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.
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.
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?
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.
“What is your depression like? We’ve never discussed that,” asks my newer counselor at the not-free free clinic. They are post-graduate level interns, which hasn’t done wonders for me in the 2 years I’ve gone there. At least they know that depression accompanies chronic illness like a fever accompanies the flu.
“It’s like a dark cloud over my head and everything goes black—the light at the end of the tunnel went out years ago,” I reply. “Then,” I say, “I go to the past—before I got sick—when my life was good.”
I get a stare.
“What about the future?” my counselor asks.
“I never go to the future anymore—it’s too scary. The future means more illness, more pain, more medical bills, maybe living in my car next month,” I answer matter-of-factly.
My counselor says, “Most people are stuck in the past and the future.”
“Not me,” I reply back. “I just go to the past and I’m stuck with the present, but I never go to the future.”
“The past is just an illusion—it doesn’t exist,” states my counselor. It feels like I have been socked in the face.
“WHAT?” I reply, and give a thousand examples proving otherwise. My counselor’s explanations make no sense while my brain goes through those thousand memories like still photographs in my mind.
I’m supposed to appreciate small things in the present. I already gave the example of my cat weeks ago. My eyes wander in the small room and then to the bushy, palm tree blowing against the window that I can see fairly well.
“I like that palm tree,” I tell my counselor. “Those trees have the fronds that look like a fan. You need the fan-shaped fronds to build a palapa—those thatch roofs made of palm fronds in México. I had one built and they had to go high up in the jungle to get to those trees.”
“You found something simple that gives you joy,” responds my counselor.
“It makes me happy because it takes me back to a better place—a time in my past,” I reply back.
I leave the appointment with my head swirling. Did I have anything left? Where was my past if it was just an illusion?
I came back to the motel and Google spied on an ex from over 15 years ago who I’d been trying to find forever—and finally did. A little trip down memory lane. Then, I was even more depressed that the only Jewish guy I ever dated—even if he was so neurotic that I dumped him—hadn’t turned back into the Super Size American he was before I met him at 22 and that he was married and actually had his good hair still. His face had aged and I really didn’t recognize him very well, although I have a photo of him somewhere from the brief time we dated so long ago. Was the past an illusion, after all? He wasn’t the same. How different do I look? At least his wife was a hot mess—he used to tell me I looked like a supermodel, minus the height thing. Then, I had my usual meltdown. So much for memory lane.
So, today I Googled this concept that makes no sense in my mind: the past is just an illusion. An illusion is a rabbit in a magician’s hat. I figured this is what I get for going to the not-free free clinic. It’s actually physics it appears. I underestimated my counselor. Albert Einstein first described it in his theory of relativity. Stephen Hawking and all the big physicists follow the theory that all time is an illusion: the past, the present, and the future. Einstein said, “The distinction between the past, present, and future is only a stubbornly persistent illusion.” For those science-types who would like to learn more, you can read this boring article.
I don’t have any interest in the theory of relativity. It’s not going to give me my life back, which is all I really care about anymore, aside from my 3-legged cat. I hate physics and I never even studied it. Physics is that weird uncertainty principle on that huge blackboard in A Serious Man that Larry, the physics professor, dreams about—as seen in this clip. Throughout the movie, he always asks, “Why?” and never gets an answer. It’s a Jewish thing—this need to know why all the time. Maybe I would have done well in physics. I was only one of two students in my class to pass logic in college and with a 4.0. My friends watched as I wrote 20-something page solutions and were convinced I could crack codes for the government.
Maybe physics is logical, but it seems abstract—like why I can’t see anything due to keratoconus despite my good visual acuity per the eye chart.
“High order aberrations,” said my dry eye doctor when I decided to ask him 2 weeks ago due to my inept corneal specialist. “It’s physics. Let me give you an example.”
He mentioned waves and I was already lost. I even took oceanography. He drew side views of misshapen corneas like mine, which I’ve seen and I understand. I’m a visual learner. Aberrations are refractive problems, which leads to less-than-perfect vision. In my case, light isn’t refracted correctly due to my Rocky Mountain-shaped corneas, or one of them after cross-linking. This creates a high order aberration, or more specifically, a vertical coma. Due to keratoconus, I also have nearsightedness and farsightedness, which are low order aberrations. It’s all just physics.
“Why can I read 20/40 then?” I implored. Actually, I read 20/70 that day, but the DMV doesn’t need to know that, or that I drive in two lanes.
“Well,” said the good doctor, “The exam is under optimal conditions—it doesn’t represent how you see in real life.”
Finally, an answer to my why. My distorted vision isn’t an illusion—or is it? My brain remembers that there is only one moon, not the several moons that I now see in the night sky. Maybe my entire life has become an illusion.
This is what I know. I am who I am because of my past. The good memories gave me life and the bad memories made me a survivor in every sense of the word. Some events from years ago are so clear they play like a video in my mind. Certain smells can transport me in time or bring people back from the dead. The simplest things can trigger a memory from my past. They are as real to me as the present moment. No illusions and no smoke and mirrors. The past is so tangible to me—it is alive for as long as I remember it.
I remember when my father was a dad. I remember when I was a roller skating queen. I remember when I had acquaintances and best friends and boyfriends. I remember when I fell head over heels in love. I remember when I went out on the weekends—every weekend. I remember when I drank too much and I don’t care. I remember when I had fun and laughed all the time. I remember when I spent the whole afternoon at the mall. I remember when I sat for hours people-watching. I remember when I was in college. I remember when I had old apartments in Seattle that I loved to decorate. I remember when I was a good cook and could eat almost anything. I remember when I read books and sipped soy lattes on the weekends in coffee shops in rainy Seattle. I remember when those who are now gone were alive. I remember when I enjoyed the present, but lived for the future. I remember when I was an expat in México. I remember when I had a life.
And I remember that every Sunday in my early 20s, when my business was closed like nearly every other in the pueblo, my stray dog and I walked to the puesto de cocos—the coconut stand—and I would buy un coco and the vendor would hack the top off with a machete, give me a straw, and my dog and I would walk to the sandy beach on the warm Pacific Ocean and I’d drink the water from my coco and talk to my friends while my dog ran free and life was as picture-perfect as a postcard.
This was real. I lived it. I can’t go back, but I can re-live it in my mind and I am someone again—someone who was healthy and pain-free and not visually impaired and didn’t stay in a motel and lived life to the fullest and could be anything I dreamed of. That is where I go when the sky becomes black and the light at the end of the tunnel is still gone and there is nowhere else to go because the present is a nightmare I can’t wake up from and the future is a slow and painful death.
Physics answers the big mysteries of the universe and explains why planes don’t usually fall out of the sky. It doesn’t delve into the human experience and try to make sense out of the nonsensical. Isn’t that what counselors are for?
By the way, according to the theory of relativity, everything you just read is an illusion—it’s in the past now.
I dedicate this post to the memory of my beloved, paternal grandfather, whose yahrzeit—the anniversary of one’s death in the Hebrew calendar—falls today. I lit a candle, said Kaddish, and made a small donation at sunset, when everything begins. This is what we do. My grandfather was larger than life, the strongest man I ever knew, a traveler of the world, and as seen here, a self-enlisting Marine in WWII who fought in Okinawa and survived. He died the year I got sick, but the smell of rye bread brings him back in an instant.