What Requires Maintenance?

I started this blog about 2.5 years ago after joking with my mom when the maintenance required light came on in my car. I want to use this as a way to encourage others going down the same road I am, as well as to be a resource for others starting this journey. For many people managing chronic conditions, especially rare diseases, the internet can be a major resource. In my case, we used the internet to ultimately find the doctor who diagnosed me and has been a huge part of managing my symptoms. Hopefully, this blog can be a resource for others on this same journey!

Here is what "requires maintenance" for me:

I was diagnosed with mast cell disease in January 2015 after about 8 years of progressing symptoms. Mast cells are produced in the bone marrow and located throughout the body. We need mast cells to survive as they are a part of our immune system; however, in my case, mine are hypersensitive and do not have an "off switch." This presents as my body having allergy-type reactions ranging from mild (flushing, hives, itching) to severe (full-blown anaphylaxis) that are caused by the mast cells degranulating (releasing their contents) in response to a trigger. Because mast cells are located in every organ, along the nerve pathways, and pretty much everywhere in our bodies, symptoms are systemic (i.e. impacting multiple organ systems) and present differently from patient to patient.

I do have systemic symptoms, but for me, there are a few areas that are more impacted than others.

  • For anyone who knows me, you know I have significant GI involvement. Part of my diagnosis was based on GI biopsies taken during an endoscopy. There are multiple ways my mast cell disease presents in my stomach and intestine. Mast cells are located along the nerve pathways and can impact the nervous system signals that tell the stomach and intestines to contract and move food down the GI tract. This can cause slow stomach motility which results in food not being digested and just sitting in my stomach. It can also impact intestinal motility as well can result in food (sometimes undigested) getting stuck in the intestines. This can cause nausea, what we call "rancid burps" (as I literally have food rotting in my stomach - think leaving food outside in the summer, i.e. 97 degrees, and add stomach acid to the mix...), vomiting, bloating, pain, etc.
  • Another way my mast cell disease impacts my GI tract is during digestion. Mast cells are involved in digestion so when we eat, they release their contents to help break down the food in the stomach. Because my mast cells are hypersensitive and don't have an "off switch," when I would eat, my body would be overwhelmed by the mast cell activity and instead of interpreting the signals as digestion, it would react to food as though it was an allergen. It got to the point where my reactions to eating were so severe that I was going into anaphylaxis from the digestive process. This is the main reason that I had Herman (my feeding tube) placed.
  • The last main issue that the mast cells cause in my GI tract is swelling. Just like if you are stung by a bee and have localized swelling, I get that same allergy-type reaction, just in my intestinal tract. When my body is reacting to food (or sometimes just any reaction not necessarily just food), I can get swelling in the lining of my stomach and intestines. This causes severe pain and discomfort and can mess up my intestine motility as well.
  • As I mentioned before, mast cells are located along the nerve pathways. When mast cells are reacting, the chemical mediators they release cause permeability of the blood vessels and disrupt the nervous system signals. This can result in my body not responding properly to changes in position (i.e. lying to sitting, sitting to standing, etc.) and the body being unable to regulate blood flow properly. This can result in abnormal changes in heart rate, rapid blood pressure changes, both of which can result in passing out.
  • The autonomic nervous system is the part of the nervous system that controls the "automatic" functions of the body, i.e. breathing, heart rate, digestion, temperature regulation, etc. When mast cells are triggered, they can interrupt the normal nervous system signals that regulate these "automatic" body functions. This can cause all sorts of fun (insert sarcasm here) symptoms like I mentioned above regarding GI motility issues, blood flow regulation, breathing, heat/cold sensitivities, etc.
  • Just like the rest of the body, there are mast cells located throughout the brain. For me, the biggest issue that I have with this (other than what I mentioned above) is brain fog. This is where my ability to think and process information like I used to be able to is impacted. I often have trouble with remembering things (who doesn't =P) so I have to write everything down. I know, everyone forgets things, but this is a major change for me. I used to have an incredible memory, and now I struggle to remember things that used to come easy. I also have difficulty processing information that I didn't used to deal with. In school, I used to be able to read a bunch of articles, pull the information I need, and communicate the ideas in my own words in research papers or journal proposals. Now, I really struggle with this. When I read an article (only one at a time or I really can't process things), I can retain the information, but being able to reword in my own words is incredibly difficult. Reading multiple articles at a time and remembering what I read is almost out of the question. I know that for some people what I just described may be normal. What I am hoping to communicate is that the difference in what I used to be able to do and how I process information now is how my mast cell disease has impacted the way I think.
  • I have also been dealing with balance issues and a loss of coordination. To be honest, we are not entirely sure of the mechanisms behind how or why this is occurring other than knowing that because mast cells are everywhere, they can present as all sorts of different issues. Thankfully a lot of my balance issues appeared to be connected to my low blood sugars (see below) but I am still working on figuring out where the coordination issues came from and how to regain that. 
  • For me, my pancreas is the main offender in the endocrine system. Part of it is related to me having been on tube feeds for over a year now. Because my pancreas is constantly being stimulated to produce insulin because I am on continuous feeds, I am experiencing what is known as "pancreatic burnout." Basically my pancreas is overwhelmed with the constant stimulation and while it was able to keep up for 8-9 months, now, it is struggling. This means that I deal with hypoglycemia often and also occasionally have high sugars. 
  • The other way my pancreas is impacted is in the production of digestive enzymes. Because the mast cells interrupt nerve signals, as well as interfering with general pancreatic function during reactions, my body does not produce normal amounts of pancreatic enzymes to digest food. When I do eat, I have to replace these by taking the enzymes in capsule form so I can actually digest the food and get the nutrients from it. 
  • I also have borderline adrenal insufficiency. Again, it's not entire clear if this is specifically mast cell driven or may be a separate issue. For now, we are monitoring it and choosing not to treat (since as soon as I start treating, I'll be on steroids for the rest of my life) and hoping that as we are better managing my mast cells, my adrenal function will improve as well.
  • The last major system I'll mention is breathing. I was misdiagnosed with asthma for years before we realized that it was likely a mast cell issue rather than asthma. Once we started treating my symptoms as mast cell related, things started to resolve. On any given day I can have shortness of breath, coughing, some wheezing, or chest heaviness/tightness. Usually these symptoms are triggered by an airborne allergen (such as perfumes, smoke, scented products, cleaners, etc.). Obviously while my other symptoms can be debilitating, my respiratory symptoms can be extremely dangerous. These are typically the reason I need my epi pen (sometimes it's due to throat swelling, but usually due to breathing issues).
These are the major issues I am managing (or not managing as the case may be sometimes...). There are many other symptoms I live with on a regular basis that I haven't specifically mentioned here (flushing, hives, itching, rashes, tremors, headaches, fatigue, etc.), remember, mast cells are everywhere in the body so I can experience symptoms in pretty much any body system.

Mast cell disease is not considered terminal and does not necessarily shorten my life span. The main risk with mast cell disease is anaphylaxis. Mast cell can be triggered by anything I mentioned above (food, scents, heat/cold, etc.), unknown triggers, or even things that happen in my body (i.e. low blood sugar can cause mast cell degranulation which can then, in turn, lower blood sugar...see the challenge?). The unpredictability is the hardest part of managing this condition. About half my reactions are to unknown triggers, so I always have to be prepared with emergency medications, epi pens, and just knowing that sometimes, there is nothing I can do.

Everyone with mast cell disease experiences it differently. That is one of the reasons it is so hard to diagnosis and treat. This is just my experience living with it and how it presents in my body. I hope this was at least somewhat interesting, and I'd love to hear from anyone else living with mast cell disease!


  1. I love the title of your blog! And as I read through I definitely relate although I do not yet have an "official" diagnosis. The only doc that somewhat gets it so far is my allergist PA. I found your blog because I was researching service dogs for MCAS. I jam in the midst of a terrible reaction, and was thinking how nice it would have been to have a warning and maybe it would t be so bad. My husband travels a lot for work and while I have kids and other animals, having a dog that has a job to take care of just me, I think would help greatly.

    1. Thank you for the comment! I'm not glad you're dealing with this stuff, too, but I'm glad this blog could be of some help. Leni has been a great addition to help manage my mast cell disease. I am so very grateful to have her! If you think a service dog could help, it is definitely something to research and look into. Feel free to find me on instagram (@kboka27) because I post a bunch about Leni and what she does for me. And I'd be happy to answer any questions if you have specific things you are wondering.