Chiari Malformation, Writer's Blog

Help to Share the Friday Feeling

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I sent my husband a Whats App message during the morning, mentioning I felt tired and needed a walk in the sunshine. He offered to meet me for a coffee at lunchtime and duly arrived, meeting me with bare arms in the warmth of early Spring sunshine.

We wandered to the Cathedral Refectory, conveniently located a short distance away, both it and my place of work being located in the grounds of Norwich Cathedral. We enjoyed a drink and each chose a slice of home-baked cake which we hungrily devoured. Feeling stifled, I suggested a walk in the Cathedral Close. The infinite blue sky offered a vibrant backdrop for the cathedral, resplendent in its majesty. We are humbled to be afforded the enjoyment of witnessing such beauty during our midday break.

I was reminded of a story one of my friends from dancing had drawn my attention to while we were at Ceroc last night. The local news yesterday evening had shared the story of a young lady who is very sick and needs help to raise over £300,000 just to give her the chance of surgery to relieve and, indeed, save her. One of the conditions she is seeking help with, is a condition my own son suffered with and had subsequent surgery for, just four years ago. The condition is known as Chiari Malformation which is neurological disorder whereby the lower part of the brain is pushed down from the skull and interferes with the spinal cord. In short, it gets in the way of fluids needing to make their way past and the pressure on the base of the brain results in a number of painful and debilitating neurological problems.

The surgery required is called decompression and is normally available, as it was to my own son, on the NHS in hospitals such as Addenbrookes. The lady, Sarah Jackson, has other related conditions which prevent even the experts in the UK from attempting the surgeries she requires and she has to see one of the top specialists in the world who is based in America. He can operate and give her the best chance of a normal life but she needs to raise around £300,000 for that and the other costs such as flights, scans, etc. This all has to be raised within two months which will not be easy.

I found her ‘gofundme’ page online and on Facebook and researched her story further. If only I could win the lottery – this lady would be high on my list of those I would wish to help. So far, just over £14,000 of the £300,000 has been raised and I felt I would dedicate today’s blog post to sharing her cause in the hope that those who have a few spare pounds could click on the link and donate whatever they can.

If you can help by donating or even if you could just share this blog post or the relevant links below, you could just be helping to give this young lady her life back. A life she cannot remember as normal. Surly she deserves the chance to walk out at midday and see similar sights to those I have had the privilege of seeing today?

Sarah’s ‘Go Fund Me’ page can be found here:  https://www.gofundme.com/slsnbs

Sarah’s Twitter page can be found here: https://twitter.com/LifeForSarah

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Chiari Malformation

Living with a Chiari Malformation

My son was about 21 when he sat down one day and told me he was concerned about the head pains he had been experiencing on and off for a few months. It was the first time he had mentioned it because he thought it was nothing but then he started to realise that the shooting pains were happening every time he coughed, sneezed or bent his head forwards. He had a dull ache at the back of his neck which never seemed to ease, and the shooting pains were lasting for longer each time they happened and he was clearly quite concerned.  

We booked him an appointment with his GP and he asked me to go along with him to help reiterate how worried he was. The GP was thorough but felt it was more than likely to be a kind of migraine, especially as Daniel had experienced migraine in the past, and sent him away with some medication to try for a month. The pills didn’t work well for Daniel and his GP offered some mild anti-depressants which were for nerve pain but they just made him feel constantly tired, so we went back to see the GP. Each time, he saw a different GP. Dr Thomas was due to retire at the end of the week, so I am always thankful that we were able to see her as she listened as Daniel explained his symptoms and told us that she had seen a case many years ago of a teenager who had a Chiari Malformation. She offered a referral to a Neurologist at the local hospital and advised us to look up the condition to see if we felt it fit. It did.  

A Chiari Malformation is a structural defect in the base of the skull and the part of the brain which controls balance (the cerebellum). This usually sits in an opening in the skull, allowing the spinal cord to pass through it and is called the foramen magnum. When the cerebellum extends (a little like a tonsil) into this area of the upper part of the spinal canal it is called a Chiari Malformation. It causes pressure on the brain stem, can affect functions controlled in these areas and block the flow of cerebrospinal fluid (CSF) which surrounds and cushions the brain and spinal cord. 

Daniel attended the appointment with the consultant at the local hospital but as they had no expertise in Chiari Malformations they referred him on again to a consultant at Addenbrookes Hospital in Cambridge. The consultant at Addenbrookes, Mr Laing, was very attentive and made note of all of Daniel’s symptoms before carrying out some simple motor tests and requesting both CT and MRI scan appointments.  

Daniel’s scans showed that he did, indeed, have a Chiari Malformation and Mr Laing spent time going over the medical and surgical options and what they entailed. Daniel was not prepared to feel this level of shooting pain, insomnia, weakness and disorientation for the rest of his life, so he chose to undergo a surgical procedure called a Posterior Fossa Decompression which creates more space for the cerebellum, thus relieving the pressure and the pain. There are other symptoms which present in patients with the condition such as hearing problems, dizziness, difficulty swallowing and speaking, tinnitus, scoliosis (curvature of the spine), depression and hand coordination but not everyone suffers from every symptom. 

Daniel arranged to have his head shaved for charity (knowing he would need to have patches removed for the surgery anyway) and the local press came in to his place of work to interview him and take pictures which was a boost. 

The day of the surgery was, as always in hospital, a waiting game and we sat for hours until his slot came around. He eventually left the ward at around three in the afternoon and didn’t return from the surgery until gone eight that evening. The back of his neck was bandaged and sealed, and he was in a lot of pain, unable to move. I wanted to be nearby, so I rented a room on the Addenbrookes campus rather than travelling ninety minutes twice a day just to see him.  

In the days following his surgery, he was in so much pain which was traumatic for all concerned but immeasurable for Daniel. Unable to sleep, we kept his mobile fully charged to enable him to read the numerous messages from well-wishers.  The most poignant moment was when his twin brother, Matthew, visited him. They had not always been close (as many brothers aren’t) but the concern on his face when he sat at his brother’s bedside was an image which will never leave me. Daniel had so many people who wanted to visit him, his sister and brother-in-law, two step brothers and friends so we had to keep to a very strict rota and, although visitors were almost all from as far away as we were, we had to keep visits short as he was in so much pain. 

Daniel improved over the next few days and was discharged to come home to our house a mere eight days later. He seemed brighter and was asked, after discharge, to wait in the dayroom for various drugs. At that stage he seemed to go downhill, sitting upright for that long and he felt sick, dizzy and in terrible pain. The staff felt it was all post-operative pain and advised us to go home and monitor the situation. I should have been more assertive at that stage because we had to stop on the way home as Daniel felt so sick and didn’t know where to put his head to try and get comfortable. He was in sheer agony. Seeing a grown-up son crying in pain is heart wrenching for a mother. 

We monitored the pain for a couple of days but even the strongest painkillers didn’t help for more than a short while, so I called the GP who sent a locum around to check Daniel out.  Daniel was clearly in so much pain and the locum wanted him to walk up and down the living room and carry out motor tests which Daniel simply couldn’t do. He was moving Daniel’s head from side to side and making the agony so much worse. I had to intervene and get him to stop as he clearly didn’t know anything about the condition and the procedure. I was scared he would do more damage than good. We gave it one more day and I called Mr Laing who advised me to call an ambulance and get Daniel back to Addenbrookes straight away. 

Having been advised to go through A and E for processing, Daniel was seen quickly, and we were advised that he would go for another scan. He was given some stronger painkillers and we were told to go home and wait for news. He was sleeping soundly when we left him. 

I was awoken by a text message from Daniel in the early hours of the morning telling me that Daniel was about to be taken down for emergency surgery as the scan had shown that he had a build-up of fluid on his brain which was causing the extreme pain and that they needed to drill into his skull and get the fluid drained away. Daniel was panicking, and the text begged me to get to Addenbrookes. I called the neurological ward and was advised that he had already left and would likely be in surgery for an hour or so and he would be under anaesthetic so would not be fully awake until later in the morning. When we got in to see Daniel (visiting hours are unrestricted on the neurology wards) he was sitting up in bed and feeling what he described as much fresher. After a few days he went downhill again, and further scans revealed that the hydrocephalus (build-up of fluid) had returned. It was clear that his body had lost the ability to drain its own CSF and he would need a more permanent solution. The consultant discussed further surgery to insert a VP shunt which would remain in place.  

A VP shunt is inserted via a surgical procedure under a general anaesthetic. A catheter is threaded through an opening and into the brain and another is inserted under the skin travelling down to the chest and abdomen, where the drained fluid will be absorbed. There is a pump (or valve) placed behind the ear which automatically activates when the fluid increases. This can be set to different levels. 

Daniel came through this, the third surgery to his brain in as many weeks, and his recovery then started to pick up pace. He had to come to terms with no longer being able to play 11-a-side football due to the risks involved.

Daniel was lucky. He had an excellent surgeon and has come through the worst of this with his life intact. He still suffers with dizziness and disorientation, needing his shunt to be readjusted at times and still has some degree of constant head and neck pain. He is waiting for a referral to a head pain clinic at our local hospital where he has been advised that his best route now might be to have Botox around the decompression scar site. Time will tell but we hope he is over the worst of this horrible condition now.