The Perspectives on Scoliosis stories first appeared in the Information Point newsletter Our World in 2011 following a webinar in which the Myotubular Trust, the Joshua Frase Foundation and Cure CMD joined up to help patients and families with myotubular and centronuclear myopathy and congenital dystrophies. The webinar was a free web and audio facility for all attendees and involved perspectives from eminent surgeons and clinicians in both the US and UK. To coincide with the webinar, Our World featured information about scoliosis and interviews with people who had experience of scoliosis surgery. Shaun’s perspective is below.
What form of CNM/MTM do you have?
I still don’t know what form I have, and am currently waiting for results of tests to find this out via my doctor who has sent bloods etc to the London lab for genetic testing centre at Guy’s and St Thomas Hospital. I hope to hear soon. I had had a biopsy done at one year but that was nearly 20 years ago and very little was known about the condition then. They said that it didn’t show anything. I was meant to have another at age seven but they didn’t recommend me undergoing surgery, so they did it as part of the scoliosis operation and that’s when they told me I had a form of myotubular myopathy, but they haven’t been able to say yet which gene is affected.
How old were you when you had scoliosis surgery?
I was 12 when I needed surgery, in my second year at secondary school. I am now 22 years old.
Where did you have your scoliosis operation performed and by who?
I had my operation at Bristol Children’s Hospital – by Mr Nelson and another surgeon who I can’t remember. It was a 9 hour operation so they split it between the two surgeons.
Can you describe what issues you had which lead to the need for surgery (eg curvature, discomfort, difficulty with ventilation and squashed lungs, difficulty sitting)?
Before I had my operation it was becoming dangerous to breathe, because the bend of the curve in my spine was squashing one of my lungs. The surgeons originally wanted to do Scoliosis correction surgery when I was 16 after I’d gone through my growth spurts, but my breathing was becoming an issue. I also had a big S shape curve, my hips went one way, my back went another way and my head another, but fortunately it counterbalanced all the way up meaning that I was still able to walk, but not brilliantly. Sitting was okay, although I couldn’t sit back in a chair or anything.
Were you given anything to help alleviate the symptoms you had before surgery, and did it help?
I was asked to wear a body cast, from about the age of 9. At first the curve was only 10% so not too bad and it was easy wearing the body cast, but two years on, it began to get uncomfortable because my curve increased, this was even though I was getting fittings for a new body cast every so often. At first I wore the body cast for 7 hours a day, but at one appointment later on the consultant said it should be worn over 24 hours! I couldn’t bear it – it was worse at night and really tight on the chest so I felt I couldn’t breathe in it. So in the end, I just used to take it off to sleep.
What did you feel about the prospect of corrective spine surgery?
I wasn’t too impressed at first about needing it, because I was only 12 and it seemed scary. But I knew I needed it really so I started to accept. I spoke to one other person’s mum about surgery – he didn’t have MTM or CNM, and that helped but it also scared me – because she said he was in hospital for three weeks and that sounded horrible!
How did you prepare yourself for surgery – do you have any hints or tips?
I mainly I rang up the other person’s mum and tried to find out what the operation was like. I asked the surgeon a lot of questions too. I was exercising anyway so didn’t need to do anything different than I was already doing. I would recommend that you speak to as many people as possible about it.
Describe the first days immediately after surgery and what things did you do to try to cope and get through it?
It took a while for me to come around after the operation properly – I was quite ‘high’ on the painkillers – out of my head – and I remember it felt really unusual, I kept seeing faces and didn’t know who they were! But I didn’t feel any pain, I just felt weird and a bit stiff.
I was drifting in and out of sleep a lot as well the first two days. After that it started to get better and all I felt was stiff. I was on an Intensive Care ward for five days after the operation and then went onto a normal ward for four days so I was only in hospital for nine days in total. I got through by watching lots of TV and Mr Bean Movies, but it was boring on the wards so I got up and started moving around quite quickly after the operation just for something different to do!
After the operation the doctors came in and said that my breathing was still dangerously poor and they decided I would need bipap. At the time they didn’t know what my condition was either. So not only did I have to have scoliosis surgery, but I also had to start night time bipap ventilation. Shortly after I’d had the surgery I also found out that I had a form of myotubular myopathy.
Everyone buys you loads of things when you’re in hospital so you don’t need to take anything! I did take a Game Boy computer console in though to keep me busy. I felt okay about having visitors when I was in hospital. Obviously, only family came into see me in intensive care, but on the ward everyone could come in and at one point I had seven people around the bed!
What happened when you went home? Did you need much time off school?
When I got home I fell over the first day. It was a bit frightening falling so soon after surgery, but fortunately no damage was done. I was at home for 2 1/2 months before I could go back to school. I had a tutor come in and help me do school work, but I never managed to catch up with my language courses again after so much time out of school.
What things do you think most helped you recover from surgery (eg; equipment, nursing help, activities, exercise, friends, etc, etc)?
Just taking it easy and walking around but not going mad, but relaxing, and not pushing myself too much. I went back to school for half a day at first, because I felt too tired to do a full day after so much time off, but it didn’t take me long to get back to school full time.
When did you start to exercise again and what type of exercise did you do?
I started exercise again pretty quickly. They got me sitting up two or three days after the operation in a chair and it felt pretty weird, and after the 3rd or 4th day I started to walk again. I visited upstairs and walked around the hospital but they took me up in the lift. I felt really tall afterwards – in fact I grew six inches taller! You should measure yourself before and after the operation because you might be surprised by how much you do grow.
I like to exercise as part of my general routine and always have done. I train six days a week, in a routine where I work out a different muscle group each day, just to keep my muscles strong and intact. I started to do those exercises again from about two months after my operation, and until then just took things easy.
On reflection, is there anything you may have done differently?
No, I wouldn’t do anything differently.
Is there anything that you can’t do after surgery? Or perhaps there are things that you can now do that you couldn’t do before?
Well, I can’t do roly polys any more, as I used to be able to do them. I can’t twist like I used to. But I can walk a lot better now after the surgery and my back is generally a lot stronger.
On balance, are you glad that you had scoliosis surgery done and has it improved your quality of life?
Oh yes I’m definitely glad that I had the scoliosis surgery done, it’s made my life a lot better and built up my confidence at lot.