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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My introduction to Marfan Syndrome + Marfact #2

First things first, here’s your Marfact for the day (provided by the Marfan Foundation)!

Marfact #2: Marfan syndrome is a disorder of the connective tissue, which holds all parts of the body together and helps control how it grows.

Today I want to talk a bit about how and why the words Marfan Syndrome first came into my life.

1st Eye Surgery

I was 4 years old the first time a doctor ever suspected that I had Marfan Syndrome. To be more specific, it was my optometrist the moment he found out that I had ectopia lentis, or in other words: dislocated lenses. This is a fairly rare ocular finding overall but is commonly found in cases of Marfan Syndrome and a few other connective tissue disorders. My mom had taken me to the eye doctor thinking that I may have had lazy eye. Little did she know the foundations of her world were about to be shaken.

Not long after that initial finding we were being sent 8 hours away for surgeries and I was undergoing evaluations at BC Children’s Hospital for a rare genetic disorder no one in my family had ever even heard of before.

Looking back now, the part that seems the most frightening to me is the outcome. Even at that young age I did have a few alarming characteristics of Marfan Syndrome, most notably the dislocated lenses. As always, a cardiologist did an echo-cardiogram to see if my aorta was enlarged (another common finding in MFS). Thankfully, at the time my aorta was perfectly normal, which at such a young age isn’t all that uncommon in MFS.

The scary part was that based on this finding we were told by the geneticists evaluating me with 100% certainty that I did not have Marfan Syndrome and that I wouldn’t need any future follow up care or screening. And, that was that. We were never told that I should get echo-cardiograms as I aged to make sure things continued to look normal, or that sometimes a negative diagnosis at a young age can change into a positive one as you grow and more symptoms appear. We were simply told that I did not have Marfan Syndrome, end of story.

At the time this was obviously fantastic news. But it could have ended tragically had my aorta not been so stable during my childhood and early teens. I was always on our school’s sports teams and participating in anything gym class threw my way, never having any followup screening done on my heart. And, it wasn’t until almost 11 years after we initially heard the words Marfan Syndrome that I was properly diagnosed with a connective tissue disorder.

It’s frustrating looking back on it. I spent so many years scared and in pain with increasing amounts of unexplained medical problems. I saw countless doctors and out-of-town specialists, went through endless tests and sleepless nights. All for the sake of so desperately trying to figure out what was wrong with me and why my body seemed to be falling apart, when the answer had been there all along.

And, my hope is that with increased education and awareness that instances like this will become less and less. Doctors, specialists and even patients will see the signs and symptoms of a connective tissue disorder and recognize it for what it is and get the proper help. I’ve heard so many stories of people who’ve spent years – decades even – in unexplained pain with countless medical issues, before they were properly diagnosed. Hopefully those stories will become fewer and fewer.

To learn more visit the Marfan Foundation at www.marfan.org

Struggling & Taking Notes From an Oak

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

Much love to you all. ♥
-Katie

oak

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

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

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

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

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

A long night.

The last couple of days have been pretty lousy. Sorry, I know I haven’t been blogging anything very positive lately, my body just seems to be going through one of it’s really low points.

I’ve been dealing with some major urinary retention issues which are just miserable, especially during the night. Even when I can hardly go at all I still have to go often, which is a terrible combination. I spent the entire day yesterday thinking I was going to have to go to the hospital again like the last time this happened.

This is how my night went:

12:30 am I go to bed.
1:30 am I’m almost asleep but I have to pee, which I spend the next hour relentlessly trying to do before I actually can – though not very much.
2:30 am I’m back in bed but I can’t fall asleep.
3:30 am I have to pee again which takes me another 35 minutes or so.
4:15 am I’m back in bed but my bladder hurts and I still can’t catch any sleep.
5:30 am I get back up again for another bout of the same.
6:10 am I finally get back into bed and manage to fall into a light sleep.
7:00 am I’m awake again.
8:00 am I give up for a while and try to go pee yet again with more difficulty.
8:30 am I head back to bed but can’t fall asleep and am in a lot of pain.
10:30 am I get up again to try to go pee (for what feels like the millionth time).
10:50 am I get back into bed and still can’t fall asleep.
1:00 pm I finally just give up and pull myself out of bed.

My doctor thinks this is probably from my back. This time we’ve ruled out all of my medications as the primary cause so I’m afraid that it is too. I’m feeling really frustrated and exhausted. My neurologist’s appointment is still so far away and my referral to the urologist to make sure nothing else is causing this seems to have gotten lost on it’s way.

The minutes pass like hours.

Nightshade by Marc

Well my dear readers, today has been one long miserable day to say the least. I woke up at around 5:30 am or so with my signature headache and the overwhelming urge to vomit. I hadn’t hardly had anything to eat so there wasn’t really anything to vomit up but the waves of intense nausea and stomach spasms kept coming. This cycled on about 3 times, at once per hour and every time was worse than the last. Please make it stop; please don’t make me dislocate anything are the thoughts that kept running through my mind.

I never thought I’d miss my old headaches. But, to have a headache that’s so severe in it’s throbbing and overall pain that just turning my neck makes me cry out, then having to run to the bathroom and then traumatize my body with the vomiting that follows, is just about as miserable as it gets for me. And to make it even worse, I can’t keep anything down so any type of pain relieving medication is off of the table.

I was only finally able to take my medication a bit ago and every inch of my body was screaming at me for it. My headaches never used to come with nausea at all and now all of a sudden every severe headache I get the nausea isn’t far behind. I’ve thrown up more in the past 2 years than I ever had in my entire life.

I’m so relieved that it’s eased off now. The nausea has lifted and the headache is more background noise than anything. I always feel so exhausted and kind of hung over after these episodes, so I’ll probably be in bed early tonight.

Thank you for listening and much love.

xx

-Katie

Up and about.

Stunning picture taken by Jim Greer @Flickr

Hi to all of you beautiful people. Sorry that it took so long for me to update, I’ve still been having a pretty rough time pain-wise. That being said, my pain medication was upped last week and since then it’s been getting a bit better. Yesterday I was even able to go buy some (desperately needed) new pairs of pants after my doctor appointment. It feels good to accomplish things – no matter how small – after barely being able to make it out of bed for a week.

I’m hoping that things will continue to improve and I’ll be able to lower my medication back down soon. For now I’m mostly just biding my time until I can see the neurologist and maybe get some treatment options for my spine.

I want to say thank you, again, for all of your wonderful comments and emails of support. You all have such amazing, kind hearts and you really do mean the world to me.

Sadly, I haven’t been able to do too much drawing lately but I have started a new piece that I’m really excited about. I’m trying some new things with it and attempting to use my prismacolor markers. On that note, I got a new OTT-Lite lamp with a magnifying glass and I must say, looking through it is like looking into a whole new world! As most of you know I don’t have very good eyesight but with this new lamp I can actually see what I’m doing on an entirely new level! I didn’t know just how poorly lit my work-space was before now.

It’s really nice to have a hands-free magnifying glass too. Before I would just have a pencil in one hand and the magnifying glass in the other which is a bit of a pain when you need your free hand for other things.

I’m not going to post any pictures of my new drawing until it’s all done. I want it to be a surprise. :)

Much love to all of you.
And thank you for everything. xx

I’m tired…

More Dead Flowers by Bill Gracey

I’m tired of leaving my doctor’s office and coming home to the reality that I’m still going to be in loads of pain all day everyday.

I’m tired of waking up every hour of the night and realizing how much I’m hurting and how hard it will be to fall back asleep like that, only to do it all again in an hour.

I’m tired of finally believing we’ve gotten somewhere with my pain management, only to soon realize that I’m basically where I started.

I’m tired of being given all these fantastic ideas on different types of exercise that can actually help those living in chronic pain, while instead I’m being left in so much pain that I’m not sure how I’m going to make it out of bed at all.

I’m tired of getting up each day and not having the slightest clue how I’m going to make it through the entire day, never mind how I could possibly enjoy doing it.

I’m tired of knowing that someone who doesn’t suffer like I do 24/7 is the one who has the power to make my life more bearable, but doesn’t.

I’m tired of doctors who think that only treating “about 30%” of my pain is enough, when I’m only 20 years old and will have to spend everyday of the rest of my life with it.

And most of all, I’m tired of fighting so hard for so little.

Pain in Canada and the need for a national pain strategy.

First off, I want to thank all of you for your support during this National Pain Awareness week! I’m thrilled to say that Brittany’s story – deservedly –  brought in the most traffic I’ve ever had on this blog! Go Brittany!

Now, back to the reason I’m blogging tonight. I know that I’ve posted some of the following facts from the Canadian Pain Coalition before but here they are again (just as they appear on the website) for my new followers and those of you who haven’t seen them. It gives some startling insight into just how many people are directly impacted by chronic pain in Canada alone.

Pain in Canada Fact Sheet

• Although we have the knowledge and technology, Canadians are left in pain after surgery, even in our top hospitals
• Only 30% of ordered medication is given, 50% of patients are left in moderate to severe pain after surgery and the situation is not improving (Watt-Watson, Stevens et al. 2004; Watt-Watson, Choiniere et al. 2010)
• Growing evidence has identified that many common surgical procedures cause persistent post-operative pain that becomes chronic (Kehlet, Jensen et al. 2006)
• Pain is the most common reason for seeking health care and as a presenting complaint accounts for up to 78% of visits to the emergency department, recent research continues to document high pain intensity and suboptimal pain management in a large multicenter emergency department network in Canada and the United States (Todd, Ducharme et al. 2007).
• Uncontrolled pain compromises immune function, promotes tumor growth and compromises healing with increased morbidity and mortality following surgery (Liebeskind 1991)
• One in five Canadians suffer from chronic pain, children are not spared and the prevalence increases with age (Moulin, Clark et al. 2002; Schopflocher, Jovey et al. 2010)
• Many cancer and HIV survivors have greater quantity of life but unfortunately a poor quality of life due to chronic pain conditions caused by the disease or the treatments that cause irreversible damage to nerves (Levy, Chwistek et al. 2008; Phillips, Cherry et al. 2010)
• Chronic pain is associated with the worst quality of life as compared with other chronic diseases such as chronic lung or heart disease (Schopflocher, Jovey et al. 2010).
• Chronic pain costs more than cancer, heart disease and HIV combined. Estimates place direct health care costs for Canada to be more than $6 billion per year and productivity costs related to job loss and sick days at $37 billion per year (Phillips and Schopflocher 2008; Schopflocher, Jovey et al. 2010)
• People living with pain have double the risk of suicide as compared with people without chronic pain (Tang and Crane 2006)
• A recent review of opioid (narcotic) related deaths in Ontario, identified the tragic fact that pain medication related deaths in Ontario are increasing and that most of the people who died had been seen by a physician within 9-11 days prior to death (emergency room visits and office visits respectively) and the final encounter with the physician involved a mental health or pain related diagnosis. In almost a quarter of the cases the coroner had determined that the manner of death was suicide (Dhalla, Mamdani et al. 2009)
• Veterinarians get 5 times more training in pain than people doctors and 3 times more training than nurses (Watt-Watson, McGillion et al. 2008)
• Pain research is grossly under-funded in Canada (Lynch, Schopflocher et al. 2009)

Here are a few things that you can do to help:
Find your member of parliament and write them about the significant public health issues chronic pain in Canada presents.
• Volunteer with the Canadian Pain Coalition, click here to learn more.
• Spread the word about chronic pain on social media.
• Hang informative posters in your local doctor’s offices and hospital lobbies.
• Ask for permission to operate an information booth at your local hospital.
• Contact your local paper about doing a story on chronic pain in Canada.

Call to action

We haven’t yet been successful in getting the federal government to implement a national pain strategy in Canada – the main reason behind the Canadian Pain Summit. But, with continued effort, hopefully we’ll be able to accomplish this in the near future. The pain strategy aims to improve and target 4 key areas in relation to chronic pain: awareness and education; increased access to best care practices; enhanced pain research capacity; and ongoing monitoring of delivery of care, patient experience, quality of life and level of function. 

Clearly, there are so many things that need to be addressed in order to improve the lives of those with chronic pain. It’s a very complex problem and it’s not going to just disappear with a few simple fixes by a few specific people – it’s so much bigger than that. But, I can say having met some of the members of the Canadian Pain Coalition as well as Sandy Smeenk of the ILC Foundation, that we are extremely lucky to have such dedicated, compassionate and hardworking people out there, fighting so tirelessly to make change. I can’t even begin to thank them for all that they have done and continue to do for not only me but all of us.

I never could have imagined just how much sending in that essay to the faces of pain writing contest would forever change my life. If I hadn’t done so I never would’ve met all of these incredible, life changing people.