Natalie is a bright teenager who has definitely been through the mill with Chiari amongst other things. Natalie's Syrinx seemed to cover most of her spine, yet she rarely complained about any pain. Her story is is told by her Mum Anne Marie, a nurse herself, but above all things a very proud mother!

Natalie was born in March 1993, the 3rd of 3 girls, ‘normal’ was diagnosed with asthma at 9 months but otherwise development was normal. Infact she probably did some things faster as she wanted to be the same as her sisters 14 months and 8 ½ years older.

Natalie continued with an uneventful childhood apart from Millennium Eve when we took a trip to our local A&E department courtesy of an asthma attack and that is where we heard the chimes of Big Ben - what a way to start the millennium, maybe it was on omen? Apart from one further episode she was not a sickly child and we hardly ever visited our GP apart from asthma/ medication reviews. Actually all 3 girls and myself have asthma must be in the genes.

A few years ago Natalie went through quite a dark period where she was constantly worrying about who was going to look after her if anything happened to her dad and I and would often lie in bed crying. Eventually she did grow out of this but I wonder if this was the start of her problems it was about this time she started to notice memory issues but never told anyone. We carried on with our life happily but oblivious to the bombshell that was about to be dropped. We did often wonder why Natalie could not tell the time and tie her own shoe laces, both insignificant as she found ways of dealing with both – lost her watches I had bought for her as presents, then got a mobile phone – can manage digital clocks ok – and always chose shoes/trainers with no laces – rather ingenious really! However because she used to get upset when asked about it, we never pursued either. No point when you can manage ok with adaptations. We have since found out that these behaviours are typical of hydrocephalus. In October 2007 Natalie started to complain about pins and needles following a visit to our local animal shelter. So I looked up all the usual animal borne parasites/viruses that could possible cause these symptoms. However I found nothing so I thought it was maybe a trapped nerve. When it refused to improve and I noticed she was veering off to the right when walking I started to take more notice of things to do with Natalie and what was normal and what was not. Next her voice changed to the point where she sounded like she had permanent laryngitis (one good thing was she lost the ability to shout) and she was no longer able to sing – she had quite a good voice up to this point. She also developed almost permanent hiccups. With these things occurring so fast I could no longer ignore them (alarms bells had started ringing) So I made an appointment with our GP who knows we only go when things cannot be dealt with at home, she took on board what we said and sent her for blood tests to rule out any of the straight forward (i.e. deficiencies) reasons for her symptoms. Apart from being slightly deficient in folic acid these were all normal she was referred urgently to a paediatrician.

I at this point became a bit naughty to ensure that Natalie saw one that I trusted as I had had a previous bad experience with one of my other daughters, but with a previous good experience too. So, 3 weeks later, a week before Christmas 2007 she had her appointment.

The Paediatrician did all the classic neurological checks i.e. blunt/sharp, walk in a straight line – she couldn’t, stand on one leg – she couldn’t, touch nose with eyes closed – she couldn’t. She agreed that something was wrong but didn’t at that point know what, so arranged for an MRI scan (again urgently)of Natalies head, neck and cervical spin which took place on 3rd January 2008 – Christmas was a stressful one that year.

The head one was done without incident or comment but part way through the spine scan the senior radiographer came in to add some tracing dye as they had found something in the spinal column. (The central canal was enlarged) that they wanted to get a better look at. Loud alarm bells started ringing and as soon as the scan was over we hot footed it home to get on the internet (oh the joys of technology)

Once there I didn’t like what I found what I did find was syringomyelia secondary to trauma or Chiari and as Natalie had not been involved in any accidents that Chiari was a probability.

I decided we wouldn’t worry too much (yeh right!) and to await the follow up appointment in February. However 5 days later I got a phone call from a Paediatrician saying that they had found something on the scan that I probably hadn’t heard of. By this time I considered myself converse in syringomyelia this needed a closer look at by a Neurosurgeon but he didn’t think it was too urgent, somewhat, but not much reassured I remained calmish only to be sent into another mild panic an hour later when said Neurosurgeon secretary rang to say he had seen Natalies scans and that he would see her in clinic on Friday – this was Tuesday – help! World just turned upside-down.

Before the scan I had been worrying about it being MS but thought that at 14 Natalie was probably too young. I also thought about the possibility of a tumour, but had also partly ruled that out as she never had any headaches and was never an ill child.

The appointment at Alder Hey Hospital was stressful. Here we have Natalie, a fit and well teenager and 2 parents that do not know what to expect. However we are prepared to go along with whatever is recommended. After weighing up who was who Natalie decided after watch them all call names out that she liked one particular Doctor as he appears to be a bit loopy (her word). Well she got him.

He explained all about the syringomyelia, that it was caused by Chiari and then he asked how her school work was affected by the hydrocephalus? Well, the first 2 I was expecting, but I wasn’t expecting the hydro so my chin hit the floor. As far as we were aware she was not affected at all but with hindsight and research we now know she is. Think back to watches and shoelaces.

He was very good and explained to Natalie as well as her Dad and I what it was all about and what the future may be with or without surgery. She was OK up to the point where he mentioned aspiration pneumonia caused by swallow issues, and although people don’t die directly from Chiari/syringomyelia but you can from pneumonia. Also that as symptoms were progressing then this was a distinct possibility. Once aspiration pneumonia was mentioned my mind was made up. If an operation was needed then she would have it. I’m a nurse, I work with the elderly and have seen too many with aspiration pneumonia. No way was I risking my daughter not getting to adulthood and beyond.

Surgery was arranged for 12 days later, not long enough away for us to panic for too long but far enough away to arrange time off from work. I was asked by one of my colleagues before the operation how I had remained so calm. I think the nurse took over, I decided I was no use to Natalie or the rest of the family if I was a quivering wreck. So I soldiered on, but inside I was like a jellyfish.

Surgery took place, very straightforward apparently and a pleasure to do because of this and slowly Natalie started to improve. Nausea and vomiting was the problem but Natalie was determined we were going away in half term which was less then 3 weeks later. Booked on a coach trip to Scotland which is one of her favourite places anyway; go we did although she saw it from a wheelchair and from an angle of almost 90 degrees.

When we cam home however she went down hill very fast, vomiting became a major issue as did the headache and nothing the GP could prescribe would make any difference. She ended up back in hospital and back to theatre on 2 more occasions – one for a temporary drain as the CT scan showing the hydrocephalus had worsened then the following day for the placement of a permanent VP shunt. Within a week she was getting back to her old self and went back to school on her 15th birthday (wonder what the motive was). 6 weeks after the decompression surgery and 2 weeks after the replacement of the shunt she was back in school.

Natalies symptoms are still there although they are no worse, infact some are a little better. The hiccups are now infrequent, she can walk in a straight line without veering off to the right and her voice is not as wobbly.

How did we as a family cope? I don’t know, but both myself and my hubby can fully understand how marriages and relationships can fall apart during the stressful periods. Hubby went through a patch of feeling that all he was, was a glorified taxi driver. I don’t drive and Natalie wanted me not him. Not anyone’s fault, but that’s what happens – kids what mum when they are ill and it wasn’t helped by the fact that only one parent is allowed to accompany a child going into theatre.

Last summer was hard when one of Natalies class mates died she was also 15 and had hydrocephalus although from a different cause. One night she had a seizure in her sleep and never woke up. That brought it home to all of us however easily we could loose Natalie and that these conditions are a real ‘sword of Damocles’- No one knows what the future holds. Natalie realised that she needed to start taking notice of her symptoms and tell me if things changed so we could do something about them.

Natalie calls me paranoid and I suppose I am really – she’s a bit like an ostrich and is happy to believe that she is like any other teenager. She is, but she needs to be a little more aware of what can go wrong I think these days she is better than she was but refuses to be held back and good for her. Because of her memory issues, rubbish short term memory she is going to need that optimism to keep herself going and we will be behind her all the way. I also worry that as soon as prospective employers see her medical conditions that they are going to run a mile. It’s a pity really as they will miss out on the opportunity to get to know someone who is witty, loyal, very determined and would be an asset to any employer. She currently volunteers with various youth groups and throws herself into anything she signs herself up for. Her ambition is to become a social worker dealing with kids with disabilities. I hope she succeeds as she will have more insight then most others.

Would we do it again? Definitely, because this is the only way that Natalie gets a shot at a future. Our jobs as parents is to do what is best for our children and hopefully this is what we have done for Natalie. Decompression is not a cure but it is an attempt at halting symptoms. We could not sit back at let those conditions progress without attempting to stop them Natalie deserves a future - she did not ask for this – she has been set a challenge and we as a family are rising to it.