Tuesday, May 10, 2016

May is Ehlers Danlos Syndrome Awareness Month!

May seems to be a prevalent designated awareness month for numerous chronic health conditions.

Ehlers Danlos Syndrome is one of them.

Now, I know what you are probably thinking - "Oh no, here she goes on another awareness rampage." Those thoughts may be correct. However, consider waking up tomorrow rather ill. Perhaps with the flu, because that is the extent of sickness most healthy people have experienced. Now, imagine having that flu every single day of your life for the remainder of your existence. That flu impacts all aspects of your life: each decision, your abilities, your relationships, your schooling, your career, your independence, and so on.

Chronic conditions are similar to the above imaginary scenario (with the exception that they are generally life threatening when the flu usually is not for a healthy person). The influences chronic conditions have on life are vast. This makes awareness extremely important. The only way to a cure is through awareness. How can that occur if afflicted patients like me do not go off on awareness tangents on occasion?

So, what is Ehlers Danlos Syndrome? 

 “Each type of Ehlers Danlos Syndrome is defined as a distinct problem in making or using one of the types of collagen. Collagen is what the body uses to provide strength and elasticity to tissue; normal collagen is a strong protein that allows tissue to be stretched but not beyond its limit, and then safely returns that tissue to normal. An analogy: If one builds a house with bad materials, with cheap nails or only half the wood required, problems will arise. Some problems are more likely to show up than others, but because the bad or missing materials are everywhere and not necessarily visible, one can be surprised where some problems occur.

It is much the same thing with EDS and collagen. The collagen with which a person with EDS is built is not structured the way it should be, or only part of it is produced. With a badly built or processed collagen, the tissue that relies on it can be pulled beyond normal limits and thus be damaged. Collagen is the most abundant protein in the body and types of collagen can be found almost anywhere: in skin, muscles, tendons and ligaments, blood vessels, organs, gums, eyes, and so on.

The problems resulting from one’s body being built out of a protein that behaves unreliably can be widespread, in a wide range of severities, and show up in places that seem unrelated until the underlying connection to EDS is recognized.” [ x ] 
Upon googling EDS, you might take notice that the joint and skin problems are abundant. To be specific, joint laxity "double jointed-ness" soft and stretchy skin are all signs. Those lead to frequent joint dislocations/injuries, as well as poor wound healing. Did you know that some with Ehlers Danlos dislocate or partially dislocate their joints multiple times a day? Are you aware that the frequency of "multiple" times a day can be hundreds depending on the severity? We fall apart with every step...literally!

While the above symptoms are rather troubling, Ehlers Danlos Syndrome is more than the patient's ability to contort their body or not healing well. The tissue fragility in the various types of Ehlers Danlos Syndrome can potentially result in dysfunctional organs. After all, 80% of the human body is composed of collagen. Point blank: our organs can fail and/or rupture on us at any given time.

In summary, other signs and symptoms are: easy bruising, organ prolapse, organ rupture, hernia, chronic pain, muscle weakness, heart problems (mitral valve prolapse, aortic dissection, heart murmur, etc), bleeding, gastrointestinal conditions (Gastroparesis and other motility disorders), and more. [ x ]

Although I am personally not effected as severely as others in respect to the joints and skin,  Ehlers Danlos Syndrome causes a multitude of secondary conditions in certain cases. A common secondary ailment is called Dysautonomia, which is the dysfunction of the autonomic nervous system. A common subset of Dysautonomia is Postural Orthostatic Tachycardia Syndrome. To put it simply, upon standing blood fails to pump back up to the brain due to the laxity of the vessels. If you notice an EDS patient fainting after moving to an upright position, that is probably why. A plentiful number of patients are also diagnosed with Mast Cell Diseases; although, the correlation is not understood one hundred percent.

Other comorbids are: like mentioned above - Gastroparesis, Chronic Intestinal Pseudo Obstruction, Osteoporosis, Hiatal Hernia, arthritis, Chiari Malformation, Raynauds, and more. All patients are not identical. Presentation varies. While some have all of the above additional diagnoses, others have none.

It is not rare for an EDS patient to go undiagnosed or misdiagnosed for years while they suffer from the unknown malady plaguing their bodies. Who would have known children at school remarking "ew, gross" while gawking at my arm resting in a strange, contorted position or simple joint dislocations as an infant was indicative of the medical mess I would eventually become?! Not me. Not my family. And not my doctors either. This must change.

Overall, Ehlers Danlos Syndrome is a serious condition in dire need of awareness. What will you do for awareness this May?

For additional information, visit EDNF.org.