Talk About Your Medicines

ARC_Talk_About_Meds_Banner_TissueTales

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

A guest post by Destiny

1535016_684084964965780_1392250340_n

“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.

988809_706025416105068_2121916912_n

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

Thanks for sharing your story with us Destiny!

Marfact #25 + My lens journey: Part 4

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

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

Visit www.marfan.org for more information.

118359

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/

118355

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 #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

118358

Marfacts: 18 + 19

Marfan Awareness

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

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

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

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

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

118363

Marfact #17 and if I had known earlier.

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

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

Visit www.marfan.org for more information.

118354

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

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

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

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

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

Marfact #16, quality of life, and thanks!

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

Marfact #16: There is no cure for Marfan syndrome. However, a range of treatment options can reduce and sometimes prevent complications.

When the words Marfan Syndrome were first mentioned to my parents as something that I could potentially have they carried with them a very bleak and devastating outcome. It was thought that should I have the syndrome I would likely only live long enough to become a young adult at best. Today however, because of the treatment options offered – mainly where our cardiovascular systems are concerned – and the ever-expanding knowledge of connective tissue disorders, our lifespans are now on average the same as those in the general population.

It’s truly amazing how much things can change in the span of 20 years. Length of life isn’t so much of a worry anymore, but with that instead comes the increasing fear of quality of life. Our faulty connective tissue now has far longer to carry us than it ever has before. It’s scary to think about what kind of shape I’ll be in physically or how much more the chronic pain will have increased a year from now, never mind 40 years from now.

But, fears for the future aside, I’m extremely thankful that I live in a time where so much is available to us medically. And, if that much can change in 20 or 30 years who knows what treatment options we’ll have in another decade or two.

Visit www.marfan.org for more information.

I want to thank the Marfan Foundation for supporting Tissue Tales and sharing it on their Facebook page. I have so much love and appreciation for all that they have done and continue to do for those of us who live with connective tissue disorders and to have their support where this blog is concerned is an enormous honor.

And a ton of thanks to the wonderful Ben for his reblogs of the Marfacts! Please do stop by Ben’s awesome blog and have a read – and a look at his art. You’ll be glad that you did!

Marfact #15, genes, and connective tissue disorders.

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

Marfact #15: Marfan syndrome is caused by a defect (mutation) in the gene that tells the body how to make fibrillin‐1 ‐‐ a protein that is an important part of connective tissue.

While Marfan Syndrome affects connective tissue through it’s influence on fibrillin, the connective tissue faults found in Ehlers-Danlos Syndrome are instead due to mutations affecting the body’s collagen. Both fibrillin and collagen are very important in the function of our body’s connective tissue.

Also, connective tissue disorder causing genetic mutations have been found and identified on the gene that tells the body how to make fibrillin-2. This ties into what I was saying before about the complexity of our genes and connective tissue and how we’re still in the process of learning so much about both.

When I had my genetic test done they only looked for mutations affecting fibrillin-1. The test is pretty expensive and in my case the cost was only covered for that specific gene (and only after my aortic root started becoming enlarged). That’s one of the reasons that we weren’t overly surprised when the test came back negative.

For now I’m alright not having the test done on any of the other genes but it’s definitely something that may be worth considering more in the future.

Visit www.marfan.org for more information.

Know the signs; save a life.

118362