Struggling with POTS

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One of the conditions that I’ve been dealing with—that I don’t often talk about—is known as Postural Orthostatic Tachycardia Syndrome, and it frequently co-occurs with the type of connective tissue disorder that I have. What this “syndrome” means is that, though I usually have a healthy resting pulse, when I stand it increases, often dramatically so. The effects that this can have and the list of symptoms that it causes seems near endless—and while my POTS isn’t as debilitating as it is for others, it does make an already difficult disorder sometimes seem an even more monumental task to cope with.

As an example, here is an incomplete overview from Wikipedia on just some of the most common symptoms:
• palpitations
• light-headedness
• chest discomfort
• shortness of breath
• weakness or “heaviness” in the lower legs
• blurred vision
• headache
• decreased concentration
• mental clouding
• extreme fatigue
• nausea
• near-syncope
• difficulty sleeping
• fluctuations in weight, memory
• pallor, or sweating

I experience every single one of those effects—most of them on a daily, continuous basis. And, this is despite the fact that I’m on a high dose of another difficult medication to control it. The medication itself also causes it’s own problems. While it does lessen the amount that my heart rate rises upon standing, it also causes my already low-normal blood pressure to fall quickly—and dramatically—along with it. This, ironically, also causes many of the same symptoms I’m on the medication to treat in the first place. Despite this, the medication seems necessary for the time being because, while it doesn’t prevent a large increase in my heart rate entirely, it does keep it from going as high as it often did without—and prolonged tachycardia (of any kind) can be hard on the heart, particularly with my disorder.

POTS is also a difficult disorder to navigate, as symptoms may be exacerbated with things like: “prolonged sitting, prolonged standing, alcohol, heat, exercise, or eating a large meal“. I seem to have to tip toe around it in order to help keep it controlled, but frustratingly, the path that I need to take to do so often conflicts with the things I’m supposed to do to minimize the effects of some of my other health issues. For instance, nearly every recommendation for helping someone with POTS (like increased salt and fluid intake), is the opposite of what I’m supposed to do to help stabilize my intracranial pressure.

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This was my pulse and blood pressure while sitting down.

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And this was my pulse and blood pressure shortly after, while standing up. My heart rate increased greatly, while my blood pressure fell just as much.

This can be a frightening thing to live with—the possibility of it’s effects worsening, hanging always over your head. That’s something that can be hard to ignore when you’re dealing with such prominent (and at times all-consuming) symptoms. I know that on the whole I’m lucky. I can still walk around normally without a wheelchair and haven’t begun to lose consciousness whenever I stand. That’s something that I absolutely know to appreciate with this syndrome. But—at the same time—dealing with all of the other ways it’s effecting my quality of life on top of a debilitating disorder can be difficult, and exhausting.

The hospital stay: part 2

Continued from yesterday.

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The spinal fluid leak and post-dural puncture headache.

During the lumbar puncture they were instructed to get fluid for diagnostic testing, but also since they were so sure my problems were caused by increased intracranial pressure, to both measure said pressure to prove that theory, and to drain some extra fluid to see if my symptoms improved. But, they were really surprised to find when measuring my pressure that it was well within the normal range. I was confused, surprised, and worried. They were also confused and unsure what to do next, so they decided to drain some fluid anyways just to see if my symptoms improved (something that I also thought was a good idea at the time). Now I should mention that before the LP another big concern of mine was a potential complication that they call Post-dural puncture headache, and the continued leaking of spinal fluid from the puncture site. My connective tissue disorder puts me at a higher risk for this because my tissue is weaker and doesn’t repair or heal itself properly. Within hours of the LP it was clear that I was suffering from exactly that. The pain from the headache if I’d sit or stand up came on quickly, and severely. Likely made worse by the spinal fluid that was already drained during the LP and the fact that it was drained despite my pressure being perfectly normal to begin with.

While most leaks eventually seal themselves and the headaches and other symptoms then resolve, I knew that in my case my tissue likely wouldn’t be able to do so by itself. When this happens they do another spinal injection, called a blood patch, where they inject some of your blood into the area around the puncture site, which triggers an inflammatory response and usually successfully seals the leak. Despite my telling them that my connective tissue disorder would almost certainly make a blood patch necessary, they said that they won’t do blood patches until it’s been a week because by then most leaks have resolved. This brings me to something that I noticed often in the hospital: when mentioning my connective tissue disorder, those who even knew what it was, would act as if it changes nothing in the way that they handle my case. This can be both frustrating and scary because your connective tissue is everywhere, and when it’s weak it has the potential to change nearly everything big and small about how your body reacts to virtually anything that done to it from a procedure to surprisingly medications. For instance, as I observed and pointed out near the end of my hospital stay, my tissue was necessitating the frequent changing of my IVs, because otherwise the IV would fail and my vein would end up inflamed and sore. Up until that point, they’d been trying to leave the IVs in for as long as they would with any patient, and my weaker tissue couldn’t handle it. This happened many times without anyone taking into consideration my disorder, despite me frequently mentioning it.

As for waiting an entire week for a blood patch; the thought was terrifying for me. The pain in my head was nearly unbearable at times, and even laying down didn’t fully resolve it. But what made it worse was that with my chronic pain, particularly my back issues, having to lay flat on my back for very long without being able to change positions is very painful. And now that was the only way I could lay. I couldn’t begin to imagine having to do it for a week, yet there was no other position that I could tolerate from the severe headache.

Pain, vomiting and frustration.

The next morning, I was told that my doctors had decided that they wanted to do an MRI. The MRI didn’t show anything that could be causing the swollen optic discs either. It did show that I had an enlarged pituitary, a sign of intracranial hypo-tension, resulting from the spinal fluid leak and confirming that the severe headache I was experiencing since the hours following my LP were from that. Considering all of the information my doctors in the hospital had on how my ICP hadn’t been increased, was now far too low, and continuously lowering still from the leak, and the fact that I was in that much pain because of it; I still can’t understand why they would ever think that it was a good time or decision to start me on a diuretic in order to further lower my ICP (which can be dangerous!). Given that my first dose of the medication was that evening (after doctor’s rounds) and I wasn’t aware that they were planning on putting me on it and hadn’t been spoken to about it, my nurse essentially gave me the option of either just taking the medication until my doctors could talk to me and authorize them taking me back off of it, or she could write down in my file that I was uncooperative and refusing to take my medication. So, since I’m not a very confrontational person and I was too tired and sick to argue it further, I took the medication, knowing it could likely make a nearly unbearable problem even worse.

Once I saw my doctors the next day they said that they agreed that I shouldn’t begin taking the medication yet (you think?!), with no explanation as to why they didn’t just agree with that in the first place without making me worse off with it instead. My pain was pretty excruciating at this point and I hadn’t really slept much in the past week with everything going on. My mom wasn’t a whole lot better off but she was there for me no matter what, and it was a lifesaver to have someone there to help advocate on your behalf because frighteningly at times, my treatment in the hospital (supposedly one of the best in Canada) was dismal and at times bordering negligent. I was left to deal with the horrible acute pain of everything going on with little more than what I take at home on a daily basis to begin with, and for the first few days because of some mix up with my medication forms, they couldn’t even give me ibuprofen. They eventually, after prodding, started to give me what they called a “headache” cocktail every 8 hours, which I was relieved and hopeful about. The cocktail was an IV anti-nausea medication, followed by an IV drip of an NSAID (ketorolac). I thought maybe I noticed a slight improvement at first but soon – maybe from the still lowering ICP – it wasn’t doing anything but making me sick.

One of the things that they say is incredibly important with a spinal fluid leak is to get plenty of fluids. But by this point the low ICP (and the ketorolac) was making me vomit even just from trying to drink water. Despite being well aware of that, it wasn’t until I told my nurse that I was probably getting dehydrated and should be put on fluids that she said she’d mention it to my doctor. A few hours later they had eventually hooked me up to fluids, and I continued to not be able to eat or drink without throwing up. But still, after 24 hours of not having to urinate once, I was pretty sure that I was still dehydrated, perhaps severely at this point. This was when I found out that the amount of fluids they had put me on was equivalent to about a cup of water a day, which is well under the needed amount for an average person, never mind someone who’s both leaking spinal fluid and continuously vomiting. I told my nurse that I couldn’t drink anything and wasn’t getting enough IV fluids and despite all signs clearly and obviously pointing to that being a major problem, she still seemed unsure about it. So, instead of upping my fluids immediately, instead, to see if that really was the reason that I hadn’t been urinating, she insisted on first scanning my bladder for an obstruction. I had to so strongly point out all of the reasons that dehydration was going to be the problem that I was starting to get weepy and exhausted because I couldn’t understand why I was having to fight for this in the first place. It’s hard to explain how this feels; when you’re in severe pain and unable to move, frequently throwing up, and having to literally argue the importance of the most basic human need (water) to the people who are supposed to be taking care of you. I felt like I was constantly fighting for the simplest things in the hospital.

I want to say here that I’m a very easy going, compliant and non-confrontational person, often to a fault. I have a difficult time sticking up for myself, even when it’s right and needed. I also understand that being a nurse is a very difficult job. They deal with an incredible amount and they do one of the most important and demanding jobs that there are, often without thanks. But, that being said, there’s also a standard of care in hospitals, and it’s frustrating, and frightening when that standard of care is repeatedly not being met, or when the simplest things are made difficult simply because you’re not being thought about as a human being or listened to. It’s an incredibly helpless feeling to be bed bound, and in severe pain, and to have the people with power over you, who are responsible for taking care of you, often failing in the simplest areas of that job. To end up dangerously dehydrated while your in the hospital seems unacceptable to me, particularly when someone is repeatedly pleading with you to do something about it.

There were so many instances in the hospital that made me feel that way. There were days where I was informed that they hadn’t ordered a medication that I was on from the pharmacy yet (spoken as if it were no big deal) and was told that I’d have to miss a dose, and I’d then have to make a point of fighting just to get a medication that I had managed to never miss a single dose of at home. There was also the time that my IV machine kept repeatedly beeping in the middle of the night indicating a problem in the flow of my IV and despite me telling my nurse multiple times that I could feel fluid dripping down my hand, instead of simply looking at it she kept trying to adjust the machine or untangle the cords. Then she’d go away for a few minutes only to have to come back again when it would start beeping. I felt pretty badly for my room-mate that night. When she did finally turn on the big light to check, my IV was leaking blood and fluid down my hand which had been the problem, so now she had to try to do another IV in the middle of the night. Two hours since the first beep, two nurses trying, and 3 IV pokes later the problem was finally resolved when it could have been almost immediately instead.

Unanswered questions, an uncertain diagnosis and it’s connection to my disorder.

My neuro-ophthalmologist was surprised that my ICP hadn’t been high. He was certain that must be the problem because none of the other scans and tests had shown anything else that could be causing the optic disc swelling. He wasn’t entirely sure of what to do to help my eyes at that point, so decided (as mentioned earlier) that the easiest course of action for now would be to put me on a diuretic, just to see if there was any improvement and then to watch me closely in case my vision deteriorated. We decided that the med should wait until my ICP recovered from the spinal fluid leak, and then we would slowly begin the new medication (which can be difficult to tolerate). I felt really frustrated, not at anyone, just at the fact that here I was again, in a difficult medical situation with no clear answer.

Luckily for me, someone with the ILC foundation that I often correspond with and who knows and works with many others with my disorder, as well as with possibly the top neurosurgeon in the world dealing with problems related specifically to our rare disorders, helped me get some answers. I later found out that there is indeed a link between both high and low ICP, and connective tissue disorders such as mine. It seems that for whatever reason (they haven’t been able to figure it out yet), our bodies can’t always regulate our spinal fluid flow and “draining” system properly, which can result in an ICP that can fluctuate between being normal, to too high, to too low. This can cause symptoms like headaches that can come and go, and when the ICP irregularities are prolonged this can cause symptoms like swollen optic discs. And while I’m not happy to have another rare, often unknown side-effect, of an already rare, often unknown disorder, I am glad that I’m not the only one and that there are some answers out there. It made sense as to why my ICP was completely normal when it was measured despite all of the symptoms and signs I’d been experiencing pointing to it being too high before. This problem, whatever the cause, can be difficult to treat, but that was a bridge I would cross once and if I got there.

I wasn’t sure how I was going to do it but slowly each day dragged on and ultimately passed by. My symptoms briefly improved enough for me to walk up and down the halls outside of my hospital room a few times, holding on to my mom, but they quickly went back to how they had been, picking up new symptoms along the way. I started having problems with my hearing in both ears which I was certain was from the low ICP, but at the time the resident neurologist, who I saw the most during my hospital stay and who tried to do my LP the first time, told me that it was physically impossible (not even just unlikely, but impossible) that my low ICP and my ear problems were related. This scared me because he said it with such certainty, and I was now thinking that either something else was now wrong with me, or that I was losing it and it was just in my head. Frustratingly, we later found out the ear related symptoms I was experience were in fact one of the most common symptoms of low ICP and was absolutely caused by my spinal fluid leak. Which strikes me as the kind of thing that he should know, and if he didn’t know then I wish he would’ve just said so instead of telling me that the thing I know that I’m feeling is actually impossible.

Blood patch and being discharged from the hospital.

Eventually, when it was finally nearing the week mark since my lumbar puncture, it was decided that it was time to do the blood patch to seal the spinal fluid leak. I can’t even begin to explain what an enormous relief that was to hear. I was also a bit afraid, knowing that they’d have to do another spinal injection in the same spot, but without radiology. Luckily for me the anesthesiologists had a much easier time than the neurologists had. It took him three attempts, but he interestingly used the nerve pain it was causing to help him find the right spot. When he brushed a nerve (unfortunately for me) the first time, he asked me which leg I felt it in, and when I said the left, he seemed to move the needle over to the right side, and when I felt the pain again, but this time in my right leg, he moved the needle ever so slightly back to the middle, which worked.

Blood patches are not a guaranteed fix for a spinal fluid leak which scared me. I was told that they work about 80% of the time, but I felt like it was doomed to fail after everything else that had gone wrong. They often work immediately but as much as I wanted to say that it had worked when the doctors asked me, I noticed no improvement whatsoever in the hours following. So, we were told not to give up hope and that it was very important that I lay flat on my back and not move, because that just might get it to work. And eventually, ever so slowly, I started to notice a gradual improvement. By then, I was so desperate to get out of the hospital and so happy that I could see a light at the end of the tunnel, that I played up how well I was already feeling as much as possible and told them I was certain I was good enough to go back to the hotel. Even then I was fully aware that I should have waited at least one more night in the hospital, but that stubbornness kicked in again. I was still in pretty bad shape, and both sitting and standing for too long were still incredibly painful. By the time I exited the taxi and made it to my bed at the hotel it was nearing my limit of tolerance, but every hour I found myself able to sit and stand a little longer each time I tried.

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Saying goodbye to my hospital bed!

We had one last appointment with my ophthalmologist to see how my eye infection was doing, which was also pretty hard to get through pain-wise, but it also signaled the longest that I’d been able to sit and stand since my lumbar puncture, and that I really was on the mend.

Getting home, and how things are a year on.

Before long we were on an airplane home, finally, a few weeks after we’d left. But frustratingly, we were returning with a lot of unanswered questions and uneasiness, on top of the incredible relief. My optic discs weren’t better, and the lack of a concrete, simple explanation for what was going on left us worrying about how or if we’d be able to treat it.

During my hospital stay, because of the trouble we’d had with my IVs, I also ended up with thrombophlebitis which is an “inflammation of the wall of a vein with associated thrombosis”. It was in the hand that 2 (out of 4 or 5) of my IVs had been in, both of which ended up having problems and leaking. The veins on that side of my hand have hardened and were at first red, visibly inflamed and often painful. While this condition can usually be avoided by changing the IV once a week, it seemed that with my fragile tissue even a couple days of an IV in the same spot ends up being too long.

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The residual redness and inflammation in the back of my hand and veins.

All in all, things have been up and down since then. The medication has been very hard to tolerate at times particularly in regards to my P.O.T.S. It takes many of the difficult symptoms of that and exacerbates them, particularly the fatigue and faintness. Often at times it can cause very painful pins and needles in my legs, but as my dose has decreased this symptom has nearly disappeared.

We travel to P.G. to see an ophthalmologist, at first once a month, but since my eyes have been relatively stable, now we go every 2 months. Then we travel back to the neuro-ophthalmologist in Vancouver first every 3 months and now every 4. Sometimes my ICP doesn’t seem to be too high and sometimes there are signs that it is indeed too high again (like a lack of pulsations in the back of the eye caused by increased ICP). The swelling in the optic discs tends to still be there, though this last appointment in Vancouver it was decided things were looking really good so we could try lowering my medication to see how things went. Now I’m waiting for my followup appointment in Prince George to see if lowering my medication caused a worsening of the swelling or pressure.

It was a bit of a difficult and scary year at times. And by the time I finally got home I was covered in bruises and reminders of everything that I had been through. But now, it’s hard to believe that’s how long it’s been since all of that kicked off. I can’t imagine going through any of it without my incredible mom. She was there every single step of the way, no matter how awful she was feeling or how difficult things got. I’m so, so lucky to have that support through things like this.
And, thank you to all of you for sticking around, and to the amazing few who actually made it through this novel of a post (I don’t blame you if you didn’t!).

– Katie ♥

The hospital stay.

Things can change so fast. And, likely not so dissimilar to a perfectly healthy person, life is full of highs and lows, peaks and valleys. I won’t deny that often during the lows, as a chronically ill person, it’s very easy to get lost moment to moment in how you wish things were and how unfair it feels that your life has been and will continue to be this way. You can forget to just be grateful for the things that you do have, because you’re constantly being reminded of the things that you don’t or can’t. I think it’s a pretty normal way to feel about things under difficult circumstances, and yet, even when I’m struggling with those feelings, I’ve always kept myself reminded of how lucky I am for my vision. Even – or perhaps especially – when something happens to decrease it further. I know that the vision that I have at any given moment isn’t guaranteed and so I make a point of reminding myself often to cherish it – and I absolutely do, above nearly everything.

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It’s been a long year and there’s a lot to cover, so I’ll have to break it up and stretch it out a bit. Hopefully it doesn’t end up being too disjointed, but with everything that happened and my brain it very well might be. To start with, I wanted to explain a bit about everything that happened last summer, and what has changed since then because of it.

The first signs that something’s wrong.

On the 1st or 2nd of July (last year) I woke up with an intensely painful right eye, (which is actually supposed to be my good, “stable” eye). When I was trying to figure out if it was an emergency or not, I lifted up my eyelid and could see that a couple of the sutures (visibly sitting just below the surface of my eye) from one of my previous lens surgeries were very red and irritated. Shortly after, the optometrist confirmed that one of the sutures was indeed poking out (again) and would likely need to be removed via laser by my longtime surgeon in Vancouver. Since we were already scheduled to see him near the end of the month to check on the lens issues that I was having in the other eye, we were assured by my optometrist that as long as the pain wasn’t unbearable then it would be perfectly alright to leave it be until then. He had even suggested that maybe if we got lucky the friction caused by my eyelid rubbing over the suture repeatedly might even file the suture down on it’s own (yes, ouch!).

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Believe it or not: this wasn’t even the worst my eye would end up looking that particular week.

This all happened to be going on at the same time that my baby nephew (who I hadn’t seen for months) was finally in town staying with us and I desperately didn’t want to leave him almost as soon as he arrived, so I kept trying to hold out, despite how surprisingly severe the pain was often getting. As the days passed though, I started noticing alarming visual symptoms in that eye, at increasing frequency. Looking back on it now, it seems like things started to happen and change very quickly in the days that followed. I had now started encountering flashes, trailing images and tunnel vision in that eye with shocking intensity. Despite everything that I’d been experiencing throughout my life with my eyes, nothing had ever come close to effecting my vision like what I was now going through. Reds appeared dull and faded, and I started finding that I often couldn’t see anything at all in dimly lit rooms. And I knew that even if it was to blame for some of my symptoms a protruding suture shouldn’t be causing all of them – not to that degree. I knew that something serious had to be wrong, how could it not be with what I was seeing on a daily basis? And yet, I kept making emergency trips to my (very understanding) optometrist, who kept looking in my eye and seeing nothing that indicated any problems aside from the still protruding suture.

I was stressed out, confused, and frightened. The fear and sense that something was dangerously amiss kept me going back to the doctor, nearly every single day, despite being routinely told that everything looked perfectly fine and that it was still safe to keep my end-of-the-month appointment in Vancouver. Finally, after nearly three weeks had passed since that day I woke up with a sore eye, I started experiencing transient episodes of extreme double and blurred vision. This was essentially the last straw for both my mom and I’s nerves and fears about what was going on. I was frazzled and exhausted but I felt that I had no choice but to pack up that night and head to Vancouver first thing in the morning by bus, wondering the whole time if maybe I was just crazy or overreacting. In the morning, we figured with the drastic change in my vision so quickly the night before that we should go see my optometrist one more time before I left. He decided to dilate my eyes to get a better look (which seemingly should have been done already), and was very surprised to find that my optic disc was swollen – an unusual find, especially at my age. The optic disc is the head of your optic nerve (what connects your eye to your brain and transmits visual signals). It’s also the entry point for the major blood vessels that supply the retina, and a swollen optic disc can dangerously compress those blood vessels and lower blood flow in the eye. It also indicates the presence of a potentially serious condition.

Many things can cause optic disc swelling, all of them pretty serious, and none of them particularly pleasant. At the time he noted that only one looked swollen, which suggested it wasn’t anything as serious as increased intracranial pressure but none the less, after speaking with my ophthalmologist in Vancouver about the find, it was decided that it was best we get down there immediately. We hadn’t really slept in days with everything we’d been dealing with but following that my mom and I ended up catching the next flight to Vancouver so that we could see my ophthalmologist early the next morning.

Vancouver appointments, swollen optic discs, and an abscess.

Thus, we began the first of what would end up being weeks of exams and scans and were soon informed that both of my optic discs were in fact swollen, with the right being worse than the left, and that blood flow was also noticeably decreased in the right eye. I was really surprised and genuinely hadn’t been expecting any of those things to be happening. I was also admittedly scared, knowing that both discs being swollen signaled something potentially very serious. This was entirely new territory and nothing that I was aware of having a direct connection to my tissue disorder, so, I was potentially looking at an entirely separate medical condition, on top of the debilitating one that I already have. My eyes were being dangerously threatened again, despite not having completely recovered from everything else that had previously gone on (and was still going on) with them.

Swollen optic discs have the potential to cause both retinal hemorrhages and complete blindness, especially when advanced, so it was terrifying when my ophthalmologist said that he suspected that the discs had been swollen for quite some time. We hadn’t even had time to fully process the implications of all of that before we’d been hit with another equally as frightening problem, one that seemed to alarm my doctor even more; my protruding suture had developed into an incredibly dangerous abscess/infection. Yet somehow and worryingly, I had absolutely none of the hallmark signs or symptoms of that type of serious eye infection and if we hadn’t gone to Vancouver for the swollen optic discs it would never have been discovered in time. What makes these infections so dangerous is that, if they reach the inner parts of the eye they can cause catastrophic damage, sometimes to the point of necessitating the removal of the eye itself. The chances of these two serious, completely unrelated conditions happening at the same time are nearly non existent. And yet, here I was, in the midst of exactly that.

Both issues were going to have to be dealt with as soon as possible. First, anything that causes optic disc swelling in both eyes is caused by something that is wrong with the brain, so I needed to see a neuro-ophthalmologist first thing on Monday morning (it’s nearly impossible to find one in an emergency situation on the weekend). As for the abscess, we had to fight it hard with antibiotics and watch it very closely. I was told to put an antibiotic drop in it once every hour, around the clock (even during the night) and a steroid drop twice a day. I had to see an ophthalmologist at about 6 or 7 am both Saturday and Sunday morning to see if the infection had improved at all, and both times it hadn’t gotten any worse, but it also still hadn’t improved despite the high dose of antibiotics. So, we were prescribed another antibiotic drop on top of the one that I was already using, (both still every hour) making a grand total of 50 drops a day, including the steroid drop I was also using twice a day. The dose of antibiotics was so high for an eye infection that the pharmacist dispensing them at first thought that the prescription must be a mistake. With waking up once an hour, to do two drops, 5 minutes apart each time, it’s safe to say that neither mom or I were getting any sleep.

The neuro-ophthalmologist, and ending up in the hospital.

I can say this now, I’m really glad that I had no idea what was in store for me Monday. I was relieved to find that my neuro-ophthalmologist seemed to really know his stuff. He was quick and direct to the point, but also empathetic and personable. I felt like I was in very good hands for once. He said that he suspected that I had increased intracranial pressure, but that he also couldn’t rule out other things like a brain tumor or MS. He thought it best considering the seriousness of what he found that I be admitted to the hospital where they could immediately start investigating what was going on. Especially since we’re from such a small, far away town and wouldn’t be able to leave the city until they knew what was happening.

Looking back, everything seems like it moved so quickly after that, but living it was a very different story. It took about 9 hours for me to be taken to and fully admitted to the hospital (a tough day for two chronically ill people). The morning after, 5 neurologists of varying experience levels (I was in a teaching hospital, which I sometimes learned the hard way), came to do another visual and neurological assessment. They also suspected that given my age that I was most likely suffering from increased ICP as well. They let me know that they’d be doing a CT scan and if that was clear, a lumbar puncture. At this point, of all of the things that could be going on, increased ICP was the best diagnosis that I could hope for, so I was happy when the CT scan was clear of things like brain tumors.

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Abscess, antibiotic injection and suture “removal”.

Also on the day after being admitted to the hospital I was taken by hospital bus with my mom (because I was a patient on the neuro-ward I wasn’t even allowed to walk outside by myself, understandably), back to the ophthalmologist dealing with my eye abscess. We were still doing the 50 drops a day and yet even after all of that it somehow hadn’t improved. So, at that point we decided that it would be best to inject the antibiotics directly into my eye, and the infection, to hit the abscess even harder, and to let me get some sleep at night finally. I was not looking forward to this. In spite, or maybe because, of everything that I’ve been through with my eyes I’m quite squeamish about the thought of anything poking my eye. Although, I doubt that feeling’s unique to me. But, despite my reservations, I knew it needed to be done, so while my mom quickly walked to the pharmacy to get the antibiotic to be injected, I sat quietly and steeled my nerves. After the injection he would also be lasering off the part of the suture that had caused the infection in the first place. I’ve had this particular procedure done before, so I already knew that it was a fairly unpleasant one.

The eye injection was very difficult. It was quick, but the pain it caused was very jolting and unexpected. He said “little pinch” to let me know that there’d be some pain when he moved the needle into my eye a little bit, and it hurt quite badly, but I thought it was over. When he said “okay, now big pinch” and moved the needle in further, I had but a fraction of a second to realize what was coming. And it hurt. I can still, nearly a year later, vividly recall exactly how it felt and how shocked I was at the severity of it. I could tell that my doctor felt bad about it all and he apologized profusely. Afterwards my eye took a while before I could finally get it to open and it had stopped watering. It was definitely difficult, but it was necessary, and in the end, I was glad that it was done. Maybe now my eye would start to mend.

After the injection the laser procedure, though unpleasant, was a walk in the park. When it was all over I felt really relieved, but also proud of myself for enduring it all and doing it so calmly. I know I didn’t have much of a choice, but when we’re pushed we’re often surprised at how strong we can be, and that’s definitely how I was feeling. And, it was all well worthwhile because afterwards my eye began to get better continuously, and the worry of just how close I’d been to something catastrophic, began to subside.

The multiple lumbar punctures.

The lumbar puncture was another incredibly punishing experience. To start with, whatever’s wrong with the part of my spine that they need to do the puncture at, makes it unbelievably painful if it’s pushed on, even slightly. And for a lumbar puncture (which I was already a bit anxious about) they need to push on that spot, a lot, in order to landmark where they need to put the needle. That itself was so painful that I kept thinking to myself if the lumbar puncture hurts less than this it’ll be a breeze. I had a bad feeling about the way the lumbar puncture was going to go from the start. But I kept telling myself that it was just my nerves talking and that everyone probably feels that way right before getting one. But the resident neurologist that was doing the LP was having a really hard time finding the place that he was supposed to mark for the puncture. So, we decided that he would try to do the LP twice and if he couldn’t do it then they would just get radiology to do it with an x-ray another day.

The thing is: I’m stubborn, and I knew that if they couldn’t get the LP to work that day it could mean days longer that I’d be in the hospital waiting for an opening in radiology. I desperately didn’t want that. The injection of the freezing wasn’t particularly pleasant but it wasn’t too bad, and they usually say that’s the worst part. So when he had tried unsuccessfully 2 times, I felt okay to tell him that he could try a few more times. He did, but was still having no luck. So he went and found a colleague to see if he’d have better success. I was feeling pretty stressed at this point and the longer they both tried it the more frequently they were brushing the nerves that send signals from the brain to your legs. I was told that this in itself wasn’t really anything to worry about, but it can be very painful and sometimes it was excruciatingly so, especially if they weren’t fast enough in withdrawing the needle once I’d told them that they’d hit a nerve again. To be honest, I lost count of just how many times they tried back and forth. My conservative guess is somewhere around 9 but I think it may have even been more.

By the end of it I was an emotional and physical wreck. The hardest part was not knowing when that awful pain in my legs would start again, how long it would last, or how bad it would be, and by the time they finally gave up it had happened so many times to such a horrible extent that I was incredibly tensed and stressed and I was close to not being able to handle it anymore. I was also so frustrated that I had gone through all of that for nothing and would still have to stay in the hospital and wait to go and get it done again. I had so badly just wanted to get it over with so I could stop stressing about it and we could finally figure out what was wrong. I tried so hard to tough it out, even when I was at my limit for pain, and it ended up being for nothing. I also wouldn’t have even worried about that if I had known just how quickly a spot was going to open up for them to do it with the X-ray (the next morning, not even 24 hours later).

I was pretty stressed going into the lumbar puncture the second time. My spine was severely bruised from the last time (even the radiologist was surprised at the extent) and still very sore, and now I knew how much pain it can cause when a nerve is brushed, which was what stressed me out the most, by far. But, I was told that it would be quick and easy with the X-ray, even though I – rightfully – had my doubts. The doctors doing the LP this time were having a harder time than they expected right off of the bat. And from their conversations I could tell that wasn’t a very common occurrence. I kept thinking “oh great, just great, here we go again” and “how am I going to get through this again?”. The first attempt didn’t work and when I asked what was wrong I was told that the degree to which my spine was crooked at that spot was making it hard for them to do the LP, even with X-Ray guidance. To my incredible relief, they were able to eventually get the second attempt to succeed, and I foolishly thought to myself “I did it, the hardest parts are over now”. What I thankfully didn’t know at that moment was that I still had another week of hell to go through.

I split this post into two parts (the second of which I’ll post tomorrow) because of how ridiculously long it ended up being. If you even made it this far I would consider it a miracle.

It’s hard to go through it again.

On Saturday something fairly heartbreaking happened to me medically. I wasn’t sure whether to post anything about it until I have some more answers, but I found it fairly therapeutic to vent about at the time and would like to post it. This is from the day that it happened. I’m sorry that it’s not the most positive of posts, but this has been really hard and it’s how I sincerely feel about what’s been happening.

It’s 5:30 am when she decides that she can’t fall back asleep anymore. She finally got a decent night’s sleep though, so she’s happy to get up today. She turns on the light and immediately realizes that something is wrong – out of the corner of her eye she notices the edge of her lens implant has come largely into view. Every time she moves her eye her entire visual field bounces and distorts along with it. Her heart rate shoots up and all she can think about is how badly she doesn’t want to go through this again. She’s been here before. She knows how this works and largely what’s coming next.

The day ahead of her now consists of ER and emergency optometry visits.  And she knows that the next few days will be filled with wait and worry. These are the things that she must now face, but it’s the uncertainties that frighten her the most. She will need surgery again, that is all but guaranteed and she knows that this time they may not be able to fix her eye. They have to be smart about this decision because anything they do to her eyes now would result in further damage – her tissue is weak to begin with and it’s been significantly further weakened with every lens subluxation she’s suffered. She’s only 21 and her vision is the most important thing in the world to her – and now the future of it is up in the air yet again. Every option they have is filled with uncertainty and potentially devastating consequences. She hates to be afraid but she is. The last two eye surgeries she had were filled with so many complications. They were far more painful than anything else she’s been through and instead of taking a month or two to heal it took 6 months. Her vision hasn’t been the same since – never nearly as good. But she’d give anything to have that vision back right now.

On Monday they will begin to put a plan into place of when she will likely need surgery. It would be so much easier if she didn’t have to travel so far for them, and during the holidays too. The surgeon that she’s had since she was 4 might not even be in the country right now, and she fears ending up with someone different with her complicated disorder and history. Her other eye concerns her too – if history repeats itself the lens in this eye could also dislocate within the next few months, just as it did last time. She tries not to dwell on those what ifs right now, they do her no good. So instead she puts one foot in front of the other, no matter how desperately she wishes she didn’t have to. She’s thankful that she’s not going through it alone and has her mom by her side. She’s coping fairly well, but it’s hard not to think about because it’s in her vision every second of the day reminding her. Eventually she starts covering it with a patch – it aches and makes her dizzy and the patch eases those issues and lets her think about something else.

The lens is still hanging on for now – last time it took a week to fully let go. She’s not even sure what she wants at this point – part of her just wants to get it over with, the other part of her is hoping beyond hope that it keeps hanging on just a little while longer. She worries that leaving the lens to pull through the tissue where it’s still attached is a bad thing to do and will cause a lot more damage in the end. But, she knows that her surgeon wouldn’t risk operating on her while it’s still attached. There are no good options, she feels suspended and numb, like she fell asleep and is just now waiting to wake back up. If only she could.

Marfact #25 + My lens journey: Part 4

In honor of Marfan Syndrome awareness month, here’s today’s Marfact (provided by the wonderful Marfan Foundation).

Marfact #25: People with Marfan syndrome should be treated by a physician familiar with the condition and how it affects all body systems. Careful management includes an annual echocardiogram to monitor the size and function of the heart and aorta; an initial eye exam by an ophthalmologist, including a slit‐lamp exam, with periodic follow up exams; careful monitoring of the skeletal system by an orthopedist, especially during childhood and adolescence; medications such as beta‐blockers to lower blood pressure and, consequently, reduce stress on the aorta; and lifestyle adaptations to reduce stress on the aorta.

Visit www.marfan.org for more information.

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My lens journey part 4:

Part 3

The healing process from the last surgery to re-attach my left lens implant was painful and slow going thanks to the complications that occurred. It was only 3 months later and a few weeks into December of that same year when I noticed a worrisome ring forming around the vision in my right eye. I was pretty sure that this was the lens but my optometrist said everything looked fine for the moment. Regardless of how it looked on exam, I knew that something was wrong and after a few days of the ring continually increasing in length and thickness it became obvious. I bent forward to get a pair of pajamas out of my dresser drawer and my entire lens slid forward into the front of my eye.

This was different than the last time one of my lens implants dislocated in that it happened gradually and was still holding on to some extent. The last time it had happened instantly and it wasn’t hanging on at all. My surgeon again wasn’t available for a week, so this was another long week of sleeping upright and worrying about what I was about face. Only this time I had every reason to worry, after all of the things I had gone through during the last two surgeries and everything that followed. I had just lost the vision in my remaining “good” eye and I was looking at what could be another horribly painful, complicated surgery with months of healing time. I was afraid that the vision in this eye would turn out as poorly as the vision in my left eye had or worse.

I held onto the hope that because 4 of my 6 previous surgeries had gone perfectly that this one likely would too, despite how the last two turned out. After all, the odds were technically in my favor. The surgeon decided to re-attach my lens as he had done during the previous 2 operations and before I knew it I was being wheeled into the OR again. The first thing I remember after waking up from the surgery is being in tremendous pain. Because of this I was kept in the recovery room far longer than I’ve ever needed to be and the nurses would return every five minutes to administer more pain meds in order to try to get the pain under control – which was largely unsuccessful. After about an hour of this they wheeled me back to the holding area. I remember laying curled up in a ball on the bed clenching my fists and waiting for them to bring my mom in – sometimes a girl just needs her mom.

It was a long time before they finally brought her in and they still hadn’t been able to get my pain under control, though not for lack of trying. I was told that my eye had hemorrhaged again and that there was severe inflammation – just like last time. The pain was really intense and on top of that it’s not uncommon for people in my family – especially my mom and I – to not respond very well to pain medications (or local anesthetics) to begin with. Eventually I just started vomiting uncontrollably from all of the pain medications, the violence of which did not feel good on my eye. Eventually, because nothing they did was helping much and all I wanted to do was go back to the hotel and curl up in bed, the nurses agreed to let me go home. All in all I was in the hospital for 9 hours after this surgery, instead of the usually 2.

The recovery for this surgery was by far the longest I had ever experienced. It took well over 6 months before my vision had healed to the full extent that it would and the pain had largely and finally subsided. Sadly, my vision never recovered to what it had been before the surgery. While I thankfully don’t have floppy iris or double vision in my right eye, my visual acuity as a whole was largely reduced and my distance eye can no longer see distances very well at all. It’s been hard to get used to and it’s been a very long and frustrating journey.

I miss things the way they were and it’s been hard to adjust to not seeing the world as well as I had all those years. But, as hard as it’s been to cope with these changes, it’s these experiences that have also renewed in me a feeling of appreciation and gratefulness for the vision that I do have. I’ve been reminded that nothing is guaranteed, and that’s something I’ll always hold on to.

Marfact #23 and 24 + My lens journey: Part 3

In honor of Marfan Syndrome awareness month, here are Marfacts 23 and 24 (provided by the wonderful Marfan Foundation
and Maya over at Marfmom respectively).

Marfact #23: Related conditions that have signs and treatments that somewhat overlap with Marfan syndrome include Loeys‐Dietz syndrome, Ehlers‐Danlos syndrome, Beals syndrome and MASS phenotype. The differences are critical so it’s important to get the right diagnosis.

Marfact #24: Do you know the signs of a pneumothorax and how to treat it? A pneumothorax is “a collection of air or gas in the space between the lungs and the chest that “collapses” the lung and prevents it from inflating completely.” It’s an emergency situation, although usually not life-threatening. http://marfan.org/marfan/2444/Lung-Emergencies/

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My lens journey part 3:

Part 2

The day after surgery I woke up in a tremendous amount of pain. It hurt pretty severely to look anywhere, but if one eye moves so does the other, so it was almost entirely unavoidable. When we got to the eye doctor’s for my post surgery checkup (with a surgeon’s helper, not my actual surgeon) I was very agitated in a way that I never am. But I had done this 5 times before and I knew the drill: they were going to take off my patch and hold open my eyelid, shine a light in it, put drops in it – all things that I usually wouldn’t bat an eyelash at. But, this time I was in so much pain that the thought made my stomach turn – and it turned out to be for good reason. The pain I felt just from the weight of that tiny drop hitting my eye was enough to make me tear up and cry out.

Something was definitely wrong. Never had I ever experienced even a fraction of this much pain after a surgery. At this point I’d been living with a lot of pain every single day for the past 3 years and I was pretty good at handling it, but this pain was frightening in it’s severity. Lucky for me that I didn’t know how much worse things were about to get or I wouldn’t have been able to find the courage to carry myself towards them.

To find out what was wrong I would need an ultrasound on my eye. My eye that was still mushy and flat from the previous day’s surgerywhere there were stitches sticking out everywhere and fresh wounds, and where the weight of a tiny drop was followed by a shocking amount of pain. I thought to myself there’s no way. But yes, that’s exactly what they were going to do.

As they explained the procedure I wanted so badly to run as far away from there as I could. But what do you do? If I didn’t get the ultrasound they wouldn’t be able to figure out what was wrong, and then what? I couldn’t risk losing my vision because I was scared. It had to be done, which I guess is what made it so doable. That being said, the 20 minutes it took seemed to drag on for hours. My fingers hurt from gripping the chair arms so hard. I stayed silent with my jaw locked tight and kept every muscle in my body tensed to the point of exhaustion – I couldn’t help it. Up until that point I had never been in that much pain in my entire life. You know that horrible, intense shooting pain that you get in your eyes sometimes during brain freeze? The pain was a lot like that, only worse and for 20 minutes straight.

But, what the ultrasound revealed was that my eye had hemorrhaged and was severely inflamed. This explained the amount of pain that I was in and if left untreated could have severely and irreversibly damaged my eye. I was immediately put on a high dose of steroid drops four times a day (more misery), along with steroid tablets to help the healing and zantac to protect my stomach from the steroids themselves. I was also on a few other drops that I always take after surgery starting four times a day everyday.

After all was said and done I spent the rest of the day a bit traumatized by everything, but I was also impressed with myself for handling it – not that I had much of a choice. It’s pretty amazing though: just how much pain we can endure when there’s no other options. Things were still a bit difficult from there on out. Because of the complications my eye was taking a much longer time to heal than it ever had before. I was impatient with the slow progress – I wanted to know how my vision would turn out this time around and if it would be like it had been before the past two surgeries.

I can say now that I wish things had turned out differently. After all of the pain and worry; the drops, the traveling, the money; this surgery turned out worse than the last one. While I didn’t have the floppy iris anymore, I now had severe double vision and the acuity of my vision itself had decreased considerably. I could no longer read nearly as well as I had been able to or see close up things a fraction of how I had (my left eye is my nearsighted eye). It was all so frustrating. I couldn’t understand why everything had gone wrong when I used to breeze through the same surgeries like they were nothing. I was also inescapably terrified that my right lens would soon dislocate too and that I’d have to go through it all over again.

Coma

This is an example of how the double vision in my left eye looks (the center and far right images) compared to my previously normal vision (far left).

Part 4

Marfact #21 and 22 + My lens journey: Part 2

In honor of Marfan Syndrome awareness month, here’s today’s Marfact (provided by the wonderful Marfan Foundation).

Marfact #21: People with Marfan syndrome are at an up to 250 times greater risk of aortic dissection than the general population.

Marfact #22: Marfan syndrome can affect many parts of the body, but has “variable expression,” so each person is affected differently, even in the same family. While there are features that are frequently seen in many people with the disorder (such as tall, thin stature, disproportionately long arms and legs), not all people exhibit these features.

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I’m actually going to be splitting this into 4 parts because of how long it actually is once I sit down to write it.

Part 1

About a week after my 16th birthday my life changed – and I will never mean this more literally – in the blink of an eye. Meaning that I blinked and all of a sudden the vision in my left eye was entirely blurry. I knew what had happened immediately: my vision was identical to when I had no lenses at all and would take off my glasses. My implant had dislocated and I was devastated. At the time we had been told that should anything ever happen to my lens implants that they would likely have to be removed and that would be that.

My eye surgeon wasn’t available for a week, and because of the risk of my lens implant lodging in my pupil and causing serious problems I had to spend that entire week sleeping practically upright to keep it from doing so. This coupled with thinking that I had just lost one of the most precious things I had, made it a pretty long and melancholy 7 days. To my enormous relief though, my eye surgeon decided to re-attach my lens instead of removing it. I wish that had been the end of it, but it wasn’t.

My eye healed quickly and very minimally painful as they always have, but once my vision began to come back I noticed that every time I moved my eye everything in my field of vision would bounce. Mom and I left Vancouver and made the 8 hour trip home hoping that as my eye continued to heal this would go away, but it didn’t. I went to my local optometrist for a post operative check and was told that the cause of the bouncy vision was “Floppy Iris Syndrome”. As far as he could tell my lens implant was reattached further back this time to help keep it from rubbing on my iris as it had before, but now it was too far back and not supporting my iris at all, causing it to “flop”.

6th Eye Surgery

My eyes a month and a half after surgery #5 and a day before surgery #6.

So, about a month and a half after surgery #5 – my eye red and still not fully healed – we headed back to Vancouver for another operation. This time it was decided that he would replace my lens entirely with a new one (a bigger and riskier surgery) and for the first time ever before an operation I felt dread. I was hoping that because he would be replacing my lens this time that things would be better but when I woke up my mom told me that it had been decided during surgery that my old lens would be reattached instead of replaced as it had last time. Aside from that, right away things felt different this time when I woke up than it ever had after the previous surgeries. And though it wasn’t unmanageable, my pain level was a lot higher than it had ever been following eye surgery.

It was the day after though, that I ended up going through one of the hardest things I’ve ever been through.

Marfact #20 + My lens journey: Part 1

In honor of Marfan Syndrome awareness month, here’s today’s Marfact (provided by the wonderful Marfan Foundation).

Marfact #20: Eye problems associated with Marfan Syndrome include early nearsightedness, early glaucoma, lens dislocation and retinal detachment. Treating eye problems early is key to maintaining quality of life.

Visit www.marfan.org for more information.

*I’m splitting this post into two parts because it’s a bit of a long story.

Lens dislocation and the complications that come with it is something that I’ve dealt with in some capacity my entire life. I was 4 years old when I was diagnosed with ectopia lentis and not long after that I was undergoing the first two of seven eye surgeries because of it. These early surgeries were done in order to remove the lenses and fit me with highly magnified bifocal glasses. I hated those glasses with a passion for how they made me look, but at the same time I was actually seeing the world for the first time in my entire life.

When I was 10 years old my dream of ditching the glasses and being able to see out of my own eyes, without any vision aids, finally came true when it was decided that I was a good candidate for lens implants. And so, it was with surgeries number 3 and 4 that I received one of the most amazing gifts I could have ever imagined. Things were relatively calm for the first few years afterwards but then I gradually began getting unexplained bouts of Iritis and Uveitis that would cloud up my vision, as if I was looking through a frosted window. These episodes were gradually becoming more frequent and severe, until one day I woke up without being able to see anything out of one eye and subsequently landing in the ER, wondering if I had gone permanently blind in that eye.

It turned out that my lenses were rubbing on my irises, causing tiny pieces of them to flake off. This would then clog my pupils, disallowing any fluid to escape and causing the pressure within my eye to skyrocket. To remedy this without trying to re-position my lenses my ophthalmologist decided to use a YAG laser to make a small hole in each of my eyes, underneath my upper eyelid (too small to see) so that if my pupil did become blocked there would still be a way for the pressure within my eye to escape. And, although the procedure itself was really unpleasant because my eyes wouldn’t freeze properly and I could feel the holes being burned, it ended up working really well and afterwards the episodes dissipated entirely.

Part 2

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Marfacts: 18 + 19

Marfan Awareness

This story tragically highlights why it’s so important to raise awareness about Marfan Syndrome: so no other families have to suffer this same devastating loss, and so no more lives are cut short needlessly. I dream of a day when there are no more stories like this one. My heart goes out to Stevie’s family and friends, as well as all of the other families who’ve lost a loved one this way.

In honor of Marfan Syndrome awareness month, here’s yesterday and today’s Marfacts (provided by the wonderful Marfan Foundation and Marfmom).

Marfact #18: While there is no cure for Marfan syndrome, continuing advances in medical care and research are helping people with the disorder live longer and enjoy a better quality of life.

Marfact #19 (courtesy of the awesome Maya over at Marfmom): Consistent monitoring of the heart and aorta is vital for patients with Marfan syndrome. This leads to a greater likelihood of preventative surgery, which has better outcomes than emergency surgery after a dissection (tear of the aorta) has taken place. Most people with Marfan are on some sort of medication to keep their blood pressure very low in order to slow the growth of the aorta. Typically this is a beta blocker, but ARBs like losartan and irbersartan are currently being researched as well.

If you suspect that you or a family member may have Marfan Syndrome or a similar connective tissue disorder, please get evaluated by a medical professional (who is familiar with these disorders). Visit www.marfan.org for more information.

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Marfact #17 and if I had known earlier.

In honor of Marfan Syndrome awareness month, here’s today’s Marfact (provided by the wonderful Marfan Foundation).

Marfact #17: People with Marfan syndrome should not play competitive or contact sports because of the effect on the fragile aorta, as well as the pressure they put on the fragile bones and joints

Visit www.marfan.org for more information.

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This is something that would have been different for me growing up, if I had gotten the diagnosis of Marfan Syndrome earlier in life. I loved sports in school and participated in any school sports team that I could. I also played a lot of games of dodge-ball that probably should have been avoided for someone with my tissue disorder.

Looking back on it, even without the diagnosis of Marfan Syndrome, my history of lens dislocation and implants should’ve taken things like dodgeball out of my physical education curriculum. But, even the eye surgeon who did all of my previous surgeries never suggested that I avoid sports, contact or otherwise in the long run.

I can remember getting hit in the head more than once with a dodge-ball or volleyball in school, and on one particularly rough occasion that it precipitated one of my earliest instances of Iritis. I think it was either later that night or the next day that the vision in one of my eyes began to cloud over and fill with thousands of tiny floating dots.

Obvious risks aside, because of how much I loved sports in school and how much they meant to me, I can’t help but be glad that I was able to participate in them growing up. Don’t get me wrong, if I had known the risks I wouldn’t have done it and I’m extremely thankful that nothing too bad came of it, but it was still nice while it lasted.

I do often wonder though, if those instances and injuries may have contributed to my lens implants dislocating later in life. Or even some of the particularly weak tissue I have in certain joints. It was right around when I started joining the school’s basketball and volleyball teams that my first tissue injury and chronic pain started (a painful ganglion in my left foot at 10). But I guess I’ll never know for certain how things would’ve or could’ve turned out under different circumstances.