Tethered Cord - A Personal Story
Birth
Childhood
Family
Progress
of condition
Thoughts
and Feelings
Surgery
and postoperative progress
6
months report (February 2002)
12
months report (July 2002)
22 months report (May 2003)
36 months report (July 2004)
Almost 5 years (February
2006)
Acupuncture
Birth
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. It was covered and did not extend into the depths of the spinal column, although a bony element was very close to the surface. 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 (L5-S2 and diastematomyelia at S2) and loss of spinous processes. Congenital spinal problems were relatively unknown by the general public in the 1960s, and while I was growing up, despite the visible symptoms of feet deformities (see below), the medical people did not seem to realise how much it would progress and affect my life. No-one explained fully the significance of the sacral dimple and its connection to my bilaterally deformed feet. Once I learnt about and researched the problem of tethered cord syndrome, the fact that the medics knew about spina bifida occulta and tethered cord in the 1960s but never offered me or my parents any advice, used to make me really angry and resentful. But now I realise that surgical techniques for spinal cord detetherings were crude in those years and it was fortunate that I was brought into contact with Mr Ian Pople at the right time (in my 30s) and place (living in Bristol).
I am told 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 2˝ years old, my feet started to show the symptoms indicating a tethered spinal cord, although the name of the condition was never given to me or my parents: 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. My parents were assured that there would not be major problems but after being seen by a paediatric orthopaedic surgeon at Stoke Mandeville Hospital, Bucks, 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 toe tendons. This did not cure the problem of pes cavus, but it eased some of the symptoms, i.e. my heels could again touch the ground and 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.
I had a relatively "normal" childhood, running around with my younger sister, being as mobile as any other youngster until my legs weakened, and not being cocooned and protected. In my teens and 20s, I was OK, although I regularly had a very aching left leg, both legs felt tired and burning if I walked too much, I could not stand in one place for long, or, for example, go hiking or do lots of tourist walking in cities. I was very active, sportswise, as I did gymnastics (how? I don't know!) and trained, and still do, with triathletes for the swimming section. There was no inkling of the ongoing deterioration of my tethered cord and I had to learn the hard way, as detailed below, and I experienced very rapid neurological deterioration and horrendous nerve pain in my 30s.
My condition had a certain effect on the rest of the family but there was never an assumption that I had a disability. My sister (three years younger) says that she has always known me with different feet, but that they never bothered her. She did wonder when she was 7 why I was "lucky" enough to be getting hand-made shoes, and she did have to endure hospital visits at an early age. However, she and Mum have told me independently that they became defensive of me. One of the biggest problems for all of us to cope with was that people used to stare at my feet, and still do. I am tempted to point out to the starers how rude they are, but my response depends on what sort of mood I am in! I know that people are just ignorant of the true reason for the feet problem and just plain rude but even at my age, their actions still hurt emotionally.
In 2003, I wrote this to a reader of my website about the "staring"
issue:
"My first reaction used to be "how dare you stare at me, you ignorant
b....". However, with further acceptance and knowledge about my condition
I have come to the opinion that sometimes I like people to ask me directly why
my feet are deformed, rather than the usual staring-looking away-looking back
routine that usually happens.
Adults should know better that staring is rude, but I get just as annoyed with kids, and the snide remarks behind their hands to their chums really gets on my nerves (excuse the pun!). However, my reaction is now not so full of hatred and violence. If the kids are old enough I try to explain the problem in simple terms so that the deformity is not so frightening to them. I also try to show them how it feels to be stared at, by gently questioning how they would feel if they constantly had people looking at them.
It depends how you feel about yourself as to how much you want to explain to adults what the condition is. One person I know says "oh, just a problem I have with my back" and "it's a weird form of spina bifida" and if they continue to question, then she stuns them with her knowledge of the minutiae of all forms of tethered cord syndrome!"
Spina bifida occulta with a tethered cord may not necessarily be a totally physically disabling condition, although it can place limits on the amount and type of physical exercise that one can do comfortably. OK, I can't run the 4-minute mile, but when I could not afford a car and before my spinal cord started to deteriorate, I walked everywhere, and took suitable stops to rest my weaker left leg and ankle. I am also a very strong swimmer as my upper body is not affected by the cord damage.
Progress of the condition and worsening symptoms
Spina bifida occulta and cord tethering is a congenital condition and without careful monitoring, the neurological damage and other problems are progressive (see Tethered Cord page). In my case, 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. During these years, I achieved my qualifications, I attended Keele University, Staffordshire, which included extensive travel in the US on an education exchange to Ball State University, Indiana, and earned a good degree in 1994. I now enjoy a rewarding and responsible job at the University of Bristol.
In 1990, I contracted cellulitis which would have long-term effects on the condition of the vascular system in my feet and began the first symptoms of Erythromelalgia (EM). In the mid-1990s new symptoms relating to the tethered cord began appearing. My hip, knee and ankle joints began to stiffen up, especially after periods of inactivity. My left foot, always the weakest and most deformed, experienced excruciating pain within the ankle joint and would collapse sideways, often without warning. My ankles swelled to huge proportions whether it was hot or cold weather or whether seated or taking exercise. The left foot became less and less responsive and its flexibility diminished, with the toes also becoming tighter and more curled; the right foot gradually tightened to a lesser extent. I just thought it was me getting older, never realising it was linked to the original problem of spina bifida occulta, and never ever knowing that I had a tethered cord.
These problems were very frustrating particularly as I did not know the cause for their occurrence. All the symptoms were reported to my GPs at university and in 2 different cities, 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. They were not linked by the specialists, or myself, to the spina bifida occulta and at that time, in my ignorance, I had no idea that I had a tethered cord that was gradually deteriorating, or that the swelling etc. was the first sign of the EM which would come to figure so large from mid-2001 onwards.
Early in 2000, I began to get excruciating sciatica-type pain down the back of my right leg, with a deep aching and throbbing on some days. The nerve pain was quite unique and in 2004 I characterised it as:
"Shooting, aching, fizzling,
Burning, crawling, numb,
Hot or cold, dead or alive
Walking on a bed of nails
Friend to warn of potential for damage,
Foe for not letting me go.
Feet burning like the oven's fiery flare
Toes dead and suddenly coming back to life.
The unknown, the fear,
Will I ever get out of here?
Could it be, that due to this pain
I may never walk again?
Risks, benefits, surgery or not
Will it turn off the Pain and the Rot?
Blasting, tearing, my whole body cries "ouch",
Relief in drugs, but coming back again,
I just want "out"."
As I was usually so active, I attributed the pain to "too much swimming" or "digging the garden too much". However the pain did not go away as muscle pain does and indeed it increased daily and exponentially. Although I continued working full time, with no sick leave taken, on some days I could not climb the stairs, or I became rooted to chairs as my legs were too stiff, tight 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. After I stressed the debilitating effects of my condition, I was referred by my new GP to an orthopaedic consultant at Bath's Royal United Hospital. He 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 suffer from too much stretching and tension. He booked me in for an MRI scan in double quick time, and soon after I was referred to Mr Ian Pople, at Frenchay Hospital, Bristol, who is an international expert on spina bifida and its associated conditions.
I had to wait three long months to see him. I still worked full time and also did freelance work but the nerve pain continued. It took the form of a constant dull ache in my legs, my right hip and my back which would unexpectedly emerge as a "bad period" of pain flare-up, with "burning" legs, sharp sciatica nerve pain in my right hip and down the back of both legs, alternate fiery tingling in the toes or numbness in the front of my feet, and sharp twists of pain in the lumbar and sacral spine and buttocks. These symptoms would always appear without warning and without precipitating circumstances. I would fruitlessly take over-the-counter painkillers.
In my diary I raged against this condition: I had never heard of tethered cord, or tethered cord syndrome, and felt so angry that why, when my life was at last on track, did this THING have to rear its ugly head in such a dramatic way? I had thought that the operations as a child would be all that would be required. At this time I was not aware of the full nature of the condition. I also did not realise the possible devastating consequences (to motor and sensory function, and continence) of progressive neurological stretching and damage to the spinal cord.
I tried to find information on the internet about tethered cords, but most UK sites seemed to be focussed on open spina bifida. That was all very good, and such resources are needed, but I felt so lost and alone. Even the Asbah site does not cover the subject in any detail. There was very little about covered (occult) tethered cords, and any information I could find was scattered on American sites, often from university or hospital medical schools. The American medical establishment is very different to that of the UK and some of the information posted on chat pages appeared to be alarmist, incorrect and uninformative, or alternatively, very complicated and hard to read and find relevant information. When I read that the filum terminale had to be cut to release the tether, I just panicked - surely that would mean instant disability as the spinal cord would not be able to send messages to my legs. How misinformed I was! Having fought against my feet all my life to be as normal in activities as possible, despite my deformed feet, how on earth could I deliberately opt for an operation that would take away all that I valued?
How could I go through spinal surgery with the potential for failure, neurological deterioration and further damage, and incontinence? I just wanted to put it off further and further into the future so as not to face those possibilities. With hindsight (in 2004), that was a limited attitude and point of view, particularly when I was being operated on by the leading (and best) neurosurgeon in the country. I am sure that the delay had detrimental effects on the outcome, although it has been a success overall, in that I am still walking, despite now living with chronic pain. Also, I should have trusted Mr Pople more - he is the best in his field, with high success rates and clinical outcomes, and a wonderfully kind person as well.
I also detest the appearance of my feet so much, and my dearest childhood wish of perfect feet and legs never comes true. All the associated problems and pain just compounded the "differentness" of my feet. These three months were full of pain and frustration as I had so many questions that needed answering but I had no-one qualified to talk to. At that time I had not made contact with the medical advisors at Asbah or anyone with this condition using the internet.
At last the appointment 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 the symptoms and progressive nerve damage would continue and I would probably be incapacitated within two years. He assured me that although my cord was tethered "pretty severely" at S2 in a stretched bowstring formation, the type of tethering was at the low end of risk to correct, and that the operation would give me relief of about 80% from my symptoms.
In the following weeks the fears, worries and hate all crowded in and dominated my life. I tried to keep all these worries to myself but inside my tearful depression grew. Everything seemed black and threatening, my concentration at work suffered, and 5 months later I was signed off sick for stress. However I contacted Asbah in Peterborough and Paula Thompson and Rosemary Bachelor, the medical advisors, were full of information and very helpful. In the local area Mr Pople's secretary put me in contact with Julie Knight, who was an advisor for Asbah; she was a godsend as she listened to my concerns especially when they all seemed too overwhelming. She could give impartial advice and guidance, drawing on her experience of talking to others with this condition. I obtained another appointment with Mr Pople and we addressed all my fears, the small risks and, of course, the benefits of the surgery. The operation seemed less frightening, and although the fears and worries remained, after talking over the details of the surgery and the recovery period, I felt better able to cope with whatever was coming. We set a date for the end of July 2001.
In the time between seeing Mr Pople in March and the surgery, I was so busy at work that the forthcoming hospitalisation was put to the back of my mind. However, my preparation for it had to continue. I researched and wrote the first drafts of this website, I prepared a job handbook so that my long absence from work would not be too disruptive, and I gathered information about the logistics of hospital admission by visiting the ward at Frenchay Hospital to which I would be admitted. The Sister-in-charge was extremely helpful, answering my many questions on pain relief, bed rest, subsequent mobilisation, and the immediate recovery period. At the time of writing this section (end of June 2001) I wanted to get on with the operation and begin the recuperation period, and ultimately to get my life back on track.
At 6 months post-op an update on my situation is required.
The operation did go ahead on 31st July 2001 and it has taken a lot longer than expected to return to any semblance of fitness. 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.
To help readers who are facing a detethering operation, the following points may be useful. However do remember that each person's pain threshold is different and the individual circumstances of your cord tethering will cause you to have different experiences of surgery and recovery.
Hospital Period
Right after surgery I did not want to move at all as everything hurt far too much. I was told to lie totally flat for 3 days to ensure no CSF leak or other complications, and this was always on my side, usually propped up by a pillow to avoid pressure on the operation site. I didn't dare roll on my back due to the horrendous pain that would ensue. Generally if patients are able, they are encouraged to turn themselves. On the first couple of days, I found it excruciatingly painful to turn over, especially as my legs were very weak but the nurses helped me and nothing was too much trouble if I asked. Unfortunately I kept compressing the midline operation site accidentally as I turned over. The pain was an incredibly sharp, severe stabbing pain, all up my back, down both legs, along the incision site and of course inside my lower back where the filum had been divided.
Lying in bed for long periods can also restrict circulation, possibly leading to DVTs, and so I was told to keep flexing my legs below the knees to keep the blood flowing. I did this as soon as I could on the operation day to assure myself that I could still move my legs and that I was not paralysed after the surgery. Catheterisation dealt with toilet issues.
On days 3 and 4 moving around in bed became easier. The fiery pain diminished slightly for short periods with the help of regular painkillers. Some leg strength also gradually returned. This gave me more leverage to turn over without putting undue pressure on the incision site, and I was allowed a slightly bigger pillow, which was more comfortable. On these days, the stress of the operation and continuous pain took its toll, as I felt extremely tired, weak, exhausted and constantly weepy.
On the 5th day I was mobilised i.e. got out of bed. This moment was anticipated for so long and when it came it was so painful! One does not realised how much pressure is put on the lower back even just getting out of bed and as I have a 4"-long scar down my lower back the pain from it all was overpowering and literally took my breath away (there is no way to disguise that fact!). Once I was upright it felt as though my spine below the ribs was being squashed and compressed, but it was such a good feeling to be up and walking again. My legs were still relatively weak but I took short assisted walks from my bed to the sun-filled day room and by the end of the day I could manage to walk the length of the long ward independently. Unfortunately one unexpected consequence of mobilisation was the most humungous migraine as the CSF fluid readjusted, but that only lasted one day.
I was allowed home on Day 6 to recuperate as the doctors had monitored my voluntary mobilisation and said I was mobile and safe enough to leave hospital. I was fortunate to have close relatives with me to help with recovery at home. I certainly needed their help, especially for personal care, until the stitches came out 10 days post-op at the local GP's surgery.
I think my stay in hospital was relatively short as my consultant had originally indicated a 7-10 day stay, but my initial progress was much better than expected. The important thing to remember is to do EXACTLY what the health professionals (your consultant and the nurses) tell you as they have extensive experience of detethering patients. The key points to a successful mobilisation seem to be:
Pain and sensations
In my case after surgery I had pain in my back, and buttocks, and down my legs, much the same as pre-op. I soon worked out which pain was which - bone pain (dragging pain/ache), nerve pain (like sciatica, which remained from before the surgery), operation pain (muscles and skin healing) and detethered pain. This is the hardest to describe: it was a sharp but blunt type of pain that would suddenly jab me in the lower back where everything was starting to heal. All these types of pain remained for approximately six weeks, but gradually diminished. The detethered pain still occurs very occasionally even at 3 years post-op. The sciatica in my right hip and the hip stiffness in the mornings both remained at 12 months post-op.
The pain was not constant though. I would have good days when the pain levels were relatively low and on those days Mum and I would be tourists, visiting the Eden Project, Exmoor, and south coast beaches. However the following day I would pay for my (over) activity by being forced to lie on the floor due to the return of the pain, unusual nerve-related sensations and total exhaustion.
For adult patients, it must be noted that I was not allowed to drive for 3 weeks, or swim for 4 weeks. Driving places extreme pressure on the surgical scar (both inside and outside), compromises the ability to react quickly to situations, and ultimately is extremely tiring. I am an experienced, long distance, motorway driver, but it took me at least a year to be able to do 120 miles to my mum in one trip - I had to take at least 2 stops to get out and stretch or rest. Swimming had to be avoided until the incision was completely healed and the external scarring had fallen off. The consequences of getting an infection in the CSF would be unthinkable.
Other sensations post-op included very "buzzing" feet. It was as though an electric current was passing through my feet, but it did not travel up my legs. It was also as though pins were being stuck into my toes and along the sides of both feet. A friend suggested that this could be the nerves trying to work but not quite being successful. This sensation of varying intensity still comes and goes without warning and depends on how much walking I have done. Along with this, the right forefoot still goes numb (late 2003). Another (very unwelcome) new sensation was that the underside of my feet would go cold . Again this was probably all the nerves trying to work correctly after 35 years of sending distorted signals. Thankfully this sensation did not stay long.
The skin on my back around the operation site felt "dead" as though it was anaesthetised, and had no sensation whatsoever. This was due to the skin nerves being shocked, but it did not affect motor function. Sensation gradually returned over the year, and by 2 years post-op my back has become oversensitive again.
My feet also continue to swell up with apparently no specific precipitating reason, except being hypersensitive to heat. 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.
See the 12 month and 22 month updates for more on this aspect of tethered cord syndrome and the 30 months update for the diagnosis of Erythromelalgia
A unique nerve sensation appeared at ~24 weeks post-op. At the point of detethering in the lumbar/sacral spine, I felt tickly sensations and as though something was loose inside my back. I was assured that this is a common occurrence after a detethering. It could be 2 things: either 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!!
Pain relief
When I had my feet operated on as a child aged 11 and 12 I was, amazingly, refused pain relief. This was therefore one of my biggest concerns for this period of hospitalisation, but my worries were unfounded. The nursing staff kept a close eye on my pain levels and for the first two days I was given a PCA (Patient Controlled Anaesthesia) machine, whereby I could press a button and a measured amount of morphine and cyclizine (an anti-emetic) would be delivered. However, I didn't use this particularly well, especially immediately post-op, and it made me feel very giddy and gave me very odd dreams, so my pain levels were constantly high. The nurses therefore gave me oral painkillers (Diclofenac and Paracetamol) at regular intervals, which were much more effective and I would recommend them over the PCA. To rest at night I was prescribed sleeping tablets. They were also very effective and enabled me to have relatively long periods of restful sleep rather than waking every 10/15 minutes.
When I left hospital, I was given a bag of potions consisting of Paracetamol, Diclofenac, and a week's supply of sleeping tablets. In the first week at home I used the sleeping tablets to ensure I got some rest, but the specific painkillers were used sparingly. I regarded the pain as a warning sign not to overstretch my physical limits and gradually it diminished over time. In February 2002 I used Diclofenac on a regular basis to dampen the latent nerve pain and to ensure I could get through a working day.
By May 2003, I am not using any painkillers, but see the 22 month report concerning continuing pain.
Rest and recuperation period
It felt good to be home, but this was just the start of a very long recuperation period. I felt overwhelmingly tired for about eight weeks. I spent a lot of the time lying flat on the floor, as this relaxed my back and took away the downward pressure on the injury. I must say that you get a different view of the world from the floor! I found that in the early days even the smallest task needed so much effort and my stamina and strength would suddenly desert me. I was also advised that walking should be limited, although not totally eliminated, and that I should not lift anything for at least six weeks. Both of these instructions I could easily obey without question! It has been shown (in spine-health.com) that gentle exercise may help prevent the build up of scar tissue. This also enables the tissues, muscles and limbs to remain in a good condition rather than weakening and atrophying with lack of use.
I went back to swimming after four weeks and I felt so weak! It is amazing how much fitness is lost in such a short time. However, I gradually built up my strength and stamina again but I cannot swim as far or as fast as I would like.
Work
As I have outlined adults may require detethering surgery after many years of conditional stability. This inevitably has implications for work commitments. I originally planned 6 weeks off, but due to various complications (including bladder infections, tissue inflammation and the disturbance of sacral nerves relating to the urinary tract), and a longer than expected recovery period, this was extended to 10 weeks in total. It is advisable that activities should initially be taken slowly and small adjustments should be made to ease yourself back into work. I made the mistake of thinking I was fit enough to work and therefore returned too early, expecting to be as active as pre-operatively. Big mistake! This exacerbated the latent back and leg nerve pain and so I was forced to take a 2nd period of sick leave. My working hours were part-time at first and I returned to full-time hours 14 weeks post-op. The tiredness and varying degrees of pain still return (2003) but I make the most of weekends to rest.
Since February 2002 my progress has slowed, with many episodes of the recurrence of nerve pain, UT problems and other issues.
Nerve Pain
This remains in the form of sciatica and hip, back, leg, and groin pain. I feel sensations that are very familiar - tightness along the sinus tract and at the point of detethering, nerve pain down the backs of both legs now, a stiff and painful right hip. New problems include numbness of my right foot and leg in patches, and continued feet and ankle swelling. All in all, a worrying catalogue of symptoms that I had hoped would not reappear.
As I am relatively uneducated in this field, except through personal experience, I always fear the worst - retethering. The likelihood of a filum retethering is low but it can happen. Two cases are reported by M. Souweidane and J.M. Drake in the literature (Souweidane, M.M. and J.M. Drake, "Retethering of Sectioned Fibrolipomatous Filum Terminales: Report of Two Cases", Neurosurgery, 42(6) (June 1998): 1390[-]93). There is also the possibility of scar tissue wrapping around the nerves and causing some form of compression and tightness. However, I have been told that
UT problems
I have experienced months of still ongoing bladder problems and UTIs, but they are slowly settling down. As this is an issue that stresses me immensely, I do not feel able to write very much on this subject.
Feet issues
My feet have experienced both positive and negative results from the detethering:
Positive results: the tension on the tendons, bones and muscles has been released. My left foot has visibly untightened, the pes cavus arch has dropped slightly and the toes have become unclawed to a degree. The forefoot flexibility has improved enormously. The right foot, which was always the better foot in terms of shape and function, remains tight with little sign of improvement.
However, I am hoping that after another year or so of neurological relaxation I can undergo an orthopaedic assessment. I may possibly have surgery to improve their distorted shape and change my footprints!
Negative results: my feet and ankles continue to swell, and feel hot and burning particularly when exposed to heated environments (hot summer days or indeed winter central heating) and physical over-activity. If I walk too much I still lose the feeling in the forefeet and the hot tingling and burning comes on. Paradoxically, as I am not so active now, this situation is relatively rare. On other occasions as mentioned above, my feet go numb in patches, a situation which still has to be investigated.
However my neurosurgical consultant hopes that as the nerves continue to settle they will desist from sending messages wildly, will reduce the amount of blood being sent to the skin and the vascular system may also settle. Again it is a matter of time and patience waiting for settlement.
Emotions
In the middle of July 2002, I am feeling very under the weather, exhausted and weepy. The pain in the hips and legs, and the constant deep aching all over is all so familiar and I am awaiting a clinical assessment after an MRI scan. I am angry that this situation is continuing so long and just wish I could recover some energy and physical strength.
At the end of August 2002, I have had a few consecutive days of no pain and without the use of painkillers! I hope that this is the start of a new pain-free life with occasional bad days. After careful perusal of the MRI scans my neurosurgeon has informed me that I am not retethered, about which I am very relieved. The feet problems, pain and feet numbness still remain in varying degrees and occurrence, but a potential source of pain and deterioration has been eliminated.
Further updates will be done in approximately 12 months time.
Time for another update on my position. It has been 10 months since my last posting and I have experienced some progress and now intermittent decline in my condition. I have been through a really bad 3-month period of pain, starting with a flare-up + cellulitis in February.
Nerve pain
This remains and is intermittent in its occurrence and intensity. Some weeks
have gone by when I have very little pain, and suddenly something will cause
a flare-up and it takes ages for the pain to die down. Unfortunately, since
the end of January 2003 the pain has spread into my left leg and both legs are
starting to feel numb in large patches just below knee level, which is a totally
new development. I experience nerve cramps in both calves, and a tightness down
the thigh nerves.
I cannot exercise to the intensity that I am used to and I am getting tired
more quickly. I tried to use the University gym in January 2003, but due to
the extended flare-up that has not been continued.
Overall I am finding it difficult to sit for any length of time as my hips and legs stiffen up quickly and I get a deep aching in the buttocks and upper thighs. I cannot do as much as I would like to independently (e.g. gardening), and if I do too much I am slayed for about a week after.
This scenario is very reminiscent of the period 3 years ago when I first became aware of the cord tethering, so it is a worrying situation.
Feet
This is not good reading. The right forefoot goes totally numb overnight and takes a half-hour or so to feel "normal" again. It is also the worst affected by the TCS neuropathy and is now constantly fizzing and pricking all over or, alternatively, numb in patches. In order to try to avoid this very painful neuropathy I don't walk as far as I would like. I also have to undo my sandals so that they turn into flip-flops: this helps to avoid compression on the nerves and the right forefoot (hopefully) doesn't go numb if I walk for longer. It is ironic that this neuropathy has worsened since my surgery and it is an entirely unwelcome consequence of the cord detethering. The right foot tendons are seemingly tighter than previously.
The intermittent bilateral swelling of my ankles continues with no sign of disappearing. However I have found that the major trigger factors are heat and immobility. Heated environments, such as hot offices, the cinema or theatre, shops with central heating, or even sitting in the sun on a hot day, will always have the same outcome - swollen ankles, deep aching of the bones in the feet, and overall body tiredness. However if I know I will be in such situations, I go prepared. I try to keep my own office as cool as possible, with a fan constantly on my legs and the windows open (even in winter!). As soon as I am out of the hot place I put on my Aircast braces for at least an hour, and the compression acts like support stockings, helping to massage the legs and ankles, thereby dispersing the oedema. I am not able to wear support stockings such as provided by MediUK due to the heat intolerance in my feet and legs. In the evenings at home, I try to lie on the floor in the cool house, and if necessary use the braces to prepare my feet/ankles for the next day. It is indeed a complicated situation!
Emotions over the period from
July 2002 to May 2003
Pain Management Programmes
Around August 2002, I began to experience some of my blackest days in dealing with the whole tethered cord scenario. The constant pain, abnormal nerve sensations, detestation of the appearance of my feet and legs, and the fact that there seemed to be no "end date" to the tethered cord situation, plunged me into a depressed mood. My diary is full of notes like "fear of something gone wrong", "mood heading south again", "really in the dumps", "feel so alone, lonely, isolated, abandoned". I found I was all brusque and bristly at work, and my personality was snappy and distressed.
I still feel traumatised by it all, from December 1999 when the pain began, through the day of diagnosis in October 2000, through the surgery in July 2001 which produced the worst pain I have ever experienced, to the overall weariness of constantly having to appear normal on the outside, apparently fully recovered from the surgery, but having to deal with the physical and emotional pain on the inside. There is still, even now, the underlying impression, very real to me, that my physical condition is declining.
I have spent many an hour in tears. I find that people assume "oh, she has had the surgery done, she is all fixed now", but as in most cases of a tethered cord release, this is most certainly not true. The operation was just the start of a new episode with tethered cord syndrome. I find it particularly frustrating and frightening that the pain still returns so unexpectedly and stops me doing things. I was always so active prior to the cord deteriorating (considering how tight it was tethered without me knowing!) and to have to slow down and/or stop is very annoying and indeed distressing. As a fellow TCS'er has said to me, it is hard to deal with the feeling that I am sometimes in the body of a 90-year old.
Around July 2002, my neurosurgeon was concerned that my ability to cope with the emotions around my tethered cord syndrome and the ongoing pain and sensations that go with it was relatively poor. He referred me to the Pain Clinic at Frenchay Hospital. Although they undertook a thorough assessment of my condition at that time, to my dissatisfaction and distress they only prescribed strong painkillers (Neurontin, aka gabapentin) and I was too wary of the potential side effects to take them. I also don't want to become dependent on painkillers, especially if they mask future pain and/or deterioration. However after that initial meeting I was referred to the Pain Management Centre, also at Frenchay, which runs a Pain Management Programme. Please click here to read about Pain Management Programmes and my notes on the one I attended.
Of course, life two years post-op does have its good points. i.e. the surgery ensured I am still walking, driving, swimming, etc., and fully continent. When the flare-ups come along I do try to keep these positive outcomes in mind, and the Pain Management Programme helped me identify further positive ideas and coping strategies.
May 2003
With the unpredictability of the situation at present I veer between happiness that the pain is at a relatively low level, with a few twinges, to a full-on panic that the flare-up pain (excruciating back and leg pain), and reduced mobility is indicative of my biggest fear - retethering. I have been advised that nerve root irritation and the leg cramps can mimic the symptoms of a (re)tethered cord, but are not actually a tethered cord. In the bad periods, I am yet to be convinced.
The questions that I try to answer are:
In the year post-op I could always explain away the flare-ups as "nerves settling down after the surgery" or "re-enervation of nerve circuits after years of being suppressed by the tethering" but now I am not so sure.
Further updates in ~12 months' time
Well, we are back in the summer again, just after my 3rd anniversary of the detethering and a short report is required.
Tethered Cord Syndrome:
The good news is that a new MRI scan shows there has been no change or deterioration in the condition of my spinal cord, with no syrinxes or any evidence of retethering. To get this news was a great relief and I can now approach the "coping with the painful days" on a more informed, and yet relaxed basis.
However, the TCS pain is very variable, with long episodes of flare-up pain, stiffness of feet and ankles, right leg nerve pain to the point of incapacitation. These flare-ups usually come on if I have not paced myself and are interspersed with short periods of relatively low level pain. However I have constant chronic pain on walking and in the lower half of my body. Two examples of pain episdodes are below.
Flare-ups: For various reasons I felt under the weather and a massive flare-up appeared at the end of September. The leg nerves, especially the long sciatic nerves felt very irritated and compressed on both sides, the left ankle felt continually strained, and my whole lower body felt drained, heavy, lethargic and exhausted. The usual feelings of nerve pain appeared in the back of both legs and the neuropathy in both feet increased. This flare-up lasted about 1 month, and only gradually died away with rest and a reduced exercise programme. I really had to take things slowly overall.
In the 2nd week in November, an even worse pain flare-up appeared with both excruciating, lancinating nerve pain, and subsequent muscle spasms, all across the sacrum, so much so that I was confined to bed for 2 days, and had to take a week off work. This pain was comparable to that I felt on waking up from the detethering surgery, so I was literally knocked flat by it. Thankfully, with a lot of rest and gentle swimming I got back on my feet, but the recovery was the slowest to date (see section on 5-year update for a possible explanation for this pain).
One can see that this condition is extremely variable, with very good and very bad days, with absolutely no warning that deterioration will occur, or that flare-ups will appear.
Erythromelalgia (EM): another positive from the summer 2003 is that I now have an official diagnosis from my new rheumatologist of bilateral Erythromelalgia (EM) after 13 years of trying to fathom out and cope with the symptoms of swelling feet and ankles, burning pain, erythema, and pins and needles. Over the summer months I can control the symptoms quite well by keeping my legs and feet cool, wearing sandals and light clothes. However when the heating comes on at work, over which I have no control, my EM symptoms increase, and every night I have to lie on the floor in my cool house, with my Aircast braces on to try to keep my feet cool and reduce the swelling for the next day.
However, since October 2003 my EM has become unbearable - the swelling, burning pain, red to black discoloration of the skin on my legs and feet, and consequent pressure on the nerves in my feet and ankles has been overwhelming, especially over the winter months. It has literally driven me to distraction and lots of tears. In April 2004 I tried a vasoconstrictor medication, but this made me feel exceedingly weak and exhausted, like a wrung-out dishrag, and it exacerbated the Raynaud's phenomenon in my hands. I stopped taking this after a month and slowly regained my strength. In a few weeks time (October/November 2004), I am due to be admitted to hospital to try stronger supervised medication and will report back on the results when I can. I am also going to be assessed for orthotics to support my feet and to try to stop them cracking and splitting due to the SBO/TCS deformities.
The EM has caused me many emotional and psychological traumas. I have been trying to get my head around yet another chronic, lifelong condition, on top of the spina bifida occulta/tethered cord syndrome. I have just finished 7 months of counselling to help me cope and come to terms with the emotional and mental wear and tear of having chronic conditions. I am eternally grateful to Brian Jenkins, who got me through some of my worst ever days. He also helped me discover tools, knowledge, and more inner strength and maturity to try to cope with the conditions in the years to come.
I would be interested in hearing from anyone who suffers from EM due to neurological damage and how they manage it.
Website: this website has had a lot of traffic on it (see Feedback page) and I have made new contacts with people with varying types and symptoms of tethered cord syndrome. This is what this website is for - to disseminate information and to give help, even a little, to anyone who needs support at such an uncertain and frightening time. Updates have been made to the pages on Tethered Cords, Spina Bifida Occulta, Information and Resources, Pain Management and Articles, and a new page on Erythromelalgia has been added (July 2004)
It has been nearly 18 months since my last update and it has been a varied time. I will split this update into three sections
August-Winter 2004
After I had had a bad time with the erythromelalgia detailed above (although I didn't get admitted to hospital but coped with it myself) I decided to try acupuncture to see if it would alleviate the painful symptoms and constant flare-ups. Please go to the page on acupuncture to read of this experience.
Winter 2004/05
As usual the EM/CRPS emerged with a lion's roar in October 2004 and overall my "winter" was full of EM flare-ups, pain, feet swelling and all the concomitant problems of EM (see below). The skin on my feet constantly split, I got recurrent skin and soft tissue infections and the problem seemed unending. I went through periods of depression and despair, as nothing seemed possible in terms of a cure, although the acupuncture helped to alleviate some of the worst symptoms.
The TCS pain seemed to go in cycles as usual. Flare-ups appeared with no warning and no precipitating factors, but I also had a few weeks of low pain levels and I took advantage of these weeks to do as much as I could within my own limits.
During the winter I was referred to Dr Kennedy, a dermatologist at the Bristol Royal Infirmary, and after extensive tests we got two results: the erythromelalgia was diagnosed more correctly as Complex Regional Pain Syndrome and I got a referral to the Bath Royal National Hospital for Rheumatic Diseases. Professor Blake is a world authority on CRPS and therefore I am now with a dedicated team in this regard. The 2nd result was that it was discovered I had a chronic fungal foot infection which had probably been in my feet for about 10 years (all the time I have had split and unhealing skin on the undersides of my feet) and which had not allowed my feet to heal. I was put on strong antibiotics and with intensive personal care of my feet, they are now all healed up, with no cracks or splits and therefore the risk of further fungal (or other) infections is minimal. I also know the warning signs of further infection - a heavy, pressurised feeling in my feet, intense soreness of the skin, and intense pain on walking. I take prophylactic antibiotics at the first sign of infection and for 9 months now (April 2005 - February 2006) they have been clear!
Diary extract of EM/CRPS symptoms
"I usually try to start a diary on an upbeat note, but I regret this time I
can't. Of course it is all to do with the EM/CRPS - so painful, feet very swollen
and discoloured. Let me give you some idea of what it is like:
I go to bed each night, with relatively good feet, usually freezing cold due
to lying on the floor with no heating. This suits me but other people would
feel like ice blocks. I have to have a hot water bottle as the top half of me
is cold and I lie there desperately trying to either warm up or keep my feet
cool on the Chillow
- what I did without that I don't know. Eventually I sleep but wake up several
times in the night with either mid-backache, back pain, hip pain or burning
feet.
Eventually the alarm goes off - ohmigod, time to face the pain. I leave it as long as possible until the call of work, travel and the sun gets me up. I swing my legs around and put them vertical … and the pain begins. It is not just pain - it is shafting, daggers, blood pressurised into vessels damaged by all the swelling etc, and the skin is stretched, split and damaged. The thudding pain pours into my feet like nothing you can imagine - it is like having a ten ton weight on my feet, yet from the inside. The Achilles Tendons throb, the splits in the skin threaten to deepen into chasms - I fall onto my knees to take the weight off them, kneel on a stool to reach up and shut the window. I try to put my feet flat on the floor, but cannot bear the weight on them - again I crawl to the bathroom, trying to keep the insteps of my feet from shearing against the carpet. My right foot is currently the worse, but of course this is my strongest side, and so all my weight goes onto my weaker left foot and leg. This throws the TCS pain up my back…
Trying to get dressed, I have to sit on the edge of the bath, take all the weight on the left foot, and brace against the right. One false move and the pain strikes lightning and the tears come. Going downstairs is either on my backside or one hand gripping the left bannister and the other holding onto the wall. My lounge is a large space, so I have to crawl across that too. The coolness of the hall laminate floor is welcome, momentarily, but then I have to face putting socks on against the subzero temperature outside. As the fabric touches my toes, my feet, my ankles, all the burning pain strikes again, the toes so swollen they are unresponsive to bending. Up the Achilles Tendons, and the swelling and rasping on the skin is almost unbearable.
Then … I have to get out the front door with two bags. Oh, I forgot, my shoes, or sandals rather, to put on, the leather, misshapen in the deformed outline of my feet, touches my skin and the straps find their place, but again the pressure and the pain makes my face screw up and tears come to my eyes. At the door, gingerly stepping over the threshold, taking care not to move too fast or bend my feet too much as they would break even further. Holding onto any part of the car I can find, walking on my toes so as not to tension the Achilles Tendons, eventually I get round to the driver's side and can go.
Next day: even worse. With my Pain Management counsellor, I voiced doubt about my current coping, daily living with it, the future with it. The coping strategies are bearing very rapidly eroded and destroyed, and are wearing very thin at the butt end of winter. I feel like I am sinking into darkness again, tears readily come, and I feel like I am in a tunnel with no light at the end of it. There is no prospect of a cure or alleviation, I feel really disabled by this now, old before my time, facing limited job, leisure, day-to-day activities, and yes, I was in floods of tears.
All this is written for the record, for the CRPS consultant, for the DLA, for me, so I know I haven't dreamt all this. After 15 years at least of being dismissed, not believed, misdiagnosed, I want to be looked after and helped through this annual struggle of living with TCS, EM/CRPS and later in 2005, the diagnosis of spondylolisthesis.
March/April 2005
In March/April I went to the USA for a 3 week vacation. I had a great time with Trek America, camping under the stars, watching the sunset over the Grand Canyon, waking up under canvas to 3 inches of snow, walking in Yosemite National Park, despite the pain in my left foot (see February 2006 report) and visiting both San Francisco and Utah National Parks. Once I got home, I realised that the EM/CRPS swelling in my feet had reduced. It seemed that the chance to get away from the heating/cooling/stressful environment of work had enabled further recovery and this promised a good EM/CRPS summer.
April - October 2005
Of course with such a complicated mix of rare conditions as mine, nothing is ever simple. The EM did die down to a minimal amount during the summer, with a few flare-ups when the heat and humidity rose again. However the TCS pain seemed to compensate - the summer seemed to be a permanent flare-up, with a new type of pain across my sacrum and in both buttocks and thighs. It seemed a crushing pain, worse on lying down or swimming - the lack of rest added the issue of extreme tiredness to the mix. In the acupuncture clinics, Jason and I worked on desensitising the skin and nerves in my lower back and we got to the point where I could tolerate being touched - this facilitated needling of my back and we had success in pain reduction and relief of the EM flare-ups.
October - December 2005
With the onset of the winter heating again (which I was stressing about in August!), I was dreading the start of winter and the full-on return of the EM/CRPS. However Jason had developed an acupuncture protocol of needling my back and it seemed to be successful in deferring the onset of the EM for 7 weeks. My feet didn't swell so much, the pain level from the EM remained low and I was cautiously optimistic of a relatively good winter. However at the end of November, the EM emerged again, full-blown symptoms and intense pain, but this came after a particularly bad soft-tissue infection and tendonitis, which probably didn't help the tissues of my feet and legs withstand the nerve-related EM mechanisms. In December I got a referral to Mr Pople's colleague, Mr Mike Carter, Neurosurgeon, and he queried whether the new, more mechanical pain in my back and sacrum is spondylolisthesis, i.e. slippage of the vertebral column, due to the congenital spina bifida occulta, the detethering surgery and general wear and tear as I am getting older. I was put forward for CT scans and plain X-rays to see the dynamics of my back, 4.5 years post-detethering surgery.
January 2006
As I write now, I only have 8 weeks left of the winter and Jason's acupuncture protocol is alleviating the worst of the EM with brief flare-ups only. Let's hope when spring comes and the heating goes off I can look forward to a better summer.
Unfortunately the left foot structure and strength - bones, ligaments, muscles and joints - is deteriorating fast. I have constant pain either at rest or walking. I now wear a foot brace during the day which prevents the ankle straining or spraining, and supports the joint. However, despite this my mobility is significantly impaired, which is ironic as my legs feel stronger with swimming and I feel 99% recovered from the detethering surgery
February 2006
I have just had the CT scans and X-rays done to investigate the possible spondylolisthesis. I will update this as soon as I can, but for now that pain is lying low and even the short-flare-ups are relatively easy to cope with.
...and finally...
Many people have commented to me all along on how I have coped with all this. One person close to me wrote "I am full of admiration for the way in which you have coped with all the distress and pain"; another said "you do more than three average people, you are an inspiration to us all", and a colleague at work really made me feel emotional when he just threw into the conversation "you really have some pluck, I admire you".
I write this all here, not to blow my own trumpet and pat myself on the back for getting through all this relatively unscathed (at least on the outside! and despite all the observations I have outlined above), but
Further updates will be done in approximately 12 months' time.
Feedback
I do hope that these notes have helped you with your preparation for detethering surgery and given you an insight into a post-operative condition. Although I had learned a lot about the TCS condition, its aetiology, clinical and physical manifestations, and surgical requirements to prevent further neurological deterioration, no-one told me what to expect from all the after-effects of the surgery. I was therefore most unprepared for all the degrees of severe physical pain, the complications of L5-S2 nerve-related issues, and the very slow, lengthy, physical and emotional recovery period.
If you have comments and advice for other readers, email me on nina.bunton@bristol.ac.uk and I will post your input on the feedback page