The hospital stay: part 2

Continued from yesterday.


The spinal fluid leak and post-dural puncture headache.

During the lumbar puncture they were instructed to get fluid for diagnostic testing, but also since they were so sure my problems were caused by increased intracranial pressure, to both measure said pressure to prove that theory, and to drain some extra fluid to see if my symptoms improved. But, they were really surprised to find when measuring my pressure that it was well within the normal range. I was confused, surprised, and worried. They were also confused and unsure what to do next, so they decided to drain some fluid anyways just to see if my symptoms improved (something that I also thought was a good idea at the time). Now I should mention that before the LP another big concern of mine was a potential complication that they call Post-dural puncture headache, and the continued leaking of spinal fluid from the puncture site. My connective tissue disorder puts me at a higher risk for this because my tissue is weaker and doesn’t repair or heal itself properly. Within hours of the LP it was clear that I was suffering from exactly that. The pain from the headache if I’d sit or stand up came on quickly, and severely. Likely made worse by the spinal fluid that was already drained during the LP and the fact that it was drained despite my pressure being perfectly normal to begin with.

While most leaks eventually seal themselves and the headaches and other symptoms then resolve, I knew that in my case my tissue likely wouldn’t be able to do so by itself. When this happens they do another spinal injection, called a blood patch, where they inject some of your blood into the area around the puncture site, which triggers an inflammatory response and usually successfully seals the leak. Despite my telling them that my connective tissue disorder would almost certainly make a blood patch necessary, they said that they won’t do blood patches until it’s been a week because by then most leaks have resolved. This brings me to something that I noticed often in the hospital: when mentioning my connective tissue disorder, those who even knew what it was, would act as if it changes nothing in the way that they handle my case. This can be both frustrating and scary because your connective tissue is everywhere, and when it’s weak it has the potential to change nearly everything big and small about how your body reacts to virtually anything that done to it from a procedure to surprisingly medications. For instance, as I observed and pointed out near the end of my hospital stay, my tissue was necessitating the frequent changing of my IVs, because otherwise the IV would fail and my vein would end up inflamed and sore. Up until that point, they’d been trying to leave the IVs in for as long as they would with any patient, and my weaker tissue couldn’t handle it. This happened many times without anyone taking into consideration my disorder, despite me frequently mentioning it.

As for waiting an entire week for a blood patch; the thought was terrifying for me. The pain in my head was nearly unbearable at times, and even laying down didn’t fully resolve it. But what made it worse was that with my chronic pain, particularly my back issues, having to lay flat on my back for very long without being able to change positions is very painful. And now that was the only way I could lay. I couldn’t begin to imagine having to do it for a week, yet there was no other position that I could tolerate from the severe headache.

Pain, vomiting and frustration.

The next morning, I was told that my doctors had decided that they wanted to do an MRI. The MRI didn’t show anything that could be causing the swollen optic discs either. It did show that I had an enlarged pituitary, a sign of intracranial hypo-tension, resulting from the spinal fluid leak and confirming that the severe headache I was experiencing since the hours following my LP were from that. Considering all of the information my doctors in the hospital had on how my ICP hadn’t been increased, was now far too low, and continuously lowering still from the leak, and the fact that I was in that much pain because of it; I still can’t understand why they would ever think that it was a good time or decision to start me on a diuretic in order to further lower my ICP (which can be dangerous!). Given that my first dose of the medication was that evening (after doctor’s rounds) and I wasn’t aware that they were planning on putting me on it and hadn’t been spoken to about it, my nurse essentially gave me the option of either just taking the medication until my doctors could talk to me and authorize them taking me back off of it, or she could write down in my file that I was uncooperative and refusing to take my medication. So, since I’m not a very confrontational person and I was too tired and sick to argue it further, I took the medication, knowing it could likely make a nearly unbearable problem even worse.

Once I saw my doctors the next day they said that they agreed that I shouldn’t begin taking the medication yet (you think?!), with no explanation as to why they didn’t just agree with that in the first place without making me worse off with it instead. My pain was pretty excruciating at this point and I hadn’t really slept much in the past week with everything going on. My mom wasn’t a whole lot better off but she was there for me no matter what, and it was a lifesaver to have someone there to help advocate on your behalf because frighteningly at times, my treatment in the hospital (supposedly one of the best in Canada) was dismal and at times bordering negligent. I was left to deal with the horrible acute pain of everything going on with little more than what I take at home on a daily basis to begin with, and for the first few days because of some mix up with my medication forms, they couldn’t even give me ibuprofen. They eventually, after prodding, started to give me what they called a “headache” cocktail every 8 hours, which I was relieved and hopeful about. The cocktail was an IV anti-nausea medication, followed by an IV drip of an NSAID (ketorolac). I thought maybe I noticed a slight improvement at first but soon – maybe from the still lowering ICP – it wasn’t doing anything but making me sick.

One of the things that they say is incredibly important with a spinal fluid leak is to get plenty of fluids. But by this point the low ICP (and the ketorolac) was making me vomit even just from trying to drink water. Despite being well aware of that, it wasn’t until I told my nurse that I was probably getting dehydrated and should be put on fluids that she said she’d mention it to my doctor. A few hours later they had eventually hooked me up to fluids, and I continued to not be able to eat or drink without throwing up. But still, after 24 hours of not having to urinate once, I was pretty sure that I was still dehydrated, perhaps severely at this point. This was when I found out that the amount of fluids they had put me on was equivalent to about a cup of water a day, which is well under the needed amount for an average person, never mind someone who’s both leaking spinal fluid and continuously vomiting. I told my nurse that I couldn’t drink anything and wasn’t getting enough IV fluids and despite all signs clearly and obviously pointing to that being a major problem, she still seemed unsure about it. So, instead of upping my fluids immediately, instead, to see if that really was the reason that I hadn’t been urinating, she insisted on first scanning my bladder for an obstruction. I had to so strongly point out all of the reasons that dehydration was going to be the problem that I was starting to get weepy and exhausted because I couldn’t understand why I was having to fight for this in the first place. It’s hard to explain how this feels; when you’re in severe pain and unable to move, frequently throwing up, and having to literally argue the importance of the most basic human need (water) to the people who are supposed to be taking care of you. I felt like I was constantly fighting for the simplest things in the hospital.

I want to say here that I’m a very easy going, compliant and non-confrontational person, often to a fault. I have a difficult time sticking up for myself, even when it’s right and needed. I also understand that being a nurse is a very difficult job. They deal with an incredible amount and they do one of the most important and demanding jobs that there are, often without thanks. But, that being said, there’s also a standard of care in hospitals, and it’s frustrating, and frightening when that standard of care is repeatedly not being met, or when the simplest things are made difficult simply because you’re not being thought about as a human being or listened to. It’s an incredibly helpless feeling to be bed bound, and in severe pain, and to have the people with power over you, who are responsible for taking care of you, often failing in the simplest areas of that job. To end up dangerously dehydrated while your in the hospital seems unacceptable to me, particularly when someone is repeatedly pleading with you to do something about it.

There were so many instances in the hospital that made me feel that way. There were days where I was informed that they hadn’t ordered a medication that I was on from the pharmacy yet (spoken as if it were no big deal) and was told that I’d have to miss a dose, and I’d then have to make a point of fighting just to get a medication that I had managed to never miss a single dose of at home. There was also the time that my IV machine kept repeatedly beeping in the middle of the night indicating a problem in the flow of my IV and despite me telling my nurse multiple times that I could feel fluid dripping down my hand, instead of simply looking at it she kept trying to adjust the machine or untangle the cords. Then she’d go away for a few minutes only to have to come back again when it would start beeping. I felt pretty badly for my room-mate that night. When she did finally turn on the big light to check, my IV was leaking blood and fluid down my hand which had been the problem, so now she had to try to do another IV in the middle of the night. Two hours since the first beep, two nurses trying, and 3 IV pokes later the problem was finally resolved when it could have been almost immediately instead.

Unanswered questions, an uncertain diagnosis and it’s connection to my disorder.

My neuro-ophthalmologist was surprised that my ICP hadn’t been high. He was certain that must be the problem because none of the other scans and tests had shown anything else that could be causing the optic disc swelling. He wasn’t entirely sure of what to do to help my eyes at that point, so decided (as mentioned earlier) that the easiest course of action for now would be to put me on a diuretic, just to see if there was any improvement and then to watch me closely in case my vision deteriorated. We decided that the med should wait until my ICP recovered from the spinal fluid leak, and then we would slowly begin the new medication (which can be difficult to tolerate). I felt really frustrated, not at anyone, just at the fact that here I was again, in a difficult medical situation with no clear answer.

Luckily for me, someone with the ILC foundation that I often correspond with and who knows and works with many others with my disorder, as well as with possibly the top neurosurgeon in the world dealing with problems related specifically to our rare disorders, helped me get some answers. I later found out that there is indeed a link between both high and low ICP, and connective tissue disorders such as mine. It seems that for whatever reason (they haven’t been able to figure it out yet), our bodies can’t always regulate our spinal fluid flow and “draining” system properly, which can result in an ICP that can fluctuate between being normal, to too high, to too low. This can cause symptoms like headaches that can come and go, and when the ICP irregularities are prolonged this can cause symptoms like swollen optic discs. And while I’m not happy to have another rare, often unknown side-effect, of an already rare, often unknown disorder, I am glad that I’m not the only one and that there are some answers out there. It made sense as to why my ICP was completely normal when it was measured despite all of the symptoms and signs I’d been experiencing pointing to it being too high before. This problem, whatever the cause, can be difficult to treat, but that was a bridge I would cross once and if I got there.

I wasn’t sure how I was going to do it but slowly each day dragged on and ultimately passed by. My symptoms briefly improved enough for me to walk up and down the halls outside of my hospital room a few times, holding on to my mom, but they quickly went back to how they had been, picking up new symptoms along the way. I started having problems with my hearing in both ears which I was certain was from the low ICP, but at the time the resident neurologist, who I saw the most during my hospital stay and who tried to do my LP the first time, told me that it was physically impossible (not even just unlikely, but impossible) that my low ICP and my ear problems were related. This scared me because he said it with such certainty, and I was now thinking that either something else was now wrong with me, or that I was losing it and it was just in my head. Frustratingly, we later found out the ear related symptoms I was experience were in fact one of the most common symptoms of low ICP and was absolutely caused by my spinal fluid leak. Which strikes me as the kind of thing that he should know, and if he didn’t know then I wish he would’ve just said so instead of telling me that the thing I know that I’m feeling is actually impossible.

Blood patch and being discharged from the hospital.

Eventually, when it was finally nearing the week mark since my lumbar puncture, it was decided that it was time to do the blood patch to seal the spinal fluid leak. I can’t even begin to explain what an enormous relief that was to hear. I was also a bit afraid, knowing that they’d have to do another spinal injection in the same spot, but without radiology. Luckily for me the anesthesiologists had a much easier time than the neurologists had. It took him three attempts, but he interestingly used the nerve pain it was causing to help him find the right spot. When he brushed a nerve (unfortunately for me) the first time, he asked me which leg I felt it in, and when I said the left, he seemed to move the needle over to the right side, and when I felt the pain again, but this time in my right leg, he moved the needle ever so slightly back to the middle, which worked.

Blood patches are not a guaranteed fix for a spinal fluid leak which scared me. I was told that they work about 80% of the time, but I felt like it was doomed to fail after everything else that had gone wrong. They often work immediately but as much as I wanted to say that it had worked when the doctors asked me, I noticed no improvement whatsoever in the hours following. So, we were told not to give up hope and that it was very important that I lay flat on my back and not move, because that just might get it to work. And eventually, ever so slowly, I started to notice a gradual improvement. By then, I was so desperate to get out of the hospital and so happy that I could see a light at the end of the tunnel, that I played up how well I was already feeling as much as possible and told them I was certain I was good enough to go back to the hotel. Even then I was fully aware that I should have waited at least one more night in the hospital, but that stubbornness kicked in again. I was still in pretty bad shape, and both sitting and standing for too long were still incredibly painful. By the time I exited the taxi and made it to my bed at the hotel it was nearing my limit of tolerance, but every hour I found myself able to sit and stand a little longer each time I tried.


Saying goodbye to my hospital bed!

We had one last appointment with my ophthalmologist to see how my eye infection was doing, which was also pretty hard to get through pain-wise, but it also signaled the longest that I’d been able to sit and stand since my lumbar puncture, and that I really was on the mend.

Getting home, and how things are a year on.

Before long we were on an airplane home, finally, a few weeks after we’d left. But frustratingly, we were returning with a lot of unanswered questions and uneasiness, on top of the incredible relief. My optic discs weren’t better, and the lack of a concrete, simple explanation for what was going on left us worrying about how or if we’d be able to treat it.

During my hospital stay, because of the trouble we’d had with my IVs, I also ended up with thrombophlebitis which is an “inflammation of the wall of a vein with associated thrombosis”. It was in the hand that 2 (out of 4 or 5) of my IVs had been in, both of which ended up having problems and leaking. The veins on that side of my hand have hardened and were at first red, visibly inflamed and often painful. While this condition can usually be avoided by changing the IV once a week, it seemed that with my fragile tissue even a couple days of an IV in the same spot ends up being too long.


The residual redness and inflammation in the back of my hand and veins.

All in all, things have been up and down since then. The medication has been very hard to tolerate at times particularly in regards to my P.O.T.S. It takes many of the difficult symptoms of that and exacerbates them, particularly the fatigue and faintness. Often at times it can cause very painful pins and needles in my legs, but as my dose has decreased this symptom has nearly disappeared.

We travel to P.G. to see an ophthalmologist, at first once a month, but since my eyes have been relatively stable, now we go every 2 months. Then we travel back to the neuro-ophthalmologist in Vancouver first every 3 months and now every 4. Sometimes my ICP doesn’t seem to be too high and sometimes there are signs that it is indeed too high again (like a lack of pulsations in the back of the eye caused by increased ICP). The swelling in the optic discs tends to still be there, though this last appointment in Vancouver it was decided things were looking really good so we could try lowering my medication to see how things went. Now I’m waiting for my followup appointment in Prince George to see if lowering my medication caused a worsening of the swelling or pressure.

It was a bit of a difficult and scary year at times. And by the time I finally got home I was covered in bruises and reminders of everything that I had been through. But now, it’s hard to believe that’s how long it’s been since all of that kicked off. I can’t imagine going through any of it without my incredible mom. She was there every single step of the way, no matter how awful she was feeling or how difficult things got. I’m so, so lucky to have that support through things like this.
And, thank you to all of you for sticking around, and to the amazing few who actually made it through this novel of a post (I don’t blame you if you didn’t!).

– Katie ♥

We always carry on, and sometimes it gets easier.

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

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

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

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

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

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

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

Big hugs to you all. xx
– Katie

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

The fear that follows.

Generally, I try not to read too much information when it comes to aortic dissection in Marfan Syndrome. It scares me too much because it reminds me that it does happen and that it can be fatal, and I don’t want to die. I know that the odds are generally in my favor, it’s not like having MFS is a death sentence anymore but I’ve heard some very real horror stories and I don’t live in a good area with a hospital that could quickly save my life in such a case. When I read about the life saving surgery and the fact that it can leave one paralyzed, or that it can cause heart attack or stroke, I can’t help but be terrified and feel reminded that survival life is never a given.

I also have a hard time entrusting something as important as my life to another person (doctors). There’s always a fear and a possibility that mistakes are being made in one’s care, after all, they are only human and there’s good and bad ones in the medical community just like everywhere else.

The reason that this is on my mind is because I’m considering going off of the beta blockers. I would think that it’s okay because it was my own initiative that got me on them in the first place (my heart rate was becoming increasingly high, not good for someone with MFS, beta blockers slow the heart down) but I’m trying to do all of the research that I can before making that decision. I’ve been on them for a little over 4(?) months now and I think that they’re the cause of my unbearable exhaustion and sleepiness. I have to nap multiple times a day now, sometimes even only 30 minutes or so after waking up in the first place. When I’m not sleeping I seriously wish that I was. The odd thing is that I still get insomnia a lot. I’ll be so tired that I can’t keep my eyes open during the day but when I do try to sleep I still can’t a lot of the time. My exercise tolerance has also decreased in a huge way. Getting my heart rate up to 100 takes as much effort now as getting it up to 160+ did before. In the end I guess that doesn’t really matter, I’m supposed to keep it 100 and under when exercising anyways to protect my heart. It’s kind of depressing but necessary. 

Since starting them sometimes I’ve caught my resting heart rate as low as 48. I haven’t panicked about it or gone to the hospital. mostly because usually when that happens I don’t feel much worse than compared to usual. Generally my pulse has been at around the mid to low 60s, sometimes the high 50s. 

Anyways, I’m nervous that were I to go off of them that it would be a huge and possibly dangerous mistake. I’d feel better about it if my next echocardiogram/cardiologist appointment wasn’t so far away still (April). From what I do know, with my aortic root size, it should be fine (as long as my resting heart rate doesn’t go back to being way too high), but I need to be sure. Talking to my gp about it seems pointless because she knows less than I do about the subject, she even forgot why we decided to start them in the first place (she thought it was high blood pressure when mine’s always been low), I could call the cardiologist but I haven’t met him yet (he’s my new adult one instead of my children’s hospital one) so I don’t know if he could make an accurate decision before our appointment.

If anyone has an opinion or more information on this subject I’d be happy to hear what you have to say, if you’d like to email me at or comment here I’d really appreciate it!

Head full of doubt, road full of promise.

I’ve been drawing again lately, for the first time in a long time. I used to do it in some capacity every day, just as I would write poetry often. But, the neck and head position required by drawing would aggravate my headaches to a degree that I couldn’t take anymore. The same thing would happen with my rib pain from the sitting position. This has been another bitter blow that pain has dealt me. The list of hobbies and activities that don’t make the pain worse is dwindling down to nothing. But that’s just it; I can’t let the pain take away everything that makes me happy. And when I truly think about it, why should I? I’m in pain anyways, whatever I do. So, I might as well be doing the things that I love and finding some happiness in the process. And I’m not saying that I’m going to pick up all of my old hobbies – like basketball and volleyball. I know that doing those things aren’t an option – pain-wise and Marfan-wise.

Moderation is the most important factor that I’ve found in containing the pain to a bearable degree. I draw for about 20 minutes, take a break and then when my neck and ribs feel a bit better I pick it up again for another 20 minutes. This doesn’t fix the problem entirely but it helps. I try not to do it for more than an hour a day, sometimes though, I break that rule – and pay for it. It’s given my attitude a bit of an uplift. It’s silly but I feel like I’m taking something back from my condition, like I’m not letting it get the better of me for once. Looking at it solely that way, like I’ve somehow won this one battle, makes me feel a bit better about my current circumstances. 

I’ve been sleeping, a lot because of the beta blockers. And when I’m not sleeping I’m either trying to or wishing that I was. I’ve spent about 42 of the last 48 hours asleep. Impressive for an insomniac. I think my days of being up all night may be behind me – although, the beta blockers do list insomnia as a common side effect so I guess I’ll see. That’s not very nice; that they can make you super tired but also make it so you can’t sleep.

Now for a coincidence – my mom was just prescribed the exact same beta blocker (Propranolol) as me – only for headaches and not her heart. I was disappointed because my headaches haven’t gotten any better since starting it but I read something online that said it may take 4 to 6 weeks after getting to the appropriate dose (mine may not be high enough) before you notice a change in your headaches. I really hope that’s the reason, but not as much as I hope that it helps my mom’s headaches. It’s been 12 years now since the initial headache started. She had that one all day every day until a couple of years ago. She still gets it at least once a day but it has mellowed a bit.

During the first couple years of having the headache she was scared that she was going to drop dead at any moment – the pain was so severe that she was sure there was something very wrong. I was shocked to learn that no scans or tests had ever been done to make sure that there wasn’t. Her doctors had never thought it necessary, no one (medical professionals) did anything to help her. I’ve watched my mom suffer with this for a long time now. I’ve seen that look in her eyes that only someone in a lot of pain has, everyday for the past 12 years. I didn’t understand it when I was younger; why she stopped going out as much and being able to attend as many of my school events as she used to. I was 7 when this started, and how it feels to live in chronic pain is something most adults can’t even comprehend. I often thought it was something that I had done, like I was in trouble or that she was just being mean when she said no to things. Now regrettably I know how much harder those things were on her than me, especially if she knew that it was making me unhappy. I seriously hope that I kept that to myself and didn’t hurt her more with it.

Soon the chronic headaches turned into widespread chronic pain, and eventually she was diagnosed with fibromyalgia. A diagnosis that we still don’t know whether to believe is correct or was just handed to her by doctors too lazy to find the real answer. And I 100% believe that fibromyalgia is a real condition so don’t get me wrong on that. I just wonder if because it covers so many symptoms if they just stuck my mom with that diagnosis instead of putting in the effort to find another cause. Or if the original cause of the headaches remaining untreated did in fact morph into fibro and start causing the widespread pain – since we know that many cases of FM stem from acute injuries not being diagnosed and properly managed. Either way, she’s suffered and continues to. I would do anything to give her a body that doesn’t hurt, to let her live her life again instead of just surviving it.

If the Propranolol helps my mom’s headaches that would be the first step forward in her illness in 12 years. It wouldn’t fix everything but it would fix something

“There was a dream and one day I could see it.
Like a bird in a cage I broke in and demanded that somebody free it.
And there was a kid with a head full of doubt,
So I’ll scream ’til I die and the last of those bad thoughts are finally out.”
– Head Full of Doubt, Road Full of Promise by The Avett Brothers

Seeing things?

This may sound odd and it could be very improbable but I’m just relaying exactly what I feel, and what I feel is: my body seems to go through phases where the tissue is weaker than other times. Like for instance, sometimes many of my joints will continuously subluxate at the same time more often for a while and then go back to “normal”. My pain level also rises in the same areas, not even only after the subluxation but before too. Like today, my tail bone is out again (it’s been doing this for as long as I can remember and I think I can place the initial injury that weakened the tissue back to when I was 5 and dropped down on a bicycle extremely hard, I remember only because it hurt so bad), my shoulder is continuously grinding and popping in and out, as well as my hips. I’ve only been awake for about an hour but I wouldn’t be surprised if my ribs and throat or jaw joined in as well. I had an awful headache all through the night and my left hip was aching the entire time I was in bed making for a not so restful sleep (my mattress is also extremely worn down and hard on my body, spine especially).

Another new development; I had a visual hallucination last night. Something I’ve never experienced before. I had been tossing and turning and only sort of half sleeping, when I woke up to see my red heart shaped pillow floating up in the air around my room. The more I looked and processed the more my mind tried to make sense of what I was truly seeing, while searching with my hand for the switch to my lamp. I had wondered if it was my garbage bag (hot pink) being blown up and around by my fan because a floating pillow is obviously impossible. When I finally did find my lamp switch and flooded the room with light there was nothing in the air. My pillow and garbage bag were in their rightful places and I was left shaken, blinking and staring up at nothing at all. I felt so awake, but the more probable solution to such a thing would be that I was not entirely so…right? But I was seeing clearly and thinking clearly. There was plenty of time before I switched the light on for me to stare at what I was (or thought I was) seeing. 

I hadn’t taken any sleeping or pain medication in the last 24 hours. The only thing in my system was my beta blockers and the natural hormone melatonin that I’ve taken 1000 times before. I’ve been trying to come up with possible explanations to the hallucination and though I feel so sure that I was fully awake and that was not the cause, it’s the most realistic reason that I can come up with. After all, people don’t just hallucinate floating objects for no reason. It was light enough in my room with the setting sun that I saw what I “saw” clearly. Whatever the cause, it definitely gave me a bit of a fright once I realized there was nothing there. I lay there thinking about it for a long time before getting up and checking on my mom (just in case it was a “sign”) and finally deciding to try sleeping again. I thought of telling her at the time when she asked me what was keeping me up but I figured it would sound even crazier out loud than it did in my head. 

Anyways, there’s your dose of strange for the day, and it’s only 7:00 am! I’ll probably be going back for a nap at some point today, the longer I sit here writing this the sooner I think that I’ll be doing that. The beta blockers like to make me super tired and at the same time make trying to sleep hard work. Oh, and I think there may be many grammatical and punctual errors in this post because of sleepiness, a headache and a general unwell feeling. Thank goodness for spell check though, at times like these I’d be so much worse off without it.

An update of sorts.

First of all, I want to extend my thoughts and prayers to everyone and anyone affected by the theater shooting that occurred last night. I am so sickened and saddened that this happened. I can’t even fathom what the victims and their families were and are going through, but from the depths of my heart and soul; I wish them well. It feels like a sad day to be human, when reminded that our species is capable of such senseless horror and cruelty. May those who were injured make speedy recoveries with as little added suffering as possible and those who were lost find peace.

Now, I realize that I haven’t been blogging about any medical related things in these last few posts. Mostly because nothing too interesting has been happening.

My shoulder did dislocate for the first time the other day. Well, actually, my guess would be that it was more of a subluxation than a full dislocation, but painful none the less. And disheartening. Another reminder that my tissue is weakening and that things will and are only going to get worse with time. To make things even more pleasant, a couple of my ribs decided to also subluxate today. They popped back into place fairly quickly but as you can imagine, the surrounding tissue and what not are going to take a while to heal. Sadly, I’m becoming very used to this. It seems like every time I finally heal from the last subluxation another one occurs. I kept my arm in a sling for the first little while after the shoulder incident but it seems unnecessary now, it doesn’t seem to be helping as much.

I recently ordered an orthopedic corset off of the internet for the purpose of compressing my ribs and hopefully keeping the lower ones in place. I got the idea because when my ribs are moving around it always helps to hold my arm down against them. The sizing got messed up on the first order so I’m just waiting to get this one and try it out before I give my review on it. I’m really hoping that it does something.

In other events, I’ve been avoiding my doctor like the plague. Not for any specific reason really, only that I’m just tired of doctors. And I’m also still contemplating what to do about my beta blocker situation and I need to decide on something before going in. I finally made an appointment for next Friday so I really need to make a decision.

Now I’m going to be super cliche and talk about the weather. Mostly only because we’ve had more thunderstorms in the last couple weeks than I can remember having throughout my entire childhood. Which is awesome because I love lightning. Watching it at least. I love the rain too.

This picture was taken in my hometown where there’s been some flooding. Where the geese are is actually a road. It floods like this once a year, and every once in a while someone is silly enough to attempt to drive through it.

If It’s Meant To Be

There’s a debate I see in different places on the internet, about whether a person with a genetic disorder should – or even has the right to – have a child when there’s a chance that they too will suffer from the same disorder. In this case I’m speaking of Marfan Syndrome and similar disorders. Is it okay for someone with this disorder to risk passing it on to a child? And, as with any issue, there are a lot of very opinionated people, people who strongly and loudly object, saying that it’s selfish and wrong. And just to clarify, this is my opinion on this matter only in relation to Marfan Syndrome and similar disorders, not the many other genetic disorders in which I’m unqualified to have an educated opinion.

Now, my mom didn’t have the disorder, neither of my parents did. I received the defected gene that causes a tissue disorder via what’s known as “spontaneous mutation”. Meaning, the gene wasn’t inherited, it just mutated on it’s own. This is the case in approximately 1 out of 4 instances of MFS. But, had one of my parents had the disorder already and still chosen to risk it by having biological children here is what my stance is on the issue:

I suffer because of this disorder, I do. I’m always in pain, I’ve had 7 surgeries, I take many medications with many side effects. I worry, about my future, about my heart, about in what way this disorder is going to affect my body next. So yes, clearly, I struggle because of this disorder.

But the thing is, I am who I am because of this disorder and the things that suffering has taught me. I feel like this life has opened my eyes to things that I wouldn’t miss for the world. Things like compassion and understanding and passion. You need to be passionate about the things that you do have in your life, or the struggle wouldn’t be worth the reward those things give you. I’ve been given the ability and the need to fight in order to live and in the end living becomes something that holds so much more meaning because of it. And maybe I live a life that hands me days where all I’ve accomplished is not giving up, but I also live a life that’s been blessed with only the most amazing people to help me through it. I am happy to be here, no matter how much it hurts, no matter how often I question whether or not I can handle it anymore. So, if you asked me; had I seen this life – the good, the bad, the people, the tears, the love, the pain – and been given the choice, would I have chosen to be born to this life, to this disorder? Yes.

Had my mom known there was a chance that I would have this disorder, and decided that it wasn’t worth the risk then none of this would’ve happened. None of the laughter, none of the smiles, the heartbreak, the hugs, the lessons about life that only pain – physical or emotional – can teach you. No “Marfamily” to lean on and learn from, no words of strength from the other side of the world, from strangers in nature but family and dear loved ones in circumstance. And, maybe I’m crazy for it but I would not change those things.

There is suffering everywhere in the world, in numbers incomprehensible, and in ways unimaginable. I have a roof over my head, food in my stomach, a home, a family and yes, I happen to have Marfan Syndrome, I happen to be in pain. But it’s part of life, it’s part of my life. And one day, if my life should allow it, and I need to make a choice: to risk having a child with my disorder, or not, I know my decision. And I know that it’s the right one because no matter what that child may face because of it, they will face it with a love unwavering and unconditional, and a strength unlike any other. If it’s meant to be, it will be.

Dear Marfan

*I’m re-posting this with my name at the end as I’ve seen it being blogged and reblogged on many different sites by many different people – some who also suffer from Marfan Syndrome, some who have family members with chronic illness and some who themselves have other chronic illnesses. And, don’t get me wrong, that makes me unbelievably flattered and happy, that so many people can relate to my words, I just would like people to know that it was me who wrote it because I take pride in my writing. Thank you so much to all of the people spreading this around, it feels amazing to be heard when living with a pain that often makes me feel so alone.

Dear Marfan,

First thing’s first, I do not hate you. Sometimes I’d like to, for all of the people that you’ve taken from this world too soon, for all of the pain and heartache you so often cause. But, you are what has made me who I am today, and showed me strength that I never would’ve thought I had. So, I don’t hate you, but I am angry with you. I’m angry with you for trying so hard to make me hate my own body and for all of the things that you’ve taken away from me, all of the things that I’ve missed out on because of you. You were not supposed to be a part of my life. You were never part of the plan.

I’d like to ask you why you chose me, but in all honesty, that’s not as important to me anymore. I wouldn’t wish you on someone else instead. But I do wish you didn’t make me feel like a ticking time bomb. I wish that I never had to think about things like aortic dissection and emergency heart surgery, that I didn’t feel the need to have my affairs in order, “just in case”. I wish I didn’t have to wonder if you’d cause any future child of mine suffering, or if I’d lose my own life in the attempt to have one.

I wish you hadn’t taken my sports and many of my friends, and replaced them with medications and doctor appointments. You’re constantly reminding me that no part of my body is safe from your consequences and that it’s only a matter of time before something else goes wrong. You hurt me, day in and day out, standing or sitting, year after year, from head to toe. Because of you I talk more often to doctors than I do to people my own age.

I had dreams before I knew you. Now, I’m afraid to get excited for the future because I know, eventually, you’ll get in the way of it. You always do. As soon as I let myself want something, you give me a reason why I can’t have it. You’ve given me a body that is always telling me “no”. No, you can’t play basketball anymore, no, you can’t have a job, no, you can’t get your heart rate up, no, you can’t lift that. No.

These are all of the reasons that I’m angry with you, these are all of the reasons that you scare me. And honestly, it feels good to get them off of my chest. But, you haven’t been all bad. You’ve given me a chance to have this blog and to talk to and meet people that I never would’ve otherwise known. You’ve taught me a lot about the important things in life, like telling those you love that you love them, as often as possible. You’ve showed me the ignorance and cruel judgement of some, but you’ve also showed me the uncompromising kindness of others. You’ve taught me to be compassionate, and not judge others by their looks, because you never know what they’ve struggled through.

I wish that I could know a future without you in it. I wish that you hadn’t changed everything so much. But most of all, I wish that my body belonged to me, not you.

By Me, Katie Robertson

“A tragedy need not have blood and death; it’s enough that it all be filled with that majestic sadness that is the pleasure of tragedy.” – Jean Racine

Beta Blocker Decisions

I wrote here about my last cardiology appointment and how my doc left it up to me as to whether or not I wanted to start beta blockers now or later, saying that he couldn’t really recommend I go one way or the other. Now, for those of you who don’t know, beta blockers are used in cases of Marfan Syndrome, in order to lower the heart rate, therefore decreasing the amount of stress put on the aorta, with the objective being to postpone heart surgery longer and to make the chances of aortic dissection lower. At the time, because my aorta is still fairly stable and because beta blockers can cause many highly unpleasant side effects (which with my medication history, I’d be very likely to pick up) I made the decision to stay off of the beta blockers for now. This was not carelessly decided mind you, I did a lot of research and spent many hours discussing and contemplating my choice. And of course, I’ve often wondered if that was a reckless or stupid decision.

This was back a few months when on average my resting pulse was always in the 60s and my blood pressure usually around 110/70.  And while my blood pressure hasn’t changed at all, my heart rate certainly has. Now it’s usually 100, often spiking to and staying at 120 – which is the recommended maximum limit for those with MFS who are not on beta blockers. This is very odd for me, nothing’s changed in my eating and exercising habits, caffeine intake etc. But it has me worried that I should definitely be on the beta blockers now. I was thinking of giving it a few more days and continually checking both my heart rate and blood pressure on the at home monitor we have, to see if anything happens before making an appointment with my GP. But this has been going on for a few weeks now and it doesn’t seem ready to quit.

I wish my cardiologist lived closer, right now he’s 8 hours away. My GP is good but she doesn’t know MFS the way he does. I don’t want to be stupid with my life, but I also seriously don’t want to deal with the monstrous list of effects that beta blockers can have on you, like, for instance, zapping all of your energy and making you feel tired all of the time. And headaches, I certainly don’t need help getting those, I do just fine all on my own.  I’m really struggling already and don’t know if I could keep any more fight in me if it got any worse. And for some silly reason, I feel like being put on beta blockers would be a huge negative turning point – like another notch in my “look what Marfan Syndrome is doing to me” belt.

What to do?