Happy Birthday Little Zebra

It was Lucy's 11th birthday yesterday. This week her secondary school have been having the new starters in for a summer school. She went in Monday and Tuesday and really loved it. She was looking forward to going in yesterday as it would be the first time she'd ever been to school on her birthday. It was not to be, however, as she decided she didn't want to go to school after all. It wasn't compulsory so to avoid upsetting her we let her have her way.

We didn't plan anything special for her birthday as we thought she wouldn't be here and we are all going out for a big family meal on Saturday. So she had to make do with a birthday shopping trip for school shoes...at least that's one more thing ticked off the back to school list.

Her main present was a drawing tablet for her PC. It's a flat device with a pen, which when connected to the PC it allows you to draw as if you were drawing on paper. It really makes a different if you want to draw on the PC and she's really pleased with it. She really does love her art work and already has her unique style. I'll share something with you when I get the chance.

Today she didn't go into school again as it was her appointment with her Neuro Surgeon at the hospital. She had her scan about six weeks ago to see how things were going. She has had some pain and soreness in her neck so, although we didn't think there was anything wrong, we were anxious for this appointment. The Neuro Surgeon, Mr S. was very pleased with her scan. Everything was fusing nicely. He said the discomfort she had been feeling was probably due to the bone graft fusing and growing along with her other bones, so it's a good thing not bad. He's promised to send us before and after scans of her neck, but we will have to wait and see if they ever appear.

In other news we took Lucy for her cardio appointment last month. They were incredibly thorough in their investigations including an ECG and an ultrasound scan. The results were all good, she does not have any heart problems. Again, we didn't really think she had but all these tests are to help the geneticist in her study to find out which type of Elhers Danlos Syndrome that she has.

The tests are all done now so we will wait for the geneticist to call us back. I'm not sure what she is going to say. She was considering a diagnosis of Marfan Syndrome but that would mean she would have heart and eye problems and she doesn't. It can't be ruled out for a future diagnosis though as these problems can develop later in life.

We still have to contend with her stomach problems on a daily basis too. Maybe one day everything will just suddenly get better, I'm forever hopeful.

The next big step in Lucy's life is starting secondary school just two weeks from now!

 

Not The Best News

Today we went to the hospital to get the results from Lucy's CT scan.
It wasn't good news. The bones have definitely slipped again, but much worse is that the bone has deteriorated quite a bit and no longer fits in with the rest of the spine so it will keep on slipping.

We have been told that Lucy will require an operation and possibly the halo will be returned.

Lucy's consultant will be discussing the CT scan with neurosurgeons at GOSH tomorrow and then will decide what the next step will be.

I don't have a picture of Lucy but I found this photo of scan which looks very similar. You can see the two bones at the top of the spine. One is square, the other is triangular. They should both be square. The one on the left of the picture has deteriorated changing the shape. This means the skull does not stay in position and tilts to the side.

photo credit: http://neuroradiologyonthenet.blogspot.co.uk/

A possible cause could be Rheumatoid Arthritis. Something I've suspected before.

I will be asking lots of questions, and pressing for our appointment with the rhuematologist.

It could be that the bone deteriorated because it was rubbing against the adjoining bones when they slipped out of place the first time. Will we ever know?

Cock Robin

We had a hospital appointment today to see how Lucy was getting on after having her halo removed.
The Doctor straight away noticed that her neck was not straight. He asked several times when the halo was removed, I'm not sure why, it was two weeks ago, it seemed like he didn't believe me. He said that she had a xray on 3rd March and her neck was straight. I said 'yes, that was two weeks ago, when she had her halo removed.'

He took off her collar and asked her to look to the left, which she did (and grimaced). The he asked her to look to the right, which she couldn't do (and grimaced even more). He asked her if she was in pain, she replied 'a little bit' She hates admitting to being in pain for some reason.

Then he said he would need to speak to her consultant, but was certain that she needed a scan. So we waited around for a while. Then he got back to us and said that they couldn't arrange a scan until tomorrow so could we come back. On the way home I had a call to say we had to come in Thursday instead.

So, still no answers. We really don't know what is going on.

I have two theories.

1. The muscles are too week to hold her head up straight and that's what is making her lean. Perhaps if they can strengthen her muscles then she will be recovered.
I worry that maybe her muscles were lax in the first time and that's why the bones subluxed. In which case there may not be a way to strengthen them. I'm not sure what the answer would be if that was the case.

2. Her joints are starting to sublux again. In which case then they will need to find another way to fix her, if that's possible. It also means the last three months of enduring the halo have all been for nothing!
It also brings up the question, why are her joints subluxing all the time?

One thing that the consultant has mentioned in the past is fusion. The joints in her neck would be screwed into place to stop them from moving.

I hope it doesn't come to that, but I do want her fixed.

Sometimes the Road can be Rocky

Right from the beginning things have not been plain sailing with Lucy's neck problems. It took more than two weeks of regular A&E visits before they actually realised what the problem was and admitted her. She was sent home after a week with  the promise of a six week check up to see how she was doing and regular physio therapy. The physo therapy department were great and Lucy was seen every week at first and then twice a week when she didn't seem to be getting any better. The six weeks came and went and I tried to get her a follow up appointment with the consultant but to no avail. The physio thereapist contacted the consultant with her worries but still nothing. Then I contacted PALS the hospital patient liason support team and eventually, after twelve weeks, Lucy got her appointment...for a CT Scan. I was so fed up at this point my plan was to go for the scan then to go and sit on the ward until the consultant agreed to see us. However, I did not have to make a nuisance because the consultant turned up at the scan and then talked to us afterwards. That's when Lucy was admitted for the manipulation.

After another week in hospital Lucy was sent home with weekly appointments for the next four weeks. I had to call and make appointments for the next two weeks. Then at the last appointment I was told she would be sent for another CT scan and if that was ok then they would take her in and remove the halo. The CT scan was supposed to have been this week. Today I called radiology to ask why they hadn't sent her an appointment and was told that the instruction was to see her in six weeks!

So what were we supposed to do for the next six weeks with no appointments booked? The halo was supposed to be fitted for 8 to 12 weeks, it's been 8 weeks already, another 6 would take us to 14 weeks. Also, the screws are supposed to be checked and tightened every week. It's just like before, they've forgotten her again.

Last week we had some discharge from her points, it did stop so I didn't fuss. This week Lucy has been experiencing more pain than usual. I've already called the hospital and they promised to call back but didn't. I can't wait for PALS again, I will have to go to the hospital and make a nuisance of myself until she is seen. Why does it have to be like this? Why do they seem to have so little regard for an eight year old child?

We often get asked, how does she sleep?