Perspectives on scoliosis: Sukhi’s perspective

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. Sukhi’s perspective is below.

What form of CNM/MTM do you have?

Congenital Myotubular Myopathy, but I’m not sure which one yet.

How old were you when you had scoliosis surgery?

When I was 18 years’ old, I am now aged 20.

Where did you have your scoliosis operation performed and by who?

I had my operation at the Royal National Orthopaedic Hospital under Mr Lehovsky.

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)?

I was permanently sat forward, leaning on my elbow for support only having one hand free and sometimes not having any hands free. I also had shortness of breath, so that when I spoke I would say individual words before needing to take another breath. This is because my lung capacity was restricted.

Were you given anything to help alleviate the symptoms you had before surgery, and did it help?

I did have a brace which I wore but by this point it made no difference to my posture.

What did you feel about the prospect of corrective spine surgery?

I was really scared, I didn’t want it at all as I have had bad experiences in the past which has clouded my judgment in having spinal surgery. This was because my previous operation was a tendon transfer and they did not manage my pain well at all. Therefore my fear about going through a spinal fusion was based on my fear of pain.

How did you prepare yourself for surgery – do you have any hints or tips?

I didn’t really prepare for it, I buried my head in the sand and got upset when people spoke about it. Then, my parents collected me from college one day stating they had a date for the operation which was in a few days time. Looking back I’m glad it was done this way as I had less time to be scared and it forced me to realise how bad things had got.

Describe the first days immediately after surgery and what things did you do to try to cope and get through it?

This is difficult to remember because they started to operate on me but then had to abort due to some problems they faced. (Please don’t be afraid by this as this was just something that happened to me and is not normally what happens when going through spinal fusion). So, after this I required a tracheostomy and the fusion went ahead. I was heavily sedated for about a week after the operation, and I certainly don’t remember pain from the back operation but naturally when I came round my family were concerned and they kept asking me was I in pain? My reply was no I wasn’t. The tracheostomy was temporary so I no longer have that.

What happened when you went home? Did you need much time off school?

I was attending a residential college at the time, so I went to college from the hospital with a short weekend home for my birthday. I returned to the college where I received the care I needed but did not go back to my normal lessons until about four weeks later when things were ‘back to normal’ ie being able to be back in my chair all the time.

What things do you think most helped you recover from surgery (eg; equipment, nursing help, activities, exercise, friends, etc, etc)?

Support from friends and family and the good nursing care I received from ITU really helped me recover. At first I was only able to sit in my wheelchair for a little bit of time but as things improved I was able to get back to being in my wheelchair as normal. This was still worked on when I went back to college.

I was not able to sit up after surgery until I had been fitted with a brace. This, for me was the hardest part as I find it very uncomfortable to lie on my stomach. The brace is only temporary and fitting it is done in two parts – you lie on your back to do the front and then they remove that and turn you onto your stomach to do the back. Then, when you get the brace it is in two pieces – so to put this on you roll on your side, the back piece is positioned and you roll into it onto your back then the top half is placed whilst you are on your back and then it is fastened. The brace wearing after the operation was only a temporary thing, but I can’t remember how long this was needed for exactly. Boredom during the recovery period was an issue!

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?

Before I had the operation I would be lifted everywhere i.e from my wheelchair into the car. But after the operation this is not the case. Hoisting is the way I had to be transferred but this will need to be discussed with the surgeon beforehand so that necessary arrangements can be made to provide you with hoisting equipment, etc if you don’t already have it.

Surgery has meant that I have both hands free, no brace and I can wear anything without being conscious of a brace showing. Having both hands free, for me, is the main benefit from the surgery, because I am now able to do simple things like using a Wii remote more easily.

I can also now swim on my back using just a head float. It has always been difficult in the past to do this because of poor neck control. The increased lung capacity is also a major benefit.

On balance, are you glad that you had scoliosis surgery done and has it improved your quality of life?

Yes, I’m glad I had it done.

Perspectives on scoliosis

Perspectives on scoliosis: Steven’s perspective

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 patients who have had scoliosis surgery. Steven Pepi gave his perspective on son Steven’s surgery. At the time the Pepi family, dad Steven, mom Michele and children Nicole and Steven lived in New Jersey, USA. Steven had turned 10 years old on 25 July and in March had undergone scoliosis surgery. The operation was the first of three (maybe four) back surgeries to start to correct his severe scoliosis. 

What form of CNM/MTM does Steve have?

Steven has Congenital Muscular Dystrophy. Although not diagnosed – symptoms mirror X-linked myotubular myopathy.

How old was Steven when he had scoliosis surgery?

Steven was nine years old.

Where was the scoliosis operation performed and by who?

The operation was performed at N.Y. Presbyterian Hospital by Dr. Michael Vitale. We made a trip there for an initial visit back in November 2010. During this meeting Dr. Vitale had taken x-rays and confirmed that if we did nothing Steven’s breathing would become more labored and this would lead to other organ problems. It helped us make the difficult decision of putting Steven through this major surgery that much easier. We knew we had to do something and we knew it had to be sooner than later. A couple of months later (January 2011) he had a short hospital stay and we were thinking it was Pneumonia, but it was actually a partial lung collapse. With this, we knew we had to start to get the ball rolling with his back surgery and we knew Dr. Vitale was ‘our guy’.

It was a seven hour procedure – the longest seven hours of our lives! The anesthesiologist had told us that Steven would be sedated for 24 hours to help with the pain. We were antsy to see him as soon as possible and as they were wheeling Steven out of the operating room, we snuck by the elevators to catch a peak and he was alert saying “Let’s go home … ” What? You just had major back surgery and you want to go home? It is amazing the strength these kids have.

Can you describe what issues Steven had which led to the need for surgery (eg curvature, discomfort, difficulty with ventilation and squashed lungs, difficulty sitting)?

Severe curvature upper and lower spine causing twisting of the spine and rib cage – affecting lung function. Could not sit straight up.

Was Steven given anything to help alleviate the symptoms he had before surgery and did it help?

Full scoliosis vest. Helped with support and slowing the curvature.

What did you feel about the prospect of corrective spine surgery?

We had to get it done. If left alone would have caused more severe health issues.

How did you prepare yourself for surgery – do you have any hints or tips?

As parents, it was one of the most difficult decisions to make. Steven was the one dealing with it and always has a smile on his face. He had a partial lung collapse in January 2011 and the doctor in the hospital at that time who never met Steven before stated “You need to do this surgery sooner than later … the longer you wait the more collapses Steven will encounter.”

Describe the first days immediately after surgery and what things did you do to try to cope and get through it?

We were so impressed on the immediate effect it had on his improved lung function. We noticed Steven’s vent settings were not right when we saw him and when my wife questioned the Pulmonologist, he stated “I actually lowered the pressure support settings in the operating room just after the procedure”. As they straightened his spine the rib cage came back to where it belonged and everything fell back into place making more room for his lungs. Michele spent the night with Steven, and every time he woke up, she would ask him “Are you feeling any pain? Do you need anything?” his reply was “No, Mommy, my back does not hurt.” Again, WOW these kids are amazing. If it were me, I would have been playing ‘Jeopardy’ with the Morphine button.

What happened when you went home? Did Steven need much time off school?

Steven was out of school for quite some time. The risk of infection was great and we had to avoid this happening at all costs.

What things do you think most helped Steven recover from surgery (eg equipment, nursing help, activities, exercise, friends, etc, etc)?

Help from in home nursing and family is a huge part of the recovery process.

On reflection, is there anything you may have done differently?

If we knew months, years ago it would have went as smooth as it did we would have done it sooner. But there is always the doubt in your mind that “God forbid something happens during the procedure…we decided to do this, not him”. Pretty scary….

Is there anything that Steven can’t do after surgery? Or perhaps there are things that you can now do that you couldn’t do before?

Steven is able to now sit up in his bed (plays video games, draws, surfs the net) straighter than ever. His center of gravity is where it should be now (while sitting) so he does not require propping with pillows and no longer has to wear that very uncomfortable scoliosis vest.

On balance, are you glad that Steven had scoliosis surgery done and has it improved his quality of life?

Steven continues with the lowered vent settings and is sitting up straighter with less concern for falling over – prior to the surgery. Steven has a new found love with dinosaurs. He knows the names, if they are carnivores, herbivores or omnivores, the periods in which they lived and so on and so on. He loves anything electronic – The Wii, Nintendo DS, Mom and Sister’s iPhones – he loves all of the talking animal and dinosaur apps and plays with them for hours. He loves the movie The Lion King and has taken on such a passion for family trees comparing it to The Lion King. “Daddy is Mufassa and Steven is Simba”. Whenever family members come over he tries to put the pieces of that puzzle together. “Nana is Mommies Mommy – Pop Pop is Mommies Daddy”.

Perspective on scoliosis

Perspectives on scoliosis: Shaun’s perspective

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.

Perspectives on scoliosis

Perspectives on scoliosis

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.

Scoliosis is derived from the Greek word meaning crooked and is a medical condition in which a person’s spine is curved from side to side. Although it is a complex three dimensional deformity, on an X-ray viewed from the rear, the spine of an individual with scoliosis may appear more like an ‘S’ or a ‘C’ than a straight line.

Scoliosis is typically classified in three ways:

  • congenital (caused by vertebral anomalies present at birth)
  • idiopathic (cause unknown, subclassified as infantile, juvenile, adolescent, or adult, according to when onset occurred)
  • or neuromuscular (having developed as a secondary symptom of another condition, such myotubular and centronuclear myopathy)

Around 3 out of every 100 people have some form of scoliosis, though for most people it’s not a problem. For a small number of people, including children and young adults with neuromuscular conditions in particular, the curve may get worse as they grow and they may need an operation to correct it. While small curves generally do not cause problems and may not be very noticeable to others, larger curves can cause discomfort and lead to respiratory problems when the lungs become compressed.

Shaun’s perspective | Steven’s perspective | Sukhi’s perspective