Friday, April 13, 2012




Children with SMA have weak muscles and in the case of the spine these weak muscles can not hold it straight so an abnormal lateral curve can occur: frequently - according to the paper I have just read, all - children with SMA 2 will develop this abnormality known as scoliosis.  A severe scoliosis can effect breathing and can make sitting more difficult with spinal surgery and correction of the deformity being a treatment option.

Eilidh has a scoliosis and was referred to a spinal specialist by Dr Neuron six months ago.  We were seen recently by Mr Spine, an orthopaedic surgeon for Eilidh's initial appointment and assessment - a formality really and very much a "meet and greet" but necessary all the same.  Eilidh had x-rays taken on arrival which took a wee bit of time to get -

"please try and sit still, eilidh" i said as she tried her hardest to sit on the chair, smiling at mummy who was wearing a really funny (lead) pinny with cartoon animals all over it.
"don't wobble!" cautioned the radiographer - try telling that to a 3 year old with poor sitting balance - but Eilidh did try hard and she smiled at the camera, too!

- but we did get them!

Mr Spine and I talked a little about Eilidh, her SMA and her mobility.  He examined her and then looked at the x-rays, measuring Cobb's angle, which measures the degree of side-to-side curve in the spine, and explained that a "normal" spine will have less than 10 degrees of curve and that anything over 10 degrees is regarded as a scoliosis; E has a 17 degree curvature and so - no surprise! - has a scoliosis.

He continued on to say that surgery is not required at present - as way of reassurance - and may not be necessary as the natural progression of the scoliosis, and subsequent problems as a result, is unknown and can therefore not be predicted.   A spinal jacket or splint may at some point be necessary, but again not just now as she is reasonably mobile and has good trunkal control and sitting balance.  Mr Spine explained that rigid splinting can lead on to further problems as they somewhat limit the use of the abdominal (tummy) and respiratory (breathing) muscles.

With no intervention required at present and no further questions from me, we were dismissed with a "see you in 2 years but please contact me if you notice any changes before then" and a smile.  Another appointment over... phew!

For me the thought of a scoliosis isn't a biggie really - and perhaps that's because of my medical background - and I can easily dismiss it, putting the idea into a box and pushing it to the back of my brain.  But it's not just me that I need to consider and D needs to hear about the appointment and be told a little more scoliosis and its treatment.  So we talk and he begins to worry - for in his mind scoliosis was something of the far, far, far off distant future - and he asks questions and you know what?  I can't actually answer them because I have already consigned the problem to the recesses of my mind and to a future many years away: I realise, however,  that it's ok to say "i don't know" because he knows that I'm trying so very hard to be Eilidh's mummy and not her doctor.  Soon he - quite quickly - too has placed her scoliosis in a box and firmly shut the lid.

Today, only for the purpose of writing this post, I reopened the scoliosis box and read a few articles - and then had to stop my research as I got too caught up in the "medicalness" of it all; this is about my daughter, not a patient or a case study, my little girl...   and this is what I found:
  • "expert opinion" suggests that all children with SMA 2 will require surgical intervention - with this improving quality of life and activity of daily living (1)

  • a non-rigid spinal jacket may be used prior to surgery; rigid jackets can reduce respiratory function (1) (making chest infections more common)

  • there is no data to suggest that spinal jackets will alter the progression of scoliosis in children with SMA but they can provide comfort and  functional support, aiding sitting balance, making the use of the upper limbs easier and could also help breathing (2)

  • post-operative complications are common, particularly in relation to respiratory function (1)
So now I'm a bit better informed - great! - and the scoliosis box has a few tit-bits of information in it; and here I am just putting the lid back on the box and confining it to the "not so far, far, far off distant future":

 until then...

(1) Mullender et al "A Dutch Ggideline in the treatment of scoliosis in neuromuscular disorders" - the full article can be found here
(2) Kotwicki er al "Bracing for neuromuscular scoliosis: Orthosis construction to improve the patient's function" - an abstract can be found here


Lynsey Summers said...

This is a good thing to do, you have released some of the worry from your mind by posting, then filed it back away until the need to think about it again. I do this when it comes to my sons hips (he has Cerebral Palsy and has had to have two big hip operations already, we just got news a 3rd isn't required yet :-). It is nice to think you have a while to get your head around dealing with stuff. As and when, it's the only way forward! So pleased it's boxed until 2014 for you all.x

BavarianSojourn said...

You are dealing with it very sensibly. Close the box and open it again if and when you have to, on another day. xx

Anonymous said...

My son age 9 just had his spinal surgery. The one thing we didn't even think about til we met a girl 13 who had just had hers, was to set up rehab following surgery. Our doctor fought us on this. We won and it was the best decision. So just file that away. He was in a body jacket from age 2. What the brace does is not to prevent the scoliosis, but to keep the spine flexible. Once the scoliosis starts getting rigid it gets harder to correct with surgery.