Velvet
My skin is softer than most of the general population's. Apparently, when freshly smoothed, it is particularly pleasurable to the touch.
I've been told this many times by the men who have been given the freedom to put their fingertips upon me. This might sound arrogant. It's actually a medically documented fact.
One of the ways my type of EDS is diagnosed is through examining our skin, and the official medical term for the way it feels is 'velvety soft'.
Sounds good, right?
Appealing.
Something so many feminines strive for. It's a subject worth exploring because to you, we're a pleasure to touch. But for Bendies, our velvet could kill us...
Close Shave
One of the ways my type of EDS is diagnosed is through examining our skin, and the official medical term for the way it feels is 'velvety soft'.
Sounds good, right?
Appealing.
Something so many feminines strive for. It's a subject worth exploring because to you, we're a pleasure to touch.
But for Bendies, our velvet could kill us...Honestly, getting that freshly smoothed feel is not easy for an EDS body with skin like mine. Shaving cuts, no matter how careful we are, are always a hazard. One of mine left a notable a scar on the back of my knee.
I still remember picking the slice of skin out of the blades. Done that more than a few times - not my favourite kind of close shave...
My legs can only really take a shave once or twice a month now, because my collagen production and quality has and will continue to worsen with age. Creams don't work and waxing is just useless to me for a few reasons. The hairs snap rather than being removed, it pulls at my fragile, velvety coat and it hurts a body already sensitised to and in constant pain. Been there, done that, not doing it again.
My body is covered in small scars from the slightest of injuries that didn't even break the surface.
My skin's inability to properly heal itself was first noticed when I was around two-and-a-half years-old. I'd fallen, smacked my forehead on the corner of a unit in our front room.
Blood was everywhere and my young parents, still as yet undivorced, sped down the dark country lanes to the local hospital, my tiny frame on my mother's lap and a wad of toilet paper pressed up against the bleeding. I can still see that tissue. It was a peach colour, with brown lines forming diamond shapes, their junctions marked by a little flower. For this memory alone, I thought I must have been older, but no.
Being numbed wasn't a problem then, and I have no recollection of my cut being stitched, but it was, with six sutures. Having those sutures removed is an event I remember - though, fortunately, not in full detail. The tickling of the thread as the nurse pulled it though my flesh still sits with me, as does my curiosity and the matchbox I took to collect the removed threads.
Always an arachnophobe, I still see myself cautiously opening that box in the back seat of the car on the way home to peep at what had held my head together, thinking the remnants looked like spider's amputated legs. I didn't look at them again and the box was lost.
What stayed forever, was the scar. It is not a thin line down the middle of my forehead as you would expect stitched wounds to be once healed. This is where the knowledge of my mother comes in. My wound reopened. "When she took the stitches out," my mother once told me, "I thought she'd cut you! She hadn't. Your cut just opened and started bleeding again."
This scar is a couple of inches long, about 4-5ml wide and sits in the centre of my forehead. Others have asked the younger me if I would have it removed if I could. My answer has always been no. It's a unique feature. It doesn't bother me at all. It never has. What does bother me is the fact that the nurse did not think to flag my reopening wound or order further investigation into this obvious abnormality in healing.
Especially given that my mother had noticed several issues with my body prior to this in my infancy and I was learning to walk, but was dismissed every time she tried to discuss it with my doctor. A medical trend only too well-known and often experienced by people living with Ehlers-Danlos and the parents trying to get care and help for their children.
The medical skin examination I mentioned? It includes looking at your scars, particularly the ones that were sewn up, for signs that the wounds reopened or widened (the edges drifting apart instead of staying together in a nice line) once the sutures are removed. Of course, this immediately rules out surgeries in the future. If the condition requiring the operation doesn't kill you, the inability to heal surgical incisions will...
Growing up, I was fortunate in that I didn't suffer any more accidents that needing stitching and I didn't need surgery. Life-saving surgery, anyway. Things would get very complicated in my Twenties and Thirties, but before explaining those complexities, it's time for the sciencey bit.
Both of these facts are
contrary to old-school
medical thinking on EDS.
This is problematic.
In certain cases,
it ends lives.
Sciencey Bit
Most people know what collagen is, kind of, nowadays. Back in the Nineties and Noughties when trying to explain Ehlers-Danlos Syndrome to people, even this was a stumbling block. Collagens are the proteins our bodies are made of.
Our bodies synthesise different types of collagen proteins according to the body part they make and that part's function. For example, our blood vessels and internal organs are not made of the same collagen proteins as our skin.
Different types of EDS are caused by a malfunctioning in this synthesising process. We know that the gene COL3A1 causes Vascular EDS (VEDS) and my Hypermobile EDS sees mainly a different set of collagen proteins being effected than the Vascular EDS type, where the aorta, blood vessels and internal organs are not made properly.
Sounds simple enough. But in 2005, a study found something that wasn't widely talked about then, but that the two main EDS charities talk about today as if they have made a new discovery.
Prior to 2005, there was no proof for us to take to any medical professionals that backed-up our claims of internal ruptures or bleeding issues. The EDS establishment refused to budge on the idea that internal organ rupture only happens in the Vascular type (VEDS), which is known to shorten life expectancy and kill through your aorta popping or splitting, or your internal organs rupturing, and bleeding to death. Sometimes, surgical attempts are made to save lives by wrapping a kind of net scaffolding around the aorta or threatened internal organ. However, the surgery itself carries a high risk of death because of the inability to properly heal.
In 1998 and 1999, I surveyed many EDS bodies online, speaking to people in seven or eight different countries. We all agreed that the experts were wrong, and our type does rupture - we just couldn't back it up in any way other than with our own physical experiences and losses.
Enter 2005 and the TNXB gene.
TNXB is the gene responsible for Tenascin-X protein production. Think of Tenascin X as a glue that binds our collagen proteins together and helps structure our very bodies properly, providing support for your muscles, ligaments, tendons, internal organs...It's pretty important glue...
While it is said that the gene causing Hypermobile Ehlers-Danlos Syndrome has not yet been found, what is known is that many of us HEDS bodies have problems correctly making Tenascin X. This recorded fact immediately opens the doorway to aortic and internal organ rupture. Of course, if HEDS is not traceable in your family's history, it is entirely possible that a gene mutates and one of your relative then has the Syndrome.
From that point forward, the condition can run through your future family. If one gene can mutate, so can a second one, and so, from a medical perspective, having two types of EDS because of genetic mutations is entirely possible.
It may just not be well observed or recorded. Both of these facts - the Tenascin X involvement and the mutations - are contrary to old-school medical thinking on EDS. This is problematic. Not only is it behind-the-times, it also impacts negatively on our lives. In certain cases, it ends lives.
In short, people with HEDS who have the deliciously velvety skin can and do have issues with Tenascin X expression and production which can lead to blood vessel and internal organ fragility and rupture, as well as poor skin healing, easy scarring and wounds that reopen when stiches are removed. Stretchmarks are another thing that come with this soft skin - which will be covered in their own page some time.
On an everyday level, this means that big bruises that didn't cut my skin can scar me, scratches that don't even bleed scar me, as do IV needles and the stitches themselves. It rules out surgeries I may need in the future to treat things that can hit anyone. Earlier, after re-researching this area, I found information on how the COL- genes responsible for causing various types of EDS also act in bodies without EDS. Something that stunned me and that I'll talk about another time.
I hope you're starting to see that what seems like a nice feature to have is actually the mark of a condition that not only causes lifelong intractable pain, but that also kills in hideous ways - including making routine surgeries too dangerous to even contemplate.
The extra bagginess is
a physical manifestation
of our bodies being unable to make
themselves properly, and all the
pain, debilitation and loss
this brings to our lives.
My Skin Coat
For this page, I chose images that showed my skin coat getting baggier and baggier. Of course this happens with age, and that is an ignorant cry from a non-EDS body's insensitive mouth.
Getting baggier naturally as you age is not the same as having a connective tissue disorder and experiencing the bagginess. Why?
Because the extra bagginess is a physical manifestation of our bodies being unable to make themselves properly and all the pain, abuse, debilitation and loss this brings to our lives.
Believe me - ageing and ageing with EDS are not the same.
There are certain angles and poses that make my skin coat catch and wrinkle more than they would in a normally constructed body. To many eyes, it will be unattractive. The care I have for their opinions could not be less. Yes, I would prefer tighter skin. Who wouldn't?
Especially when in middle age. But my body, despite all it's struggles, all the times it nearly couldn't cope, the times it would have given up had the medics not intervened, the carrying and birthing of my son, the experiences I have managed to have in life, the love and sex it has given and received, the lives it has allowed me to save, the things it has allowed me to learn, the abuses it has taken from myself and others, even the pain from it...For the compassion and strength, the toughness it has given to my mentality...My fragile, soft, scarred, velvety skin has survived them all.
I would not be the same person if I did not have Ehlers-Danlos and all its companion conditions. Many times I have thought about a life without disability, without never-ending agony. Would I choose it, if I could?
On one hand, of course I would prefer a life without increasingly bad pain and limitations, knowing both are only ever getting worse.
On the other, that would mean accepting a different me and I don't know if I would want that.
Neither do I know if I would want a thicker skin when this harsh world needs more velvet.
