Jean Shepherd

Firstly I feel it is necessary to state the following is not a criticism of the NHS for the time it took to receive a diagnosis or of the length of time between acknowledgement of something amiss and the resolution of that problem - it is just what happened. In March 2001 to be finally diagnosed with  Chiari Malformation (CM), a condition which could be helped by surgery, came as overwhelming relief. Although I appreciated the enormity of the situation at last there was confirmation of something wrong, and I had not been imagining symptoms as I had been led to believe. Personal research has shown how difficult this condition is to diagnose without a scan and many neurologists, let alone GPs, will not see cases throughout their working lives.

During childhood I had been labelled clumsy and the family joked I could trip over fresh air. There were many occasions of falling backwards instead of forwards, up and not down, together with severe headaches and occasional faints, but nothing to indicate an underlying cause. Other symptoms became apparent in my thirties but over the next twenty years the medical profession assured me it was the menopause - probably the longest one in history!

In May 1999 with the headaches increasing their intensity I had an accident which to this day I am eternally grateful for as it kept me going to the surgery until I had a resolution. I fell over a hosepipe left out by an attendant at the local swimming pool, twisted as I fell, slide along the floor and hit my head on the shower wall, no apparent damage just shaken up. However, the next day I woke with severe discomfort in the back of my head and neck. First visit to the surgery saw a locum who said, 'take two paracetemols regularly until the pain goes'; second visit again a locum with the same advice; third visit saw my own GP and told him 'it felt as though my spine was rammed up into my head'. I was quickly reassured this is not humanly possible (after diagnosis I realised I described the reverse action my tail pushed into my spinal column). A referral for a neck x-ray showed compression in the 3rd and 4th vertebrae and four weeks physiotherapy was recommended. The final day of treatment consisted of my head being held firmly and my right arm being pulled across my back, the pain was intense to the point of nausea and I began to black out. When I asked to stop I was told it couldn't be that painful. The pain in my head and neck continued until November eased by constant paracetemol doses.. Then I woke one morning to no neck pain, which seemed strange but was welcome relief, however the headaches continued.

Other symptoms were occurring: intense pain in my arm between my right shoulder and elbow; my hands going numb whilst driving; pounding headaches when sat on the loo; veering to the left or right when I walked; variations in hearing and vision; earache; twice I had blacked out momentarily; and sitting down at work made the bottom of my back pain.

Finally, after many visits to the surgery I had an appointment on a very bad headache day, and two weeks later in July 2000 saw a Neurologist as pressure had been noted behind the eyes. At the appointment the Neurologist stated he could not understand why the GP had referred me (no headache that day) but would arrange a scan to rule out possible causes. Finally in January 2001 I was scanned but was not given the result until March 2001 when I was told I had a condition which I was born with - CM. This could be corrected by surgery and referral had been made to a neurosurgery unit with the operation taking place within a fortnight. This proved not to be correct, my first hospital appointment was May 2001, when I was also told that next to my CM  I had an arachnoid cyst. Both conditions could be dealt with surgically and I had been placed on the waiting list. Whilst waiting for the operation my headaches intensified, due to the continuing growth of the cyst of which I was unaware, and medication gave no relief at all.

Eventually in January 2002 called for a pre-surgery testing day which included a scan. Then joy oh joy admission day came in February 2002. Ringing at 9.00 am to see if a bed was available, I was told there were none but to ring again at 11.00, then 12.00, then 1.00 pm still no bed. Then the surgeon rang and told me to make my way to the hospital, I would be having my operation the next day and there would be a bed available. We arrived at the ward about 3.30 pm and we were sent off to the admissions department, then back to the ward at 5.00 pm - still no bed. I didn't realise that behind the scenes juggling was taking place and eventually at 7.00 pm I had a bed. After more tests and finally a chest x-ray at 10.30 pm I was so tired nothing disturbed me that night. The next morning sped by and I was so relieved at 12.00 noon to go to theatre, I woke in recovery after six hours not really believing it was all over. Following the surgery I was told the cyst which had been golf ball size on my first scan in January 2001 had doubled in size by the next scan in January 2002, hence the increasing severity of pain. Also not all of the cyst could be removed and there was a small chance it could form again, and I will worry about that if it ever happens.

However, four days later I went home. The wound had internal stitches but no external stitches just butterflies holding things together and healed very quickly. It was wonderful to shampoo my hair ten days later. Apart from soreness at the operation site and occasional balance problems all other symptoms had disappeared. I felt so good after feeling so unwell for so long, that I returned to work in nine weeks, and in retrospect a few more weeks recuperating would have been beneficial. This didn't last and in October 2002 tests showed excess fluid in the ventricles and I had a tap placed in my head. I returned to work but six months later my husband was confirmed with Parkinson's disease so I retired early at 59.

Seven years on and occasionally the balance problems make themselves obvious also the hearing and vision can vary from day to day. Recently for over six months I had headaches which were beginning to approach the level of those I experienced prior to the operation but a scan showed nothing of significance. Acupuncture treatment has reduced the incidence and intensity of the headaches.

Assessing my experiences I would like to see more action taken to publicise the CM condition across all health disciplines, particularly those who work on head and spine including those in the alternative field. I understand the rarity of CM but far more people are being diagnosed. Unless there is more publicity, misconceptions about the condition will occur. I have been asked the following (i) did they operate by going up your nose (No wrong side of head); (ii) you are now cured (No apparently my CM was there at birth it will be there when I pass on, the operation has enabled me to live with the condition); and (iii) after explaining to a radiographer the scar on the back of the neck was an operation for CM got a raised eyebrow and, Oh really are you sure!!! Finally, I am very thankful there was an option available, with a successful outcome, to help me live with this condition. And immensely grateful for the skill of the surgeon, George Malcolm, and his team who carried out the operation at Frenchay Hospital.