding pharmacies for custom formulations
- • Keep a record of which excipients you tolerate and which you don't
Compounding Pharmacy Recommendation
Compounding pharmacies can often produce drugs with just the active ingredient. It's important to do research when choosing a compounding pharmacy to ensure that you're working with a trustworthy entity that's following all the rules for sterility, cleanliness, etc. I work with and trust Harbor Compounding.
Learn from the Expert
Expert Resources
Learn about living with and diagnosing MCAS from compassionate expert, Dr. Anne Maitland.
Management
Understanding the Challenge
Mast cells are found in connective tissues all through the body, especially under the skin, near blood vessels and lymph vessels, in nerves, and in the lungs and intestines. It is because mast cells are located in all parts of the body that people can experience such a wide range of mast cell reaction symptoms.
Everyone is different and everyone's body and needs change over time. The management recommendations listed below include things that I currently use and benefit from, things that I have used in the past and benefitted from, and things that I know others use and benefit from.
Allergic-Immunologic Management
Prescription and Over-the-Counter Drugs
Histamine 1 Blockers (H1 Blockers)
Help with itching, abdominal pain, flushing, headaches, brain fog and general mast cell stability.
Hydroxyzine (Atarax), Doxepin (Silenor), Cyproheptadine (Periactin), Loratadine (Claritin), Fexofenadine (Allegra), Diphenhydramine (Benadryl), Ketotifen (Zaditen), and Cetirizine (Zyrtec, Reactine).
Histamine 2 Blockers (H2 Blockers)
Help with gastrointestinal symptoms and overall mast cell stability.
Famotidine (Pepcid, Pepcid AC), Cimetidine (Tagamet, Tagamet HB), and Ranitidine (Zantac).
Mast Cell Stabilizers
Help with gastrointestinal symptoms and overall mast cell stability.
Cromolyn (Cromolyn Sodium, Gastrocom—oral form, Nasalcrom—nasal spray, Opticrom—eye drops, nebulized cromolyn, homemade cream), Ketotifen, and Hydroxyurea (Hydrea).
Additional Medications
Leukotriene Inhibitors
Help with respiratory symptoms. Montelukast (Singulair), Zafirlukast (Accolate), Zileuton (Zyflo).
Anti-IgE Therapy
Helps with asthma, anaphylaxis. Omalizumab (Xolair).
Low-dose Naltrexone (LDN)
Can boost immune system, lower inflammation, improve motility, improve sleep. Must be compounded.
Emergency Treatment
Epipen should be used in response to anaphylaxis (life-threatening allergic reactions).
Supplements
Mast Cell Stabilizers:
- • Quercetin, HistaQuel, Bromelain
- • Stinging Nettle, N-Acetyl-Cysteine
- • Ginger, Vitamin D, PEA
- • Green Tea, Curcumin, Chamomile tea
- • Resveratrol, DAO enzymes, Vitamin C
Binders:
- • Charcoal, apple pectin
- • Bentonite clay
- • Fulvic acid, humic acid
Lifestyle Tools
- • A glass eye flush cup for rinsing out allergens (use only reverse osmosis or distilled water)
- • Saline nasal sprays for flushing out the nose
- • Air purifiers for filtering allergens and impurities
- • Humidifier, dehumidifier as needed
- • Lymphatic drainage techniques
- • Stress management
- • Flush out the body via hydration, lymphatic drainage, sweating (sauna), gentle movement
Gastroenterologic Management
The Gut-Mast Cell Connection
Addressing gut health is essential for managing mast cell issues. I went from having a frighteningly restricted diet (and undiagnosed SIBO) and reacting to everything I came into contact with to having an increasingly abundant diet and decreased reactivity to the world once my SIBO was resolved.
Unfortunately, in my experience, most conventional gastroenterologists and mast cell specialists do not pay sufficient attention to diagnosing and treating underlying gut health issues that have an enormous effect on mast cell diseases. Once I started branching out to add functional and integrative medicine specialists to my team, my ability to manage my mast cell issues and gut issues improved drastically - and my diet continues to expand.
Important Caution About Restrictive Diets
I spent a lot of time on incredibly restricted diets without adequate guidance. I learned later that, while this type of restriction can be essential short-term and can seem like the only option in the moment, this level of restriction can be detrimental long-term and it's important to work closely with a team of providers to ensure that these restrictions don't last longer than absolutely necessary.
This is not to say that anyone should push themselves into a less restrictive diet that causes symptoms or to make anyone feel guilty for having a restricted diet; it's simply a word of caution against thinking that restrictive diets are a safe permanent option without consequences.
Common Problematic Foods
The internet is full of lists suggesting people with mast cell issues avoid certain foods, but it's important to pay more attention to your own symptoms than to generic lists. Some of the most common foods that cause many people with mast cell diseases to experience troubling symptoms include:
- • Fermented foods
- • Alcohol
- • Fish
- • Food dyes and flavorings
- • Preservatives
- • Leftovers
Conducting an elimination diet can be helpful to identify baseline foods while working with a qualified team to address gut health and other issues that will hopefully allow for a more full diet. Check out my nutrition guide for more information.
Key Takeaway
Mast cell diseases are incredibly complex, and there's no one-size-fits-all approach to navigating the symptoms and the underlying causes. Patience, perseverance, and out-of-the-box thinking have been key to addressing my gut and mast cell issues.
Dermatological Management
The Gut-Skin Connection
In my experience, skin issues are generally connected to, if not directly caused by, gut issues. For me, addressing my gut issues has made the most enormous difference in my skin reactivity, rashes, hives, blemishes, etc.
Masto Cream Recipe
For the first few years after being diagnosed with MCAS, I relied heavily upon the masto cream recipe (below). If I didn't cover every single millimeter of my body with this cream after showering, I would experience horrible itching and hives in the uncovered areas.
Recipe Option 1:
1 tsp glycerin + 1/4 cup of Vanicream or Eucerin or other thick lotion + 5 ampules (tubes) of Gastrocrom (cromolyn sodium)
Recipe Option 2:
2 tsp glycerin + 1/2 cup of Vanicream or Eucerin or other thick lotion + 1 whole bottle (0.44 fl. oz.) of NasalCrom
Eventually, after years of healing my gut, addressing issues with my nervous system, changing how I ate, lowering my overall histamine load, and overhauling the types of products I put into my body and onto my skin, I slowly noticed that I wasn't relying on the masto cream anymore…and I haven't needed to use it for at least 3 years!
Product Changes That Made a Difference
One of the biggest impacts for me has been moving away from using products that are filled with chemicals and preservatives (e.g., sodium benzoate, etc.) and instead using products that are more natural and made with a simpler (shorter) list of ingredients.
Doing my best to eliminate coconut and coconut-derived ingredients from my skin and hair products has also been a huge gamechanger (and nearly impossible to do!).
Recommended Resources:
- • The Detox Market
- • Credo Beauty
- • This New Knew
These are go-to resources for "non-toxic" beauty products (note: "non-toxic" is an unregulated term, which is why it's important to learn how to read labels).
Environmental Toxicity
The Modern Toxic Environment
Our ancestors' environments were vastly different than the environments in which we are living in modern times. This enormous difference can cause our mast cells to go a little crazy in their attempt to keep up with our increasingly toxin-laden world where our vast exposure to environmental toxins provoke our mast cells to act up and release the inflammatory mediators that cause our deeply unpleasant symptoms.
Exposure to concentrated doses of environmental toxins or chronic low-level exposure to these toxins can trigger a process known as toxicant-induced loss of tolerance (TILT) meaning that, as your immune system is exposed to these toxins, its tolerance of them steadily decreases until a tipping point is reached, disrupting your mast cells to such a degree that they begin malfunctioning.
Lifestyle Tips for Reducing Toxic Load
I recommend Dr. Aly Cohen's books Non-Toxic: Guide to Living Healthy in a Chemical World and Integrative Environmental Medicine, as well as other resources she provides on her website. A few examples of how to reduce your toxic load include:
- • Avoiding smoke
- • Removing shoes at the door
- • Steering clear of pesticides and chemicals when being sprayed
- • Avoiding gasoline fumes from power equipment
- • Choosing glass or stainless steel water bottles instead of plastic
- • Becoming an educated shopper for beauty products, cleaning products, and furniture
- • Opening windows for fresh air whenever possible
- • Using air purifiers throughout home and office
- • Filtering water using carbon or reverse osmosis filter
- • Eating organic foods when possible
- • Choosing grass-fed, grass-finished meat
- • Using shower water filters
Recommended Products
Beauty Products:
The Detox Market, Credo Beauty, and This New Knew
Cleaning Products:
"Non-toxic" cleaning and laundry brands include Better Life, Dirty Labs, and Seventh Generation
Safety Checking:
Reference the Environmental Working Group's website to check the safety of certain ingredients
Testing & Detox
Testing Options:
- • Great Plains Laboratory MycoTox Panel (tests for mycotoxins from mold)
- • Great Plains Laboratory TOX Panel (tests for toxic non-metal chemicals)
Detox Tools:
- • Supplements: Sulforaphane (Avmacol), Glutathione, N-Acetyl-Cysteine, Glycine, Milk Thistle
- • Binders: charcoal, chlorella, bentonite clay, apple/fruit pectin
- • Sauna
While our bodies are designed to detox naturally, it's important to work with a practitioner who can assess your individual capacity for detoxing and develop a plan to boost your ability to detox as necessary.
Stress Management
The Stress-Mast Cell Connection
Stress activates mast cells, causing them to release prestored and newly synthesized inflammatory mediators and induce increased blood-brain barrier permeability, recruit immune and inflammatory cells into the brain, and cause mast cell degranulation in several tissues.
In other words, stress can worsen mast cell symptoms. Stress can stem from physical, emotional, and/or environmental factors. Incorporating stress management techniques into your lifestyle and eliminating avoidable sources of stress are critical for managing mast cell diseases.
Neurological Balance
When the nervous system is unbalanced, patients with mast cell diseases are more likely to struggle with symptoms. Treating mast cell diseases without paying attention to how the nervous system is functioning will prevent a person from making as much progress as they otherwise could.
Moving to a calmer, quieter, more nature-y part of the country where we're surrounded by the beach, mountains, and have bunnies in our yard, has played a tremendously profound role in lowering my overall stress levels, raising my neurological trigger threshold, and allowing my body to truly begin healing in a way I've never experienced before.
Developing a feeling of safety in my body and in the world via medical hypnosis and neuro performance training has been hugely beneficial in managing my neurological and mast cell reactivity.
Nervous System Calming Modalities
- • Sleep optimization
- • Breathing exercises
- • Meditation, yoga nidra
- • NuCalm
- • Nature exposure
- • Hypnosis
- • Neuro performance training
- • Vision training
- • Vestibular training
Read my recommendations for stress management for more detailed guidance.
Lymphatic Activities
Educational Resources:
- • Lisa Leavitt Gainsley is a Certified Manual Lymphatic Drainage Specialist - her Instagram account is filled with lymphatic drainage video tutorials
- • "The Big 6" - Dr. Perry Nickelston shows his "Big 6" technique for lymphatic drainage
Activities & Tools:
- • Bounce lightly up and down or use a rebounder (mini trampoline)
- • Any movement, including walking
- • Gua sha, dry brush
- • Mini trampoline
Care Team
Recommended Care Team
Medical Specialists:
- • Allergist-Immunologists specializing in mast cell diseases (or willing to learn)
- • Gastroenterologists specializing in mast cell diseases (or willing to learn)
- • Functional/integrative/naturopathic medicine doctors
- • Primary care provider
- • Pain management specialist
Complementary Care:
- • Medical hypnosis provider (MD, DO, or PhD)
- • Acupuncture, acupressure
- • Mental health professionals, including psychiatrists, psychologists, trauma specialists, counselors, etc.
Care Team Recommendations
Visit our Provider Directory for recommended specialists who understand mast cell diseases and related conditions.