Tethered spinal cord syndrome

Tethered spinal cord syndrome is an unusual condition, thank goodness. I was almost certainly born with it, but for 58 years was unaware of the fact. By and large I fitted into the broad spectrum of “normal kids,” despite a well concealed secret sense that I was not quite right. I accepted reluctantly that I couldn't run as fast as other boys in Bath, or play football as well. I got on with the things I was good at, as most people do.

In my 50s I began to suffer more and more with my lower back and legs, for which pain relief and physiotherapy were offered. Bladder control became more tricky and daily life a bit of a struggle. I kept working, reluctant to admit all this to myself. In the mid- to late 1990s, while living and bringing up a family in south London, I saw a urologist in a London hospital. He assured me I didn't have prostate cancer. I had never thought that I might have prostate cancer. The specialist put aside his colleague's findings that I was retaining large quantities of urine in my bladder. Prostate cancer was his enthusiasm, whereas bladder control was someone else's department.

In 1998 my wife and I moved back to Somerset. The episodes of back pain became more frequent. It was a physiotherapist in Frome, in 2002, who set me on the road to recovery. Studying how my body, legs, pelvis, and spine moved in obvious disharmony with one another, she finally confirmed that incoherent boyhood feeling of something not quite right about me. When my general practitioner, a kindly compassionate man, argued that I had managed 58 years with my funny body and would probably manage a few more, my physiotherapist put considerable pressure on him to refer me to a specialist.

The next stage (early 2003) was the most frightening period. I had to cancel a holiday at short notice “just in case.” In case of what? Various explanations were offered, none of which I understood. My body became a site of special scientific interest—or so it seemed—as I was subjected to more and more tests at the Royal United Hospital in Bath. The scans were fine: the staff told me what was going to happen, and it did. But the lumbar puncture hurt a lot and no one warned me of possible after effects as my body tried to compensate for the loss of those few precious drops of spinal fluid. As for having electric currents passed along nerves, the “light electric impulse” I was warned to expect struck me as more like jumping feet first into a mains electric circuit.

It didn't help that I had three consultants in turn, although eventually I did meet someone who understood my condition. That was Mr Ian Pople, consultant neurosurgeon at Frenchay Hospital in Bristol. I knew he was the man. He had seen my scans, and instead of wanting to look at my tired back he asked me to remove my shoes and socks and roll up my trouser legs. It was a thrilling moment. He admired the crabbed toes on my right foot and then whipped out his tape measure and confirmed the 3 cm difference between the healthy left and shrunken right calf muscles. We smiled at one another in mutual recognition. His assistants gazed in awe.

That was in February 2004. Mr Pople wanted to operate in the spring, but it wasn't quite that easy. As a self employed writer and researcher (and far from being a bestseller), I needed time to build up a fighting fund to see me through a long convalescence. No problems about waiting lists here—it was I who needed more time, not least to sort out my feelings. I had put aside those childhood doubts, that feeling of being not quite right. I had spent a lifetime pretending that all was well. Now that was coming to an end. A lifetime habit of trying to be strong and independent would have to be set aside, at least for a little while. I had never been a hospital inpatient. I couldn't see beyond the operation. I was scared. But I did keep up my exercises and my swimming, knowing that a fit patient might stand a better chance of recovery.

On 10 August I woke up singing my heart out in the recovery room at Frenchay Hospital. It was a good omen. Much to my surprise, and I think that of my family too, I settled into the hospital routine. For five days I was required not to move. I had expected this to be an ordeal but it wasn't, largely thanks to the people around me. I was a patient patient (“stoical,” said one nurse, a word no one had ever used about me), phased by morphine, but pathetically and genuinely grateful for each cup of tea, each pill, each wash, each visitor, each whispered conversation with the night nurse who came to turn me in the predawn grey. I owe so much to those nurses.

I was in ward 3 of the old (1942) wartime emergency hospital, which had been put up quickly next to Frenchay Common for North American servicemen. I loved the fresh air—windows open all day, and night too—and the delicate light flooding each morning and evening through the high, rusty Crittall windows. Everything and everybody in the place helped to make it easy to be a good patient. I was proud to have been born at the time of the NHS, perhaps the most solid national achievement of the often depressing 20th century. Nothing that I do from now on in my life will erase the memories of the unstinting patience and care I received at the hands of the staff at Frenchay Hospital.

Within 12 months of the operation the world looked a very different place to me. I get occasional aches and pains, but what 60 year old doesn't? I am not in daily, constant pain any longer. I am back at work again, as busy as ever. I have joined a community choir after a lifetime of claiming that I can't sing. In 2005 I celebrated my 60th birthday, surrounded by family and friends. I wake every morning—OK, most mornings—feeling that I am a lucky man.

As a writer it would have been neat for me to leave the story at that happy ending. But real life is not like that. The one aspect of my health that has not resolved itself is my bladder problem. Since the spring of 2004 I have been on a programme of regular self catheterisation. Over the past two years many of the problems relating to my early contacts with the neurology department have recurred. I am a non-urgent case in a urology department full of urgent cases. Some tests have been quite painful, and the hospital has not been good at communicating test results. I try not to let my bladder problem interfere with my sense of optimism and wellbeing, but inevitably it does.

The skill and care of individual doctors, nurses, osteopaths, and physiotherapists have given me a quality of life I would not have dared to hope for a few years back. Yet there are still questions in my mind about how well the various parts of the health service actually combine with one another. All too often I have felt stranded in a medical no man's land between doctor, hospital departments and clinics, tests, and laboratories, and not least my own ignorance of medical matters. At times being a good patient has been hard work.

A medical perspective

Compared with the ubiquitous lumbar spondylosis or disc prolapse the tethered spinal cord syndrome is a rare cause of back and leg pain in adults. The underlying cause of the tethering process is usually congenital spinal dysraphism (a mild form of failure of neural tube development or occult spina bifida). This ranges from a simple thick, fatty filum terminale to more complex lipomas or congenital malformations in the lumbosacral region, holding the spinal cord in a taut low lying position.

A tight, thickened filum terminale can sometimes be missed on routine magnetic resonance imaging of the lumbar spine, so a high index of suspicion during the investigation period is often required to clinch the diagnosis. The pain is caused by progressive stretching and ischaemia of the distal spinal cord and its emerging nerve roots. Usually an associated impairment of bladder function and lower leg function occurs, with clawing of the toes, sensory impairment, and discrepancies in shoe size.

The symptoms and signs of the tethered spinal cord syndrome are often insidious in onset and subtle, as aptly shown by Mr Payne's account of his protracted diagnostic investigations. His initial presentation in childhood with a weak bladder and problems with his legs were early clues, but the lesson here is that the syndrome can progress beyond the growth spurts of childhood and adolescence, well into adulthood. External signs of spinal dysraphism, such as midline dimples or patches in the lumbosacral region, may not always be evident, and leg asymmetry or clawing of the toes can develop as new features in adulthood.

The onset of unremitting back and leg pain that Mr Payne experienced is common as the tethering process worsens but usually responds well to operative detethering of the spinal cord. Bladder impairment is much less likely to resolve after surgery because of the vulnerability of the nerves supplying the bladder to permanent damage from progressive tethering. In some patients the best that can be hoped for from surgery is cessation of neurological deterioration, but in over half of patients there is the hope of sustained symptomatic improvement, even after many years of having the syndrome as an adult.

Lingering difficulties after successful detethering are the norm with such a spectrum of chronic neurological damage, and early referral of patients with this condition will help surgeons to optimise the timing of detethering. This would hopefully lead to fewer patients having to endure the same convoluted pathways through the NHS described by Mr Payne.

Ian Pople, North Bristol NHS Trust, Frenchay Hospital, Bristol BS161LE ian.popl