Hannah Richards
On the 22nd of January, 1992, I made a dramatic entry into the World, with a small curve in my back, being delivered by caesarean section because I was stuck in the flexed breach position. Within days, the paediatric physiotherapist who had treated another girl with my symptoms diagnosed me with Beale-Hecht syndrome. The doctors weren’t sure what to do so they watched and waited; for too long.
At first, the local orthopaedic consultant just put my curve down as positional but in the middle of May, over just a few days, my whole rib cage twisted. It was only by August, when my curve was really pronounced (my mum guesses around 100 degrees) that they referred me to a back specialist and put me into my first brace in September. With over a 6 month wait for a brace, this delay meant that they could only hold off and try to stop any further twisting or curving, instead of using the brace to straighten out my back. I can’t emphasise enough how much early detection really does make a massive difference! I had a plaster jacket from that moment on until I was 10. From then I had a removable brace – I could bath properly at last!
I have an S shaped curve syndromic Beales syndrome and I have the Marfan heart dilated aortic route and flop mitrovalve, which is checked yearly. I also have arachnodactyly in the hands and feet. The curling of the feet can make it difficult to find shoes, they have to be quite soft and my hands make touch typing difficult and other tasks that require a flat hand, especially when something gets stuck behind the sofa!
In 2001, when I was 9 years old, I had spinal fusion to my back. I was in hospital for a month and a half, and had two operations and the traction bed in between; my mum and I called it the modern-day rack. Fortunately, I have a very dedicated and caring mother who stayed with me every day, and she cooked me food (I’m a bit picky and the hospital food was disgusting!), she washed me and she fought with the doctors about when more weights should be added. They wanted to add more and more weight quickly, while my mum knew I needed more time. Anyone who’s about to have the spinal operation listen to yourself and your body and your mum or dad. I’m not saying don’t listen to the doctors, because they do know more than you medically but they might think you’re coping when you’re not, so listen to your body. Also, make sure they have the correct amount of morphine as well; it’s surprising how much is needed. For an hour or so (well at least it felt like an hour), my morphine dose reduced and the nurses were hurrying about making more at the correct amount but the pain for that hour was near unbearable.
At my second operation during which they put in the two rods, they couldn’t straighten out my back as much as they liked, my oxygen stats went down by 20 and they had to stop. We think my curve is now around 60 degrees, but there is some concern that it might be twisting around the rods, one of which has snapped at the neck but it’s not moving which is very good. I still have the staples that they used in my back in a pot that they gave to me. Some friends visited which was great and they decorated my halo–traction thing that was in my head with stickers. By the end of my stay, I had lots of get well soon posters from my school class and a couple of presents from friends, family and families who were in the ward as well, which really helped keep my spirits up.
When I came out of traction I was very weak and thin and I had to build up my muscles all over again. My mum modified some knickers by cutting the sides and sticking Velcro down the sides so I didn’t have to move my legs and she also bought dresses that could be buttoned all the way to the top and bottom so I didn’t have to move much to get dressed. After traction, my neck went right over to my left shoulder, hence why in the photo by Lord Lichfield my head was severely over to the left. None of us had twigged that my neck muscles wouldn’t be strong enough to hold my head up. If you’re going to have a spinal operation be sure to give support to the neck afterwards. I still have plaster jackets, even after my operation. They added a lip on the left shoulder to encourage my neck back into the centre; it’s not quite central but it is hugely better than before. I never had brilliant health and after the operation I was tired for quite a while and over the summer I had to build my muscles back up to full strength. With all my time off school, I missed half of year four but I still managed to keep up in 5th year.
The photo shoot by Patrick Lichfield for the SAUK Getting it Straight campaign was a blast and a half! We arrived with my tutu which we had bought just weeks before, and Lord Lichfield greeted us with a smile. Linford Christie was also there having his shot and he was also very welcoming. Lord Lichfield’s assistants whisked me off into the back where they did my hair and put on a tiara – a tiara! (What! I was 10 and excited). I put on my tutu and then I had to sit on a box and smile with a sparkler in my hand (with the fireworks ads running through my head!) and apparently I looked great.
As I went into secondary school, I was finding it hard to have enough energy for the whole day and missed a lot of school. When I fell and damaged my left knee in 2005, I took a long time to recover and still have scar tissue from when they operated. This setback meant that I missed even more school and became even more tired. For my GCSE’s, which I’ve thankfully just put behind me, I got 4 A*s, 3As and a B, which equates to eight that may not be as many as my peers but they don’t have my limitations or easy exhaustion and I’m really happy with my results. I did two of my subjects by home learning, where I was given a large folder with work in it and I could call my tutor if I got stuck. It did require a lot of determination and effort. However, I could at last do half a day without missing the other half of my lessons which meant I wasn’t stressed about catching up.
Health wise, I still get tired after long days, but I can usually last a lot longer now. At school I never did PE, which I regret, since although I can’t do cross country running, small muscle exercise really does help back pain. I now do Pilates once a week which helps and I probably should do more, but without a zest for sports I’m a little reluctant. I still get back pain and when it’s really bad I do wear my plaster cast. However, it is really cumbersome so instead I try to use cushions to alleviate the pain, and doing Pilates back stretches can help. I dislike using pain killers because they don’t solve the problem and only mask it, but if it’s really bad then I have to. Good posture and small bits of exercise really help the pain. I also have to use a wheelchair for long distance walks, and although I hate the wheelchair and how heavy and obvious it is, it means my back’s not sore from the walk. Also, I never wear very high heels. I have one or two really soft 2 inch heels, but that’s the highest that I’ll wear, and never stilettos – they might look good on the shelf but they won’t when you go splat on your face! I can’t swim because I had my plaster on from when I was little and I tried later when I could remove it but got too tired.
I have some really good friends who help me to carry my bags and who are there for me if needed – even when accidentally tipping me out of my wheelchair onto a curb! We have fun and laugh, but I know my limits and I know the things I can and can’t do. I won’t say it’s fun to watch as others do fun things but I won’t try and attempt things when I’ll only get more pain. So, what I am saying is know your limits and don’t push yourself too far – you’ll regret it the next day and possibly longer, but you can still have lots of fun too.