Eye and heart updates (again).

With everything that has been going on with my optic discs and brain lately, I never took the opportunity to write about the outcome of my cardiologist and ophthalmologist appointments from last March.

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My cardiologist appointment didn’t go as well as we’d hoped; my aorta had grown a bit since my last visit, which is never the greatest thing to hear, but it was also still quite stable overall. To get a better idea of how quickly it may or may not be progressing—and to maybe start me on treatment with Losartan if necessary—the cardiologist wanted to see me again in 6 months for an MRI. And, the MRI results showed that in those 6 months my aorta hadn’t grown any further, so for the time being it’s still safe to leave it be. I’m due for my next checkup in about 2 months, so we’ll see how things are looking now. I’m quite relieved at the results, and as far as I know my aorta is doing pretty well for someone my age with MFS, so, no complaints there.

As I’ve mentioned before on here (in “It’s hard to go through it again.” and “We always carry on, and sometimes it gets easier.“), the lens in my right eye suddenly shifted early one morning as I was getting out of bed. This was the third time that I’d experienced something like this with my lenses, so I was pretty certain I knew what was going on. It was somewhat devastating at the time because both times this had happened before, I would end up needing surgery soon after, and my vision would never be the same. But, thankfully it was (and is!) still hanging on because of some factors we weren’t aware of.

When we got to my ophthalmologist in Vancouver he told us that my lens was indeed holding on by one suture instead of two, which was why it had shifted and was now relatively (but not completely) loose. What we didn’t know was that during my last lens reattachment surgery, only one suture had come loose, so he only had to replace the one. We had always assumed both had been replaced. So now, the new suture is still stable and hanging on, but the original suture from my lens implant surgery—13 years ago—has finally let go. This can help account for why this lone suture has managed to hang on two years longer than last time—it’s relatively new and strong.

The trouble is, with only one suture holding all of that weight—in combination with the weak tissue from my disorder, trying to support it—we were told that it’s not a matter of if it fully dislocates again, but when. Sometimes it’s hard to sit here waiting for the other shoe to drop, not knowing when we’ll have to rush back to Van for yet another eye surgery and all of the difficulties that entails. But, I was already expecting another eye surgery, and having these extra years in between has been a gift, so the news wasn’t too bad.

My ophthalmologist was (and is) a bit concerned about some of the complications that a loose lens can cause regarding inflammation, hemorrhages and retinal swelling, so,— as long as the lens is hanging on—we’ll have to go back to Van every 6 months to check on all of those things. On a positive note, the lens in my right eye has had both of it’s sutures replaced, so it should be much more stable and less likely to subluxate like this one did, which is an incredible relief.

So, those are my—as per usual—long overdue eye and heart updates. I can’t believe it’s been nearly two years since everything with my lens kicked off. I’m so surprised and grateful that it’s still holding on.

Thanks for reading!
– Katie

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 ♥

Better late than never.

Apparently the writer of this blog has fallen off of the planet altogether. Or, at least that’s what it may have seemed like after over 18 months of complete radio silence from me.

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Things can change very quickly when you’re living with a chronic, degenerative disorder, and when they do writing updates about it (on the blog you chose to start specifically for that purpose, mind you) can rapidly evolve into a fairly daunting task. My disorder’s rare and complicated and it can drastically change how my body reacts to even the most routine of medical interventions and procedures, so explaining any new developments in it’s progression can often require that I take many branching, long-winded detours into explaining all of the relevant factors involved. So, inevitably I procrastinate writing the update, all the while my illness continues to throw new complications and side effects at me, adding to that ever-expanding list of what needs updating, and so on.

Fortunately for me, in the grand scheme of this thing called life the difficulty with which I maintain this blog – that I elected to do in the first place – is about as trivial an issue as it gets. Still, that’s how it might just end up that before you know it it’s been a year and a half since your last update, instead of the week or two that you had planned on making it to begin with. Good job Katie.

The other reason that I found it difficult to commit to writing was the severity of the effects that my disorder was (and  often still is) having on me over this past year. This was both because it’s difficult to think and write when you’re very ill, but also because this was a dark and frightening time for me and I’d been stubbornly hung up on wanting to write some posts that were lighter in tone after the more serious ones that I’d been churning out since my eye started having problems again. I worried that people may not want to check in anymore if the tone of the blog seemed constantly dark and dire (apparently I figured people would prefer to check in and read absolutely nothing, instead). But, avoiding the worst of my disorder goes against one of the core things that I’d set out to do with this site, which was to write about the reality of my illness. Not how I wish it was, or how I want others to think it is, but how it actually is, even when that means that the tone of my writing will at times be dark and depressing.

In all honesty though, I do apologize for disappearing without so much as a word for such a long time. Luckily for me, the most serious consequences of that would likely have consisted of a simple thought here or there about where the Tissue Tales girl may have gone off to, or whether she’ll ever post again. That being said, I received a number of really kind and much appreciated emails from some of you, who were just checking in or saying hi, and I thank you very much for that. It’s nice to be thought of once in a while and the support that writing here has afforded me from and through all of you guys has been incredible and deeply cherished. There is such a sense of understanding and caring from the individuals and groups that I have been fortunate enough to have been introduced to through this blog, and I do miss it immensely when I’m struggling or away for very long.

So much has gone on since my last proper update that I’ve been wanting to vent about and share with you; starting with what has essentially been the catalyst of everything that’s gone on in the last year – my stay in the hospital – and the complications that lead to it in the first place. That’s the first and lengthiest thing that my update needs to cover, so from here on out I’ll be working on posting that, and afterwards some of the other things that have gone on.

Once I’ve more or less filled in all of the gaps of the past 18 or so months then hopefully I can get back into the routine of blogging regularly again. I have desperately missed this, and all of you, that’s for sure. For those of you that are still here after all this time: thank you so much for sticking around, I really appreciate your patience and continued support. You’re fantastic, as always.

– Katie

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.

I’m Still Here

By Lukasz Szmigiel
It’s been a long time, hasn’t it? I never thought that I’d end up needing, or choosing, to step away from this blog for so long. I had come to rely on it so heavily to help me cope with the various aspects of living with a chronic illness, and it’s been so incredible to be able to vent on a platform that gives me access to so many wonderful, supportive and understanding people. I really have missed that, and all of you so very much.

I never intended for my hiatus to be so long, but the longer I stayed away the harder I found it to come back. So many difficult things have happened since I last wrote and I haven’t known where to start. While some of those difficult things have been to do with my illness, most of it involved my personal life. I’m so used to being able to honestly and openly talk about all of the difficult, painful things that my illness results in on this blog. But, when the hardest things that I was facing became within my personal life – where there’s the need to protect and respect the privacy of everyone else involved – I found it hard to work up the desire to post. That was what I wanted and needed to vent about the most, so instead of having to pretend that those things weren’t happening I ended up just preferring to stay silent entirely.

I do apologize and regret not letting everyone know where I’d been for so long and that I was alright – not that I’d expect people to be sitting there consumed by it, of course. But nonetheless, I think I owed it to you to have explained my absence after all of your continued support over the years. To those of you who sent me emails and messages asking if everything was alright, thank you so much. You never picture your absent having any impact or even being noticed, so, receiving these messages was really touching and appreciated.

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It will take me a few posts to explain a bit of what’s happened (medically) in the past 6 months, but I do intend to catch you all up very soon. But, first off, I was asked by the American Recall Center to participate in their November Awareness “Talk About Your Medicines” campaign, by writing a bit about my personal experiences with various medications. I’m really honored that they asked me to be involved and this is a really good thing for me to write about having been tried on so many different things since being diagnosed with my illness. That post will be published tomorrow. I invite any of you who’d like to participate to either leave a comment about your experiences – good or bad – or, if you’d like to guest post: email me at tissue.tales@hotmail.com.

I can hardly believe so much time has passed; the last 6 months seemed to have somehow gone by almost in the blink of an eye. Maybe it’s because so much has been going on. I’m really looking forward to picking up where I left off 6 months ago and I sincerely thank you all for sticking with me during the hiatus. You are, without fail, what makes this blog such a joy to keep.

'Til the Cows Come Home By Kenneth Thewissen

Much love to you all.

– Katie

There are days.

Broken Flower

There are days in life that have the potential to permanently alter the course of your future for the better; days that could make some of the things that you live with easier from that point onward; days with the ability to get the ball rolling towards some positive, long overdue change. Yes, there are days like that.

But sometimes those days instead end only with missed opportunity; another lengthy appointment with disappointment, and another added mark on your overflowing wall of let downs and discouragements. Those days that, instead of giving you a piece of your life back – no matter how small – end with you being left to go back and suffer endlessly, in the same painful reality, once again feeling defeated and abandoned by the only people with the power to truly make a difference in your life.

The 8 long months leading up to that day passed me by in slow succession: cloaked in a bleak and melancholy air that hung heavy over and all around me. The weeks were almost entirely marked only by the increasingly debilitating and all-consuming pain in my lower spine that brought about the need for that day in the first place. Things were consistently deteriorating right before my eyes, and I was frighteningly often struggling desperately just to pull myself out of bed each day.

While time passed and I waited, I forced my mind not to linger too long or too often on the possibilities of that coming day, which sadly – experience has taught me, over and over – would most likely end in the exact disappointment, frustration and – should I allow it – devastation, that it did. Or, worse than letting my mind focus on the fear of things ending badly, I couldn’t let the hope in. If you have hope it only hurts so much worse when that hope is shattered. I couldn’t take the crippling pain of getting your hopes up and then having them destroyed, on top of everything else.

But, I did get my hopes up a bit – I couldn’t help it, it’s all that I had to prop me up. And so, as hard as I tried not to let it, some hope did manage to find it’s way in. But, I think that was what had carried me these last months through all of the pain: that little bit of hope that maybe someone was about to help find a way to lessen it for the first time, and that maybe this person would finally be the one. My fears about hope ended up being perfectly reasonable in the end – despite not having all that much of it to begin with, it was still a devastating thing to have and then lose.

I’m back at home now, back to my reality, and that day has gone and passed. The pain continues on in the same ever-worsening way that it has for the past 10 years – exactly one half – of my life, while my body continues to fall apart in a constant string of new and unexpected ways. And that day is ever further behind me, without having given me anything to help carry me forward. And now? Sometimes, I don’t know how I’m going to be able to. But yet, somehow I always do.

Sorry it’s been so long again, things have been tough.
Lots of love to you all. ♥

-Katie

A guest post by Destiny

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“My name is Destiny, and I am 20 years old. I love Panda’s. I’m a pretty plain girl…I love the colors black and white! Oh yeah, I’m also dying. Yep, I’m 20, and I am dying. How wonderful, right?”

I was introduced to Destiny’s story and Facebook page a few months ago by a fellow EDS zebra. Destiny is the same age as me and has Ehlers-Danlos Syndrome Type 4 which is the vascular type. This is considered one of the most severe forms of EDS due to the extreme fragility of the blood vessels and organs that it involves, which can lead to the rupture and tearing of many of the body’s major organs and blood vessels. The first thing that I learned about Destiny was that she’s incredibly strong and brave. The second – which you’re about to see for yourself – was that she’s an amazing writer with a gift for helping her readers see things through her eyes. The above paragraph that she wrote, in my opinion, sums up the kind of person she is; despite everything she’s lived and is living through – which is more than anyone should ever have to endure – she’s kind, cheery, funny and optimistic. She’s stoic beyond belief and there’s such a warm bright light within her no matter what she faces. And, she was sweet enough to let me use the following post as a guest post on Tissue Tales.

Enter Destiny:

This day started out just like any other day. She woke up with only 20 minutes to spare, her alarm still ringing in her ears. She gently and quietly climbs down her bed, so as not to wake her slumbering roommates. She goes to the mirror and brushes her hair back into a ponytail when the world spins and tries to go black. Her cold, white hands cling to the futon as her heart pounded and the blood rushed everywhere but her head. The sparkles in front of her eyes began to lessen and the girl, with a face as pale as the moon pops her morning pills, downs a salt packet, and fills her cup with orange pedialite; flashing back to her days as a child, grabs her backpack, stands – slowly this time – and heads off to her first class of the morning. Her hands still shaking and her heart still racing.

She sits in her class with her heavy textbook on the table way in the back row. Listening to all the students talking about the parties of the night before, her mottled hands begin to warm up and she sips some more pedialite. The shaking begins to subside and she prepares herself for the next 2 hours. Her hands begin cramping an hour into the lecture as each second is another second of her body attacking itself. She puts her purple pencil down on her crisp white notebook for a break when suddenly the nausea hits. Deep inside her belly a heat hits her and it travels up to her head, which in turn fills with a pressure words cannot begin to describe. She grasps her desk tightly and tries to take a deep breath only to find her chest was too tight, she couldn’t breathe.

Her frantic green eyes searched around her for what to do. She noticed people were staring and their lips were moving but she couldn’t hear them. A hand on her leg, a feeling, someone can see something is wrong, she reaches for that hand, anything to keep her in the here and now, desperately trying to convey what is happening as she is a prisoner in her own body. She tries to move, and her dry, blue tinged lips try to form words when the pain rips through her chest and the world goes white.

Fast forward a few days later and the girl lies in a hospital bed with an icepack across her chest from a fractured sternum. The CPR done to save her life fractured her porcelain bones. Tears pour from her pale green eyes as she replays the words in her head again and again.”…too sick…medical leave….close call…can’t risk it…focus on yourself and family…you need to withdraw…”. On the bedside table lies a copy of the medical note from her team of doctors, a copy of a form she signed officially withdrawing her from college. On the bedside table lies a copy of all her hopes and dreams completely crushed by a disease no one has ever heard of.

This isn’t my best writing…but it is pieces from my last week in college before I was forced to withdraw due to my medical problems. Looking back, I agree it was the right decision, and I have accepted VEDS won that battle, but it still breaks my heart in half. School was an escape for me…even in Elementary. It was a place I could focus on something besides my disease and my pain. It was a place to express myself and learn who I was. In High school I was in so many activities, the honor roll, and was even teaching a class come senior year. School was my sanctuary…College became a more hectic, scary sanctuary. One that opened my world to limitless opportunities, all of which I wanted at one time…I loved the friends I made, the classes I took and all that I learned…and the main thing being to never give up.

My hopes and dreams are still on that bedside table. The travelling abroad, the tutor program, the German club, community choir, becoming a teacher or a psychologist, specializing in Autism…all of those are still on that table and never will I be able to pick those up, dust them off and hop back in….But with that loss comes growth.

My new dream: to raise awareness for rare diseases. To raise money for research. To find a doctor willing to do research on such a little known condition. To share my story and inspire others. To have my story reach as many people as possible…and eventually I’d like my dream to come true, true to the point that my slogan will no longer be needed. That “Awareness for a Cure” will not need to exist because people will have heard of Ehlers-Danlos type 1,2,3,4… KLS, Dysautonomia and the other rare diseases.

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If you’re as inspired as I am by Destiny and you’d like to let her know you can find her Facebook page here.

Thanks for sharing your story with us Destiny!

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