Tag Archives: Ehlers-Danlos

PREMONITIONS & REALITIES

Photo Source: http://www.novedadestarot.es

Years ago, I needed a bottle of essential oil to ward off the desert spiders that had invaded my former rental.  I found it at a store that sold odd herbs, good luck charms, amulets, candles, things pertaining to black magic, and items usually used by fortune tellers, or psychics as they’re referred to nowadays.  There had been a sign in the window that there were psychics available, but I really couldn’t justify spending money I didn’t have on an unproven science, although I was curious.

So, I perused the hamsas and evil eyes after I grabbed my oil and then a woman with wild, blonde, curly hair came out from somewhere and looked at me and then started to talk. She had noticed my necklace and told me she was a Russian Jew.  I said that I was, as well, or at least most of my father’s side came from there.  Wouldn’t she know that if she were psychic? She rambled on a bit, presumably to get my business and then left while saying, “Jews are very psychic,” to which I replied, “Well, I’m not.”  I paid for my oil and never went back to that store, but I go to a shop in the same complex every couple of months and think about that comment.

I get premonitions.  When my paternal grandfather was dying in the hospital, I visited him every night after work.  He was on a respirator and pumped full of morphine and every breath sounded like his last, but I knew when he was going to die. No one had told me that it was probably his last night and he looked the same: a comatose body in a bed in the ICU, but I knew.  On that last night, I kissed him on his high forehead, not so different from my own, and said, “I love you, Papa.”  He died early the next morning and I wasn’t there, but at least I got to say goodbye.

When I was young, I had this premonition that the number 22 was bad luck.  On the 22nd of that month, my brother and his friend were sliding down our staircase on vinyl pillows—a rather stupid thing to do—and my brother’s friend didn’t land right and broke his arm.  That was just the beginning of bad luck with the number 22 and I wouldn’t even fly on that day. When I hit my head this January, it was on the 22nd.

Oh sure, most people probably can relate or have had similar experiences—I never claimed to be psychic if you recall, but I wish I were.

But, one premonition that came to me within the last 10 years seems to be coming true, once again.  I said that I wouldn’t make it past a certain age: a milestone birthday which most people dread that is just around the corner.  I’d make it to that age, but not any further.  Do I care?  No I don’t, as long as my cat is okay.

You might wonder about that.  Life is so great; there’s so much to do and so little time; there are friends and lovers and soul mates; there is travel and adventure.  That’s not my life anymore.

My life starts the same everyday and has for the last 13 years: I wish I’d died in my sleep as soon as the alarm wakes me up from my medication-induced shallow sleep and the chronic pain hits me like a ton of bricks.  The pain feels like someone took my skin off, shrunk it 4 sizes too small, and put it back on my body so that the muscles and fascia are literally bruised to the bone from the compression.  I have to get out of bed and move and try to see with the new vision I was also blessed with.  I need hours in a recliner with a down pillow to get my pain down to a level 7 on the 1-10 scale, but I have to keep getting up, moving around, and stretching or it just gets worse as my muscles chronically go into spasm. I have to figure out what to eat when I’m down to less than 10 foods, and I’ll still break out in painful rashes and my GI tract will be a mess all day and night.  And at some point—usually after midnight if I don’t have a ridiculous appointment, I have to shower and deal with the upkeep that a human body requires unless you want to look like the vagrants in the parking lot here.  Then, there are the chores that are so hard, like cleaning my 300 sf room here at the motel and using the laundry room down the hall.

Most people do all this everyday without a second thought.  I know, I used to do it too, and I worked more than full-time and went to the gym and hung out here and there and had friends that I did things with.  But, now it’s sheer torture to just get through a day filled with nothing.  There is no meaning in a life without purpose and to stay alive, whatever that even means, just to live everyday in a body that’s a torture chamber is beyond my comprehension.  And if anyone thinks there’s a meaning in any of this, I’ll let you know right now that there isn’t.

However, I try.  I’ve been trying since 2001 and while I manage to make it through this same routine, or add more fun to it by throwing in the painful sclerals if I go out, doing errands, rushing the routine to get to an appointment, driving a 5-speed with seats that feel like they’re made out of concrete, and on and on, I’m so very tired of it.

I forced myself out tonight, in fact.  It’s been a bad week, month, year, decade, 13 years.  There was yet another shooting around the corner off a street I refer to as The Jungle.  Gun violence hits way too close to home for me and the PTSD I never wrote about gets triggered and everything goes haywire. Then, a guy who stayed at this motel the 1st time I was here came back—fresh from being released from a mental institution, which is where he should have been for life.

Back in late 2010, he was my neighbor and threw me from one side of the hallway to another after I got on him about all his illegal activities and highly annoying behavior in here.  He also destroyed at least 3 rooms in this motel, had a huge Rottweiler at one point that was anything but trained, got his ghetto cousin and mother, who was an old prostitute, a room in here as well, and the general manager could have cared less.  He only left when the rent went up and most of his type of people couldn’t afford to stay, and I left a few weeks later as I’d found a cheaper and seemingly nicer studio down the street which later became a sea of raw sewage, but this guy left owing over $4,000 in back rent and damage.  Well, seeing that the GM has some sort of white guilt and just feels so sorry for this poor, half-black guy and his phony sob story of being a combat veteran which must be part of his delusional disorder, he’s now back and if I could see like I used to, I’d buy the .380 I had 20 years ago because I apparently could have been a sharpshooter I was that good.

But, I digress.  I forced myself out tonight.  I had to do a few errands and thought I’d stop at a relatively inexpensive clothing store and perhaps cheer myself up by buying a new top that wasn’t from the thrift store, and I rarely even do that.  I found a couple of things after a lot of searching because I’m picky and don’t like cheap looking clothing and extra small is not a common size in this town.  The prices were too high for synthetics and cotton blends, another favorite down here, and I sure wasn’t paying for a label at that store, but I headed to the over-lit dressing room, regardless.

And there I was in the mirror and I had my sclerals in.  The old, gaunt-faced skeleton covered in black bruises and aubergine purpura and a venous pattern like a road map all from Ehlers-Danlos syndrome.  Everything looked awful because I looked awful.  I wanted long sleeves to hide the blue veins and hundreds of weird wrinkles stacked on my elbows due to the faulty collagen in my skin.  I wished I could wear my winter gloves to hide the acrogeria on my hands that makes them look like an old lady’s.  I only like V-necks, but all I could see were the bones and veins in my chest and my neck that’s so thin and vein-y it looks like my head is too big and that it’ll snap in half at any moment.  That was enough.  I know what I once looked like.  I remember being attractive and normal and that was stolen from me like everything else.  No wonder someone found my blog by typing “Ehlers-Danlos freaks” into Google.

So, I bought a long sweater with a hood that wasn’t worth $30 bucks—when it’s already warm here and will be 115° soon.  I went to my car, got on the freeway with the cars speeding past me at 90 mph, and cried the whole way back to the motel with the mashugana who just got out of the cuckoo’s nest on the top floor.

I have a joke with the staff here that the only way I’ll ever get out of this motel is in a body bag.  It’s a family legacy: my paternal great-grandfather died of a massive heart attack in his suite at the Biltmore, where he was residing while doing business in another state.  But, at least he died in the Biltmore and not a 2 star motel that now serves as a halfway house for mental patients.

My, what a confusing post.  It’s going backwards and forwards and sideways, rather like how my brain is these days.  It’s no secret that I don’t like blogging.  I already tried to stop once and even wrote a post that I was, but quitting is for failures said my father long ago, and as all I’ve done is fail since getting sick due to my body being some degenerative thing that I’m just stuck in, I forced myself to keep going.  I had my search engine crowd who needed information and then I met bloggers along the way, which I didn’t even know was part of blogging as I’d never really seen a blog until my youngest brother created this very blog for me to document my corneal collagen cross-linking experience—and that I have done.

To tidy up this ridiculously long and rambling post, which happens to coincide with the one year anniversary of the death of someone I cared about very much and is just adding to my depressed state, I am bidding adieu to my blog for now.  I may be back in 2 weeks and I may never be back, but I need a huge break from it and from WordPress and this unsettling feeling of giving so much of my time to others on here and getting very little in return.  To the very few people who have been a friend on here, I thank you and am sorry I can’t be there for you right now, but I can feel the sand trickling through the hourglass—that old premonition—and need this time to focus on myself and fold my cards if all else fails.

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WHY ARE YOU A DOCTOR?

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Recently, I had my follow-up visit with the PM&R doctor who agreed to see me after I was diagnosed with Ehlers-Danlos syndrome (EDS), classical type.  I had seen him a couple times prior to that and was originally his patient for my misdiagnosis.  PM&Rs are physical medicine and rehabilitation specialists, and I personally feel they can be a good fit for EDS patients as they specialize in people with chronic, debilitating diseases and conditions.

I originally saw my doctor before my collagen cross-linking surgery and long before I had my scleral contacts that I’m forced to wear when I have to go out and I literally couldn’t see his face or make eye contact with him.  However, it wasn’t solely due to my vision—it was also due to something I didn’t pick up on for awhile.  You see, my doctor has some type of social phobia and does things like stand as far across the room from me as possible, tear through my file like a mad scientist while not really looking for anything, avoid eye contact with me at all costs, and the most bizarre: putting his head down on the counter when we were in a very small room and he couldn’t escape my gaze or me.  So, the faceless doctor image could also be replaced by a faceless patient I suppose, or an image of this patient talking to the wall to not upset the doctor.

Now, his behavior makes any type of interaction rather difficult, but I’m not one to make fun of anyone with a phobia—I just think they should choose their profession better.  For example, as I tend to have panic attacks on airplanes and yes, I saw the news about the triple-7 that is somewhere at the bottom of the South China Sea southern Indian Ocean, I didn’t become a pilot. Perhaps, I would have been an excellent pilot as I’m very systematic and prefer to be in charge, but darn it, I just can’t get over that free fall back to earth.  So, I went into the non-profit sector instead.  I have many reasons to wonder why this man became a doctor, so here’s a rundown of how my appointment goes to justify my thoughts, and mind you that I have to drive about 45 minutes to get to his office with my less-than-stellar vision.

  • I arrive and have to fill out the same form that doesn’t really address any of my issues and is too hard to see and best of all, my doctor never even looks at it.

  • The medical assistant calls me back and then I get to sit and wait in a chair that kills my body in a boring exam room for up to 45 minutes, when I’d be more comfortable in the waiting room with padded chairs and a big plasma TV I can actually see.  Per the wait, I was going to be 5 minutes late once due to an accident and called from my cell (these are things I should not do with my vision) and my appointment was cancelled and then I was slapped with a huge, no-show fee that didn’t go away until I called someone I knew in the office who took it off my bill.

  • The doctor finally comes into the exam room and asks how I am.  How should I respond when I already wrote “the same” on the form?  I’m also in more pain and beyond irritated by that point.  So, I tell him that I’m hanging in there as he doesn’t really care what I say.

  • He doesn’t ask me about my EDS or how it affects me physically.  He doesn’t offer to get involved with my physical therapy to make it more effective, and he writes my scripts for it.  He doesn’t ask how my EDS affects me emotionally, even though I circled on the form that my anxiety has been worse since I saw him last.  I really question how much he knows about EDS, even though he said he was familiar with it before I saw the geneticist a little over a year ago.  He tends to speak in a hushed tone and when a medical student was with him once, I vaguely heard him say that people with EDS are tall. Sure, a subset of people with EDS have a Marfanoid habitus, but I’m not one of them—that was my maternal grandfather.

  • I basically try to think of something to say to make my appointment worthwhile, but what’s the point when you don’t have a doctor who cares or is interested?  I’ve even asked the one medical assistant I like if he’d rather not have an EDS patient since I formerly saw his nurse and now he’s stuck with me, but she said that’s not the case. So, what is the case?  And if you’re wondering why I don’t just go elsewhere, trust me that I’ve been everywhere in this town and this is as good as it gets.

  • I have my few prescriptions written and there’s a problem with them every time, but as my scleral lenses don’t fully correct my vision and other factors affect my near vision, I tend to need help reading them.  This time, he wrote the dosage of one of my drugs wrong, so I would have run out early.  His handwriting is awful even for a doctor and the assistant wasn’t sure what it said for a couple minutes, but then she rewrote it and had him sign it and he asked her what the problem was and she lied and said that the pharmacy would have trouble reading his handwriting which really bothered me, but I decided it was best to stay quiet about his mistake.  On another prescription, the medication was crossed out and then rewritten, so I hope that won’t raise any flags.

  • And that was it!  Nearly an hour of driving, who knows how long spent waiting to see him, five minutes or less accomplishing nothing in the exam room, and 15 minutes of wasted time and frustration dealing with his inability to write prescriptions.  Then, I had to check out with the rude girl who feels so important behind her counter—the same girl who had informed me on the phone that my fabulous nurse had left the practice and slapped me with the no-show fee in the saga that I wrote about in this post.

So, why is he a doctor—especially for patients like me?  I could easily walk into an exam room, naturally not look at people without my lenses in, not offer much help—although I feel I know much more than this one does—and then write poorly written scripts due to my vision just like him.  He’s not much older than I am so the burn-out hasn’t started yet, not that I like that excuse because I knew some great, older doctors back home.  And if you have a social phobia, then go into research, because those of us with chronic, life-altering diseases that will only get worse need empathetic doctors who actually care and can look us in the eye and put their hands on us in order to feel a pulled trapezius muscle, or tendinosis in a rotator cuff, or subluxated shoulder joints, or any of the other things my physical therapist does on a regular basis and all the doctors missed from day one.

If my PT could write my scripts and order the imaging I have to pull teeth to get, I’d just have her be my doctor because it takes a lot more than having the initials M.D. after your name to actually be one.  No one deserves such sub par care when they’re sick and in pain and the worst thing is that I could be writing some semblance of this post about the majority of the doctors that I have and the hundred I saw before them.

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MY LITTLE ACCIDENT

Accident

This was my forehead after I spent 8 hours applying a compress to stop the bleeding.  I probably needed stitches, but I couldn’t afford to go to the ER so in the middle of the night, I super glued my forehead together, which is actually better than sutures for my fragile skin.  I heard this method of closing wounds was used during the Vietnam War, so good enough for me.  In fact, I can source that fact right here, although having training as a medic would’ve been helpful at the time.

I wasn’t in a car accident; I was trying to grab my cat’s litter box from under the built-in desk, but due to being visually impaired from keratoconus, I slammed my head into the sharp, laminate edge of the counter top and because my defective collagen makes my skin so weak from Classical Ehlers-Danlos syndrome (CEDS), I split my forehead in half, or however long that gash is.

Now, in the grand scheme of all my problems: living with severe, chronic pain, having a body falling to pieces, dealing with major visual distortion which makes it hard to tell how far away a counter top is, surviving off 6 foods, being semi-homeless and on and on, hitting my head shouldn’t be a big deal, right?  Well, it really is and here are a few reasons why.

People with CEDS, a genetic connective tissue disorder, have defective collagen V, which causes poor wound healing and wide, atrophic scars—also known as cigarette-paper scars—along with 1 million other things.  I think I hit my head 2 weeks ago or so and the scar is looking very purple and keloid-like right now, but if history proves correct, it will keep widening and then the center will turn into thin, papyrus-looking skin—right across the middle of my freaking forehead!

But, that is just part of my little accident.  I apparently hit my head so hard that my brain literally shook.  Yes, it took me a few days to realize I’d sustained a concussion from this thing once I could get back online and Google my symptoms.

At first, my forehead just felt like someone was driving a stake through it, so I went through every bag of frozen veggies in the freezer icing my head.  Then, I got v-e-r-y sleepy and just had to lay down and I fell asleep without Ambien CR for the first time in over 8 years and I kept sleeping like that: in 3 to 4 hour bursts throughout the day and night.

Now, while all this was going on I started to get really nauseous, which isn’t normal unless my pain gets above a level 9 on the Richter scale.  And even worse, the lights were driving me absolutely insane and I got double vision, so instead of seeing 6 of everything like normal, I saw 12. Therefore, I just stayed in this dark, distorted cave in my sleepy stupor with frozen veggies on my head and prayed I wouldn’t throw up, which I luckily didn’t.

Now, how many days was I in the cave?  I have no damn idea. I don’t remember much of anything, other than trying to text someone and not being able to do that at all, not that I can text well in general due to my vision.  I know I posted a draft that I had saved on my blog a few days out.  I also remember contacting my mother, but I’m not sure if that was by phone or e-mail.  I know I was trying to get help and she lives one state over from me and can drive here, but she was too busy getting some cosmetic procedure done to her face to be bothered, so as usual, I was in the cave all by my lonesome, aside from my beloved cat, who really could care less about her litter box being a little stinky I suspect.

To add insult to injury, literally, I got kicked out of physical therapy until I received clearance from a doctor stating it was safe for me to come back, but I had fired my rude and inept GP, so I missed a couple weeks of my much needed PT, not that I should have been doing any exercise, but my brain didn’t seem to comprehend that.  In fact, my brain wasn’t comprehending much of anything.  For example, I did a bunch of labs in December for a disorder associated with Ehlers-Danlos and I had to twist that doctor’s arm to even get him to order the tests for over a year.  However, I couldn’t recall what the tests were for all of a sudden.  How odd: this disorder I had researched in the medical journals for so long had just flown right out of my head.  It definitely started with an M, but what was it called?  Hmmm…

Since the concussion, I don’t sleep with Ambien CR anymore, not that I’m sleeping well and unfortunately, my circadian rhythm disorder is definitely here to stay.  I’ve also made some changes in my life, like deciding to not tolerate rude and discourteous people for 1 second anymore (yes, if you are one of them, goodbye) and getting rid of some useless online activities that were anything but helpful.  Who knew about the benefits of cracking your head open?

If you ever hit your head and anything in this article on concussion rings a bell once your brain is working a bit better, I hope you have the following if you are in the States:

Money 
Good insurance
A doctor
Transportation                                                   

My mother suggested that due to my disabilities, such a nasty word coming from her mouth, I should wear the following on my head.  Come join me so everyone will know how special the disabled are and you might just get a trophy, too!

For the 1st time, my home team won the Super Bowl! Go Hawks!

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A FALSE ALARM, BUT KEEP YOUR SEATBELTS SECURELY FASTENED

Image Source: http://www.thesun.co.uk

I don’t like to fly.  I get extreme anxiety and have had full-blown panic attacks at every elevation imaginable, even after taking a fear of flying course nearly 20 years ago.  So, when I went to see my optometrist a couple of weeks ago to find out why my vision was worse and more ghosted in my right eye, I had that fear-of-flying feeling the whole time: being completely out of control and waiting to crash and burn at any second. After all, if I’m not holding that plane up, then who is?

As usual, an assistant had me read the Snellen chart—a useless tool for assessing the visual acuity (VA) in anyone with keratoconus (KC).  I just wanted to know how much my VA had changed in my right eye.  Well, this woman didn’t want to tell me, but I do know that the VA in my left eye was unchanged because I turned around and read it on the wall when she walked out and saw 20/60 written next to the letters. I couldn’t see that while staring straight at it in the mirror. Again, the Snellen chart is worthless and there is no way I really have 20/60 vision in my formerly better eye pre-op and then formerly worse eye post-op.  I didn’t get near that line with my right eye, so I believe I lost around 4 lines of vision over a month in that eye.  I’m sure that sounds impossible, but I was progressing so quickly pre-op that it really is possible.  I had that awful feeling of sitting in a plane knowing we would be taking off very shortly.

Next, I had to have the dreaded corneal topography done, as that is the gold standard for diagnosing and tracking KC.  I looked into the machine and stared at a tiny hot air balloon that I saw multiples of with each eye while a photo was taken. My fate was sealed, just like when they close the door to the plane.

We headed back to the exam room and I waited for my doctor with my heart pounding.  What did the topography show? Was I progressing 13 months after collagen cross-linking (CXL)? Why doesn’t anyone research people who get keratoconus from Ehlers-Danlos syndrome?

My doctor came in and he knew why I was there as we had spoken on the phone, which is a rarity in the States.  He looked over a few things on the computer and then my topographies came up, but I couldn’t see them in detail.  I think he spent 20 seconds comparing them to my previous ones from this June, but it felt like 20 minutes and I could hear those huge, jet engines starting up: the clear sign of impending doom.

Finally, he spoke and said that there was no difference between my topographies.  I actually cried right there in the exam room out of relief, which is how I feel when a plane firmly touches down on the runway.  I was horribly embarrassed and it only lasted 5 seconds and I wiped my eyes and apologized. But, what was wrong with my eye?

My doctor checked my eyes and looked to see if the slight scar I have in the right eye was worse, but it wasn’t.  Another thing that can initially cause ghosted vision is a cataract, which may be a side-effect of CXL due to the UV light, but my lens was clear.  He thought everything else looked normal in both eyes, aside from the scar partially blocking my vision in my left eye from CXL.

He decided to do a refraction and apparently my prescription has totally changed since I saw him a few months ago, but not for the worse.  I’m still confused on what that means exactly, but that is precisely what he told me and I wish I had gotten copies of my refractions, but you have to pay for records.  I can still drive with my scleral contact lenses, but either due to the sun or the lights at night it’s really hard.  As I rarely wear them due to the pain, I noticed that my vision had gotten worse without my lenses in, which is the bulk of the time.

I told my doctor again my concerns about CXL lasting due to Ehlers-Danlos.  He told me that he had no idea about that or what was wrong and that I had surgery that totally effed up my eyes—that would be both eyes due to the prescription change and yes, I cleaned up his language.  What did he mean by “effed up my eyes?”  I believe he was referring to the fact that my corneas are changed at the molecular level, not truly effed up, but maybe a casual relationship with a doctor isn’t as great as I thought.  He said that maybe my left eye had gotten better, but I’d been doing the cover-one-eye-and-then-the-other test for weeks and just looked at him like he was yet another inept doctor in this city.  The free fall was starting.

I can’t even read on my hugely blown-up laptop with my right eye, so that is the one that is worse.  That was my better eye 6 weeks ago and I was also right eye dominant due to that, so my visual acuity was based on that eye which used to read the 20/40 line with great difficulty, but previously read 20/100 pre-CXL.  I am now left eye dominant as my brain is trying to compensate for the vision loss in my right eye.  Do I need to have fancy initials after my name to figure that out?

Again, the Snellen-based VA means nothing with KC as you can get a driver’s license with 20/40 vision and I flunked the test almost 2 years ago when the KC was starting in my left eye and I could read 20/30 from that eye without correction. I got a license as I could get enough correction with glasses in my better eye to read it back then, but I have a huge restriction stating that I am blind in my right eye, which was at 20/60 at that time: my current VA.  Legally blind is defined as 20/200 in the States; I rest my case on the idiotic Snellen chart.

So, my optometrist doesn’t know what is wrong or if the CXL will continue to halt the progression of keratoconus.  Oh, he called it keratoconus three times so I’m going back to that. A Google search for “secondary corneal ectasia from Ehlers-Danlos syndrome” yields zero results, unless my posts come up, so keratoconus it is.  Many disorders are associated with KC and until the medical world catches up and comes up with new names, I’m staying in the KC club. My topographies give a diagnosis of keratoconus and the big specialist who did my CXL had no clue I had Ehlers-Danlos syndrome, although I did mention the fact that I thought I might have it and he told me to see a geneticist.  If the only real difference is the typical age of onset and the lack of disease burn-out, I’m sticking with keratoconus and not changing my tag line.

What is wrong with my eyes again?  My vision is a bit better today so I wrote this post.  Who effing knows?  The plane crashed and there are no survivors.  I’ll post an update when they recover the black boxes.

Where all the answers are.

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IMPENDING DOOM: MY VISION IS WORSE & ALL ABOUT MY PSEUDO-KERATOCONUS

dominos-1

Photo Source: 40ans.ehess.fr

I was in deep thought about my collagen cross-linking (CXL) surgery awhile ago.  I’ve read every journal article online about epi-off CXL, poured over the interviews with the top surgeons and their speculations on whether CXL will last in the long-term for keratoconus (KC), and thought about everything my top-notch surgeon told me at my pre-op appointment about the efficacy of CXL.

Since epi-off CXL was first performed in Dresden, Germany in 1998, the studies have shown there is no progression of KC and in the small percentage of people who don’t benefit from CXL from the get-go, it is effective when the surgery is repeated. This abstract from 2006, while not long-term, demonstrates the halt in progression.  So, why was I worried?

Well, it wasn’t until I found my great optometrist this summer that I learned that although I look like I have KC per my scans and tests, my vision is that of someone with keratoconus, and despite all of the medical literature stating that keratoconus, a primary corneal ectasia, or thinning, can be caused by Ehlers-Danlos syndrome (EDS)—I really have secondary corneal ectasia caused by EDS.  Should I change the tagline on my blog? As my optometrist stated, “It’s the same thing more or less.”

Per the “more or less,” here are the differences:

The true cause of KC is not known but there is a connection, at least in part, to faulty collagen in the cornea, but KCers don’t have a systemic disease that causes faulty collagen throughout the body like EDS does.  A dry abstract discussing collagen fibrils in KC.

KC tends to show up in one’s teens or early 20s and runs a course of progression before burning out—usually within 20 years.  With my secondary corneal ectasia, which apparently has never been researched, there is obviously no burn-out cycle as my body will continue to produce faulty collagen V for the rest of my life due to EDS type II.  Therein lies the big problem, or so I think.

With CXL, the weak collagen fibrils in the cornea are tightened up by creating cross-links, much like adding rungs to a ladder. That sounds like a win-win for true KCers and for me.  By creating all the cross-links, the cornea is stiffened and aged in essence (see abstract), which burns out the KC as cross-linking and stiffening are naturally occurring processes in normal, aged corneas.  That’s good for KCers and in my mind, has no affect on my ectasia with no burn-out cycle and continued production of faulty collagen.

So, why all the worry again?  I began to think about cell turnover.  Most of the cells in our body turnover in time. What would happen to those nice, cross-linked collagen fibrils when the cells in my cornea, or within the stroma more precisely, turned over?  With true KCers, I don’t think it matters as the disease has burned out, but for me, I believe that all the good collagen fibrils will be replaced by the old and it will only be a matter of time before the pseudo-keratoconus—what I’m calling this disease—shows up again.

Then, what are the options?  I see none.  When KC is severe enough, corneal transplants are the only option, but they reject sooner or later and the healing process is very long.  I also have abnormal connective tissue due to EDS, which would make finding a match rather difficult, but that’s an assumption. Lastly, transplanted corneas in KCers who are still progressing can occasionally develop keratoconus again since the disease process is still active.  I believe that would be a huge cause for concern in my case due to my faulty collagen production, not to mention my healing and scarring issues from EDS which would make transplants so problematic.  A single case report highlighting the recurrence of keratoconus post-transplant.

Now, I was just thinking about all of this and then I noticed my vision got worse all of a sudden, rather like when I first noticed the psuedo-keratoconus.  I thought it was from the sclerals I have to wear a few times a week to drive and that it would go away in a couple of days, per usual.  That was about a month ago, so it’s not from wearing the sclerals.  I don’t have any symptoms aside from more ghosting in my right eye, which was my better eye post-CXL.  Now, my left eye with the scar is the better eye and my brain has made that the dominant eye. As the vision in my right eye is worse than the left, it means I’ve lost more than 2 lines of vision in a month or so, which is how this whole debacle started by losing a line every 4-6 weeks on the Snellen chart.

I did have a lot of testing at my 1-year follow-up in late September for the FDA clinical trial, but due to my corneal specialist being the worst ophthalmologist known to humankind, he came into the room for 30 seconds and didn’t say a thing.  The reports, which are required by the FDA, never seem to make it to my surgeon in L.A., so who knows if anyone even reviewed them.  No one told me anything was awry and I had no reason to suspect anything at the time.  I do remember that my VA was the same, not that they’re very accurate at that practice or that it truly represents keratoconic vision.

I spoke with my optometrist about my eye and my concerns and have an appointment next week for an eye exam and corneal topography to see if there is progression.  The CXL did work as I got 6 lines of vision back, which is very rare as most get 1 to 2 lines back or none at all.  I do know that the cell turnover of the collagen in the stroma is several years and I’m shy of that at about 1 year post-op, but I have no idea what else it could be unless the very mild scar I have in my right eye that’s been stable for 8 months suddenly went wild, in which case I still won’t get my former vision back, and that’s all I want right now, even though most people would be crying in the corner if they had to see the world through my eyes.

I wish I could just get a break and I had to pay so much money for CXL that I didn’t have—I’m sure more than most Americans have in their savings account.  When I had the talk with my optometrist about my concerns with CXL, EDS, and the pseudo-keratoconus, he agreed and said, “It’s a whole other monster.”  Why didn’t he say that the first time? Maybe they could state that in the medical literature: that a condition that looks, but doesn’t exactly act, like keratoconus can be caused by EDS and it’s a whole other monster, rather than stating that keratoconus can be caused by EDS, period.  Perhaps the inept corneal specialist who has M.D. after his name could have told me that after I was diagnosed with Ehlers-Danlos syndrome.

I also found out from my optometrist that if I had had an EDS diagnosis in September of 2012 when I had CXL, instead of in November, that I would have been excluded from the clinical trials.  That’s doing wonders to quell my nerves while I sit and twiddle my thumbs and ponder writing a letter to the German ophthalmologist who developed CXL to get his take on its efficacy on my pseudo-keratoconus.

Theo Seiler, M.D., Ph.D., the creator of CXL who also looks exactly like my late grandfather.  Hmmm…

Photo Source: http://www.meaco.org
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ON LOSING A HEALTHCARE PROVIDER

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Image Source: http://www.clipartsheep.com

Years ago, I needed a new doctor for my now misdiagnosis and I found a specialist for that syndrome, which was odd for this city.  He was a PM&R physician and did a very thorough exam, but somehow was yet another provider who failed to realize that I actually had a genetic disorder: Ehlers-Danlos syndrome, classical type (EDS).

I remember that at my first consultation and after my exam, he told me straight out that he had no magic pill for me, but that what he could offer was compassionate care.  It was what I—a patient with a chronic, painful, and debilitating illness—needed to hear, even if he thought I had something else.

No one knows what happened, but a year later, all of his patients received a letter stating that the doctor would no longer be in practice there.  No other information would be given and I was transferred to an associate, but that was the first time I had a complete meltdown over losing a healthcare provider.

Prior to getting sick, I just had the doctors most have and maybe saw them 6 times a year for annual exams, refills of a few medications I took, and then the endless upper respiratory infections I’d get from every cold, which only turned into massive bronchitis because I was too busy working to go to the doctor.  But, things change when you get a chronic illness. I now have more than 20 medications, I have over 10 specialists who largely do nothing for me, and I spend 3 or 4 days a week in some waiting room, which literally makes me sicker.  When healthcare providers become your entire life, and not by choice, losing one is utterly devastating as the process of finding another and dealing with records and forms I can’t see and every other hurdle you have to jump over in the U.S. is an utter nightmare.

I have been to 4 doctors for my EDS, formerly called something else, since I lost the one who disappeared and then the entire practice closed.  They were all inept or rude or left me waiting hour upon hour or all of the above. One, who I absolutely believe was an anti-Semite based on his country of origin and how friendly he was with everyone but me, actually said something so rude to me that a tear fell down my cheek in the exam room, and I wish with all my might that he had never seen that.

Right before my corneal surgery and my diagnosis of EDS last year, I found another PM&R who was willing to see me, as most weren’t interested in the misdiagnosis.  I had come from a rundown and horribly unprofessional practice where my doctor literally walked out the front door while I was waiting for him. I really didn’t know him as he spent maybe 2 minutes with me signing prescriptions and nothing else. The staff were rude and complacent and I was months behind in scripts for physical therapy and they could care less.

I knew this new doctor I had was smart and he actually questioned my misdiagnosis, but he seemed rather uncaring and disinterested by my second appointment like the others. I couldn’t see him well, but noted that he preferred to stand on the opposite side of the room and wouldn’t make eye contact with me as I can see color, like the sclera of the eyes.  If he has a social phobia, then he should have gone into research and not private practice.  It was a place to get my refills and scripts for physical therapy, which is how I view most providers in my life: robotic people who do the basics of their job while I get an extended stay at the Hanoi Hilton courtesy of my broken-down body.

One day, I was told I’d be seeing the nurse as the doctor was busy.  Well, that figured.  Unlike the doctor, his nurse was actually a person and she was friendly and talked and asked questions and was concerned.  So, I became her patient and didn’t see the doctor anymore.  I always took the last appointment since I can’t wake up and am really slow to get ready due to my pain and stiffness, and she would spend over 30 minutes with me and it felt like I finally wasn’t just a number or a nuisance anymore.

We tried a few other medications without much success as usual, she attempted to appeal the insurance issue with my physical therapy being cut-off, she was researching EDS as fast as I was, and since she was from Ohio, she always said she wished she could just stick me on a plane and send me to the Cleveland Clinic because she was so frustrated, and not by me, but for me.  I always left my appointment feeling better, even if there was no magic pill.  I had compassionate care again.

I’m on a 3-month schedule with the practice now and I had an appointment earlier this week.  There was a huge plumbing issue with the central line that affected my bathroom here at the motel, so I was running a hair late, but had enough time to get to my appointment if I drove fast and everyone does.  I get that I can’t see well, but I can drive better than the 20/20 crowd here and had my sclerals in, with enough lubricating ointment to make them somewhat tolerable, but it was like someone smeared Vaseline over the windshield.  The clinic is in a nearby town, so it’s a bit of a drive, but I can do it if I really try since it’s not in the direction of the sun.  What I didn’t expect was an accident on the arterial that leads to the freeway that created a backup for miles.

I called the clinic to let them know I’d be late due to the accident, but not more than 10 minutes, as everyone gives you a 10-minute grace period from my experience.  I was told that the doctor had to leave to meet with his lawyers and so I would have to reschedule.  I didn’t understand and had about gone through the wringer waking up earlier and getting ready and just driving.  I explained that I didn’t see the doctor—that I saw his nurse and I’d never been late.

It was then that the rude receptionist told me she was no longer at the practice.  It hit me like a ton of bricks, and she made up a lie as to why she was gone.  I knew I needed refills soon, so I was pleading with her, and she told me that I was talking in circles.  I just kept trying to be the squeaky wheel, but could hear my voice cracking and then hung up.  I was by a shopping center, so I pulled into the parking lot and just lost it.

How would I find my nurse?  Who could I talk to that would understand how complex chronic illness is?  Who would offer me compassionate care?  Certainly not anyone I currently see and not the doctor there.  My counselor, who is supposed to help me, has been putting me through the Inquisition for weeks and I rip the cuticle off my thumb in every session from the anxiety he’s causing me.  I felt abandoned, like I have felt my entire life.  The feelings about the former doctor disappearing came flooding back, as did everyone else in my life who had betrayed me: my family, my traitorous, best friends, the guys I had been with, the entire world.

I’m tired of this disease and the subsequent vision loss.  It’s been 12 years and there’s no hope.  The cure is not coming in my lifetime and the degeneration will continue—that much I know.  A million thoughts were flooding my mind and I went to a very dark place while sitting in my car, which is not uncommon—I’ve had enough and there is no meaning in my endless suffering.  I couldn’t breathe and was on the verge of having one of my major panic attacks.  I called the only person I know here—the one who gives me rides here and there—and asked that he take my cat, Moush Moush, but he wouldn’t.  I was begging him to take her to no avail and in the end, it was Moush Moush who saved me again, just like I am trying to do for her.  I promised her that I’d never leave her, and I won’t.

Very few people will understand this post.  A healthcare provider is someone most see for an infection or an injury or an acute condition that will either get better or kill you. There’s a gray area where those with chronic illnesses that steal your entire life live, and that’s my land.  I have staked a claim and it’s a lonely place that hurts my body and my mind. My nurse brought more light in, but now she is gone, like so many before her.

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GOOD YONTIF, MY FATHER

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Photo: My father and me on Mt. Rainier in 1978.

It’s Yom Kippur today, or was: the Day of Atonement and the holiest of days in Judaism.  I’ve been very sick the past two months due to the never ending monsoon where I live and its affect on my body due to Ehlers-Danlos syndrome, on top of other issues in my life.  It’s been very difficult, painful, and depressing, so my mind has wandered to my father as I’ve sat for months in utter isolation, even though we are estranged yet again and have had no contact in months. Last night, before the sun set on Erev Yom Kippur, I sent him an e-mail.

This is a time for reflection: to think of where one has erred and where one can improve.  I wasn’t expecting a reply to my e-mail; I just wanted to share my thoughts at this time.  I was raised Reform and am not overly observant, but there are traditions I choose to follow.

I wrote in the subject line of the e-mail the traditional greeting on Yom Kippur: Good Yontif, my father—anglicized Yiddish for Good Holiday. The rest was highly personalized on purpose, as I know how to get my father to understand my point, if nothing else.  So, I wrote:

My thoughts this Erev Yom Kippur:

My father taught me to be strong and tough, so I am.

My father taught me to be outspoken, so I am.

My father taught me to be responsible, so I am.

My father taught me to be the squeaky wheel, so I am.

My father taught me to be funny, so I am.

My father taught me to be a Jew, so I am.

My father taught me to be affectionate, so I am.

My father taught me to love doo-wap and Motown, so I do.

My father taught me to not let others disrespect me, so I don’t.

My father taught me about tzedakah, so I give back.

My father taught me business sense, so I use it.

My father taught me to speak up against injustice, so I do.

 

My father taught me to be stubborn, so I am.

My father taught me to talk down to people, so I do.

My father taught me that asking for help is a weakness, so I don’t.

My father taught me to hold grudges, so I do.

My father taught me to not rely on people, so I don’t.

My father taught me to cut people out of my life, and so I do.

 

I learned all of these traits—both good and bad—from you.  I atone the latter: these horrid traits that go back generations. My mother taught me very little that I can recall going back to 1977 and I have few memories of her and so many of you, a man who is long gone now and who has no memories of my formative years.  I have many traits that you don’t have and vice versa, but I am my father’s daughter, and it wasn’t from mere observation.  You wanted me to be like the man you were years ago before you lost interest, and so that is what I became and still am, despite becoming debilitated and disabled so early in life.  I don’t know who you are now, but I knew who you were back then: a father who, despite having many flaws, loved me and who I could always rely on, and who I loved in return.

May you be inscribed in the Book of Life this New Year.

Your daughter

My father replied to my e-mail today and let me preface this by saying that list was abridged and purposely focused on the good traits he taught me.  He apologized for some things, which was unheard of, and made excuses for others, but then the e-mail turned sour and was focused on how my family had reached out to me and that this was the time to not be so strong and tough and to let others in, which is just absurd and completely false.  I was also told I needed to learn how to apologize. To whom do I need to apologize—my grandmother who told me I was “the broken tile in our family’s mosaic?”

My father stopped talking to me, and by that I mean by e-mail, because I hinted at the fact that my physical therapy benefit was cutting out and as my income is mainly from SSDI—federal disability that pays for my prescriptions and little else—I would need help paying cash for my much needed physical therapy.  My father was a successful businessman who lives a very comfortable life, so this was not asking for much.

I won’t even touch on the lack of emotional support from the bulk of my family in general or the verbal assaults thrown my way simply for falling ill due to a genetic disorder.  As the day progressed, the back-and-forth e-mails got downright offensive and I was told to not contact him again—all for speaking the truth.

I tried and failed and the impasse continues, and on a day when things should be resolved, as is our tradition.  I’m left with a heavy heart and am as frustrated with this relationship as I am with this disease, both of which are utterly incurable.

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GETTING AN ACT IN THE FREAK SHOW

Image Source: http://www.medical-dictionary.thefreedictionary.com

Why does Ehlers-Danlos syndrome (EDS) have to be a freak show disease?  My skin doesn’t stretch enough to be the India Rubber Woman and I’m not hypermobile enough to be the Human Pretzel, but I look like a hybrid of marbled, blue cheese and the Purple People Eater from the venous pattern and purpura and petechiae covering my body from nearly head to toe.  The icing on the cake is the beyond frustrating keratoconus, which turned my corneas into little cones. Alas, the latest problems that have been discovered could surely guarantee me an act in the freak show.

Possible employment? http://www.plasticjoe.com

 

I’ve been working with my great, new optometrist over the past couple of months to try and get scleral contact lenses, which can correct keratoconic vision to a point by creating artificial corneas in essence.  As I’m post-CXL surgery, the progression of my keratoconus (KC) has been halted and my vision is stable, not that it means I can see well.  The trial pair of scleral lenses had about 3 pluses and a lot of minuses, which I typed up in a list with bullet points for my doctor.  He thought that was very amusing, but in a good way, and it actually helped to narrow down why I was having so many issues with the sclerals, aside from my dry eyes which will prevent me from wearing them daily it seems.

My doctor got down to business getting the prescription just right as it was a hair off and he can get my far vision a little clearer, although that is where things are very good for me with sclerals.  I mentioned in my bullet points that I still can’t see well close-up and that while the T.V. image is crisp, it often has a double image, as do all the illuminated signs at night which makes it hard to drive still and is different from the multiple, faded images I see with keratoconus.  My doctor thought that the better prescription would help, as sclerals work on both nearsightedness and farsightedness.  I wasn’t that positive, though.

I had also mentioned in my list that the intermittent strabismus, or intermittent exotropia to be exact, that I developed when my brain shut off vision to my worse eye pre-CXL wasn’t gone as I suspected.  It reared its ugly head again when I started inserting the new sclerals.  As the contacts are akin to plastic bowls filled with saline solution, I have to use a rubber holder of sorts to put them into my eyes while looking into a mirror laid flat on the vanity with my head parallel to the floor.  It’s really not that easy as the lenses are so huge and I have to hold my eyelids open very wide.

The right contact goes in well, but when I go to put in the left one, I can literally see my right eye turning out—see Figure B in the 1st image—and then my left eye that I’m trying to work with does the same!  In other words, both of my eyes are turned out and it’s a little freaky to say the least.  Now, I’m not making fun of anyone but myself here—I dated someone in high school who had exotropia and it wasn’t the intermittent kind.  Regardless, a scleral lens has to fit over the cornea, so if my left eye has wandered to the far left, out falls the lens and saline solution and then I have to repeat the process 10 more times.  Suffice to say, I’ve been a little late to appointments the last month.

My setup for scleral contacts lenses

 

It’s a very good thing I made a bullet point about that strabismus issue because my doctor did a couple of tests. He had me focus on a letter on the wall and then flipped something over my eyes in a rapid motion, so that every second I was using either the right or left eye to read.

“You do have strabisumus!” he told me, as he’s always amazed to have a patient who speaks doctor talk. Apparently, the test made my eye turn out as I started to lose vision in my right eye and couldn’t see the letter, only a blank blur.  Yet, I can somehow make both eyes turn out when putting in the sclerals for some odd reason.  That sounds like a freak show act if I ever heard one.

“Great,” I said, as I thought this was just some transient thing from having low vision for 2 years and thus some atrophying of my extraocular muscles, which control the movement and alignment of the eyes.

“Am I going to need surgery?” I asked my doctor. “You know about my scarring issues with EDS.”

He told me, “Not now at least.”

Extraocular muscles–what are lax from EDS and where I may need surgery. http://www.medicalgeek.com

 

Then, he was on a mission and started rifling through my purse, which is fine as we spend most of the time joking like we’re old friends.  I asked him what he was doing and he told me that he was looking for my phone.  I grabbed my cell and he told me to type a text.  So, I started to and he noticed how close I hold the phone to my face and he pulled my arms down.  Well, that’s not fair.  I knew he was trying to figure out my difficulty with seeing close-up and I mentioned that the screens on cell phones and my blown-up laptop aren’t horrible with my slcerals in, but print text was awful.  That was in a bullet point, but I’ll cut him some slack since he’s my favorite doctor and knows all about EDS.

He gave me the paper I had typed up in 12 font at 200% in Word to read, which now looked very small and wonky.  With the sclerals, the letters tend to move around a bit and make me feel sick; that’s the best way I can describe it, unless it’s very small text and then my head starts to hurt.  He kept putting different lenses in front of my eyes and asking if it was better, but I kept saying that I didn’t notice a difference. I figured this was the best I would ever be able to see if none of the lenses over my sclerals helped.  However, I must admit I like the rows of little lenses lined up in long drawers—it’s all so old-world and I got to wear the opera glasses again for a minute.  You sure wouldn’t get any of this from the optometrist in the mall.

 

Then, my doctor had me look up and read the paper with the bullet points directly in front of me.  He must have been watching my eyes because he had a rather deflated look on his face when he stopped me.

“You have convergence insufficiency,” he said in a rather sad voice.

“Noooo!” I whined. “No more diagnoses.  What is that?”

He grabbed something and told me to watch his eyes while he brought it closer to his face.

“Do you see my eyes moving?” he asked me.

“Oh,” I replied. “Your eyes are moving inwards as it gets closer to your face.”

“Right,” he told me, “But your eyes don’t do that and they aren’t in perfect alignment in general, either—they both turn out a bit and all of that is causing double vision.”

“So, I have more vision problems than just the keratoconus?” I asked.

“Yes,” replied my doctor, “But convergence insufficiency is not that rare.”

More information on convergence insufficiency can be found here.

Per my eyes not being in perfect alignment, he tried to reassure me that it wasn’t noticeable, but I swear that one eye looked a little off when the KC first started.  I demanded to know if I looked like Jaws from James Bond, and my doctor couldn’t believe that he was the image I associated strabismus with.  Jaws actually had acromegaly and I don’t think he had strabismus.  I should have said Marty Feldman, who was only in my favorite movie ever growing up and does the Abby Normal line I use all the time.  Here’s the video clip. He had strabismus from Graves’ disease, but I see a resemblance, and it’s not just the eyes.  He may have been a second cousin. How long until the freak show comes to town?

Igor in Young Frankenstein–my new, male twin. http://www.screenjunkies.com

 

My doctor put lenses with prism in front of my eyes and I could see the text, but my head started to hurt.  Prism bends light so that people with convergence insufficiency can see close-up and I could get readers made with them, but my doctor said they can be hard to get used to.  Contacts and glasses are just too much right now.

I remembered that when I had the intermittent strabismus pre-CXL, I did some eye exercises I found online as I was so worried my wandering eye would prevent me from getting the surgery.  I asked if that might help to tighten up my muscles, just like I do in physical therapy for my loose joints.  My doctor handed me a plastic, Popsicle stick with a tiny E on it. He told me to focus on the E and pull it towards my face and then away, which I was able to do.  I will now be doing this for 5 minutes every day, as it sometimes can help, but now I can’t remember with which issue, so hopefully it’s both.

My doctor also confirmed this could all be secondary to Ehlers-Danlos, but that maybe I had had it since childhood like most people.  I never had problems with my near vision or any strabismus before keratoconus showed up, which is around the time my EDS started to affect more of my body, so I’m betting on EDS being behind all of this, or that and and KC combined.

I left with more health matters on my mind and then got a call from the assistant of yet another specialist I need to see as I was sitting in the parking lot of CVS.  How many body parts can fall apart from faulty collagen?  I went in to get my pricey prescription and got $5.00 in Extra Bucks—free money to spend at CVS for those not familiar with the chain pharmacy. I almost make money from all my prescriptions.

Then, I remembered I needed a Popsicle stick, but couldn’t find any, until I found the little freezer that had real, lime Popsicles, one of the last fruits I can still eat.  Eating the lime Popsicle that I got for free in the desert heat made the bad news a little sweeter, especially if an employment opportunity where I can just stand while people gawk at me may be in my future.

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AN ANEURYSM? ADD IT TO THE LIST.

Image Source: http://www.commons.wikimedia.org

I really thought I had an honorary M.D. after my name by now. I’ve only spent 12 years of my life researching a misdiagnosis, and now a correct one—digging deep into medical journals with such frequency that I only speak doctor talk even if discussing the weather.  Well, apparently I needed a good dose of reality.

After 5 months or so and two or three cancellations by the clinic, I finally had an echo of my heart and saw a real cardiologist.  Omg.  I know, I thought this was America—land of the world’s best healthcare system per the wealthy conservatives.  What was this, that maple leaf country to the north or something?  I had an echo twice in the past 10 years and was told I had trace mitral valve prolapse and mild tricuspid valve prolapse with regurgitation and a slightly enlarged heart chamber as a result.  Well, apparently the cardiologists who interpreted my former echos needed a good dose of reality by way of more CEU credits.

Now, these are both consistent diagnoses with Ehlers-Danlos syndrome (EDS), not that I can find that information anywhere at the moment.  For a broader description of the typical cardiovascular issues that can occur with EDS, click on this link and scroll down to Cardiovascular.  Please keep in mind that Type IV (VEDS), which has a shortened life span usually due to cardiovascular complications, is included in this description.

Regarding cardiovascular issues, I know what a stroke is.  I couldn’t claim to have an honorary M.D. without that basic knowledge.  My grandfather who had EDS had one, but he was a chain smoker.  His mother had one, but she was a rather zaftig woman from photos I’ve seen.  I recently heard some random, first cousin once removed that I never knew of died of a stroke in her late 30s or so, but she’d been a former drug addict it seems.  Did any of these women have EDS?  I have no idea and I really don’t know my mother’s side of the family, with the exception of my late uncle and grandparents.  Then, we have aneurysm.  I always thought an aneurysm was when a blood vessel in your brain bursts due to a weak spot and then you go bye-bye.  I do recall that it is a type of stroke: hemorrhagic stroke.  Due to my very leaky blood vessels, I figured this would be my one-way ticket out of here.

So, what is an aneurysm really?  According to the NIH:

An aneurysm is an abnormal widening or ballooning of a portion of an artery due to weakness in the wall of the blood vessel.

Source: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002109/

It’s before the artery bursts, if it does.  Well, you learn something new everyday.  In fact, I learned I have an aneurysm in my heart.  It’s in the septum dividing my right atrium and left atrium, or the atria as the docs would say.  I apologize that the septum isn’t labeled in the colorful image above; I think you need to dissect the heart to see it.  The aneurysm is in connective tissue as it’s in a septum—what a surprise, but that means it’s not in an artery if it’s in a septum (see aneurysm definition).  Odd?  What about the deviated septum in my nose?  Now, I always thought that was just a nuisance, but my snotty cousin had to get a nose job on account of hers.  On to more pressing matters, I have a bulging balloon of who knows what size in some connective tissue dividing my atria.  More precisely, I have a congenital heart defect called an interatrial septal aneurysm. What does that mean?

I don’t really know because even though the older cardiologist was nice and knowledgeable and had seen three—make that four—EDS patients in his entire career, he never mentioned the heart defect.  Did it slip his mind?  He even took me to the back to view my echo and was considerate enough to ask if I would be able to see it due to my keratoconus.  I do okay with computer screens if I’m viewing larger images and he pointed out this and that and used doctor talk—oh, goodie.

The cardiologist did tell me my heart was fine, and while I don’t have any prolapses, I do have leaky mitral and tricuspid valves with mild regurgitation, but no enlargement of my heart chambers.  He suggested we do an echo annually to monitor things and due to my leaky blood vessels, I need to stay away from aspirin and omegas and everything I already know turns me into more of a black and blue (and purple) mess.  That sums up the consultation, which was very pleasant aside from the long wait.  In the end, I happened to see mention of the heart defect in the report when I got home as the font was large enough for me to read.

Of course, I hit up the medical journals as soon as possible to figure out what this aneurysm was.  Would my heart burst open at any moment?  I did find a connection with EDS—how could I not?  Here’s what I found, or rather didn’t find, as every site refers to atrial septal aneurysm, so what is the “inter” that is before “atrial” in my report?  I did read a non-scholarly article that referred to this septum as interatrial, so are they synonyms?  Where’s a medical thesaurus when you need one?

An atrial septal aneurysm (ASA) is a rare but well recognized and localized saccular deformity of the atrial septum that bulges into the right or left atrium with uncertain clinical significance. Although these abnormalities are considered clinically benign entities, they have been independently associated with ischemic stroke. 

Source: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3327030/

Ischemic stroke?  I went right past the benign part.  Wouldn’t you just get a hole in your heart if that thing ruptured?  Well, that’s what medical school and those residencies are for.  I do believe an ischemic stoke is what my grandfather had, not that it killed him.  He just had a miserable downward slide until he died from pneumonia after his electric scooter tipped over, shattering his hip and leg due to early-onset osteoporosis—the latter presumably due to EDS.  So, what will it be?  An ischemic stroke or a ruptured aneurysm in my brain due to my weak and leaky blood vessels as presumed. Blood thickeners or blood thinners?  The doctor said taking vitamin K would probably be beneficial when I asked, but what about the ischemic stroke risk?  It’s the ol’ Catch-22.

Maybe I should just start smoking, but then I’d have to go out to the freaky parking lot with the wandering junkies and it’s nearly 120°F here and it doesn’t cool down at night.  I possibly used to smoke years ago and may have enjoyed it. Something menthol-y rings a bell.  Lately, cigarette smoke makes my nose stuffy and my eyes burn, not to mention all those wrinkles and other bad stuff, but my grandfather smoked like a chimney and lived to nearly 80.  Oh, what to do when you can’t be healthy even if you try?

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I SAW AGAIN, IF ONLY FOR AN ARIA

Photo Source: http://www.timeout.com

I saw the world with near perfect vision this week.  It was so surreal that my logical brain is still processing it: low vision to seemingly crystal-clear vision and back again.  I think I fell down the rabbit hole like Alice, or more accurately, I finally climbed back out.

It wasn’t really a Lewis Carroll tale.  I finally saw an optometrist who works with scleral contact lenses this week. These medically necessary contact lenses, which can work for those who are contact lens intolerant like I am, are my only hope to ever truly see again.  Scleral lenses can correct keratoconic vision and actually help severely dry eyes like mine, as the lenses are filled with saline solution which keeps the cornea and most of the sclera nice and moist.

Scleral lenses, like other specialty lenses, need to be a perfect fit.  The optometrist, who was very knowledgeable with a great personality to boot, had to determine the correct diameter lens for my eyes and then try various lenses out for fit.  The lenses seem very difficult to get in due to the fact that the saline solution can’t spill out, so he had to put them in with my face parallel to the floor, which is what I will do on my own in due time.  If I blinked while he held my eyelids wide open to get the huge lenses in, an air bubble formed and then the process needed to be repeated.  The lenses were inserted and removed with a little suction cup on a stick.

After much trial and error, I had two scleral lenses in my eyes and they gave me a smooth corneal surface, instead of a keratoconic one.  However, the doctor pointed out after looking at my corneal topographies, which at least haven’t changed in 2 months, that the center of my left cornea is now completely flat, which is not normal at all after cross-linking (CXL) and finally explains why my refractions for the FDA clinical trial show farsightedness in that eye now, not that I can see far or near for that matter.  I get a + rather than a – reading, to put it in layman’s terms.  No wonder the scan says the severity of keratoconus is 0%—the steep, cone-shaped cornea I had got bulldozed by CXL for some reason.  Well, so much for a safe surgery with hardly any complications.

The scleral lenses need a prescription in them like regular contacts, which I’m sure my good doctor was dreading by then, but regardless, I needed the scleral lenses in my eyes to get an accurate refraction.  I looked through the refractor and the doctor began flipping lenses over my right eye. Which lens was better?  I should get a lollipop after refractions at this point.  The odd thing was that I could see a difference in the lenses nearly every time for once. Suddenly, I had dark-black, crisp lines of letters in front of me.  The ghosting—or multiple images—was gone!  I read 20/20 with ease and I knew it was really 20/20.

He repeated the test with my left eye with good results, but not superb.  There was still some faint ghosting and the letters weren’t as crisp, but I read 20/30 and with ease again. I quickly rambled off the letters instead of taking 5 minutes and guessing like I normally do.  There’s a reason why the left eye—my better eye before CXL—can’t read 20/20 even with scleral lenses.  That eye has developed central corneal opacity, a type of scar, that’s in front of my pupil and is affecting my vision. The optometrist discovered it in a simple exam of my corneas, and I thought he was confusing it with the right eye, which developed opacity months ago that’s not affecting my vision. He wasn’t.  I have a serious scar in my left eye now.

This was just maddening because my idiotic corneal specialist wanted me to return to L.A. to see my CXL surgeon due to the mild opacity—more like a slight haze—in my right eye and has been monitoring this rare side-effect of CXL for months.  I literally saw him 2 weeks ago and he never noticed the scar in my left eye that has stolen more of my vision? The optometrist, who should be the M.D., luckily has a better corneal specialist for me to see who I’ve never heard of, so possibly he’s new to the area.  I wish I had been told that my vision wasn’t fluctuating at all per my scans, rather than the opposite, so I could have seen the optometrist sooner, who would have gotten me to the new corneal specialist in time to possibly treat the scar.  I trust this new doctor, and that’s a rare thing.

So, now my doctor had my prescription, which must be the strangest in the world.  He put it into what looked like opera glasses—ah, the post is making sense now.  He handed them to me and told me to look through them.  I noticed they were heavy as I held them with two hands and placed them in front of my eyes.  I could see!  I saw my doctor and there was so much detail in his face that I hadn’t seen before.  Even the color of his skin had more tones and shades.  I looked around the room as if I hadn’t been sitting in there for more than an hour.  He told me to walk around with them, so I eagerly did.

I think I was falling down the rabbit hole again.  I became extremely dizzy and felt like I had heavy moon boots on.  I noticed I was walking at some strange angle—almost on a backwards incline, yet I felt I was falling backwards so why couldn’t I lean forward?  I tried to keep walking, although I looked like a cat in kitten mittens.  I saw the waiting area that was down the hall and it seemed so close.  How could I see it? I walked towards it with my opera glasses and kitten mittens walk.  I turned the corner and saw all the glasses in display cases for sale.  I think I could have read the price tags if I cared to, but why bother when glasses don’t correct my vision.  How surreal it all was.  The dizziness was getting to me so I headed back to the room with my weird walk.

I told the doctor how I could see everything, but I felt so dizzy and couldn’t walk—as if my depth perception were off. Perhaps the prescription was too strong, although I wasn’t getting a headache and my eyes didn’t feel like they were crossing, which I’ve experienced with poorly prescribed glasses for mild myopia in years past.  He said that the opera glasses are very thick and aren’t exactly the same as the thin scleral lenses that will be right on my eyeballs.  He also reminded me that I will need to adjust to seeing again.  What an odd concept!  Then, I handed him the opera glasses and re-entered Low Vision Land.

I could see less than 2 years ago.  They say that you don’t know what you have until it’s gone, but one could also say that you don’t know what you lost until you get it back. During the aria, while I ungracefully walked around the optometry clinic with my opera glasses on, I was in la-la land—maybe I was seeing Madame Butterfly in there like I did years ago at the now demolished Seattle Opera House.  I forgot that I once saw the world this way, sans opera glasses.  In such a relatively short time, I’ve become resentfully accustomed to this horrible vision.  When it was briefly gone and then came back, I realized how abysmal my vision really is.  I truly felt blind.

That made me think this week, as I replayed my aria of sight in my mind while waiting for the scleral lenses to be made in Texas.  I should have them within a month and then I’ll know if they will truly work for me.  I’m used to my low vision again and only have snapshots in my head of that near perfect vision I experienced.  What if science invented something that could take away the unbearable, chronic pain that I’ve lived with for 12 years from Ehlers-Danlos?  What would that aria be like when my collagen acted properly and all the connective tissue in my body came back together and healed itself?  Would I just sit for hours since I can’t sit long due to the pain?  Would I feast on the hundreds of foods that my GI tract can no longer digest?  Would I regain 20 IQ points from not having a brain on pain?  Would I catch the next flight out of this horrid city and be an expat again—my ultimate dream?

I would forget what the pain is like during the aria, just like I did with my low vision.  Then, when the doctors took away whatever medical miracle made the pain go away and it all came rushing back into my body in a nanosecond, much like removing the opera glasses, I would scream the most bloodcurdling scream imaginable and it would be heard all the way in Japan—just as Madame Butterfly commits suicide with her father’s hara-kiri knife.

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