One of the possible features of Beals Syndrome is scoliosis. It does not develop in all Beals cases, and some cases will only develop in adolescence or later in life. Due to the connective tissue disorder the scoliosis will continue to worsen with age if not treated. One article I read, stated it has a 'tendancy to rapid progression'. If not treated this can result in restrictive lung disease and the possibility of other issues with internal organs.
In Evelyne, we discovered the scoliosis at 4 months of age. The first x-rays showed a small but distinct scoliosis (first x-ray below). She was booked into the spinal clinic to monitor the curve. Each x-ray showed the curve clearly worsening.
At this stage they wanted to monitor the curve, and await the diagnosis before proceeding with any treatment. The hope was that the spine might stabilise. The physios made a special chair for Evelyne in order to try to protect her spine and limit degeneration. She was known as "Queen Evelyne" at playgroup - sitting on her throne!
In December 09, when the diagnosis of Beals was confirmed, the Doctors went into overdrive! We had a meeting with about 5 specialists in the room to discuss treatment. It was decided that Evelyne should be put straight into a plaster cast.
Evelyne was in the cast for 3 months. This was an extremely difficult time. The cast was made from plaster covered with fibreglass. It was heavy and hot, and difficult to change nappies and keep her clean. She could not bath or swim and struggled under its weight as she tried to master standing and walking.
We moved onto a fibreglass brace from this point which can be removed for about an hour a day. In this first brace the curve was reduced to 35 degrees. Although I was surprised it was still so high, the doctors were happy with 'maintaining' the curve at this level. In Evelyne's case, the aim to cast or brace was not to 'cure' or 'fix' the curve, but to maintain it to a safe level until they operate at some stage in the future. In the second brace the curve had worsened to 40 degrees. The third brace was thus organised. We will find out how the curve is progressing in this brace later this week.
Brace 1 Brace 2 Brace 3 - with brother Sam
I plan to write more in the future about: my tips and tricks for living with casts and braces; dealing with general anaesthetics; casting without sedation; future surgery.