I wear a large, gold pendant on a thick, gold chain around my neck. My hand frequently gravitates to the pendant to make sure the necklace is still there. When I am nervous, I rub the six points and the intricate grooves on the surface and feel the smoothness of the two Hebrew letters—Chet and Yud—that spell Chai. I never take it off, unless I need imaging done and the techs force me to unclasp it and then I hide the necklace in my purse, as it is really worth its weight in gold these days. The large pendant—a popular style from the late ’70s and designed for a man—has sat on my chest for almost 25 years, but the chain is not the original and was replaced less than a decade ago.
The pendant was my father’s. He bought it for himself most likely to follow the trend and display his pride of being a Jew when most men had large crosses, and wore it until the disco look went out of style in the early ’80s. The pendant is solid gold and heavy—far too heavy for the near 30 years of wear on the original, thinner, gold chain. I never noticed the links were wearing down in spots or that the chain was slowly weakening, like that old bridge on I-5 in my home state of Washington that just gave way and collapsed into the river below.
Around 7 years ago, I was living in a dumpy, rental condo across town. One afternoon, I was standing in the living room next to the laminate pass-through from the kitchen. I bent down for some reason and when I came up, one point of the pendant caught on the edge of the pass-through and the chain ripped off my neck. I saw it fly through the air in slow motion in a state of shock—as the necklace had so much significance to me. I picked it up off the floor and saw that there were numerous thin spots in the chain that I had never noticed, but it was that one spot—the weakest link I suppose—that had broken the chain in two. I knew at that moment that like the chain, the relationship with my father would never be mendable, and thus far, I have been right.
My father and I were estranged, as usually is the case, when the chain broke. We have semi-mended ways and then become estranged again at least a dozen times since that fateful day. He stopped talking to me—and by that I mean via infrequent e-mails—two weeks ago. I had responded to one of his chain e-mails, usually something Jewish: a little humor, a story of the Holocaust that he adds a memorable comment to, or some randoms facts that come his way.
I told him I was about to reach my insurance’s limit for physical therapy for the year and that I could not get an override, despite numerous attempts to do so. My Ehlers-Danlos and subsequent tendonosis is getting worse and I need physical therapy like a diabetic needs insulin. I have not been able to work for years and despise even hinting that I need money. He gives the same stock answer to any problem I mention, even though he would never really do anything. The e-mail came back with the familiar, “What can I do to help?” It sounds so wonderful and caring, like the father I knew as a child who I would search frantically for in our house after a day of being called derogatory slurs at school, but it is just smoke and mirrors now and I already knew the game we would play.
As he would never part with a dime to pay cash for my physical therapy, I gave a smart-ass reply and asked if he could grow a money tree outside of his home—the one that is half a block from Lake Washington with a 180° view of Seattle and the lake. The argument ensued. He claimed he was broke, his other stock answer, and I asked how a broke man has that home, and a custom Mercedes, and two country club memberships, and takes vacations every year. I would have mentioned shopping at Nordstrom’s and his young, gold-digger girlfriend, but that gets very messy. He replied with, “How easily you forget.”
That was a reference to my childhood: growing up as the daughter of a successful businessman who bought me rabbit fur coats and diamond earrings for holidays, living in the beautiful homes that he, a high-end contractor, built, and taking vacations to warm places with palm trees and swimming pools. The poor-little-rich-girl saga that he loves to put me in lately. I suspect the gold-digger, pulp-mill-town girlfriend is behind this as my father would never insinuate such things about me. I was as self-made as he was and he knows that.
How easily he forgot that he, my only real parent, emotionally abandoned me by middle school, made me start working when I was 14 so I would have a work ethic, that I never had anything in common with the few, spoiled, Jewish girls I knew growing up, and that if I wanted something as I got older—I bought it myself. Let us not forget that I rarely even lived at my home once I was a teenager due to the dysfunction swarming inside it.
I have had enough of this Jewish-American-Princess story he has invented over the past few years to avoid looking like the horrible father he has unfortunately turned out to be. His story is almost laughable considering I am on SSDI and live in a motel, but I suppose this is how he saves face while golfing with his old friends who have the princess daughters who aren’t even disabled. I ended the e-mail argument by replying, “I do not even know who you are anymore.” I really do not know this man who was so great at times in my early childhood—memories that have nothing to do with material possessions.
Before the price of gold went through the roof, I replaced the broken chain with the thickest twist chain I could find that would fit though the loop of the pendant. It is strong and sturdy and has only had one weak spot that I had a jeweler fix for next to nothing. The newer chain—of a lower karat and different style—is like the father I remember: the rock in my life, the bridge that would never crash down into the cold Skagit River north of Seattle. The old chain, with all the weak spots that eventually broke due to the heavy pendant, is my father now. He is a damaged version of his former self, unable or unwilling to carry any load, and like the chain that was beyond repair, so is he.
Getting a diagnosis for a chronic illness is everything. It does not mean you will get better per se, but you finally find that weird box that all your symptoms fit into and some solace comes from that. When I connected the dots after getting keratoconus and stumbled upon Ehlers-Danlos syndrome (EDS) and had my “Things that make you go hmmm” moment and then got a diagnosis of EDS type II (classical type) confirmed by a geneticist, it all made sense. I have zero doubts about my diagnosis and personally, I think my textbook case should be used in the textbooks so doctors can learn to connect the dots, as well.
It seemed like a closed case 6 months after my diagnosis, but EDS is a genetic disease—in type II it is autosomal dominant—and aside from those who are born with it via a spontaneous mutation, you only need one parent to carry the gene and the chance the affected parent will pass that gene on to their offspring is 50%. Flip the coin. It is quite clear that EDS type II came from my mother—who has a rather mild form but some obvious type II symptoms—and she inherited it from her father, who had a more severe case. This is documented in my genetic report. More information on classical EDS (types I and II) and basic, genetic information can be found here.
Just to up the ante, I do not have a good relationship with my mother. She never bonded with me, I have little recollection of her from my early childhood, and then have awful memories of her from around age 11 on as my father took an emotional hike and I got stuck with her until I decided that chillin’ in skeezy, 1980s, downtown Seattle at 13 via a bus pass and living with friends was a better option than dealing with my mother and the dysfunction in my home. As an adult, she was a chronic burden, and I got stuck raising my younger brothers for awhile when I was in college due to her inability to do so after my father divorced her. Then, I had to deal with her chronic phone calls for no apparent reason while at work during my pre-illness, workaholic, career years. She was a ball and chain tethered to my ankle for too many years to count and she was supposed to be the parent—not I.
Once I became sick, things deteriorated further with my entire family and her lack of ability to be a mother was more apparent than ever. She did take advantage of my illness to bolster her poor me story, however, which is the only story she seems to tell. She has many acquaintances, but no close friends due to her closed off nature, yet in time her entire circle knew about her hard knock life due to her sick daughter, although they had no idea that she did not even have a relationship with me. Nonetheless, this became her story for over a decade as it apparently worked for her—ol’ Johnny One Note.
Suddenly, however, things began to change once I got a diagnosis. My mother revealed more and more EDS symptoms that she had, which she previously dismissed when I inquired about them for myself. She would call my cell and not pick arguments with me for the first time in my life and while she is a passive listener who never has any suggestions or advice, at least she was not verbally abusing me on the other end of the phone. Dare I say she had a guilt complex that my 12 years of torturous pain, illness, and now impaired vision—not to mention losing my career, financial security, and entire life—were all from her defective genes? After all she had done, or not done, for me—I do not hold her responsible for giving me EDS. She did not even know she had it herself and we thought a lot of my grandfather’s symptoms were from his rheumatoid arthritis. Who would really know about Ehlers-Danlos back then? Most doctors still do not know what it is.
Well, all good things come to an end, of course. My mother, who is passive and does not have a voice of her own or an opinion on anything for the most part, was just trotting around China with my step-father, a retired physician, as they take extravagant vacations a couple times a year. She does not have a clue about medicine or medical research, let alone how to use a search engine or a library. Somewhere on the guided tour in China—so typical they take tours—she met all sorts of new friends from Australia. Note that making new friends is her entire reason for living and then she needs to tell everyone about how popular she is. Obviously, my mother told the sob story to her new friends. Well, as a result, she soon learned—possible over a lunch of pricey, shark fin soup—that one of her new BFFs had Lyme disease and my mother hung on to every word.
Let me preface this by saying I was tested for Lyme disease twice within the last 10 years, although it was the test that is not very sensitive. I had a couple of positive antibodies each time, which is not uncommon, but when discussing if we should order the pricey, sensitive test the second time around, my doctor and I decided to pass as I had no symptoms of Lyme disease, never had a bullseye rash, and the tick that carries it and transmits it to humans is very rare in any area I have lived in or traveled to. If I look at the symptoms of Lyme disease even now, it is so far out in left field I would never consider it. Therefore, it was crossed off the list.
My mother knows my EDS symptoms, or at least I told her what they were a thousand times. The more I research EDS type II, the more and more it fits. How she heard this new BFF describe Lyme disease and then came to the conclusion that this is really what I have and that my textbook EDS type II is all wrong is beyond me. Was my Johns Hopkins-trained geneticist having an off day when I saw her, as well? Now, I would so love to blame this on her lack of knowledge on everything aside from shopping, lunching, and throwing parties with lots of pricey alcohol, but I suspect she decided to play doctor for a reason, without the honorary medical degree I have acquired after more than 10 years of hardcore medical research on the wrong disease: my misdiagnosis. Why would she even be interested in my health when she never was before—and now that I have a clear-cut diagnosis, why would she question that?
It is so obvious, after all. It is all about her, like it always is. She gave me a genetic disease and now she is not able to tell the sob story without implicating herself in the process. Instead of acting like a mother who offers support or even someone who can relate to a couple of my symptoms as she has the same disease, even if in a non-disabling form, she is now set on finding a differential diagnosis in order to not be the one responsible for my illness and disability. That is precisely why she was never a mother to me and never will be, not that I would have a clue what having a mother is even like.
The final stage of the five stages of grief is acceptance. It seems that a genetic disease, as opposed to my former misdiagnosis, would be a hair easier to accept. There is literally nothing one can do to prevent it, so it removes all of the, “If only I had done x, y, and z.” Yet, how can I learn to accept all that has been put on my plate if some people cannot even accept the diagnosis? I would so love to put my mother on the next cargo ship back to China, but as she cannot do anything on her own, I presume she will fall overboard, be eaten by sharks, and then be ladled out as shark fin soup to the next round of wide-eyed tourists savoring the exotica in Hong Kong.
I had near-perfect vision less than 2 years ago. It was somewhere close to 20/20, aside from some mild myopia, or nearsightedness, that I had developed in my early 20s and did not even wear glasses for.
Then, the keratoconus (KC) developed and went wild and you can read my blog for that saga. I was at my corneal specialist’s recently as he wanted to see me for a 6 week follow-up after discovering central corneal opacity, or scarring, in my right eye months after my bilateral cross-linking (CXL) surgery.
Despite what I have felt has been rather negligent care, I got the full workup at the appointment: reading 2 eye charts, a refraction, a corneal topography, a corneal pachymetry, a check of my ocular pressure, and who knows what else I forgot. I was in a bad mood due to the disorganized clinic and was not really sure why I needed all the tests when I just had them done, but asked if I could at least have a copy of my topographies since nothing was initially sent to my CXL surgeon in L.A. for the clinical trial.
The techs are nice and had no problem with my request of having 4 papers copied, as opposed to the last time when I asked the front desk girl, who said she would have to charge me. Now, I finally had the scans I needed to see what my corneas and KC really look like at this stage, although my surgeon confirmed the other week that per my scans, the CXL was successful in both eyes and the progression of KC has been stopped in its tracks. Thank the Lord for modern medicine.
After all the testing and finally seeing what I could of those scans myself, the doctor rushed in talking a million miles a minute. He never seems to remember who I am or what my post-op issues are, which is really frustrating. He asked if I had any improvement since I had last seen him and I replied that I had some intermittent watering in my left eye—possibly due to the Restasis starting to work after 5 months—and that my right eye with the scarring had the same vision, which is a good sign, as well as the fact that the horrid light sensitivity (sun burning my retinas) was better, but not gone. He did a quick check of my eyes and said, “Good. Good.” That told me nothing, per usual, so I began with the questions that he hates, as he is already making notes in my chart at this point.
Here we go with the issues to preface all this. I read 2 eye charts—from right to left my visual acuity (VA) is now 20/40, 20/40 on one chart and 20/30, 20/40 on another. My left eye had gone up to 20/60 at my last appointment, so I was dealing with a post-op fluctuation that has cleared up. To err on the side of caution, I will say my vision is 20/40 with great difficulty on those last 2 lines, as no one has had me read the charts with both eyes, or binocular vision, which often gives a better VA. Would my true visual acuity not be of importance post-CXL? Well, of course, but I am dealing with sub par healthcare here. However, the big question is: why can I not see?
I should add that I do have a bizarre zone where I can see somewhat normally—it seems to start 1 foot in front of me and stop at 4 feet or so and there can be no sunlight or bright, artificial lights or the whiteout sets in. This is the safe zone and aside from a plasma-type TV on a not-too-far wall, I get staggered vision of everything outside that space in small rooms. For a description of how I see outside, in big spaces, and in the world in general—also known as the Ghosted Mess—refer to this post.
I have to ease my way into questions or my doctor gets irritated and the appointment ends, so I started with the basics. I wanted to know if the Restasis was causing the intermittent watering in my left eye, or if that was just from my corneas finally starting to heal from the surgery, as Ehlers-Danlos syndrome (EDS) is causing very poor and slow wound healing. The doctor could not answer that one. He kept saying my eyes were dry due to my meibomitis (MGD), which causes a lack of lipid (oil) layer in the tears, and that I just need more IPLs. I told him I had my last IPL 2 months ago and his associate—the doctor I like who specializes in dry eye—said more IPLs would do nothing at that point and I would not need a touch up for possibly a year.
I had 5 IPLs with no improvement that I noticed. However, the doctor thought they were successful in terms of the meibomitis, but as my eyes stopped producing the water layer in my tears post-op, they were still dry as a bone. Yes, this is all confusing, but for more information on MGD, as well as post-op dry eye, refer to this post. The corneal specialist gives me an odd look every time I explain that my complaints are related to my lack of a water layer and he says that I could barely open my eyes due to the meibomitis before the IPLs—although I only saw him once since 2010 and never had that symptom so I think he has me confused with another patient. Can I just see the dry eye specialist instead?
That leads to the big question—if my vision is roughly 20/40, why am I visually impaired? 20/40 vision is legal to drive in most, if not all, of the U.S. and I am not too sure I should even be on the road late at night, let alone left to wander in a big box store, which I would like to peruse with a stiff drink to offset the anxiety from the chaotic mess. Why do people look like shapes and colors and nothing more, unless they are in my safe zone? Well, the doctor is not sure, of course. What is he sure of? Why is he a doctor? Why is he the best corneal specialist in this horrid city? I may have better luck getting answers from the local fortune teller, who happens to be in the jenky strip mall next to my motel.
Now, I am not an ophthalmologist, but I am quite capable of interpreting my scans. Per the topographies, my better eye pre-op has no evidence of KC now in either the scan—showing no corneal steepening and 0% severity—or the K-readings, which are in the normal range now. My worse eye pre-op decreased in severity by 32.5%, although my K-readings are relatively the same in that eye, thus showing keratoconus—although it will not progress further due to CXL. K-readings measure the curvature of the cornea and there is still a significant steepening, which I can also see in the color-coded scan, but it appears to be much less severe, which makes no sense, especially since I got 6 or 7 lines of vision back in that eye. Both topographies do look quite different from my pre-CXL ones, which is rather fascinating. So, it would appear that I would have no keratoconic vision in my better eye, yet there is from my vantage point and I do not have my old 20/20 vision, regardless. May I also point out that at my last appointment, my doctor could not even tell me if the CXL had worked in that eye. Can I please move to L.A. with a giant dehumidifier and go see my surgeon for follow-ups instead? As mentioned, even I could interpret that scan with my impaired vision!
Back to the question at hand—my doctor asked when I would be seeing my surgeon again, as he seems to think I am able to go to L.A. at the drop of a hat—even though I would need an all day ride, have to pay a huge cash sum to see my doctor, and rent a motel room there, while living in one here. I reiterated for the 100th time that I have not seen him since my surgery in September of last year due to X, Y, and Z—not to mention that my local doctor referred me there and said he would handle all my follow ups. My patience was wearing thin.
Then, he asked how soon I could have laser vision correction surgery done there, even though that is my local doctor’s specialty (hence, corneal specialist). Is he crazy? I am 7 months post-op and a poorly healing, scarred mess due to my EDS and he thinks laser vision correction—namely PRK, which to my knowledge is still being studied for safety in post-CXL eyes (please fact check that), is a good idea? Due to the weak collagen structure in keratoconic corneas prior to the development of CXL, laser vision correction is contraindicated and is why all laser vision correction patients are given a topography to look for any evidence of KC before LASIK or PRK surgery. My eyes are so dangerously dry, plus the central corneal opacity that is no worse 6 weeks later but has not resolved, and he is even considering this? Furthermore, upon researching this issue just for the hell of it, the only scholarly, research articles regarding PRK following cross-linking involve or reference this ophthalmologist in Greece, who is on the payroll:
Financial Disclosures: Dr. Kanellopoulos is a consultant for Alcon, Inc., maker of the Wavelight excimer laser that he uses for topography-guided photorefractive keratectomy [PRK].
Maybe it is best to just see the local shaman wandering in the desert at this point. Perhaps some native shrub, like tumbleweed, can fix my vision—or is the local shaman in on the hustle to further destroy my corneas for some exorbitant fee, as well?
I told my doctor—who I now have absolutely no trust in—that there was no way I would go through surgery again with all the complications I have had and he mentioned something about my vision being bad from astigmatism. What astigmatism? Irregular astigmatism aka keratoconus? 9 months before my diagnosis, the optometrist I saw had misdiagnosed my keratoconus as mild astigmatism in my worse eye. Was this something new that my doctor pulled from the refraction done that day that I was not given a copy of? Add it to the list of copies I will be paying for very soon.
So, aside from residual keratoconic vision and little improvement in my K-readings in my worse eye, which I still don’t understand if the cone is so much flatter per the topography, is massive astigmatism in both eyes—the normal kind—contributing to the significant visual impairment now? If so, why can I not see any better with a refraction, just like when I had one before my diagnosis. I just answer, “Same” every time they flip the lens and ask, “Which is better—1 or 2?” Is the KC cancelling out any hope of lenses correcting the astigmatism? Why can I not get correction in my now non-keratoconic eye like my brother who has astigmatism and gets by fine with glasses? I am so utterly confused and Dr. Corneal Specialist is of no help.
As I have no choice but to wait and hope my vision improves a little more as my corneas continue to heal, patience has now been added to the survival box, and while I twiddle my thumbs, I will keep crunching the numbers in attempts to resolve the elusive mathematical equation involving my VA: 20/40 = X.