…I used to have two of them, but I decided to downsize in 2006 — I'm a minimalist at heart.
The good news is the tumors are/were benign. The bad news is they live just outside of my brain wrapped around the 8th cranial nerve, which is responsible for both hearing and balance functions.
I found out about the tumors after they showed up on an MRI scan in January 2006. I had gone to see an ear doctor with complaints of ringing in my ears and some minor hearing loss. Hoping it was the simple results of too many rock concerts and too much drumming, the results of the scan were a bit more than I had bargained for. I was clinically diagnosed with a rare neurological disorder named Neurofibromatosis Type 2 (NF2).
Around the time of the diagnosis I became overcome with vertigo and balance issues, which is caused by both the nerve being crushed and the tumor pressing on my brainstem.
It was quickly decided that the larger of the tumors (left side) needed to be removed surgically. I would lose the hearing permanently on that side as a result.
March 2006: Surgery
St. Vincents, NYC
Ten and half hours long
– Four $100 drill bits used to get through my thick skull
– Head clamp with sixty pounds of pressure holding things still
– A bunch of stitches
– A ‘good sized silver dollar’ titanium plate to seal up the hole
– Complete removal of the tumor
– Permanent hearing loss on the left side
– Temporarily unable to swallow; Five days
– Seventeen pounds lost in three days
– Additional swallowing issues; Six months
I spent the next two and a half months at home healing and getting my strength back. Since the nerve was killed during the surgery, I no longer have balance function on my left side, leaving me with a bit of 24/7 dizziness. I spent a good amount of time trying to recalibrate my brain. Simple things like turning my head from side to side while I walked and using a two by four as a balance beam in the back yard proved to be helpful. I was even able to hop back on my bike, which impressed the doctors.
Because the tumor on the right side was much smaller at the time of diagnosis, it was decided to just ‘watch and wait ‘ to see if it grew or became symptomatic. This is achieved by getting MRI scans of my brain and audiograms (hearing tests) every six months.
Because these tumors are considered slow growing, there is no documented growth rate or pattern — everyone is different. They suspect that my tumors have been in there since I was twenty one (fifteen years).
Fast forward to 2009
Unfortunately, the little effer has decided to go on a growth spurt recently, and is now causing ‘mischief’ (a term that one of my doctors once used as a reference which I've always enjoyed.)
I was referred to another doctor at NYU, who I'd met with in the past who specializes in NF2. The first words out of his mouth were
We've got some exciting things happening in drug therapy.
This was not the news that my wife and I were expecting to hear from this guy, needless to say, we were excited to learn more.
Because this disorder is so rare and delicate, the treatment options are minimal:
1. Watch and wait
2. Radiation therapy (not really proven with good results for NF2)
3. Invasive surgery
To hear that there was possibly a new treatment option that would not endanger my nerves, we were beyond excited. Apparently, some mad scientist figured out that the already existing drug, Lapatinib (Tykerb) had some positive effects on some mice in the lab. Go rats!
The basic goal for the drug therapy is to slow down, stop or at best, shrink the tumor. This is not a disappearing act, but my situation will be greatly improved if I'm able to achieve either three of these results.
E-mails and phone calls were made, meetings were set to learn more about the clinical trials and the drugs.
Then my hearing dropped. While sitting at work in early May, I noticed that the voices around the office had become very distorted. I packed up and left. By the time I got home from the office, I was unable to understand what anyone was saying to me. My wife sounded like the teacher from Charlie Brown:
Wah wah wah & wahh whha wah.
Needless to say, I freaked out.
More phone calls and emails were sent to my trusty team of doctors and it was decided that my steroid dose (I was already on a lower dose due to failing hearing) would be cranked up in an effort to restore some of the hearing. Twenty four hours later, the steroid magic kicked in and I could hear again.
Unfortunately, the steroids are only a temporary fix. I’m in the process of figuring out what the next step is. I've spent the last month not working (short-term disability) and spending a good amount of my time meeting and emailing with a very kick ass team of doctors and experts at NYU Medical.
Since the Lapatinib trials are being held up by FDA nonsense, the team has acknowledged my dire situation and offered some other options in drug therapy.
I have been approved for another drug called Tarceva, which is for lung cancer and actually have in my possession but I was informed by my oncologist, that I will be exempt from the Lapatinib trial if I take these.
I have a gut feeling that the Lapatinib is my best shot at shrinking this thing, so I need to choose carefully. We're thinking things through before making any decisions.
To be continued…
Originally written in 2009 as a note to friends on Facebook.