Worsening Scoliosis

Annie had her 6 month scoliosis check-up on Monday.  Ever since she was about 2 years old she has had scoliosis.  It held steady at 11 degrees for a couple years, then held steady around 18 degrees for a couple years, but last July her curve jumped to 37 degrees.  They are wanting to monitor her more frequently now, so on Monday she had another x-ray. 

Now, just a note about how scoliosis x-rays are taken... Annie has sometimes had her x-rays laying on a table, and sometimes she's been sitting up.  With an 11-18 degree curve, the difference in the curve sitting versus laying hasn't been much.  However, generally speaking, the curve degree is more accurately measured if the patient is sitting up, which also usually shows a more severe curve.

In these images below, the one on the left is from July 2010 and was taken laying down.  The one on the right is from Monday and was taken sitting up.  Last July her curve was measured at 37 degrees.  We have not gotten an accurate measurement from her x-ray on Monday, although the computer model calculated it at 69 degrees.  (However, the same computer model calculated her July curve at 52 degrees.)  So, taking into account that she is laying down in the first one and sitting up in the second, we're guessing that her current curve would measure close to 52-57 degrees.  All of that is just our guess--the doctor really couldn't tell us an exact measurement now since it hadn't actually been accurately calculated.

The thing is, you might say, "Well, Annie is obviously not sitting up straight in the second x-ray."  Let me just tell you that both Justin and I were supporting her on the x-ray block, and when they took the x-ray, I commented to Justin about how glad I was to have her sitting so straight on the block--that way the curve of her spine wouldn't be influenced by how she was sitting, and I knew they'd get an accurate measurement not influenced by the way she was sitting.  I thought we had her sitting so perfectly straight, and from what we and the technicians saw, she WAS sitting as straight as she would go.  That's why I was so surprised to see the x-ray!

July 2010 X-ray (37 degree curve)         January 2011 X-ray (52-57? degree curve)

Just a couple other things about these x-rays...  We were rather appalled to see on these x-rays the amount of fat that Annie has gained around her chin and cheeks.  We know she's been gaining lots of weight being on prednisone for nearly 3 months now, but I was surprised at how clearly these x-rays show that weight gain in her face!

Also, one reason some of her past x-rays have been laying down is because Annie get her hips x-rayed every year to monitor for problems and another dislocation, so they seem to like to get both hips and spine in one image and be done with it.  So in the image on the left from July, you can clearly see her dislocated left hip and her rotated pelvis, which in turn is causing her scoliosis to worsen.  You  can also clearly see how that dislocated hip is causing her left leg to be about 3" shorter than her right.

So, now you know almost as much about Annie's insides as we do (at least orthopedically)!  The plan now is to continue to monitor her scoliosis, with x-rays every 4-6 months.  Annie is not a candidate for bracing because this is not the type of curve a typical child might develop who has normal muscle tone.  Bracing would only cause more pressure sores and potentially cause respiratory issues.  Any amount of bracing at this point also would never work to correct her curve.  It might prevent it from getting worse, but most children with Aicardi Sydrome have continued to have a worsening curve even with bracing, and for us, the side effects aren't worth it when results would be so poor.

So really the only option (if we do decide to treat it) is to surgically correct it with a major spinal fusion surgery where two rods are inserted on either side of the spine with loops threaded through the spine.  Most doctors don't like to do spinal fusions on children until they are 10 years old.  So right now we'll continue to monitor her curve and start discussing surgery in about a year, unless things start getting really bad really fast.  The doctors believe that Annie's curve is already at the point where it is going to start putting stress on her heart and lungs, so we're anticipating that within the next 18 months we're going to be facing some tough decisions.  For now, I'll just try to not think about it all and be grateful that we have Annie and that she is so happy and doing relatively well!