Patient Perspective on HαTS

As of March 2022, I can finally see the end of the journey I began in 2017. I was so ambitious and blithely unaware of just how difficult it would be to earn a clinical doctorate in Traditional Chinese Medicine (or is it Integrative Medicine). The colleges in California are giving this degree different labels these days. I was in the middle of a major midlife overhaul, and I still had adolescent kids at home. Ah, what the hell, I said. Sure, I lived with chronic migraines and an autoimmune disorder, but time is going to pass anyway. I might as well pursue something meaningful. I didn’t want to let my health problems get in the way of my personal ambition and hope.

I laugh at that idealism now because chronic health problems do indeed get in the way of personal goals and hopes! I can’t begin to describe how much they have interfered with my personal agenda, but there’s a wonderful seam of good news running through this narrative. I moved to California to begin a new chapter in my life as well as continue my doctorate. An unexpected benefit of moving was new eyes on my health history. I met with a new allergist, new rheumatologist, new neurologist, and new hematologist. With their fresh perspectives, they saw something that my former doctors did not, and, of course, they ordered all new tests including genetic testing and the dreaded bone marrow biopsy.

And they did indeed find something–Hereditary Alpha Tryptasemia–the underlying genetic condition that has been the bane of my existence for, well, decades. With a correct diagnosis I now receive better treatments and more effective interventions. I can now research any complementary and integrative medicine (CIM) interventions that may be helping individuals with mast cell diseases since HαT is classified as a mast cell disease. I can also make modifications to my lifestyle and diet in an attempt to prevent mast cell activation events and anaphylaxis.

Truthfully, I have found that living with Hereditary Alpha Tryptasemia Syndrome (HαTS) is challenging. The biggest challenge is the anaphylaxis. In my case, I have exacerbations or clusters of anaphylaxis usually brought on by accidental exposure to a known allergen–most often a tree nut. As an example, two weeks ago I had an accidental almond exposure while dining out. The restaurant had accidentally garnished a pie with ground almonds instead of coconut. I had a primary anaphylactic reaction to this accidental ingestion and a secondary rebound reaction the following day. Even when treated with epinephrine, 50 mg Benadryl, 20 mg cetirizine BID, and 20 mg famotidine, I will likely have a cluster of anaphylactic events which require a round of prednisone. Annoyingly, the anaphylaxis usually triggers a round of migraine headaches as well. As is common for mast cell patients, NSAID usage is now causing some mast cell activation for me which makes treating migraine pain challenging. I have also become allergic to anti-CGRP drugs (both Emgality and Aimovig injectables). This progression of drug “allergy” is not uncommon for patients with mast cell disorders.

This is a weird disease. While there is an actual diagnosis now which brings such relief rather than the former sexist medical shaming (“Are you sure that you’re not just getting your period, sweetie? Do you need attention? Here’s a Tylenol.”), I will say that my tendency is to overdo it on the symptom-free days because I fall behind on the sick days. And, I don’t know what to do about that. My guess is that’s the Chronic Disease Cycle, and it likely contributes to a perpetuation of symptoms and exacerbations.

From a Conventional Medical Treatment Perspective, a treatment protocol for Hereditary Alpha Tryptasemia Syndrome accompanied by a mast cell activation event might be:

Daily:

  1. cetirizine (Zyrtec): 10 mg BID or twice daily. After anaphylaxis episodes: 20 mg BID or 2 pills twice daily
  2. quercetin (Weng, Z., Zhang, B., Asadi, S., Sismanopoulos, N., Butcher, A., Fu, X., Katsarou-Katsari, A., Antoniou, C., & Theoharides, T. C. (2012). Quercetin is more effective than cromolyn in blocking human mast cell cytokine release and inhibits contact dermatitis and photosensitivity in humans. PloS one7(3), e33805. https://doi.org/10.1371/journal.pone.0033805)
  3. luteolin (Theoharides, T. C., Stewart, J. M., Hatziagelaki, E., & Kolaitis, G. (2015, July 3). Brain “fog,” inflammation and obesity: Key aspects of neuropsychiatric disorders improved by Luteolin. Frontiers in neuroscience. Retrieved March 22, 2022, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4490655/
  4. berberine (Fu S, Ni S, Wang D, Fu M, Hong T. Berberine suppresses mast cell-mediated allergic responses via regulating FcɛRI-mediated and MAPK signaling. Int Immunopharmacol. 2019 Jun;71:1-6. doi: 10.1016/j.intimp.2019.02.041. Epub 2019 Mar 9. PMID: 30861392.)
  5. Vitamin C, D, E (Kakavas, S., Karayiannis, D., & Mastora, Z. (2021). The Complex Interplay between Immunonutrition, Mast Cells, and Histamine Signaling in COVID-19. Nutrients13(10), 3458. https://doi.org/10.3390/nu13103458)
  6. Zinc (Kakavas, S., Karayiannis, D., & Mastora, Z. (2021). The Complex Interplay between Immunonutrition, Mast Cells, and Histamine Signaling in COVID-19. Nutrients13(10), 3458. https://doi.org/10.3390/nu13103458)
  7. Omega-3 fatty acids (animal or plant-derived) (Kakavas, S., Karayiannis, D., & Mastora, Z. (2021). The Complex Interplay between Immunonutrition, Mast Cells, and Histamine Signaling in COVID-19. Nutrients13(10), 3458. https://doi.org/10.3390/nu13103458)

Please note that you have to be careful with taking berberine as it inhibits certain liver enzymes which can affect blood levels of certain prescription drugs that use those enzymes. So, be sure to discuss any berberine supplementation with your doctor. A website that I frequently use is www.drugs.com. It’s very handy for looking up drug side effects and pharmacokinetics. Another extremely useful site is www.naturalmedicines.therapeuticresearch.com. You can find an abundance of information on herbal drugs and supplements on this site, and I use it frequently when looking up patient supplement information and writing case studies.

There are, of course, dietary interventions that we can and should undertake, and Eastern medicine has an entirely different perspective. But, that’s another post.

Some takeaways:

  1. All of us have natural limitations, and our bodies may speak to us about those limitations when we don’t hear them in various somatic “voices”. Two of those somatic “voices” may be pain and chronic conditions.
  2. Stress of all kinds can manifest in the body and cause symptoms of illness, pain, and disease.
  3. The Standard American Diet (SAD) is causing preventable disease and premature death.
  4. Sometimes just a few lifestyle changes can bring about dramatic changes in health.
  5. Patients need allies and advocates rather than blame and shame.
  6. Most people are doing the best they can with what they know and have.
  7. Many chronic diseases are preventable.
  8. Lack of equitable access to healthy and whole foods is a public health crisis.
  9. Trying to do anything while living with a chronic condition is very difficult.

Wishing you all good health, MJ

Plum blossoms blooming in Japantown Peace Plaza, San Francisco, CA