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 (1 in 40,000) called Neurofibromatosis Type 2 (NF2).

Learn more about 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 tumors 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

• 10.5 hours long
• (4) $100 drill bits used to get through my thick skull
• Head clamp with 60 lbs of pressure holding things still
• A bunch of stitches
• A 'good sized silver dollar' titanium plate to seal up the hole

The Results
Complete removal of the tumor
Permanent hearing loss on the left side
Temporarily unable to swallow (5 days)
17 pounds lost in three days
Additional swallowing issues: 6 months

This bad-ass scar

I spent the next 2.5 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 re-calibrate my brain. Simple things like turning my head from side to side while I walked and using a 2x4 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 21 (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.]

Recent MRI Scan

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 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.

Emails 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.

24 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 them 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.

UPDATE: After some thought and ‘digestion’ by my rock-star oncologist, I received an email stating that they were willing to prescribe the drug to me ‘off-label.’ Which means I don’t have to wait for the trial to begin - I just need to pick up a prescription, go to a pharmacy and walk out with, what I guess is $10,000 worth of drugs, paid for with a $25 co-pay, compliments of Oxford. It's all a bit surreal and amazing.

Thanks CVS

When it rains, it pours
In between the excitement of getting approved to receive the Lapatinib, our inboxes lit up on Sunday afternoon with more emails from my docs, informing us of another clinical trial that was about to open in Boston.

Apparently, a few of my doctors are out in Portland attending the annual NF convention and they got word of some promising therapies coming out of Boston by a doctor who has done some exciting work for NF2 in the recent past.

My docs spoke directly to the Boston doc regarding my case and I am now in the process of getting involved. I can't stress enough how fortunate I am to be working with these guys from NYU, they really are just awesome. I'm going to take them all out for steak dinners at Peter Luger when this nonsense is done.

So, I’m probably headed up to Boston for a day trip sometime soon. My father has agreed to drive me with the hopes of catching a game at Fenway. Hopefully we can work that into the itinerary.

What if the drugs don’t work?
In the case that the drug therapy doesn't work (or time) out properly, I'll need to have the surgery again. It is the last resort and most invasive of the options.

Besides the risk of losing my all of my hearing; which will most likely happen, there are concerns with other nerves in the area including the facial nerve and the nerves that affect swallowing. Damaging either of these nerves will be a real problem.

Although, if I do end up having to have the surgery again, it s somewhat assuring that I am in very good hands with the team at NYU. They are some of the best surgeons in the world for this procedure, which will make a world of difference with the results.

The Two Options:

Debulking Surgery
An attempt to preserve the hearing by only removing ‘sucking out ‘ the middle of the tumor, basically making space and taking the pressure off of the nerves. This is far from a guaranteed solution to save the hearing, but it’s the best that they ‘ve got. Basically a 50/50 chance.

Surgery with an Audio Brain stem Implant
Complete removal of the tumor causing deafness. Placement of an audio brainstem implant, which is a very hi-tech device that would allow ‘sound awareness ‘ to be transmitted to my brain without using my auditory system. Very few people with these devices are able to understand conversation, but it is better than being profoundly deaf.

Written in June 2009