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.