We’ve gotten our store open in record time and we want to celebrate with you by giving big discounts for our Grand Opening. From now until December 1, use our exclusive code to get 25% off every order! Just read on to get your code and visit the store. First, we’ve got a wee bit of business to cover and we hope you’ll read on to learn more about what the Zebra Pit Shop is all about and our plans for it!
Why Open a Shop?
The easiest answer to this question is that it helps us to keep doing what we love to do, and that’s bring health and wellness information and resources to help people with Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders and/or their common comorbid syndromes and conditions. This includes ME/CFS, Fibromyalgia and other soft tissue pain disorders, MCAS and other MCADs, POTS or other forms of dysautonomia, gastroparesis, IBS, Chiari, CCI and other cranio-cervical junction disorders, migraine, degenerative disc disease and related disorders, arthritis, costochondritis, and so much more we could spend all day listing them. Here are a few more reasons we opened our shop:
To provide products that raise awareness and help build a sense of community in ways unique to a variety of personalities and that are reflective of the times and spoonie life. It’s our mission to make EDS and all of its comorbid conditions well known across the world and these products help us serve that mission both literally and figuratively.
To display and sell some of the wonderful items we’ve found that truly help with some of our most difficult to solve symptoms. While we could just continue to act as an affiliate for some of the products we’ll be adding, this means the Zebra Pit gets a little more of the cut and has more control over what we can do with those funds.
So we can support the organizations that are making the biggest impact on these conditions. [UPDATE: 11/20/19] We planned on doing a dedicated retail giving program, but as it turns out, several states require you to register to do so. The cost to do so is in upwards of $10K! Obviously, that isn’t possible. We will definitely still be supporting the charities that matter to us, but we cannot do it by donating a percentage of our proceeds at this time due to legal/financial constraints. This won’t stop us from giving, but it will stop us from advertising it and allowing you to designate a specific program.
So we can better support the community we serve by getting the best prices possible and ensuring you always have a place to get the things you need to help you achieve your best health. Whether it’s a product we’re recommending or a post about promising therapy techniques, you know it’s all vetted by people who share these conditions, Understand how it works and whether it needs to be modified. We always want you to check with your doctor before taking any of our advice, but it can be reassuring to know that something works for similar people before investing the money on it.
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In addition to the plan to add on as many of our recommended health and wellness products, we have several goals we hope to attain as we grow:
Adjust pricing as we grow. We may not get the bigger discounts to begin with, but as we grow and they come along, rest assured the Zebra Pit will be sharing any decreases we see with our community. We understand the financial toll of chronic illness because we also live with chronic illness.
To provide a comprehensive specialty shopexclusively for spoonies and medical zebras that include supplements, therapy devices, health and fitness gear, a whole slew of over-the-counter remedies, CBD products, books both informational and entertaining, self care and personal care products, mobility devices, and more.
To provide products which are ideal for most people with sensitivities to use. For example, while I couldn’t find tagless shirts for our printed products, everything we’ve selected has tear away tags for easy removal so those of us living with any level of sensory overload can feel good in our clothing.
To work toward creating and offering some of our own products.
To source our products as ethically as possible.
To support our community and customers by implementing fair practices and providing good quality products.
Shop with Confidence
In regards to our awareness products, if the item you receive arrives damaged or is not printed perfectly, you simply need to send us a photo and we’ll send you a replacement that’s printed just for you.
If your order isn’t right, we’ll do our best to make it right as quickly as possible.
If you’re unsatisfied with your purchase, ordered the wrong item or size, you can return it to us for a refund of the product price or exchange within 30 days, provided it’s returned in brand new condition.
As we grow, these policies may change, but our commitment to quality will always remain. To view our shipping and return policies, click here: Shipping and Return Policy.
Other Changes Around the Pit
I’m sure you’ve noticed that our menus and structure has changed recently to accommodate our store. We like our new design quite well, but it may take some a bit of time to get used to it. Here are a few tips that may help:
Our Blog page looks quite different, but it now has 3 different ways you can view our posts. My hope is that it offers something to suit every browser.
The first is a 20 post carousel that shows the featured image, title, date and brief description. It always begins with the most recent post.
The second section is an image catalog of our sections, so you can view posts by subject.
Finally, our last 100 posts are listed at the bottom so you can scroll through them much like our old page, but they only include titles, dates and descriptions.
Finally, we have everything arranged in convenient drop down menus in our header (the upper purple section) you can access both on a computer or smart phone. To go to the blog, simply click the word “blog.” To visit a specific section page, choose it from the drop down menu.
Search Boxes: As always, we have a search box near the top of the side menu so you can search for anything you may be looking for. If it’s an item from the shop you seek, use the search box in the purple header instead.
Now finally…. the news you’ve waited so patiently for…..
Now that we’re done with the boring stuff, why not take a tour of our shop? Be sure to take this code with you:
The code is good for 25% off your entire purchase on anything in our shop. Want to shop more than once? Use it as many times as you’d like! The best part? While everyone else is having “Black Friday,” “Small Business Saturday,” or “Cyber Monday,” sales, our grand opening sale will last two weeks! From today through Sunday, December 1, 2019.
Can’t afford to shop right now? If you reside in the U.S. you can enter to win 1 of 3 prizes in our Name Our Mascot Contest between now and November 25, 2019.
October 16th is World Spine Day. World Spine Day is part of Bone and Joint Decade Action Week. The day was formally launched by the World Federation of Chiropractic in 2012, with the goal to raise awareness about spinal health and spine disorders.
World Spine Day will be celebrated on every continent, with health professionals, exercise and rehabilitation experts, public health advocates, schoolchildren and patients all taking part.
If you’ve ever experienced back pain before, you know how much it hurts. Often the problem is muscular, but occasionally the problem is directly related to the spine.
This link to the website Spine Universe gives you a complete list of all the various spinal conditions and information about them.
Causes of Spine Disorders
Spine disorders have a wide variety of causes depending on the particular condition. For some conditions, the causes are unknown. Common causes include:
Abnormal Bone Growth
Accidents or falls
Congenital disorders (present since birth)
Degenerative wear and tear that comes with ageing
Injuries ranging from minor to traumatic
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Risk Factors for Spine Disorders
Factors that can increase the risk of developing a spine disorder include:
Excess weight or obesity
Improper lifting techniques
Nutrition and lifestyle habits such as sedentary lifestyle, low calcium intake, or smoking
Other conditions such as osteoarthritis, rheumatoid arthritis, or thyroid disease
Overuse from exercise or occupational movement
Repetitive strenuous activities
Symptoms of Spine Disorders
Signs and symptoms depend on the specific spine disorder and often affect other parts of the body, depending on the area of the spine or spinal cord that is affected. Common symptoms include:
Abnormally rounded shoulders or back
Back or neck pain that can be sharp and stabbing, dull and aching, or burning
Bladder or bowel dysfunction
Nausea and/or vomiting
Pain radiating in the arms or legs
Stiffness or tightness
Uneven appearance, such as one shoulder or hip being higher than the other
Weakness, numbness, or tingling in the arms or legs
Diagnosis of Spine Disorders
Spine experts will conduct a thorough evaluation, including:
Discussion of personal and family medical history
Discussion of symptoms and risk factors
Neurological exam, if a nerve injury or disorder is suspected
Depending on each patient’s individual case, your doctors might recommend one or more tests, such as:
Magnetic resonance imaging (MRI) scan: MRI uses radio waves and a strong magnet to produce detailed images of the spine. MRI is useful in detecting injuries and disorders in soft tissue such as muscles, ligaments, tendons, spinal cord, and nerves.
Computed tomography (CT) scan: CT uses specialized X-rays with or without a contrast agent to produce cross-sectional, 3D images of the spine. CT provides images that are more detailed than plain X-rays for evaluating bone injuries or disorders.
X-ray: X-rays of the neck or different areas of the back to check for bone problems such as fractures, other injuries, and chronic disorders.
Biopsy: If cancer is suspected, neurosurgeons can take a small tissue sample for analysis under a microscope.
Electromyography (EMG): Electrodiagnostic examinations measure electrical activity generated by muscles and nerves. They generally involve seeing how different parts of the body react to stimuli.
Treatment for Spine Disorders
Spine specialists often use one or more treatments, depending on the specific condition or injury. Treatments offered include:
Cancer treatment such as surgery to remove tumors, radiation therapy, radiosurgery, and chemotherapy
Injections, such as corticosteroids or nerve blocks, for pain
Massage for relief of back pain
Medications such as anti-inflammatories, pain relievers, or muscle relaxers
Rehabilitation using physical therapy to strengthen and stretch the back and abdominal muscles
Surgery to replace discs, fuse (connect) vertebrae, open up the spinal canal, or repair nerves
Lifestyle Is Important
It’s important to pay attention to your lifestyle when it comes to spine problems. If you are overweight, it puts added pressure on your spine and can cause issues like arthritis to become even more painful. Exercise on a regular basis and do spine strengthening moves such as stretches and yoga.
Be aware of your surroundings in order to avoid accidents and falls. Use proper safety equipment at all times, especially if you are working at heights such as roofs or trees.
Lift properly, using your legs and not your back. Even a minor change in your lifting habits will help to protect you. Engage your core muscles and using lifting straps for heavier items.
Quitting smoking is always good as it helps to increase blood circulation which is always good for the body including the spine.
A healthy spine is a happy spine. If you are experiencing problems with your back, see your Family Physician first to rule out any serious problems. Massage or Chiropractic care may be the first step to healing, or you may need to see a specialist. The important thing is to seek treatment early so you have the best chance of gaining full recovery.
Pamela Jessen lives in Langford, BC Canada. She is a blogger who writes about Chronic Pain, Chronic Fatigue and Invisible Illness at pamelajessen.com. She also writes for The Mighty, PainResource.com and various independent publications. Pamela is also a Patient Advocate with the Patient Voices Network in BC. She sits on 4 committees and one Provincial working group and has also been involved in advocacy work at the Canadian National level as well. Pamela is married to her amazing husband Ray and they have one cat named Dorie.
To complete our series on Chiari malformation and Craniocervical Instability, I wanted to share more of the interviews that I didn’t have the opportunity to use during my exploration of the Issues of Diagnosis and Treatment. These interviews are great supplemental material to that discussion and help to see the problem from the perspective of those who have the condition. We wrap it up with my own information, as representation of what I suspect are hundreds of thousands of people suffering from undiagnosed forms of craniocervical junction disorders and why I believe I have one. While this group is by no means insignificant, ultimately none of us will know what affects us until finally put through proper testing, as described in this post.
This is the third post in our Chiari Awareness Month Series: The first two are available here:
Out of respect for the privacy of participants, I am only publishing their first names and general location. Some people preferred to use a pseudonym, which is denoted with an asterisk (*) next to the participant’s name.
Location: Sacramento, CA
Time from onset of symptoms to diagnosis: 25-30 years
Jackie underwent posterior fossa
decompression with craniotomy, C-1 laminectomy and duraplasty in November,
2017. Prior to surgery, Jackie suffered severe head pain, persistent weakness
in her right arm, and by 2015, extreme dizziness, lightheadness and near
syncope that was caused by orthostatic hypotension that had no other cause. It
was believed that Jackie’s Chiari malformation which was classified as Chiari
1.5, was congenital in nature, due to the small size of her posterior fossa,
which caused everything to get pushed forward. When asked if she considered the
surgery a success, she noted “For me the procedure was extremely successful. It
eliminated my neck pain and chiari headaches completely. It also completely
cured my chronic hiccups and motion sickness (which I wasn’t expecting). The
only thing that remains is the orthostatic hypotension which is less severe. I
manage that with increased salt intake only.”
Despite the successful outcome of Jackie’s surgery, achieving it was a bit of a feat. “My primary care doctor sent me to a neurologist who ordered the MRI that revealed the Chiari. The neurologist completely blew it off, insisting it couldn’t possibly be causing my symptoms. He thought my problem was “vestibular migraine.” Even though my headaches never completely fit the migraine profile, that’s what doctors assumed I had. I had to keep trying to find the cause of my dizziness, and it took until September 2016 to pinpoint orthostatic hypotension. It was tough navigating the different workups. Once I was able to get two neurosurgery opinions, things got much easier. I was pretty lucky in that I had good insurance (a plus in the United States) and I had access to a brilliant neurosurgeon at Stanford University.”
Jackie’s case demonstrates how even when Chiari is clear cut and easy to diagnose; the conclusions of doctors who aren’t really qualified to make a determination about intervention, but who do anyway can be a huge obstacle to intervention and treatment of Chiari. Lucky for Jackie, she didn’t let his ill-advised treatment plan (to do nothing and assume her problem was something else) stop her from seeking treatment elsewhere. I also feel it’s important to note that Jackie’s doctors were smart enough to evaluate her for the presence of hypermobility before making a final determination on how to treat her.
South East England
Time of Symptom Onset to Diagnosis:
Christina has EDS related Chiari malformation. It took Christina 30 years to get a diagnosis of EDS. She had an upright MRI because of awful pressure headaches. A horizontal MRI missed the presence of a malformation, but she persisted and was given an Upright MRI at the Medserena in London, which finally confirmed the diagnosis. To date, Christina hasn’t had any procedures. For her Chiari, if symptoms continue to progress, she will need surgery: However, Christina’s chiari malformation and symptoms are complicated by Craniocervical Instability. The procedure which she needs, a spinal fusion, isn’t available through UK’s National Health Service, forcing Christina into the position of raising funds to so she can have both surgeries with a doctor in Spain.
Location: Newark, DE
Time from onset of symptoms to diagnosis: unknown
Maura was diagnosed with Chiari I in 2007 at age 6 via a traditional horizontal MRI. “I had a decompression shortly after diagnosis and had significant relief for 8 months. After that symptoms returned but slightly differently, so I had a full decompression. Turns out it was craniocervical instability that caused the symptoms to return after the first decompression so the second decompression was ineffective.” Despite this setback, today Maura’s only residual symptoms of CCI and Chiari is that she struggles with fine motor coordination. In additiona to chiari I and CCI, Maura lives with hypermobile Ehlers-Danlos Syndrome, Gastroparesis and POTS.
In Maura’s case, had she been
evaluated for the presence of hypermobility like Jackie was, it’s conceivable she
would have been able to avoid two separate surgeries. It is unlikely the
condition developed in such a short span as eight months.
Time of Symptom Onset to Diagnosis: Unsure. Kaitlyn had symptoms from early childhood that were ignored due to similar familial symptoms until an injury in high school revealed her condition.
Kaitlyn was diagnosed in July of 2006 with Arnold Chiari Malformation type 1 via traditional horizontal MRI following her cheerleading accident. Treatment for Kaitlyn came quickly, being performed in a matter of months after diagnosis. She had posterior fossa decompression surgery for a 2.2cm herniation. During the surgery they performed a laminectomy of her C1 to create more space in the spinal canal, opened the dura, cauterized some of her cerebellar tonsils and inserted a stent for cerebrospinal fluid drainage.
Kaitlyn considers her surgery successful and has no plans for further intervention: However a number of her pre-operative symptoms remain. “I have debilitating [hemiplegic] migraines that replicate stroke symptoms, neck stiffness, neck pain, trouble swallowing and my head shakes.”
notes location and lack of knowledge to be the biggest obstacle in her ongoing
care. “I live in a small town in Kentucky and the doctors around here have
little to no knowledge about Chiari Malformation. There have been doctors [who]
Google ‘Chiari malformation’ in front of me and then act like experts. Recently
I have traveled to Cleveland Clinic and Johns Hopkins to meet with doctors who
were knowledgeable about Chiari Malformation. Being able to meet with a doctor
who knows about Chiari Malformation has been the hardest part of this journey.”
Kent, England, UK
of Symptom Onset to Diagnosis: None
Martha’s Chiari I malformation
diagnosis for Chiari was incidental. At the time, she wasn’t experiencing any
symptoms. That was in 2012, but lately she’s found things have changed. “I very
recently have developed tingling in my lower jaw/around my mouth, chattering of
the teeth, TMJ type pain and severe headache mostly on the right side.” Martha
is currently waiting for another neurology consultation to discuss her new
symptoms and next steps. During another MRI for her symptoms in 2017, they diagnosed
her with possible facet joint compression due to posture, which has been helped
by regular physiotherapy. In addition to Chiari, Martha has Ehlers-Danlos
Syndrome, Fibromyalgia, hypothyroidism and lipedema.
of Symptom Onset to Diagnosis: 15 months
has Chiari I malformation which was discovered through MRI, but she couldn’t
recall which kind. In addition to Chiari, she has pseudotumor cerebri. Jennifer
had posterior fossa decompression, which failed. Multiple procedures, from
nerve blocks to a spinal stimulator were attempted in an effort to reduce her
pain, but none were sufficient. Her final surgery in January ’18 entailed a
fusion of C1-3 at Johns Hopkins.
Today, Jennifer regrets all these attempts. “If I had to go back, [I] would not have any of the surgeries or procedures. The 8 years that I was sick was nothing but obstacles. It was a nightmare. I almost took my life last Halloween, because that final surgery just about broke me. We have struggled financially. It’s been hell for my children, and it destroyed my marriage. I’m now a single mom and rebuilding my life from nothing.”
Since Jennifer has given up on surgical intervention, she
lives a different sort of life now.
I walked away from doctors and pharma last year, I really, really did. Makes me
feel icky to even look at medical stuff. None of the surgeries or procedures
helped me. They just made me worse. I got off of pharma and trained myself to
process pain in healthy ways. Found the app, Curable, super helpful, and I
started CBD oil, which began healing the damage to my cerebral cortex.
pain level varies with the weather, and today it’s kicking my ass. In another
month or two, I may have a totally different story. I’m currently in spasm and
probably functioning at 6-7… have to slow down to a snail’s pace during the
summer… and that’s very hard on me mentally, because I often feel like I
missed out on 8 years and don’t want to waste time.
“I’m in as much if not more pain, now, compared to 2013, but I don’t process pain the same. I’ve dealt with the emotions that came along with pain… guilt, fear, disappointment, shame, etc. So, now pain is just pain. All humans function in some type of pain, whether physical or emotional. It’s up to us to find a healthy way to walk through it.”
of Symptom Onset to Diagnosis: Unsure.
Rose has a somewhat unusual story in that she was first diagnosed with Chiari at birth, but while the surgeon discussed her hydrocephalus and the intervening shunt they would place, he failed to report and explain that she also had a Chiari malformation. When she was diagnosed in 2004 with Chiari, it came as a total surprise to her. It wasn’t until after that Rose discovered the paperwork in her parents’ records, which briefly mentioned the diagnosis.
The onset of Rose’s symptoms are hard to determine due to a cascade of health problems that probably coincided with onset. “I started having problems that I thought were allergies about 9 months before. Then in September I had a shunt failure. I was officially diagnosed June of 04 through the use of traditional head/ abdominal CT, MRI and x-rays. There was still concern that the shunt was failing again. Decompression was done June of 2004, after 3 shunt revisions had been performed.
“[I] haven’t needed any other surgeries for that. My symptoms after surgery were stable, despite a six month recovery.” In addition to Chiari, Rose was diagnosed with Ehlers-Danlos Syndrome in 2010 and developed POTS a few years after her Chiari treatment, so while she’s had favorable outcomes to her chiari, she still struggles with many other symptoms.
of Symptom Onset to Diagnosis: 10 Years and Counting
I believe my own story belongs here like that of many others. While I do not have a diagnosis of Chiari or CCI, I have symptoms consistent with CCI. My earliest started in my early 20’s, when I started passing out every time I tried to extend my neck backwards to look up. My symptoms have compounded over time and I struggled the most over the last ten years with the following symptoms, which have fluctuated over time; severe head and neck pain, tremors, feeling like my head was simply going to drop off, occasional speech impairment, significant impairment of cognition and memory, weakness in my arms, dizziness, difficulty with my fine motor skills, tinnitus, insomnia and depression. I have gone through jags of vomiting for days to entire weeks due to my head pain and go through periods where I get hiccups several times in a single day.
As I explained in this post, like many of the men and women who are absent diagnosis despite such strong symptomology, I do not have a diagnosis because my malformation doesn’t become apparent until I am in the upright position or my neck is extended and flexed and the only testing available to me is a traditional MRI. Despite my request for an upright dynamic MRI or flexion and extension MRI, I have been told by multiple neurologists that “upright MRIs don’t work” or “I have no idea how to order that test,” or “let’s see what we can do about this in other ways,” which usually entails failed drugs with side effects too great to bear, attempts at nerve blocks, and other unsavory options like botox.
Another EDSer in
my hometown had to go to New Jersey to be treated by a surgeon who first
ordered and read her MRIs through email for a fee. Without doing so she would
still be living with her symptoms and yet we both live and go to the best
teaching hospital in the area, where one would hope the doctors would be aware
of the most cutting edge techniques.
Recently, I had
a lumbar puncture to relieve the pressure my CSF was causing. At this time, the
source of my increasing pressure is considered “idiopathic” or of unknown
origin, but I feel strongly that there is a cause that could be easily
determined with the proper test. Removing some CSF fluid helped initially with
my tinnitus, light sensitivity and positional headaches, but they have returned
in just a matter of a few weeks and I feel it is likely the next steps will be
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My symptoms have
fluctuated over the years and I feel I’ve made good headway in shoring up my
neck through physiotherapy and excessive rest. Still, my neck occasionally
slides out of joint and that familiar, unbearable pain returns. One of my
uppermost vertebrae now has so much play in it, I can move it freely with only
a gentle push, but for whatever reason, it hurts me much less and I have
greater range of motion than I used to. Still, my head pain persists and I have
to take daily abortive medications for migraine and limit the amount of time I
spend in upright positioning. Even sitting in my recliner can cause a nasty
headache that’s impossible to relieve without lying flat.
While I want to know if Chiari and/or CCI are the cause of my misery, I am not at all sure I would agree to surgery, so I haven’t contacted an expert outside of my area for consultation. I may yet, depending on the evolution of my symptoms.
During the course of my interviews, I had no less than a dozen people come forward with similar stories and I see others on my friend’s lists, among my followers and in my support groups who suffer with these symptoms and also suspect strongly the cause to be the same. Like me, these people have neither the means nor the access to do the proper testing. My belief is that Chiari and CCI contribute heavily to the missing millions of ME/CFS and is largely occupied by undiagnosed people affected by EDS. I feel certain one of these conditions is indeed responsible for the years I spend in bed, barely able to function and fear regression. Though I treat my symptoms well through the use of diet, exercise and supplementation, my life is greatly affected by this condition, which I’ve come to privately term “the condition that shall not be named.”
Have your own story to tell? Please add it in the comments. This story is far from over and the more patient voices who get heard the better.
As always, thanks for reading. I hope the stories of people with Chiari and CCI have helped you to understand firsthand what it’s like to live with these rare conditions. If you’d like to know more about these conditions, please take a look at the other posts in this series meant to bring awareness to the issues of Chiari, CCI and craniocervical junction disorders overall.
For More Patient Perspectives on Chiari and CCI, check out these posts:
*The names of some subjects
interviewed for the post have been changed to protect their privacy. These
changes are denoted by an asterisk (*).
In my first post on Chiari malformation, I outlined the standard procedures for the treatment and diagnosis of craniocervical junction disorders, primarily Chiari malformation, though also touching briefly on Craniocervical Instability. I also highlighted a few of the issues with getting a proper diagnosis, due to the improper use of traditional horizontal (supine) imaging techniques as the standard of diagnosis, which is propelled by a lack of knowledge, training and availability, even in some teaching hospitals and medical centers that tout their use of cutting edge technology.
While my focus in this series is on Chiari malformation and craniocervical instability because these are the two most commons forms of craniocervical junction disorders associated with Ehlers-Danlos Syndrome, it’s important to understand that there are many forms of craniocervical junction disorders and that diagnosis and treatment of all of these conditions are problematic. At times, I will discuss CJD as a whole, because there is research which impacts them all and I feel they’re all interrelated, as are many of the secondary conditions that tend to develop with CJD.
I do this also in part because, as one researcher noted in the literature I reviewed for this series, there is little consensus on the diagnosis and treatment of these disorders, even their classification. For example, while some experts consider craniocervical instability as part of Chiari type I while others have labeled it Chiari 0 and others insist it is an entirely separate entity and shouldn’t be categorized as a Chiari malformation at all. These discrepancies aside, all of these disorders can exist alone or in unison and whether wholly congenital, caused by illness, injury or hypermobility, every patient with CJD deserves a thorough workup with the use of proper imaging to ensure the most successful procedures possible.
The underdiagnosis and mistreatment of Craniocervical junction disorders are experienced worldwide, in both private and public healthcare systems today. We will explore this phenomenon through the use of two primary sources; a review of current literature available on the subject and direct patient feedback I collected through interviews conducted in writing with people who have been diagnosed with Chiari and craniocervical instability (CCI), also known as Occipitoatlantialaxial Hypermobility.
While I am happy to be able to share a few success stories, my review was rather disheartening. Information on the subject of the proper treatment and management of craniocervical junction disorders is readily available, yet it is not being utilized by the people making decisions about the processes and procedures necessary to come to or rule out a diagnosis of a craniocervical junction disorder (CJD), let alone how to treat it with precision for better surgical outcomes for patients, with the exception of a select few “Chiari experts” worldwide. These doctors are few and far between and are often difficult for patients to connect with due to issues of location, socio-economic status (including insurance coverage, if any is even accepted by these doctors).
Poor Training and Availability of Testing Persist in the World’s Richest Countries, Regardless of the Healthcare System’s Structure
The best information I found was out of India, where the prevalence of CJD is higher than anywhere else. Given the range of techniques performed to gain accurate measurements based on positioning (the use of Upright [functional] MRI, flexion and extension positioning in MR imaging) and investigation of CSF flow and movement through the use of dynamic cine MRI, one must ask themselves if perhaps the higher number in cases in India can be partially accounted for by their use of advanced techniques. They have taken the time to carefully investigate the most useful and varied methods of diagnosis, therefore uncovering the actual variety of these disorders that do not show up with the supine positioning of the patient in traditional MRI.
In many cases, available options for patients in publicly funded healthcare settings are even worse than those living in countries with privatized healthcare, where patients with rare disease requiring specialized care are often left to fend for themselves. Privatized healthcare systems can’t attract top experts and are unwilling to pay premium pricing to help what they consider to be a small portion of the population. In Canada and the UK both, patients are forced to seek care in foreign countries, as their own privatized healthcare systems refuse to diagnose these conditions or honor cross-border diagnoses.
Take for example, the case of Marjorie*, a UK resident aged 21. Marjorie had to go to France for evaluation and diagnosis of her symptoms. When the doctor diagnosed her with CCI, she felt utterly triumphant, but the realization that she couldn’t get treatment for her condition, a much needed spinal fusion, in the UK. In order to be treated, Marjorie and her parents have been forced to turn to fundraising, so they can return to France for the needed operation. The price tag for her surgery is estimated to be $60-80K.
While in the US, Gina* aged 38, traveled from Kansas City, MO to a doctor in Florida for diagnosis and treatment of her Chiari Malformation and craniocervical instability, a process which took her over 5 years to complete because she had to crowd source donations to meet the cost of treatment, as well. Even after everything Gina went through to achieve treatment, she continues to live with the same debilitating symptoms as before; her surgery a complete failure.
While these problems have differing sources, it’s the same for patients in both settings; the rarer the condition, the more expensive the doctors and treatments. Both situations leave treatment attainable only to those who can afford travel expenses and exorbitant charges for their healthcare needs.
Poor Surgical Outcomes Due to Inadequate Testing
Even when you do luck out enough to gain access to medical intervention, these surgeries are only successful for some. In the course of my research, I came to understand this was largely due to the outdated use of imaging techniques which provides a lack of clarity in both the severity and variety of issues faced in this population. It is my feeling that many patients are undergoing these surgeries without successful outcomes because surgeon are effectively going in blind, or with only a partial view and understanding of the full problem. Because of this, many patients are no better off post-operatively than they were before or experience only mild improvements.
Atul Goel, department head of neurosurgery at K.E.M. Hospital and Seth G.S. Medical College in Mumbai states
The treatment of basilar invagination [a form of craniocervical junction disorder in which a vertebra at the top of the spine moves up and back, toward the base of the skull (3)] must be based on the understanding of its pathogenesis. As complications related to an inappropriate treatment can be devastating in nature, exact anatomical and biomechanical evaluation and precise surgical treatment is mandatory in these patients.
How does Goel recommend this investigation take place for basilar invagination and other conditions within CJD? Through the extensive use of modern day MR imaging techniques, including those mentioned earlier, dynamic MRI, upright (functional) MRI, and MRIs performed in the flexion and extension positions. In addition to MRI, Goel recommends the use of CT (over that of x-ray) to get accurate measurements of the size and bony features and protrusions of the neck, foramen magnum and posterior fossa, which could further complicate treatment or need to be addressed through the use of additional surgical techniques.
His argument that the origin of CJD is necessary to treatment plans are best explained in his own words:
In our recent study, we identified patients in whom there was ‘vertical mobile and reducible’, wherein there was basilar invagination when the neck was flexed, and the alignment was normal when the head was in extended position. Although such mobility is only rarely identified, it does indicate the need for dynamic flexion-extension studies to preoperatively assess the craniovertebral instability. In Group B the alignment of the odontoid process with the anterior arch of the atlas and with the inferior aspect of the clivus remains normal and there is no instability in these patients.
Majority of patients (58%) with Group A basilar invagination had a history of minor to major head injury prior to the onset of the symptoms. The pyramidal symptoms formed a dominant component. Kinesthetic sensations were affected in 55% of patients. Spinothalamic dysfunction was less frequent (36%). Neck pain was a major presenting symptom in 77% of patients. Torticollis was present in 41% of patients. The analysis of radiological and clinical features suggests that the symptoms and signs were a result of brainstem compression by the odontoid process.
This view is quite a bit different from doctors in the US who often seem to believe Chiari and similar malformations of the foramen magnum are always congenital in nature. It could also explain why some surgeries are much more successful than others.
In “Image Based Planning for Spine Surgery,” Elsig and Kaech note
“The planning of decompressive and reconstructive spine surgery is based on clinical findings and diagnostic imaging. The evaluation of segmental instability, but also of the risk of destabilization following a needed decompression of the spinal canal and/or neural foramina make complex spine surgery a challenge, bearing in mind the risk of failures in case of an inadequate operation. The insufficient correlation between imaging and clinical symptoms originating from the spine and its nerve roots has been frustrating for some decades.”
They list several of the same problems with relying on traditional horizontal MRI and note the need for the use of the same modern imaging techniques to ensure successful outcomes for more patients overall. Problems which are only present in load bearing positions can easily be hidden in a patient laying on their back (2). Positioning can have dramatic outcomes on diagnosis.
Another study by Elsig and Kaech notes some of the
differences seen in patients in the horizontal position versus dynamic upright
MRI throughout the spine, noting an increase in positive outcomes for surgeons
who have this information prior to intervention:
A Postion-dependent appearance or increase of posterior disc protrusions, a varying degree of central canal and foraminal stenosis, and of mobile spinal instability was demonstrated in cases with preceding less remarkable or even negative recumbent MRI examinations.
A Type I Chiari Malformation, with positional increase of cerebellar tonsils downward herniation and brainstem compression was identified in a patient studied for a c5/6 degenerative disc disease. At this level an increased disc protrusion and segmental kyphosis was seen during upright imaging.
Other Issues Barring Positive Outcomes for CJD
Of course the failure to thrive post-operatively can’t
always be blamed on outdated imaging or surgical techniques. In the case of
Chiari I, researchers found “that abnormal changes in the DTI metrics in
patients with Chiari I malformation indicate microstructural abnormalities in
different brain regions that may be associated with neurocognitive
abnormalities (5).” In other words, in some cases, Chiari 1 malformation may
drive the development of microstructural abnormalities throughout the brain
that could be affecting patient symptoms.
Dr. Pellay notes in another study that “the presence of
syringomyelia implies a less favorable response to surgical intervention,” with
only 9 of 20 syringomyelia patients seeing significant post operative
improvements versus 13 out of 15 patients without syrinogomyelia seeing
significant improvement (6).
According to research, age can also have an effect on outcomes, which also seemed to hold true in my interviews. In the case of Graeson, whose Chiari I malformation wasn’t addressed until age 2 and a half due to one surgeon deciding his problems weren’t pronounced enough despite his CSF flow being completely blocked. Once the surgery was completed after his mother, Angela sought a second opinion. For Graeson, the outcome was good despite the delay. Now a year older, Graeson’s speech and swallowing problems have all disappeared. Angela’s advice to other mothers and patient who suspect chiari is to keep pushing to find a doctor who is truly knowledgeable of the condition.
Unfortunately, not all young Chiari patients see the same results. In the case of Tom*, a young man with chiari in the US, developed a CSF leak after his decompression surgery and contracted meningitis during a lumbar puncture. He must now undergo spinal taps every 6 months to relieve ongoing intracranial hypertension. A year after surgery, Tom describes a surreal feeling, as if waking from a fuzzy dream. His headaches remain and he is under pain management to try to keep them under control.
Conclusions and Further Considerations
There are many reasons which affect the level of access to the diagnosis and treatment of this condition, from proper training of the doctors doing these procedures, to the processes and standards set forth by the healthcare organization, to standards and regulations which bar the use of non-native diagnosis and testing. In addition, not all of the imaging techniques mentioned are widely available, which can create further barriers.
All of these obstacles to proper treatment affect the lives of people with CJD, from diagnosis to treatment, to overall successful outcomes after treatment. I continue this discussion in the final portion of this series in which I present information about several people with CJD in the United States and UK. That post is now available here: 7 People with Chiari and CCI Share Their Stories
Part I of our Chiari Malformation Awareness Month Series
It’s Chiari Malformation Awareness Month and time to put this important neurological condition in the spotlight. It’s a subject near and dear to many EDS zebras, as it is a common comorbid condition to the Ehlers-Danlos Syndromes. Chiari Malformation can also be congenital and is believed to be the widest known cause of CM today, however it is possible this may be a misconception due to the failure of many to recognize the role that hypermobility and craniocervical instability may well play in the development of Chiari later in life.
There are also a number of socio-political factors that
block the treatment and discovery of Chiari, which will be explored in the second
part of this two part series. Part two will also include the firsthand
perspectives and experiences of people who have a confirmed Chiari Malformation
(CM) diagnosis so we can get an intimate picture of what it’s like to live with
the condition, though it’s important to understand that like most chronic
illnesses, no two people seem to experience CM quite the same. In this first
post, we will walk through the basics of CM, learning what Chiari Malformation
is, the four known types and how they are classified. Of course no
informational post on a condition would be complete without exploring the known
causes, symptoms, and treatments.
What is a Chiari Malformation?
It is said that the Chiari Malformation affects 1 in 1,000 people: However, the increased use of diagnostic imaging has shown that Chiari malformation may be much more common(1). Chiari Malformations are structural defects located at the in the base of the skull where the spine meets it. These structural defects can cause the brain to sag into the spinal canal, causing herniation of a portion of the cerebellum, cerebellar tonsils and/or the fourth ventricle. Depending on the severity, there are a variety of symptoms and complications that come with Chiari Malformation.
The cerebellum is the part of the brain that controls
balance, memory, cognition and motor skills. Normally the cerebellum and
parts of the brain stem sit above an opening in the skull that allows the
spinal cord to pass through it. This is called the foramen magnum. In CM,
part of the cerebellum extends below the foramen magnum and into the upper
spinal canal, causing pressure on the cerebellum and brain stem that may affect
functions controlled by the cerebellum and block the flow of cerebrospinal fluid
Learn about Cerebrospinal Fluid and Lumbar Puncture: here.
There are four types of chiari malformation. According to
the American Association of Neurological Surgeons, they are classified in the
Type I occurs during fetal development and is characterized by downward displacement by more than four millimeters, of the cerebellar tonsils beneath the foramen magnum into the cervical spinal canal. Anomalies of the base of the skull and spine are seen in 30-50 percent of patients with Chiari I malformation.
Type I is also believed to include the formation of Chiari Malformation through Craniocervical Instability, where CM often meets with EDS or another Hypermobility Spectrum Disorder (HSD), such as JHS, Marfan Syndrome, or Loey-Dietz Syndrome. According to the Pain Relief Foundation, “Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to a pathological deformation of the brainstem, upper spinal cord, and cerebellum. It primarily occurs in patients with Ehlers-Danlos Syndrome and other hereditary disorders of connective tissue (4).”
The Ehlers-Danlos Society notes that we need to develop a
better understanding of the prevalence and variance of CM in EDS/HSD, along
with educating neurosurgeons on the role of hypermobility may play in chiari
Type II is characterized by downward displacement of the medulla, fourth ventricle and cerebellum into the cervical spinal canal, as well as elongation of the pons and fourth ventricle. This type occurs almost exclusively in patients with myelomeningocele, a congenital condition in which the spinal cord and column do not close properly during fetal development.
Type III includes a form of dysraphism (a spectrum of congenital anomalies resulting from incomplete or aberrant neural tube fusion, 3.) with a portion of the cerebellum and/or brainstem pushing out through a defect in the back of the head or neck. These malformations are very rare and are associated with a high early mortality rate or severe neurological deficits in patients that survive.
Type IV is the most severe form and the rarest. The cerebellum fails to develop normally. There may be other associated malformations of the brain and brainstem. Most babies born with this malformation do not survive infancy (2).
Symptoms Associated with Chiari Malformation
According to the NIH’s Chiari malformation Fact List, the hallmark sign of Chiari malformation is headache, especially after sudden coughing, sneezing, or straining. Other symptoms may vary among individuals:
hearing or balance problems
muscle weakness or numbness
difficulty swallowing or speaking
ringing or buzzing in the ears (tinnitus)
curvature of the spine (scoliosis)
problems with hand coordination and fine motor
Some individuals with CM may not show any symptoms and symptoms may change or fluctuate, depending on personal physiology, the compression of the tissue and nerves and on the buildup of CSF pressure.
Infants with Chiari malformation may experience the following:
irritability when being fed
a weak cry
gagging or vomiting
a stiff neck
inability to gain weight (1).
Of course, this is just a basic list of the most common symptoms of CM. As mentioned, it is possible for a Chiari malformation to be present, but completely asymptomatic (free of any noticeable symptoms). In these patients, the condition is usually discovered through MRI taken for unrelated reasons. However, patients may experience symptoms in the future. It has also been known for symptoms to fluctuate over time.
As the condition
progresses, there are many comorbid conditions that can develop with CM and
different types have different symptoms. According to the NIH, common comorbid
Hydrocephalus is an excessive buildup of CSF in the brain. A CM can block the normal flow of this fluid and cause pressure within the head that can result in mental defects and/or an enlarged or misshapen skull. Severe hydrocephalus, if left untreated, can be fatal. The disorder can occur with any type of Chiari malformation, but is most commonly associated with Type II.
Spina bifida is the incomplete closing of the backbone and membranes around the spinal cord. In babies with spina bifida, the bones around the spinal cord do not form properly, causing defects in the lower spine. While most children with this birth defect have such a mild form that they have no neurological problems, individuals with Type II Chiari malformation usually have myelomeningocele, and a baby’s spinal cord remains open in one area of the back and lower spine. The membranes and spinal cord protrude through the opening in the spine, creating a sac on the baby’s back. This can cause a number of neurological impairments such as muscle weakness, paralysis, and scoliosis.
Syringomyelia is a disorder in which a CSF-filled tubular cyst, or syrinx, forms within the spinal cord’s central canal. The growing syrinx destroys the center of the spinal cord, resulting in pain, weakness, and stiffness in the back, shoulders, arms, or legs. Other symptoms may include a loss of the ability to feel extremes of hot or cold, especially in the hands. Some individuals also have severe arm and neck pain.
Tethered cord syndrome occurs when a child’s spinal cord abnormally attaches to the tissues around the bottom of the spine. This means the spinal cord cannot move freely within the spinal canal. As a child grows, the disorder worsens, and can result in permanent damage to the nerves that control the muscles in the lower body and legs. Children who have a myelomeningocele have an increased risk of developing a tethered cord later in life.
Spinal curvature is common among individuals with syringomyelia or CM Type I. The spine either may bend to the left or right (scoliosis) or may bend forward (kyphosis).
Diagnosis of Chiari Malformation
CM is typically diagnosed by a neurologist, preferrably one with an expertise in treating CM. The doctor will perform a physical exam and check the person’s memory, cognition, balance (functions controlled by the cerebellum), touch, reflexes, sensation, and motor skills (functions controlled by the spinal cord). The physician may also order an MRI, X-ray or CT. The most common imaging used is MRI. There are problems with diagnosing Chiari through the use of traditional supine positioning: However, this will be covered in our next post in this series.
Other tests the doctor might order,
according to the AANS, include sleep or swallowing studies, a BAER test,
myleogram, or SSEP (2).
Chiari Malformation Treatment
In the case of asymptomatic patients, no intervention is needed. However, monitoring of the patient, including regular check-ups, is usually recommended. In many cases, Chiari Malformation symptoms and disease progress may only be relieved or halted through surgical intervention. According to the NIH, these procedures are as follows:
The most common surgery to treat Chiari malformation is posterior fossa decompression (PFD). In the case of type II CM, a PFD is usually followed by a spinal fusion, where hardware is placed to create a supportive structure for the head and neck. This creates more space for the cerebellum and relieves pressure on the spinal cord.
The surgery involves making an incision at the back of the head and removing a small portion of the bone at the bottom of the skull. This procedure is known as a craniectomy. In some cases the arched, bony roof of the spinal canal, called the lamina, may also be removed to create sufficient space for the cerebellum. This procedure is referred to as a spinal laminectomy. The surgery should help restore the normal flow of CSF, and in some cases it may be enough to relieve symptoms while other patients show no improvement or even a worsening of post-operative symptoms.
Next, the surgeon may make an incision in the
dura, the protective covering of the brain and spinal cord. Some surgeons
perform a Doppler ultrasound test during surgery to determine if opening the
dura is even necessary. If the brain and spinal cord area is still
crowded, the surgeon may use a procedure called electrocautery to remove the
cerebellar tonsils, allowing for more free space. These tonsils do not
have a recognized function and can be removed without causing any known
The final step is to sew a dura patch to expand the space around the tonsils, similar to letting out the waistband on a pair of pants. This patch can be made of artificial material or tissue harvested from another part of an individual’s body.
Infants and children with myelomeningocele may require surgery to reposition the spinal cord and close the opening in the back. Findings from the National Institutes of Health (NIH) show that this surgery is most effective when it is done prenatally (while the baby is still in the womb) instead of after birth. The prenatal surgery reduces the occurrence of hydrocephalus and restores the cerebellum and brain stem to a more normal alignment.
Hydrocephalus may be treated with a shunt or tube that drains excess fluid and relieves pressure inside the head. These tubes drain the excess fluid into either the chest cavity or the abdomen so it can be absorbed and disposed of by the body.
An alternative surgical treatment in some individuals with hydrocephalus is third ventriculostomy, a procedure used to improve the flow of CSF. A small hole is made at the bottom of the brain cavity, diverting the CSF flow to relieve pressure. In cases where surgery was not effective, doctors may open the spinal cord and insert a shunt to drain a syringomyelia or hydromyelia, which is increased fluid in the central canal of the spinal cord (1).
Today, we learned about what Chiari malformation is, the four types and their symptoms, diagnosis and surgical interventions. The next post in this series on CM will explore the outcomes for patients with Chiari Malformation, along with the socio-economic impacts that affect receiving treatment of this complex disorder in both private and public healthcare settings. It’s now available here: The Issues of Diagnosis and Treatment of Chiari and Craniocervical Instability
Then our final post will include interviews by people who have either received, or are awaiting treatment along with information about another group of patients, who strongly suspect CM, but cannot even get the proper the imaging for confirmation due to the strictures these socio-economic factors create. It’s now available here: 7 People with Chiari and CCI Share Their Stories
Let’s start the conversation early! Are you one of the suspected undiagnosed? Have you been diagnosed and/or treated? Let us know your story in the comments! And as always, thanks for joining us for this educational post about Chiari malformation.
This site is not intended to replace the medical advice of a trained medical professional. Readers are advised to consult a physician or other qualified health care professional regarding treatment of their medical problems. The author disclaims liability, loss, or risk, personal or otherwise, which is incurred as a consequence, directly or indirectly, of the use or application of any of the contents of this site or its articles.