YA Jaw Update: Part III, the return

Tom the Melaniephile · Apr 27, 2023

Well, a bit over 15 years later the dental Xray shows a hole in my jaw again. Last time it was an ameloblastoma (rare, noncancerous but locally aggressive tumor). Fortunately it looks smaller this time, but it's around the root of another tooth - so that one's a goner if it is a recurrence.

Ameloblastoma - Symptoms and causes

www.mayoclinic.org

YA Jaw Update - 2009 Part II

Prior thread with links to even earlier backstorySo, bone graft went well. Healed nicely.Monday, I went in and was put under to have the 3 screws pulled (see photo prior thread) and 2 posts put in for implants. Opted for the general.Took digging in 3 different areas for them to actually get an...

arstechnica.com

After some back and forth, my health insurance is going to be covering me (well the normal 80% anyway).

Oral surgeon consult in a few hours, and my anxiety is through the roof. The original biopsy was by far the most painful experience I've ever had - the local anesthetic did nothing and the doc was rooting around in my jawbone for 20 minutes of "let me give it one more try to get a bigger piece." Not an estimate - there was a clock in sight. I have always needed a lot of novocaine (or whatever 'caine) - I remember with prior dentists they would have to do 3 rounds of "Okay, this should numb you up, I'll be back in a few" "What, you can still feel that?" The dentist I've had since 2006 was great - confirmed it once, then every time after he gave me enough without having to come back and add more.

On top of that narcotics have little effect on me, so I'm concerned about being flagged for "drug seeking behaviour" if I ask for the heavier stuff to use after a probable surgery.

I am seeing a different oral surgeon this time. Besides the biopsy experience the original oral surgeon did a crummy job placing the implant posts.

Gah. Not been a good spring in several respects.

fitten · Apr 27, 2023

Thanks for the info... if I ever have to have a bone biopsy, I'm telling them to knock me out.

Good thoughts, hoping for the best!

Tom Foolery · Apr 27, 2023

Sorry to hear, fellow Tom. I will echo @fitten in sending good thoughts and hoping for the best!

timezon3 · Apr 27, 2023

Good luck. Dental work is perhaps one of the most unpleasant things in life. I'm thankful that drugs have a good effect on me, but I know not everybody responds as well as I do, and that's gotta make things tough. Can you ask for Ativan to help with the mental aspects of it? When I had a root canal done, I had Ativan + nitrous + novocaine and it was nearly as good for me as just being knocked out.

Tom the Melaniephile · Apr 27, 2023

Appreciate the thoughts.

With better imaging at the oral surgeon, it's not one location of concern - it's two, and the second one includes the main nerve on that side of the jaw. The likelihood of a more radical treatment plan increased significantly.

So, sometime I should be getting a call to set up the dual biopsy appointment.

Carhole · Apr 27, 2023

Jesoouch. I’m missed the original installments so wow, what a thing to endure.

Don’t be concerned about the medicine; thankfully the overreaction in the US to over-prescription and blatant grey market sales of opioids is starting to even off a little bit and legitimate pain patients don’t get treated as often as criminals as we used to, with a big serving of YMMV and all that. Legitimate reasons for wanting to knock out anxiety==ask for help there, then you can get to the important part in dealing with the procedure. It sounds like a general anesthesia could be warranted so have the discussion openly regarding your tolerance and they can use alternate means to send you off. Post-operative pain is more challenging, and you should plan to have a strong but short treatment on what would be a serious medicine for most (such as oxycodone or fentanyl) at your doctor’s recommendation, with a sound plan to get you tapered down to lesser medications as you heal.

The involvement of nerves may be worth consulting on that issue alone. Loss of the branch past the point of your pathology should be well understood prior to the procedure, as well as possible complications and remedies such as ablation to shut up potential nerve pain(s). You may end up being a lucky candidate to have anything painful simply blocked locally for the procedure, then if the tract is cauterized where your lesion is, hopefully there’s a simple lack of feeling. Things aren’t quite so simple around a surgery site so learn as much as you can from the team ahead of time. They do not want you to suffer and will work with you. Also, ask for a decent diamond grille or a T-1000 smile while they’re messing around.

Hoping for the best.

Tom the Melaniephile · Apr 28, 2023

Potentially good news, and I probably should hop over to the Observatory and ask for some expert help.

It's good for me that the science has moved on since the first round in my jaw. There's a high correlation with a handful of genetic mutations (especially BRAF V600E, followed by SMO). BRAF V600E mutation is associated strongly with an "early" ameloblastoma matching my age at onset, and with location in the jaw instead of the skull.

BRAF V600E is also associated with malignant melanomas (normally that wouldn't be good) - but it means there's an existing approved drug protocol to block the signalling pathways triggered by BRAF V600E- and it's been used successfully for a small number of ameloblastomas!

Still a long way to go to make this happen. Emailing the oral surgeon to request Prior Authorization for the genetic screening is the next step.

Tom the Melaniephile · Apr 28, 2023

Just to complete the circuit, here is the Observatory thread: https://arstechnica.com/civis/threa...n-datamining-contacts-help-requested.1491677/

yd · Apr 28, 2023

Had trouble reading through this. Sending you good vibes. Also, if it were me, seek out the fucking drugs, all of em.

Tom the Melaniephile · Apr 28, 2023

Appreciate the vibes.

Carhole · Apr 30, 2023

Tom the Melaniephile said:
Potentially good news, and I probably should hop over to the Observatory and ask for some expert help.

It's good for me that the science has moved on since the first round in my jaw. There's a high correlation with a handful of genetic mutations (especially BRAF V600E, followed by SMO). BRAF V600E mutation is associated strongly with an "early" ameloblastoma matching my age at onset, and with location in the jaw instead of the skull.

BRAF V600E is also associated with malignant melanomas (normally that wouldn't be good) - but it means there's an existing approved drug protocol to block the signalling pathways triggered by BRAF V600E- and it's been used successfully for a small number of ameloblastomas!

Still a long way to go to make this happen. Emailing the oral surgeon to request Prior Authorization for the genetic screening is the next step.

The study is very promising, and I hope that you get a near opportunity to speak with a medical oncologist about setting you up as a patient to undergo the neoadjuvant treatment.

What I really like is how much emphasis was noted in your linked study concerning mandible-preserving outcomes. When you get seen by a professional they’ll ascertain the defining characteristics of your recurrence as the first step (local, locally advanced, etc), via imaging and likely the biopsies you had feared but maybe not depending on your case’s aggression—your old jawbone chunk may be in a freezer somewhere for retrieval, then all of the above will help inform everyone on your best choice of treatment. I doubt it will be the horribly invasive surgery that was originally posited in light of the advances of the immunotherapy unless there is distant metastases or activity suggesting it, and even then my money is on more recent means to preserve the jaw and reconstruct anything that’d look funny or impact your ability to chew or speak.

They will learn how to kill the growth(s), and get you back to feeling as good as possible. In a best-case scenario you may not need surgery with an effective immunotherapy and ongoing suppression of the mutation’ expression.

Keep us posted.

Tom the Melaniephile · May 11, 2023

Minor update - biopsy is scheduled for 5/30. Not sure why it takes so long to schedule, but it is what it is.

My GP does seem willing to give a referral for a second opinion from an oncology perspective - MD Anderson was discussed.

Tom the Melaniephile · May 17, 2023

Pretty frustrating when I reach out to the (apparently) appropriate group from FDA, and the response is basically "Sorry about your troubles, have a look at clinicaltrials.gov and PubMed!"

Dude. There isn't shit on clinicaltrials.gov for ameloblastoma, and yes I've been using both PubMed and direct Google searches.

WM314 · May 17, 2023

What were you looking for from FDA? I wouldn’t have expected much of a role for them in your situation?

Carhole · May 17, 2023

WM314 said:
What were you looking for from FDA? I wouldn’t have expected much of a role for them in your situation?

Likely trials, and approved treatments based on his other thread but right, getting into a good cancer center is going to probably provide the best actionable plan being established and they will know anything from clinical trials and likely have their own underway as well, so the team work-up of the case can’t be stressed enough as the best way to go. If MD Anderson is being an asspain about evaluation and opening the door to you, keep bugging any good cancer center within reasonable range. It’s highly relevant when talking to admissions your reasoning for wanting doctors with the most recent treatment options because this is such a highly invasive surgery—the cosmetic factor is important here. Both insurers and doctors alike will want to do the very best to leave you without visible disfiguration, and speaking openly with anyone on the phone about your own concerns in this area and others is total fair game.

I only stick to those guns as vehemently as I do in that you’ve already unearthed multiple possibilities that an academic cancer center could harness to reduce the volume of affected tissue thus providing a better overall elimination of the recurrence and the least scarring operation(s) needed to get you well again. Keep advocating for yourself, and sorry for your frustrations.

Tom the Melaniephile · May 17, 2023

Carhole said:
Likely trials, and approved treatments based on his other thread but right, getting into a good cancer center is going to probably provide the best actionable plan being established and they will know anything from clinical trials and likely have their own underway as well, so the team work-up of the case can’t be stressed enough as the best way to go. If MD Anderson is being an asspain about evaluation and opening the door to you, keep bugging any good cancer center within reasonable range. It’s highly relevant when talking to admissions your reasoning for wanting doctors with the most recent treatment options because this is such a highly invasive surgery—the cosmetic factor is important here. Both insurers and doctors alike will want to do the very best to leave you without visible disfiguration, and speaking openly with anyone on the phone about your own concerns in this area and others is total fair game.

I only stick to those guns as vehemently as I do in that you’ve already unearthed multiple possibilities that an academic cancer center could harness to reduce the volume of affected tissue thus providing a better overall elimination of the recurrence and the least scarring operation(s) needed to get you well again. Keep advocating for yourself, and sorry for your frustrations.

MD Anderson isn't being an asspain yet. Wanting me to have an official diagnosis first is pretty reasonable.

....of course, taking over a month to get a biopsy is really, really annoying.

They're the only place in Texas participaing in ComboMATCH:

https://ecog-acrin.org/clinical-trials/eay191-combomatch/

Tom the Melaniephile · May 28, 2023

Okay, biopsy is a go for (stupidly early on) Tuesday!

Of course, when the hospital called me to confirm details they described it as "removal of two bone lesions" - which sounds to me like a notably more significant undertaking than "biopsy". Not a complaint - I'm going to be sedated, take out as much as you want to - just leave my mandibular nerve intact and close up the holes before I wake up. Probably won't be getting the genetic screening immediately, just having the samples retained for future genetic screening. Frustrating, but I can understand the logic.

I already have my prescriptions (antibiotics, pain meds, oral rinse) - so that's a good sign for good organization in the oral surgeon's office. Antibiotics and oral rinse start tomorrow for prep.

We're now 6+ weeks since the initial alert from the dental Xrays. While I couldn't feel anything at the time, or at the initial poking by the oral surgeon - I've been feeling internal achiness/pain/pressure in the jawbone for weeks now. Not terribly intense, more a constant background irritation.

fitten · May 28, 2023

Good thoughts and speedy recovery!

Tom the Melaniephile · May 28, 2023

fitten said:
Good thoughts and speedy recovery!

Appreciated!

Unfortunately, this is just the first phase. I'm still hoping to avoid the "radical resection" by using drug therapies proven in cancers with the BRAF V600E mutation (the vast majority of mandibular ameloblastomas have BRAF V600E). Totally not standard practice for ameloblastomas, since they're "not cancer" and really rare. Biggest trial I could find was 12 patients in Israel, but 100% had significant tumor shrinkage and bone regrowth in 6-8 weeks, the focus changed from radical resection to jaw preservation surgery.

Tom the Melaniephile · May 28, 2023

WM314 said:
What were you looking for from FDA? I wouldn’t have expected much of a role for them in your situation?

Apologies, I missed responding to this.

As noted above, the majority (and likely nearly all) mandibular ameloblastomas are associated with the BRAF V600E mutation in the tumor cells. FDA has made a series of approvals for a drug regimen to treat a variety of BRAF V600E linked cancers (Many melanomas, etc.). Unfortunately, it's never been approved directly for ameloblastomas.

The standard of treatment for mandibular ameloblastoma is "radical resection and fibular flap" - when I got some details from the oral surgeon and read from from someone who had it performed.... it's even worse than it sounds. Frankly I got lightheaded and had to ask the surgeon to stop with the description.

The following is probably both disturbing and TMI, you've been warned.

~8 weeks totally off work, followed by ~4 weeks of minimal part time. Permanent reduction in rigidity in that side of the jaw since it's now a chunk of fibula and calf tissue. Physical therapy. Speech therapy - ie, learn how to eat and speak functionally again. Quarter of my teeth gone. Permanent nerve loss - meaning numbness in a quarter of my face, half the tongue, etc. The chunk they take out of the fibula and muscle gets closed, but recovery on that takes quite awhile too and I'll always have a missing bone segment in the fibula, plus other fun.

Anxiety has been in overdrive (duh!) and now I need to try and sleep fairly soon. Ugh.

WM314 · May 28, 2023

Tom the Melaniephile said:
As noted above, the majority (and likely nearly all) mandibular ameloblastomas are associated with the BRAF V600E mutation in the tumor cells. FDA has made a series of approvals for a drug regimen to treat a variety of BRAF V600E linked cancers (Many melanomas, etc.). Unfortunately, it's never been approved directly for ameloblastomas.

The standard of treatment for mandibular ameloblastoma is "radical resection and fibular flap" - when I got some details from the oral surgeon and read from from someone who had it performed.... it's even worse than it sounds. Frankly I got lightheaded and had to ask the surgeon to stop with the description.

Yeah, we ended up discussing it in the other thread, but I suspect key for you is finding a physician willing to go off-label / start a clinical trial of 1 (neither of which is a thing where FDA will need to talk to you directly, which probably contributed to them being less helpful).

Anxiety has been in overdrive (duh!) and now I need to try and sleep fairly soon. Ugh.

Very understandable, both on the anxiety front and on the front of trying to not do a radical resection unless truly necessary. Best of luck Tuesday.

Carhole · May 29, 2023

Fingers crossed that you get some actionable pathology results and with little waiting time to fester. Gooooood luck, high fives, voodoo healing vibes and home brews aimed your way, Tom.

Tom the Melaniephile · May 29, 2023

WM314 said:
Yeah, we ended up discussing it in the other thread, but I suspect key for you is finding a physician willing to go off-label / start a clinical trial of 1 (neither of which is a thing where FDA will need to talk to you directly, which probably contributed to them being less helpful).

Yep, I've basically been casting around for leads. Realistically, it's probably premature since I don't have pathology confirmation that it's the ameloblastoma again.

Emotionally, I've been pretty frantic once I started feeling it in my jaw. It's incredibly unlikely to be something other than the ameloblastoma recurring.

In addition to FDA, I contacted:
rarediseases.org/NORD
National Institute of Dental and Craniofacial Research (NIDCR) @ NIH
Genetic and Rare Diseases (GARD) Information Center @ NIH

Too rare for the rarediseases.org folks (1-page description, 14 years out of date, zero subgroups for ameloblastoma). Actually I was able to give them a starter for an update of the page...
NIDCR, GARD basically offered nothing other than stuff I already know (PubMed, clinicaltrials.gov, rarediseases.org)

Actually, the best luck I had was the Mayo Clinic discussion groups - there were actually two other ameloblastoma patients.

Tom the Melaniephile · May 31, 2023

Well, saying yesterday was a "biopsy" was underselling it. I was put under a bit before 7AM and was out for well over 2 hours. I'm pretty sure the surgeon basically took out all the tumor material he could find without significantly cutting extra bone. I actually prefer him being thorough, so that's good. Surgeon did a really good job sewing up my gums. At home I was mostly in bed, mostly asleep til about 3PM. Slept poorly last night - insanely vivid dreams and I was waking up about every 30 minutes because I started to do something in the dream and I also did it for real (Raise my arm, talk loudly, etc)

Anaesthetic wasn't just propofol. It was 2 ampules of Versed, get rolled into the OR, propofol to knock me out (fast) nasal tube, then gas* to keep me thoroughly out. Beforehand they said something about fentanyl as well, which would explain the constipation I just resolved manually. Whatever they did, I ended up with an insanely sore throat, was periodically coughing up blood until around 5PM and had lots of annoying, crusty dried blood in my right nostril.

Lab report may take up to 6 weeks, there's a fair chance the lab will do a genetic test on their own - but if not, samples will be retained.

Mandibular nerve was pissed off, but recovering. Started out with numbing in the (left half of each) tongue, lower lip, lower face. Tongue is back to normal now, lip shifted to burning sensation yesterday evening and is still there, face is still numb. Same nerve recovery process as my original surgery 15 years ago, but the nerve recovery is faster so far.

My 20% OOP ended up being well over 2 grand between the surgeon and the facility fee. Someday I should get a separate anesthesia bill in addition. Isn't American healthcare the BEST? /s

*I had asked what the gas would be and they told me beforehand, but I'm not certain of what it is. Of the list here, isoflurane sounds right. https://www.ncbi.nlm.nih.gov/books/NBK537013/

Cognac · May 31, 2023

Hopefully recovery is progressing well, and there's minimal additional operating required.

Carhole · Jun 1, 2023

Wow, that’s a major progress note. That little bit of paper you signed beforehand was in part giving the surgeon permission to go ahead and take out bad guy flesh, so it’s not surprising. If it had been something even more serious then your wife likely would’ve been involved before they moved on, so that good to have your advanced directive shit all figured out before going under for something with a suspiciously lurking presence such as what you’re up against.

But the good points: first, malicious tissue out of the way will help your body cope with regular intruders far more readily than while stressed by cells going haywire, so I bet that you heal up nice and quickly. Second, you got it done and that’s huge. Take it easy and I hope that your lab is faster than ours are out here.

Also, great job keeping up the research on the nature of the affliction. It sounds very well like you may end up teaching the medical world a thing or two by the time you finish recovering from this ordeal.

Good luck, stay comfy. I hear that there’s a decent forum nearby to read when you’re awake and killing time. Cheers!

Tom the Melaniephile · Jun 1, 2023

I appreciate the well wishes folks.

Things have gotten rougher as the nerves wake back up and I didn't sleep much (again). Burning lip and itching around the edges of the numb skin. I probably overdid it with talking and working yesterday as well.

Going to take it easier today.

Tom the Melaniephile · Jun 2, 2023

Minor update: Actually got decent sleep last night!

No other significant changes. It will be nice when I stop having chunks of dried/semidried blood come out of my nose.

Tom the Melaniephile · Jun 12, 2023

Realized I hadn't updated in awhile. Last week I had a followup with the oral surgeon - he gently prodded the area and apparently was pleased with the progress. Still some swelling evident to him. I was surprised he didn't take out the sutures, but he just wants to leave them til they dissolve. I had weaned off most of the pain meds before then, which is mixed. Sometimes I hardly feel any pain, other times it gets quite sharp. Most of the time it's just achy/annoying inside the bone. I presume that's just based on the nerve recovery. I'm mostly just living with it, I might take a single dose of 600mg ibuprofen during the day. I can open my jaw further now, and have started using the recovering side for some gentle chewing.

On the outside of the face, the numb area continues to slowly shrink, still maintaining about 1" of itchy/burning around the numb patch, so that's receding at approximately the same rate.

In other good news I haven't had a chunk of dried/semi-dried blood come out of my nose in awhile.

Diabolical · Jun 12, 2023

Crossing fingers, Tom! Sounding good so far!

Tom the Melaniephile · Jun 12, 2023

Diabolical said:
Crossing fingers, Tom! Sounding good so far!

So far so good - just in a holding pattern until the pathology report shows up so that we can plan followup treatment. Only update on that front was that the normal pathology lab has forwarded the samples to a specialist pathology lab.

Tom the Melaniephile · Jun 13, 2023

While only marginally related to the jaw (because projectile vomiting) - it's medical anyway.

I got to have a nice trip to the ER. My very first kidney stone! First: the positives - I didn't notice any jaw pain whatsoever.

The kidney area on the other hand? Second worst pain I've ever had. They initially gave me fentanyl, which definitely took the edge off for the back pain and made me woozy for about 20 minutes, once stone was confirmed I got some toradol as well. Unfortunately it didn't do all that much for the pain around/behind the testicles. Hopefully I can sleep sometime.

The original jaw biopsy (read: excavation) from 15 years ago without effective anaesthesia still ranks #1 on the personal pain scale.

Anyway, now I get to pee into a strainer til it finishes coming out.

Cognac · Jun 13, 2023

Damn man. Did you anger some deity somewhere that is getting their revenge?

Glad to hear the jaw is getting better. Only 4 weeks until the lab report!

Tom the Melaniephile · Jun 13, 2023

Cognac said:
Damn man. Did you anger some deity somewhere that is getting their revenge?

Glad to hear the jaw is getting better. Only 4 weeks until the lab report!

Apparently so, pain came back with a vengeance and I'm back in the ER getting a couple bags of IV fluids in addition to the toradol, fentanyl and dilaudid. At least the pain is under control now.

It kinda sucks that pain meds work so poorly on me. I felt kinda woozy again for about 20 minutes, then it cleared like last time.

Cognac · Jun 13, 2023

Oh man. That really sucks. If there's anything a random poster on the internet can do to help just let us know.

Tom the Melaniephile · Jun 13, 2023

Cognac said:
Oh man. That really sucks. If there's anything a random poster on the internet can do to help just let us know.

I appreciate it. After a trip to the 24h pharmacy, I'm home, reasonably comfortable and trying to chill out enough that I can get to sleep.

Tom the Melaniephile · Jun 14, 2023

Stone just came out! Yay! Looks kinda spiky.

ER had said there was just one - I'll take it along to my followup with my PCP today. Hopefully they can send it out for analysis - apparently there are 4 different "common" types.

Diabolical · Jun 14, 2023

Tom the Melaniephile said:
Stone just came out! Yay! Looks kinda spiky.

Good news!

Tom the Melaniephile said:
kinda spiky.

Tom the Melaniephile said:
spiky.

...

Tom the Melaniephile · Jun 14, 2023

Well, I didn't go to the ER twice in one night because it was fun... Way more painful than when I broke my leg, and the second trip approximately matched the original jaw biopsy where the oral surgeon was digging in the bone without effective anesthesia.
which sets the high water mark for worst pain I've experienced.

I was unaware that during normal operation the ureters (vessels from kidney to bladder) continuously squeeze and relax in order to pump urine into the bladder. According to the ER doc who says he's treated >1000 kidney stones - when the ureter squeezes and there's an object (like say a spiky kidney stone) it's not uncommon for the ureter to freak out and clamp down in a death grip. Which is really fucking painful.

Ajar · Jun 14, 2023

Yeowch. Glad it passed and you can recover.

YA Jaw Update: Part III, the return

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