Hattie's Arnold Chiari Malformation diagnosis story

Hey everyone, I'm Harriet, the little sister. I was diagnosed with Arnold Chiari Malformation in 2010 and have already undergone the brain surgery my sister is yet to have. I have been so busy recently so haven't been able to post anything yet, although I think my beautiful big sis has done an amazing job so far! This is my first post all about the build up to being diagnosed with the brain condition.

I was a normal 13 year old girl, I loved school and my teenage life was just beginning. I didn't really have a care in the world (apart from trying to steal all of Georgia's make-up and clothes). Everything was changing at this time in my life; I was really starting to grow up. In 2010 I started to get horrific headaches and begun to notice that my balance was getting really bad… ‘Oh it's just hormones!’, I always said to myself. But then weirder things started to happen to me and I asked myself: ‘why can't I swallow properly without choking?, why do I have disabling headaches countless times a day?, why am I throwing up every morning?, why can't I walk without nearly falling over?, why have I started to mumble, nobody understands what I'm saying?!, why can I never remember anything?...what is happening to me…?’. 

I knew it wasn't just hormones.

I decided to now tell my family that something wasn't right with me. My mum and I went to the doctors and as soon as I told the doctor I was sick after breakfast every morning, she asked me what I ate for breakfast, I replied with porridge...thinking, why the hell is she asking me that?!. ‘You are probably lactose intolerant, cut out dairy for a month and see how you get on’. Oh great, that explains the sickness! But what about all my other problems? I knew I wasn't okay and that there must be something else causing all of these problems. So, I cut out dairy for a month and of course it did not stop me being sick and my other symptoms were just getting worse, I was not lactose intolerant. I returned to the doctors once again and I had baffled them - they had no idea what was happening to me and referred me to Watford General Hospital where I saw a paediatrician who checked all my reflexes. I will never forget the worry I saw in her face as she was checking me. She sent me off to have an MRI scan of my brain, neck and spine. Even now, I wasn't really thinking anything of it all, I was worrying more about boys and what was going to happen on Hollyoaks that week, if only I knew what was coming!

That same week, we got the results of my MRI back (normally they take weeks to get back to people), with the quickness in which they got back to us, we knew something wasn’t right. ‘We have found something wrong with Harriet's brain, however we are not trained to tell you what is wrong. You have an appointment at Great Ormond Street Hospital in London this week, where you will be told’. YOU CAN'T TELL US?! Torture is the only word to describe the next two days building up to my appointment at GOSH.

The day came. Georgia, mum, dad and I all got the train to Great Ormond Street, I have always loved London, so I was excited to be going there and wasn’t really thinking about why I was going!

We were all in the waiting room, when my name was called. Georgia wasn't allowed in, just mum, dad and myself. The neurosurgeon was so lovely and just started off doing a few reflex tests on me, and then he sat us all down taking us through my MRI scan and showing us where the problem was. ‘Harriet is in urgent need of decompression surgery. Decompression surgery involves removing a chunk of the skull, to relieve the pressure off her brain. This pressure is causing the tail of your brain to fall down and crush your spinal cord - and is causing all of these problems. If Harriet does not have this surgery, she will slowly deteriorate and eventually be unable to walk, talk, or feed herself.’

The only way I can describe how I felt at this moment was like in films, when the character has taken drugs and their vision goes blurry and it sounds like they’re under water. I couldn't hear a word the neurosurgeon was saying after that. How was this happening? This can't be real, I thought to myself, this stuff is only in films and Holby City! I had never even had a tooth out and they wanted to take some of my skull out?! All I remember after we came out of the room is my sister running over and I collapsed into her arms, crying like I have never cried before and probably never will again. This was real and was happening to me.

It was August 2010 when I was diagnosed with Arnold Chiari Malformation, I was booked to have my surgery on 19^th of November, 2010.

My next post will be all about my experience having the decompression surgery. Thank you for reading, if you have any questions please feel free to comment or contact me personally! x

Having ICP Monitoring

Last Friday I went into the national hospital to have an ICP monitor put into my head. This was the first operation I have ever had so as you can imagine I was pretty terrified and being thrown in the deep end that is, brain surgery. If you have Arnold Chiari Malformation and are yet to see your surgeon, he or she may suggest that you have an ICP monitor in order to determine what would benefit you most; a shunt or a decompression. This is a really accurate way of reading the pressure inside your head every minute of the day. As mentioned in my previous post, this all depends on wether your surgeon believes a shunt could be an alternative to the decompression as this is such a big and invasive operation if it can be avoided, it will be. 
The ICP monitoring procedure sounds rather daunting. I have to admit that I was so anxious about it! It involves your surgeon drilling a small hole into the top of your skull and inserting a small wire and bolt into the tissue area surrounding your brain. This is then secured with a second bolt outside your head to make sure it doesn't get pulled or come out during the monitoring time. The remaining wire on the outside is left so that it can be plugged into a laptop which monitors the pressure of your CSF over 24 hours or more if the surgeon needs to collect more data. 

What the procedure feels like... awake!  

I arrived at hospital at 7.30am and luckily got told that I would be first in theatre with Mr Thorne, so I didn't have long to let my nerves get the better of me. Most people having this operation are given the option of having a general anaesthetic or a local with lots of sedation. I obviously opted for a general as I'm sure like most people, the thought of having a hole drilled through my skull whilst awake was just unimaginable. Within half an hour of being at the hospital I was called down to theatre and greeted by Mr Thorne and my lovely anesthetist who made me feel so relaxed and reassured that I was in the best hands. Then she gave me the news I did not expect to hear... I was going to be AWAKE during the operation! Although I had opted out of being awake Mr Thorne decided that it was too high risk to be asleep throughout the procedure. The main reason for this being that they wanted to talk to me throughout the operation to make sure they had not aggravated my brain or damaged anything as some (very few) patients might experience a seizure during the op - a risk that I did not want to take. So off I went, walking in what felt like a weirdly calm bubble into the operating theatre. Waving goodbye to my teary eyed mum was definitely the hardest part. When I got into the operating theatre I was asked to lay down on the bed and place my head onto what I can only describe as a small hollow cushion in the shape of a toilet seat - weird I know but that is literally the only way I can think of describing it. One thing I must say to anyone having this done awake... DO NOT look around the room! Look at the floor until you are lying down. I very regrettably looked at a trolly that reminded me of something from a SAW film, really not a good move! So anyway, after I got over that little blip the lovely anaesthetist got me a heated blanket and put my cannula in. Everyone was talking to me not that I remember any of the conversations as the after effect of being sedated is luckily memory loss of most of the experience. When they put the sedative in it feels cold and odd for a few seconds and then you feel absolutely wonderful. I'm not joking. I felt like I was floating on cotton wool and didn't have a worry in the world. I don't remember much after that until the drilling begun when I remember my head shaking a lot and somebody had to hold my head still because it was so aggressive. That makes it sound awful but honestly, you are so highly sedated you hardly remember anything and don't really batter an eyelid when it is happening. After 45 minutes from drilling time I was ready to go to recovery and rest up for half an hour until my drugs had worn off. I do remember this part quite well as I remember asking the nurses what they were gossiping about and then told a lady that she had great skin... I was still pretty much completely off my face but they seemed to like telling me all the nurse gossip...I think! Shortly after my lovely chat I was wheeled back to my ward and left to sleep for half an hour. Then my mum, sister and boyfriend came up which was the most delightful sight! 
Here is a picture of me looking my best with my new head accessory a few hours after the op...

The orange/ pink bit around the bolt is just bandage covered in Povidon-iodine (surgical anti bacterial solution) and the wire is connected to the computer as you can see here...

Once the monitor is plugged in you can sit up and unplug yourself to go to the toilet and you can even go for a short walk to stretch your legs. Unfortunately my Chiari related dizziness was at it's all time worst for most of 4 days in total so I was unable to get up unassisted and to be honest, just the thought of walking around made me feel nauseous. Apart from that, having the bolt/ wire wasn't as bad as it seems. I have been so unwell recently that laying in a bed for most of the weekend wasn't such a bad thing. Also, having great family and friends around to visit made it much more bearable along with plenty of magazines and a trusty i-pad for catching up on 'American Horror Story' - a miserable choice I know, but I am addicted to it at the moment and anything to put my mind off the fact I was attached to a computer by my head was worth a try! 

Having the bolt removed... with no pain killers...

After 24 hours of being monitored the doctors had collected enough data for me to have the bolt and wire removed. I think I was dreading this part more than the putting it in part as I was aware I would be without pain killers. The reason for this is that it is too dangerous to inject anaesthetic into your head with an open wound as I think from what the doctor said, it can make you brain dead and also cause seizure. So, the doctor wheeled his little trolly over to my bed and moved my bed so that I was lying flat. I then got my trusty neck pillow, grabbed my mums hand and calmly breathed as the doctor unwound my bandage. I have to say that this was really sore. It stung a lot, so much so that I ended up with my neck pillow in my mouth. Then comes the unscrewing of the outside bolt which was also very painful and uncomfortable but it is also over before you have time for it to get unbearable. Then the worst and almost final part is taking out the inside bolt... I'm not going to lie, I found this part absolute agony. My head was pounding, the wound was stinging like mad and I could hear the bolt squeaking and grinding through my skull. My boyfriend had to hold my legs still because they were flapping everywhere as I was trying so hard not to make any noise. After that little nightmare is over you are stitched up. Luckily the stitch is already in and all the doctor has to do is tighten it, unfortunately mine was not tight enough so the doctor took it out and put in two new stitches, which were the icing on top of the cake of pain. This just stings like a bitch. After the ordeal I was given lots of morphine and left to lay flat for two hours, which was good because I was still so dizzy and exhausted. Here is a lovely picture of me around four hours after having it taken out, looking erm... great... 

After this I stayed in bed for another day and night until I was ready to get some not so fresh London air and try out my new set of wheels... 

Getting the results... I'm having more brain surgery! 

A week later, now out of hospital I was going back in with my mum to meet with Mr Thorne to get my results. We drove into London this time as my dizziness means it is almost impossible for me to go on the tube and I am still a little nervous about the use of a stick or wheel chair on London Underground (I must read up about that!). Long story short Mr Thorne explained that they found the data very useful and that it was a successful outcome. He then went on to say that my CSF was of a normal pressure and that because of this, a shunt would do nothing for me. So the only outcome is to relieve pressure from around my brain stem in order for it to ease the pressure it is putting on my spinal cord, causing my debilitating symptoms. Mr Thorne then reminded me that this operation will not cure my condition, it will most likely alleviate my symptom progression and mean that I can have a better quality of life to how I'm living now. The operation is called 'a posterior fossa decompression' for anyone who was wondering. You can find details of it here: http://chiaricare.com/chiari-1-malformation/treatment/traditional-decompression-surgery.aspx

Harriet, my sister, has had this surgery and will be posting all about her experience sometime this week for anyone who is due to have it or just curious. 

Stay positive and strong and have a great week everyone! 

Please feel free to leave any questions you may have on here or you can follow us on twitter and instagram @chiarisisters   

    

Neurosurgeons are simply wonderful

Hi everyone, apologies for my late post. I was supposed to write this two weeks ago but unfortunately I have not been well enough and have found it really difficult to concentrate (Chiari Malformation messes with my concentration a lot). 

Finally, here is my update on my experience with a neurosurgeon... 

Two weeks ago I had my first appointment with neurosurgeon Mr Thorne, who will be attempting to eliminate some of my debilitating CM1 symptoms. I am being treated at The National Hospital for Neurology and Neurosurgery (NHNN) in Queens Square, London. Luckily for me the hospital is just a short train journey away and it happens to be the best neurological hospital in the country so I already felt extremely lucky and at ease about being treated there.

However, I did find myself feeling really anxious about meeting my surgeon. I wasn't really scared of what he was going to do but more so what he might not do. I have read and heard lots of stories about neurosurgeons who have limited experience with CM1 due to it being so rare and so patients often feel let down and have to get a second opinion. Many people also get told, like I did (explained in the previous post) that their symptoms are not related and that the CM1 was found whilst scanning for something else. It is true, many people do live with the brain herniation and never present any related symptoms so therefore surgery is only performed if the symptoms have become debilitating. I guess surgeons need to be very careful about making the decision to operate, after all it is a serious operation which is not only very dangerous but can also be unsuccessful in the long run.   

I was greeted by Mr Thorne's registrar, who was very welcoming and jumped straight in to asking me to number my symptoms in terms of which of them effected me the most. This was difficult to do as all of my symptoms effect me so much in different ways. I started with my memory and concentration, explaining that these had deteriorated dramatically over the past few months. I then went onto my balance, headaches and chocking. He then turned the lights off and did a quick basic eye test and also tested my reflexes and balance. After doing so he explained that there was some damage to my left eye - on top of everything else! And that I am clearly being effected by the condition. 

More news I was not expecting - ICP Monitoring 

He went on to explain that after looking at my MRI and completing various tests he and Mr Thorne were unsure if the typical CM1 decompression operation was the best thing for me. This was quite startling news as I hadn't herd of any other treatments for the condition before then. Mr Thorne then came in to meet me and explain what was going to happen. Firstly I'd like to say, what an amazing human! Mr Thorne was so friendly and reassuring, he explained everything in immense detail. He told me all about a procedure that has been introduced to CM1 patents which is set up to understand the exact cause of each symptom. This procedure is to determine whether I would benefit from a shunt, which would reduce my CSF pressure or if it would be better go straight in for the big decompression - I will explain what all of this means below. The procedure is called ICP monitoring. It involves having a wire and bolt placed into the skull which is connected to a computer which monitors the pressure of your brain fluid to determined if you have too much fluid pressure (which is caused by the brain herniation/CM1). He went on to say that it is urgent that they perform this procedure as soon as possible so they booked me in for the following Friday and explained that I would be in hospital for up to five days. Because the operation was so soon, I was sent off straight away to have my pre op, which just consists of taking blood and answering questions about my health history. 

And so that was pretty much it! I will be writing a detailed post of my experience with an ICP bolt, for anyone who may be getting one or just curious as to what it's all about. I'll leave you with a picture of my MRI compared to a 'normal' MRI so you may be able to get a clearer idea of what all of this means. 

A short description of CSF and why a shunt can be used 

*please remember I am not medically trained these are just rough summaries from my understanding* 

Cerebrospinal fluid (CSF) is a clear fluid found around the brain and spine. CSF pressure is when this fluid is blocked or there is too much of it causing dangerous and irreversible damage to the brain. 

A shunt is commonly used to treat an excess buildup of cerebrospinal fluid. Shunt systems drain excess fluid from the brain to another part of the body where the fluid is absorbed as part of the circulatory process. 

Chiari Malformation is a form of abnormality in the lower part of the brain know as the cerebellum. Here is my MRI - I have circled the herniated cerebellum.

If you have any questions please feel free to leave a comment. Have a great day everyone and stay positive! x