Brain drain for real: Adventures in cerebrospinal fluid leakage

A few months ago my wife began experiencing a nonstop headache that became increasingly severe over the space of a week and a half. Having experienced tension, sinus, and migraine headaches with some regularity throughout life, my wife is very familiar with headache treatments and their relative effectiveness. But nothing she tried eased the pain at all.

Eventually my wife began experiencing tinnitus and an odd hearing disparity, along with cognitive fog. My wife visited the local instacare one day when she was at her wits' end. The doctors there said that an MRI was needed but that "they" (whoever that is) don't like it when instacare doctors order MRIs.

Due to my wife's persistence, an MRI of the brain was ordered anyway. Then she called the imaging center and pestered them. Conveniently, the imaging center had a cancellation so that my wife was able to get her MRI done later that evening. We perused the results as soon as we knew they were available and discovered that the observations were consistent with intracranial hypotension.

The information we read online about this condition seemed rather alarming. At this point the instacare folks said that this was outside of their area of treatment and that my wife needed to see a neurologist. But due to the way things work in our heavily regulated, insurance focused healthcare industry, my wife would need a referral from her primary care physician.

The whole reason my wife had gone to instacare was because her primary care doctor was out of town for a couple of weeks. She ended up working with the office staff of one of the doctors who was covering for her doctor. It's a good thing that doctor was not physically within reach when a staffer told my wife that the doctor didn't think the finding was significant. She would have reached through the phone line and throttled the guy.

In reality this doctor isn't a bad guy. Let's just say that he comes across as more detail oriented than patient oriented. And let's just say that my wife was persistent enough to get that doctor to refer her to a neurologist. It still took several days to see the neurologist after taking the first available cancellation spot.

During the visit we were pleased that the neurologist had gone to the trouble to personally analyze my wife's MRI. Since intracranial hypotension is generally caused by a cerebrospinal fluid (CSF) leak, the neurologist carefully questioned my wife to try to determine the cause of the leak. Spontaneous CSF leaks are considered very rare (see National Organization for Rare Disorders discussion). But all indications were that this is exactly what my wife was experiencing.

The neurologist explained that he understood that most spontaneous CSF leaks can heal up on their own. He ordered four weeks of strict bed rest accompanied by relatively high doses of caffeine. It turns out that caffeine's vasoconstriction properties can help alleviate some CSF leak symptoms, as can lying down. Painkillers are completely ineffective for this condition.

My wife felt somewhat OK while lying down and taking caffeine. She could get up to take care of necessities, but she couldn't stay up for long before the nasty headache returned. That was a terribly boring month for my wife. She isn't much into watching TV. She loves to read but the cognitive fog she was experiencing made that difficult.

When a month of bed rest yielded no positive results, the neurologist ordered an extensive MRI of the spine. Upon reviewing the results he ordered an epidural blood patch procedure. In essence, they suck some blood from your arm and inject it into the epidural space of the spine. It is supposed to work like slime in a bicycle tube to form a clot to patch the leak. But it costs $6,000 more than a bike tube repair.

The blood patch was helpful. My wife was soon functional, but far from 100%. In follow up appointments with the neurologist it seemed clear to us that his expertise in my wife's precise condition was quite limited. Our research suggests that this is common. Very few neurologists have knowingly interacted with anyone experiencing a spontaneous CSF leak.

The UK-based CSF Leak Association explains that spontaneous CSF leaks "are not rare, yet are generally under-diagnosed; misdiagnosis of migraine, sinusitis and other headache disorders is common place...." Specialists in the condition believe that it is far more widespread than understood by the medical community and that ignorance of the condition and its treatments is rampant among healthcare professionals.

A couple of months after the blood patch it became clear that my wife was experiencing more CSF leaking. It seemed obvious that another blood patch was needed but the neurologist wanted more MRIs to get a better idea of what was going on. This chagrined my wife. Even with decent insurance, MRIs are not cheap and she couldn't imagine what benefit additional imaging would provide.

Hospitals use MRI machines like cash printing presses. Last year Forbes reported that "the national median network rate for a scan at a freestanding center is $504—a third of the $1,567 rate in a hospital." Which is why hospitals and their industry compatriots work to squeeze out private imaging centers. Moreover, a full set of MRIs covering the brain and the spine amounts to four images. So multiply that $1,567 by four every time my wife makes a trip to the imaging center. Ouch.

Thanks in part to the MRIs and the blood patch, for the first time in our lives we were able to take advantage of the medical expense tax deduction for last year and we're shaping up to repeat that performance this year. That's small consolation. Since you can only deduct the amount of qualified medical expenses that exceed 7.5% of your adjusted gross income, any tax benefit covers a tiny pittance of actual out of pocket medical expenses.

We didn't want to pay for more MRIs but the neurologist would not budge on the requirement for a third round of MRIs. The thinking was that locating the leak source would make the second blood patch procedure more successful; although, the doctor had told us that even with the best imaging they can't find the actual leak location in 80% of cases.

The third round of MRIs revealed nothing new, which made my wife even less happy about incurring the related expense. Still, the neurologist ordered a second blood patch. But when we went for the procedure a physician's assistant explained that the neuroradiologist who was to perform the procedure wouldn't do it until my wife had yet another set of MRIs that were tuned to look at fluids. I didn't even know there was such a thing as a neuroradiologist until that moment.

This fourth round of MRIs revealed a number of perineural cysts (aka Tarlov cysts) on the spine but there was no clear indication of where the current leaks were. The doctor was especially suspicious of a very large cyst near the base of the spine. When we took my wife in for the rescheduled second blood patch, the doctor took us into an alcove that had three large computer screens. He rotated the images around to show different angles and slices so that we could see what he was seeing. He called my wife's case very unusual.

The second blood patch temporarily caused intracranial hypertension. But after a few days my wife was doing much better and was much more functional. In a follow up appointment the neuroradiologist suggested that no further treatments would be helpful unless significant leakage is once again detected.

The doctor explained that my wife must appreciably alter her lifestyle so as to prevent rupture of any of the multiple cysts on her spine. She must avoid anything strenuous and any kind of motion that might cause problems. No riding on bumpy roads. No picking up anything heavy, including small children. Only very light yard work or housework. All bending must be undertaken with great care. Etc.

Even extreme care will not guarantee freedom from problems. It is believed that the large cyst near the base of my wife's spine acts as a CSF reservoir. The longer my wife is upright the more CSF drains into the reservoir, causing brain sagging which results in nasty headache, hearing problems, and cognitive issues. Caffeine can help. But the real thing is a significantly different lifestyle than she has been used to.

We aren't complaining. Many people deal with far worse conditions. This isn't fatal; just unpleasant. We are learning more about spontaneous CSF leaks, perineural cysts, and other factors surrounding my wife's condition. We will see where this adventure takes us.

Update: See part 2 (8/7/19) and part 3 (11/18/19) for the rest of the story.