Living with the EDS/POTS/MCAS trifecta, plus some.
**Note: for someone else's thorough explanation of my diseases, click here!
In the last few months, as I’ve begun figuring out strategies to feel better, I’ve encountered some interesting reactions from the people in my life. I’ve had the pleasantly surprised “look at how much energy you have after 8:00 pm!” and “it seems like this diet is really helping!” I still have bad days (the joys of elimination diets -- you have to repeatedly make yourself sick by adding new foods), and I’ve become less ashamed of expressing how I feel, so some people are actually seeing negative change. From these people, I’ve received an interesting mix of concern, support, and unsolicited advice (some helpful, some quite aggravating). Since most people have only observed the surface of what’s going on and are naturally curious, the most common response I get is something along the lines of “so… what exactly is wrong with you?”
What a fascinating question.
First of all, being pretty inept at social cues, I never quite know whether to give them the short answer -- I’m on a diet to figure out what I’m allergic to -- or a much longer answer:
I have a genetic disorder called Ehlers-Danlos Syndrome.
For an in-depth description, check out www.ehlers-danlos.com/, but the gist is that my collagen isn’t made correctly, meaning that all of my connective tissue is defective. Anything that needs glue holding it together is extra stretchy and prone to dysfunction. This includes skin, intestines, joints, blood vessels, etc. As you can imagine, this can cause a whole host of multi-systemic problems, but the most common issue for zebras (people with EDS) is joint problems and widespread pain. Over-stretched muscles can be strengthened and tightened, but ligaments are like old rubber bands -- once you stretch them too far, they never quite bounce back to their original length.
So, if you let your muscles get weak, even temporarily, you end up stretching your ligaments until they are permanently made useless.
Another complication of EDS is that our blood vessels are too stretchy. To someone with hypertension, this may seem like a dream. True, it does come with certain benefits -- I can eat as much salt as I want and will never have high blood pressure -- but it also causes some issues. Blood pools in my legs and arms, like water in a loose balloon. This causes swelling, to the point where sometimes my feet won’t fit into my shoes and rings become painfully tight on my fingers. It also causes low blood pressure, because the vessel walls stretch rather than push the blood along. I am often light-headed. My heart has to work extra hard to get blood to my head, and sometimes it just gives up. You know that head-rush feeling you get when you stand up too fast? That’s pretty much every time I stand up. Any change in my posture takes awhile for my body to react.
This leads to the next diagnosis, Postural Orthostatic Tachycardia Syndrome. POTS is a form of dysautonomia, or dysfunction of the autonomic nervous system (the part of your brain controlling heart rate, blood pressure, breathing, sleep, adrenaline, digestion… you know, all of those fairly important automatic reactions that keep us alive and standing). When you change posture, your blood pressure shifts due to gravity, so your heart rate naturally reacts in order to keep your blood pressure steady. When someone with EDS stands up, our blood pressure drops super low. If that person also has POTS, their brain overreacts to the BP drop, and tells the heart to freak out and get the blood pressure back up. This causes head rushes, tachycardia (heart pounding/racing), and various other symptoms.
Speaking of dysautonomia, my team of doctors is still trying to tease out mine. In addition to POTS, I have odd sleeping patterns, messed up hormones, periodic fluctuations of anxiety, and a disastrous digestive system. These are all controlled by balancing the sympathetic (fight or flight) and parasympathetic (rest and digest) wings of the autonomic nervous system. The theory is that people with EDS have to keep themselves in a constant state of adrenaline in order to just stay upright, since our skeleton is naturally too floppy to stay up on its own.
When the sympathetic wing is active, the parasympathetic doesn’t function as well, so it makes it tough to sleep or digest.
In addition to these more common forms of dysautonomia, I also have isolated incidents of extreme nausea, vomiting, diarrhea, and/or presyncope (difficulty staying conscious). This could be caused by irritation to my vagus nerve (more on that here), some other form of dysautonomia, or potentially from my third diagnosis…
Mast Cell Activation Syndrome was my most recent discovery. It has not been officially diagnosed, since it is still a relatively new discovery and testing can be pretty hit-or-miss. I have almost all of the symptoms, though, like diarrhea, sensitivity to a million foods, constant allergies, nausea/GERD, headaches, etc. Thankfully I do not experience any anaphylaxis, as some with MCAS do, because mast cells are the white blood cells that cause allergic reactions.
White blood cells are supposed to protect us from germs, but sometimes they see danger in things like tree nuts or cat fur.
For people with MCAS, pretty much anything could trigger a mast cell reaction -- foods with histamine (egg whites, acids, anything not super fresh… there’s a long list and it varies from person to person), excessive heat or sun, vibrations (from cars, loud music, movie theaters, etc), and a million other random occurrences in daily life. I’m lucky enough to not only react to histamine, but also foods with salicylates and oxalates (pretty much all fruits and veggies). Reactions can cause subtle allergic symptoms like itchiness or post nasal drip, or extreme fatigue, migraines, diarrhea, full-body inflammation/pain, and much more. Each person is wildly different, which is part of why it’s so difficult to diagnose. Some good resources for more info are mastcellaction.org, tmsforacure.org, and a great support website: mastcellsunited.com. The good news about MCAS is that treatment can be really helpful! Low-risk, over-the-counter allergy meds can make a big difference, and for some people diet and lifestyle changes can pretty much eliminate any symptoms.
Although many people with MCAS don’t have POTS or EDS, there is a strong co-morbidity (frequency of people having both). It is likely that they are genetic mutations that developed together and are therefore passed along by similar groups of people. Living with one of these diseases can be tough. Living with all three, what I’ve often heard called “the trifecta,” is difficult to describe. It often requires having to decide which disease to take care of, as the care for one often triggers the others (see "Catch 22" -- coming soon).
Added to all of this, I am a Highly Sensitive Person. It might seem silly that I am capitalizing these words, but I do not just mean that I am emotionally sensitive (although I am).
HSP is a way doctors are describing people whose nervous systems are wired to take in extra input. Check out the link or google it for more info, but it means that I am very intuitive, in touch with my surroundings, have stronger emotions/senses, etc. It also means that I feel everything -- the wind and the sand, as well as pain, hunger, and nausea -- stronger than normal people. I can feel everything happening inside my body. I have gone to doctors knowing exactly what was wrong and then been told there’s no way I could actually be feeling that (only to then have testing show I was right). I also get overwhelmed by the vast amount of stimuli that my brain is processing. Strong sounds, smells, even emotionally exciting experiences, can make my body start to shut down in the form of migraines, fatigue, and an intense need to be alone. Like many Highly Sensitive People, I am extremely introverted. So, even without all of my various health complications, being out and about in a society that values extroversion can leave me exhausted.
I am deeply grateful that my particular case of EDS is very mild.
I am not wheelchair bound and am able to minimize my pain with simple exercises and heating pads. My POTS is also quite mild and is fairly easy to regulate by just eating disgusting amounts of salt, wearing compression stockings, and trying to remember to stand up slowly. My MCAS is not particularly mild, but I can currently eat about 10-15 ingredients (I know people who are stuck at fewer than 5) and I do not experience life-threatening reactions to my triggers. Any one of these diseases would barely affect my quality of life. I may never have realized I was different than anyone else if I’d just had EDS or POTS. But when people try to understand what it is that’s wrong with me, I find myself struggling to convey the cumulative effect of having the trifecta, plus being a hypersensitive introvert.