Is EDS progressive?

Is Hypermobile EDS progressive?

I would say the answer is yes, and no, and there is a lot of debate about this.

Genetics:

From a genetic standpoint, many argue that it is not progressive, and as far as the genetic codes are concerned, that is true. Our defective collagen and connective tissue will always be coded by our genetic code. However, epigenetic changes increase through life with environmental, nutritional, and toxic influences as well as lifestyle choices, and that is shown to speed up aging by cause misfolding of proteins which does increase over time, even in healthy individuals. This is progressive and cumulative to each individual, and since we already have defectively formed collagen, and it is a protein, there will be progression over time in epigenetic misfolding which can make our tissue manifestations progress, even with the same genetic code. Some of this progression can be slowed or reversed with lifestyle modification, nutrition, avoiding toxins, etc., as is true with any person aging in the modern world. But epigenetic changes are progressive through a lifetime.

Cumulative wear and tear:

This is cumulative in any human being, but individuals with EDS are show to have more wear and tear on their joints and, therefore, more rapidly progressive arthritis. Arthritis really means “inflammation of the joints” from the Greek roots of arthritis. “Arth” originates from the Greek árthron,which means “joint” or “jointed.” “-itis” is also of Greek origin and means “inflammation.” With EDS we have increased, more widespread, and earlier arthritis due to instability in the joints that causes increased wear and tear throughout life. Our joints and skeletons show aging changes earlier than our non-affective counterparts. We accumulate more injuries, joint damage, and tissue damage faster than the general population. We accumulate progressive instabilities faster, although this does happen with the general population also, just not as rapidly. And, for the record, when you get a radiograph that says “no evidence of arthritis,” that does not mean you do not have arthritis. If your joint hurts then you have inflammation of the joint! It should actually read, “No bony changes YET evident due to arthritis”

Hormonal:

Estrogen and Progesterone -

Menopause causes hormonal changes and can make EDS feel much more progressive during perimenopause and after menopause. It is hard to find definitive “scientific studies” on this, but as my geneticist told me, progesterone makes collagen more stretchy and estrogen stabilizes it. She also said that the dose of estrogen to help would be toxic, so they don’t use that as a treatment. There are, however, some who have said in patient forums that hormone replacement after menopause can help with symptoms. This also explains why some women have issues with cyclical worsening of instability during their monthly cycles. Think of our ligaments as “old bathing suit elastic.” When you have a new bathing suit, it stretches and snaps back, but when the garment gets old it stretches… and stays stretched. That is my metaphor for what happens with our ligaments, either cyclically when young, or progressively through perimenopause and menopause. But at this time in history, we can’t reverse menopause, so the tissue changes are progressive with menopause even if the genetic code is still the same.

Testosterone -

Aging causes a progressive decrease in testosterone in both men and women. Testosterone is key in building and retaining muscle mass. In skeletal stability, ligaments are the primary stabilizers connecting bones to bones, and collagen defects cause ligaments to be too stretchy when young, and break as we lose that stretch (particularly with loss of estrogen, but this also happens with “normal” aging) Or it can break due to “normal trauma” when young just because of the excess stretching. However, muscles are the secondary stabilizers of the skeleton. That is why EDS specific physical therapy and exercise are essential to functioning. Our muscles have to do extra work to make up for our defective ligaments to try to give stability to the skeleton. This is also why we can have unexplained muscle spasms as our muscles fight to keep our skeleton aligned.

Postural integrity:

Our entire body communicates. If we injure a knee it can put excess stress on a hip and all the way up the spine. So one injury can lead to more injuries in the postural chain. Also, with our current lifestyle of sitting in school and being sedentary in work, we are constantly stressing the ligaments of our spine, especially since we sit to be comfortable, and are not always thinking of our posture. But regular slumping, and even the forward head posture in our texting world, puts stress on the ligaments that hold your spine straight. That is one reason why many of us have spine issues including degenerative disc disease and disc herniations. This can be worse with EDS because our primary stabilizers, our ligaments, put more strain on our discs. At my last thoracic MRI, I had 7 herniated thoracic discs out of 12! And when the discs herniate, you start showing bony changes in the vertebrae due to excess pressure because of lack of the disc cushioning. Our spine is the center stability of our entire body, and we do get progressive damage. And then we walk and sit funny which causes damage to other joints due to mechanical defects in our movement.

So is EDS progressive? Not in a genetic code sense. But as with normal aging, damage is cumulative and progresses in rate as we age through hormonal changes, as well as lifestyle causing epigenetic changes and problems with protein folding. And aging is progressive, EDS causes us to manifest aging changes faster so we develop arthritis at an increased rate compared to the general population. In that respect, EDS causes more rapidly progressive skeletal changes, arthritis, and instability than the general population.