It came on very slowly, first the stiffness, along with more spasticity, making different movements more difficult.
I’m a “fake quad,” an incomplete C5-6 with enough muscle sparing and leg strength to stand and do some crutch walking, but the spasticity made both much more challenging. While in my wheelchair, my legs would shoot straight out of the footrest straps and trigger spasms in my trunk strong enough to straighten my entire body, occasionally sliding me out of my chair. Transferring to my handcycle became all but impossible — I found myself on the floor or ground all too often. Getting up to sitting in bed was harder, and often the spasms were strong enough to lay me back down. Then my posture began to deteriorate and the hours of computer work grew more exhausting. Transfers, ramps and wheeling in general all got harder. Pain and fatigue came on more quickly, and I just couldn’t do all that I used to do.
Friends and professionals told me this was what 60 felt like. That stung, but didn’t sound unreasonable.
I saw my doc about the spasticity and tried baclofen, upping the dose until I needed naps, then tried dantrolene, then the two in combination, all to little avail. I took enough ibuprofen to give myself an ulcer, but not enough to kill the pain. MRI results failed to raise any red flags.
In January 2008, three years or so after this all began, I got a full re-evaluation (my first in a dozen years), muscle/sensory testing and another MRI. I was also referred to Scott Falci, a neurosurgeon at Denver’s Craig Hospital, who specializes in untethering surgeries of the spinal cord. I wasn’t sure why I’d been referred, but I followed orders, figuring what the hell?
He explained spinal cord tethering — formation of scar tissue at the injury site that limits cerebrospinal fluid flow and cord movement in the canal — and its symptoms. He added that virtually all cords injured by trauma are tethered to some degree, that some deteriorate to the point of developing cysts (cavities within the cord) and that surgery could stop the progression of losses.
All this was interesting, but no one ever mentioned or even suggested that perhaps a tethered cord might be my problem. No one — no doc, no OT, no PT — had suggested that my function had deteriorated so badly that I might need surgery. Always somewhat wary of self-diagnosis, I tend to put a lot of trust in medical professionals. I thought somewhere along the line someone would have said, “Dude, you’re a rolling description of a tethered cord, get your ass to a neurosurgeon.” That never happened.
It also never occurred to me to take some initiative, do some research and aggressively seek answers for all the changes and losses I was experiencing. Besides just being a little long in the tooth, I saw spasticity as my main problem, so I asked about a baclofen pump. Denial is a beautiful thing.
Denial Turns Ugly
That once-beautiful denial turned ugly over the next month as my balance and strength rapidly deteriorated. I finally came to recognize the previous three years as a slow, insidious, almost invisible loss of function. Much of the function I’d fought so hard to regain in rehab years and years ago was evaporating. A couple articles on cord tethering put it all together: my symptoms — loss of sensation and function; decreased strength; increased fatigue; a decrease in bladder and sexual function; increased pain and spasticity and more edema — strongly suggested a tethered spinal cord. Panicked and depressed, I was in the neurosurgeon’s office the next week trying to schedule an untethering procedure.
In the past year I’ve learned far more about spinal cords, tethering, progressive myelopathy, myelomalacic spinal cords and syringomyelia, along with the havoc they wreak, than I ever wished to.
Tethering, Falci explains, is usually the formation of fine, web-like bands of scar tissue around the site of injury, which anchors the cord to the dura — the cord’s protective covering within the canal — and limits spinal fluid flow. That anchoring stresses the cord, inhibiting it from floating and stretching freely within the canal. Some cords deteriorate to the point of developing cysts, or cavities within the cord, which then fill with spinal fluid, causing further stress.
With or without cysts, that stress and pressure exacts a toll and can cause bad stuff to happen, like all my symptoms and a few others as well — excessive sweating, hypothermia and more.
“In my experience,” Falci says, “all people sustaining traumatic spinal cord injury develop tethered spinal cords, though not all of them develop significant symptoms. In over 800 surgeries, I’ve always found cord tethering, even when the MRI doesn’t show it.”
Though tethering can be seen on an MRI, the imaging can’t diagnose damage or loss of function and makes diagnosis tricky, as most of these symptoms can also be tied to aging, overuse, wear and tear, or other things such as UTIs, bladder or kidney stones, decreased activity or muscle use, skin sores, prolonged bed rest or neurodegenerative problems such as MS. The most telling symptom, Falci says, is progressive sensory loss, which can’t be masked by any other problems.
Once all the above-mentioned problems are ruled out and there’s no evidence of structural problems in the spine such as instability, cord compression or stenosis, an individual’s personal assessment of significant motor and sensory loss or other symptoms, coupled with professionally-administered motor/sensory testing, best determine the need for surgery. The presence of a cyst makes it an easier call.
And how often does this happen? Falci suspects less than 10 percent of all spinal cord injuries develop tethering to a degree requiring surgery, but UCLA’s Ulrich Batzdorf thinks it may be closer to 20 percent. So much for spinal cord injury being a stable condition. Lucky me.
Ken Riesse, Brian Johnston, Al DeGraff, Dave Denniston, Kris Gulden and even nondisabled Catherine Deist have all had the same kind of luck.
Neurosurgeon James Guest of the University of Miami Miller School of Medicine and The Miami Project, concurs with most of Falci’s description of tethering and cysts. He emphasizes the need for professional assessment of both motor/sensory loss and levels of spasticity, coupled with progressive changes. Guest, a colleague of Barth Green, the other recognized go-to guy in this area, also stresses that the primary rationale for surgery should be the prevention of further loss of function or sensation.
I’m not looking to encourage paranoia and a ‘sky-is-falling’ attitude whenever any of these symptoms show up. I’m certainly not looking to flood rehab centers and neurologists’ offices with panicked wheelers. I spent 20 years assuming SCI was a pretty stable condition and figuring that as long as I was healthy, things were probably okay, and for the most part that assumption still holds. But still, even a 10 percent risk is significant, which makes self-monitoring important, asking questions essential and constant vigilance critical.
And I’m Not the Only One
Ken Riesse, a North Dakota C5-6 incomplete quad, was stable and problem-free for over 20 years when, at about 40, he began noticing some changes.
“I began losing some strength and having some trouble getting my chair in the car in the mid-’90s, but figured I just had to get used to it, so I didn’t pay it any more mind. When I saw some sensation changes and more weakness in ’02, I started aggressively looking for answers. I went to the rehab in Grand Forks for an MRI, then for another one in ’05, but neither one showed anything. When the problems persisted, I went to Minneapolis where they looked at them, described tethering and referred me to Falci in Denver.
“He explained tethering, the surgery and all its dangers to me,” Riesse recalls. “He said he couldn’t guarantee I wouldn’t get any worse without surgery. I understood him to say that if I did the surgery I wouldn’t get any worse. I felt myself going from a C6 to a C5 and decided to do the surgery because I saw it as a way to stop the losses, to not get any weaker. You know, for me the fear of losing my independence is simply horrific.”
Not long after Riesse’s adventure, Brian Johnston, a C5 quad from Pewaukee, Wisc., noticed some loss of sensation in a couple of fingers, then numbness in his elbow. He’d had sensation throughout his body since injury, but in January 2006 those losses suddenly spread. Within a week he’d lost all light touch sensation from his toes almost all the way to the point of injury.
“My doctor checked me out for a stroke, did an MRI and CT scan. He told me I had stenosis and needed a laminectomy, rods and screws. I’d always gone back to Craig since rehab in ’73 so I called the medical director, who suggested sending the MRI to Falci. His office called, told me I had a tethered cord and needed surgery. I had them send me some information, did some calling around and went out to see them in March.
“When I saw him, he showed me the MRI and described what he would do – a discectomy to stabilize my neck and another to untether the cord. He explained the dangers, that if I didn’t do surgery I could lose more, and that I might regain some of what I’d lost. I thought that maybe I could get some sensation back. I was worried about losing more, thinking, ‘Is motor next?’
“Look, when you’re a quad and have light touch throughout your body and can feel your feet, losing sensation is a big deal. I thought the longer I waited, the less chance I had of getting anything back. My motivation for going forward was wanting to get the light touch back; not losing motor was secondary. Maybe my intentions were backwards.”
Losing function or sensation slowly can be easy to ignore, not recognize, or attribute to aging or sedentary lifestyle. That describes Al DeGraff’s reaction to losing strength and range of motion over a few years — he thought he needed more exercise. But when Degraff, a Fort Collins, Colo., C5-6 quad in his 50s, lost half his lung capacity overnight, he knew something was very wrong. He sent his MRIs to nearby Craig Hospital and quickly set up an appointment with Scott Falci, who confirmed tethering and two significant cysts, and promptly scheduled surgery.
“I was very frightened and thought I might lose more,” says DeGraff. “I kept wondering, ‘Would I be able to breathe?’ In our conference, Falci prefaced everything by saying there were no guarantees and that the best possibility of regaining function was what was lost most recently. I went into surgery hoping for some return, especially lung capacity.”
DeGraff was scheduled for a 13-hour surgery due to the presence of a cyst that required a shunt and the extensive untethering. He spent 15 hours on the table. Riesse was out of surgery within six hours. Johnston, on the other hand, spent closer to 10-11 hours on the table. Mine lasted about seven-and-a-half hours.
Degraff developed pneumonia post-surgery, and Johnston had to deal with both a clot and a severe urinary tract infection. As a result, both were hospitalized for six weeks. Riesse and I were problem-free and out the door in about two weeks.
And the pain?
“Words can’t describe the pain the first three or four days after surgery,” Johnston told me. “Even opening my eyes hurt.”
DeGraff’s experience was similar to Johnston’s: “The pain was absolutely excruciating when people would turn me. Dressing changes were just as bad. I didn’t get much pain relief for the first week or so and the drugs gave me horrific hallucinations.”
“To be honest, it wasn’t that bad,” Riesse says. “When my doctor saw that I wasn’t taking many pain meds, he kept telling me I didn’t need to be a hero. I just didn’t have much pain.”
My experience was closer to Riesse’s, perhaps because both of us had shorter surgeries and incomplete injuries.
And getting back to normal or regaining function?
Riesse and I used a slide board for transfers for a week or so and had help for about a month. Johnston wasn’t totally comfortable with solo transfers for about three months. Driving his pickup took even longer. DeGraff, who uses personal assistants, was in fairly good shape upon discharge, six weeks post-surgery.
“It’s kind of hard to judge just what I had before surgery,” Johnston says, “but since then I take inventory daily. I think I got some sensation back in my right leg but not much else. My left side remained pretty dead except for a little bit of sensation in my foot.”
“Things never got better,” Riesse told me. “Return never happened. Actually, I’m probably somewhat weaker now.” And a bit older.
DeGraff regained his lung capacity within a week, as well as some range of motion and strength. He began to lose strength again in 2006 and quickly scheduled another surgery, but as the date approached, he began having horrible nightmares and flashbacks of the previous surgery. Despite progressive losses, which have recently cost him the use of his wrists and the ability to write and drive, he ultimately cancelled.
DeGraff’s initial experience seems closest to Falci’s surgical outcome statistics, which claim 90 percent success in arresting the progression of symptoms, and over 50 percent reporting at least one lost activity regained. About the same number say their spasticity and/or pain levels decreased. Over 80 percent felt the surgery was worth the pain, time and effort involved. About 11 percent said they were not as functional after surgery as they were prior.
The need for untethering surgery does not follow predictable SCI demographics.
Dave Denniston, a T10 para and swimmer, was only two years post-injury in 2006 when he developed a persistent pain in his ribcage. When it spread and turned to numbness, he sought medical help. Unable to get any answers in San Diego, he went to UCLA, where Ulrich Batzdorf, a nationally recognized expert, diagnosed him with syringomyelia — a cyst from T4 to C4, eight vertebral levels.
“He explained the surgery to drain the cyst and restore the normal flow of cerebrospinal fluid before the cyst got worse and went to my brain. I wanted to get rid of it.”
Batzdorf drained the cyst, untethered the cord and reinforced it with Gore-Tex. But within months Denniston developed weakness and pain in his left arm that even neurontin wouldn’t touch. Batzdorf referred him to Scott Falci in December 2007, who told him the next month that the cyst was even larger and recommended a shunt.
“The pain was pretty bad and losing function was very frightening. That was my motivation for surgery,” Denniston recalls.
“I was in the middle of training for the Beijing Olympics and didn’t want to miss my chance, so I scheduled surgery for after the qualifying meets.”
He had surgery in early April and spent two weeks in Craig.
“I had quite a bit of pain, so I pushed that pain button like a champ,” he told me with a laugh. “Taking a week to get back in my chair was really frustrating. The hardest part was that three days in a flat bed.”
Denniston saw quick improvement in both strength and pain. He was back in the pool in June and pretty much back to normal by July.
“I wasn’t counting on getting anything back. I haven’t regained any sensation and still have a little pain, but the strength is back to normal. It was frustrating trying to find someone who could diagnose what was happening to me, but I don’t have any misgivings about the surgeries.”
Kris Gulden has lots of misgivings. After Gulden, a former Alexandria, Va., police officer, was hit by a car in May 1998, she was a T4 walking para with bowel and bladder function. By September 1999 she was losing strength in her left hand.
“I went for a myelogram that November. As soon as they injected the dye, I felt severe burning and lost all function in one hand. The doctors speculated that the dye caused inflammation of the dura and damage to nerve roots, a rare side-effect of the procedure. Eventually they diagnosed me with a C4 syrinx”
The following month Gulden traveled to the University of Virginia for a second opinion and surgery to remove rods and drain a cyst which ran from C3 down to T4. Days later she lost all leg function and bladder control. In little more than a year -and-a-half, she had gone from a walking para with full bowel and bladder control to a full-time chair user, incontinent and with one good hand.
The cyst kept returning and she underwent repeated surgeries to have it shunted. Each time she regained some function, only to quickly lose it again. After endless Web searches and facing yet another shunt surgery, Gulden went first to nearby National Institutes of Health, then to Barth Green in Miami in early 2001.
“I just wanted a consult, but within 10 minutes I knew this guy understood the problem and could probably fix it. My mom and I stayed and went ahead with the surgery.
“It was an 11-and-a-half hour surgery, followed by a lot of pain,” Gulden says. “They kept me off my back following surgery. I could be on my side or stomach, but not on my back. I don’t remember much pain after the first few days.
“I was in the hospital for about five days and then stayed in a Miami hotel for three or four weeks before going home. I was very careful for a long time, afraid of doing anything. It was a couple months before I was driving.”
Spinal cysts can even show up in nondisabled individuals.
In 2003 Catherine Deist, a home health hospice nurse from Illinois, was diagnosed with meningioma, a benign brain tumor. She had the tumor removed but within months developed a very unsteady gait.
“I was nondisabled before the tumor. After surgery I began having balance problems and even fell a few times,” Deist remembers. “I was diagnosed with a thoracic cyst and the surgeon recommended going in and trying to drain it. I didn’t feel I had a choice, so I had surgery that October.
“But by Christmas I was using a wheelchair, experiencing pain and numbness, and losing bowel and bladder function. When my St. Louis surgeon told me this was out of his league, we sent 25 pounds of films to the Mayo Clinic. They told us nothing could be done, that there was no help, no hope.”
By this time the paralysis had progressed up to her chest. Deist frantically searched the Web for answers, eventually finding Craig Hospital and Falci. She sent her films in July 2004, heard back quickly and went to see him.
“The adhesive arachnoiditis [tethered cord] had descended to the cuada equina [nerve roots descending from the base of the spine]. He diagrammed the cord, explained how cysts formed and what tethering was, and gave us lots of ‘whys.’ It was the first time we really understood things. He recommended surgery to try to stop the losses but made no promises.”
Deist had surgery in November 2004 again, twice in three weeks the following March, and another in October of that year, to untether the cord and rebuild the dura.
“Each time I’d get better, then quickly regress. He put a shunt in during the last surgery in January 2007, and now I have a complete flow of spinal fluid. I was in the hospital for a couple weeks each time and the pain was pretty severe.”
Deist says she could barely move her legs prior to that final surgery but was able to bend both of them immediately afterwards.
“I was so excited that when I saw Dr. Falci right after surgery, I was able to take a kick at him.”
She’s still out of the chair and kicking, but the constant pressure from the cyst left Deist with permanent damage. Still, she’s discarded the braces and is much stronger. She treadmill-walks about two miles a week and gets around using a couple of canes.
“My sensation remains impaired from breast level on down, along with some chronic burning pain. But I’m off some meds and the spasticity isn’t nearly as bad. Last year I climbed to the top of Diamond Head in Hawaii with my husband. We were both in tears. This has all been pretty unbelievable. We feel very blessed.”
What You Get is What You Get
Understandably, people’s feelings about surgery are often linked to its outcome. If the result was good, the feelings are positive. If the outcome is less than expected, there is some disappointment. DeGraff says he still carries great respect for his surgeon, but that he’d never go through another surgery. Riesse and Johnston hold that respect as well, though both express a good deal of ambivalence regarding their outcomes.
“I’ve said I’d never go through that again,” Johnston reflected, “but when push comes to shove, if you’re losing function, it’s hard to just watch it go without doing something about it.”
Deist and Denniston have no such qualms, suggesting they’d do it again in a heartbeat. Gulden says most of the damage to her cord came well after that car hit her, but steers clear of ‘what ifs.’
“I still have a good hand. I can push my chair. I can be independent,” she says philosophically.
As for me, I never got back to where I was the day before surgery, and sometimes feel considerably weaker than I was four months post-surgery. Like Riesse and Johnston, I’m ambivalent.
But like Gulden, I try to stay away from the ‘what ifs.’ Water under the bridge over the dam and out into the ocean. I’m with Johnston about feeling a need to do something and always saw the surgery as an intervention to prevent further losses rather than about getting something back.
I don’t know if I would have continued to lose function, and I think the only way to know if the surgery was a success is if loss never reoccurs. But if it does and I feel myself rolling backward again, I suspect I’ll give it all another whirl.
Cord Tethering and Cysts: Causes and Treatment
“Cyst formation is simply the endpoint of cord deterioration following injury and tethering” [formation of scar tissue], Dr. Scott Falci, of Denver’s Craig Hospital, says. “It’s related to impaired cerebrospinal fluid flow, the pressure difference and/or loss of cord elasticity tethering causes, and leakage of spinal fluid into the cord itself — thus [causing] a cyst cavity.”
The overwhelming majority of people seek intervention because of motor and/or sensory loss. Less than 20 percent cite autonomic dysreflexia alone; an even smaller percentage are motivated solely by increased spasticity or pain.
The microsurgery, whether for untethering alone or in combination with cysts, is a major one, definitely not to be taken lightly. It’s complicated, extensive, delicate and potentially dangerous.
“This isn’t something people should rush into,” The Miami Project’s Dr. James Guest counsels. “They need a sound rationale, and if possible, they should definitely get a second opinion. This should be performed by someone experienced, preferably at an SCI center. Scar dissection is very delicate surgery.”
First, all the muscles are retracted to gain access to the spine. Then a laminectomy gives the surgeon access to the dura (outside membrane of the spinal cord], and in turn, the spinal cord itself. He then painstakingly dissects the scar tissue from the cord, releasing the tension and allowing the cord to float freely and the spinal fluid to flow uninhibited within the canal. If cysts are present, they often collapse during surgery; if not, they are drained and, if necessary, shunted. The entire procedure is monitored for electrical activity up and down the cord to ensure no further damage, and for possible increase in flow of signals below the surgical site and to the cord. Once the tethering of the cord has been eliminated to the greatest extent possible, the dura is repaired and the surgical site closed.
This can be an unusually painful surgery, because of both its length (typically eight to 10 hours) and extensiveness. People are placed face down on the table for the surgery, and the positioning alone can take as long as 90 minutes. Much of the pain comes from having major muscle groups retracted for that long. Quads, who theoretically are at greater risk of needing surgery, have an especially hard time, as the majority of the shoulder girdle muscles are attached to the neck, and most movements they make will stress the surgical site.
Dr. Falci requires people to stay flat in bed those first three to four days to insure proper healing of the dura and prevent a spinal fluid leak, followed by an additional week or more of hospitalization prior to release. Barring complications, most people are on the way home within two weeks and back to a somewhat normal level of activity within three months, though regaining lost conditioning may take considerably longer.