I’m a bit late, yesterday was Rare Disease Day 2020 and I wanted to do a post. We have lots of rare to share! Instead, I’ve decided to show you my brave little girl back in 2014 in the halo.
Star had to wear the halo twice, for three months at a time. She has a rare condition called Elher’s Danlos Syndrome, which means her connective tissue is lax.
We knew something wasn’t right from when she was very young. At the age of three the hospital thought she may have a type of juvenile arthritis because of the pain in her joints. But despite everything it was only after the muscles in her neck gave way that anything was done.
I remember it so well, the kids had broken up for the summer and we were getting ready to move house. Then one morning Star woke up with a sore throat. By the end of the day she was holding her head onto one side. When we realised she couldn’t straighten it, we took her to the GP who said she had tonsillitis and gave her anti-biotics.
The next day we decided it was so much worse she needed a trip to A&E. We were told she had a torticollis and it would correct itself within 48 hours. Two days later we were back at A&E, nothing had changed. Because Star had not had an accident they didn’t seemed concerned, but I was. On our fourth visit to A&E I told a white lie and said she’d fallen off her scooter. She had, before she hurt her neck, but she hadn’t seemed hurt at all at the time, maybe a bruise on her knee. But this meant action and she was taken for a scan. Then she was immediately admitted to hospital with a rotary subluxation of the atlanto occipital joint. In fact the subluxation was so bad that part of her disc had deteriorated.
Star’s lax neck muscles had meant that the discs at the top of her spine had twisted away from the joint that connects her spine to her skull. Pretty scary stuff.
It took 18 months to fix. First with physio, which made it worse. Then with a manipulation under a general anesthetic and the first halo, but that didn’t work. Then finally it was fixed with a bone graft from her skull and two screws with a titanium plate to hold her neck in place. Oh, and the halo returned for another 3 months.
Now, 5 years later, she is fine. She has some impressive scar down the back of her neck, and a spot on her head where her hair doesn’t grow properly from where she had the bone graft taken. Also, she has several round scars where the halo was screwed in but they are fading with time.
My Rare Girl in the Halo
We were often asked how Star slept in the halo so I took a photo of her. Some people believed the halo came off at bedtime but that’s not the case. The halo is screwed into the skull, yes, I had to clean the screws. The bars where attached to a hard plastic vest which was locked in place. It came with it’s own alan key strapped to the front in case of the need of emergency entrance to her heart. The inside of the plastic vest was lined with fur for comfort, good job it was summer! Finding clothes was a nightmare, thank goodness she was too young to really care. Bathing and washing her hair was even worse. I’d spend at least 10 minutes wrapping the vest in black bags so it wouldn’t get wet, and then she’d sit in about four inches of water in the bath while I washed her.
It was during this time that the surgeon had noticed that her ligaments were lax and this eventually led to her diagnosis of Elher’s Danlos Syndrome. Her joints can pop out at any time. Only last week her ankle popped out while she was walking down the stairs and I had to pop it back into place. You do get used to it after a while.
So I may have missed Rare Disease Day yesterday but today I’m celebrating my brave, special and very rare not so little girl. She copes so well and I’m so proud of her.
Do check out my competition to win a fabulous bean bag chair from Beanbag Bazaar
Also, I have a code offering 25% discount from Photowall, wallpaper, canvas, prints, framed prints etc.