6.4 Well, At Least I Won’t Die from It

 

By Stephen P. Fleming, MBA (USA)

Those words eased through my mind when I learned my diagnosis of trigeminal neuralgia (TN).

That was in 2009, and I was in my late 40s. In hindsight, there had been signs of what was to come. For example, one morning at the breakfast table I noticed a slight pain on the right side of my face, just above my teeth. This “twinge” only occurred as I was eating and eventually went away, only to return weeks later. After a few cycles of this, I reluctantly made an appointment with my dentist, thinking I must have a bad filling—here comes the drill!

If only.

At first, my dentist thought that maybe this was a lingering sinus infection, so she prescribed an antibiotic. But the twinge lingered—barely noticeable and only when I ate, and just during breakfast. Back to my dentist, where I made the self-fulfilling statement, “I still think it’s this tooth with a bad filling,” to which my dentist gladly agreed. Because the filling was extensive, this meant that the tooth required a root canal procedure by an endodontist. As root canals go, it proved rather easy.

Unfortunately, instead of this being the end of my story, it was just the beginning of my unexpected journey.

I’m a very blessed individual with overall good health. I have a loving and incredibly supportive spouse of 36 years and two beautiful daughters. They have helped me serve as an aging-services CEO for the past 25 years, and along with my work team, they have allowed me to lead in ways I could only imagine. And unlike so many who may be experiencing this disease, I have never been fully debilitated by TN. So, in so many ways, I have been blessed. Although I hesitate to “tell my story,” I hope that it can lead to someone else’s understanding of TN and how one can still lead a full and joyful life in spite of it.

Soon after my root canal, the “twinge” paid a return visit. Once again, the endodontist found no abnormalities. In time, the twinge became a pain far beyond a breakfast companion—it hurt even when the wind blew against my face, when I showered and water hit the right side of my face, and when I brushed my teeth. At this point, however, I turned to the internet, which revealed to me the condition known as trigeminal neuralgia (TN).

A few aging-services colleagues led me to a local neurologist who conducted a complete work-up. “This could be a brain tumor, or it could be multiple sclerosis, but it’s most likely trigeminal neuralgia.” Several subsequent MRI scans proved him right, and he prescribed the tried-and-true medication: carbamazepine (Tegretol).

Carbamazepine’s side effects were significant: I had trouble staying awake at work, especially in the afternoons, and I had trouble with memory recall. Everything took just a little longer to get from my memory to my mouth; at times, this was very embarrassing, especially when I ran into someone whose name I should have easily recalled.

Worst of all, the initial dose of carbamazepine was not effective at alleviating the pain, so my neurologist threw in gabapentin, a medication used for nerve pain. Although ineffective, the side effects were unbearable, and this medication was stopped. On top of this, a brief steroid treatment made everything worse.

It hurt—sharp, knife-like pain—in the maxillary branch, referred to as V2, of my trigeminal nerve. This is the area above the upper lip, in my case, on the right side of the face, running diagonally up the cheek to the temporal area. Normally, this pain lasted only several seconds to maybe a minute, although it often felt longer. At its worst, the pain brought me to my knees and seemed to present more frequently, with the time between episodes decreasing—so much so that, after a day of multiple attacks, a dull ache ensued, only to be replaced by an episode of truly debilitating suffering.

Tweaks to my treatment regimen continued. As is common with TN pain management, my neurologist kept increasing the dosage of carbamazepine until I reached about 1100 mg/day. Eventually, the pain began to subside. Whether it was the correct dose of carbamazepine or simply my time for a pain-free “holiday,” the pain became manageable to the point of being non-existent.

After a few years on medication and the side effects that go along with it, I began contemplating a more invasive procedure to, perhaps, provide a longer-term solution. Again, through online sleuthing, I learned of a surgical procedure known as MVD, which I discussed with my neurologist. Here, he was cautious, spending more time on the risks than the benefits of going under the knife. He ultimately referred me to two neurosurgical specialty groups.

My wife, Anne, accompanied me on these consultative visits, and in addition to helping me “hear” what the surgeons were saying, she was and continues to be a supporting, understanding, and patient counselor. Relationships are vitally important when living with TN. They must be purposely maintained, nurtured, and grown. In my case, it was my spouse who was there for me. Not everyone is that fortunate; regardless, find your supporter. It will greatly improve your outlook and ultimate resolution of living with TN.

With neurosurgeons—as with most any relationship, especially one where you’re interviewing someone to cut a hole in your skull and perform microscopic surgery—you want to feel a connection, as well as confidence rooted in knowledge:

• How did they make you feel?
• Did they take the time to carefully explain your options?
• Were they empathetic to your plight?
• How experienced are they with your condition, and how many similar surgeries have they performed?
• Where were they trained, and who did they train under?
• Is MVD surgery their specialty or subspecialty? …

After asking all of these questions and talking through who we “connected” with, Anne and I chose a neurosurgeon.

On November 16, 2015, he performed my MVD, a surgery that lasted 3 hours, to address both arterial and venous compression of the trigeminal nerve. Having been prepared to anticipate about a 90-minute surgery, Anne became worried. My situation was simply a bit more complicated than expected.

The whole premise of the MVD procedure is to “decompress” the trigeminal nerve and, in my case, to keep the artery and vein from touching or compressing the trigeminal nerve. Most neurosurgeons do this through the placement of a simple piece of Teflon-coated fabric or spacer between the nerve and, in most cases, artery. Although this sounds simple, it is far from it. The neurosurgeon must weave his way through the base of the brain, avoiding other nerves, including the auditory nerve for hearing, to reach the compressed area. Think of that classic childhood board game “Operation,” in which you “play” surgeon, removing as many objects as possible from the body without touching the sides of an electrified “cavity.” If you touched the side, a buzzer sounded, and the nose of the clown-like figure on the game board lit up to let you know you had lost your turn. Similarly, with MVD surgery, too many touches of nerves are not good, and there are, in fact, probes used to effectively monitor activity to best ensure that the surgeon is steering clear. Yes, it’s a delicate surgery.

My surgery went well, with no complications and a speedy recovery, and even with me returning to work within 2 weeks. Pleased, I was completely off my medications within the next 3 years. Relief, at last.

Unfortunately, I was an outlier, and my MVD procedure did not keep the condition from returning as long as most.

In 2018, my pain returned—with a vengeance. This time, I probably waited too long to up my carbamazepine dosage, and as a result, I had a hard time getting the pain back under control. My local neurologist referred me to a neurological specialist in the non-invasive treatment of TN. He was also a renowned researcher, working to find new paths to alleviate the pain associated with TN. He prescribed several new medications, most of which required the use of a “compounding” pharmacy. These new meds helped me get my pain under control, so much so that I could get back on my normal dosage of carbamazepine. The new meds consisted of a compound nasal spray of a small dose of ketamine. This proved extremely helpful in “settling” the nerve, so that the carbamazepine could do its more regimented work. Through this experience, I learned of the correlation between high blood pressure and pain. Pain, unabated, will cause blood pressure to increase, starting what I call a “pain spiral,” where one plays off the other, especially in TN. Getting blood pressure under control is an important side aspect of controlling TN pain.

In addition to the neurological specialist, I returned to the neurosurgeon. Even though the MRI scan he ordered showed that the Teflon spacer was still in place, he recommended that we try a nerve ablation procedure known as a gamma knife procedure to partially ablate (destroy) the trigeminal nerve. The key here is to burn and scar the nerve just enough to stop the pain—but not deaden the nerve. For most TN cases, the procedure is expected to offer temporary relief with relatively low levels of risks—say, perhaps, up to 5 years, on average, before any pain recurs. But should it recur, the pain would most likely be less.

For me, this was worth trying, so in 2018, I had the gamma knife procedure. The outpatient procedure itself is rather benign; the only discomfort—despite Novocain injections just under the scalp—came with the mounting of a stabilizing “halo” device into the skull. The halo locks your head in place during surgery. For obvious reasons, you don’t want your head wiggling around while a pinpoint laser beam is directed at it! In all, this lasted only 30 minutes and was akin to an MRI scan, although in a much more open setting.

Free of the halo, I drove myself home around midday.

Unfortunately, I cannot say that the gamma knife procedure produced great results for me. In the end, I could not stop my medication, as I had after my prior surgery. But I was able to get the pain under control and avoid all but the carbamazepine. So, there were some positive effects.

I have since sought an additional neurosurgical opinion from a well-respected neurosurgeon. After reviewing my nearly 4-year-old MRI results, he determined that my Teflon spacer was most likely not fully decompressing the trigeminal nerve. Simply put, the nerve and artery were still in contact with one another, just what the initial surgery hoped to correct. To confirm this, I would need new MRI imagery, and if these images proved definitive, the possible “fix” would be a second MVD surgery. By this point, I’m not of the mindset to pursue a second MVD. I tolerate the carbamazepine well, and for the most part, I remain pain-free at a dosage of 900 mg/day.

I’ve learned many lessons over the past 12 years.

The first was to take the time to do more research. For example, I did not learn of TN Support Groups until well after my second procedure. I’m not sure that having this resource would have changed the course of my condition or the decisions I made to treat it, but the information most likely would have allowed me the option of consulting ultra specialist neurosurgeons who are nationally—and, in some cases, internationally—known for their surgical treatment of TN.

Perhaps more importantly, however, I would have learned that I am not alone in this condition. Even though it is rare, there are still thousands of people with TN like me, and we all need a support system and educational offerings for treatment options. Support Groups provide that. I wish I had found them sooner.

Second, I wish one of my physicians had informed me that any improvement is a win, something I realize and appreciate now. I incorrectly thought that I could have the surgery and that would be it, at least for a long while. Although statistically correct, that’s not always the case.

But after all the treatments, my condition has improved and is better than when it started. I’m now relatively pain-free, have few side effects, and enjoy a full life. I have a wonderful family and have enjoyed a great career in aging services.

So, life is good!

For me, TN was just one of those bumps in life’s journey. I empathize with those who have experienced complete debilitation from TN. I hope and pray they find treatment options that provide at least some relief. Through the work of the Facial Pain Association in the USA and many others, research is ongoing to assist with this most painful condition.

Finally, I want to thank my neurologist and neurosurgeon who provided me with a better quality of life.

My wish is that you find that, too.