Adventures in cerebrospinal fluid leakage: part 6 (conclusion?)

Part 6 in a series about my wife's spontaneous cerebrospinal fluid leak condition. See part 1, part 2, part 3, part 4, part 5.

TL;DR  My wife's central nervous system (CNS) spontaneously sprang a leak two years ago. Nobody knows why. Too little pressure in the CNS causes a raft of persistent problems, including nasty head pain that never goes away, hearing problems, mental fog, etc. We have been on the hunt for successful treatment since then, with some successes and a lot of failures. But now we may be at the end of the story.  

The end of August found us at Cedars-Sinai Hospital in Los Angeles for the fifth time in 13 months for yet another attempt to resolve the cerebrospinal fluid leak (CSF leak) my wife had been experiencing for two years. We saw success after a leak was identified and surgically repaired last October (see part 3), only to have additional CSF leak symptoms develop a few weeks later.

We returned to Cedars-Sinai in February (see part 4). The CSF Leak Expert Team searched for the leak via digital subtraction myelogram (DSM) without success. They hoped that this meant that the leak was small enough that it could be resolved with an epidural blood patch. When that didn't work, we returned in June for another epidural patch, but using fibrin glue instead of my wife's own blood (see part 5). When no improvement resulted, the team scheduled another surgery.

When we returned in August, the team did three DSMs in an unsuccessful hunt for the leak. Each DSM is a surgical procedure that occurs under general anesthesia in an operating room. Only a portion of one side of the spine can be inspected during a single DSM. That's why they did so many DSMs.

Dr. Schievink felt certain that the leak existed, despite being unable to find it with the best technology available. He explained that venous fistulas like the one he repaired last October are very small, more like the size of a capillary than a vein. So it is possible for imaging to miss them.

My wife's spine is riddled with perineural cysts. The presence of these cysts is not a problem by itself. Many people have spinal cysts without ever experiencing any kind of problem. But it seems that venous fistulas are more likely to attach to a cyst than to any other part of the dura mater because the membrane is thinner where cysts bulge out.

The good doctor offered the option of having him place aneurysm clips on the two cysts that he thought were probably the most problematic, based on his experience as the world's leading expert in this condition and having performed thousands of these surgeries. He couldn't guarantee that this would solve the leak. But he suggested a 70-75% chance of success.

My wife said that there was no way she was going to just turn around and go home without making some attempt to fix the problem. Going home would certainly leave her with CSF leak problems, while she only might continue to have problems if she had the surgery. So we went ahead with the surgery.

The surgery occurred late in the day because it was done on the same day as her third DSM. Unlike our first three visits to Cedars-Sinai, the hospital had many COVID-19 restrictions this time around. Only one visitor was allowed and only at certain times for limited periods. But with my wife being one of the final surgery patients of the day, I was able to spend quite a bit of time with her in post-op.

It was late night by the time my wife was settled into her in-patient hospital room. It was directly next door to the room where she had stayed last October. By luck of the draw, she ended up with the nicest, largest hospital room I have ever seen. It had its own breakfast nook. We were told that the room had been occupied by many celebrities. But once my wife was settled, they kicked me out. The residential hospital wing was very serious about limits on patient visiting hours.

Being kicked out wasn't a bad thing. I walked a block and a half back to our Airbnb apartment, which is the nicest Airbnb place we have ever been in. It was a penthouse apartment in a nice, safe building that had a private gated garage and a swimming pool. It featured two bedrooms, two full baths, a nice living room, full (but small) kitchen, nice dining room, and a balcony. I'm guessing that it was reasonably priced due to the pandemic. I crawled into the king size bed and quickly dozed off.

I was pleased when I returned to the hospital the following day to find that my wife no longer required caffeine to regulate CSF leak symptoms. Dr. Schievink dropped by to check on my wife. He asked how the surgery had gone for her and then cheerily stated, "I had fun!"

The previous surgery was in the lower thoracic portion of the spine that curves in. This surgery was in the mid thoracic section that curves out. So the surgical site is right where the back normally touches against the back of any chair. This means that the recovery has been somewhat more challenging than for the first surgery.

The great news is that recovery is proceeding well. Following the required four-week wait, my wife recently started prescribed post surgical physical therapy to help rehabilitate the back muscles that were affected by the surgery. So far things look good, so we are optimistic.

Still, the doctor could not guarantee that my wife will never spring another CSF leak. Chances for developing a third spontaneous CSF leak are statistically very low. But the possibility still exists. To me the chance might seem higher for someone who has already developed two leaks, but Dr. Schievink suggested that his experience dictates otherwise.

So for now we are going forward optimistically. My wife has hope of getting back to life without restrictions following physical therapy. Compared to when the local specialist who told us that my wife would be permanently impaired, this is very welcome news indeed.

This has been an interesting (and expensive) journey. We started out with many MRIs and hitting dead end after dead end. Local specialists were nice but were ultimately not very helpful. After months of disappointing treatments, we discovered the Spinal CSF Leak Foundation through an internet search.

This led to my wife tuning into portions of their annual Leak Week symposium online. When she admired Dr. Schievink's presentation, I searched and discovered that he and Cedars-Sinai Hospital where he practices were both preferred providers on our insurance. After months of disappointment, this seemed almost too miraculous to be true.

We discovered that spontaneous CSF leaks are tricky. The expert team at Cedars Sinai tries to approach each case with care and caution. So far it has required five trips to LA to get to this point. But we kept feeling that we were on the right track.

If you or someone you know is dealing with CFS leak symptoms, please know that help is available. Maybe not locally. It is, after all, classed as a rare disease. But there is hope for once again living a normal life.