Child with Geniculate Neuralgia: Lessons Learned, Fears and Questions

My 10 year old daughter was recently diagnosed with Geniculate Neuralgia. She woke up on New Year's Day with an earache, but after 24 hours we knew something was wrong beyond an ear infection. After a week of trips to different doctors who all told us that her ears looked fine, her pediatrician suggested we see a neuralogist about Trigeminal Neuralgia.

She was admitted to the hospital for extreme pain and we stayed there for two days. They were unable to do anything to ease her pain other than Oxycodone which only really helped her drift into sleep. They completed a CT scan and MRI as well as had multiple individuals examine her including her surgeon from a vascular malformation resection that had been done years earlier. Their conclusion was probable TN and they started her on Tegretol.

We took her to her dentist to doubly rule out TMJ or anything related to her braces. He suggested that perhaps her wisdom teeth could be putting pressure on the nerves and referred us out to the Orofacial Pain clinic to a wonderful physician, Dr. Nixdorf, at the U of MN. He provided some recommendations for medication and partnered with her neurologist at the children's hospital and didn't think her pain quite fit TN.

During this time, I began pushing to have her be seen at the Mayo Clinic in Rochester, MN. We also continued to work with her neurologist to keep trying different medication combinations to see if we could touch her pain at all with no success. Her regimin to date has included various combinations and dosages of Tegretol XR, Neurontin, Dilantin, Clonidine, Propronalol, Amitriptyline.

In mid-February we were able to get her into the Mayo Clinic to see a pediatric neurologist and neurosurgeon there. Her diagnosis is Geniculate Neuralgia which much more closely fits her symptoms. She is still in constant pain that she relates as always at least a 9 on her pain scale and most of the time it's like being "punched in the head" on her right side with periodic "knives stabbing" on top of it. We're continuing to aggressively try to treat the pain with medications to exhaust all options (per the surgeon) prior to doing surgery, but I think that is the road we are heading down and quickly.

Lessons Learned so far:

  • ALWAYS advocate. Keep copies of everything in triplicate and organized. One copy for yourself, one that is "copy-ready" (not stapled that you could hand someone so they can make copies of what they need during an appointment, and one copy that is just ready to be handed over. Do not be intimidated into not seeking additional opinions. You can still respect the phsycian you are working with while getting more information. Doctors should be willing to partner with each other to find the best possible care for the patient especially in the case of rare disorders.
  • The book "Striking Back: The Trigeminal Neuralgia and Face Pain Handbook" has some great information in it, especially about medications. Even though my daughter doesn't have TN, GN is mentioned in the book as is GPN and it has been my go-to reference book as we work through medication management for her as the drugs seem to be very similar for the different neuralgias.
  • Don't be afraid to ask friends/family for support. They genuinely want to help you and often just don't know how. We actually had to kindly request that people not ask our daughter directly how she's feeling as it increases her pain when she has to think about it to answer and then she feels bad if she lies and says "fine" when she doesn't feel that way or that she disappoints them when she doesn't respond that she's magically better. I dreaded asking her school for support, but they were genuinely very happy to help and we made reasonable accommodations to get her back into the classroom where the distractions help her manage the pain.
  • Think outside of the box. We've engaged a psychologist for her to help with coping skills, chiropractic and acupuncture for complementary pain relief, aromatherapy, meditation, reiki. Being brave enough to try things others may scoff at may at least bring some secondary relief.

A Parent's Fears:

  • I'm terrified that I won't be able to find a way to help her no longer be in pain.
  • I'm scared that her personality will change and that she'll "shut down" because she can no longer handle coping with the pain especially as she goes into her teenage years.
  • I'm frightened of the lack of information there is about treatment/surgical options. Even with the Chief of Neurosurgery from the Mayo Clinic on our side, it still seems like there's very few of these surgeries that have been performed and even fewer on children.
  • I'm scared that I've somehow missed something obvious that either caused this and can be fixed or that could reduce her pain significantly.
  • I'm concerned that if we move forward with surgery and something does go wrong that results in a lasting side effect that she will blame me for the rest of her life. It's a risk I'm willing to take as it's certainly nothing I could ever ask her if she wants to do because in the end we simply cannot ever set it up so that she ever has any reason to blame herself - EVER.
  • I fear that I'm underestimating or overestimating her pain. I can't feel her pain myself. I simply know she hurts and I have to trust that. I have to trust a very subjective viewpoint of a child's pain to make incredibly hard decisions. I have to shove aside those doubts that creep up when people tell me "She looks fine" or "Wow. She's making it to school, she must be feeling better."

Questions:

  • At what age did you start dealing with your condition? Is there anyone here who was under 18 or is a parent of a child suffering?
  • Has anyone had surgical intervention that included transection of the nervus intermedius? What was the result? Any side effects? Any residual pain?
  • If anyone is continuing to deal with GN has there been any relief from medication and if so, what combination?
  • Any tips, tricks, lessons learned that I should add to my list as I'm just learning how to navigate this path?

Sorry for the novel out of the gate, but I think I've been storing it up in my head for a couple months.

Thank you.

Sorry to hear about your daughter Lel, so young to be dealing with all this.

One question that sprang to mind as I was reading her story - how long has she been in braces now and did they do extraction orthodontic work on her at all?

It's a good question. She has been in limited braces (just four brackets) for 2 years. There were no extractions and we did have them taken off about a month ago just to make sure we weren't causing her additional undue discomfort. There hadn't been adjusting wires in them though for over 6 months prior to the pain because she was losing teeth and the wires kept popping off.

Good to hear no extractions were done. Do her frenums line up? They are the little flaps of skin above and below the upper and lower incisors, behind the lips.

And what did the chiropractor have to say about her cervical spine? Do adjustments help at all?

I don't think I've ever specifically asked about her frenums, I believe so but will look tonight.

Nothing jumped out about her cervical spine, but the adjustments seem to help as complementary pain relief for when she's tightening her muscles in her neck and shoulders. The limited acupuncture also seemed to help with sleep in the first month or so, now not as much. He also worked with a nutritionist they use to recommend a supplement that is heavy on the B vitamins called Neurosol that she takes daily now.

Hi Lel,

Did you look at her frenums?

So nothing jumped out about her cervical spine but the chiro still did adjustments on her? I would hope there was clear evidence of misalignment before they treated her?

Yes, there was misalignment. It's a chiropractor we've seen in the past and trust very much. He adjusted her for secondary relief as her neck/shoulders have been holding most of the tightness from her stress.

The frenums line up.

We go back to Mayo near the end of April. There is another specialist in Neurology they'd like her to see before sending us back to Neurosurgery.

Hello, I'm writing to you from MN as well. I see it's been a while since you posted this... but how is your daughter doing now? I believe I am suffering from GPN and possibly GN. I am looking for a dr. either at Mayo or U of MN. Who did you take your daughter to at Mayo Clinic? I would appreciate any info you could pass along and I pray that your daughter is finding relief from this! Thank you!

Hi Sarah. Thankfully, my daughter is doing very well and has been pain-free and medication free for the past 10 months. We saw Dr. Nixdorf at the U of M in the Orofacial Pain/Dental clinic. At Mayo, she was seen by a pediatric neurologist (Kenneth Mack) as well as Dr. Fredric Meyer and Dr. Carrie (Beth) Robertson in Neurology there.

I’d highly recommend any of them. I’d probably start with Dr. Robertson if you try for Mayo as she seemed to have the most experience with GN/GPN. Dr. Meyer was the one we consulted with about surgery.

Let me know if I can be of additional help.
-Jana

Hi Lel,

I've had the geniculate neuralgia on and off - Topimax which is an older anti epleptic worked quite well, though I'm not sure of the suitability for young ones.. I became allergic to it, (being an allergy queen) but it calmed the nerve enough to reset it over a 12 week period and has been calm since (for a couple of months now). Not sure if you've visited our TN site but there is a geniculate group and also a kids group on the site that may be helpful for you both. Its awful having the condition, but worse still when its your baby girl. Links below:

http://www.livingwithtn.org/group/tn-atn-and-ear-pain

and http://www.livingwithtn.org/group/childrenwithtrigeminalneuralgia

KcDancer flagged a youtube / vimeo with Dr Casey, if you search for "The Tides of Neuropathic Pain"

This is a great video of Dr Casey on YouTube that goes for a couple of hours but has a lot of information that can help you understand the relationship between different neuralgias and their treatment, including pain management.The video is a few hours long, and I suggest you have a pen and take notes, but its really worthwhile because although he talks about TN and ATN, he explains concepts that are relevant to many forms of neuropathic pain.

Not sure whether you've gone down the trigger path, which is trial and error, but if you could identify specific things that make it worse, it becomes easier in that avoidance doesn't provide a cure, but can help in maintaining a semblance of management with the medication. For example, noise, light temperature, screen brightness etc. Too much happening with sensory input - music with multiple layers, visually videos that flash and cut around etc. can all potentially act as triggers.

Hope these are helpful and thank you for sharing your learnings with us. As you mention Striking Back is really great reference for many forms of neuralgia, not just the TN.

Regards,

Smiley.

Thank you..I'm so glad to hear she is doing better!!! Thank you for that info. Will have my family dr. try to get me in to see Dr. Robertson at Mayo.

Lel said:

Hi Sarah. Thankfully, my daughter is doing very well and has been pain-free and medication free for the past 10 months. We saw Dr. Nixdorf at the U of M in the Orofacial Pain/Dental clinic. At Mayo, she was seen by a pediatric neurologist (Kenneth Mack) as well as Dr. Fredric Meyer and Dr. Carrie (Beth) Robertson in Neurology there.

I'd highly recommend any of them. I'd probably start with Dr. Robertson if you try for Mayo as she seemed to have the most experience with GN/GPN. Dr. Meyer was the one we consulted with about surgery.

Let me know if I can be of additional help.
-Jana

Hello, it's me again :) Just another question, as I feel I'm at an important crossroads in finding a provider to help me right now. I have the choice of having my dr. refer me to a neurologist at Mayo in Rochester or at the U of M in the Cities. Which, in your experience, would be the "best" place to go for the GPN, especially if I need surgery for this someday. I saw there are many more neurosurgeons on the U of M webpage than there are at Mayo..but does that necessarily mean anything? I hate to rely on any of my preconceived assumptions...I'd rather ask someone who's been thru all this and has been to both facilities as you have. Thank you so much for any info you can give me. :)

That's a hard one. Both places have very good doctors. We really spent very little time at the U of M for Alanna because she is a child and they didn't feel as comfortable treating her there.

Our experience at Mayo was amazing. It was not easy to get in, but once we were in then it was truly a team/collaborative approach. They all partner together for treatment and it's a well-oiled machine. Dr. Meyer, the Chair of Neurosurgery, would have been the one to do Alanna's surgery if we'd needed it and honestly I would have trusted him to do so. Dr. Robertson was willing to think outside the box and try a topical compound. She also probably asked the best diagnostic questions out of anyone we had talked to.

If we were back at the point we were when Alanna was in the worst of the pain, I would go back to Mayo hands down even though it was further away for us.

That’s what I needed to hear. Again I’m so happy she’s doing better now…its such a terrible problem to have. Thank you…shooting for Mayo!

Hi Lel,
I’m not sure whether you’re on this group anymore? Just in case you are, I was wondering how your daughter was doing? I think my 9 year old daughter has geniculate/glossopharygeal neuralgia but we can’t get a diagnosis here in the UK - we’ve been told that her symptoms don’t match up - constant ear pain that dials up and down for the last three years, not the shooting pain more commonly experienced.
I just read your earlier comments and wondered how your daughter had got better in the end? Did she end up having MVD? Would love to hear from you.
Thanks,
Charlotte

Dear Lei, I have a 10 year son who was diagnosed with geniculate neuralgia. It took 3 months to get a diagnosis. He tried gabapentin and is now on carbamazepine. The pain is less than in the beginning but still a long way from being pain free. I’m wondering what helped your daughter and how long it took for her to be pain free?

Hello. It’s been a long while since I’ve visited this site so I deeply apologize if I haven’t been as responsive. I just am catching up and should have time today for some longer replies. I want to doc out my timeline, med list and give you some detailed answers.
Hugs

@Trey @Barlotte

In February of 2013 after her first diagnosis of Trigeminal Neuralgia, she was started on the following medications – 500mg of Tegretol, 1200mg of Gabapentin, 12mg of Prednisone, and .1mg of Clonidine. These were changed over the course of the month after we went to Mayo Clinic and received the revised diagnosis of Geniculate Neuralgia to up the Tegretol to 900mg daily and wean completely off the Gabapentin.

By end of March, her medications were Tegretol 900mg daily (400mg in the a.m. and 500mg in the p.m.) and 60mg Amitriptyline in the p.m.
These stayed fairly consistent through early May. The Tegretol was switched to 300mg 3 times a day and we added in a topical gel of Amitriptyline/Lidocaine/Ketamine 2x daily.

The gel helped take some of the sharper pain away which Dr. Carrie (Beth) Robertson from Mayo Clinic thought was an indicator that her pain was likely more auriculotemporal neurology versus geniculate. With geniculate, the nerve is so deep that a topical wouldn’t have helped.

Ultimately, we worked very closely with the team at Mayo and once we had her pain to a point where she could get through most of her day we transitioned her care with their support to Dr. Stefan Friedrichsdorf at Children’s of Minnesota Pain & Palliative Care Clinic. She began physical therapy, self-hypnosis, and biofeedback training. These techniques gave a lot of the control back to her which she really liked. She felt that she wasn’t helpless to the pain. During the 8 months that followed, she was weaned very slowly off of the medications. It was described to us as a way to help retrain her brain. That the pain was very real, and some of the worst he’d seen, but that the nerve was at a point that it was misfiring a signal to the brain and that if we could stop the brain from reacting to the pain it would calm the nerve versus continue the cycle.

I will be the first to admit that I was VERY against going to anywhere with Palliative care in the name. I truly thought that by doing so I was giving up, that we were just going to stop trying to find a way to make it stop and that instead we were choosing to ‘live with it’. This could not have been further from the truth. The process was incredibly hard, and there were many many days were we felt like awful parents for pushing her to do her exercises and to make it through the school day or her workbooks. But in the end, it worked.

Alanna graduated from pain clinic in January of 2014 and has been medication free since. Aside from seeming to be more susceptible to illness in general, she has not had a recurrence of the sharp pain she was living with constantly. I’m still afraid it will come back, but as of now it has not.

Please let me know if there any additional questions I can help with. Best to you both – I am so sorry your children are going through this.

Not sure if you can view this, but here is Alanna’s exit interview with Dr. Friedrichsdorf when she graduated from pain clinic. https://www.dropbox.com/s/h3akef2ldykdodg/2014%20Feb13%20Alanna%20Worrall%20Facial%20Pain%20Exit%20Interview.mov?oref=e

Thank you so much for responding! It sounds like a long road and we are still toward the beginning. I am going to share your response with Trey’s doctor (an orofacial pain specialist) to see if it’s helpful with meds. We have been waiting for an appointment with CHLA pain clinic since November. Our appointment is later this month. I am hopeful they can help with some of the techniques and therapies that have been helpful for your daughter.

Again, thank you so much for responding so quickly. This has been such a nightmare and I have felt so alone and hopeless. Your daughter’s story surely gives me hope. Thank you again! Traci