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7 People with Chiari and CCI Share Their Stories

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.

7 people share their journey with CCI, or Chiari Malformation, from diagnosis and treatment, to symptoms and barriers to care. A special Zebra Pit series in honor of Chiari Awareness Month

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.

Name: Jackie

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.

Name: Christina

Location: South East England

Time of Symptom Onset to Diagnosis: Unsure.

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.

Name: Maura

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.

Name: Kaitlyn

Location: Worthington, KY

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.”

Kaitlyn 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.”

Name: Martha

Location: Kent, England, UK

Time 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.

Name: Jennifer

Location: Fairhope, AL

Time of Symptom Onset to Diagnosis: 15 months

Jennifer 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.”

7 people share their journey with CCI, or Chiari Malformation, from diagnosis and treatment, to symptoms and barriers to care. A special Zebra Pit series in honor of Chiari Awareness Month

Since Jennifer has given up on surgical intervention, she lives a different sort of life now.

“When 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.

“My 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.”

Name: Rose*

Location: Chicago, IL

Time 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.

Name: Michelle

Location: Cincinnati, Ohio

Time 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.

7 people share their journey with CCI, or Chiari Malformation, from diagnosis and treatment, to symptoms and barriers to care. A special Zebra Pit series in honor of Chiari Awareness Month

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 a shunt.

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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:

Resources:

All interviews were conducted through internet correspondence from September 5-13, 2019.

An eighth story is also included, one of a person who can't achieve diagnosis for clear cut symptoms of a craniocervical junction disorder and EDS, myself. Learn what factors prevent diagnosis and how people with Chiari and CCI live before and after surgical intervention of their condition.

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The Issues of Diagnosis and Treatment of Chiari and Craniocervical Instability

*The names of some subjects interviewed for the post have been changed to protect their privacy. These changes are denoted by an asterisk (*).

Chiari Malformation Awareness Ribbon displayed on "The Issues of Diagnosis and Treatement of Chiari and Craniocervical Instability, an investigation in the barriers of access for patients.
September is Chiari Awareness Month

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

A comprehensive investigation on why many patients with CCI, Chiari Malformation and other CJDs are being left with no where to turn.

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.

(1)

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.

(1)

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.

Traditional MRI may be the least helpful type in diagnosing CJD, while uprightand dynamic MRIs are the preferred methods of imaging among top experts.

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.”

(2)

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.

(4)

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

Craniocervical Instability Awareness Image displayed on "The Issues of Diagnosis and Treatement of Chiari and Craniocervical Instability, an investigation in the barriers of access for patients.

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

Resources and Related materials

  1. Goel, Atul, et al. “Basilar invagination, Chiari malformation, syringomyelia: A review.” Neurology India.
  2. Elsig, Jean Pierre and Denis Kaech. “Imaging Based Planning for Spine Surgery.” Taylor & Francis Online.
  3. “Basal Invagination.” The Spine Hospital at the Neurological Institute of New York, Columbia Spine.
  4.  Elsig, Jean Pierre and Denis Kaech. “Dynamic Imaging of the Spine with an Open Upright MRI: Present Results and Future Perspectives of FMRI.”
  5. Kumar, Manoj, et al. “Correlation of Diffusion Tensor Imaging Metrics with Neurocognitive Function in Chiari I Malformation.” Science Direct.
  6. Pellay, Prem K, et al. “Symptomatic Chiari Malformation in Adults: A New Classification Based on Magnetic Resonance Imaging with Clinical and Prognostic Significance.” Neurosurgery; Congress of Neurological Surgeons.
  7. Mossman, SS, et al. “Convergence Nystigmus Associated with Arnold-Chiari Malformation.” JAMA Neurology.
  8. Furtado, Sunhil. “Correlation of Functional Outcome and Natural History with Clinicoradiological Factors in Surgically Managed Pediatric Chiari I Malformation.Neurosurgery; Congress of Neurological Surgeons.
  9. All interviews were conducted through internet correspondence from September 5-11, 2019.

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An exploration of the barriers to the diagnosis and treatment of craniocervical junction disorders such as chiari malformation  and craniocervical instability (CCI) for patients worldwide in both public and private healthcare systems in the US, Canada and the UK. Includes a review of current literature on treatment & dx, plus patient interviews in this awareness month investigation.
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Guest Post: ME/CFS, POTS, Fibromyalgia, Hypermobility, CCI. What’s the connection and is science missing it?

Please help me offer Tracy Hagler a warm and friendly dazzle welcome! Tracy is the author of today’s guest post. A nurse with many of the same conditions as myself, Tracy and I were admiring each other’s work and decided we had to do a blog swap. In this piece, she writes about the onset of her conditions and the interconnected nature of conditions like ME/CFS, Fibromyalgia, HSD/EDS, CCI, along with some studies delving into these connections. I think you’ll enjoy it and find some similarities in our theories! After you read her post, be sure to go and give her blog a like so you can keep reading her posts! There’s a link available below, in her bio.

Without further ado, here is Tracy’s post, originally run on Unspoken Words of the Heart on 7/2/19.

ME/CFS, POTS, Fibromyalgia, Hypermobility, CCI. What’s the connection and is science missing it?

I know I have quite a few readers who battle with me/cfs, pots syndrome, fibromyalgia or Hypermobility Syndrome/EDS. Although, this is a blog to share my faith, I also want to share health updates and things that I’m learning so that we can educate one another.

The one thing I can look back for sure and see is that I’ve always had a very sensitive nervous system, I just didn’t realize it at the time. I was overly sensitive to medications and caffeine. Other people could drink several cups of coffee per day and do fine but a few sips, for me, would make me crazy. From twenty years old and on I had GERD and always reacted to gluten and sugars. I just felt bad after eating them. I just thought all of this was “normal”, at the time. I was always a type A personality. I was a nurse and I worked abnormal crazy hours (16 hour shifts, swing shifts, etc.) When I had time to sit down, well I never would. I always felt tired but wired up, if that makes any sense. I had been working as a nurse for about eight years before I became sick. The year I got sick (2017) was a busy, exhausting year for us (mentally & physically). My fiancé’s Dad passed away In February. We got married in July and my younger brother passed away in a car accident in August. In July I started having a severe pain that wrapped around my hip and into my inner thigh. I couldn’t stand, sit or walk without being in severe pain. It was actually a deep hidden inguinal hernia, with my small bowel protruding through. The hernia was pinching my inguinal and obturator nerves. (We have all heard of sciatica nerve pain, well this is the same excruciating pain only it involves different nerves.) They didn’t find out that it was a hernia until almost a year later. I was out of work and could barely do much due to the pain. (Please keep reading, I promise I am going somewhere with this story.) I had no clue what was wrong.

I went to a chiropractor for a spine adjustment because at the time no one could figure out that I had a hernia. Everyone presumed that the pain was from, possibly, a pinched nerve in my back. I go to get the adjustment and he uses an adjustment gun on my back and neck and does some deep tissue massage. He worked on me for thirty minutes. I was desperate to get back to work and wanted this severe pain to go away. What I didn’t realize is that my life was going to take a drastic change that night. I wish I could go back and change that day but I can’t. That night I awoke with my spine and neck burning hot and they felt inflamed. I was vomiting, my whole body felt feverish and I was chilling. The next day, I awoke unable to stand up. When I would stand my heart rate would shoot up. I had massive headaches in the back of my head every time I would stand for long periods of time. I couldn’t eat anything and had extreme nausea. I would lose 10 lbs over the next couple of weeks. I called the chiropractor and told him the extreme symptoms and he said I had anxiety and to come in for my second adjustment. That made me mad because it was obvious that something was severely and physically wrong with me and I only wished it was anxiety. But crazy me went back thinking he could fix what had happened. This second adjustment only worsened my condition and I ended up at my moms for a week unable to get up or eat. I thought I might die that week and no one would ever know why it happened. I truly didn’t think I would make it. By this time people probably didn’t know what to think of me. I couldn’t go back to work. I could barely make it to the bathroom. No one had ever heard of an adjustment doing this to someone. But what I have found is that it’s actually happened to a lot of people. They call it “toxic reaction”. But I never got over it.

I developed pots syndrome and months later would come to find out I had me/cfs. Over the next few months, I found that exertion would send me into severe crashes with severe symptoms. Everything was post, several days after an activity. I could see a pattern. I would wake up gasping for air at night, I had air hunger, my nail beds were cyanotic. I had severe orthostatic intolerance (pots), severe heat intolerance, blurred vision, it felt like I had the flu times ten. My spine was sore to touch. The skin on my back felt sunburned and I couldn’t stand the slightest touch. I couldn’t look at lights or phones or tv. I just laid there in complete darkness and felt close to death. I was then diagnosed with me/cfs, fibromyalgia and hypermobility (People with Hypermobility Syndrome or EDS have thinner tissues and often times have hernias at a younger age, which also helps to explain my hernia issues).

I tell this long story because it all makes sense looking back. It seemed like a perfect storm. I do feel like my body had a strong reaction to the adjustment and inflammation set up in my spine that night. It was like my body overreacted and attacked me in order to protect itself (kind of like an autoimmune response). During my sickest times, I had chronic UTI infections that antibiotics did nothing for (8 to be exact). Later they said I had Interstitial Cystitis which can be set off by mast cell activation. I started taking Benadryl nightly and it helped decrease the pain of the Interstitial Cystitis. They checked me for mast cell activation and it was negative but at the time I was taking a daily antihistamine. So I’m thinking that might be why it was negative.

This illness definitely originates, in my opinion, in the spine, brain stem and at the base of the skull. I always feel so much pressure and swelling there especially during a crash. I love to write and sometimes just the effort required for that will cause a crash. This really is a cruel disease with severe suffering (which I am happy to say, has only made me a stronger person). I keep putting my story out there because I’m a minority where my me/cfs was caused by something other than a virus. Although, I have heard of spine adjustments triggering or reactivating viruses in the body. I was checked for EBV and they just said it wasn’t currently active but I had been exposed to it in the past. I also have heard of people getting sick from deep tissue massages because it releases toxins from the tissues into the body. Studies are also finding dorsal root ganglionitis in the brains and spines of those suffering with severe me/cfs, postmortem.

I’m not exactly sure what happened to me that day. I’ve got a few theories. However, the one thing I do know is we all have the same illness. Where science has gotten it wrong for so long, they seem to be on the right track, for the moment at least. The recent stories of people recovering from Me/cfs and Pots after having craniocervical instability surgery (a surgery of the neck) has left me with a lot of questions. Especially, since my me/cfs was triggered by a neck adjustment. It has been found that people with hypermobility or EDS can have laxity in the ligaments that hold the skull up causing instability and pressure on the brain stem therefore causing POTS, ME/CFS and Fibromyalgia. There have been several people that have had CCI surgery done and all their pots and me/cfs symptoms have disappeared. Jennifer Brea, the producer of the Netflix documentary film “Unrest”, also had severe Pots and Me/cfs and is now in a complete remission after CCI surgery. Her Me/cfs was triggered by a virus/infection.

In my case, was I a ticking time bomb? Did it just take one or two more millimeters of instability at the base of my skull to cause me to develop Me/cfs, Pots and fibromyalgia? Or, did my spine set up inflammation that led to dorsal root ganglionitis? I may never know but I will never give up hope that I WILL someday get better. My trigger was a chiropractic adjustment. Your trigger may have been a virus, a concussion or head trauma. All the science behind this is kind of intriguing and it starts to come together after studying on it for a while. It’s like five separate illnesses operating under one specific cause. If we keep telling our stories, hopefully it will help science fit the pieces of the puzzle together and they can find out the cause and develop a cure. Most importantly if you have this illness, don’t give up, there’s still hope. We have a God who is greater than science. Let’s continue to support and lift one another up in prayer!

(This is in no way medical advice and should only be taken as part of my story and personal opinions.)

Resources


Tracy Hagler is a nurse who was diagnosed with ME/CFS, POTS, Fibromyalgia and Hypermobility Syndrome within the past two years.  She started writing a  blog where women can share faith, hope and encouragement. She has become an advocate for theses conditions and shares information and education about them. She also writes on mental health and her Christian Faith. You can check out her blog at Unspoken Words of the Heart.


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In this guest post, Tracey Hagler explores the connections between ME/CFS, POTS, Fibromyalgia, CCI, and Hypermobillity Spectrum Disorders. She talks about the progression and diagnoses of her own conditions, some compelling research and more to put together this thought-provoking post about the interrelated nature of these illnesses.
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Traction Collars & the War Against Neck Pain

Please note this post contains affiliate links. Thank you.


Spinal problems never run on short supply for a person with Ehlers-Danlos Syndrome (EDS) or another Hypermobility Syndrome Disorder (HSD), conditions which cause a person to produce weak, faulty collagen. Collagen is often described as the glue that holds our bodies together. Over 80% of our bodily tissues are comprised of collagen,from our skin, hair, nails and joints, to even our bones, organs, and muscles. Everything has some collagen in it. Our spinal columns are comprised of many tiny bones which are stabilized and cushioned by collagenous formations known as discs and the whole Jenga party is kept together by various connective tissues (see the diagram to the left). All of these structures are comprised of… you guessed it: Collagen. In people with EDS/HSD, naturally these structures will be weak and prone to early degeneration, leading to a life of instability and easy injury, chronically dislocating vertebrae that pinch nerves, impinge the natural movement of fascia and other soft tissues, abnormal bone growth that cause tears in the surrounding tissue and even a collapse of the structures which hold the skull in place at the top 3 of the spine, creating craniocervical instability and chiari malformations.

Wins and Losses in the Jenga War

My spine began breaking down quite young and I suffered back pain regularly as a teen. I herniated my first disc as a nineteen year old working as a certified nurse’s aide in a full service residential program. I lifted a patient and nearly ended up on the floor, crippled by pain.

I was told by the urgent care doctor they sent me to that I was young. I couldn’t possibly have done any damage to my spine. I must have just torn the muscles on both sides of my spine. He gave me pain pills, an order to rest for a week and sent on my way. It took 4 weeks before I could even think about getting back on my feet. I tried returning to my job 3 times, only to realize my career in nursing was clearly over, as any little thing now re-injured my back and doing my nursely duties was no longer feasible.

It started an avalanche of back problems that would be occasionally medicated and rehabbed, but never imaged or properly examined to see why I was in so much pain until I was in my thirties. By then, I had herniated discs and signs of deterioration throughout my entire spine that had been repeatedly ignore because I was “too young.”

The fact that pain in young people, most especially young women and women of color, who face constant racial bias and neglect from our healthcare system, is a major sticking point for me. I may never stop rehashing how neglected I was by the medical community or those closest to me who couldn’t bother to support me enough to believe in or fight for what I needed in those early years. I could be living a different life had a single person taken me by the hand and said “I believe you. Let’s see if we can get to the bottom of this.” We have to keep voicing these things until the day the problem no longer exists.

These problems are ten fold when you have a chronic illness. So here I am, sharing products like this one and talking to other women about ways of healing, because the system that’s supposed to help us is failing. While I personally have better doctors than ever before, they still aren’t trained well enough to evaluate me properly for craniocervical instability or Chiari malformation, even though they know I’m an excellent candidate for these conditions. I don’t even lived in an isolated rural area. Kentucky may sound rural, but in reality, I live 15 minutes from Cincinnati, Ohio where there’s a very good medical teaching university.

I don’t have the answers to these complex problems other than to do my best to learn for myself and care for this wacky Ehlers Danlos body to the best of my own abilities, relying on the doctors I do have to fill in the gaps when I need them.

Adding a New Weapon to My Arsenal

Why yes, that is a great shirt I’m wearing. Thank you!

When I run into problems like my cervical spine popping out of place for several weeks, I don’t go to the doctor anymore unless I feel certain the pain is new or very different. I get started on my own rehab. I won’t lie, the pain was a zebra level 10 and there were days I’d have been thoroughly satisfied if I could just behead myself and be done with it, but I made it through. I made it through because I have tools like the fasciablaster, an ultra cavitation machine with far infrared (more info coming soon), AloeMD, epsom salt baths, physical therapy exercises, rest, ice, muscle relaxers and more Advil than I care to admit to (max doses only, no worries), but sometimes we have to compromise to make life more bearable. Why bother the doctor or go through the pain of leaving the house when I’ve already learned what I need to rehab my neck myself? If your answer was pain pills, let me assure that he would neither offer them nor could I take them because of severe MCAS reactions to codeine and opioids.

Knowing that this is a chronic lifetime condition with no real solution, I’m always looking for the best new solutions to address my pain and keep what mobility I have. I decided that while I’m not crazy about braces, it’s time to begin using one to support my head and neck during strenuous upright activity or when I’m going to be bending over a task for some time.

The Branfit Neck Traction Device

I chose this inflatable traction style collar because I felt the way it worked would offer the most support for my head and neck while up and around. Turns out it was a good choice. It definitely fully supports the head and helps to position the neck properly, all without causing any pain. It’s also incredibly affordable. Before you celebrate by popping one in your Amazon cart, let me cover a couple of caveats for this particular item.

First, I can’t guarantee this is the exact collar I bought, but it looks and is described in exactly the same way and even has the same air control and deflation valves. The collar I actually bought came with an overseas shipping nightmare and price tag no one experiencing severe pain should have to pay, so I found this one on Amazon that appears to be the same and ships quickly. Often we need these things fast, not in 6 weeks! It’s also $10 less (no more impulse buys on Instagram for me) and for whatever it’s worth, it’s FDA approved.

Shows a woman holding her neck in pain. Overlay text says "Branfit Neck Traction Device: Fixes posture, relieves stiff neck pain, relaxes neck and shoulders, restores natural cervical curve, headache and migraine relief, supports back and spinal decrompression.

This style of collar does what it’s supposed to great, but you may experience some of the frustrations I see in it. For one, when it arrived, as very strong chemical smell came wafting from the package and I quickly developed a migraine. I put it out on my porch a couple of days and it really seemed to help. After it was unboxed about a week, I no longer needed to wear my mask and the odor was no longer apparent.

The other thing about it is that whatever poly/vinyl material the collar is made of has to be airtight so it can be inflated, so of course it’s a bit like wearing pleather; there’s no breathability to the material wrapping your neck so you end up pretty hot and sweaty under the collar. If you have heat intolerance, you’ll probably see this as a drawback, like me. If you’re always looking for your sweater, it could be an advantage.

On the claustrophobia front, it presses a tiny bit on the jugular and can make you feel a bit uncomfortable at first, but it’s only Velcro straps that hold it in place, so you can be out of it in less than a second. Not only is this great for anxiety or PTSD issues, it’s necessary in case anything were to go wrong and you need to take it off fast.

The thing about braces of any kind of course, is that they shouldn’t be worn excessively, so I actually think this will work just fine. Now that my neck is almost back to it’s normal state, I will only use this brace if I’m going to be doing something for an extended period of time that forces my neck outside of the neutral position. Most importantly, I’m working out my neck and shoulders so they can grow stronger and do the work themselves. That way, I only have to go into my brace during injury flares. Never go into a brace without a definitive plan of how to cut your dependence on it, no matter the path to healing you choose.

At this point there isn’t much I can or am prepared to do about my neck regarding surgical measures. Even though I had a wonderfully successful surgery on my low back, I want to delay surgery on my neck for as long as possible. Surgery can be helpful, but it can also snowball into more problems and yet more surgery, or even worse symptoms than previous. My symptoms are neither life threatening nor debilitating with the exception of occasional flares like this one, so I would refuse surgery anyway.

In light if this, it doesn’t really make sense to push to see other specialists. My fascia work and strengthening usually get me by. My migraines are now very well controlled. The problem with my neck  this time was probably the combination of a mutli-condition flare while also being off my usual exercise routine during my heart issues; this is exactly why I preach reducing workouts over skipping them on bad days. Unfortunately, my buddies anxiety and depression had other thoughts about what we should be doing. We all have our bad moments, even preachy health bloggers.

I may eventually look for a cooler option in braces for the summer months and keep this for the rest of the year, but for now, I think this collar will work just fine to support my head during moments of vulnerability while I try to stretch any possible surgeries as far into the future as I may.

If you decide to purchase a collar like this or already have one, I would love to hear how yours is working out for you. Alternatively, if you have another to recommend, we’re all ears! 😊

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Neck Strengthening Exercises

These resistance exercises for the neck are so simple, I had a tough time conveying the concept. Basically, you’re going to push against your head in order to create resistance; something for your neck to have to fight against. You can’t really see it in the video, because you’re not supposed to move your head, you just meet your hand with equal resistance. It should remain in basically the same position as you resist the force of your hand. You want to do these exercises coming from all four directions, both sides, front and back. Keep your hand position toward the top of the skull on each side. Your hand should be positioned on the center of your forehead when facing the front, so the pressure is high on your skull, but well balanced. On the sides of the head, your hands should be slightly in front of and above your ears. In the back, it should be a mirror of the positioning on your forehead.  It doesn’t really matter if you do a whole set before moving to another side or one repetition; just do what’s most comfortable for you and gets you in a good rhythm. They will help you to gradually build up the muscles in your neck. You can hold them beginning at a count of three as I do in this video and work your way up to a longer hold and more repetitions. You should not experience any pain with this exercise. If you do, you may be exerting too much pressure and need to ease up. You may also benefit from more magnesium in your diet and/or myofascial therapy. if you have persistently sore muscles.

I look at these exercises as a way to maintain a healthy neck. If you already have neck issues that you’re struggling with, seek a therapist and imagining, if necessary. I’ve gone through five or six different head and neck therapies and there’s one I felt was most effective, but of course I’ve lost all the information I had on it and haven’t seen the practitioner for years. I had terrible neck pain for the longest time, but never could quite determine how much was attributed to coat hanger headaches and how much was the damage to my cervical spine caused by my degenerative disc disease. I do have instability in my neck from a couple of herniated discs, but once I got my POTS under control and had been working on  my head, neck and shoulders by fasciablasting them for about a year, my neck pain resolved, with the exception of the occasional twinge.

The important things to remember during this exercise is that you maintain good posture while doing it, try not to hyper extend or overexert your neck by pushing too hard or doing too many reps, and be sure to keep your movements slow, measured and steady. Jerking movements to the neck can be damaging and of course that’s exactly what we want to avoid. Since you can vary the amount of pressure, number of repetitions and length of time you hold the movement, it’s easy to increase the challenge of this exercise. Finally, in my opinion these exercises are safer than flexion and extension exercises for zebras, which primarily involve stretching and could damage our already fragile collagen.

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