Eye and heart updates (again).

With everything that has been going on with my optic discs and brain lately, I never took the opportunity to write about the outcome of my cardiologist and ophthalmologist appointments from last March.

Wind blown.
My cardiologist appointment didn’t go as well as we’d hoped; my aorta had grown a bit since my last visit, which is never the greatest thing to hear, but it was also still quite stable overall. To get a better idea of how quickly it may or may not be progressing—and to maybe start me on treatment with Losartan if necessary—the cardiologist wanted to see me again in 6 months for an MRI. And, the MRI results showed that in those 6 months my aorta hadn’t grown any further, so for the time being it’s still safe to leave it be. I’m due for my next checkup in about 2 months, so we’ll see how things are looking now. I’m quite relieved at the results, and as far as I know my aorta is doing pretty well for someone my age with MFS, so, no complaints there.

As I’ve mentioned before on here (in “It’s hard to go through it again.” and “We always carry on, and sometimes it gets easier.“), the lens in my right eye suddenly shifted early one morning as I was getting out of bed. This was the third time that I’d experienced something like this with my lenses, so I was pretty certain I knew what was going on. It was somewhat devastating at the time because both times this had happened before, I would end up needing surgery soon after, and my vision would never be the same. But, thankfully it was (and is!) still hanging on because of some factors we weren’t aware of.

When we got to my ophthalmologist in Vancouver he told us that my lens was indeed holding on by one suture instead of two, which was why it had shifted and was now relatively (but not completely) loose. What we didn’t know was that during my last lens reattachment surgery, only one suture had come loose, so he only had to replace the one. We had always assumed both had been replaced. So now, the new suture is still stable and hanging on, but the original suture from my lens implant surgery—13 years ago—has finally let go. This can help account for why this lone suture has managed to hang on two years longer than last time—it’s relatively new and strong.

The trouble is, with only one suture holding all of that weight—in combination with the weak tissue from my disorder, trying to support it—we were told that it’s not a matter of if it fully dislocates again, but when. Sometimes it’s hard to sit here waiting for the other shoe to drop, not knowing when we’ll have to rush back to Van for yet another eye surgery and all of the difficulties that entails. But, I was already expecting another eye surgery, and having these extra years in between has been a gift, so the news wasn’t too bad.

My ophthalmologist was (and is) a bit concerned about some of the complications that a loose lens can cause regarding inflammation, hemorrhages and retinal swelling, so,— as long as the lens is hanging on—we’ll have to go back to Van every 6 months to check on all of those things. On a positive note, the lens in my right eye has had both of it’s sutures replaced, so it should be much more stable and less likely to subluxate like this one did, which is an incredible relief.

So, those are my—as per usual—long overdue eye and heart updates. I can’t believe it’s been nearly two years since everything with my lens kicked off. I’m so surprised and grateful that it’s still holding on.

Thanks for reading!
– Katie

A new step: Beta blockers.

As I’d mentioned a few posts ago, I had a doctor appointment last Friday. I told my gp about the change in my heart rate, and we made the decision that it was time for me to start beta blocker treatment. In many ways this was a bitter step for me. It was an affirmation that there is something wrong with my heart, it’s reality. It’s a scary thing to think about. When I write and talk about the aspects and dangers of my disorder in regards to my heart there’s a certain technical feel to it, I feel like I’m just talking facts and medical jargon. It’s important to me that I switch of any emotions that might personalize what I’m saying because I feel stronger and safer that way – it helps me cope. But, there are certain moments and milestones that hit home hard for me. Being reminded three times a day when I take this pill that I may very well need heart surgery someday or that I’m at 100 times greater the risk of aortic dissection makes it that much harder to keep those thoughts out.

The beta blocker my doctor decided on is Propranolol. It is also prescribed to treat many different types of headaches so she was hoping that it may help mine. To start with I had been taking 40mg three times a day, equaling out to 120 mg, but my heart rate started to climb again, so we upped the dose to 60mg three times a day. They definitely do make me more tired and weak, some days being worse than others. They’ve also made my circulation and Raynaud’s syndrome worse, where my thumb and sometimes other fingers and toes turn dark purple and become freezing cold. I’ve been experiencing the vivid dreams that can come with this particular beta blocker, but so far not the nightmares that they warn of. As for insomnia, I don’t know whether the pills are making it worse or if I’m just going through another bad spell. My headaches haven’t gotten any better, but I don’t know if I’m on a high enough dosage yet for that. My current dose is still not as high as I’m meant to get it up to, but they recommend a steady, slow increase so that you can safely monitor your blood pressure.

I feel very overwhelmed today. Family and social stresses in combination with the fact that my body is wreaking havoc is beginning to take it’s toll. I’m exhausted and in a lot of pain. I’ve been very lethargic while on the beta blockers and if it weren’t for the intolerable heat I’d be trying to nap right now. I truly, truly hope that those who are healthy do not take it for granted. I’d give anything in this world to have that one thing.


On an entirely different note, here’s a photo that I took at the height of flooding season. I loved the way that the reflection on the water looked with the beautiful sky.

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?