I recently wrote a post intending to address this but got into a tangent, so here goes take two!
Connective tissues are in every part of our body, as are nerves, blood vessels, and mast cells. So, any one disease affecting these can affect your body pretty extensively, but the combo is crazy. I sat down today to list all of my symptoms and came up with more than 80. And I'm sure I'm missing some. Here's a quick rundown of how each system can be affected:
Musculoskeletal -- obviously the joints are made of collagen, therefore EDS affects mobility and joint function. EDS/POTS can also cause swelling. The constant movement of lax joints can cause small-fiber neuropathy, which can in-turn cause other issues with fine motor skills etc.
Mouth -- joint issues in the jaw, Sjogren’s (common comorbidity that I might have) causes dry-mouth, this plus iffy collagen in your gums causes receding gums and mouth sores.
Esophagus/swallowing -- whether from nerve issues, general lack of proprioception, crappy collagen, or some combo, EDS patients often have trouble swallowing. I also have some instability in my cervical spine (neck), which makes swallowing painful/more difficult at times. Note: it’s great that people with swallowing issues have to swallow a bajillion pills, huh?
GI tract -- poor collagen = weak sphincters, which leads to GERD and heartburn. MCAS also causes an increase in stomach acid, which further exacerbates the GERD. POTS can cause nausea and vomiting, as can vagal irritation and disk slippage from cervical (neck) instability. Gastroparesis is also common with EDS, and although it’s not as acknowledged, intestinal paresis may be as well. At any rate, the motility issues from poor collagen/propreoception in the gut can cause constipation, diarrhea, or a combo. Because of these motility issues, bacterial overgrowth is common, from the colon bacteria getting into the small intestine. Also, when we eat, our blood rushes to the gut causing postprandial depression (aka food comas), which can then trigger POTS, since our low blood volume and dysregulated vasoconstriction/dilation doesn’t leave enough blood in the brain. Mast cells are also very present along the whole GI tract, so mast cell flares can occur in the gut, causing pain, vomiting, gas, diarrhea, etc. Finally, central sensitization makes nerves extra likely to send pain signals. The gut houses a huge portion of our body’s nerves, so pain can be triggered even when the other complications are quiet.
Cardiovascular system -- blood vessels are made of collagen, so they are too stretchy and in some cases (especially with vEDS), prone to tear/rupture. Hearts are also made with quite a bit of collagen, so there are structural issues that can happen (I thankful do not have any of those). POTS, as I’ve mentioned, can be caused from too much blood pooling in our feet (or wherever), causing overall blood pressure and brain oxygen levels to plummet. This causes the brain to freak out and ramp up the heart rate to get blood back to the brain. This can cause fainting, presyncope (almost fainting), tachycardia (racing heart rate), palpitations, shortness of breath, etc. The stretchy vessels also cause swollen hands/feet/legs and red splotches on our skin. It can make it difficult to shower comfortably as well.
Breathing -- this one is tricky. I’ve been complaining that I can’t breathe forever, but it was assumed to be anxiety. The thing is, I think a lot of times it is anxiety. But it can also be caused by a number of other issues, and it can be hard to tell what’s what. Vocal Cord Dysfunction (VCD) is fairly common with EDS, again because our tracheas and vocal cords are made with collagen. They can be stretchy, sensitive, and easily irritated. My airways sometimes feel swollen and irritated. I get a cough for days on end, but without having a cold. If I try to breathe too deeply, it often triggers a bad cough that hurts deep in my chest, like bronchitis, followed by many minutes of wheezing. I also respond well to albuterol, which means airways in general can get narrowed. It’s tough to say what’s EDS and what’s MCAS/allergies. I certainly get wheezy when exposed to MCAS triggers. On top of that, POTS can make it tough to breathe. I can’t put my finger on why, I just know that the more horizontal I am, the less I feel like I have to gasp for breath. One theory is that the brain is deprived of oxygen, so in addition to setting your heart aflutter, it also makes you hyperventilate. Another theory is that our vasoconstriction functions are off, so blood pressure in the lungs/brain goes up and down, affecting how much oxygen we get.
Cognition -- I’ve often written about how frustrating brain fog, memory issues, aphasia, and other cognitive issues are. They can all be caused by a number of factors -- EDS forces the sympathetic wing of our autonomic nervous system to activate all day just to keep us upright. This can slow down the prefrontal cortex, which makes speech and things like logic or math difficult. It’s also exhausting, so we often feel brain fog simply from fatigue. Lack of oxygen in the brain can obviously cause cognitive dysfunction. I’m not sure of the mechanism, but MCAS triggers often cause brain fog as well. In fact my friends now fondly tease me for getting “cookie drunk” -- eating certain trigger foods, like cookies, makes me floppy, foggy, silly, clumsy and make my speech a bit slurred.
Nervous system -- the impact here is staggering. EDS can cause damage to nerves all over your body. The dura, membrane around the brain and spinal cord, is made of collagen. You can have spontaneous leaks of fluid from your brain. POTS and EDS cause imbalance of the autonomic nervous system (fight/flight vs rest/digest). Nerves can get pinched when joints, especially vertebrae, move around. Central sensitization happens from the constant sensation/discomfort/movement/pain in your joints → my gut tells me that central sensitization has a bigger role to play in all of this than experts realize as of yet.
Skin -- MCAS can cause skin to flush, itch, have hives, etc. Sjogren's causes extreme dryness. EDS causes weird bruising, scarring, and tearing of skin, as well as unexplained stretch marks and varicose veins. I've had capillaries pop from pressure (like lifting a box or vomiting) which makes my skin speckled. Central sensitization and MCAS can make skin feel very sensitive to pressure, rough textures, etc. POTS makes skin feel taught from swelling. MCAS and POTS can both make skin sore/tender. The combo of dryness and weird collagen makes skin crack easily. In the winter, I typically have at least five bleeding cracks in my fingers, often upwards of ten or more. In fact, my iPad stopped recognizing my fingerprint this week, because I have so many cracks and scabs.
Headaches -- tension headaches can occur from the muscle spasms holding our heads up with floppy spines. As mentioned above, blood pressure in the brain can be volatile (from vasoconstriction/dilation dysregulation and/or blood pooling in the extremities), brain fluid can pool and/or leak, among other complications that can all cause types of headaches. There are many structural issues that can happen in the brain, such as chiari malformation, which again I thankfully do not have. MCAS can also cause migraines.
Fungal infections -- it’s unclear whether it’s an immunodeficiency issue or something else, but MCAS patients seem to be prone to fungal infections. I have some on my toes (since infancy), have had ringworm in the past, and many of us have candida overgrowth (fungal infection in the intestines).
Endocrine/reproductive systems -- The research on why hormones are tied into all of this hasn’t really happened yet, but it’s clear that hormones play a role. Many of us have PCOS, endometriosis, or other hormonal abnormalities. Many zebras also have thyroid issues, and structural abnormalities in pelvic organs can happen due to stretchy collagen. Temperature regulation seems to also be a common issue.
So yeah, when I don't take impeccable care of myself and symptoms flare, it's fairly all-encompassing. When I go to the doctor and they ask for my top two concerns that day, well... what would you say?