Tumor Regrowth, Facial Spasms, CyberKnife Radiation

Here is a very tardy update on my situation. My brain works best in chronological order, so we'll approach it like that.

In February 2015 I started having hemifacial spasms on the left side of my face (AN side). They would come on in times of stress or big smiling that seemed to aggravate the facial muscles. The spasms lasted for up to 30 seconds at a time, during which my mouth would pull up toward my eye, and I would feel it down into my neck. Needless to say, this was uncomfortable both physically and psychologically.

I went to visit my doctor at Johns Hopkins, who suggested that it could be a latent herpes virus affecting those nerves/muscles, and his treatment suggestions included Botox injections to freeze my face in place, which I did not do. I did take a course of antibiotics in case it had to do with the herpes virus. The spasms subsided for the most part in late 2015 without any additional treatments.

My husband and I moved from DC to Denver in mid-2016, at which point the spasms came back in a very urgent manner; they were very frequent and seemed to be triggered by anything involving my face, even just talking in a calm manner. Moving across the country is a stressful event, so that was underlying the entire episode.

Within a couple of weeks, I had an appointment with a neurotologist at the University of Colorado Anschutz Medical Campus and my AN journey basically started over again. He was pretty convinced from the start that I was experiencing a regrowth of my tumor, which was irritating my facial nerve in the IAC and causing the facial spasms. We requested a copy of all of my MRI scans from diagnosis to current-day, and he pointed out the difference between scar tissue illuminating on the MRI vs how it looked at that point, which was much more tumor-like. He presented my case to the entire skull-based tumor department at UCHealth, and the consensus was that my tumor had regrown. 

He did not recommend surgery to me for a second time because it's really hard for any surgeon to operate in the same small area of the brain twice. The structures in the skull that typically serve as landmarks in the first surgery can get damaged during surgery and will not be available to assist the surgeon in the second surgery. Also having the surgery performed by two different surgeons would not be ideal since everybody does things a little bit differently. I then met with a neurosurgeon who does radiation, and he explained why that would be a good treatment option for regrowth.

As I learned the first time around, I wasn't going to move ahead with any treatment without getting additional opinions. I had read great reviews of Dr. Chang at Stanford on the ANA discussion forum, and my brother lived near Palo Alto at the time, so I headed further west for my second consultation in August 2017. 

My meeting with Dr. Chang was extremely reassuring. He does both microsurgery and radiation treatment, so he knows the risk vs reward of both options, and because he is sought out by patients from all over the country, he is more familiar with regrowth situations than other doctors may be -- so basically he knows more about the rarest of this rare condition. He, too, explained that microsurgery a second time would not be not advisable and recommended that I have CyberKnife radiation (which was invented at Stanford). There were basically no downsides to CK other than that the tumor remains in your head and you have to have patience as the radiation does its thing over the next 3-5 years. 

I'm not generally a patient person, but the risks of microsurgery seemed too great, and there would be no guarantees that all the tumor would be removed a second time either. I decided that I would rather deal with some uncertainty about the radiation vs recovering from another brain surgery too. I could come back to California later in the year and have the radiation treatment completed in a week--sounded like a vacation to me!

I went ahead and scheduled the CK for the week after Thanksgiving 2017. My husband and I would come out and have Thanksgiving with my brother and sister-in-law, stay with them, and we'd all hang out in between my appointments...

And that's exactly how it went! It was actually even easier than we'd thought. The week went something like this:

• Monday we met with Dr. Chang and he explained everything that was going to happen during the week. 
• Tuesday I met with the radiation oncologist for neurosurgery who would be planning the radiation with Dr. Chang. I also had some pre-op testing done (kidney functions) and then a super-detailed CT scan they would use to plot the points for the radiation. And I had the mask made that holds your head/face tight against the table so you don't move while the robot arm is delivering the radiation.
• Friday I had the CK done for a total of 22 minutes (I forget the amount of radiation that was delivered but the overall dose was relatively small because the tumor was pretty small).
• Sunday we flew home to Denver.

I just realized I didn't explain CyberKnife and its benefits. The robot arm delivers the programmed dose of radiation to the plotted points in a similar way to the Gamma Knife machine, which is the older technology. With Gamma Knife, they have to screw a frame onto your head to hold you exactly still during the time the radiation is being delivered. As I mentioned, I had a plastic mask made that conformed to the shape of my head to hold me in place--nothing had to be physically attached to my skull to hold me in place because CyberKnife is also doing checks for movement the entire time it's operating. It can automatically adjust the placement of the radiation if it detects subtle movement. They told me that if you sneeze while receiving CK, the machine will automatically stop. That is the main difference between CK and GK that I know of; they both use fractionated radiation to pinpoint a single location for the radiation from different angles to reduce the amount of radiation going to parts of your body that you don't want it. 

The other cool thing about my CK experience was meeting the radiation oncologist. I had read that patients usually get a dose of steroids after radiation, and I was hoping to avoid taking those due to the issues with blood sugar levels (blood sugar goes very high, insulin doesn't seem to work very well). I asked him if I needed to take them, and he said no, that he also has type 1 diabetes and totally understood my concern, and he said that people don't usually need them after radiation, it's more so that they feel like they "got something." That was quite a relief and also pretty amusing.

I had my first follow-up MRI in June 2018, and it didn't show anything unusual. The tumor looked approximately the same size, and any time there's no growth, that's a win. I wasn't having any headaches or facial spasms, or any other post-radiation symptoms, so that was all good too.

The next MRI was at the one-year mark in December 2018 with a similar report--not much change in size or appearance, and I was still symptom-free. 

The last MRI as of the date I'm writing this was at the two-year mark in December 2019. They said it was maybe a tad smaller with some darkening of the overall appearance, which would indicate that it is dying. Basically it looked like what they would expect at this time interval, so they said the next MRI will be two years out, in December 2021.

I am totally fine with that since they've seen more of these than I have, and I trust their expertise. I haven't had any facial spasms since 2016/2017, though I can tell that the left side of my face seems "tired" during stressful times or when I'm really tired. The signs are that my smile isn't quite as symmetrical, and my left cheek/eye don't move up during the smile as they would when not "tired." Other than that, I'm (obviously) still deaf on the left side, and my balance is still nearly normal. 

I feel very fortunate to have moved to Colorado when I did so that I was forced to address the issue with another set of doctors. I think that would have happened eventually if we stayed in DC, but it was good to address it sooner than later. I also feel very fortunate that I was able to travel to Stanford for the CyberKnife treatment and meet with experts for this "rarest of rare" situation. I am hopeful that my next MRI will continue to show that the tumor is shrinking and/or dying, and that "Manny's cousin" will not make a reappearance in the future. 

Thank you for continuing on this journey with me!

5-Year Post Surgery Update

Back in May, I went to Johns Hopkins for a five-year post-surgery follow-up with Dr. C and Dr. T. Dr. C said things were fine with my hearing and to come back whenever Dr. T wanted to see me again. Interestingly enough, though, Dr. T looked at my MRI and said that the area on the MRI that lights up a bit is unchanged over the five years since the surgery, so the assumption is that it is just scar tissue. Then he said that I am essentially cured, so I don't need to come back. I was surprised to hear that, and that freaks me out a little, so I think I will see about getting a check-up in another five years or so just to make sure (unless anything changes in the meantime, in which case I would go back sooner).

3-Year Post-Surgery Update

I got an MRI last week for my three-year follow-up appointment this week. Of course, I looked at the films afterward and didn't see anything tumor-like, but I'm not exceptionally skilled at reading MRIs, so I figured I'd just wait to see what the surgeons said.

I went up to Johns Hopkins on Wednesday to meet with both Dr. T and Dr. C. Dr. T said that everything looked fine and that the abnormality on the MRI in the area where I had the tumor is just scar tissue. He said that my scar looks good, and he asked about my family. All in all, it was a pleasant, five-minute visit, and I don't need to see him again for two years.

Dr. C reiterated that the spot on the MRI was only scar tissue and showed me a printout of the scan so that I could see the spot in greater detail. He also compared it to last year's MRI and said that it's unchanged, which is another indication that it's just scar tissue and nothing to be concerned about. He explained that the MRI contrast travels through blood vessels and lights up areas with a large concentration of blood vessels like tumors. Scar tissue also lights up because it's an area where the blood vessels were messed with during surgery. I asked if it would ever go away, and he said that it is unlikely to go away completely, but it doesn't pose any long-term problems.

He also tested my balance by doing the head thrust thing where he turns my head really fast and watches my eyes to see how well they're able to stay looking at the tip of his nose. My balance continues to compensate really well, and he has to "trick" my vestibular system into righting itself more slowly than normal (he explained this to the med student). I asked Dr. C is there is anything else I can do to further improve my balance, as I would like to be better able to look over my shoulder when I'm riding my bike without worrying about losing my balance. Overall, he said, you just need to practice more, and that he would recommend doing tai chi and yoga in part for the balance practice and also for the core strengthening. If you strengthen your core, you'll be able to right yourself quicker and reduce any wobble. That made sense to me, and I downloaded a yoga app and a tai chi app when I got home. I have yet to use them though...

Dr. C asked if I had changed my mind about a BAHA system, and I said no, that I seem to be getting along fine without it. I explained to him that I'm usually interacting with just a few people at a time, which doesn't pose any real hearing issues. The only time I have problems with SSD is at a networking thing in a loud place, since I can't easily hear people on my left over the other noise. I told him that I use my eyes a lot to watch for people approaching me on my left so I can hopefully avoid ignoring them on accident. I also told him that I've only recently become more comfortable telling people up front that I can't hear them if they're on my left. I usually just try to nonchalantly switch with them, but if they then try to switch back, I will tell them that I can't hear them. Up until now, I've avoided telling someone I've just met because I tend to tell them the WHOLE story instead of just the part about not being able to hear them. But then what happens is that I end up continuing to interact with them, and then it's three months later and I still haven't told them and then I finally do, and they're like, why didn't you tell me before? So I've kind of made a pact with myself to be upfront that I can't hear them and then if I still know them later, I will tell them why.

I thought it was nice that Dr. C listened to that whole thing because I think it's important for doctors to know how the results of surgery or health issues continue to affect people's everyday lives even after the immediate issue is resolved. The same thing kind of goes for diabetes too. I have a chronic, non-curable disease that I live with everyday and have generally adapted to after having it for 21 years, but sometimes it frustrates me to no end, and unless you've experienced something like that too, you're not really going to understand how I feel. I appreciate my endocrinologist and think he does a great job, but he doesn't know what my life is actually like living with the disease. And Dr. C doesn't know what living with single-sided deafness is really like either, so unless I (and other patients) explain it to him, he won't know how to advise future patients who are nervous about possibly losing their hearing after acoustic neuroma surgery.

Anyway...

Dr. C said to come back in two years, and I'll have another hearing test at that point as well. If the MRI still looks unchanged then, we might extend the subsequent visit to more like three years.

So I've been tumor-free for three years, and there's no sign of anything similar happening on my other side, so things are good. Hope you're doing well too :)

Updates

I've been remiss in updating the blog, but I think that's a testament to the fact that I am definitely in my "post-AN" phase of life and that I really don't think about it too much.

I visited Dr. C a couple of months ago for a two-year follow-up, and everything was fine. The MRI looked good, and any irregularities attributable to scar tissue had faded some since the one-year follow-up MRI.

My balance is normal, no headaches, and no facial weakness. Obviously, I still can't hear out of my left ear, but even that is just part of my "normal" now and I rarely give it much thought. I'm still not interested in a BAHA. Right now I'm looking forward to spring finally arriving in D.C. so that I can ride my bike to work again.

I hope that this blog is still helping to give newly diagnosed AN-ers a sense of what the journey is like and the knowledge that there can be a happy ending after finding out that there's a thing growing in your head.

One Year Ago

One year ago, I was in the hospital after having my tumor removed. I was off-balance, my shoulder hurt, and I was desperate to go home. Luckily, I've come a long way since then. I've completely recovered from my surgery, and the only lasting reminder is the silence on my left side. I go back to Johns Hopkins at the end of March for a follow-up with the surgeons and to go over my first post-surgical MRI. I'll be sure to post the results then. Thanks for following along, and best wishes to all of my fellow AN-ers!

The results are in

I went up to Johns Hopkins on Wednesday for a hearing test and to meet with Dr. C. On the cab ride over, I had a strange feeling, like I was returning to the scene of the crime. First I had a hearing test, and the results were not surprising: I am completely deaf in my left ear, but I have perfect hearing in my right. Even though that's what I expected to find out, it was still a little sad to get that news. I had another strange feeling when the audiologist was testing the left ear and I literally could not hear anything. She would say, "OK, listen for the beeps," and I would sit there in anticipation until the test was over, having not pushed the button once.

Then I went to my appointment with Dr. C. One of his residents came in first to talk to me about things. We discussed the BAHA, and I agreed to do the demo, even though I'm not really interested in getting it at this time. During the demo, a headband with the processor mounted on one side is placed on your head, so the BAHA processor can pick up sounds and transfer them through bone conduction. It's not as good as having the real thing (the post screwed into your head), but the results are interesting, nonetheless. The resident and I walked and talked for a few paces, with him being on my left side. I could hear him pretty well, so it definitely works.

Dr. C came in later and we talked about the BAHA a little more. He said that some people get along fine without it, and for some people, it makes a world of difference. I told him that I've been getting along fine without it so far, that there's maybe one incident per week where I get frustrated that I can't hear on that side, but that I seem to have adapted pretty well overall. It hasn't stopped me from going to work, going out to dinner in restaurants, or attending happy hours where I don't know anyone. So for me, I think it makes sense to wait until I really feel like I need it, and maybe there will be even better technology at that point. No sense in getting a post permanently screwed into my head if I don't really need one. Plus, getting a BAHA would make me one step closer to being completely bionic (I already have the insulin pump), and that's no good!

Then he checked my balance by doing the thing where I look at the tip of his nose and he turns my head back and forth quickly to see how well my eyes stay focused. After many head whips (not really sure what to call them) to test the knowledge of the resident and scare the medical student who was in the room, Dr. C declared that my right side has completely compensated for the loss of the vestibular nerve on the left side, and I was dizzy. It went away pretty quickly though. He also asked me to raise my eyebrows, shut my eyes tight, smile wide, and whistle. I passed this test with flying colors.

The next thing I have to look forward to in my "adventures with manny" is an MRI next February or March. Then I will meet with Dr. T to go over the results of that test, and Dr. C would like to see me again as well.

I am very thankful that things have turned out so well. Except for a couple of days after discontinuing the steroid, I have had no facial weakness. My balance is excellent. I have been able to adjust to single-sided hearing. No headaches, no pain. And my tumor is gone. I wish that everyone could be so lucky.

Seven-month update

I had a scare over the past couple of weeks where I thought I had made negative progress. My neck was a little stiffer than it had been, and my scar area was more sore than usual. I think I irritated the muscles in my shoulder area by carrying some heavy groceries for too long. Now that it's two weeks later, it's feeling better again, and I have a reminder to not overdo it.

No new news on the crying/tears front. I haven't been able to test it again...guess I need to watch a sappy movie or something.

My hair loss has stopped; it lasted about a month. My endocrinologist said that it's common for that to happen after a stressful event. I think brain surgery counts.

I go to see Dr. C in two weeks for a hearing test and discussion about what I'd like to do in terms of getting a hearing aid or not. As frustrating as it is sometimes to not be able to hear, I definitely don't want to do anything at this point. I'm bionic enough as it is. And it's kind of funny when I think voices at the office are coming from one direction, only to find out that they're actually behind me. Yeah, funny. In a groan-inducing way. I dunno, maybe it's not that funny. But I don't want any more surgery any time soon.