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.

The Halo is Removed

On Monday March the 3rd, after exactly thirteen weeks, Lucy had her halo removed.

Daddy took her to the hospital while I got her sister to school. I figured I should just about make it to the hospital in time for her to come out of theatre. Thanks to traffic I was five minutes late, and Lucy wasn't happy with me. Not the best of starts, but she soon forgive me.

The removal was quick, probably only took about 20 minutes with her being under anaesthetic. The pin wounds are clean and look like they will heal quickly and well. I was told that with adults they do not bother with a general anaesthetic to remove an halo, but it's better for children because it's less distressing and less risk of the child moving or trying to move while they are taking it off.

I was surprised to see that Lucy's skin looked fine under where the vest had been all this time. You can't wash under the vest, just wipe with a baby wipe or damp flannel. I thought she might have been marked or wrinkly but she just looked normal.

Lucy woke up hungry and the nurse fetched her some toast and squash, which she devoured. She seemed a little quiet at first, which is understandable, but she was soon her smiley self.
At lunch time we were asked if she would like to go to the playroom as they were having party food for lunch. I got Lucy out of bed and took her to the bathroom before dressing her. It seemed strange putting 'normal' clothes on her again. She has put on weight, so it looks like I'll be shopping again. We walked the length of the ward to the playroom and Lucy chose some food. We sat at the table but she said she felt all wobbly and wanted to go back to her bed. I think she was out of bed a total of fifteen minutes. She did eat and drink a little more though.

The nurse caring for Lucy told us that she just had to have an xray, and drink one more glass of squash and she could be discharged. We went for the xray around 3pm by which time Lucy was feeling a little pain and looking pale. The nurse decided to take her in a wheelchair rather than make her walk. By this time I was getting worried that Lucy just wasn't doing as well as we thought. Her head was floppy and she was sticking her chin out, which is something she does when she's in pain. As usual, when asked if she was ok she gave her usual reply, 'I'm fine'

I have to say at this point that Lucy's nurse this day was really brilliant, she was so attentive and told us what was going on all the time. Despite being an incredibly busy day she never left us for more than half an hour and listened to everything we said. So when I told her I was worried that Lucy wasn't acting like herself, she actually listened to me.

The Doctor came around and said Lucy's xray was good and she could go home. He said she should be sitting up though, and not in bed anymore. So I took her out of bed and she sat in the chair watching some tv. Then her nurse came to take her candula out and Lucy started vomiting, everywhere, tons of it. She was crying and complaining of headache, not like Lucy to complain at all so she must have been feeling really rough. The nurse said to put her back in bed and we were to delay going home for a couple of hours at least.

Thankfully, Lucy wasn't sick again, and after a dose of medicine her headache cleared up and she regained some colour. She was refusing to eat anything, scared of being sick again. The nurse said she'd be happy if she just kept a cup of squash down.

At 7pm Lucy had perked up a little, she'd kept her squash down and was talking to me again. The Dr came and said it was ok for her to go home, so the nurse finished the paperwork and we got ready and left to spend the night in our own beds.

As we were leaving the ward, literally just about to go through the door, Lucy's consultant was just coming in and he saw Lucy and said 'no, no, no' My heart sank, I thought he meant he wanted her to stay in. He took off her coat and then the collar and carefully straightened her neck before putting the collar back on. Her neck flopped again. He said 'the xrays were good, her bones are straight, but they will come out of place again if she can't keep her neck straight. The collar she has is not strong enough to hold her neck straight, and Lucy's tendons are not strong enough. I'm not sure what is going to happen, but we have to keep telling her to straighten up and keep our fingers crossed that we can buld up strength in her neck to hold her head up again. She can't start physio for two weeks as it's too risky, so it's going to be down to us and Lucy to try and keep her neck in place.

We have been referred to a rhuematologist for Lucy's hypermobility. I want to know if it's her loose tendons that caused the slipped discs in the first place. I also want to know if it can be prevented from happening again. My biggest fear now is that it can't be prevented and Lucy has what is known as cranial instability...i.e. her neck isn't strong enough to hold her head. Only time, and the doctors, will be able to tell.

Is it ironic that the hospital uses zebra wristbands?

12 weeks, but still one more to go!

Well Lucy reached the 12 week mark of halo wearing but she's not having it taken off until next Monday. The good news is, this is definite, she had the first theatre spot booked for Monday morning.

Apparently, taking off the halo is not as bad as putting it on, phew. However, it is a long and uncomfortable process and in the case of children they prefer to do under a general anaesthetic. Lucy is fine with this, she doesn't like having a general anaesthetic, but who does? She is happy, however, that she will wake up without the halo. We are all really looking forward to it coming off. I'm looking forward to a cuddle with my girl.

We've just finished a week of half term here. This means Lucy has had her brother and sister around to keep her company. We tried to get out of the house and do some fun stuff, but we were limited. First up was a tript to the cinema on Saturday morning. We were there for the first showing at 10am and there were only three other families in the theatre. We watched Monsters University and all really enjoyed it. I wanted to go home for lunch, I do feel uncomfortable eating out with Lucy. Her dad says I'm being stupid, but in reality it can't be nice if you have to sit and eat your food opposite a child with a halo screwed into her skull, can it? I just remember my feelings when I first saw her in it, I felt sick. We can't forget that we are used to seeing it and it doesn't bother us anymore, for others it's more distressing.

I didn't win my argument and we went to Mc Donalds. We did, however, manage to find a quiet table right at the back of the restaurant. Yesterday we all had lunch in the hospital restaurant, I felt much more comfortable there!

Then on Tuesday we went to Toys R Us. This is a trip I've been promising the kids for a long time. They have been missing out on sweets and treats and saving up the money for a special treat. They managed to save £25 so I made it up to £45 so they could have £15 each. I was a little worried because there is so much in Toys R Us, and such a lot of expensive stuff. I really thought I'd have trouble on my hands as they rushed around the shop wanting everything in sight. It wasn't like that though, they were really good. The girls both wanted My Little Pony toys and Lucy really wanted the wedding castle. It came with two ponies, so I suggested that I bought it between them as it was £30. They agreed and were really happy with the idea.

We had to get to the shops by bus and again we had lots of stares, but also plenty of questions. I really prefer it when people ask what's wrong with her instead of just staring. Of course, after three months it's getting a little tiring explaining it over and over, but I'm still glad that we've been asked.

We didn't get to go out again. Lucy does find it really tiring, and we end up having very disturbed nights as her body catches up with the exercise she has taken. Thank heavens for Junior Ibuprofen and wheat bags, it's the best way to stop her pain.

She's had a lot of pain in her neck too which has been very worrying. We even had an impromptu visit to the hospital at one point when her screw came loose and I found blood in her hair. It was ok though, soon sorted. The pain seems to have eased in the last week though, so fingers crossed everything will be ok when the halo is taken off.

Just one more week to go...YAY!!

The End is Nigh, But is the Future Bright?

Last week I took Lucy to the hospital for a CT scan. Since having the halo she has had x-rays but this was her first CT scan. I was told three weeks previously that Lucy would have a scan at eight weeks and if it was fine they would remove the halo.
Well, Lucy had her scan at around nine and half weeks and we didn't hear anything from the hospital. Then over the weekend we noticed one of the pins was really loose and Lucy was crying because it hurt her. The next day I noticed blood around the point. So Today, without warning, I turned up at the hospital. We were seen straight away but because Lucy was her bright little cheerful self she made me look like some neurotic stressy mother.
That's the thing with Lucy, she's almost always bright and cheeful, she takes everything in her stride. Don't get me wrong, she does have meltdowns and we really know about it when she does, but they don't happen often and most of the time she's just lovely. You can watch her without her knowing and see her grimace in pain, but ask her if she's ok she'll smile and say "I'm fine" so when she does say she's hurting we know to take her seriously.
So the Doctor today asked her when and where it hurt and she replied, on this side (pointing to the left) and when I lie down on this side. The Doctor replied, "well, don't lie down on that side then"

He checked over her points and said they all looked fine. He found dried blood in her hair and said that was normal. He asked how long it had been since the halo had been tightened and I said more than three weeks, he said she'll be ok for another two then.

Then he looked at her scans and xrays. He said there had been some improvement during the last month, but there was still rotation noticable in the atlanto occipital joint, and there was still a slight bend. Therefore he suggested leaving the halo for another two weeks and then see about taking it off. So an appointment was made for two weeks time.

I came home and told Lucy's dad what had happened. I was confused because I was under the impression that the halo was there to keep her neck perfectly straight while it healed. Lucy's dad agreed because he'd been told by another consultant that it was there like a brace to heal a broken bone, to keep her neck in the same position. This contradicts what the Doctor said today about there being improvement in the position over the last month. How would the position improve if it was meant to be held in place? However, I do know that the halo is a traction brace (it actually says so on the back of the vest) so maybe it is stretching her neck straighter? Which Doctor is right? Which should we trust? Can we trust anyone anymore?

I have a plan. I am going to write down all my questions and concerns and when we take Lucy back to the hospital on 25th February I'm going to make sure the Doctor that sees us hears them all. Hopefully, he will have some answers, because I have a feeling I'm going to need them.

In the meantime I am praying for my little girl. I know from the scan pictures I saw today that she is still not healed. My hope has been drained. The Doctor may have been fobbing me off saying that it will improve over the next two weeks, I can only hope that he was telling the truth, but in my heart the hope is lost. I've been saying for a long time that her neck was still not straight, but Dad believes it's because they over compensated for the bend. I can still feel that lump in the back of her neck, the bone that is not where it should be. I keep telling myself I'm imagining it, I'm being negative, I'm a neurotic mother, anything. Will I have to accept that she is much better but not perfect? I think I will, so long as she is safe and free from pain.

Moving Forward

We had another hospital appointment today. It's amazing how quickly you become familiar with a hospital, well I say quickly, we've been going there for nearly six months now! Lucy has her appointments on the ward where she stayed, now as soon as we go in the nurses and helpers all know who she is.

Today we saw one of our favourite consultants. I know you shouldn't have favourites, they all do a very important job. However, there is always one that seems to listen more, take more interest and remember things better. This doctor has promised to get us a referral to a rhuematologist so we can find out what type of hypermobility she has and get a proper diagnosis. I know that in the long term the actual diagnosis is not relevent, but it will give us some answers and allow us to find the right support groups. There is no cure, no real treatment, all we can do is manage the symptoms. Of course, with a diagnosis it will mean that if Lucy has problems in the future the doctors will know where to begin. It took a long time for them to take us seriously this time, it was as if they didn't really believe that Lucy had anything but a crick in her neck at first.

Today's appointment went well. There was not much tightening to be done which pleased Lucy. She has been complaining for a couple of days of a pain in her groin area. She told the doctor and he examined her. I didn't think he would because I thought the pain was nothing to do with her neck. However, it turns out that it's possible for her lymph nodes to swell, which could be connected to the lymph nodes in her neck. She could also have pain there because of the restriction of the vest. People see the halo and think that it looks uncomfortable, but the vest is just as bad. It is fixed tight around the abdomen and can be tight under armpits too. If it gets too bad then the doctor (and only the doctor) can release it a little. Anyway, Lucy is ok at the moment, we just need to keep an eye on her and get in touch with the hospital if it gets worse, or doesn't get any better.

people don't realise that the vest is uncomfortable too.

Next step is a CT scan in a couple of weeks time. Then if all is well they will take away the halo. I asked about the procedure for taking the halo off. Normally, for adults they just take it off with a topical anaesthetic, but it does take a long time and can be distressing. For children, they prefer to give them a general anaesthetic. So Lucy will go to sleep and wake up halo free. We can't wait now.

Hospital Appointment

Yesterday we went to the hospital for a halo check. It wasn't a good experience, two of the screws had come out, although they were still in the skin. Most of the screws needed tightening. Getting it all tightened up again was a distressing and painful experience for Lucy.

When you have a halo then you should have the screws checked weekly. We were not told this at the start. We were given weekly appointments and at the first one they were all tightened, but they were not that loose. At the second appointment they were tightened again but the third appointment was during the Christmas holiday and there was no-one available to tighten them. Then for some reason we were told not to attend the following week. So this week it had been nearly three weeks since they were last tightened and that's why they had got so bad.

I was told yesterday that I should have attended last week, which is exactly what I thought and did phone up to ask, but nothing was done and we missed an appointment which we shouldn't have missed. If you are reading this because you or your child have been fitted with a halo, I will advise you to make sure you get them checked weekly, it really is necessary.

As well as having to endure this horrible experience we were also given the news that the halo wouldn't be removed any time soon. When we had the chat with the consultant before the halo was fitted we were told that they are normally on for six weeks. As we are approaching our six weeks in a few days time I had built my hopes up that it would be removed soon. It was quite deflating being told that it would be at least another two weeks and possibly another six weeks. I really do want the best for Lucy, this has to work and I wouldn't want to jeopardise her recovery, but I just can't wait for it to be gone now.

While at the hospital we were asked to meet a family who's son was having a halo fitted tomorrow. I was happy to do this and answer their questions as well as give them a few tips on how to cope. This is my aim, to raise a little more awareness so people know what to expect.

Living With a Halo - part two

Another post taken from my other blog about how we are getting on with the halo.

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My Lucy is a real trouper. She is coping so well and I'm so proud of her. We had a hospital appointment yesterday where she went to have all her screws tightened up. They also gave her an x-ray and things are still looking good. Her Daddy got to see the scan pictures all in order from the start and was a bit shocked at how bad it actually became before they did the manipulation. Thankfully things are looking much better now. It's still not perfect because the consultant over compensated, but he insists that it will go back once the halo is off.

Sleeping has become easier for Lucy. It's probably down to just getting used to the halo more than anything else. She only has pain relief when she asks for it, which is usually last thing at night and first thing in the morning. She has really adapted to wearing the halo and never lets me forget to clean her pin points regularly.

We have found a way of washing her hair.
First I put towels around the edge of her sheepskin. Then I cover her with a black plastic bag. Then I use a plastic hair dressing cape across her shoulder bars.
I put the Little Man's step in the bath for her to sit on. Then I wash her hair trying to use as little water as possible.
It's not easy but it gets the job done and so far we've not got the sheepskin wet.

Clothes are still a problem but we've found that spaghetti strap vests that are a couple of sizes too big will go up from the bottom and stay on her shoulders. They manage to keep her tummy and side bits covered up.
Then we can put button through cardi's or blouses over the top. Luckily there is not problem with her wearing her normal jeans and trousers. I did buy her some dungarees but she's not keen on them.

We took her to her school for the Christmas concert and they all made a fuss of her. We even had seats reserved for us at the front. We may consider sending her back to school after Christmas for a couple of mornings a week.

Living With a Halo - part one

This is a post copied from my other blog which I've decided to include as it explains how we have coped with the halo.
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One week on from Lucy's operation and we are learning how to cope with the halo. Just over a week ago I was thinking of how I would cope with Lucy's long hair if she had the halo fitted (it wasn't definite then) but now I see that really was the least of our problems.

 Starting with the hair though, it really isn't too bad. The halo doesn't go all the way around the head, there is a gap at the back, perfect for reaching the hair and putting it in a plait or pony tail. We do have screws in the side of her skull which means hair brushing is difficult, but it's not so impossible to keep it tidy. Also, I've been told that it may be possible to wash her hair next week when the pin point wounds have healed more. We just have to find a way of doing it without getting the vest wet.

 Now, our biggest problem is clothing. I didn't realise just how big the vest is, both at the front and the back. And the bars come right down onto the chest making putting clothes on almost impossible. So far we have managed to fit her in a cardigan which just covered her arms and fitted around her back. So I tried one of my bolero type cardis which has a fastening at the front. It just about does up at the front but tends to slip off her shoulders. The other day I took an old vest top apart and sewed on some ribbons to see if I could adjust it to fit around the vest. I had a little success, although the vest was way too small. I think with a larger vest it just might work but it's going to look weird. I've ordered her some dungarees, and praying that they fit. They seem the perfect solution as they open up at the shoulders. Also, they are adjustable at the sides so we could just leave them open if they don't fit over the vest. Fingers crossed they do the job.

Another huge problem is getting out, especially to hospital appointments. I currently don't have a car but I would so appreciate having one right now. We can't really take her on the bus, it just wouldn't be safe for her, I'd be scared of her bumping into one of the bars, or someone else. The ride can be quite jolty too, even if I ask the driver to stop while she sits down or gets off. I think she could cope with the stares, we're kind of used to that with her having to wear a collar for so long. However, the halo is unpleasant to look at and I wouldn't want to distress anyone on public transport. Taxi's are a good alternative but so expensive, and we don't exactly have a lot of money right now. I've tried to find some hospital non-emergency transport but so far had no luck. I'm managed to get two offers of lifts from friends and family, that leaves four journeys until the halo is removed. Also, I can't really take her anywhere else. We have been invited to see a play at the town hall at Christmas but I have no idea how to get her there.

Now, I'm quite used to not having much sleep. The Little Man has only been sleeping through the night for a short while, and Lucy has never been a good sleeper anyway. However, I'm finding looking after her during the night a big strain. She calls out to me often needing me to turn her over, or rub her knees (this is usually what keeps us awake) or give her painkillers, or just comfort her because she's distressed. This is happening all night long and I'm lucky to get an hour in-between. I do hope she settles more as she gets used to the halo, for both of our sakes.

Other things we've had to get used to are;
Cleaning the pin points. I found it quite hard at first, but now I don't think too much about it and just get it done. The ones at the back are the hardest because you can't see past her hair, but I'm so glad they didn't have to shave her.
Washing. We can't get the sheepskin under the vest wet, so no showers. She can have a shallow bath, but to be honest, I'd be terrified of her getting in and out, she's not the best at this without a halo. So it's wash downs. And we can clean under the sheepskin with baby wipes. I'm dreading what her skin will be like when it comes off.
Stairs. Lucy has always been a little awkward on the stairs and now it's ten times worse. Now she has to balance herself and has limited vision. I have to follow her every time she goes up, and come down in front of her just in case.

The halo is big and hard, I'm already sporting bruises on my arms and shoulders from it, and every time I go to kiss her it feels like I'm close to losing an eye. It's really not a kind or friendly contraption, hugs are out of the question. Also, when you collide with her, and believe me we are trying very hard not too, you worry about hurting her, although she hasn't complained yet.

On the plus side, and it's really hard to find a plus side, lucy has been brilliant. She hasn't complained much at all It has to be uncomfortable, and sometimes I watch her facial expressions and know she's in pain, but when I ask she says something like "it doesn't hurt too much, mummy" She's a proper trouper. I really hope this is all worth it and she comes out of it fixed and pain free.

Lucy's Story

In August 2013 my little girl, Lucy, woke up with a bad neck. A visit to the GP didn't really help, he thought maybe tonsillitis but she wouldn't open her mouth far enough for him to see inside. I wasn't happy and took her to A&E. We were told she had torticollis (bent neck) and that it would likely correct itself within 48 hours. It didn't and my little girl was finding it difficult to eat or drink. A CT scan was done one her brain, but that came back ok. A couple of days later a CT scan of her neck revealed a rotated bone at the top of her spine. In medical terms, a subluxation of the atlanto occipital joint.

Now, I tried to Google this term and all that came up were medical records, difficult to understand and some quite scary. I figured that the condition might be quite rare, and apparently it is, but it does happen and it's quite scary when you can't find anything that can offer advice or help. This is why I have set up this blog. Hopefully, the next time someone searches for subluxation of the atlanto occipital joint, or even atlanto axial joint, or even C1, then maybe they will find some support in my words and the story of my little girl.

Another reason I have set up this blog is to raise awareness of Hypermobility Syndrome, because I am certain that this is why my daughter had the subluxation. I'm still travelling this journey so I want to share my story as I go and maybe help or inform others of our experiences along the way.

Lucy was first diagnosed as hypermobile when she was six years old. She'd been having pain in her knees and I'd asked the GP to investigate. He sent her to the hospital for x-rays but nothing showed. They did point out that she was hypermobile. Shortly afterwards I was having her assessed for Autism as her teacher suspected that she might be on the spectrum. I did too, but I'd kept it to myself until it was noticeable by others. As part of her assessment she was seen by a physio therapist who diagnosed her as hypermobile. She was also diagnosed as high functioning autistic, but I'll talk more about that later.

Now, being hypermobile is quite common, most people know the condition as being double jointed. However, when the hypermobile joints cause you pain and discomfort then it's known as hypermobility syndrome. The assessment is based on the history of the patient and their score on two charts known as the Beighton score chart and the Brighton Criteria 

Lucy scores 9 on the Beighton score chart. She's not been medically assessed yet on the Brighton Criteria, but seeing as she currently has a subluxation of a joint and has suffered chronic pain for years now, then it's likely that she would get a diagnosis of Hypermobility Syndrome. 

When it was first discovered that Lucy had a subluxations of cervical joint the consultant was convinced that she had had a fall. I know that she didn't (not even at school because it happened during the holidays). She woke up with condition which is very unusual. It's also scary because it means it could happen again.

In my next post I will write about what happened when Lucy was admitted to hospital and how her treatment has progressed.