I promised in an earlier post to explain about ACM, a condition I do not have. As I explained, I have so many friends online who are suffering with this and related conditions, AND my surgeries will be happening at The Chiari Institute in New York. Unless someone you know has ACM, you probably don't know what it is. I know I certainly did not. And for my friends who
have this and are battling to get the word out, I will paste here an article I wrote for ChiariConnectionInternational members. I felt this article could be used to send to their hometown papers and hopefully generate enough interest that a story might be requested.
Here is my article:
LITTLE-KNOWN SPINAL DISORDER SHOWS FEW OUTWARD SIGNS
Vicki is a 28 year old mother of two rambunctious children both under the age of six. She appears healthy as she enters through the doors of the gym where she works as a personal trainer. Pony-tail swinging and accompanied by the muffled sounds of her cross-trainer shoes upon the sidewalk, she greets her clients with a smile.
Yet, Vicki is holding on by a thin thread, for she is fighting a number of debilitating health issues which belie her appearance of "normal." In spite of a broken spinal fusion for which she is seeking surgical help, she is also battling a rare condition known as syringomyelia which developed after a motor vehicle accident 2 years ago.
Once healthy, strong and outgoing, Josh earned his living as a hardworking power lineman. An onset of debilitating symptoms and subsequent diagnosis of syringomyelia now keeps him mostly homebound, advocating for improved working conditions for those dealing with high-intensity electrical wire atop dizzying power-poles.
Vince, a renowned radio-controlled airplane competitor, now stays home to watch his children and suffers in pain after an auto accident injured his spine and left him battling syringomyelia (SM) instead of downdrafts and demanding landings. His wife works full time to pay the bills.
Keesha is also a mother of young children. In her late 20's, she has earned three college degrees and is a qualified teacher. She should be continuing her teaching career, working in her Arizona yard or riding on amusement park rides on the weekends with her young family. Instead, she is filling out forms for Social Security Disability Insurance, traveling to hearings and doctor appointments while she faces the challenges of knock-you-to-your-knees headaches, weakness and pain throughout her body. Keesha suffers from not only syringomyelia, but also a related condition called Arnold Chiari Malformation (ACM), or simply "Chiari."
Once these patients receive the news that they suffer from one or both of these incurable conditions, their lives will never be the same. They may appear "normal" to the world around them, yet they deal with neurological pain and diffuse weakness which prohibits them from living any kind of normal lifestyle.
Chiari was discovered in 1898 by Professor Hans Chiari during his postmortem studies in Germany. Long before the advent of MRIs and radiological imaging, Dr. Chiari found that a part of the cerebellum (which lies at the back of the head and is believed to control movement) can herniate into cone-shaped "tonsils" which then fill up the hole located at the junction between the bottom of the skull and the top of the cervical spine. When the tonsils dip far enough down into this hole, the ebb and flow of cerebral spinal (CSF) fluid is obstructed.
One hundred years later, in 1998, Dr. Thomas Milhorat, along with his colleagues, released a study where he redefined ACM as being not just the length of herniation of the cerebellar tonsils. It was discovered that the ectopic tonsils are pushed into the spinal canal because the posterior fossa (the compartment which houses the cerebellum) is too small. Dr. Milhorat founded The Chiari Institute.
Betsy, an artist in Pennsylvania, knows well the symptoms caused by obstructed CSF flow and the subsequent pressure upon vital nerves. She has lived with Chiari for 30 years and endured 12 surgeries. Her symptoms run the gamut from simple "pins and needles-type" tingling, numbness, impaired cognitive function, inexorable headaches which radiate out from the base of the skull toward the eyes and ears and then radiate down through the neck to the shoulders, arms and hands. Tremors are typical, along with bladder and bowel dysfunction, sleep apnea syndrome and a diffuse, painful weakness that defies description.
Many people are often misdiagnosed before finding out they have Chiari, a condition which afflicts women 3 to 1 over men. They are told they have multiple sclerosis, fibromyalgia, restless leg syndrome or chronic fatigue syndrome. Or, worse, in the case of women, some have been told their symptoms are attributable to PMS. Men and women alike are often chided that it is all "in their heads" and are given prescriptions for antidepressants or psychological evaluations.
Patients begin to believe those doctors respected within their community and thus question their own sanity or work ethic. Until one day, a bright, young neurologist; an older, compassionate GP; or even a licensed homeopathic-practitioner recognizes the symptoms and urges the sufferer to have an MRI done of their brain.
The good news is that with the correct knowledge and expertise in Chiari, it is easy to diagnose the condition with a simple look at the patient's films. The bad news is there simply is no cure. Surgery can be performed but the best that can be promised is a slowing of the progression of symptoms.
Neurosurgeons enter the back of the head at the base of the skull and remove bone and tissue to increase an opening for spinal fluid flow. Some surgeons will also cauterize the cerebellar tonsils in order to encourage the hole-plugging tissue to back away from the vital opening at the top of the spine.
Linked to Chiari Malformation is another spinal cord condition known as syringomyelia (SM), a disease where a fluid-filled cyst or syrinx occurs within the spinal canal. CSF moves up and down through the space around the cord with each heartbeat. The fluid inside the syrinx can compress the cord out against the adjacent bony rings of the spinal vertebrae, thus compressing and damaging the nerve-laden interior of the cord . Some surgeons elect to divert any fluid from reaching the syrinx through use of a shunt inserted into the cord.
75% of syringes are found to be caused by the obstructed CSF flow from a Chiari condition. At other times, a syrinx is brought on by head or spinal trauma such as whiplash incurred during a motor vehicle accident, and no Chiari is present.
"While research into the causes of the severe, nerve-damage-related pain is progressing, the actual mechanisms involved are still poorly understood. Current medications are not effective in many cases or only partially successful in dampening down the pain and other symptoms of Chiari and syringomyelia," says Sarah Bates of Paradise, CA, herself a CM sufferer and advocate for her peers through her role as board member of the Wishes and Rainbows organization, which "educates, encourages and assists" those with CM and/or SM.
To Betsy, Vicki, Vince and others like them, the hardest things to face are often outside the doctors' offices. Families think they look fine and healthy so they must be lazy or obsessed unnecessarily with their health. Employers cannot understand why a previously outstanding employee begins to decline and cannot keep up an adequate standard of work. Insurance case-managers and disability examiners cannot comprehend the pain and weakness inherent to syringomyelia and Chiari because, though these conditions are not really rare, they are not widely-known or understood. The average doctor may not encounter a single case in his career.
The results are the misdiagnoses and judgment that these sufferers must deal with in conjunction with their symptoms.
A person can be born with Chiari Malformation and lead a full life never knowing their condition exists. Such a person may only become symptomatic when trauma is experienced: an accident, fall, difficult childbirth or even a lengthy bout of sneezing.
"Education and awareness are key in finding treatment and a cure for those afflicted," Sarah Bates states.
To read more about Chari Malformation, go to www.chiariconnectioninternational.com.
To learn from those who suffer, go to CCI's message board and support group:
Visit this site for more information and a chance to help Chiarians, especially children at Christmas time in their "Santa Smiles" project.