Web Posted Nov 17, 2001

Web Posted March 21, 2002

On the morning after I was born, my mum was informed that I had a small abnormal dimple at the base of my spine. However, the paediatrician had examined it and thought it was OK. Mum was so thankful to have had an otherwise healthy first-born daughter so she did not worry unduly about it.

Little did my parents know that I had congenital spina bifida occulta, manifesting as a closed dermal sinus tract with a tethered cord (thickened filum terminale) and loss of spinous processes. While I was growing up the medical people did not seem to realise how much it would progress and affect my life.

I crawled early, but walked late as my ankles were too weak to hold me for any length of time. I used to cling on to the furniture, sit down suddenly, and absolutely refused to walk between gaps - I crawled instead! I was fortunate not to have problems with toilet training. At about 2½ years old, my feet started to show the symptoms indicating a tethered spinal cord: claw toes, tight toe tendons, shortened Achilles tendons, large big toe joints, and very high arches (bilateral pes cavus), which caused me to walk on tiptoe. I had operations on both feet at the ages of 11 and 12, which included the lengthening of both Achilles tendons, and the release of the tight tendons of two toes on each foot. This allowed me to remain mobile. Running was a problem but I was determined that my feet would not stop me doing as much sport and gymnastics as possible at school. OK, I can't run the 4-minute mile, but when I could not afford a car, I walked everywhere, and took suitable rest stops. I am also a very strong swimmer.

Progress of the condition and worsening symptoms

My condition was stable between the ages of 12 and 34, with no nerve pain or warnings that the condition of the cord was deteriorating. However in the mid-1990s new symptoms began appearing; hip, knee and ankle joints began to stiffen up; my left foot, always the weakest and most deformed, experienced excruciating pain within the ankle joint, and my ankles swelled to huge proportions whether it was hot or cold weather or whether seated or taking exercise. All these problems were very frustrating particularly as I did not know the cause for their occurrence. All the symptoms were reported to my GP, were investigated separately and diagnosed variously as osteoarthritis, synovial leakage from joints, or poor vascular tone. Advice was also given just to take more painkillers. It was not linked by the specialists, or myself, to the dysraphism.

Early in 2000, I began to get excruciating sciatica pain down the back of my right leg. As I am usually so active, I attributed this to "too much swimming" or "digging the garden too much". However the pain did not go away as muscle pain usually does and indeed it increased exponentially. Some days I could not climb the stairs, or I became rooted to chairs as my legs were too stiff and painful to get me up again. I often felt as though I was sickening for 'flu, aching all over, really feeling "blah" and drained of energy and stamina all the time. An orthopaedic consultant was of the opinion that all these symptoms were linked and were caused by the deterioration of the underlying dysraphic condition, in that the tethered cord and its associated nerves had begun to complain of too much stretching and tension. After an MRI scan in double quick time, I was referred to Mr Ian Pople, Consultant Neurosurgeon, at Frenchay Hospital, Bristol, UK

Thoughts and Feelings

I continued to work but the nerve pain was grumbling away. It took the form of a constant dull ache in both my legs and my back which would unexpectedly emerge as a "bad period" of pain flare-up, with "burning" legs, sharp sciatica nerve pain down the back of both legs, and alternate fiery tingling in the toes or numbness in the front of my feet. These symptoms would always appear without warning and without precipitating circumstances. I would fruitlessly take over-the-counter painkillers.

At last the appointment day with Mr Pople came in October 2000 and my fears were realised. There was no (sensible) way to avoid undergoing surgery to release the tethered cord. If I did not have the detethering I would probably be incapacitated within two years. He assured me that although my cord was tethered "pretty severely" the type of tethering was at the low end of the risk spectrum to correct, and that the operation would give me relief from my symptoms by about 80%. We addressed my fears, the risks and, of course, the benefits of the surgery and set a date for the end of July 2001.

Operation

The operation did go ahead in July and it took a lot longer than expected to return to any semblance of fitness but ultimately the surgery has been a success. My biggest fear, of losing leg function and sensation, has not been realised, my bladder and bowel control is now assured, and most of the post-operative pain has disappeared. It occasionally returns if I do too much.

Nerve sensations post-op included very "buzzing" feet, the underside of my feet would go cold and numb and my feet are also continuing to swell up with apparently no specific precipitating reason. It is therefore evident that a spinal cord detethering may not totally eradicate this problem associated with poor nerve signals and deficient vascular tone. This is also very disappointing as I had hoped that this one distressing symptom of tethering would reduce or even disappear.

A unique nerve sensation appeared at ~24 weeks post-op. At the point of detethering in the lumbar spine, I have tickly sensations and as though something is "loose" inside my back. I have been assured that this is a common occurrence after a detethering. It could be positive nerve growth and regeneration or has been attributed to the random firing of nerves at the end of the spinal cord. At last, something good to report on the subject of nerve sensations!!

I continue to work full time. I am hoping that I do not retether and that I don't have to go through the detethering operation again. My website site at http://www.btinternet.com/~tetheredcordresources has been rewarding to write, approved by Mr Pople and well received by readers. I wrote it after considerable research into the condition, and I would hope that it helps other people awaiting detethering surgery. I felt so lost, alone, and frightened when I was first diagnosed, although Laura V. was a source of information on the condition, and of friendship which cannot be underestimated.

Web Posted Nov 17, 2001

Web Posted Nov 11, 2001