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.

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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

A new low.

Lightning Tree Silhouette

Hi everyone, I know I’ve been really absent on here as of late. Life has been kind of topsy-turvy at best, so blogging has just been really pushed aside – which is a shame because it’s such an outlet for me and all of you really mean the world to me.

I hadn’t planned on blogging about this because it’s extremely embarrassing and at first glance, not medically related. But I also know with certainty that this wouldn’t have happened if not for my medical problems and further more: the medications that I have to take to help treat them.

Okay, here goes nothing. You know the reusable shopping bags that you can buy now? The ones that you take with you when you go shopping to help lessen the use of plastic bags? Well today I went to the grocery store with one of those – which we always use – so that I could get a couple of things done before I leave town.

The day started off on a bad note because of some family issues and also because once again my bladder has almost completely stopped working. On top of that I didn’t feel quite “there” enough in my thinking, because of medications and pain, to drive to the store myself. So I got my auntie – who’s staying with us – to take me instead.

Anyways, as soon as I got out of the car to go into the store I realized that I had forgotten to bring the item that I had wanted to take back, which was 1 of 2 reasons I went to the store in the first place. So now I’m flustered and annoyed with myself too. I get into the store and decide to look at what they have for cosmetics there while I wait for my auntie to pick up some things that she wanted.

They weren’t on my list but I wanted to look anyways because there are a few cosmetics that I’d like to buy before we leave that weren’t in stock at Walmart when I went the other day. So, I see a few things that I’d like to buy and I put them in the black, reusable shopping bag like a lot of people do, so that I don’t have to hold them while I walk around the store some more.

When my auntie’s ready to go I tell her to go through the check out while I go to the pharmacy and get a medication – that’s the other thing on my list. So, I go get the medication, and pay for it at the pharmacy, and then I’m thinking to myself there, that was everything left on my list, now I can go. I completely forgot about the makeup that I had decided I would buy and put in the black grocery bag.

I walk out of the store and what do you know, a security guard pulls me aside asking to see my grocery bag and receipt. So I calmly hand him the pharmacy bag and receipt thinking okay, that’s fine, I have a receipt, but when he asks for the other grocery bag (the one with the unpaid-for cosmetics!!) that’s when it all comes back to me and I’m absolutely horrified.

Now, I live in a small town where everybody recognizes everybody. I’ve shopped at this store frequently for most of my life, without issue. My best friend worked at this store for years. Which all adds to the unbearable embarrassment of realizing what I just did: accidentally shop lifted from them.

Let me just say right now that I’m not trying to make this out as if it wasn’t my fault or shirk the responsibility for it. It wasn’t intentional, but it’s still my fault. I know that. It was me and it was unbelievably stupid, accidental or not, for a number of reasons. I shouldn’t have been using something opaque to carry items so that I didn’t have the visual reminder that the products were there. I shouldn’t have been grabbing things that weren’t on my list. Maybe I shouldn’t have even left the house in that kind of thinking state, I don’t know. But what I can say with all honesty is that I didn’t mean to do it, it was an accident, and I feel horrible.

I know that a lot of you are probably wondering why I would even write about this on here. And I can definitely understand that. The reason that I am is because this blog is meant to be about my life, and all of the ways, big and small, that my chronic illness affects it. And this is one of them. Pain, medications, not sleeping, they all affect my ability to think, remember, and concentrate in enormous ways. And that’s in combination with all of the normal life stressers that healthy people have too. How could that not mess with brain’s ability to function on point, or properly?

Not to mention, I’m on 5 different medications that list: short term memory loss, fatigue, difficulty concentrating, confusion, and changes in thinking, as side effects. All five. On top of major insomnia and chronic pain that’s been particularly bad the past week and a half. Nobody can tell me that those things don’t have an impact.

In the end – after being taken with my auntie into a back room in the store and spoken to (so humiliating) – I was given a warning and let go. Luckily for me, I’ve never been in trouble before and the police were too busy to want to bother with me beyond that. The security guard had made me pretty frustrated and angry at first. And I don’t mean by doing his job because I understand that he has a job to do and that he has no way of knowing whether it was truly an accident or not.

What made me angry was what I perceived as complete and common ignorance when it comes to chronic pain and the impact that it, along with medications, can have on your thinking, and also on the need for pain medication itself. Here’s an example of what I mean: after I had mentioned it, he asked what kind of medications I take and I told him. He then said something along the lines of “it tells you a lot about a person when they’re on those medications”. I found that incredibly offensive because it sounded like he was insinuating that I was a shady person and a criminal because I need to take opiate pain medication. Which is just such a play on an already horrible stigma that I seem to run into at every turn.

I was also irritated at first because he was acting like there’s no way that someone could possibly forget something like that so I must have done it intentionally. This, even after I explained to him that maybe he’d be right under normal circumstances – which I honestly don’t believe because there’s a lot of things that could be going on in someone’s life that could distract their mind’s from remembering that – but that there’s a lot of things that come into play in my personal situation that change that anyways.

I think he believed me in the end though, and seemed to even feel bad because the longer he talked to us the softer his tone got, to the point that he seemed to be trying to make light conversation, jokes, and even seemed to be trying to cheer me up a little bit in the end.

Things could’ve turned out a lot worse, so I’m incredibly thankful that they didn’t. But I’m so mad and disappointed in myself for screwing up so majorly, and also that that is how bad my thinking is now. I hate it. I’m 20 years old and I think more like a 70 year old with dementia. It makes me sad. I used to be able to take pride in my intelligence, and now it’s the source of one of the most embarrassing things that’s ever happened to me.

I’ve learned a few things though. First, I’m never using one of those bags to carry my stuff around the store in again. From now on I’m only using buggies or those carry cart things, or my hands.

Second, I’m not going to go out to public places when my thinking is that bad anymore because I’m afraid of making mistakes like this in the future. This really sucks because the majority of the time lately my brain is that bad but I never want to accidentally screw up like this again.

And third, it’s really shown me just how downhill my thinking has gone even just in the past few weeks. It was sort of the nudge I needed (though not wanted, that’s for sure) to make a doctor appointment and maybe we can try switching some medications around to see if things improve at all.

Today has been a long and miserable day. I’m exhausted, sad, and extremely embarrassed, not to mention very annoyed and disappointed in myself and my thinking. I still can’t really believe that I did that…how absolutely humiliating. Anyways, you may still be questioning my decision to publish this post, and again, I understand why you would. But, when I started this blog I promised myself that I would write here with brutal honesty about my disorder and the pain that it causes and how those things in turn affect my life: both the good but especially the bad – like this. So, I’m trying to keep that promise in publishing this post. 

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 do.

*I’ll write more about what exactly went on at the appointment in my next post.

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!

Rare Disease Day, last Marfacts + My lung issues.

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It seems very fitting that Rare Disease Day happens to fall on the last day of Marfan Syndrome awareness month. For those of you that don’t know Rare Disease day aims to raise awareness all around the world for the approximately 1 in 20 people living with the thousands of rare diseases and disorders that exist. Events are held in dozens of countries all over the world, as well as various social media campaigns and more. To learn more click here.

When I first learned about this project a few years ago I began researching and blogging about various different rare diseases in the days leading up to it, in the hopes of shining some light on the day itself and some of the specific illness they’re trying to make change for. In the process I learned so much about some of the things people are living and struggling with that I had never even heard of before. It ended up being a really great learning experience for me.

It’s hard to really comprehend just how many people these diseases – connective tissue disorders included – effect, and how much people go through because of them. And still, for so many diseases and disorders, so little is known about what they are and how to properly diagnose and treat them. To find out how you can help and participate in future Rare Disease Days click here. You can also raise awareness by wearing jeans for genes today! :)

And now, in honor of Marfan Syndrome awareness month, here are the last of this year’s Marfacts (provided by the wonderful Marfmom and Marfan Foundation) respectively.

Marfact #27: Do you know the 5 Ps that could signal the presence of an aortic dissection? They are pain, pallor (paleness), pulselessness (difficulty finding a pulse), paralysis, and paraesthesia (a tingling sensation, like when your foot falls asleep). Not all of these have to be present. A sense of impending doom is another common symptom. If you have Marfan Syndrome or a related disorder, make sure the ER rules out dissection with an echo, TEE, MRI, or CT scan: whichever can be done the most quickly and be expertly read. More information about how to advocate for yourself or your loved one during a possible cardiac emergency can be found here.

Dissection awareness poster featured on the NMF's website.

Dissection awareness poster featured on the Marfan Foundation’s website.

Marfact #28: There are several lung issues that can come along with Marfan Syndrome. These include restrictive lung disease (it affects about 70% of Marfs), chest deformities, collapsed lung, emphysema (at least 10-15% of Marfs, if not more), asthma, and sleep apnea.

In my case the bases of both of my lungs have what is know as “chronic atelectasis”, which is: the incomplete expansion or collapse of parts of, or a whole, lung. This can cause numerous issues such as: difficulty breathing, chronic cough, increased heart rate, low oxygen saturation, chest pain and more. For me it was coincidentally discovered during a chest CT and X-ray that was done last year as part of my annual cardiac screening. It definitely wasn’t something that I was expecting to have show up. I’m not sure how long my lungs have been like that and if that’s the reason for some of my chest related pain and problems or if those are from something else. Maybe it’s a bit of both. A lot of the complications and symptoms from that overlap with some of the other illnesses I have like POTS, so it’s frustrating trying to figure out what could be causing what sometimes.

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Thank you all so, so much for your support during Marfan Awareness month. I hope that with the continued efforts to raise awareness by not only those within the Marfan community but outside of it as well, that in the near future no more lives will be lost from otherwise preventable causes.

I also hope that the number of people who suffer for what feels like endless years alone and in debilitating chronic pain without any answers, will continually diminish until it disappears altogether. This feels possible with the tireless efforts of organizations like the ILC and Marfan Foundations, both of which I could never find enough adequate words to properly thank for the differences they’ve made in my life. ♥ 

Marfact #25 + My lens journey: Part 4

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

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

Visit www.marfan.org for more information.

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My lens journey part 4:

Part 3

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

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

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

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

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

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

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

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

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

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

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My lens journey part 3:

Part 2

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

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

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

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

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

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

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

Coma

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

Part 4

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

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

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

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

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

Part 1

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

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

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

6th Eye Surgery

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

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

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

Marfact #20 + My lens journey: Part 1

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

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

Visit www.marfan.org for more information.

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

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

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

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

Part 2

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