Into the tube

Since being diagnosed with NF2 in 2006, I’ve had the odd experience of sitting (laying) through approximately sixteen MRI scans. 

I was clinically diagnosed with the disease after a scan revealed the two original tumors. I’ve had scans to check on the growth of the tumor and to map out the location in preparation of surgery. In one case, I had an emergency scan because I thought my brain was exploding (check the archives for that gem).

One of the treats that come with an NF2 diagnosis is the lifetime need for an annual MRI scan. Because NF2 tumors are nerve-based, they can pop up essentially anywhere in the body, so there is a need for constant monitoring. My situation calls for brain and spinal scans. Because the tumors are considered 'slow-growing' — an annual MRI is the norm for keeping things in-check.

Having to lay completely still in a two-foot wide tube for 45 minutes seems like it would be the most difficult aspect of the process, but it’s not. I actually don’t mind the scan itself, I’ve taught myself to relax to a point of a meditative-like state during the scan - just hovering above falling asleep.

The most difficult part of the MRI scan is having to once again make the appointment year after year, wait for the day to arrive and then wait for the results. In my experience, 75% of the results were bad news. You get obsessed with micro-measurement.

“The tumor grew 0.1 centimeter!”

I don’t really even know what 0.1 centimeter amounts to in the real world, I don’t think it’s a lot, a tick mark on a ruler —but because your head is packed pretty tightly with brains and nerves and stuff, those little 0.1 cm increases eventually add up and cause bad things to happen along with a shitload of anxiety.

As anyone who has ever had any sort of health testing done, he time in-between the test and getting the results is the most difficult part.

When working with my original doctor, the results would take a few days and he would follow up with a phone call. I would be on-edge every time the phone would ring. If I didn’t recognize the number, I would let it go to voice-mail. I preferred the results were left on my voice mail, so I could listen to them a few times and make sure I understood everything.

My current doctor emails the results as opposed to calling (obviously). But the rules still apply; I jump every time the phone vibrates with an email alert. Since the scan is now done within the hospital, the results usually get turned around within 24 hours – which cuts down on my post-scan anxiety.

My anxiety was short-lived this year because I received this piece of ‘good news’ last night:

I finally got the reads back
Both sides have a very slight change in the enhancement pattern*, but no significant change in size
So good news and we continue to watch
The spine is good

*When they remove these tumors, it’s very difficult to remove ALL of them. In my case, the ‘enhancement patterns’ refers to residual tumor left behind in an effort not to damage the facial nerve.

Woohoo! No significant change in size!

Here’s to flipping that 75% from bad to good in the next few years and reducing some of that nasty week-ruining anxiety that comes along with it all.

The Grandaddy of Gifts

Our friends Russ and Carl and their friend Gary, are the creators of a podcast called 3 Non-Joggers (3NJ), which is a series of recordings of them hanging out in a basement drinking beer and talking about running.

Both Russ and Gary are insane and for some reason, think it’s a good idea to take part in ultra-runs, which is basically any distance longer than a marathon, which is 26.2 miles.

Carl doesn’t run (nor jog) but he’s a mailman, so he walks a lot. I believe he pounds out seven miles a day on his route.

A few months ago, they Tweeted that they had a special guest on the show named Jason Lytle. This caught my attention because Jason Lytle is the creator / multi-instrumentalist / singer for the band Grandaddy – a band that I really used to enjoy listening to.

I was introduced to Grandaddy by my friend and former band mate, Joe around 1998.  I remember listening to a few of the songs on one of Joe’s crazy multiple-speaker stereo set ups and being sold instantly. I picked up the CD the next day.

From that point on, there was a Grandaddy album within reach of my car stereo, home stereo or Discman at all times. Eventually, all of the albums made it onto my iPod too.

After I saw the blurb about Jason guesting on the 3NJ show, I confessed to Carl that I was a really big fan of Grandaddy and asked him a few silly fanboy-esque questions about Jason. Fortunately, Carl confessed that he was actually a bit star-struck during the recording, because he too, was a pretty big fan of the band.

I told Jennifer about the episode so she listened to it and said:

“Oh man, it’s really good, I wish you could hear it…. you know what? I’ll transcribe it for you.”

A few weeks later I received an email with about half of the show transcribed. Which I imagine involved a lot of stopping and ‘rewinding’ on her part. I read through it and was entertained, as expected.

Somewhere along the line, she told the 3NJ guys that she had transcribed most of the episode and she could email it to them, if they wanted it for any reason. They accepted the offer and she sent it over.

Fast-forward to tonight. I came home from work to find a package in the mail from 3 Non-Joggers. I wasn’t expecting anything from them, so the contents were a bit of a mystery to me.

Inside the envelope, I find a black folder with the some printouts and a CD. The printouts appear to be the entire transcription of the show, the CD is Jason Lytle’s new solo album (Grandaddy broke up a few years ago)

Upon further inspection, I notice a hand-written note from Jason, which talks about how Carl asked him to include his signature on this great thing that Jennifer had started for me. The second part of the note explains how he’d be ‘honored’ to be a part of this special gift and then lastly tops it off with his ‘dumb little signature” (his words).

WHOA! What an insanely cool gift to receive.

Thank you Jennifer.
Thank you Carl.
Thank you Russ.
And thank you Jason.

--

Jason Lytle
http://jasonlytle.com/

Recommended Grandaddy albums:

•  Under the Western Freeway
• The Sophtware Slump
• Sumday

3 Non-Joggers:
http://3nonjoggers.com
@3NonJoggers

2010 Wrap Up

It's been another crazy year, so here's the breakdown as it stands in its' final moments:
 

The Tumor 

The surgery in May and recovery has gone incredibly well. I basically felt fully recovered by mid-Summer, which is pretty amazing for having the surgery a few months prior. The follow up MRI showed that my brain had ‘expanded back into place’ and that there was no tumor left at the operated site, which was very good news.

The facial nerve weakness, that I experienced directly after the surgery fully recovered, as expected. 

My balance has returned to a better state than it was pre-surgery – it’s not back to ‘normal’ and probably won’t ever be, but it’s pretty good. Sometime around August, I managed to ride my bike around the block without wiping out, which seemed to impress everyone, including my docs, who labeled me as ‘brave’ for the effort. I have a skateboard in my sights for next summer… someone needs to teach the boys how to skate, right?

The Eyeballs
It was realized pretty quickly that my eyes were going to need some additional tweaking even after the cataract surgeries. The surgeries were successful, in that they were able to remove the cataract (protein in the natural lens of the eye) and replace it with an artificial lens (IOL). But, unfortunately, after the surgeries, I developed some residual clouding in both of the eyes, which was light sensitivity and focusing issues. My doctor told me that it was going to be a work-in-progress to get things looking good again, but ‘we’d get there eventually.’ 

At this point, I’m about 95% there, which is awesome, because this time last year I was basically housebound because of my inability to see clearly.

I had two separate laser treatments (simple, five minute jobbers) that successfully zapped and scattered the residual protein haze, giving me clear vision once again. I don’t have 20/20, but it’s not too far off. I currently need to wear reading glasses for the computer and reading, but distance is pretty sharp and seems to still be improving.

I’ll go back to see the doc in a few months for a check-in. If my vision hasn’t improved to my liking, he will simply set me up with a pair of glasses. I’ve been wearing glasses since kindergarten, so the idea of having to wear them again isn’t a big deal at all.
 

The Robot Ear

The ABI continues to be interesting. As I’ve said, I really didn’t know what to expect from this thing, so as I continue to wear it, I seem to keep getting more and more surprising results. 

While I can’t hear speech directly with it, it’s proven to be very helpful when combined with lip-reading. I’ve had a couple of conversations with Jennifer where I was able to follow what she was saying without her using sign language.
 
I did have one isolated incident during the summer where I clearly heard my son yell:
 
“Hi, Grandma!” 
at my mother in law, who was walking down the street. It was pretty amazing and took me by complete surprise. Unfortunately, nothing that clear has come through since.
 
I am getting pretty good results with hearing surround sounds though. When I say that I ‘hear’ sound, it’s a very different type of sound than natural hearing. It’s a much simpler and limited range of sound. 

A lot of the sound, sounds the same, if I’m in a noisy environment, it just becomes static. But now that my brain is starting to be able to recognize different pitches and volume changes, I’m able to differentiate the source of the sound.
 
I can hear doors opening and closing, toilets flushing, water running, people talking, beeping alerts in my car, and recently been able to hear ambulance sirens and horns. They tell me that it could continue to change and improve for up to five years, so I’m just really at the early stages of what this thing might be able to do for me.
 
Overall, I enjoy wearing it, it's worth having. There are times when it gets really noisy and it’s not really helping me and I think ‘Man, this thing sucks.’ But, when that happens, I usually turn off for a minute and realize how difficult it is without it. 

It’s literally (a lot) better than nothing.
 

The End (for now)

I have about two hours left of 2010, so this truly is, an end of the year wrap-up. As I had hoped, with some knowledge and some guesswork, the year did manage to get better as it progressed. It wasn’t easy, but as usual, the tremendous support from my wife and sons, family, doctors, therapist, friends and colleagues, the combination has been a huge factor for  a successful year.
 
Thank you all and cheers big ears!

“How’s the ABI?”

It’s weird, but, I expected that. Right now the sound is very much just beeps, boops, clicks and clacks. The hope is that over time my brain will be able to process the sounds with more pitch differentiation, which will allow me to recognize different sounds.

Good news on the technical side is that we were able to map 17 of the 21 electrodes – which the audiologist thought was very good.

The first session was spent checking to see if the electrode would function, then finding the right level for it. Because the device is ‘plugged’ into my brainstem, turning the level up on a specific electrode might cause side-effects – which is basically the electrode stimulating another part of your body, along with the auditory pathway. In some cases, my head would buzz, other times my right hand and arm would tingle. It felt like a very minor electrical shock, which is essentially what it is.

After this was done, he set the highs and lows. This was achieved by simply taking each channel down to its least recognizable level. Similar to a hearing test.

The second session was spent searching for electrode pairs. I’m not exactly sure how and why this is done, I need to look into that, but he had to deactivate four pairs because they were causing side effects. Even with the four pairs deactivated, he still felt that I was in good shape.

He also tested me with some simple voice recognition and I did well with that. He shielded his mouth so that I wasn’t able to read his lips and I was able to identify when he said ‘beep.’

I did not hear him say the word ‘beep’ but I heard a beep when he said it.

Because we were able to map so many electrodes, he hopes that I will be able to make out some speech in the future. This is actually very good, most of the ABI users are not able to have this happen.

The exterior device is a little strange looking. It’s the same set up as a cochlear implant – a microphone that sits on the ear, similar to a hearing aid with a small disc that is connected to a wire that attaches magnetically to the side of my head, on top of the device under my scalp.

Unlike a cochlear implant device, which has a magnet under the skin, the ABI has no magnet.

The reason for this is that almost all ABI users have NF2, which requires getting repeated MRI scans. Having a magnet under your scalp is going to be a problem when they flip the switch on a machine that functions with giant magnets.

So, the work-around is connecting the retainer piece to my head with a small Band-Aid with a magnet inside. It’s a little awkward since he had to shave a patch of my head so that it would stick properly. I need to figure out how I’m going to manage with odd grooming procedure in the future. Plus I’m going bald as it is, so I don’t need any help losing hair.

The other small problem with the Band-Aid magnet is that I have a tendency to sweat like a wild boar, even when it isn’t 100 degrees, which it was the day I walked outside with the ABI on my head for the first time. I made it about four blocks before the magnet came loose off of my scalp.

So, that’s my report after day four of wearing this crazy space-age thing. My plan is to just keep the device on and let my brain adjust to this new way of ‘hearing.’ I have another appointment with the audiologist in a month, so I’ll meet with him for a tune-up and check on my progress.

Signing off,
RoboDan

You have no idea how much I miss all of you

Pavement. Ween. Aroma. Jane's Addiction. Beastie Boys. Beck. Grandaddy. Fugazi. Flaming Lips. Radiohead. Bedhead. Iron & Wine. Shellac. Neutral Milk Hotel. Mogwai. Yo La Tengo. Weezer. Modest Mouse. Matt Costa. Violent Femmes. Van Halen. White Stripes. The Shins. Mastodon. Liz Phair. Primus. Pixies. The New Year. Soul Coughing. Sonic Youth. Nirvana. Deftones. Helmet. Interpol. Hub. Giant Sand. The Beatles. Public Enemy.

The funniest thing about having brain surgery

...is waking up the morning after and having your wife tell you:

JV: Oh, by the way, you’re some sort of Twitter Celebrity now.
DM: Huh?
JV: Yeah, that comedian that Dave knows, from Best Week Ever, found your blog and tweeted about you. All these people are leaving you messages on Twitter.
DM: Wow…that’s awesome.

After some Internet re-con, I was able to piece together that a couple of comedians came across my blog and posted some very thoughtful messages on their website and Twitter streams regarding my upcoming surgery. 

Those two gentlemen are: Paul Scheer and Todd Barry.

From Paul Scheer’s website:
Dan McGorry’s very funny, thoughtful, and amazing blog about one man’s struggle with his Brain Tumor.  Send him good wishes as he’s getting major surgery today.

Todd Barry on Twitter:
Do me a favor. Send some nice thoughts to @dfxm326. He's getting brain surgery today.
 
And they did. I received about twenty notes between Twitter, e-mail and this blog from complete strangers from around the world, wishing me good luck and sending good thoughts for the surgery. It was pretty amazing.

I’m familiar with Paul Scheer from his very funny appearances on VH1’s Best Week Ever series that ran a few years ago. He was also brought to my attention when he taped a couple of the episodes while wearing a “Poop” t-shirt, which was designed by a good friend of ours, Dave Bowles — giving Dave some national exposure, which allowed him to sell a few extra t-shirts. Apparently, Paul likes to do nice things for people.

While I’m not as familiar with Todd Barry, the Internet and Rickey Gervais, both have some very good things to say about him and his work. His Twitter stream is very funny as is his website - check out the Receipt Museum, in particular.

So, check these guys out. Go see them perform, buy their CDs and watch their shows. Help me return their kindness. And thank you again Paul and Todd.

Twitter: 
@paulscheer
@toddbarry

Robot Ear

I will meet with an audiologist in about four weeks to activate my ABI and have him patiently run through several different combinations of electrode pairs that will stimulate my brainstem, hopefully allowing to restore some 'sound awareness.'
 
I have no idea what the results will be. The sounds will be nothing like 'normal hearing' but it will be something. The results are described as being pretty primitive - horns honking, doors closing, etc. From what I understand there is quite a learning curve which can improve over years of use. I am currently profoundly deaf, which means that I have absolutely no hearing in either ears, so anything is better than nothing.

The positive news is, the surgeons were both very pleased with the placement of the device and when they stimulated it before closing me up. It tested quite well with 18 of the 21 electrodes functioning. So, we'll just have to wait and see what things sound like when they boot this thing up.

“Anxiously awaiting an update with positive news.”

OK Anonymous, here you go: 

The surgery went VERY well. They were able to completely remove the tumor without damaging any of the surrounding nerves while successfully decompressing the brain stem.
 

The actual procedure lasted about seven hours, as expected. I spent one night in Neuro ICU, two days in regular ICU and two days in a regular recovery room. I was discharged on good behavior on Friday.

The facial nerve was left intact and unharmed. I am experiencing some minor facial weakness, but that is getting stronger each day and I can expect a full recovery.

After the tumor was removed, they placed the Auditory Brainstem Implant (ABI). Word on the street was that they got ‘very good’ placement of the device, which was surprisingly good news.
 

I’m treating any results that I get from the ABI as a bonus. The main goal of this procedure was to get the tumor out safely, which was accomplished.
 

As predicted, my swallowing was not affected this time, so that’s been a huge relief. I lost a few pounds from nausea, but it’s nothing to compare to the 17 pounds lost during Surgery v.1. Having the ability to swallow also enabled me to fully enjoy the deliciousness that they call hospital food – three times daily. Mmm.

Overall, I’m feeling pretty good. I’ve had a decent share of headaches (go figure) but those are thinning out as the days go by. My balance took a hit, but my brain and body seem to working well together to get that back on track. I took a short walk around the block for the first time by myself yesterday, which felt good. I’m restricted to pretty limited activity for a few more weeks, so walking will be my main course of physical therapy. More specifically, walking to the diner for breakfast!

I met with Dr. Awesome this morning to have my stitches removed. He removed the stitches, examined the wound and let me know that everything looked good. Then I ran through my best Jim Carrey impressions and got the thumbs up for my facial nerve related movements. Some basic Q&A was done, we shook hands, I said ‘Thank you’ for the 100th time and we walked out the door. 

Not a bad way to start the weekend.

...like I need a hole in the head

This time next week, I’ll be in a deep-deep sleep with two trusty surgeons, a team of nurses, techs and an audiologist - removing the tumor from inside of my head. The idea of this is both surreal and completely factual, at the same time.

The procedure should last between 6-8 hours with an approximate 5-day hospital stay. Two days in ICU and three days in recovery.  The scar should be about half the size of the other one and located directly behind my ear. This is good - I like my designs to be asymmetrical.

Since I have very little hearing left and the tumor is considered to be large (3.5 cm), they  are going with the translabyrinthine approach, which is different from the previous surgery. The hearing nerve will be sacrificed, but the angle that they will enter the skull is the best for viewing and preserving the facial nerve – which is VERY important to me. During the procedure, after the tumor is removed, they will be implanting an Auditory Brainstem Implant (ABI). More on that later.

I met with the two surgeons separately last week. Both meetings were good and I walked away feeling less concerned about possible complications. It was a relief to get ‘their’ statistics and percentages regarding the risks and complications, versus getting the information from the Internet. Sorry to disappoint, but Wikipedia isn't always accurate!

We discussed the swallowing issues that I experienced with the first surgery. The doc explained why it happened and because the approach is different, it shouldn’t happen again.

These guys are good. I know it – they  know it. When I mentioned it took four drill bits to get through my thick skull during the first surgery, doc responded:

“Our drills are better.” 

I laughed. I’m not a big fan of arrogance, but I’ll make the exception when it comes to the two guys drilling my head open.

So, that’s it. All my tests are complete. Meetings are done. I have a week of ‘relaxing’ before I head in and have brain surgery for the second time in five years.

Good vibes, prayers, karma, and happy thoughts are all welcome. 
I don't discriminate.

Three down, one to go

1. Fungus infection of the lung

Successful surgery. No post-op complications with breathing or shortness of breath. CT scan showed no recurrence after three months. Bye bye funk.

2. Diabetes

My blood sugar level returned to normal shortly after I stopped taking the Prednisone in Mid-December. I celebrated with a can of Coke and some cotton candy.

3. Cataracts

Both of the cataract surgeries went well. The lenses were implanted and ‘most’ of the cataracts were removed. I have some residual clouding in my left eye (this happens sometimes) which will be resolved with an in-office laser treatment if necessary. This can’t be done until the three-month mark from the surgery, for healing purposes. 

There is some adjustment time for the lenses at different distances, but things are getting better and crisper by the week. I have a more detailed entry in the works that will explain things a bit more. But, in general my vision is much much better. I can cross the street again.

4. The Tumor

I’ve scheduled the surgery for May 10th. I’ve decided it needs to come out before it causes any more damage. It would've been a much more difficult decision if I could still hear, but physiology already made that decision for me. April is filled with appointments and tests in preparation. I won't be returning to work until after the surgery recovery.

I am very nervous, but will be very relieved when it is done. I’m relying on the expert skills of my docs to get the job done with minimal complications. It's in their hands now.

During the surgery, I will also be having an Auditory Brainstem Implant (ABI) put in with hopes of restoring ‘some’ hearing. More on the surgery and ABI later.

Breakin' it down for '09

Just a few facts and figures I was able to put together regarding my health issues throughout 2009. There are some pretty absurd numbers in there that make me feel very fortunate to have health insurance. Most of the appointments and medications were taken care of with a $25 co-pay.

Amount the insurance company was billed:

$94,980

Lung surgery

$30,555

Ambulance trip from Edison, NJ to Manhattan

$2,112

Clean out mastoid cavity (wax removal)

$375

Fee for Neuro-opthamologist office visit

$650

25-day course of lapatinib

$2,900

5 courses = $14,500

30-day course of VFEND

$1,400

2 courses = $2,800

Drugs that ran through my system:

• Prednisone
• Lapatinib
• Voriconazole (VFEND)
• NPH
• Alprazolam (Xanax)
• Percocet
• Oxycontin
• Aciphex
• Dexamethasone
• Toradol
• Caffeine

Types of doctors who I saw:

• Neurologist
• Neurosurgeon
• Neuro otolaryngologist
• Neuro opthamologist
• Oncologist
• Audiologist
• Endocrinologist
• Pulmonologist
• Thoracic surgeon
• Nurse practitioner
• Opthamologist
• Optometrist

Well, I still have that pesky brain tumor...

And a bunch of nonsense to go along with it. I last left off in the very early stages of getting involved with the chemotherapy drug, lapatinib, as a means of stabilizing and/or potentially shrinking the tumor. I started taking the lapatinib shortly after the note and began a six-month journey that I don’t think anyone was prepared for.

The first few weeks on the drug went very smoothly. I was expecting some diarrhea and some mild skin rashes, which is what is listed as common side effects by the manufacturer. Fortunately, I didn’t experience either of these things and figured that this was going to be a piece of cake.

My hearing was still pretty good at this point thanks to the heavy doses of prednisone (steroids) that I was taking. But combined with the lapatinib, I was left with a serious lack of motivation and creativity for a majority of the day. I would get bursts of ‘feeling better’ from around 3 pm to 7 pm, which was nice, but I would have to take the second dose of steroids around 8 pm, which would start the negative cycle all over again. I would fall asleep each night with Lucas around 8:30, sleep for a few hours, wake up, eat something and go back to bed.

Feel the Funk
In early July, while celebrating my father’s birthday, I started to experience minor episodes of trouble taking deep breaths. It wasn’t much of a big deal, they would last maybe ten minutes or so, and then things would return to normal. I had also developed a pretty nasty cough that was strong enough to send a pain into my back. Something wasn’t right.

I casually mentioned this to my oncologist on my next visit and the next thing I knew, I was standing in front of an X-Ray machine getting my chest radiated. He took a look at the films via the NYU intranet, e-mailed me and told me he didn’t notice anything unusual.

The following day I received an e-mail from him noting the radiologist had spotted an ‘abnormality’ on my X-Ray films and that I should see a pulmonologist asap. He also ordered a CT scan of my chest.

I didn’t even know what a pulmonologist was, but luckily his office took care of business and set me up with an appointment.

I met with the pulmonologist about a week later and he reviewed the CT films, pointing out the mysterious black spot on my lung. There was mention of possible tuberculosis and other nasty things, but the idea of this being anything malignant was ruled out early.

Because they couldn’t identify it from the films, he ordered a biopsy. The word biopsy instantly freaked me out, but I was (sort of) assured that this was nothing malignant and the biopsy will reveal exactly what it is.

I’ll spare you the details of the biopsy but know that it involved a 12” needle, multiple injections and a lot of me gritting my teeth on the bed of a CT scan machine for an hour.

Fungal in the Jungle
A few days later I met with my oncologist, who informed me that the results of the biopsy showed that I had a ‘fungal infection’ in the top portion of my right lung. He was as surprised as I was, being that fungal infections are quite rare.

Ideas were discussed of where the infection may have come from, which ranged from something I picked up five years ago on our honeymoon in Mexico to moldy grass clippings in our backyard. It was a bit of a mystery.

I later found out that because my immune system was so compromised due to the drugs, this fungus that everyone has in their body was able to cultivate inside of my lung due to the lack of defense..

Back to the pulmonologist, who went over a few options and decided that a course of antibiotics would be the best way to get rid of this thing. I got my hands on the 30-day supply of antibiotics called VFEND, I was ready to start fighting the funk.

A few weeks prior to the fungal fiasco, my hearing had started to become distorted again. I was starting to have trouble understanding what people were saying to me, similar to the way it sounded when it initially dropped in May.

I noted this to my team of doctors and was told to increase the steroids to a level that would give me ‘adequate hearing.’ So, I did. I bumped up the daily dose to 50 mg, which is about ten times the typical dose.

It was late July at this point and we packed up the wagon and headed North up to the Finger Lakes in Upstate New York for some much needed R n’ R. We were able to relax a bit along with some of Jennifer’s relatives at a house that bordered Lake Seneca. We ate tons of good food and spent the days swimming in the crystal clear lake.

I started taking the VFEND the day we left and felt the side effects immediately. About an hour after taking the pill in the morning, my vision would get distorted and I would notice slight changes in color perception. It was also making me extra dizzy. According to the manufacturer, this was a common side effect and it should be resolved after I stopped taking the drug.

We returned home from the Finger Lakes a day early so I could have my two-week lab work done for the VFEND. There was a risk of liver damage, so my doctor wanted to make sure that we kept a close eye on things.

Shortly after having the lab work done, I was sitting in the Apple store enjoying the A/C when I received the following e-mail from my oncologist:

your liver enzymes are a touch out of the ordinary... so nothing to do with that..
but your glucose is completely abnormal.
you officially have diabetes and should see an endocrinologist!

Say what - diabetes?! I really didn’t know how to react to this news. This wasn’t the news that I was expecting to receive. I basically lost it, walked in the bathroom, sat down in a stall and started crying.

I frantically texted Jennifer, who in her usual manner, told me that it was going to be OK and we’ll deal with it. I believed her, cleaned myself up and headed back out into the inferno they call Manhattan in August.

I later found out that my blood glucose level was 350 – the normal range is 75-100. In retrospect, I probably shouldn’t have spent the entire week at the lake house stuffing my face with hot dogs, ambrosia salad and cans of Coke.

So, at this point, I’m gradually losing my hearing, I’m wacked out on three heavy duty types of medication, I have a lung infection and now I have diabetes! Things were not going too well.

No more hot fudge sundaes
I met with an endocrinologist, who set me up with an insulin injection plan and I stripped my food intake down to a sugar free, low carb, high protein diet.

After reviewing my blood sugar levels that I’d recorded for a few weeks, she downgraded the original “diabetes” diagnosis to “steroid-induced hyperglycemia.” This was positive news. She explained that the steroids were causing my blood sugar to sky-rocket, so once I eventually stopped taking the steroids, my blood sugar should return to normal.

All in all, this actually wasn’t the worst thing to have happen. The balanced meals and increased vegetable intake was making me feel better; giving me the extra energy that I needed to compensate for all the synthetics and chemicals that were running through my body on a daily basis.

I was due for the change. I was still out of work on disability due to the constant doctors’ appointments, so I had the time to shop for the food and prepare meals. Between the new diet, a good daily walk and the insulin, I was able to get my sugar levels down to a reasonable level. Phew.

Accepting the situation
Around Mid-August, my hearing had become very poor. Surrounding sounds like traffic in the distance, birds chirping and the wind blowing all started to fade. I could make out familiar voices, but was having a very difficult time out in public when dealing with strangers.

I didn’t know how to handle the situation. I would order a cup of coffee and the barista would ask me a question. Hoping the answer was something simple like ‘yes’ or ‘no’ I’d respond with that. In most cases, the answer wasn’t ‘yes’ or ‘no’ and the barista would then stare at me like I was an idiot. It became an awkward dialogue that needed a much better solution.

I mentioned the difficulty to Jennifer who suggested that I need accept the fact that I was deaf and would now need to act accordingly. I agreed.

I started telling people upfront that I was able to talk, but unable to hear them respond. I also started carry a pen and pad with me wherever I went. This was a much better solution that took some of the anxiety out of my everyday life.

You are in the shit. I can't imagine it. And yet you have a lightness about it all. I know it's difficult for you to see it sometimes, but it's there. And that's the fucking magic during the struggle: Acceptance and dealing with your cards.

Your attitude is heroic. Seriously. To choose to share this at ALL takes massive balls. And to be completely and 100% honest while wading through the scary waters? My friend, that is the face of bravery.

Rustyboy via Flickr

I had become a bit of a hermit and needed to get out and start to adjust to different environments beyond a doctors’ office or the local coffee shop with my hearing loss.

My buddy Dave was getting married in the fall and his brother was throwing a bachelor party for him.

I decided this would be a good opportunity to venture out into the social scene as a deaf person. I could still hear some sound and make out some conversation with a lot of focus on the person speaking. My friends knew the situation and those who didn’t figured it out pretty quickly. We ended up drinking pitchers of cheap beer and eating miniature hamburgers while they kept me in most of the conversation by texting me from their iPhones.

It was a pretty great night because it showed me that I was still going to be able to do this even though I could no longer hear.

Hearing with your hands
I began taking sign language classes in Manhattan in September. My sister, Suzanne had taken classes at the school and recommended it.

The first two classes were great, I felt really comfortable being in a situation where I didn’t have to listen as a means of communicating.

By the third class, things started to get difficult. The instructor started to move along at a much quicker pace and I was having trouble keeping up. It was a harsh reminder of how quickly I failed my third year of Spanish in high school. But unlike Spanish, I really needed to learn sign language as a way to communicate.

I was getting stressed out about going to the class and just decided to coast through the remaining sessions without putting too much effort into it. I simply had too much going on and was taking way too much medication to be able to concentrate on all of this.

This is not to say that I didn’t learn anything. I learned a lot, just not as much as I would have liked to. It gave me a good base for the language that has enabled me to understand it more at home with Jennifer. She’s been teaching herself through books and DVD’s. We have recently been trying to get away from the pen and paper and use sign as a method of communicating. It’s not easy but we’ve been making pretty good progress. We’ve also been teaching Lucas, who is doing really well. Andrew just turned one a few months ago, so sign language will be second nature as a way to communicate with me in the future.

Long Live the Funk
I hated being on the VFEND, so I was pleased to finish up the 30-day course and head back for another chest CT to find out if it had done its job and killed the infection.

The CT results came back and showed that the VFEND did eliminate a majority of the infection, but not all of it. I would need to go back on the antibiotics for another 30 days.

I was pissed, but I figured another 30 days was better than having to have it surgically removed, which was the other option.

Oh cool, some good news
I went in for an MRI of my brain in Mid-September to check on the effects of the lapatinib. The results came back showing that there was no change in the tumor from the previous MRI.

I was disappointed at first because I was hoping the drug would have shrunk the tumor, but after giving it some thought I realized it was a very good thing, especially since the tumor had been on a growth spurt for the last two and half years.

This meant that either the tumor had taken a natural break from growing this round, or the lapatinib was actually working. With these results, it was decided that I would stay on the drug and see what happens in the next couple of months.

Back to the grind
I hadn’t planned on being out of work for five months, but somehow that’s what happened. I had so many doctor appointments and tests every week, it had become a full time job, just one without a paycheck.

Things started to calm down though, the second round of VFEND wasn’t as bad as the first and the appointments were thinning out. Walking around the mall and browsing books at Barnes and Noble starts to lose its charm around month four. It was time to get back to work. I chose a date, contacted my bosses and prepared mentally to return to the job.

I had lost about 95% of my hearing by now, so things were going to be a lot different for my co-workers and I.

My boss and I passed a few e-mails back and forth trying to figure out the best approach for me to work. There wasn’t much of a plan beyond e-mail, instant messenger, my laptop and pen and paper. We were all going have to work through this together and figure out what works and what didn’t.

Surprisingly, the transition back to work wasn’t as difficult as I thought it might be.

Fortunately, I get to sit in front of a computer and design stuff for most of the day. Using my eyes, not my ears. But of course it’s not all fun-time with Photoshop, I do have to attend the occasional meeting. Double fortunately, I work with a team of very generous and thoughtful people who have adapted to my hearing loss and work with me in different ways so that we can get the job done. Whether it’s typing out comments on a laptop or diagramming things out on the white board, it’s just a different, but effective way of communicating.

Meetings and design reviews are the most challenging to deal with. The easiest solution right now is for someone to volunteer to type out what is happening onto my laptop. We’re currently looking into voice-to-text software that will help eliminate this process.

Similar to the night out for the bachelor party, being able to return to my job and do it successfully was an accomplishment for me. It felt good.

You Can’t Fight the Funk; Nor can you fake it
Two weeks after returning to work, I finished up the second 30-day course of VFEND. I had another CT scan and made an appointment to go over the results. I had a bad feeling about the results of the CT. I had been coughing a lot the week before and was concerned that the infection was still there.

My gut was right. My doctor e-mailed me the results stating that the second round of antibiotics did not have any affect on the infection. Either the infection had become resistant to the antibiotics or this was something other than a fungal infection. He suggested that I meet with a thoracic surgeon to discuss having the infection removed surgically. So, I set up yet another appointment with another doctor.

I met with the surgeon a few weeks later. He discussed the procedure and the recovery time. I was looking at an hour under the knife and three days in the hospital. He came off very confident about his work and basically made it sound like it shouldn’t be that big of a deal. The goal was to be as non-invasive as possible. I believed him and we set a date for the first week of December.

Foggy with a chance of freak out
A few weeks after learning about the need for surgery, I was at work and I noticed that my right eye was kind of blurry. I figured it was just a dirty contact lens and didn’t put too much thought into it. The day after, it was still blurry and I started to get concerned.

In preparation for the lung surgery, I had to stop taking the lapatinib, which I did the week before. After stopping the medication, I started to feel really dizzy, my hands were weak and I was waking up with minor headaches everyday. The blurry eye was on the same side as the tumor and I started to freak out. I made a call to my opthamologist and was told that he couldn’t see me until the following week. No good.

I Googled a few opthamologists in the area and made an appointment with one nearby. I left work, raced down the Parkway, couldn’t find the office and arrived 15 minutes late. I was off to a bad start. The whole situation was weird, she shared an office with a plastic surgeon who had all sorts of dopey posters and brochures about cosmetic surgery around the waiting room. I thought I was in the wrong office.

I explained my history to her and she examined me with some routine stuff. She needed to dilate my eyes, which is normal for an opthamologist to do, but I told her she couldn’t since I drove there and had to go back to work. She became very frustrated with me, which was both unprofessional and not something I needed at the moment. Knowing the situation with the tumor, she advised me to go to the ER, because there could be something ‘seriously wrong.’ Freak out time.

I got back in the car, raced back to work, avoided talking to anyone in the office and quickly e-mailed my boss to let her know that I was leaving to go to the ER.

Jennifer was in California on a business trip and I didn’t really feel like texting her to let her know that I was on my way to the ER. She had enough on her mind at this point.

I e-mailed my doctors and my parents. Both of my doctors agreed with the opthamologist and told me to go to the ER. My father got the e-mail, didn’t even respond before getting in the car and drove up. My adrenalin was flowing at this point. I was a nervous wreck. I hadn’t eaten since breakfast, but wasn’t hungry. I knew I needed to eat since we may be looking at a long night in the ER. I threw together a peanut butter and jelly, packed a bag and waited for my father to show up.

My father showed up and I explained the situation. He remained calm but concerned as usual. I kissed the boys good bye and we headed to the local ER.

The ER arrival went pretty smoothly, checked in, explained the situation to the nurse, had my vitals taken and then we sat and waited.

Into the ER, not my favorite place to be. It always looks like a war zone, people laying on gurneys all over the place with doctors, nurses and orderlies running around all of them. It’s always a little nuts and in most cases, there are people in much worse shape than you. It can be a little frightening.

I met with a nurse and explained my whole history once again. My father complimented me on how well I know my medical history and how eloquently I explain things to people. I sort of laughed it off while realizing that this had become my life and I’d been explaining it so often to people, it had become like a sales pitch. A really complicated and serious sales pitch.

We finally met with the doctor and explained the situation again. I told him that I would like to see an opthamologist and possibly have an MRI done, which is what both of my doctors had requested earlier via e-mail.

The ER doctor came back shortly and told us that they would do a CT scan to look for any pressure within my head that might be causing the blurry eye. I decided to e-mail my doctors to let them know what was going on and hoped that they might make a phone call to speed things up.

My oncologist flipped out asking if they were refusing to give me an MRI and told me that he was calling the ER to find out what was going on. Awesome, but how did he know what number to call when I never told him where I was? I e-mailed him back and explained that I was in Edison, New Jersey, not at NYU, which is where he thought I was. Oops.

After some back and forth, I got him the phone number for the ER and he made some calls. At some point during all of the confusion, I had a CT scan done and was brought back to wait for the results in my curtained cove in the ER.

It was getting pretty late at this point, maybe 11:00. Jennifer had spent the day flying home from California and had no idea that I was in the ER. After a few attempts, my father was able to get in touch with her while she was taking a car service home from JFK.

I was relieved that we’d gotten in-touch with her and was pretty exhausted and were still waiting for the CT results, so I shut my eyes and tried to get some sleep. I woke up at 1:30 when the doctor returned with the results. He was explaining them to my father, who was quickly taking notes on his note pad. Since I can longer hear, I pay closer attention to peoples’ expressions and reactions. My father had a look of concern and borderline panic on his face while the doctor spoke to him. I knew before the doctor was done explaining that things were not good.

My father came over and explained that I had a ‘mass affecting my brain’ and that my doctor at NYU was having me transferred there for further investigation.

“Oh, shit.”

All sorts of thoughts started running through my head. I was pretty sure that I was going to be lying on an operating table having my skull drilled open within the next 12 hours at NYU. I asked my father to call Jennifer and her mother to let them know what was going on. My anxiety was now at Defcon 4 and my entire body was shaking. We requested something to calm me down from the nurse. She returned with a huge syringe of something that she shot into my ass. It worked, I stopped shaking and was able to focus on the situation again.

We didn’t receive much more info except that they wouldn’t be transferring me until the morning, so my father, who was exhausted at this point, decided it was best for him to drive home while I slept through what was left of the night. He’d meet me at NYU in the morning.

I was woken up bright and early at 6 am, signed some release papers and was loaded into the ambulance. The ride was smooth and seemed to go by quickly. I watched the sun rise out of the back windows of the ambulance while heading north up the Turnpike.

Arriving at NYU was pretty uneventful, which was a surprise. I was set up in a room and visited by both my doctors and the on-staff doctors and their cronies. No one was rushing me into an OR or even an MRI. I started to relax a bit more.

I brought the CT scan results on a disk to the NYU team to review. It was our understanding that there was a new ‘mass’ on my left side along with the existing tumor on the right. When the scans were reviewed again at NYU, they found nothing but the existing tumor, which actually had not changed in size from the previous MRI.

Phew, no emergency brain surgery this weekend!

While this was a huge relief, I wondered where this idea of the mass on the left side came from. Then I remembered that the films from these scans are inversed when they are printed…THEY READ THE SCAN BACKWARDS!!!

If they had shown me the scan back in New Jersey, I could’ve told them that and I’d be sitting in my kitchen with a nice cup of coffee, instead of lying in a hospital bed - amateurs!

More doctors came through and checked-in with me, I had another MRI, a chest X-Ray and finally a neuro-opthamologist came in for a consult. He looked me over and dilated my eye to get a better look. I explained to him that the vision wasn’t so blurry as it was foggy in the middle and sort of in-focus around the edges. He didn’t see anything wrong and told me that it was most likely due to the change in medication and it should clear up over time. Double Phew!

All in all, it wasn’t a huge waste of time. I had been feeling strange with the extra dizziness and weakness in my hands, so it was good to make the doctors’ aware of this. There is also something comforting about having an MRI done and told everything is stable.

After fours days in the hospital, Jennifer picked me up and shuttled me home. I couldn’t wait to get home and see the boys. Andrew, who refuses to sit still for more than three seconds, just sat on my lap and gazed at me for about five minutes. It was really nice to be home.

I returned to work (again) a few days later and got back into the groove. The vision in my right eye was still a little funky, but my left eye was crisp, so it compensated well. I figured as long as the left eyes stays clear, I’ll just wait it out and see what happens. It was manageable.

I had the lung surgery scheduled for the Monday after Thanksgiving, which worked out well because it gave me a few days to relax before the surgery and I could also enjoy the holiday.

Uh oh
The Friday before Thanksgiving, I noticed that the vision in left eye was now looking a bit blurry. When I covered my right eye, I was seeing double on small type on the screen and my iPhone. I wasn’t on the computer too much over the weekend, so it wasn’t so noticeable. I returned to work Monday, hoping that it would have cleared up, but it didn’t, it seemed to be getting worse. I adjusted my monitors and increased the type size, which helped a little, but things weren’t looking too good.

I figured I had a bunch of time off coming up, so I would just relax and it would resolve itself before I had to go back to work.

By Wednesday, I was really having trouble reading my iPhone and once again, panic struck. I told Jennifer what was going on and she basically lost it. The last few months had been one thing after another. Nothing seemed to be going in our favor with the exception of the tumor not growing. I was continually getting knocked to the ground and then getting kicked. She simply couldn’t take it anymore. Neither could I, so I completely understood where she was coming from.

With a houseful of people expected for Thanksgiving the next day, we agreed to just focus on the next couple of days and then get through the lung surgery on Monday.

Get the funk out of here
We arrived at NYU at 6 am to prepare for the surgery. We went through the typical check-in process and were taken to a room where I would get into my gown. A handful of different doctors would then brief me on the procedure. The whole process ran smoothly because they provided a translator, who typed out what was being said with an Ubi-Duo. After signing more paperwork than a real-estate deal, I was taken to the OR.

Instead of using the Ubi-Duo, we opted for a simple dry erase board for informing me what was happening. There was a good amount of prep work, so it was really helpful to know what was going on around me. Finally, I lied on my back, took a few deep breaths and it was lights out.

When I woke up I was in a lot of pain and I felt like I couldn’t breath. They had the oxygen mask on face, so I knew I was getting air, but the pain was pretty bad. I started pressing the magic painkiller button and after five minutes or so, the pain started to fade.

I was in ICU for a day, but felt pretty good overall. My breathing was normal and I was told the surgery was a success and they were able to remove all of the infection easily – no need for the rib spreader.

I was moved to a regular room the following day where they removed a tube from my abdomen. Moving around was still pretty tough at this point, but they wanted to get me out of the bed and walk around the floor. When I finally managed to take a walk, the whole right side of my upper body hurt, I felt like I’d been hit by a truck, but I managed to do a few laps and get back into bed.

I was pretty doped up and tired so I did a lot of sleeping throughout the day. Unfortunately, this catches up with you as you lay wide-awake at two in the morning. “Nurse, Xanax! STAT!”

I felt pretty good by the third day, still beat up, but I was able to get up and around. I was checked out by a few docs and they gave me my walking papers around noon. They removed the IV’s, I got dressed and texted my father to have him come pick me up.

We had decided before the surgery that I would spend a couple of days down at my parents house to help recover. Both Jennifer and the boys had come down with nasty chest colds, so it was now an especially good idea.

We made a stop at home so that I could see the boys, but didn’t stick around too long for fear of catching any of their colds.

The next coupled days spent at my parents’ house was comfortable and relaxing. I slept, ate and watched TV – it was like being in high school again. As I was feeling better and better about the lung recovery, I started to worry about the vision again. It was definitely getting worse as the week went by. I asked my mother to call the eye doctors about getting me to see someone the next day, I was really starting to get nervous.

We were able to get an appointment with an optometrist. She gave me thorough examination, dilated my eyes and told me the issues I was having were probably due to the two cataracts that had developed in my eyes. She also told me that my optic nerve ‘looked different and may be swollen.’ She suggested that I make an appointment with their ophthalmologist/retina specialist.

We were able to get an appointment for the following Monday. I was pretty stressed-out about the possible swollen optic nerve. I don’t know much about how the works, but I do know that if you lose the optic nerve, you’re dealing with complete blindness in that eye. Not good.

My mother-in-law took me to appointment and we really didn’t know what to expect. We were both nervous. After more exams, we finally got to see the doctor. Keep in mind that this is the same doctor I tried to get an appointment with when this whole thing started who I really like, so it was good to be finally meeting with him. The meeting basically went like this:

Him: I’m pretty sure your vision problems are caused by the cataracts.
They’re like frost on a window.
Me: Do you think they are caused from steroids?
Him: Most likely. Do you want to sign up for the surgery?
Me: Yeah, I guess so. What about the optic nerve, how does that look?
Him: It looks fine, nothing to worry about.
Me: Cool, thanks.

So, that was it. It wasn’t the best news, but it was good news because it was most likely correctable and I wasn’t going blind.

We set up an appointment to meet with the surgeon and left the office much more relieved that we were when we’d arrived. Some phone calls were made and everyone was happy with the news from the appointment.

It was now a few weeks before Christmas and we were scheduled to see the surgeon on January 7th. Apparently, he was on vacation and that was his first day back in the office. I hadn’t returned to work after the lung surgery because of the vision problems – I couldn’t see the monitor nor could I drive at this point. It was going to be a long couple of weeks.

The fact that it was Christmastime was a good thing. I was able to keep busy and keep my mind off of things. Christmas arrived and we all had a good time, the boys got a bunch of nice toys and I managed to get through my first Christmas without hearing.

We set up an appointment with the original neuro-opthamologist who I’d seen in the hospital in October. The appointment wasn’t exactly a second opinion but more of a follow up with a somewhat subtle hint of ‘Hey, just wanted to let you know that you diagnosed me completely wrong.’

The appointment went well, he was surprised with the cataracts but obviously didn’t disagree about them after taking those cool photos of my eyeballs. He also ran me through a couple of neuro-based tests which all came back good. Despite his earlier misdiagnosis, he does have a pretty good understanding of NF2, so he is actually a good person to have in the address book. All in all, my father and I both left the appointment feeling good, which was nice for a change.

My vision has progressively gotten worse and worse as the weeks went by. It’s not so much blurriness as it is fogginess. Everything has a fog over it and my eyes are extremely sensitive to light. It’s gotten to the point that I need to use the Walk/Don’t Walk signs for fear of getting by a car.

January 7th finally arrived and my father took me to the appointment with the eye surgeon. More tests were done, more questions were asked. The surgeon took a look at the results and a look at my eyes and concluded:

"You have the type of cataracts that young people get"
"It’s fixable."

Music to my deaf ears! We scheduled the first surgery (left eye) for February 10th and the right eye with most likely be scheduled for two weeks later. I got my medical clearance physical done last week and my eye measurements done this week, I’m ready for this to be done with. Two weeks to go!

What’s Next?
After getting the eyes fixed this month, we’ll start discussions with my kick-ass doctors (surgeons) about taking this tumor out of my head. I would like to return to work for at least a few months for both my own sanity and my bank accounts sake.

My thinking is that I’ll be going in for surgery to have the tumor removed and the ABI put in within the next few months. The meetings and decisions continue.

The idea of any more medication is kinda out of the question, since I uh… don’t do too well the side effects and complications that they may cause.

This is not to say that the lapatinib didn’t do what it was supposed to do, the fact that there was no change in the tumor’s growth was great and a very positive thing for others with NF2 who are able to tolerate the drug long-term. The clinical trial did finally get approved and is in process now. Hopefully they’ll gain some positive results and they can start to treat this crappy disease in a much less invasive way as they do now. Fingers crossed.

And finally, there’s no ‘I’ in TEAM
Like your grade school gym teacher once told you… that silly expression rings all too true when it comes to me and everything that I’ve been dealing with for the last fours years and continue to deal with.

I can’t express how lucky and proud I am to have the type of strength, support and love from my wife Jennifer, my two sons, Lucas and Andrew, my mother and father, Elaine and Frank, my sisters, Suzanne and Caroline, my mother-in-law, Linda, my bro and sis-in law Ernie and Lara, and of course the Junior Support Squad: Chloe, Kate, Brody Monster, Justin and Matthew. I love you all.

I’ve also received incredible support from old friends, new friends, friends I’ve never met, colleagues, former colleagues, relatives, our au pair and of course all of the doctors and nurses. To steal a quote that Jennifer once told my oncologist – You rock!

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