CNN Article on Ron Davis (and Family) and His ME/CFS Research — May 12, 2019

https://edition.cnn.com/2019/05/12/...cist-chronic-fatigue-syndrome-trnd/index.html

He pioneered technology that fueled the Human Genome Project. Now his greatest challenge is curing his own son.

 

Jennifer Brea, who directed the Sundance documentary UNREST and co-founded #MEAction, posted that all her ME symptoms are in remission!

"And now, thanks to three spinal surgeries — one for something called craniocervical instability and the others for tethered cord syndrome — I believe I am on my way to a full recovery."

Health update #3: My ME is in remission

 

Hi @Autumnly -- This story has created quite a buzz in the ME/CFS community, of which I'm (unfortunately) a part of. I've debated whether to start a thread on this forum on craniocervical instability (CCI), as I feel certain many here have it as at least a cofactor in their tinnitus. I definitely feel its a cofactor for me, as well as it likely being the primary cause of my own ME/CFS.

I assume CCI as a cause of tinnitus would normally fall under the category of "somatic tinnitus", but it's really in a league of its own, owing to the severity of symptoms it can cause--including ear issues. Unfortunately, there's a general lack of awareness about it, including by professionally neurologists. @Greg Sacramento -- Another person with severe ME/CFS was able to get corrective surgery for CCI, and completely recoverd as well.

But the battle he had with his neurosurgeons to recognize his situation is almost unbelievable. If you're up for one of the most compelling health stories I've ever run across, here's a LINK to HIS WEBSITE about his long odyssey. It's about 10-12 pages long. The first half is mostly about his ME/CFS experiences, and the second half is mostly about his CCI discoveries. -- I'll paste his whole story below, as I feel it's easier to read than on his website.

...........................................................................

Cerviocervial Instability (CCI) & Atlantoaxial Instability (AAI) Story – 2018
—ME/CFS Portion in First Five Pages – CCI/AAI Story the Second Five Pages— ​

Hi, I'm Jeff. Four years ago, I became ill with severe ME (Myalgic Encephalomyelitis). My story is in many ways typical: I was a healthy, active individual who became ill after an acute viral infection and a fever of 102F. After the acute viral symptoms subsided, I didn’t regain my health. Instead, I became far more ill than I could have imagined.

What is not typical in my case: I ultimately discovered the underlying cause of my severe ME. I was extremely lucky. As a graduate student trained in research, I was comfortable navigating peer-reviewed academic medical journals. I figured out what was causing my ME through persistence, sheer luck, years of research, and after becoming so ill that I nearly died. By addressing the cause of my illness, I have made significant improvements in my well-being.

I believe that it is possible to develop the hallmark ME symptoms, as described in the International Consensus Criteria (ICC) and Ramsay’s Definition, through more than one pathway. People with ME probably fit into multiple categories of underlying causes. I don’t think that my own underlying cause of ME is unique to me – it may actually represent a significant subset of ME patients. Whether that subset is small or is actually the tip of the iceberg of a much larger, unrecognized phenomenon remains unknown as I write this.

By the end of my story, you might find yourself having many questions. I hope you will keep a curious and open mind as you digest it. You are welcome to reach out to me with your thoughts. We can also have a public Twitter dialogue @jeff_says_that. – I’m sharing my story in the hopes that it can help others recognize if they are a part of my ME subset. I want others to have access to the diagnostic and treatment options that I believe saved my life – and that are restoring my quality of life. – My story:

My life became an ongoing negotiation of energy tradeoffs. – After the acute viral symptoms subsided, I still had headaches, dizziness, weak legs, and a general sense of being worn down. I initially had no reason to believe this state would become chronic, and not understanding the harm of exertion in those early days, I continued pressing forward with my graduate studies.

At first, I was still able to attend grad school, but keeping up with school and life took enormous effort. I had to stop all physical exercise. I could no longer lift weights or jog without days of payback in the form of a deep exhaustion accompanied by cognitive slowing and dulling.

I also had to begin giving up my social life outside of school. The energy expended during just a few hours of socializing left me physically exhausted and cognitively impaired for days. After giving up all outside activities, I was able to successfully attend grad school, albeit with great struggle. As time went on, I became progressively more debilitated.

I consulted numerous doctors, undergoing extensive testing by endocrinologists, internists, and other specialists. When the doctors found no abnormalities, they suggested I was probably anxious or depressed. I rejected this possibility entirely, based on my real physical symptoms. – Still not fully grasping the danger of exertion, I continued to push my energy limits. I ultimately became bedridden. I had no choice but to take a leave of absence from grad school.

From my bed, using my research training, I began an intensive investigation into what this illness might be, devouring online peer-reviewed academic medical journal articles. The viral onset, the faint feeling while standing, light and sound sensitivity, muscle weakness and pain, the exhaustion that inevitably followed activity, the cognitive impairment – all painted a clear picture of Myalgic Encephalomyelitis (ME). My symptoms exactly matched the International Consensus Criteria (ICC) for ME. My symptoms, including their timing and onset, also fit the (much older) Ramsay Definition of ME.

My relief at finding a name for my condition was overshadowed by everything else I read about ME: The illness is usually lifelong, with no known cure. The vast majority of people never recover. – I saw only an abyss before me and couldn’t imagine a viable future for myself. I grieved. – But! I finally had a name for my condition, a necessary starting point for action. From my bed, once again, I began online research. This time, I was searching for a doctor who specialized in ME patients.

I found Dr. David Kaufman, a board certified internist specializing in ME. He officially diagnosed me with ME as well as a form of dysautonomia known as Postural Orthostatic Tachycardia Syndrome (POTS). He found that when I would sit up, my heart rate would rise to the 100’s. When I would stand, my heart rate would further elevate into the 140’s. He also found high Epstein-Barr viral titers, both IgM and IgG, as well as extremely low natural killer cell function.

Dr. Kaufman said four words that I will never forget: “You are very sick.” This was the very first acknowledgement – by any doctor – that I was truly sick. When I became exhausted and had to lie down just 10 minutes into my appt., Dr. Kaufman understood. He was very kind and intellectually curious. What I didn't yet realize was how important an advocate he would become for me.

My next step was to find community. I joined the Phoenix Rising online forum for people with ME. I found thousands of people struggling as I was, with varying degrees of severity. Some were still able to work or go to school, others were housebound, while others were bedridden – as debilitated as I was. Some were even worse. – I marveled at how we all paid a steep price for engaging in minimal activity in the form of an energy “crash.” I was especially struck by how poorly we had been treated by doctors.

These online ME communities seemed to be the only places where someone could write “I finally had enough energy to wash my hair today, for the first time this month. I exceeded my energy envelope and will pay for washing my hair tomorrow and for the next week.” People in the ME community understand the energy crash that inevitably happens after activity. This energy crash is technically called Post-Exertional Malaise (PEM), but "malaise" doesn't fully capture the extreme exhaustion. PEM is devastating. PEM is very real. And yet, we are routinely disbelieved and ridiculed by the medical community.

The PEM crash was the most disabling and horrifying symptom for me. It seemed to be a particularly cruel form of payback for simply engaging in activity. PEM would at times leave me confined to my bed in a dark room, unable to speak or brush my teeth for weeks on end.

My life remained an ongoing negotiation of energy tradeoffs.

Each day, I woke up with bone-crushing exhaustion. I then asked myself while lying in bed: “Do I shower today, or do I speak to a friend on the phone for 15 minutes?” I couldn’t do both. Trying to do two tasks in one day would result in days of payback, PEM, with exhaustion so severe that I would be unable to leave my bed. – I became very sensitive to light and sound. Being subjected to normal amounts of light and sound felt physically painful and would send me crashing for days. I began wearing earplugs, and my family took care to keep noise to a minimum. I could no longer tolerate overhead lighting, so my family installed a low watt lamp, within reach, right by my bedside. The curtains remained closed at all times.

There were stretches of months that I could not even walk from one room to another within my home. In order to bathe, I had to be wheeled into the shower in my wheelchair and have others do the labor of bathing me. Just the act of being moved from my bed and bathed would result in days of payback, a PEM crash, with profound exhaustion, a near inability to move, and severely diminished cognitive ability. I also began having allergic-type reactions throughout the day, and Dr. Kaufman diagnosed and began treating me for Mast Cell Activation Syndrome (MCAS) – on top of both ME and POTS.

Most frightening was the realization that if I over-extended myself, I would inevitably descend into a more severe state of disability. These sharp declines lasted several months or longer. – The profound exertion-induced crashes happened multiple times, despite my best efforts to prevent them. During these crashes, I became not only bedbound – I also became no longer able to eat. I could not muster the energy to feed myself or to chew food. I had to drink protein shakes though a straw, fortified with spoonfuls of olive oil to maximize the calories. Despite my efforts to remain nourished, I went from 145 lbs to 117 lbs. – During these declines, I at times became entirely unable to communicate. I could not speak or write. I just couldn’t summon the energy. I was trapped.

Over time, I learned that the only way to come out of these crashes was to lie perfectly still, 24/7, in a completely dark and silent room. I couldn’t push myself at all, even to speak or eat, or else I would remain crashed. I began to carefully budget my activity level in order to have enough energy to go online. I had to figure out this illness. I again began pouring over peer-reviewed academic articles, this time seeking to figure out the potential mechanism of ME.

I found multiple avenues of exploration: the mitochondria, the microbiome, the viral/immunological, the neuroinflammation… So many possibilities to explore. I began collecting and categorizing relevant peer-reviewed articles. I spent as much time immersed in academic research as my health would allow. There were many days that I could not muster the energy to even turn on my computer. Other days, I was able to research for an hour at a time, as long as I carefully paced myself with hours of rest in between every hour of research.

I decided if I was destined to be bedridden, I would make the most of it. I resolved to search for a cure from my bed. I know so many of us follow this same instinct when we first get sick. We are driven to learn all we can about our illness. – I now had hours each day to devote to solving the puzzle of this complex and mysterious illness. My life – literally – depended on it. I would not waste what little life I had left in me. – Ironically, in order to make any lucky discoveries, I first had to get very unlucky.

The Light Bulb – One morning, my neck began to hurt. This neck pain was accompanied by a headache at the base of my skull. Also, my head felt too heavy, as though it was somehow sinking downward. –This was new.

And, when I would walk, my head seemed to “bobble,” just like those bobble head dolls. This strange sensation continued for weeks, unabated. – This was new.

And, when I would turn my head to the right, I would nearly lose consciousness. I also began having difficulty breathing. It seemed that my body was somehow “forgetting” to breathe. – This was new.

And, my heart rate began to fluctuate beyond my normal POTS fluctuations. My dysautonomia had previously been limited to moderate POTS, but it was now becoming progressively more severe, with my heart rate dipping as low as the 30’s and climbing as high as the 150’s. – This was new.

I began waking up in the middle of the night, gasping for air. Upon waking, I would be disoriented for about 30 seconds to a minute, yelling nonsensically and calling my family members by the wrong names. I realized that I was stopping breathing in my sleep. To assess the severity of the issue, I bought a medical-grade pulse oximeter to test my vitals. My heart rate was actually dipping into the 30’s while sleeping, and my oxygen saturation was dipping into the 80’s.

I was somewhat concerned that I might die in my sleep, and I needed a warning system. I set the pulse-ox alarm to wake me up when my heart rate fell lower than 45, and when my oxygen saturation fell below 91. Thanks to the pulse-ox alarm, I would awaken throughout the night in order to breathe and keep my vitals somewhat normal.

I was very angry about these new symptoms. I had already been living with severe ME for several years. How could I have developed such a severe and seemingly-unrelated new problem – on top of everything else? Despite my dramatic new symptoms, I did not want to seek medical attention. Aside from Dr. Kaufman, who was 2.5 hours away, no medical professional had ever understood that I was ill. I had actually faced outright hostility and ridicule from multiple doctors over the years. I am not alone in this. Nearly every patient with ME has shared similar stories on Phoenix Rising. There is an enormous, systemic problem.

Reluctantly, I decided to go to the ER. – In three trips to three different ERs for these new symptoms, I received three identical diagnoses: perfect health. The doctors noticed my elevated heart rate, but they dismissed that as being “just stress.” When I brought up my difficulty breathing, the doctor shined a light down my throat and said, “Your airway is perfectly fine, sir.” When I brought up my heart rate fluctuations, I was told, “That’s normal. Different people have different heart rates.” I then told the doctor the full extent of the fluctuations – that my heart rate was actually fluctuating from the 30’s through the 150’s.

The doctor then told me, “Heart rates don’t do that. That can’t possibly be happening.” I then countered the doctor’s dismissal with reality: I had been carefully tracking my heart rate changes with a pulse-oximeter. The doctor responded, “those home pulse-ox devices aren’t accurate. Stop using it.” I then countered the doctor’s dismissal, once again, with reality: I had invested in a medical-grade pulse-ox in order to verify what was happening, and this included an alarm system to wake me up. I informed him that both my medical-grade pulse-ox and my standard home pulse-ox were giving me the same readings: my heart rate was indeed fluctuating from the 30’s through the 150’s. The doctor responded without any apparent interest or concern, “Then I can’t tell you what’s going on. Have you considered that you might have anxiety?”

Ah. – So, yet again, I had been dismissed by the medical profession as having anxiety, just as I had been during my initial years with ME. Just as millions of us are dismissed – for years. – So, I did what I had done before. I buried myself in the same trusted treasure trove that had helped me discover my ME diagnosis several years earlier: peer-reviewed academic medical journals. – I poured over journal articles for weeks, analyzing the information, ruling out possibility after possibility. I was frenetic and running on fumes, as I sensed that I soon wouldn't be able to research at all. I finally came across a condition known as craniocervical instability (CCI).

I learned that CCI occurs because the ligaments of the craniocervical junction (where your skull meets your first vertebra) become too lax. Ligaments are made up of connective tissue, specifically collagen. Without tight, functional ligaments, the skull and upper spine become unstable/too mobile. This excess motion compresses neurological structures, resulting in cranial settling and brainstem compression, causing autonomic and other neurological symptoms.

I also learned that CCI very often occurs with atlantoaxial instability (AAI), instability between the first and second vertebrae. AAI would explain my near loss of consciousness when turning my head to the right. CCI/AAI accounted for all of my new symptoms, just as the ICC-ME definition had exactly matched all of my symptoms years earlier. Eureka! – What I learned about CCI/AAI was fascinating.


CCI/AAI is actually a well-established medical condition – but only if it occurs as the result of an injury, usually a high-impact trauma. CCI/AAI can also happen for no apparent reason, seemingly spontaneously. This spontaneous, non-traumatic CCI/AAI happens with rheumatoid arthritis, Ehlers-Danlos Syndrome, Down's Syndrome, and other inflammatory or genetic conditions impacting the body's connective tissue.

When CCI/AAI does not occur from a trauma, doctors almost always dismiss it as a possibility and miss it on imaging – even when severe brainstem compression is visibly present. More often than not, the appropriate imaging is never even ordered. – As I dove into the literature on CCI/AAI, I was astonished by how closely what I read matched my new symptoms: – The sensation that my head was too heavy and sinking downward? It was explicable. It was a scientifically-validated, known clinical condition, a form of CCI known as “cranial settling.” I was not wrong. My head truly was sinking downward. Far enough downward that my spinal column was impinging on my brainstem, compressing it!

The fading consciousness when turning my head to the right? It was explicable. It was a known condition, a form of cervical instability known as “atlantoaxial instability,” abbreviated as “AAI.” AAI and CCI very often occur together. I had clear, classic symptoms of both. – The breathing problems and sensation that my body was forgetting to breathe? It was explicable. It was due to the CCI compressing my brainstem, which CCI is known to do. I was not wrong. The brainstem is integral to maintaining breathing. – The worsening of my dysautonomia? Which had progressed from typical POTS to now being an extremely erratic heart rate? It was explicable. It was also due to the CCI compressing my brainstem. The brainstem is integral to heart rate regulation.

Every new symptom I had was accounted for, scientifically, in published peer-reviewed medical journal articles. My symptoms even had a single, physically verifiable cause. – Sweet relief! I smiled for the first time in weeks. – I am so thankful for research scientists and for internet access. Without online access to the published work of these scientists, I wouldn't have had the necessary pieces to solve the puzzle of my mysterious health issues.

I was now armed with real power. I had an accurate label for my new problem as well as academic research articles to support it: Craniocervical instability with atlantoaxial instability (CCI/AAI). I immediately printed out the three most relevant CCI/AAI academic journal articles. I highlighted the areas in each article that I felt would be most useful to doctors.

I took the academic journal articles, complete with my highlighted passages, to several different doctor’s appointments. I was promptly ridiculed, scoffed at, and even met with frank hostility and derision. One doctor – from a renowned institution – even seethed with anger. I told him that Dr. Kaufman had diagnosed me with POTS, ME, and MCAS, and that I was now having all the symptoms of CCI/AAI. I told him that my dysautonomia had progressed, that I had gone from having standard POTS to now having an extremely erratic heart rate. I tried to hand him the journal articles, with my highlighted passages. He wouldn’t take them. He instead sneered, “I find it awfully hard to believe that you have all these rare diseases." I responded calmly, politely asking him why he was impugning Dr. Kaufman’s diagnostic ability, and why was he also impugning the published work of academic research scientists? He immediately walked out of the room. That appointment was over.

As bad as these appointments were, they are very typical for those of us with chronic, misunderstood illnesses. For people without these illnesses, including some of our friends and family, it can be very hard to grasp that doctors routinely treat us this way. Sometimes, our loved ones don't believe us when we tell them about these experiences. Most people just can’t fathom that doctors behave this way, let alone that this treatment is the norm for us. A strong light needs to be shined on this problem.

In reality, it’s not surprising this doctor erroneously thought ME and POTS are rare. He also had no idea that ME and POTS very commonly occur together – and affect between 1 million and 2 million Americans, respectively. This doctor demonstrated the current, poor state of medical education surrounding these diagnoses.

There is a vast universe of conditions that aren’t routinely covered in medical school. In fact, in medical school, doctors are taught to assume the most common underlying explanation for a patient’s symptoms. In other words, “When you hear hoofbeats, think horses. Not zebras.” This “Horse-Zebra Rule” is often useful, because most patients’ symptoms will indeed be the result of very common underlying conditions. But, this Horse-Zebra Rule completely fails if the patient has an underlying condition that is less common, or a condition that is poorly-understood at that specific time in history.

All too often, a doctor’s response to a patient with seemingly-incomprehensible symptoms is this, “Since I can’t find a typical reason for your symptoms, then you must not be physically sick at all. You are being dramatic. You are wasting my time, and you are wasting medical resources.” – So. A better adage for medical schools might be, “When you hear hoofbeats and fail to find a horse -- suspect a zebra. Then refer that patient to a zebra doctor.”

My dysautonomia and breathing difficulties were rapidly getting worse, especially when upright. The sensation that my head was sinking downward was becoming more prominent. – I knew that all of these issues – the sinking head, the dysautonomia, and the breathing difficulties – were typical of CCI/AAI. I just couldn’t get a medical professional to believe me, let alone treat me.

In my frustration with this head-sinking sensation, I one day spontaneously pulled up on my head, forcing it upward, away from my shoulders. I immediately noticed that I began to breathe normally. When I let go of my head, it sank back down, and my breathing became difficult again. I repeated this maneuver, several times, to make sure the effect on my breathing was actually happening. Sure enough, my breathing responded the same way each time.

Thanks to peer-reviewed research articles on CCI/AAI, I was able to understand the underlying mechanism at play here. The ligaments surrounding my skull and upper cervical spine had become too lax, causing CCI/AAI with cranial settling. My upper cervical spine was compressing my brainstem when my skull sank downward. Lifting up on my head relieved that brainstem compression, which in turn relieved my symptoms. I was lifting my brainstem off of my spinal column.

Understanding the mechanical basis of my symptoms helped make the entire situation somewhat less alarming. It also allowed me to think of viable solutions. I knew that if I could just force my head up higher all day, to mechanically offset the cranial settling and brainstem compression from CCI, I would breathe better. So, I began wearing a Philadelphia cervical collar, hoping it would keep my head from sinking downward. Sure enough, the collar did improve my breathing and seemed to help buy me some time. I needed the collar to keep me stable for as long as possible while I sought competent medical help. I felt I might be running out of time.

I joined an online CCI/AAI group. And then, my world expanded. – The Facebook group “Beyond the Measurement” is comprised of people with CCI/AAI and related neurosurgical conditions, such as Chiari. What I learned from this group is nothing short of extraordinary. Most people with CCI/AAI are dismissed by doctors and remain undiagnosed for years, just as I was now being dismissed. This struck me as being very similar to how people with ME and other less-explored conditions are treated.
The fact that a cervical collar was helping me breathe and stand up was a very strong indicator of instability (CCI/AAI).

Fixing CCI/AAI requires neurosurgery, and only small handful neurosurgeons in the world are consistently competent at diagnosing and treating non-traumatic CCI/AAI. – So, thanks to the knowledgeable and helpful online community of CCI/AAI/Chiari patients, I now had a clear direction. I now knew which doctors to see – and which doctors to not see. I doubt I could have received this crucial information from any other source at the time.

My goal was to now see a CCI/AAI-literate neurosurgeon to fix the CCI/AAI with a craniocervical fusion surgery, which is the established effective surgical procedure for CCI/AAI. – I didn’t have the health insurance to immediately see the right neurosurgeons, so I went to work on this insurance problem and continued exploring the online CCI/AAI/Chiari community, learning from these patients. I spent nearly all my time in bed, wearing my cervical collar, immersed in the online CCI/AAI/Chiari community. I noticed that these patients all had POTS, just as I did. I couldn’t find a single one of them who did not have POTS. Many of them also had MCAS, just as I did.

The vast majority of these patients also had a diagnosis of Ehlers-Danlos Syndrome (EDS), a connective tissue disorder characterized by faulty collagen. There are many different types of EDS. The people in the CCI/AAI/Chiari group nearly all had EDS Type 3, the hypermobility type, abbreviated “hEDS.” A hallmark symptom of hEDS is lax ligaments. I did not have an hEDS diagnosis myself, and I didn’t consider myself to be particularly hypermobile. The only thing hypermobile about me seemed to be the ligaments of my craniocervical junction, as evidenced by the CCI/AAI.

It seemed impossible that random chance could account for over five thousand people in this group demonstrating this POTS-MCAS-CCI/AAI cluster. I began to question: Why do all of these patients with POTS and MCAS – just as I have – develop non-traumatic CCI/AAI – just as I had? I found a possible answer in an research article that discussed this exact symptom cluster: the trifecta of POTS, MCAS, and hEDS. – Did I – and the people in this CCI/AAI/Chiari group – have a specific gene underlying all of this? Did I have hypermobility-type Ehlers-Danlos Syndrome – on top of everything else? I couldn’t know for sure. But, I was very sure that I would be unable to stand up very soon, as I kept getting worse.

The Cliff Dive – One day, after wearing my Philadelphia cervical collar non-stop for over a month, I removed it in order to air out my neck and prevent pressure sores. I then put on a different, weaker collar with more breathable material. – After several minutes in this weaker collar, while sitting up in bed, I was hit by severe dizziness. I had been experiencing dizziness on and off for months, but this bout was much more severe. It felt different. I laid back down for several minutes and sat up again. The severe dizziness took over once more, and I realized I was actually having difficulty moving my limbs.

I called my family for help, and they handed me my firmer Philadelphia collar, which I put back on. I tried sitting up with my head and neck now supported by the firmer collar, but I again collapsed back onto the bed. After multiple unsuccessful attempts to sit up, I decided to remain lying flat in my Philadelphia collar. We would wait and see if I would regain the ability to sit up.

Twenty hours later, after many attempts to sit up, I was still collapsing from extreme dizziness and an inability to move my limbs while upright. My condition had become much worse. I became completely bedbound with a very rapid heart rate while lying down, as well as an incapacitating dizziness and partial paralysis while attempting to sit up. I had run out of options. I called an ambulance.

The Battle – What happened after that ambulance ride is long story. I won’t share it here. The short version is this: I was finally admitted to my local hospital. Despite my horrible condition, getting me admitted required extreme advocacy from Dr. Kaufman and my family, because my local hospital had previously and incorrectly labeled me with health-related "anxiety." – Once admitted, I asked for a halo brace. I had learned through research that a halo brace is held in place by four screws driven into the skull, stabilizing the skull and spine. This would provide much stronger stability and traction than my cervical collar.

A halo brace would alleviate my cranial settling and thus allow me to sit up, a halo would help me breathe better, and a halo would help alleviate my dysautonomia from brainstem compression. It would make me mobile again until I could have surgery to correct my CCI/AAI.

I gave the doctors peer-reviewed articles supporting the need for a halo brace, but they wouldn’t read the articles. They told me that they would absolutely not put me in a halo brace. So, I asked them for a transfer to an CCI/AAI-literate neurosurgeon, but they wouldn’t do that, either. They had no training in how to recognize CCI/AAI. And so, because they couldn't figure this out, they told me that nothing was medically wrong with me. They accused me of malingering and tried to discharge me each day I was there.

After 22 days spent flat on my back at my local hospital, each day being denied a halo and accused of malingering, I was finally transferred to what is considered one of the best hospitals in the United States. – At this world-renowned hospital, I was subjected to worse treatment by doctors than I’ve ever experienced in any medical setting. I remained 100% bedbound, with severe and untreated CCI/AAI, for a total of 122 days – over four months flat in bed between the two hospitals. I laid with my feet up and my head down, as I found that this tilted position helped me breathe a bit more easily and alleviated some of my dizziness.

I laid like this, day in and day out, for months. I couldn't sit up, let alone leave the bed. It was an excruciating experience that I will never forget. I was repeatedly berated by neurosurgeons who incorrectly asserted that nothing was wrong with my neck. They ordered me to take off the neck brace, stand up, and leave the hospital. When I tried to comply, I would collapse from profound dizziness, an inability to breathe, and limb paralysis. Despite this, they refused to examine or treat me further.

The neurosurgeons called in both a psychiatrist and a psychologist, subjecting me to repeated mental health evaluations throughout my months-long stay. With each evaluation, the psychiatrist and psychologist determined that I was mentally competent. They stated in my medical records that my cognition was intact, and I was not delusional. The psychologist described me as being "intelligent" and "knowledgeable" about my physical health conditions. Nevertheless, the neurosurgeons instructed the hospital administration to discharge me to a nursing home, in an effort to get rid of me. But no nursing home would accept me, saying I was too young.

On the 123rd day of my hospitalization, after months stuck flat on my back and unable to leave the hospital, I was suddenly visited by the hospital's highest-level neurosurgeon – the actual director of the Spine Center. He said: "This is a puzzle, and I'm going to solve it." – Then, this highly-seasoned neurosurgeon finally did the work that every other doctor had refused to do: He took a genuine interest in my symptoms, and he ordered the correct imaging required to assess for CCI/AAI – a dynamic CT scan with flexion and extension views.

In just three days of evaluating me, based on the new imaging, this neurosurgeon correctly diagnosed me with CCI/AAI. He told me that my upper spinal ligaments were hypermobile and lax, causing CCI/AAI with cranial settling. I had discovered this months earlier thanks to research articles, but I was so relieved to finally hear him say it! He informed me that he would put me in a halo – the exact intervention I had repeatedly asked for, and been denied, for 5 months. – As expected, the halo alleviated my cranial settling and allowed me to finally be upright again. When tilted up in my hospital bed, I no longer had dizziness or breathing difficulties.

After 143 days hospitalized – nearly five months total – I was finally able to go back home. The price of my freedom was having a literal cage on my head. – The screws in my skull held my head in tandem with the height of my halo vest. The vest itself is adjustable and hugs the chest. After the initial halo placement, I still had some dizziness, although not as severely as previously. This residual dizziness was particularly noticeable when getting out of bed. I had another halo adjustment, increasing the lift of my head, and my dizziness while being upright was gone.

Unfortunately, I still could not stand up for very long, as my legs were severely atrophied from 5 months of lying flat in a hospital bed. Also, the POTS that I’d had for four years was still as severe as ever, with my standing heart rate in the 140's.

I now had to strengthen my muscles. I could sit up on my own, but this took a lot of effort due to the atrophy. My legs were extremely weak and looked like toothpicks, but I could walk, barely, using a walker. Despite the muscle atrophy, I was elated to finally be free from the hospital! I set to work on the final step of this battle – securing insurance approval and a surgery date with a CCI/AAI-literate neurosurgeon. – As I moved around in the halo, I noticed that each time I pushed up on my halo vest, I’d get more neck traction. My head felt better when elevated higher above my chest, in direct opposition to when it had been sinking downward due to cranial settling from CCI.

Over the next few weeks, the halo vest began sinking again, causing my skull to sink down with it. The dizziness returned. So, I pushed up on the vest as hard as I could, elevating my head as high as possible. While holding the vest in this higher position, I noticed that colors seemed suddenly brighter. Odd. To see if my perception was accurate, I let go of the vest, and my head sank back down. Sure enough, when my head sank down with the vest, colors were less bright. I pushed back up on the vest again, very hard, providing maximal traction to lift my skull away from my spine. I held this position for a minute or so, taking stock of my perceptions. As I stood there with my head at maximum elevation, I noticed that my mind felt especially clear. Also, my heart rate felt normal – for the first time in years.

For the past four years, ever since the onset of my severe ME and POTS, my standing heart rate was usually in the 130’s. But, with maximum halo lift, this suddenly didn’t seem to be the case. I felt so different—I suddenly felt… kind of healthy? I grabbed my pulse ox and measured. Sure enough, my standing heart rate was 86! I couldn’t believe it. This seemed too good to be true, and I couldn’t accept this so easily. I had to verify this was actually happening. So I let go of the halo, and my skull sank downward. My heart rate measured 127. I lifted the halo again with maximal traction, and my heart rate was now 82.

I repeated this lifting and dropping maneuver for several days. My heart rate responded consistently, every time. Though I knew this was very significant, I was still guarded about getting my hopes up. During my years with ME, I had experienced several brief periods of mild, though noticeable, improvement. These improvements never lasted. And so, I had learned over time to continually temper my expectations for recovery. Avoiding false hope was necessary for self-preservation.

I found a local professional who agreed to lift and tighten my halo, so that my skull would stay elevated at this higher level, alleviating brainstem compression at all times. After this adjustment, my POTS once again remitted and remained gone. – The magnitude of this breakthrough, and of my sharply increased quality of life, was becoming apparent. I started to wonder: Had my POTS actually been caused by brainstem compression from CCI/AAI all along? Had I actually had brainstem compression for the last four years – mild at first, and then progressively worse – the entire time that I had severe ME? The answer to both questions seemed to be: – Yes.

Awakening – In the halo, using a walker, I attended my first physical therapy appointment. Although my POTS was gone, I was extremely concerned about exerting myself physically. I knew from years of experience that any increase in physical activity would trigger a crash – PEM – and these crashes would last for months. I did not want to become bedbound again, unable to shower on my own, unable to eat or communicate. I would need to start PT extremely slowly. I would stop at the first sign of a PEM crash.

My physical therapist was very personable and knowledgeable. In my first session, she tested my physical strength and noted severe muscle atrophy. This evaluation was an hour long, and I had to do significantly more walking and overall muscle flexing that I’d done in many years. When the session was over, I arrived home and went straight to bed in order to minimize the inevitable “crash” of exhaustion and cognitive impairment that would soon follow. – But, the crash never came.

I began venturing out to eat dinner with my family, instead of eating alone in bed in a dark and quiet room, as I had done for years. I realized that I could sit up, eat, and talk all at once – without becoming exhausted during dinner, and without crashing later. I soon discovered even more new capabilities: I could now sit up and read for hours – all without becoming exhausted, taking rest breaks, or crashing. I could now talk on the phone for hours, with more liveliness than I’d had in years. I couldn’t believe how easily my mind was processing information. I couldn’t really believe any of this at all. I kept internally bracing for the next crash to hit me.

As I began to live my life unrestrained, I would at times become overwhelmed by a strange blend of gratitude, exhilaration, and grief. After years of being sick with severe ME, having profoundly constricted my entire life and expectations, it was somewhat difficult to understand my new freedom – even as I expanded my new life.

Time, Time, Time – I couldn’t wait for my fusion surgery. The halo was giving me freedom from years-long brainstem compression that had resulted in POTS and severe ME. But, I was really feeling constrained behind its bars.

After 4 years of being bedridden with severe ME, including five recent months as a bedbound hospital inpatient and four months in the halo… I finally had my fusion surgery with one of the world’s very few CCI/AAI-literate neurosurgeons. – The surgery was successful. After the initial post-op healing period, my POTS and PEM were gone, exactly as they had been in the halo. They remain gone today.

In early 2018, I posted my story to the Phoenix Rising online ME community. As of October of 2018, six people on Phoenix Rising, all with a hallmark ME presentation, have been diagnosed with CCI/AAI after reading my story. Many, many more on Phoenix Rising have contacted me, saying they’re trying to be evaluated but are unable to access the necessary resources to do so. This is a significant barrier for many.

As difficult as my journey has been, I have faced far fewer barriers than many others with severe chronic illness. I have had consistent health insurance, an internist who advocated for me, and a supportive family who understood how sick I was. My ability to survive, and to research, wouldn't have been possible without the support of Dr. Kaufman and my family. For years, my family took care of me, physically and financially, at great cost to themselves. Most people with chronic illness are not so lucky. Many do not have access to a single doctor who will believe them, and many are disbelieved and abandoned by their families.

Thank you. Every single one of you.
My story isn't over yet. More to come!

 

What a story...

My goodness... I am speechless... love to you.

Lane, I should add, now that I've found a few words.

You are a remarkable, and inspiring person. You have endured and suffered so much and are still strong, positive and inspiring, and kick ass.

I am humbled and heartened. See you around the block sister Lane.

Daniel

 

Thank you @Daniel Lion for your very kind words. I also want to thank you for taking the time you took to read the above story. To clarify, it's not my story, but a story that has many similarities to my own. So I identify and empathize with what this young man went through, as most people can hardly fathom the difficulties that people with moderate to severe ME/CFS endure.

I hesitate to write much about my own ME/CFS story, but also feel a certain obligation to do so, as there are actually a number of crossover symtoms between the two. So I believe learning more about ME/CFS can actually help people understand their own tinnitus symptoms, such as various kinds and severity of brain symptoms. Plus the fact that both can originate in the neck and upper cervical area--and more.

Many people with ME/CFS have tinnitus and also have extreme sound sensitivity as well, which is a step beyond hyperacusis, known as noxacusis. Sometimes people who have been able to make progress with their ME/CFS make progress with their sound sensitivity as well. You may find THIS STORY interesting (on a different forum). It's a story by a man who got rid of his tinnitus after starting a ketogenic diet--after having suffered with tinnitus for 5 1/2 years.

Just to mention, that would be brother Lane. Somebody else on this forum had assumed I was female also, which surprised me, as I wasn't aware Lane was also a female name. I just did a little googling, and discovered that the female spelling is usually Laine, Layne, or Laney. But is spelled Lane as well.

One woman wrote that she was going to name her little boy Lane. She liked it because it came across to her as strong and serene. I liked that. It seemed like a bit of a blending (or balance) between the masculine and the feminine energies that we all have wihin ourselves. I've done some reading on this topic, and have come to believe that balancing these energies within ourselves has the potential to help lessen the severity of tinnitus and/or hyperacusis.

Thank you again for your kind words. I've been appreciating your posts and this forum, and have appreciated hearing about the creativity you try to incorporate into your daily life. Now that you have extremely challenging acoustic issues, your creativity may need to be exercised in ways you've never done before. But you strike me as an individual who's up to the task, and I look forward to reading about some of the solutions you come up with going forward.

 

Thank you brother Lane.

Sister Daniel here, my bad...

Take care, be strong, and better days for us I hope.