Bow Hunter's Syndrome Part 3: Wresting diagnosis and treatment from the jaws of modern medicine

The author, probably a horse

Doctors are trained, when faced with an unusual set of symptoms, to diagnose the patient as having an unusual presentation of a common problem, rather than a rare disease presenting in its normal way. As my pediatrician-mother likes to put it, "When you hear hoof beats, don't think of a zebra, it's probably a horse." This advice likely serves many doctors well. That odd looking rash on the baby's bum really is more likely to be an atypical diaper rash than amoebic tushitis.

The problem with this policy is those patient who truly are zebras. We are inevitably mistaken for, and treated as, horses. Before I knew of Bow Hunter's Syndrome, I saw two primary care physicians at the same office about my vertigo-dizziness- tinnitus-faintness-etc. symptoms. The first was sure I was depressed, asked if I had "heard of psychosomatic," and walked out when I insisted I was a zebra. The second, two weeks later, told me I was terribly allergic to my new home and would have to move out of town, closer to the sea, immediately. Shortly thereafter I had my first, and worst, "Bow Hunter's Stroke."

Luckily, none of the doctors at the clinic were free to see me that day, and I was shunted to their nurse practitioner. Nurse-practitioners, unlike M.D.s, have listening skills. I described the whole thing to her, and she immediately saw my stripes. I was no horse. She sent me for an ultrasound of my carotid arteries (to check if the main blood supply to my head was obstructed) which gave me a vital clue. While checking the carotid arteries, the technologist always did the vertebral arteries too. While both looked normal on the ultrasound (which was taken with my head in neutral position) this was my first inkling that a blockage of vertebral arteries was even possible.

I'm not going to describe in detail the extraordinarily stressful process of literature searching, doctor-switching, insurance switching, specialist bouncing, bureaucracy fighting, scans, rescans, etc. In short, I found Rotational Vertebral Artery Compression Syndrome in the literature, was struck by how exactly the case studies approximated me, and spent a year actively working toward official diagnosis and treatment, culminating in a near-fatal but successful surgery. There are several key lessons I learned along the way that I want to share with other putative sufferers of Bow-Hunter's Syndrome:

1. No doctor is going to bother to manage your care, but you have to let them pretend they are going to manage your care. Managing your own care is a full time job you have to do. Keep lists. Call every day to find out if a slot has opened up, remind people of things they already know, catch their errors, force them to communicate with you and each other, advocate for yourself. This difficult process was somewhat ameliorated by the fact that I am a white male with a greying beard. Bring an old white man with you, there are plenty of us around.

2. Bow Hunter's Syndrome can only be definitively diagnosed by comparing blood flow through your vertebral arteries when you head is in a neutral position to when it is in a position that brings on your symptoms. Make sure your doctor is explicit about this need in ordering imaging. Medical technologists are often not willing to take imaging with your head turned. They are trained to do everything in a neutral position, and if you explain why it has to be done with your head turned, they will worry you are going to pass out in their expensive machine or onto their legally-liable floor. Call ahead several days before your x-ray, CT, MRI, whatever, and insist on talking with the technologist to be sure it will be done as ordered. They will tell you they need to consult with the radiologist, who will want to talk to your doctor, who won't be available, etc.

On the day of your scan, if the technologist won't unequivocally confirm that your scan will be done both straight and with your head turned far enough to induce symptoms, you must refuse to get in their machine. Stand there shivering in the buttless gown and calmly insist that they do the scan the way your doctor ordered it. If they tell you their MRI machine doesn't have space for you to turn your head, tell them they have to put on the coil after your head is already turned. If they insist the condition you are describing doesn't exist, whip out a published description. Walk out if you have to. Failing to do this once delayed my care by four months and cost me several thousand dollars because once the scan was done wrong, my insurance wouldn't approve the same scan again. I had to change to more expensive insurance and doctors, start the process from scratch, and learn to insist.

3. The ultimate confirmation of Bow-Hunter's Syndrome comes from fluoroscopic angiography with arterial catheterization. This means they take video of x-ray dye moving through your vertebral arteries, with your head in various positions. The dye is delivered through a small tube that is inserted through an incision into your thigh and wormed up as far as the back of your brain. I found the moments when they were squirting dye into my brain to be the most painful of the entire experience, despite the sedation and pain killers. However after this procedure no one questioned my self-diagnosis.

4. The most common surgery for Bow Hunter's Syndrome is called decompression. This means they made more space around one of my vertebral arteries (one unobstructed vertebral artery is generally enough to keep your brain stem ticking) by removing a piece of the bone that wraps around it on the side of my first cervical vertebra. Such decompressions are done for a variety of syndromes that are more common than Bow Hunter's Syndrome, and it is this fact that very nearly killed me.

Surgeons are very busy people, and generally make physicians look like excellent listeners. My surgeon performs a fair number of decompressions, but mine was his first for Bow Hunter's Syndrome. His protocol for this surgery starts with having another surgeon carve a path to the bone. Then he takes over and turns the patient's head for a clearer view of his work grinding away bone without damaging other important bits. You know, and I know, and he knew, that turning my head cut off the blood supply to my brainstem. He did it anyway. That was the next step in his protocol, he had not had time to think this through, and I had failed to manage my own care on this point. Several minutes later, when they checked, no blood was flowing through my vertebral arteries. He guessed it was measurement error, and had another machine sent. Still no blood flow. They righted my head, glued me up in a hurry, and went to scare the life out of my poor wife. Luckily, they frappéd jargon with indirectness and she didn't understand what had happened until after it was clear that I hadn't been killed. My brain stem responded well to the return of blood and I was out of the hospital the next morning. Recovery to the point of feeling better than before surgery took me about three months.

5. Finally, as this is now long, they insisted I would need opioids after my spine surgery. I did not. Accept them if you need to, don't assume that you will need to.

I'm happy to be your support group of one if you're wondering if this diagnosis is yours. I'm a Ph.D. though, not an M.D., so be aware that I can only offer one zebra's perspective.

Bow Hunter's Syndrome Part 2: What, with a dab of why

Bow Hunter's Syndrome is named for people who shoot arrows at wildlife. In order to do so, they have to turn their heads hard to one side and hold that position until they are ready to shoot. Attempting this with Bow Hunter's Syndrome lands them in the emergency room, and sometimes gets the actual attention of a doctor, who will occasionally figure out what is going on, possibly provide treatment, and if they've gone to that much bother, almost certainly publish a report mentioning said venery.

That case study might refer to this condition as Rotational Vertebral Artery Compression Syndrome. The vertebral arteries are the main blood supply to the brain stem, one of those body parts humans can't do without, not even for a few seconds. There is one vertebral artery on each side of your spine, running up through tunnels in your cervical vertebrae, the spine bones in your neck. When a healthy person moves their head, each artery has enough space to wiggle in these tunnel, so that it doesn't get compressed, kinked, stretched, or otherwise lose flow. Rotational Vertebral Artery Compression Syndrome means that rotating your head pinches your vertebral arteries, usually at the joint between the two uppermost vertebrae, reducing or eliminating flow of blood to your brain stem. The various other names this condition goes by mostly include the word "insufficiency" referring to the shortage of oxygen in your brain stem, which is what causes the actual symptoms.

About those symptoms: tinnitus (a buzzing sometimes in both ears, sometimes just in my left), vertigo, dizziness, mental fatigue, and faintness, increased gradually over several years during which I did not notice that head position had anything to do with it. I would consistently feel dizzy while shopping in the grocery store, not noticing any connection to turning my head from side to side trying to find unsweetened pickles. Long after others had recovered from some cold, I would have lingering vertigo and a sense of mental fog. I gave up my habit of sleeping on my belly, which allowed me to fall asleep very quickly, but from which I eventually decided I was waking feeling tired and unwell. My first autumn in Wisconsin I wondered if I was wrapping my scarf too tight because I would start to feel like I was going to faint every time I tried to back my car out of the driveway. Attempts to discuss all of this with doctors were frequent but unproductive.

The symptoms became debilitating only after an accidental blow to the top of my spine that winter. I was diagnosed with a concussion, and because most of the symptoms of Bow Hunter's Syndrome are consistent with a concussion, I looked no further. Most of a year later, having recovered only slightly, I was a passenger in my moving van (on the way to California) when it turned over on the highway, flexing my neck too far forward. After that, the observation that the position of my neck had both immediate and lingering effects on my symptoms became unavoidable.

The blaring clue to my addled brain came from my new neighbors' fig tree. Having permission to pick, I was straining to reach a fig well above my head. I looked up, turned my head to the side, stretched my arm, and collapsed. My eyeballs were shaking, my vision went black, and the tinnitus became unimaginably loud, louder than the human ear could withstand if real sound was involved. I was breathing, but felt the urgent need to come up for air. I brought my head back to neutral and the screeching tsunami gradually receded, light started to return, the spinning slowed. I had survived my first "Bow Hunter's Stroke." I put that phrase in quotes because unlike a real stroke, no tissue damage showed on scans afterwards. My brain stem hadn't lost living tissue, just power. The medical jargon for such an event is a transient ischemic attack. My doctors were no more than puzzled.

In the fifteen months since then, I went through thousands of abstracts, scores of full scientific papers, two insurance companies, four primary care providers,  three neurologists and a wide variety of other specialists, countless imaging studies, three interventions including a poorly planned major surgery that nearly killed me right there on the table, and months of recovery before I could considered myself past Bow Hunter's Syndrome. All worth it. I'll dig around in the key pieces of that in Bow Hunter's Syndrome Part 3: Wresting diagnosis and treatment from the jaws of modern medicine.

Bow Hunter's Syndrome Part 1: A Survivors' Support Group of One

My doctor looked like I had just puked in his shoes, but eventually took the papers I was holding out to him. This was a better response than I had expected, but still I could tell he would not look at them. I switched doctors, and with the next one emailed him the papers after our appointment. He wrote the referral I needed but declined to see me for months thereafter, going so far as to change an in-person appointment for a possible broken wrist to a phone appointment, after I was already in his parking lot.

The only thing a doctor likes less than a patient walking in with an obscure self-diagnosis they found on the internet is when the patient brings the medical literature about that diagnosis for the doctor to read. I knew this, but also that my doctors had not heard of Rotational Vertebral Artery Compression Syndrome, and that I needed treatment for it. Adding insult to injury, my obscure self-diagnosis would eventually be unquestionably confirmed and successfully treated.

This same condition goes by multiple names in the medical literature including Rotational Vertebral Artery Compression Syndrome, Positional VertebrobasilarIschemia, Positional Vertebrobasilar Insufficiency, and Bow Hunter's Syndrome. This last name is the least descriptive, but also the shortest and least jargony, so I tend to use it.

Bow Hunter's Syndrome is quite rarely diagnosed. There are perhaps a few hundred cases documented in the medical literature, total, globally. This could mean that it is a rarely occurring condition, but given the lengths I had to go to get it diagnosed, the severity of those cases that are documented, and the peculiar circumstances that allowed me to reach diagnosis, I suspect rather that it is an only slight uncommon condition which generally goes undiagnosed. As a neurological condition not caused by any neurological defect, Bow Hunter's Syndrome tends to fall through the cracks.

I was able to diagnose my own case because I have a Ph.D. in biology, loquacious physicians as parents, and extensive experience finding obscure scientific literature outside my field of expertise. I knew I had something, as most patients do, but I also knew how to search the scientific literature for conditions associated with terms like "cervical vertigo," "rotational stenosis," "positional tinnitus," and "nystagmus,"  then skim through the results, read those papers that seemed most relevant, follow citations back and forth, improve my search terms, and so on to a diagnosis. What I had to figure out was how to then navigate Earth's most wasteful medical system (USA! USA!) to arrive at an official diagnosis and ultimately treatment, and I had to do all of this while suffering the symptoms of Bow Hunter's Syndrome (including vertigo, ringing in my ears, dizziness, intermittently blurred vision, and faintness).

The good news is that I am largely recovered from it, and from the surgery that resolved it.

The bad news is that I will be posting here a series of short essays with the hope of making this process slightly easier for other sufferers of Bow Hunter's Syndrome, a purpose which I do hereby gleefully acknowledge will horrify all medical professionals that happen upon this. There are, so far as I can tell, no popular accounts, no support groups, no blog posts written for the uninitiated, describing what Bow Hunter's Syndrome is, what it feels like, how to approach being a patient with it. Perhaps soon more patients will be wobbling into their doctors' offices mumbling about the blood supply to their brain stem and a condition the doctor has never heard of. One can hope.

Next: Bow Hunter's Syndrome Part 2: What, with a dab of why

Then: Bow Hunter's Syndrome Part 3: Wresting diagnosis and treatment from the jaws of modern medicine