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

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