Jessica's Story as told by Kirsty Leigh

After reading the stories in the newsletter about adults with Chiari, I felt compelled to tell you about my brave 7 year old daughter's story, and hope it will help other parents in the same dilemma.

It all started with Jessica complaining of backache which the GP's thought were growing pains, but after 6 months of it, I was not convinced and pushed GP's for a referral. An MRI scan at the local general hospital in Milton Keynes showed what they described as a 'genetic abnormality' and then referred us to John Radcliffe Hospital in Oxford, saying it was 'nothing to worry about'. 6 weeks later and, we were told that Jessica has Chiari Malformation and they needed to do another MRI scan under anaesthetic to establish if she would need surgery. What a shock it was to find out that Jessica has been living with this condition all her life and we had no idea.

Our Consultant, the fabulous Mr Magdum at John Radcliffe, told us to look up the condition on the Ann Conroy Trust website and not to look any further on the web as there would be many scary stories. Of course, we first went to aACT's website and found lots of information that explained the condition fully and could not help but search further, which frightened us completely as we worried about our very active 7 year old's future.

After the MRI it was confirmed that she had a syrinx and they advised us to agree to decompression surgery. What a horrible decision to have to make when she did not have any of the major symptoms of Chiari but were told that it would only be a matter of time. She had minor symptoms, such as clumsiness, high tolerance to pain, night sweats and sensitivity to hot water, all things we thought were normal. On 7th October 2009 we watched our daughter calmly go to sleep and we cried all the way back to the ward.

Once the agonizing 2 ½ hours were over, we were able to see Jessica in the recovery room and the simple fact that she recognised us was a huge relief. Then 4 hours after surgery her resps dropped and we were unable to rouse her, so suddenly we were surrounded by doctors and they whisked her off for a CT scan. Once in the scanner she immediately woke, and the scan showed nothing wrong, so they put it down to a reaction to the morphine. We spent the night in high dependency to be on the safe side, and were sent back to the ward the next day. The days that followed had many ups and downs, with Jessica having many headaches, the sickness following the operation making her headaches worse, on and off drips and her motivation to walk anywhere up and down every day. At JR I was able to sleep at the side of her so was there to comfort her throughout, whilst my husband did the school rum with our 4 year old son, Cameron and came over as much as he could in the day and every evening, which was a 2 hour round trip.

We were then relieved 5 days post op to be told we could go home, although the journey was a slow one. During the week Jessica continued to experience headaches, but gradually moved more and ate more each day. Then, 7 days after coming home, suddenly everything changed and Jessica was in excruciating pain and at home alone with 2 children I didn't know what to do. I phoned the ward at JR and was told that I needed to go to our local hospital so I phoned the out of hours doctors and they called me an ambulance. By the time this arrived, my parents were round to attend to my sleeping son and my husband on his way back from Leamington Spa.

To cut a long story short, it took 15 hours before we were transferred back to JR, with delays due to the CT scan, computer problems and waiting for a new ambulance shift to start. All this time, Jessica was screaming with pain every 20 seconds and we could do nothing to help her. My poor baby seemed helpless and we were scared of what was happening to her. Within half an hour of us getting to JR, Jessica was being held down as she fought off the hospital staff trying to give her an anaesthetic and she was back in the operating theatre because she had fluid on the brain. Another agonising wait, but we soon joined her in the receovery room and she was a lot quieter, so much screaming caused her to lose her voice - any other time I may have thought it was bliss!

We then spent a further two weeks in hospital as Jessica had more headaches, air pockets causing some of it, so more MRI's followed and she was confined to her bed laying at 45 degrees for the first week. After a week of not moving, Jessica found it very hard to find the motivation to get out of bed and with many nights of 3 hours sleep caused by the air pocket pain, it was hard on us all, least of all poor Jessica. This second week was even harder as my husband developed a bad cold and so had to stay away, but my parents were a rock and even my sister and nieces made a surprise visit from Liverpool which boosted spirits. Jessica was very brave and eventually did enough moving about and eating for them to allow us home on 2nd November. The journey home made her sick but she slept most of the next day. Then a week passed of a little eating and drinking and finally she found her confidence and then there was no stopping her. She went back to school a week after that, part time to begin with, but was soon back full time albeit off PE and inside for playtimes. I am glad to say that she had another MRI in March 2010 showing the operations were a complete success, fluid has settled nicely and the syrinx is half the size, and will continue to shrink. Her minor symptom have gone, along with the backache, making her settle better at bedtimes. Her zipperhead scar has almost disappeared thanks to Bio Oil and she is back to doing PE after Easter and will be back to gymnastics in September.

Although it is a scary thought that many children have this condition and it remains undiscovered until adulthood, it is reassuring that with minor symptoms Chiari can be diagnosed and resolved before it has any lasting effects.

I would like to take this opportunity to thank the staff at John Radcliffe, especially Mr Magdum, for curing our gorgeous daughter and detecting it in the first place. I would like to thank the Ann Conroy Trust for giving us the information we needed to make an informed decision about surgery and I would also like to thank my daughter for being the bravest 7 year old I know - we are so proud of you.