Saturday, 25 June 2016

My Scoliosis Story | International Scoliosis Awareness Day

Today is International Scoliosis Awareness Day and for the first time I'm going to share my whole scoliosis story with you. I've done a lot of posts in the past about my various surgeries but have never gone into loads of detail about getting a diagnosis, why I have scoliosis and the problems I've had since having surgery. I've got to be honest I know this isn't going to be an easy post to write, also it's going to be a long one, because my spine is the main problem I have in regards to pain and it's the biggest reason why I now use a powerchair to get about but if me sharing my story helps raise awareness or shows at least one person that they aren't alone then it's worth getting a little emotional over it.

scoliosis marfan syndrome spinal fusion surgery operation awareness

What is Scoliosis?
Scoliosis is the sideways curvature of the spine, it can curve either way and it can also twist. You can have either an S shaped curve, which appears to be more common, or a C shaped curve (this is what I had). There are many different kinds of scoliosis but the most common is adolescent idiopathic scoliosis, this just means that there is no reason for the curve developing, it just happens. In my case I have what is called Syndrome Scoliosis, this means that my curve developed as a result of the genetic condition I have called Marfan Syndrome. Scoliosis is also seen a lot in conditions like EDS (and other hertitable disorders of the connective tissues) and in conditions such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy.

My Diagnosis
Since we knew that having Marfan Syndrome would mean I had a higher change of developing scoliosis my Mum always looked out for the signs such as uneven hips, shoulders and ribs and a difference in leg length when I was growing up. She took me to the doctors when I was quite young as she saw a curve developing in my spine but due to doctor's lack of awareness of how quickly conditions like scoliosis can develop in those with Marfan her claims were dismissed and they didn't even do an X-Ray.

Fast forward to when I had just turned 13 and I started to notice that my ribs were uneven, they stuck out more on one side than the other. I knew something wasn't right and after ignoring it for a little while I finally showed my Mum and soon enough I found myself at the doctors. My GP referred me to straight to see a scoliosis specialist at the Royal National Orthopedic Hospital Stanmore (RNOH). I had some X-Rays done and an MRI and my curve was about 25 degrees at this point, my consultant told me that he didn't predict that my curve would progress much further and that I wouldn't need any treatment.

scoliosis marfan syndrome spinal fusion surgery operation awareness

Monitoring 
At that point there wasn't much any of us could do but keep an eye on things, in the beginning I didn't really have much pain, just a bit of back ache. When I was about 14 though things progressed rapidly, in just a 6 month period my curve progressed by about 20 degrees leaving me with a nearly 50 degree C curve. This rapid decline often happens in those with Marfan but it still shocked me. I knew something had got worse because my pain also got a lot worse. It was during Year 11 whilst I was doing my GCSEs when I was 15 that things got bad pain wise and my attendance dropped a lot. I wasn't able to climb the 2 or 3 flights of stairs up to some of my lessons so often did work in the library on my own and I also had to drop one of my GCSE subjects so I could focus on passing the more important ones.

I managed to drag myself through that year somehow though and just before I started my exams I was given a date to have corrective spinal fusion surgery.

scoliosis marfan syndrome spinal fusion surgery operation awareness
scoliosis marfan syndrome spinal fusion surgery operation awareness
scoliosis marfan syndrome spinal fusion surgery operation awareness
Surgery
I was meant to have my surgery in July 2013 but on the day, when I was ready to go down to theatre and I had prepared myself greatly, it was cancelled. I'm not going to lie it was pretty devastating, I'd been in so much pain for so long and was ready to have the surgery. The anaesthetists had failed to read my notes correctly during my pre op and at the last minute were suddenly panicking about the heart problems that come with Marfan. Before they would do the surgery they said I needed the go ahead from my cardiology team, who I wasn't due to see until September. I finally got the okay from them though and my surgery was set to happen in early December.

Having been previously let down by my team I was extremely anxious going into the surgery. I went into hospital the night before for some final tests and was wheeled down to theatre the next day at about 1pm. I'm not going to lie I did cry my eyes out in the anaesthetics room (although I'm a pro at this by now and look forward to having a nap whilst surgeons fix me!). By the time I knew it though it was the evening and I was waking up in recovery. Surgeons spent about 5/6 hours straightening and fusing my spine using titanium rods, screws and hooks. Sounds painful doesn't it, well that's because it is. I don't remember much about the surgery but what I do remember is the pain and reaching for my morphine button a lot. I spent one night in HDU and then was taken back to the ward. This post would be way too long if I went into detail about my whole 8 day hospital stay so if you want to know more about my initial recovery then I did a post about my first week in hospital, My Scoliosis Surgery/Recovery Week 1 in Hospital.

The best way I can describe the pain is it felt like I had been hit by a lorry if I'm honest, every time I moved it got worse which is great fun when nurses have to turn you in bed every couple of hours. Sitting up and walking for the first time was tough but I was proud of myself, you basically have to learn how to hold yourself up and walk again as your body changes so much. I cried when I saw my new figure in the mirror for the first time, I hated my body before and for the first time my waist and hips were more even. Even now my ribs are still pretty uneven but it isn't really noticeable through clothes so it isn't a big deal. Obviously I also have the addition of a huge scar but scars have never bothered me, I totally embrace mine and think its pretty badass!

scoliosis marfan syndrome spinal fusion surgery operation awareness
Complications
7 days into my hospital stay during the early hours of the morning I developed a very severe and intense pain down my right thigh and knee. It was by far the worst pain I had ever experienced, minus the pain a few days after surgery, and we honestly had no idea what was going on. My first idea was that it was nerve related but doctors disagreed, blamed it on my Marfan Syndrome and got physio to give me a few exercises. We thought the pain was gradually getting better but every time I sat up or stood it got a lot worse again but I was discharged without having any proper investigation.

I saw my surgeon at 3 Months Post Op  and once again the pain wasn't taken very seriously despite the fact that I had not returned to school yet and had barely left my bed. They reluctantly sent me for a MRI scan which didn't show anything major but luckily when they sent me for a CT scan at 6 Months Post Op we found out what was causing the pain. Turns out I had been right all long, it was a nerve problem. The screw on the right side of my L4 vertebrae was sitting on a nerve which meant that whenever I sat or stood the pressure that was put on it caused my severe pain. I was very quickly put on the waiting list to have a second surgery to replace the screw and put it in a better position.

My surgery was set to be done in mid September, almost a year after my first surgery. I was in hospital for about 5 days this time round, a little longer than expected due to them not being able to get my pain under control but it wasn't anywhere near as bad as the first surgery. I was told that I might not feel the effects and get any relief from the pain for a few months as my nerves needed to recover but at just One Month Post Op things had already improved a lot and they continued to do so. These days I only ever get the nerve pain very rarely and mildly so the operation made a big difference.


Chronic Pain
At this point I thought it was over, I would be able to go to college after having to leave school just a few months into my A Levels but that was far from the truth. For the next 2 years the lower back pain that I had experienced before either surgery started to return. At first I only had to rely on a crutch to get around but quickly my mobility deteriorated and I had to start using a wheelchair. During this time I told my surgical team again and again about this pain but it was never taken seriously until a few months back. After thinking for ages that the pain was related to my spinal fusion it turns out we were wrong, it was a totally different problem causing my pain, you can read about that new diagnosis here. It was a relief to know that the 2 surgeries had not been a complete waste of time, I had a straight spine and it was not the main reason for the severe chronic back pain I had that has left me using an electric wheelchair.

That's a new problem I must take on now but I suppose that is the end of my scoliosis story. I will always have the metalwork in my body and I will always have aches and pains along my spine because of it and I will never be able to bend my spine like others or go on rollarcoasters and trampolines but it's a small price to pay. My lungs and heart are no longing being squashed by the curve and my body confidence has increased so much. The aim of the operation was to improve my pain which I suppose it might have done but this new problem is causing so much pain that I don't think I'll ever truly know how well the operation worked in terms of pain relief but that's okay. I'll be 3 years post op in December and I really cannot believe how fast the time has gone!

If you've made it through this whole post then you deserve a medal for sure! If you have any questions about scoliosis or Marfan Syndrome then leave me a comment below or tweet me (@shonalouiseblog), alternatively there is loads of great information on the Scoliosis Association UK website.
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2 comments

  1. You're truly an inspiration Shona. What you've been through at such a young age is astonishing, and while it definitely sounds like things have gotten really awful at times, you seem to have handled it with such strength and grace.
    I pray for some miracle treatment to come about that can help your new condition, but for now it's great to hear how your power chair is coming along.

    I've been following you for a long time, but took a little break and lost your blog for a bit, but it was great to read about your journey and I'm looking forward to continuing reading it.

    Good luck with everything.

    Tasha

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