Marfact #25 + My lens journey: Part 4

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

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

Visit www.marfan.org for more information.

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My lens journey part 4:

Part 3

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

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

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

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

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

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

A Chance At Better Vision?

Eever since my lens implants dislocated over a year ago and the corrective surgeries left my vision much less than what it used to be I’ve been searching for something that might help to restore it. I don’t get much help from my eye surgeon in this department. He seems to think that the fact that the lenses look good to him that they are good enough as is. Easy for him to say when he’s not the one that has to look through them all day everyday. I’m constantly squinting and trying to see around the double vision the surgery left me with in my left eye. When I read or look at the computer it feels like it’s just sitting there, not doing much, and not helping me to see. My right eye (which is for distance) isn’t helping much either. I can’t see the television or any street signs etc. without being at a close distance, where as before I could see things from afar.

Like I’ve mentioned before; I am thankful for the fact that I can see at all. But that doesn’t mean that I shouldn’t hope to have my sight improved. So I started researching possible surgeries or implants that may reverse the damage to no avail. Until one day when I wasn’t looking I stumbled on what might just be what I’d been hoping for; Accommodative Intraocular Lenses. These lens implants are made to move within the eye similar to the way a natural lens would move. By doing so they are able to ‘accommodate’ both near and distance vision. But the best part is that these lenses have a much lower chance of causing refractive errors in the vision and visual aberrations. Which means hopefully they could take away the double vision.

The bummer part of all this is; I may not be a candidate. See, the way that these lenses stay in place is by being implanted within the capsular bag where the natural lenses once were. The capsular bags in my eyes were removed when I was four, along with my lenses. I’m assuming this knocks my chances of receiving this surgery. Admittedly it was hard to swallow at first. I’d thought that I finally found the answer I was looking for. But it does make me wonder if in the near future there will be technology that allows these or similar lenses to be implanted in eyes like mine. I will talk to my optometrist about these implants just for information’s sake. I’m not in a big rush to get my vision back, just the hope that one day I will is enough for now.

Here is a link to a very helpful website detailing all there is to know about the different types of Intraocular Lens Implants, how they work, and how they’re implanted. 

{I am not a doctor and the medical definitions and descriptions featured in this blog post do not and should not replace those of a medical professional. They are merely there to help give an idea of my situation and experiences. If you are in need of medical advice see your doctor.}

16 Years Old: Right Lens Implant Dislocation, Another Surgery

While I was still healing from the last surgery a few weeks into December I got an early Christmas present (sarcasm). I noticed a ring around the outside of my right eye. I assumed it was the lens but when we went to my optometrist he said everything looked fine. I knew that even though he couldn’t see a problem that there was one and it would only be a matter of time before we found out what it was. Finally after a few days of the ring increasing in length and thickness I new what was going on. I bent over to get some pajamas out of my dresser and my entire lens slid forward. I could see it sitting in my eye and remembering how I was instructed not to bend over last time so that it wouldn’t lodge in my pupil I immediately stood up. It was different this time, it dislocated slowly where as the left one had done so in an instant. Like the last time I had to wait a week before I could have surgery. I think my surgeon was already booked or something. So, another long, long week of sleeping upright and worrying. Though, I think this time I had every reason to fret. The last surgery had turned out less than what I’d hoped for and my vision with my right eye when it was good wasn’t nearly what it used to be. If the same things happened again I wouldn’t be able to see very well at all. Not to mention the pain. I was terrified I would have to go through all of that pain again. I just tried to remind myself that this would be the 7th time I’ve had to do this, and 5 out of those 7 times the pain really wasn’t that bad.

My surgeon planned to reattach the lens as he had done with the left eye. The first thing I remember after waking up from the surgery is being in tremendous pain. When your in the hospital they usually ask you to rate your pain on a scale of 1-10. First of all let me just say I hate that question because I tend to over think it. I’ve never been in the worst pain imaginable so how can I rate my pain in accordance to this? That being said I kept saying it was an 8. They kept me in the recovery room far longer than I’ve ever had to stay there and would return every five minutes to ask me if the pain had lowered -which it hadn’t- so, they would administer more pain meds via IV. After an hour or two of this they finally wheeled me back to the holding area. I just remember laying curled up in a ball on the bed clenching my fists and waiting for them to bring my mom in. Sometimes a girl just needs her mom. But they didn’t bring her in for a long time after that and they still couldn’t get my pain under control. See people in my family (me, mom, brother), tend to not respond much to pain killers as well as anesthetic. Like for instance if the dentist needs to freeze my mouth it takes a lot of freezing and a much longer time for it to kick in on me than most people and my mom doesn’t freeze at all. And with Tylenol 3 which I take for pain most of the time I can take two at once and will feel nothing. So these heavy duty pain meds; morphine, demerol, oxycodone, and whatever else they attempted were doing nothing to relieve any of my pain. Meanwhile, I was told that my eye had hemorrhaged again -the reason for all of this pain? Eventually I just began vomiting all of the medication back up. My body had had enough of that, especially considering I didn’t have any food or water in my system to help it cope. I was in the hospital for 9 hours after my surgery (as apposed to the usual 2 hours) but eventually I just wanted to go home and sleep and since nothing they tried seemed to help the nurses didn’t oppose. I was still getting sick by the time we got home but despite everything I was wiped enough to fall asleep. This recovery time was the longest by far. It took months and months before my vision had officially reached it’s potential and the pain finally went away. Sadly for me, my vision didn’t return to nearly what it had been. No double vision or floppy iris, which I am very thankful for, but for some reason now I can’t see close to as well for distance as I used to be able to. This has all been very hard to adjust to. But I try to remind myself often that I am extremely lucky for the vision that I do have and that many others could only hope for such. I am not blind, I can see. And that will have to be enough. The hardest part for me is having been given that gift, only to have it taken away again. But I’m still thankful for the time I had with my implants when I could see so well and felt as if they had been mine all along. I still hold out hope that one day they will be able to fix my vision. But until I can be sure I won’t go into surgery and have there be a chance of an even worse outcome I will learn to live and cope with my new vision.

{I am not a doctor and the medical definitions and descriptions featured in this blog post do not and should not replace those of a medical professional. They are merely there to help give an idea of my situation and experiences.}

16 Years Old: Floppy Iris Syndrome & Another Surgery

I went to my local optometrist for a post operative check up and to voice my concerns about my new vision problem. He said that I had what he called “Floppy Iris Syndrome” (sounds made up, I know). Again he reminded us he could only offer speculation but he said it appeared that because my implant was placed further back this time to prevent it catching on my pupil and rubbing my iris that there was nothing to support my iris and so it was “flopping”. This made perfect sense. Back we went to Vancouver to consult with my ophthamologist. He decided that this time he would implant a new lens. My eye was still red and not yet fully healed so the thought of them cutting into it again so soon didn’t seem right to me. But he assured us it was best to do this right away as opposed to months or years later. So again -a month and a half later- surgery was booked for the following morning. I felt doomed this time for some reason. I felt that something would go wrong again and that I would not be able to see as well. Not long after I woke up from surgery my mom informed me that yet again at the last minute my surgeon had decided not to implant a new lens and to instead reposition the old one. I was really irritated and a bit disheartened, but reminded myself that he was the surgeon and he knows best. This time my pain level was a lot higher it had ever been with my other surgeries. Not unmanageable but higher.

The day after the surgery though, would end up being one of the hardest things I’ve ever gone through. I woke up in a tremendous amount of pain. It hurt to look anywhere, but if one eye moves so does the other. When we got to the eye doctors for my checkup (with a surgeon’s helper, not my actual surgeon) I was very aggitated. I had done this 5 times before, 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. Usually I wouldn’t flinch but this time I was in so much pain that the thought made my stomach turn -and 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. Of course the doc thought I was just being a baby he didn’t know me or my history, until my mom promptly explained to him that even at four years old I had not complained once during all of this. This was not the same, something was wrong. I have a very high pain tolerance. After all I’d been living in pain literally everyday for the past 2 and a half years. And so she knew the amount of pain I must’ve been in. But things were about to get much, much worse.

To find out what was wrong I would need an ultrasound on my eye. At first I was certain they must have a different way of doing this one eyes. There was no way they were going to hold that thing down on my eye and move it around. My eye was still mushy and flat, there were stitches sticking out everywhere and fresh wounds, not to mention a drop hitting my eye was really painful and this thing would be much heavier. There was no way. But yes, that’s exactly what they were going to do. I almost swallowed my heart as they explained the procedure. The 20 minutes it took seemed to drag on for hours. My fingers hurt from clenching the chair arms so hard. I could hear my mom sniffling in the corner. I was silent, -I was afraid to unlock my jaw and unclench my teeth- but she could tell by how I was holding my body how much pain I was in. Like I said, I have a high pain tolerance, and this was the most painful thing I’ve ever had to endure. It was like someone was splitting my skull with a sledgehammer and electricuting me at the same time. But, it needed to be done. The ultrasound revealed that my eye had hemorrhaged and was severly inflamed which was the cause of so much pain. This could be very damaging to the eye if left untreated. I was immediately put on a high dose of steroid drops four times a day (more pain!) along with steroid tablets to help the healing and zantac to protect my stomach from the steroids. I was also on a few other drops that I always take after surgery starting four times a day everyday. I was a bit traumatized the rest of the day, but proud that I had handled it -not that I had much of a choice. I was surprised to know how much pain we are capable of dealing with when left with no options though.

My eye (because of the complications) was taking a much longer time to heal than it had after the previous surgeries. I was edgy and wanted to know if my vision would be back to the way it used to be before the floppy iris. After all of the pain and worry, the drops, the traveling, the money, this surgery turned out worse than the last one. I didn’t have the floppy iris now, but I had severe double vision. Along with that my vision itself had decreased considerably compared to before. I could no longer read nearly as well as I had been able to (my left eye is my nearsighted eye). I double vision I’m told is a hazzard of the type of lenses I have, along with the size of the lens. It is rare but sometimes people do end up with these visual aberrations. The name of the aberration that closest fits mine is Coma aberration.

Above is a photographic example of the coma aberration. On the far left is how a normal image would appear (in my case). The middle is how a slightly distorted image in my field of vision would look, and the far right is how bright things like lights, street signs, televisions etc, always look. This has and will prevent me from ever driving in the dark, though I suppose I’m lucky I can drive at all. Still I’m sure that not being able to get myself around after dark will prove to be an obstacle time and time again in the future.
 {I am not a doctor and the medical definitions and descriptions featured in this blog post do not and should not replace those of a medical professional.  They are merely there to help give an idea of my situation and experiences.}

16 Years Old: Dislocated Lens Implant & Surgery

It’s odd writing about my past as if it were somebody else’s. Some of it seems like another lifetime ago. The events in this blog post however were not all that long ago. It has been about a year and a half now since all of this took place.

What I’m about to write about, for me, has had a huge impact on my life. It was summer, a few weeks after my 16th birthday. I was sitting right where I am now, talking to my mom. I blinked and when I opened my eyes I couldn’t see a thing out of the left one. It was all a blur. It was exactly the same as when I didn’t have implants and would take my glasses off. I knew immediately that my lens implant had dislocated – and I was right. My optometrist knew I would need to meet with my ophthalmologis so that we could figure out what to do but this could not be done for a week because he was in China at the time. I was instructed not to bend over and to sleep upright so that the lens would not lodge into my pupil. Let’s just say it was a long week. When I was younger we were told that if anything happened to my implants that they would have to take them out and that would be it. I sat there thinking that I had lost the most precious gift I had ever been given. Sure I could see out of one eye, but it would be back to contacts and glasses regardless. Unless I wanted to remain blind in one eye. I wondered if I had appreciated my lenses as much as I should’ve. If I had remembered to stop and be thankful for the gift I’d been fortunate enough to receive. And truthfully, I had.

The week slowly dragged on until it was time to travel back to the big city. After an 8 hour bus ride (courtesy of Shriners, without which we would be lost!), we arrived at the Easter Seal house yet again. Luckily for me my ophthalmologist explained that technology had come a long way since my implants and that he would without a doubt be able to replace it. So surgery was booked for the following morning and I went through the motions of preperation. No food or water past midnight, arrive early to be checked in and hooked up to the IV etc. I was feeling nervous as one would, but optimistic. I felt certain all would be fine. Though, it wasn’t. My surgeon decided at the last minute to reattach the old implant instead of implanting a new one. To implant a new lens is a much bigger surgery altogether. It requires a much bigger incision and a longer healing time. At first I was angry at this decision as I thought it was the implant that was the problem, but now I’ve come to realize that may have been the best move.


Taken a month and a half after the surgery.

My surgeon’s decision wouldn’t be the problem though. My eye healed fairly quickly but as my vision returned I noticed a very distracting visual disturbance. Wherever I looked my vision would bounce. It was similar to looking through a bottle of water as the water waved and distorted everything I was seeing. We went home hoping that this would get better as time went on. But it did not.

{I am not a doctor and the medical definitions and descriptions do not and should not replace those of a medical professional. They are merely there to help give an idea of my situation and experiences. If you are experiencing any health issues seek medical care.}

7-10 Years Old: Contacts & Lens Implants

 
Despite wearing the glasses that made me so self concious I was very outgoing and able to make a lot of friends. Being kind always helped me in that avenue. But yes, there were times that I was teased and I never stopped longing to be rid of the glasses. So, when the opportunity arrived for me to get contacts at the tender age of 7, I didn’t hesitate to let it be known that I wanted them. I wasn’t able to put them in on my own at the time, especially since they were clear and without glasses I couldn’t see a thing. It was a pain in the butt to do everyday, but I welcomed it. I loved them more than anything. Finally knowing what it was like to be “normal” made it even harder for me to be seen in them. I stopped going on the school swimming trips, and I was afraid to have new friends sleepover because of night time when I’d have to switch them out for my glasses. My friends were always really good with them. None of them seemed to ever really even notice and if they did they did a good job of keeping it to themselves.
 
Three years with the contacts we seen a report on the news about these top of the line lens implants that were being given to cataract patients to replace their damaged ones. I almost swallowed my heart! Could I be a candidate for this? I didn’t have cataracts but it was basically the same thing. Turns out, yes, I was. At first we were told nothing could be done for my eyes until I had finished growing. But for some reason or another they decided I would be a fine candidate -at ten- for what is known as Intraocular Lens Implants.
 
After a month or two of decision making and prepping on my opthamologist’s end I was off to Children’s Hospital again. The surgeries were booked one week apart and just as they had 6 years ago they both went well. There was a minimum amount of pain and my vision cleared up fast. I recieved Conventional IOLs which are the most commonly implanted and basic IOLs. Although, they did something I’m told is not all that common when it comes to implants. They implanted a far-sighted lens in my right eye and a near-sighted lens in my left eye. They were hoping this would help me to read as well as with seeing distance and it ended up being a very good decision. This was the first time in my entire life that I was actually seeing out of my own eyes and it was incredible. No glasses, no contacts, just my eyes. It was the most amazing experience I’ve ever had and it is a feeling that I will cherish for the rest of my life. 
(I am not a doctor and the medical definitions and descriptions do not and should not replace those of a medical professional. They are merely there to help give an idea of my situation and experiences. If you are experiencing any health issues seek proffesional medical care.}

4 Years Old: The Beginning – Dislocated Lenses

The first time something was ever thought to be wrong with me medically I was four years old. My mom took me to the doctor because she thought I had a lazy eye. Little did she know I was actually found to have “bilateral ectopia lentis“, meaning my lenses were dislocated. The left lens was dislocated up and out; the right down and out. At the time I was only looking through the edge of my lenses. My vision in the right eye was 20/80 and 20/160 in the left, 20/400 at distance. It was hard for people to imagine, that I had never been able to see much a day in my entire life. Yet somehow I had always managed to do things like color in the lines of coloring books.

Next thing we knew we were being sent eight hours away to the closest Children’s Hospital so they could remove them via two surgeries (bilateral pars plana lensectomy). It was a long time ago but from what I remember – and what I’ve been told, I responded to and recovered from both surgeries well. I don’t remember much pain involved in either of those surgeries. Without the lenses I was as good as blind, all I could see was a bunch of fuzz. I was given bifocal glasses for the next 6 years which magnified my eyes and made me really self concious. Even at four the first time I seen myself in them I thought “I’m never playing with anyone ever again. I’ll have to play by myself.”
This was not a normal thing for a child to be born with and so came the first of many times in our lives the word Marfan Syndromewould be brought about. At the time not much was known about the disorder. My parents had not the slightest clue what it even was. At the time though, it was thought the prognosis for a person with the disorder was poor and that those with it would not live a very long life. It would be weeks before I would be evaluated and my heart checked for signs of the syndrome and so my mom chose not to learn anything of the disorder. She couldn’t handle the thought, especially if it might not even come to pass. Although, my grandma took the liberty of researching what little info she could get her hands on and what she learned told her I would not live long. I’m told she spent an hour or so that day screaming at God in the field of their property. At the time I didn’t have many physical characteristics of Marfan Syndrome other than my height and the fact that my fingers were long and slender. The big ruling factor would be whether or not an Echocardiogram showed any dilation of my aortic root etc. This is a major manifestation of Marfan Syndrome and 90% of those with the disorder will eventually need open heart surgery to replace the dilating part of the aorta. Lucky for me and my family, my Echo came back clean. I was out of the woods, or so we thought. We were told then and there that I didn’t have Marfan Syndrome. What we weren’t told is that it cannot be ruled out at such a young age because a lot of the symptoms including the heart problems do not develop until later in life.

But for now it was all over and life could go back to normal for a while. I could see for the first time in my life and the entire trip back home I wouldn’t take my eyes off of the scenery outside. I still hated the way my glasses looked, but I loved how well I could see with them.

{I am not a doctor and the medical definitions and descriptions do not and should not replace those of a medical professional. They are merely there to help give an idea of my situation and experiences. If you are experiencing any health issues seek proffesional medical care.}