- About the Conditions
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I was born on Easter Sunday 1965 in Whiston Hospital. This was in the days before ultrascans, so it was a shock to my parents when I was born with spina bifida, and hydrocephalus rapidly followed. I was immediately transferred to Alder Hey Childrens Hospital in Liverpool where I underwent corrective surgery. Shortly afterwards a VA shunt was implanted to control the hydrocephalus.
For the first two or three years of my life my parents were uncertain whether I would ever walk properly, or whether my bowel and bladder would function correctly. Fortunately I achieved these goals; I just took longer to learn them than my able-bodied siblings. The next challenge for my parents was to get me into my local mainstream primary school. By this time we had moved to Pennard in Gower and I have lived there ever since.
After a lot of negotiation and parental pressure I was accepted into first year at Pennard Primary School. Throughout my childhood I was an outpatient at Morriston Paediatrics Clinic, Swansea. I had annual check-ups for bowel and bladder functions. The VA shunt was also checked annually for blockages (a very common problem). I progressed through primary and comprehensive education with no health problems. At the age of sixteen I left school and then studied horticulture for a year to obtain a City and Guilds certificate.
When I was sixteen I was finally discharged from the paediatrics clinic and not transferred to adult outpatients. I was instructed to visit my GP if I felt unwell. After college I went onto a Government Scheme training course with Swansea Council Parks Dept. and was ultimately offered a job with a local golf club as Greenkeepers Assistant. The golf club did not ask any questions about my medical history and as I was fit and well I did not volunteer any information. I was afraid if I did, the job offer might be withdrawn. This was in June 1983 and I worked happily with no health problems until 2001.
Christmas 2001 marked the beginning of my life as it is today. When I awoke on Christmas Day I felt pain down the right hand side of my body; basically from my shoulder to my toes. The pain was similar to that of a nettle sting or possibly that experienced by children when they fall and graze themselves. I also began to experience bowel and bladder problems.
After the Christmas break I went to my GP who referred me to a urology specialist at Morriston Hospital. When learning of my medical history the urologist said I should never have been discharged at sixteen because of potential problems in adulthood with bowel and bladder functions. Ultimately I was referred to the neuro-surgical unit at Morriston Hospital. As my old VA shunt contained metal parts I was unable to have an MRI scan. I therefore had a different type of scan whereby dye is injected into the spine via a lumbar puncture. The results were inconclusive and it was decided to replace my old VA shunt with a temporary plastic one. During the operation I lost a lot of brain fluid because the VA valve was in fact partially blocked. I was told it could have been blocked for anything up to twenty years. (About the same time I was discharged from paediatrics).
The temporary shunt was fitted and an MRI scan duly carried out. The results showed I had Type 2 Arnold Chiari Malformation, also known as hind brain hernia, essentially the brain being too large for the skull. It also showed a Syringomyelia cyst inside the spine. As the cyst grows it makes the spinal bones rub on the nerves resulting in nerve damage and pain in various parts of the body. Following the diagnosis the temporary shunt was replaced with one utilising a low pressure antisyphon VP delta valve. This has now lowered pressure on the brain but has resulted in a chronic headache.
As neither the chiari malformation or Syringomyelia were operable, the only alternative was pain management. A wide range of oral and skin patch painkillers were tried with no success, though some did have very unpleasant side effects. During this period I also tried numerous alternative treatments including acupuncture, body temperature salt water float pools and hydrotherapy. The latter treatment was in Singleton Hospital on the NHS. Some relief was experienced but only whilst in the pool. Eventually I was admitted to the neurosurgical unit at University Hospital of Wales Cardiff. Over a period of ten days various drugs and placebos were injected directly into the spine via a lumbar puncture. I kept a daily diary but none of the drugs were successful.
Since the pain problem began in 2001 it had gradually spread throughout my body from head to toe. In 2008 I was admitted to Morriston Hospital again for an operation to check the state of the shunt. Apparently it was in fact working perfectly.
In March 2010 I was referred to the Walton Centre for Neurosurgery in Liverpool. On my first visit a neuro spine stimulator was discussed. Basically a battery operated device is implanted into the abdomen. The stimulator is then connected, via wires under the skin, to targeted areas of the spine. When it is switched on using a hand held remote, an electrical impulse is passed through the targeted area blocking the pain messages to the brain. Pain is replaced by a warm tingling sensation; a much more pleasant option. After careful consideration of all the facts I was placed on the waiting list for a trial run lasting about a week.
In January of this year I was admitted to Walton for the trial. This involved having a local anaesthetic and lumbar puncture; wires were then fed into the spine using X-rays to guide them to the target areas. The wires were then connected to a hand held remote control and switched on. The unit was linked to a computer and programmed by the consultant. As explained, it was not possible to target all the pain with one stimulator. Primarily the aim was to control the pain in the hips and legs. As the stimulator strength was altered I was asked where in my body I felt the effects. This was why the procedure was carried out under local anaesthetic. As the trial showed a positive effect on the leg pain I was placed on the waiting list for the implant which involves major surgery.
The operation was carried out in June and during the procedure I suffered a leakage of spinal fluid. This resulted in my experiencing the worst headache of my entire life. During the post op recovery I was connected to a morphine drip for the post op pain and 48 hours flat on my back for the headache. This resulted in my bladder ceasing to also violently sick. As I began to feel a little better, two members of the team began to instruct me in the use of the stimulator. This latest version of the stimulator now comes with rechargeable batteries, hopefully to prolong its life to about ten years. The downside is the batteries must never be allowed to run flat, as this would mean the stimulator could not be recharged. Consequently it would have to be surgically removed and then six months later a new stimulator would be implanted! This possibility made me quite apprehensive and I am currently charging the batteries everyday. I am slowly getting used to using the stimulator and I am very glad I decided to have the implant. My leg pain has been replaced by a warm tingling sensation and this has helped me cope better with the upper body pain.
It is now five weeks since the operation and I have just been back to Walton for my first check up. The stimulator has now been programmed into three separate files, namely sitting; standing /walking; lying down. The stimulator should now automatically be able to tell when I change position and alter the programme and intensity of stimulation accordingly. My next check up is in five months time just before Christmas. Looking to the future there is perhaps the possibility of another implant to help with the upper body pain. However it is early days yet and I need to see how the next year goes.
Summing up; after my diagnosis in 2003 I researched my conditions on the internet and found the Ann Conroy Trust and the Association for Spina Bifida and Hydrocephalus both excellent for advice. As I could not find anyone on line with exactly the same condition, namely type 2 chiari malfunction, Syringomyelia, Spina Bifida and Hydrocephalus, I felt frightened and alone on my long journey to where I am now. I hope my story will help anyone in a similar position to soldier on and not give up hope. My advice to parents with spina bifida children is to insist on regular check-ups throughout adulthood rather than just childhood. Also seek medical advice when choosing a career. Although I thoroughly enjoyed my twenty years as a green keeper it was very physically demanding and could possibly have contributed to my health problems.