Thursday, May 9, 2019

My undiagnosed chronic facial pain finally has a diagnosis

After waiting 11 months for a second opinion consultation I saw a neurologist at University of Michigan's medical center last week. He was worth the 11 month wait. Not only did he give me a diagnosis, he also gave me more insight into the condition and future testing options. But equally meaningful? He gave me honesty, empathy, and appreciation for how much work I had to do on my own since most of the medical professionals I'd sought help from for over 7 years had been kind but unhelpful at best, and condescending and dismissive at worst.

To be clear I have four unrelated (three are considered rare) conditions all currently going on at the same time and all "in my head." Literally, not figuratively. LOL. I'm listing them in order of their significance during this consultation:
  1. Undiagnosed, chronic, facial pain that began in Nov. 2010 following a dental procedure.
  2. An undiagnosed, soft tissue mass on my upper, right gum located directly between the two areas of excruciating chronic facial pain. Three medical professionals all gave me only one option a year ago when it formed to excise (cut it out) of my gum. I was afraid such a procedure could further injure my already damaged trigeminal, facial, nerve more (if it was entangled with or beside it) worsening the level and/or frequency of pain I already deal with.
  3. The discovery in February during a subsequent MRI that I have a congenital brain variant (in layman's terms: variant = defect) called a "Segment A1 Anterior Hypoplasia."
  4. The inoperable, grade 2, deep thalamic and mid brain gliomas (aka lesions and tumors) that at this point are not a concrete diagnosis because no biopsy will be done due to their locations but two radiologists, a neurosurgeon, and two neuro oncologists all concur that is what they most likely are. They showed up on the first cranial MRI that was done to try to determine a cause for the chronic facial pain in April of 2018.

1. A Diagnosis for the Chronic Facial Pain

After many long years, (even longer than this sentence LOL) including a visit to a different neurologist a year ago who was unable to make a diagnosis even after an endodontist suggested he thought it could be trigeminal neuralgia, the neurologist at UofM gave me a diagnosis of trigeminal neuralgia. (If you're curious you can Google the name of the condition or read a previous post where I described the condition in great detail with diagrams.)

When it was first suggested last April it was a devastating proposal. In brief it's an excruciatingly painful chronic condition that can worsen over time and has no effective long term treatments (pharmaceutical or surgical). Mine is short of classic symptoms in that most people develop sensitivity to the outside of their face, meaning their cheek(s), that can trigger an attack. I had pain both in my gumline and inside my right nostril, up in my sinus. I also discovered last year that the weight of my reading glasses on the bridge of my nose, pressure on a particular molar on the upper right side, as well as food, both content and hardness, were also triggers that could all set off an attack that lasted several days, several times a month. Sometimes the pain covered larger areas and lasted weeks. During this time it was almost impossible to chew or brush my teeth. But I'd still do both because they are necessary.

He also said if I have trigeminal neuralgia I have the best kind. Translated he meant that I have a very uncommon type, and the where with all, to have found a way to alleviate most of my pain through my diet and eating only soft foods since I began practicing the macrobiotics 13 months ago. He said most patients aren’t able to find their own solution. It's wonderful the macrobiotics helped give me the upper hand on the pain, but I know the condition is still there. It's as if it's lying in wait, ready to pounce if I eat the wrong thing or get so stressed I clench my jaw when awake, or grind my teeth at night.

Between the medications and/or surgery, used to treat trigeminal neuralgia, neither are a sure thing for effective or lasting pain relief. Because it is a rare condition, there are no medications made for it. Epilepsy anti-seizure medications and antidepressants are used off-label because there are no other options. But instead of treating the trigeminal nerve, they impact your entire brain. From what I researched they often only work for a short period of time and/or can cause debilitating side effects. The surgeries and physical treatments to the nerve itself are also often short lived results with pain returning and/or damage can be done that either causes the pain to worsen, or can cause permanent numbness in the face, which makes things like chewing difficult.

He said trigeminal neuralgia is rare but not that rare. That all of these doctors and dentists should have been willing to consider it as an obvious cause of my facial pain. He was genuinely surprised no one had referred me to a neurologist for over 7 years, and that once I saw one I got no further than where I'd been before I walked through his door. He also shook his head that some didn't believe there was anything serious causing my pain. He seemed as incredulous as I still am that no other doctor had helped me by giving me a referral to a neurologist for so many years while I suffered. The burning, searing, stabbing pain in my face I'd described to all of them were classic nerve damage symptoms. I didn't realize that but as medical professionals (imo) they should have.

Like I said, he was worth the wait and the 4+ hour drive to Ann Arbor. I was so happy driving home. I finally felt like I'd been heard. I've read it takes most people 10 years or more to receive a diagnosis of trigeminal neuralgia so I'm actually ahead of the curve. It only took me 8.5 years.

I told him I didn't need a diagnosis to have a label... I wanted it to know what to learn about to make sure I'm not doing things inadvertently that could make the situation worse as well as learn what can I do to make it better?

He told me I'd done a really great job getting as far as I have.

After not being believed or taken seriously for so long just hearing him say that made me feel great.

He is also making recommendations to the neuro-oncologist I'm seeing in Grand Rapids to add some other tests to the next MRI I have, if I ever have another MRI, that he feels might be helpful in gaining more insight into my situation.

2. Oral Mass

The undiagnosed mass in my mouth was another reason I wanted to see this Doctor. The mass appeared in February of 2018.

My Dentist was convinced it was an abscess as the result of a nerve dying, in a tooth that hadn't caused me any pain or senstivity, and case closed, I needed a root canal and I'd be fine. Completely dismissing I'd been in pain in that area for over 7 years. Additionally he said I could have the "abscess" removed (cut) out of my gum or treated with antibiotics if needed. He wasn't willing to consider any other possible alternative and didn't seem to believe I had been living with chronic pain for so long. He went so far as to tell met that even though my dentist in California who triggered the condition said it was possible he'd accidentally damaged a nerve giving me a novocaine injection, my new dentist in Michigan said that was so unlikely he wasn't willing to consider it. He then went on to do everything in his power to not release my dental records to me before my first neurology appointment last spring.

For over a month I requested them in person, over the phone, and by email. I received call backs but always as another hurdle I was supposed to jump to be able to receive my records. In the end I had to make an hour long, roundtrip drive, the day before my neurology appointment only to be told they didn't even have my records from California. WHAT? When I'd first called the California office they told me they didn't have them either. Ugh. When I called California back that day I was told they did have my records, they were just in a different part of the computer system. I cannot tell you how much stress this situation created. I even consulted with an attorney not to sue my MI dentist but just to ask if I had a legal right to my medical records because I was beginning to doubt I did based on his refusal to give them to me.

So my Dentist, an ENT, and the Endodontist I'd seen all wanted to cut into my gum to remove it the mass and send it to pathology only if it looked suspicious. None were willing to do a needle aspiration to see if it was the infection they all thought it was. But the Endodontist and ENT both commented it wasn’t a typical abscess because it didn't look like one. At the time there was no redness or obvious swelling but I could feel a mass approximately 1/2 in height, and 1/4" in width, beneath the gum line that was causing a prickling, pins and needles sensation in my gum directly between the two areas of chronic facial pain.

The first neurologist I saw in Grand Rapids listened empathetically, looked at my gum line, and felt the mass with a gloved finger. Then made no recommendations about what it was or how to treat it after the discovery of the gliomas upstaged it.

Still fearing that removing it might cause more damage to the already damaged trigeminal nerve I chose to not allow them to excise it and waited almost a year for the second opinion consultation with a different neurologist.

It wasn't painful but I did, and still do, feel its presence as it feels like pressure in my face even when I don't touch it and has a pins and needles sensation if I do press on it either inside my mouth or through the skin on the outside of my face.

The neurologist I saw last week is not an expert in oral masses but in his opinion it looked like a granuloma. My next question was: "What is a granuloma?" He told me a granuloma is a small, foreign particle that somehow got below the skin and caused tissue to build up around it. It could happen during a dental procedure like having your teeth cleaned, or simply eating, or I read online even a bacteria that enters a break in the skin can trigger your body to create one. The body tries to wall off the foreign matter by creating layers of tissue to surround it, then tries to push it out of the body. Which is exactly what mine has done over the course of the past 14 months.

In the past year the mass changed. It went from being below the surface of my gum to becoming a protrusion on the surface of the gum. It's also much smaller (around 2mm in diameter) than it was before having shrunk significantly in size when I started practicing the macrobiotics. It has broken open and bled a few times but hasn't gone away. A common symptom of granulomas.

It's worth noting the second Neurologist thinks the reason I was able to feel the mass developing (far earlier than most people would be aware of it) was because of the trigeminal neuralgia making that area of my face extra sensitive. That explains why the Endodontist and ENT both said they'd never seen an abscess like it before but it wasn't yet a visible granuloma they might have recognized as such.

He suggested I may want to go back to the endodontist and ask him to remove it by cutting it off with a scalpel. Since it now protrudes (and is no longer beneath the skin) there’s no longer a danger of damaging the trigeminal nerve more.

I've since read that they often go away on their own within two years. At this point I've decided to wait and see if it will and will talk to my macrobiotic counselor at our next session to see if there is a holistic way to help reverse it.

This Neurologist also suggested if it gets worse the best type of doctor for me to consult with might be an Oral Maxillofacial Surgeon. They would do appropriate vascular scans of the trigeminal nerve to make sure they wouldn't damage it more while removing the granuloma. So I am placing no blame or fault on my former Dentist, the Endodontist, or the ENT for not recognizing what it was. I only wish one of them had been willing to either do a needle aspiration which would have shown it wasn't an infection, or made a referral to an oral maxillofacial surgeon who would have been in a better position to help me figure out what was going on.

How to be Your Own Health Advocate:

What I'm about to say excludes prompt and/or necessary emergency treatments or surgeries for both acute and chronic conditions. I am not suggesting you enter a state of denial or procrastination. But if you have something like a granuloma, you can afford to take the time if you're willing to dig deep and do the research, to try to figure out exactly what is going on.

In this instance I had the luxury of time, and refused to allow multiple medical professionals to proceed with a treatment my gut was telling me not to do.

  • Had I proceeded the way my former Dentist wanted to I would have ended up with an unnecessary root canal and possibly additional damage to my trigeminal nerve.
  • Had I allowed the Endodontist or ENT to remove the mass I may have ended up with additional damage to my trigeminal nerve.
I am not a health professional but I did my own research. I turned over every rock I could find. I kept reading, looking, and asking questions until something made sense and I found the correct path to take. Despite facing a lot of pressure and dismissiveness from my Dentist last year I did not yield and took the 12+ months that were necessary to finally receive an answer, from a medical professional, that made sense to me.

3. Segment A1 Anterior Hypoplasia

About the new brain issue they found in the last MRI the Neurologist said he said he’s never heard of a Segment A1 (first segment), anterior (front of the brain), hypoplasia (malformed artery in the front of my skull) being found using a standard MRI. That a different MRI with angiographic resonance is needed to see them.

How to be Your Own Health Advocate:

When I called UofM the day before the appointment to make sure the neurology department could see the same records the neuro-oncology department was able to view last summer, they could see all of my MRI results from 2018 via a shared network the hospitals use. But, not the results from my MRI in February 2019 when this variant was found. So, I requested a CD then drove the almost 1.5 hour round trip drive to GR the day before to pick it up in case the Neurologist wanted to see the scans. He did. I could have asked that the information be placed in the file but didn't want to have to risk it wouldn't be there during my appointment the following day.

Apparently the hypoplaysia does increase my risk of an ischemic stroke and a worse outcome if I ever have one. So I'm planning not to ever have one since there isn't anything that can be done to correct it. So that’s that.

4. Thalamic and Midbrain Gliomas (brain lesions/tumors)

This appointment had nothing to do with the discovery of the gliomas. Though the Doctor did share an insight with me on why he felt the chances of the suspected gliomas were most likely not scarring because there are no bones in proximity to the thalamus that could cause damage that would produce scarring. Which doesn't mean the first neuro-oncologist was wrong to suggest it. I've read enough information online to believe and know that the migraines I suffered from for years could have caused scarring to occur. So, basically, it was confirmed that the diagnosis of the spots as gliomas can't be confirmed since a biopsy isn't recommended due to their locations.

Additionally, the Neurologist said he would try to help me find a dentist experienced at working with people with trigeminal neuralgia. He doesn’t know of one personally but would ask around the Neurology, UofM Dental School, and the oral and maxillofacial departments to try to find someone who could help me by find a dentist aware of the issues associated with trigeminal neuralgia. In most instances it is inconsequential. But if I ever need injections or when having work done, I will feel more at ease knowing my dentist will be as careful as possible to not exacerbate the damage that's already occurred.

I've also decided I am going to write a letter and send it to the 9 doctors and dentists who didn't suggest I needed to see a neurologist. Just a short, sweet, single page that explains the diagnosis and how I don't need a response or apology, I just wanted all of them to know so that if in the future they have another patient like me they'll show more compassion, consideration, AND give a recommendation or referral to a neurologist so the person can get the help they need.

It shouldn't be this hard for anyone but I know it is for many dealing with unusual or rare conditions.

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