What is EDS, POTS, MCAS?

What is EDS you ask?

EDS stands for Ehler's Danlos Syndrome. It's a hereditary connective tissue disorder that causes our body to produce faulty collagen. Collagen is basically the glue that holds everything together in our body. With collagen being in nearly all parts of the body, it affects much more than joints. This includes joints, muscles, ligaments, organs, nervous system, vascular system, eyes, ears, & more. Our joints dislocate easily, or have partial dislocation known as a subluxation. Our muscles & ligaments are weak which means our body has to work much harder just to do normal activities such as standing, walking, etc. This causes extreme fatigue. It also means that the weak muscles & ligaments in our neck, struggle to hold up our head causing cervical instability, & damage to the spine. There are 13 different types of EDS. My kids & I all have type 3, also know as the hypermobile type (hEDS). Type 3 does not have a known gene associated with it at this time while the others have. Many studies are being conducted in hopes of determining this gene as well. While our family shares many symptoms, we are also each unique. No two people with EDS will be affected in exactly the same way which is why EDSers are considered "zebras". Just like zebras, no two have the same stripes. Doctors are trained in medical school that if they hear hoofbeats, not to expect a zebra, but rather the more common horse. However, some of us actually are zebras!

There is no cure for EDS. At best some believe that certain supplements such as Lysine, & vitamin C which are known to be beneficial in collagen production, may help to lessen symptoms. Disclaimer- I am not a doctor & it is best to talk with your physician about any supplements that you are considering. Also, be aware of any & all fillers in any medications & supplements as it's possible to have a reaction to them as well.

Welcome to my very first blog post! It is my hopes that my blogging site will help to

spread some much needed awareness about EDS, POTS, MCAS, & all of the other many things that typically come with it. It's also my hope that this will in some way help to inspire someone to keep fighting whatever battles that they too might be fighting. When darkness falls, focus on the star.

Postural Orthostatic Tachycardia (POTS)

POTS is a form of Dysautonomia which is a dysfunction of the autonomic nervous system controlling all automatic functions of the body.

EDS also causes many secondary/comorbid disorders such as Postural Orthostatic Tachycardia Syndrome (POTS) which is a form of Dysautonomia. Dysautonomia is a dysfunction of the autonomic nervous system controlling all automatic functions of the body. This includes heart rate, blood pressure, breathing, digestion,& more. Due to the faulty collagen our vascular system is more stretchy which allows the blood to pool to the lower parts of our body. Gravity is our enemy for this reason. When we stand up we are not getting enough blood, or oxygen to our heart & brain. This causes our blood pressure to typically drop & our heart rate to soar (my highest 235). Some have what is called hyper POTS & they have trouble with high blood pressure. For me, mine typically drops low enough to faint, but I also have times of high blood pressure. Many people with POTS have trouble with fainting (syncope), or near faints, but not all. Our teenage daughter has syncopal seizures which means that when she faints, she goes into a seizure. POTS can be caused by a variety of things, but for us the cause is Ehler's Danlos Syndrome & so no cure, but there is things we can do to help. Many days are a battle though.

Treatment for POTS

1. The typical & more natural aproach to aid in controlling POTS is lots of salt. We use Pink Himalayan salt as we like to keep things natural & healthy. The other two things are electrolytes, & good old fashion, spring water. Yes, I mentioned spring water for a reason, tap water is generally full of chemicals which tend to set off our mast cells, allergic reactions, & chemical sensitivities. We personally drive 30 minutes away to a park who has a free flowing fountain from a natural, very deep underground spring. The water is regularly tested & many swear by it's high quality. It's about the only water that we have found that doesn't cause us to react. Many use Gatorade drinks, but some are can not due to sensitivites to some of the ingredients, I am one of those. Our oldest son recently discovered an electrolyte drink called Body Armor that has no artificial ingredients, no artificial sweeteners, & no alcohol based sweeteners. I gave it a try & I believe it just might work! No reactions so far! Just make sure to stick with the regular type & not the "lyte" version if you are sensitive to alcohol based sweeteners. 2. Various medications are used "off label" to help in controlling POTS. With our chemical sensitivities we have not been able to go that route. 3. Compression stocking are highly sought after by many with POTS & found very helpful to control blood pooling. For me, tight clothes cause a flare of my MCAS & I break out in hives so a definite no. My daughters have sensories issues & therefore can't stand the tightness. 4. Mobility devices. Some only have mild symptoms of POTS & can lead a fairly normal life. For others it is completely debilitating to the point of bedridden. I myself have been progressively declining to the point of a wheelchair, & now a custom powerchair. I can not walk very far before having a flare of symptoms, so I am currently a part time wheelchair user. There's times when I try to push myself to walk into a store for just an item, or two. After all, it won't take long (famous last words, lol), but I always pay for it with a flare. If lucky, it's only a mild flare & not passed out on the floor with people staring at me. Pushing yourself into a flare is NOT recommended so please don't follow my lead on that one. I admit, I'm to bullheaded for my own good.

Mast Cell Activation Syndrome (MCAS)

A type of white blood cell that is found in connective tissues all through the body, especially under the skin, near blood vessels and lymph vessels, in nerves, and in the lungs, intestines, & even bone marrow. Mast cells are part of the body's immune system, but with MCAS they are easily triggered into crazy mode.

Mast cell activation syndrome occurs when the mast cells in your body release to many at inappropriate times. Mast cells are actually needed & typically protect us. When we're exposed to stress, or danger, our mast cells respond by releasing substances called mediators. Mediators cause inflammation, which helps our body recover from an injury, or infection.

This also happens during an allergic reaction. Our mast cells release the mediators to remove the harmful allergen. This is done by releasing a mediator called histamine, which makes you sneeze to get rid of well say, pollen for example.

If you have MCAS, your mast cells release mediators too frequently and too often. It’s different from another mast cell disorder called mastocytosis, which happens when your body makes too many mast cells. Triggers for MCAS can be most anything from pollen, mold, dust, chemicals including smells/oders, perfumes, deoderants, air fresheners, cigarettes, asphalt, gas fumes, smoke, stress, foods, textures, bug bites/bee stings, temperature change, sunlight, & even our own sweat to name just a few. Not each individual will have the same triggers, or be affected in the same way. Some will have it only mildly, while others live their world in basically a bubble, avoiding nearly everything.

Tracheomalcia

Tracheomalacia is also known as floppy trachea, or floppy windpipe. Due to EDS causing faulty collagen, the trachea walls are to weak. This allows the walls to swing back & forth & even collapse on itself. Tracheomalacia is more common in infants, than adults, however, it's also common among those with EDS. Our youngest daughter & I both have this. It is so terrifying whenever she has complications from this as her episodes (we call them breathing attacks) are completely silent. This has surprised all of her doctors as it typically causes some kind of sound such as stridor, but not in her case. The only way that we know she is in trouble is if we can see her, or if she can pound on something to get our attention. We have plans to get her a service dog of her own that will be task trained to bark & alert us whenever she needs help.