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

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

We always carry on, and sometimes it gets easier.

First of all, I want to thank each and every one of you for all of your support. It seems so strange to think about how many people took the time to read it or share it on Facebook – I’ve never had that happen with such a personal post before and I really appreciate the response.

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Things have been easier since my last post. Of course they aren’t perfectly okay, but I’ve been coping really, really well. I feel much more hopeful about things than I have since everything happened. We’ve been waiting to hear back from my regular surgeon for the past two days but were told to call him today if he hadn’t gotten back to us yet. I’m really hoping that he’s taking the time to read thoroughly through my novel-sized records of eye history so that he can figure out what the best course of action is. Besides all of the surgeries themselves there’s so many incidents here and there throughout the past 10 years or so that could have an impact on how things need to be handled. I’m a bit nervous about what he has to say, but we’re basically waiting to find out whether or not we need to head to Vancouver to see him now, or if we could maybe just wait until it fully lets go.

Both of the above options have their pros and cons. While I want nothing more than to just wait and watch it for now, it’s a lot harder when it becomes an emergency and everything now needs to be done immediately, instead of in a way where things can be planned out – especially since we are 8 hours away and can’t drive there ourselves. I have to go to Vancouver for my cardiology appointments in March and it would be best if somehow the lens managed to hang on until then. To be honest, what I really hope is that it’ll just hang on like this forever. I know that’s very unlikely, especially with my history, but it’s still not impossible, and it’s the only thing that I’m able to grasp at right now.

Under normal circumstances the new visual change would be really disheartening, but I can honestly say that I could live like this for the rest of my life – and to be honest, I’d be incredibly grateful to do so at this point. My brain is adjusting to the vision change surprisingly well – I realized the night before last that I had been able to spend the entire day without using my eye patch. And I did so again yesterday. I’ve even been drawing sometimes, which makes me happier than I could ever say. The thought of losing that incredibly important piece of my life terrifies me more than almost anything, and to have it right now, when I wasn’t sure that I would, has meant a lot.

It also puts my mind at ease to know that my usual ophthalmologist is at his practice and in the country right now, because he’s often gone for weeks or more and we weren’t sure if we’d be dealing with him or someone we’ve never met. I do worry that he’s going to be leaving again soon and that he’ll want us to go down there to have things looked at regardless of whether or not he’ll be doing surgery at this point. I can understand why he would want to do that though, if he knows that he’ll be out of the country soon I imagine that he would want to check on things himself. Let’s just hope that’s not going to be a problem. It would be so, so much easier if we lived closer. I know it’s not the end of the world, but the travel and being away from home is definitely hard financially, emotionally and physically, on my mom and I both.

My family doctor phoned me today, wondering what was going on with my eye and wanting us to let them know as soon as we’ve talked to the specialist. It hadn’t even crossed my mind to phone and let her know what was going on and I was surprised to hear from her. Usually when anything happens with my eyes my other family doctors haven’t been involved at all. But, she told me to let them know immediately if I needed to make an appointment with them and that they’d fit me in whenever. I’m glad that they phoned, it’ll be good to be able to talk through everything with her and let her know a bit about what we might be looking at. Often when you hear that someone might need eye surgery you assume that it’s no big deal, and that is the way the first 5 were for me too. The pain was really minimal, to the point that I just remember the stitches being a little sore, and I healed unbelievably quickly. But the last two operations were riddled with complication after complication, and more pain than all of the other ones put together. I took 6 months to heal and my vision was never the same after. All in all, I really appreciate her support and interest in what’s going on, it makes me feel a lot more secure with everything.

That’s about all that I have to update everyone on for now. Again, I want to thank all of you for your incredible support and kind words. It makes such a difference in my life and makes me feel so much less alone. I know that I’m unbelievably lucky for it and to also have such a caring supportive mom by my side.

Big hugs to you all. xx
– Katie

Talk About Your Medicines

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As I mentioned in my last post, I was asked by the American Recall Center to participate in their November medication-awareness campaign. For their own awareness article they’ve written specifically about the oral anticoagulant Xarelto and some of the dangerous side effects that have been recently linked to it. To read that article click here.

As someone with an incurable illness, medication – like it or not – is, and always will be, part of my everyday life. It goes hand in hand with the quality of my life and has the power to hugely impact how I feel from one minute to the next. While I definitely wish that there wasn’t a need to be on it all of the time, there is, and that’s the reality of being chronically ill. So, here’s a bit about my personal experiences with medications.

Before I had even gotten the correct diagnosis (at about 15), I had been tried on so many more medications than I can accurately remember now. And, as is the way it often goes, virtually every one of those was ineffective or unpleasant. Some ranged from simply not working for what it was prescribed for, to being debilitating or landing me in the ER. Most of the medications I’ve been tried on thus-far have been to counteract two of the most difficult aspects of my illness: insomnia to an often extreme degree, and chronic pain. Sadly, these are two things that are very complex to treat. There’s not one simply safe, fix-all pill for either.

As with most medications, you have to weigh the benefit against the risks and decide whether it’s worth it or not. You also need a doctor who’s willing to help you find what works best for you in your unique situation. I’ve always been really disciplined with medications for both sleep and pain. I’ve always made sure to only ever use them when absolutely necessary and have kept myself thoroughly educated about the numerous downfalls and dangers of both. When my pain did eventually become crippling enough that it was necessary to have round-the-clock pain medication in order to cope with it, I found it really hard to come to terms with. I never wanted to be on that kind of medication for the rest of my life. Having to take that step made me feel weak, even though in reality by normal pain standards, my levels of pain were still at a fairly extreme level all of the time. Aside from the physical consequences there’s also a great deal of stigma and ignorance surrounding pain medication, even when it’s being used appropriately and solely for pain.

My opiate medication’s side effects are quite limited overall compared to the other classes of medications I’ve been on, but it’s still not without it’s pitfalls. For instance, when used long-term your body’s natural pain-killers forget how to do their job properly. Your body also eventually adjusts to the medication and starts to need more of it in order for it to keep working on your pain. This is called “tolerance” and is an unavoidable side effect of opiate medications when needed for an extended amount of time. Opiates can also cause nasty “rebound” headaches in many people when used for more than a few days in a row. And while it’s been proven that people who suffer from chronic pain are generally much less likely to become addicted to pain medications than others (for various physiological and psychological reasons) it’s still a risk and must be taken seriously. I wrote about all of these things a few years ago in this post.

It’s been just over a year now since I was put on a consistent and constant dose of pain medication, and while it’s made my otherwise crippling pain bearable, it hasn’t made it disappear and it hasn’t been without it’s consequences. I still struggle with my pain to some degree 24/7 and most likely always will – there’s just no simple fix for that. Still, I am extremely grateful that this has been available to me, especially knowing that in many countries and situations it wouldn’t be. I can’t even imagine being able to handle that kind of pain at all never mind being able to find any enjoyment in life with it.

Sleep medication has always been difficult as well. There’s a viscous circle that having both insomnia and chronic pain results in. I often can’t sleep because of my pain levels, which keeps my body from getting any rest or break, which in turn causes my pain levels to increase tremendously, again making sleep impossible, and so on. The medications I do use on occasion to help me fall or stay asleep often won’t work during bouts of my more extreme insomnia. They’ll often make me feel more tired, but still won’t allow me to sleep, which is horrible. Other times they’re a life-saver, allowing me to get some desperately needed rest.

I’ve been suffering from chronic headaches since I was a child, so many of the earlier medications that I was tried on (and there were many) were for this. These medications in particular were often horrible with their side effects. One of the worst side effect profiles I’ve ever seen, never mind only counting things that I’ve been on, was for a medication call Topomax or Topiramate. Topamax is primarily an anticonvulsant used in the treatment of epilepsy, but it’s been shown to improve migraines in some people. That’s why I was put on it about 3 or 4 years ago. I wasn’t made even remotely aware of the dangers of this medication and at the time had just assumed it was safe, otherwise my doctor wouldn’t risk putting me on it. I know that all medications have the potential for serious side effects, but despite the multitude of medications I’d been tried on up until this point, I had never even come close to experiencing effects as severe as the ones I had while on this.

Looking at the incredibly long list of serious and often permanently disabling or life-altering effects this medication can have, I can’t help but be angry that as a 16 year old I was put on it, without even a warning. At first, though it wasn’t helping my headaches things were generally going fine, but the longer I was on it the more symptoms I started experiencing and the more progressively severe they became. I had no idea that they were from the medication and because they were all neurological in nature I was terrified as to what was going on and why. The first thing that prompted me to start doing some research were eye tracers. I had assumed that because of my history with eye problems that it was just something else that was going wrong with them. While doing research on this symptom I was stunned to see the name of my medication come up as a common denominator, mentioned by about a dozen other people describing the exact same thing.

Other frightening side effects that I experienced while on this medication included nerve pain: I started getting these unpleasant electric shock sensations all over my body. Later I noticed this strong and unsettling pulse-like sensation in my stomach just above my belly button, which my doctor simply wrote off as anxiety. By far the scariest thing that I experienced was this sudden and extremely uncomfortable sensation throughout my body. For a moment it was like I couldn’t see or hear properly and then all of the sudden my legs went weak and I couldn’t think. I couldn’t even remember what I’d just been doing. The entire episode was brief but none-the-less terrifying. At the time I was sure that I must have just had some sort of mini-stroke. This was the final straw with this medication and without fail (thankfully) every single one of those symptoms disappeared for good once I had been weaned off of it (after doing some research and consulting my doctor).

Because everyone is so different, I do have to mention that overall my body has never tolerated medication very well. I often get the rarest of side effects, without the intended benefit. Because of this there are some medication classes that I can no longer be given at all. Two of which are SSRI’s and SNRI’s, which I had been tried on fairly early after my illness started effecting my day to day life. There are a few different reasons that these medications are prescribed in relation to chronic pain and illness. One is to help with any resulting anxiety or depression the illness may cause and the other is to help minimize the sensitization related effects that chronic pain has on the brain and body. See, when you’re in pain for an extended amount of time your brain begins over-firing pain signals. This is (as it’s been explained to me) largely because our bodies are not adapted to handle pain 24/7 – it’s unnatural – so what results is that our fight and flight response becomes heightened and overactive. So, while antidepressants won’t work on the root cause of your pain, they can sometimes help in calming or halting the amplification of the pain signals being fired out by your brain.

I’m not sure why, but early on when I tried various kinds of these particular medications I was able to tolerate them fairly well. I was never continued on them long-term due to their various moderate side effects, that weren’t worth living with because the medication wasn’t having it’s intended effect anyways. Eventually I went on a break for a year or two after being unable to find any that worked for me and being exhausted by the long process of searching for one year after year. The first medication that I tried when I was ready to start looking again was Cipralex. I had been on it for about two weeks and so far the only side effect of any note that I was experiencing was an upset stomach. But, out of the blue, I ended up with a very rare side effect known as urinary retention – the acute inability to urinate. I didn’t even know that was a thing and because I wasn’t really sure what was going on I just assumed that if I had to go desperately enough then eventually I would be able to – big mistake. I had purposefully been drinking a large amount of fluids thinking that if my bladder was full enough that I would simply and unavoidably have to go. Eventually, after not being able to for 17 hours despite trying for hours on end, it became clear that something was definitely wrong and I wasn’t going to be able to go at all. By then the pain had become so severe and unbearable that my mom had to rush me to the hospital where my bladder was immediately drained with a catheter. The nurse said that the bladder comfortably holds about 500 ml of urine and that by the time I’d gotten to the hospital mine contained 1200 ml. I was also told that I was lucky that no permanent damage had been done and that next time I should come in as soon as I know what’s happening (but they didn’t have to tell me twice!).

The doctor on call told me that it was undoubtedly from the medication and to stop taking it immediately (you’re usually supposed to be weaned off this type of medication for safety reasons). It took a little while for things to go back to normal entirely but luckily I didn’t end up having to go back to the hospital. My family doctor said I was the first person she had ever come across in her 20 years as a doctor that had suffered from that side effect. We assumed that it was just a severe reaction to that particular medication since I had been on several similar kinds before without that effect. Just to be on the safe side, the next time she tried me on that type of medication she decide to use the closely related SNRI class instead on an SSRI. Strangely, – after only two days this time instead of 2 weeks – this medication started having the same effect and I again ended up having to go to the hospital. This time, while my bladder was still working somewhat, it was becoming enough of a problem that I was unable to take any pain medication (they can also increase or even cause urinary retention) without it tipping it past the point of being manageable. Unfortunately, I happened to be in such an extreme amount of pain that day from a crippling migraine that I needed them to either try giving me a different type of pain medication or hooking me up to a catheter so I could safely take my own.

Again, I had to immediately stop taking the medication, but now we knew that for whatever reason I could no longer tolerate these types of pills at all. I don’t know what changed in the time that I stopped taking SSRIs and SNRIs to when I tried them again that made me unable to take them. I’ll probably never really know. I’m lucky that stopping them so suddenly didn’t cause any of the long term harm that it can. It’s frightening to think that just like that I could have ended up with another health problem on top of everything I was already struggling with that had necessitated the medication in the first place. My sharing these particularly negative experiences isn’t intended to make everyone afraid to take medication entirely. Everyone is different and we all react in our own unique ways to things. What may work miracles for one person may do the opposite to the next. It’s very much an often long, trial and error kind of process. I definitely understand how utterly defeating it can feel if you keep trying with no benefits to show for it. All I can really say is: try not to lose hope and keep trying for as long as you can. Keep informed: new things are always changing and being discovered and you never know what may be the answer for you.

One of the intentions I have in sharing these experiences is for people to hopefully realize that education is extremely important in regards to the medications you’re putting in your body. We so easily assume that our doctors know every side effect or interaction for every medication they place us on, when in reality that’s just simply impossible. There’s just far too many different kinds and specific variations in each individual circumstance. I’ve been prescribed medications many times that say specifically not to take them when suffering from some of the health problems that I’ve already been diagnosed with. I’ve also been prescribed medications that aren’t supposed to be taken with something else that I’m already taking. It’s definitely frightening and frustrating at times, but these occurrences are the reason that taking an invested interest in your own health – however you can – puts some of the control back in your hands and can make a huge difference. And while you always have to use the internet wisely and responsibly, we’re incredibly lucky that we live during a time where virtually any information imaginable is available to us with just a few clicks. This doesn’t mean that you should stop communicating with your doctor of course – you should always come to them about what you’ve learned and consult them before you stop taking a medication. Also, don’t hesitate to phone or go to your pharmacist with any questions or concerns that you may have. They are generally very knowledgeable and helpful when answering your questions.

Medications are often a very vital part of living as well as possible with a chronic illness. And while we can’t necessarily control whether or not we need to take it, we can control how much we know about it and what we choose to do with that knowledge.

Is this what you ordered, Sunny? by Aurelio Asiain

Do you need to take medication regularly? What have been your experiences – both good and bad – with it? Have you been able to find something that works well for you?

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.

ARC_Talk_About_Meds_Banner_TissueTales

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

Marfact #14 and MFS in my family.

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

Marfact #14: About 75% of people with Marfan Syndrome inherited it from a parent; in the other cases, it was the result of a “spontaneous mutation”, meaning that it happened randomly.

My family and I always thought that I had gotten my tissue disorder spontaneously and not by inheriting it from a parent; but, as the years go by I often wonder if I may have inherited it from my mom. Throughout her life – especially into adulthood – she’s suffered from a lot of unexplained health and chronic pain issues and some of them – like her sporadic knee dislocations as a teenager – could very well be connective tissue disorder related problems. Because of this possibility and the serious dangers of unchecked heart issues in tissue disorders she did undergo an echo just to be safe – which thankfully came back fine. But, despite her heart and eyes being okay to date, I will always wonder if she has the same disorder as me.

No one else in my family has symptoms suggestive of a tissue disorder, so in any case it seems to have either started with me or my mom. That’s one reason I wish that my genetic test for Marfan Syndrome had come back positive: so that we could’ve then tested my mom for the same  gene mutation, and maybe finally given her some answers as to why she’s had so many health and pain problems. She’s gone more than long enough without getting any and I’d really like that to change. Not that I’d want her to have my disorder, but she’s dealing with chronic pain and health issues regardless and at least then we’d know why. ♥

Visit www.marfan.org for more information.

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Happy Valentines Day everyone. ♥

Much love to you all and thanks for checking in for another Marfact.

– Katie

Struggling & Taking Notes From an Oak

Note: this is a bit of an old post/journal entry, re-worked to explain how I’ve been feeling lately. I know I’ve been absent and as the title suggests; it’s because I’m struggling. With pain, with sleep, with figuring out my purpose in life.
That being said, I do miss you all dearly and hope to be back regularly soon. Don’t give up on me yet.

Much love to you all. ♥
-Katie

oak

Resilience is an intriguing thing. It can be exceedingly difficult to come by while simultaneously appearing in places – or people – you’d least expend to find it. The way I see it, there are two basic avenues to take when handling tragedy and hardship (though, many facets here and there and in between); you shut down, break down and never truly recover; or, you grieve, accept and move on. I’d like to say that I have always chosen the latter, but in all honesty, I often find myself stuck between the two; fighting to find a balance without falling into nothingness. This often leaves me suspended, neither shutting down or moving on. Sort of just drudging my way through life.

I find myself oddly nostalgic now-a-days, and I say oddly because I always pictured nostalgia and reminiscence to be had many years later in life, when so many things have happened and so much life has been lived. Yet, when so many of my peers are living the brightest days of their lives and finding their places in the world, I instead remain vastly lost and stunted, wrestling with myself daily for the will to keep fighting for a life that has taken me places I never, ever wanted to go. I have no plans in regards to my future, no idea which roads to turn down or even how to get to them. One thing I don’t plan on is feeling this way forever. I hope – in fact it may be the very thing keeping me sane – that eventually the bitterness I feel, the sadness and the hopelessness, will depart and leave me more able to live this life. The physical pain is debilitating in many ways and that’s not going to ever truly go away, but my emotional pain is just as, if not more so, crippling in nature.

It’s odd to be writing about myself in this way. Through my words I could come to recognize myself as some angry, bitter, miserable thing to be around but in truth that’s not what I’m like on the outside and not hardly entirely how I feel on the inside. I love, I laugh, I feel the joy when it’s there to feel – at least most of the time. And my internalization of my feelings – if you can even call it that once it’s been written and made public – is what allows me to do so. I can keep the bad to myself for the most part, while still being me to the best extent that I can manage.

You’d think that keeping so much to yourself would cause you to burst, and there may yet come a time that I can no longer keep these things in my head. And every once in a while the flood gates do begin to part and I’m no longer able to carry on as if nothing’s wrong. These are the rare occasions that I lose it. It being my reserve, and have a bit of a nervous breakdown. It always seems to happen the same way; a negative event will trigger it and once the tears begin flowing there’s no going back. I turn into this shattered, weak, shell of myself. Broken beyond what I feel is even remotely repairable. There’s no going back are the whispers of the thoughts that manage their way in through the grief, I can’t do this anymore. But, such is the intrigue of coping; once I’m able to pull myself at least adequately back together, things seem to hour-by-hour or day-by-day transform back to the way they have been, and I carry on.

“The strongest oak of the forest is not the one that is protected from the storm and hidden from the sun. It’s the one that stands in the open where it is compelled to struggle for its existence against the winds and rains and the scorching sun.”
– Napoleon Hill

What did I get myself into?

Well, I finally got my procrastinating bum in gear and dealt with an enormous amount of things that needed to be done when I seen my doctor. And, as I was dreading, I walked away with an insane amount of tests and appointments.

To start with I need an ultrasound on my kidneys, and also another one specifically on my gallbladder to check on my polyps. I need to go see a urologist out of town for bladder retention problems and find out whether or not there could be something causing it other than my spine. I also need to go back to Vancouver – yet again – to see the orthopedist that we had to cancel on earlier this year when my rib subluxated (we didn’t think I’d be able to handle the appointment because I could hardly move). While we’re in Vancouver we’ll also be seeing a neurologist about diagnosing (or ruling out) several different brain related complications that can arise in tissue disorders like cervical cranial instability and chiari-malformation.

We also need to talk to the neurologist about getting an upright MRI of my spine to check for dural ectasia and tethered cord (my gp isn’t allowed to refer patients for MRI scans). There are only two upright MRIs in Canada, one is in a private clinic in Kamloops that we can’t afford and the other one is in Vancouver but used only for research purposes. We’re hoping that since my disorder is rare that maybe if we talk to the right people I could qualify as research. While we’re in Vancouver we’re going to go see my ophthalmologist to check on my eyes.

My doctor and I talked a bit about my hip problem and how in her opinion since we already know that it’s subluxating and that’s the cause of the pain that she’d rather not order an x-ray on it. I’ve already had and will need so many imaging tests that she figures it’s best not to expose me to more radiation when it can be avoided. Since there’s nothing we can do for my hip right now but treat the pain I agree with her. I’m already leaps and bounds over the recommended doses of radiation so that does seem like a reasonable decision.

I think – and hope – that I remembered all of it. My stress levels always go up when I know I’m going to be seeing so many new doctors and getting poked at so much. That’s part of what sends me into my spells of avoiding my doctor altogether; denial’s much easier that way lol. I am grateful though, that I’m being looked after medically now more than in the past. It’s nice to have a doctor that willingly recognizes the ridiculous amount of avenues that need to be checked over with a disorder like mine.

On another good note, my doctor’s agreed to work and talk with the ILC Foundation when it comes to my care, as well as pass the contacts for the EDS aware doctors they’ve provided us with to the necessary specialists that I see. Thanks a million Sandy!!

Blocked

My sleep deprived self and I are listening to music and attempting to draw. I say attempting because for some reason lately I’ve been completely unable to do so properly. It’s a problem I’ve never experienced before when it comes to drawing, it seems to stem from an inability to properly process dimensions of drawing that help me make the faces proportionate. No matter how many times I measure and re-measure and how certain I am that everything is in it’s proper place, the next time I look at what I’m doing and measure – yet again – something is always way off. And this is with taking measurements, I can usually just do it by sight. It’s really frustrating! I’m not really sure what’s up.