My name is Sarah, I am married with two children currently aged 9 and 6. This is my story about my diagnosis and operations. It all happened in October 2006…

“Mummy!!!” cried my then 3yr old son who needed the toilet in the middle of the night. I got out of bed and tried to walk to his bedroom. My legs wouldn’t work properly and I started to feel faint and had noticed that my legs and arms were shaking again. I managed to get to his bedroom and sat on the end of the bed until I felt a little better. After my hearing had come back to normal, and I had stopped shaking I took him to the toilet and went back to bed. In the morning I mentioned this to my husband, who said that my blood sugar must have been very low and next time to sit on our bed until I feel better.

This continued on and off over the summer holidays of 2006, until one night I got up to our son and as I got the to hallway, my legs would not hold me up anymore, and I was shaking that much that I couldn’t hold onto the door frame to steady myself enough, so I collapsed onto the hall floor. My husband found me shaking and a little incoherent, he took me back to bed and I fell asleep instantly.

In the morning, I felt exhausted, couldn’t talk very well (felt very slurred), was dragging my right leg whilst I walked and didn’t feel very well at all. I went to work (at that time I was a teaching assistant in a primary school). I soon found that in the classroom, I couldn’t tell where I was putting my feet, couldn’t stand the loud noises, had balance issues, couldn’t concentrate for long and my patience was very low.

I couldn’t get to see a doctor that day, but did see a practice nurse who said to take parecetomol and come back if things didn’t get any better. So a week later I went to see a female doctor who did all the usual tests on me and finally agreed to send me to see a Neuro Consultant. I was due to do out to Florida for two weeks on a family holiday in November and this was only three weeks away, so I asked for the appointment to be urgent.

I was due to fly out on the 21st November and I had my appointment through to see a consultant on 19th November!

He wanted to send me to have a MRI scan thinking that it was a tumor, but also stressed that I should stay home from America, reason being it could get worse and I wouldn’t be covered by insurance. Of course I ignored his advice and went on holiday armed with something called Stemitil, which helped a little with the headaches somehow.

Upon my return I immediately had a scan and within days, I had a phone call saying I had Chiari Malformation and would need surgery, he would refer me to see someone in Derriford Hospital which was the closest, as Cornwall doesn’t have a Neuro Surgery Dept. I decided to contact him myself and pay to see him privately which was on the 3rd January 2007.

I went to see him with my hubby, he suggested he wanted to insert a shunt, but he wanted to see the pressure of the fluid in the brain before doing this, which involved inserting an ICP Bolt in the headfirst.

This was the most horrendous experience I have ever had to go through. The operation took place on the 24th January 2007 at Plymouth Derriford. A drill was used to make a hole in my skull whilst I was still awake, the bolt was inserted and I was sent back to the ward. The machine measuring the pressure didn’t show increased pressure, so within minutes on being on the HDU ward, the surgeon was telling me I could go home! Obviously I was devastated.

After removing the bolt, the fluid then leaked in under my skin, inflaming my face so that I looked like the elephant man, of course I couldn’t go home that day. Upon release from the hospital I had to start all over again with the consultant.

My original consultant at Treliske Truro openly said he didn’t know what to do next, so I asked for a second opinion. He sent me to see Mr Whitfield privately at the Nuffield Hospital Plymouth in March 2007. By then I had a catalogue of symptoms, all of which he explained to me caused by the ACM. I also had new symptoms appearing.........

Symptoms

Light and wind sensitive to the right hand side of the head. I wear a hat and sunglasses when outside.
Headaches.

Confusion and disorientation, bumping into things, feeling of not knowing where I am putting my feet when really bad

Speech problems, -brain knows what to say, but cannot get out the correct words or in the correct order.

New – When bending over, I have a pain in the back of the neck, which then develops into a headache, like a rubber band is around my head. This then brings on black spots in front of my eyes and dizzy spells.

Activities such as putting out the washing, bending over in the classroom with the children, cooking etc.
New – having trouble trying to swallow certain foods and it gets stuck in my throat.

Memory problems - Short-term memory is really bad, instantly forget things, like telephone calls at work.

Tiredness - yet not being able to sleep at times.

New- Although my back aches has been kept under control for a few years now, the pain has returned and I cannot stand up for a long period of time, max 10 mins.

Still having muscle spasms, dizzy spells, feeling faint etc when I get up in the morning. Nearly collapsed twice but managed to get to the bathroom where I sat down until the muscle jerks stopped.

Continue to have pins and needles in the left hand.
New- I am now having pins and needles in the right hand.

New- recently been having very bad electric shocks down right hand side of face.

New- since returning to work, have noticed that my hearing on the right hand side is getting worse. Cannot hear in busy environments and rely on lip-reading.

The NS recommended that I see Mr Flint a Neuro surgeon at the QE in Birmingham who is the best in the Country. He agreed to see me in November 2007.

Mr Flint is a great man and I have a lot of time for him. He was extremely funny and understanding. He explained to us that he would be prepared to operate by doing a Third Ventriculostomy first because I had Hydrocephalus and that this was scheduled for March 2008.

This involved inserting a probe down through the top of the brain and burning a hole through the third and fourth ventricle to allow the fluid to move around more freely.

He felt that doing this first would help the fluid to disperse before he continued with the decompression surgery. Unfortunately after this surgery I had my driving license revoked for 6 months, something which I was not expecting at all. The operation went well and I recovered from the surgery within 6 weeks.

Things started to improve, until October 2008 when I developed such bad headaches, it felt my head was going to explode and this was a new experience by the severity of them.

I contacted his secretary who said that he would see me again in December 2008. He wanted to see if I had developed a Srynix and after having more MRI scans in February 2009, he said these showed that there had been a very small reduction of fluid, but that it had not moved the malformation at all. He was now prepared to do the decompression surgery to stop things getting any worse and so scheduled it for September 2009.

Surgery

I spent 9 days in hospital at the Queen Elizabeth in Birmingham, and my surgeon was Mr Graham Flint. He is a well-known surgeon through the Ann Conroy Trust and specializes in Chiari Malformation.

The decompression surgery consists of removing a small part of the skull at the back of the head, removing or shaving the C1 vertebrae to allow for more room and sometimes removing the cerebral tonsils. All this was done, but Mr Flint also found a membrane that was covering the Cerebellum.

This should have dissolved naturally when I developed as a fetus. He removed this and sent me back to the ward. Unfortunately, I was in a lot of pain, mainly due to not reacting, as you should to morphine. The operation started at 11.30am and I was back on the HDU at 4.30pm.

I was sick twice, but as soon as the medication was sorted I started to feel more human. I had 17 external stitches with the wound being about 5 inches approx. Mr Flint was very good about not shaving off too much hair and leaving the length to cover it up.

Post surgery

I traveled home to Cornwall with a car full of pillows and slept for most of the 5 hour journey. I think that I have recovered very quickly really considering having major brain surgery.

I write this at exactly 8 weeks post op and am hoping to go back to work this week. I am no longer on any form of medication on a regular basis, but may have Paracetomol at the end of the day if I have neck ache. The medication the hospital sent me home with was Codine, Diclofenac, Ranitidine, Senna and Paracetomol.

I do not have any headaches like the usual ones I had been experiencing. The operation does not cure things, but does stop the symptoms from progressing any worse.

Would I do it all over again? Yes I think I probably would.