By Angela Mack
Being disabled is the hardest job I’ve ever had. It’s 24 hours a day, every day of the week and every week of the year. There are no vacations, and the pay is lousy. Sure, I have a $20,000 wheelchair but it didn’t even come with air conditioning or a spare tire. Thanks to a disorder known as Syringomyelia (SM), I’ve been on this job for 20 years specializing in quadriplegia. Today I can discuss SM with the best of them because of the American Syringomyelia Alliance Project and the National Organization for Rare Disorders, two groups dedicated to educating patients–and doctors–on what disabled me. The more I learn about this disorder the more I realize how destructive it can be, not just to people’s bodies but to their minds, finances, relationships and the very essence of their beings.
Back in 1960, I was nine years old and looking at inkblots in a psychiatrist’s office at Boston Children’s Hospital. Neurologists and neurosurgeons had failed to find the cause of my off-balanced gait, weak legs, stiff neck and severe shooting pains down the left arm. So many tests had been tried. Everything, including polio had been ruled out. The final conclusion? It must be psychosomatic.
Medical research of that time consisted of very few articles on Syringomyelia, and those available discussed mainly males in their 30s and 40s. Who would think to look for it in a nine-year-old female? There was one final test–a panopaque myelogram–to be tried. It found a syrinx in the C-4, 5 level of my spinal cord. Orthopedic doctors performed a laminectomy so neurosurgeons could reach the syrinx, and they simply drained it that time.
With two weeks of physical therapy, I was on my way back to normal. Only a long scar and a neck brace, which I wore four months to help while my neck regained bone/muscle strength, were left from that first surgery. Doctors had explained very little to me about the operation; it was up to my parents to make the decisions, sign the consent forms and prepare for the unknown. All I did was deal with kids making fun of my neck brace. Except for the outstanding medical bills (I had no insurance), that summer’s surgery was all but forgotten. We thought I’d beaten Syringomyelia, but that nasty existent lay quietly in wait.
No one had prepared me for what happened seven years later. Slowly my legs weakened, walking became difficult and my balance was tipsy. Except for no arm pain, all symptoms were the same as before. There was no Internet to help inform us, no ASAP to help guide us. We were a lower middle class family with no medical savvy. Even though we now lived in California, the doctor I saw knew nothing about SM. His only suggestion was that I return to BCH, where I’d been successfully treated before.
Seeing that doctor’s inability to help me, his labeling it a “rare” disease, my return to Boston for another operation–all these things filled me with a kind of fear I’d never known. More lumbar punctures, another myelogram and four weeks with meningitis gave me a deadly respect for SM. But six weeks after a similar surgery as before, I was back on my way to wholeness. I’d beaten it again–I thought–but this second miracle was to be short-lived.
As I was finishing my senior year of high school in 1969, the same symptoms began to appear. My first reaction was fear. I tried to ignore the problem and continue through ’til graduation, but the symptoms became noticeable and I missed the last two weeks of school. Doctors at BCH recommended my parents take me to UCLA Hospital, and an entire regimen of tests began.
Neurosurgeons at UCLA knew to look for a Chiari malformation at the base of my brain. They found one and did a suboccipital craniotomy (more commonly known today as decompression). They collapsed my syrinx (still at the same cervical level) and placed a tiny shunt in that space. Within two weeks, I was skipping in the hospital hall to everyone’s delight (especially mine). After regaining all my strength at home, I was off to my freshman year at college having overcome SM for the third time. It never became an issue until I was planning to get married and wondered about the possibility of passing this disorder to any children I might have. A UCLA doctor, who had researched that subject in 1972, found it in only one case being passed from father-to-daughter.
Everything went well for six years. In 1975, the same symptoms came back. It didn’t happen in a day, but I felt them coming on and that same fear filled every part of me. I was married and a working journalist. Why did this insidious disorder have to come back in my life AGAIN? Hadn’t three surgeries been enough? The neurosurgeon who had done my operation at UCLA was now in private practice, so I went back to him to share the bad news.
For my fourth surgery, the surgeon opted to do a Gardner procedure. It consisted of plugging the obex with a small piece of my own muscle tissue. While strength in my legs and balance returned, I lost the feeling and use of the last two fingers of my left hand. The left side of my tongue constantly quivered, I was plagued by hiccups and occasional vomiting right after meals (this last symptom lasted about six months).
I returned to work but the strains of my illness caused a rift in my marriage, leading to divorce. It finally became apparent that SM wasn’t going to be so easily cured, that it had invaded my body and attacked at will. In less than two years, my same symptoms returned. The neurosurgeon was reluctant to try anything more in my spine, so he installed a VP (ventricular peritoneal) shunt to help drain fluid from my brain. Mild improvements followed; about 50 percent of my strength returned.
In 1979, all symptoms were back. I went to a new neurosurgeon at University Hospital in San Diego. He tried two syrinx aspirations six months apart with very minor improvement. My arms were now weak and there was no feeling in most of my hands. A syringo-peritoneal shunt was considered the only thing left to try. It didn’t help. Within three months I was a quadriplegic with no use of my hands but some shoulder and upper arm strength.
Yes, I’d read all those consent forms that listed all the possible outcomes of the various procedures. To be honest, I thought I’d either get well or die. To be 28 and alive without my job, my home, my car and the ability to care for myself had never been an option in my mind. I was devastated. PT and OT rehab workers flooded me with aids for daily living. Nine months later, after a suicide attempt, I was sent to a rehab hospital and given an electric wheelchair. This new mobility helped, but living in a nursing home didn’t. In the next four years, I had a shunt revision (my syrinx filled when my shunt clogged) and spent six weeks in a pain management program. That inspired me to get out of the nursing home and into my own. I found an apartment and a wonderful live-in caregiver. Social Security Disability isn’t much to live on, but I was as independent as possible and it felt great.
I must add here that I was fortunate to be free of chronic, immobilizing headaches or other pains that affect many with CM and/or SM. Only because of a strong and adventurous caregiver did I travel all over California, go camping in various state and national parks, have a house full of pets and spend more time outside the house than in it.
In the years that followed quadraparesis, my health slowly–but progressively–worsened. First came an indwelling catheter and bowel program. After a year of profusely sweating on my right side only, a neurologist found medication for that. Bladder infections came too often, not to mention bladder stones, and I learned the value of drinking lots of water and catheter irrigation. Choking, coughing and breathing problems developed, whereupon MRIs found my syrinx pushing higher and a cyst in my brain stem. A syringo-pleural shunt drained that cervical syrinx (and a thoracic one later in 1991). I broke my arm and was diagnosed with osteoporosis. A tiny decubitus went bad in 1997 and infection spread into my ischial bone, prompting orthopedic and plastic surgery. Then there was the mild stroke in ’98 that left my left side/arm even weaker.
Leading SM authorities (and, believe me, I’ve seen the few there are) have said there is nothing more to be done for me. Any spinal surgery, they say, could put me on a respirator and that’s one way of living I’m not ready to deal with right now. With the help of ASAP, NORD and the Internet I’ve learned so much about my disorder in the past 10 years. It’s easy to talk with specialists now that I know what arachnoiditis, dura and other things mean and how they’re affected. When an unusual new pain started ravaging my entire back and right side in early ’98, I knew it was central nerve pain but was at a loss as to why it had begun so late in my SM life. Silly me, I keep thinking it’s got to stop sometime.
As I sit on the door of my 48th birthday, I spend a lot of time on the computer communicating with others who live with this disorder. Each one of us is so unique. No two have the exact same symptoms; everyone has a different story about getting to this diagnosis. I’m amazed that almost 40 years after I sat looking at inkblots, other patients are being told their symptoms are the result of stress, “women’s problems” or just in their heads. A major educational process can eliminate many misdiagnoses, but only research can find a cure for this preternatural thief of healthy bodies and lives lived to their fullest.