Living with Neurofibromatosis type 2

A guest post by my friend and fellow NF2 warrior Michie O'Day.

May is a Neurofibromatosis (NF) Awareness Month - a busy time in the NF community. Patients, family members, leaders in medical treatment, science, research, patient care, and advocacy are all busy sharing important information - and raising money for our cause. This is all good, but I want to take a different tack... Here goes.

I have NF2 - which I describe as a special talent for growing benign tumors in my brain and on my spinal cord. Rather than talk about the surgeries, treatments and medical consequences, I want to share some of what I’ve learned over the past 39 years since diagnosis. My goal all along has been - and remains - not to give up too soon. Even though this genetic disorder can been debilitating, progress is being made. Surgical techniques are better (and less invasive!), scientific knowledge about our messed up gene, which allows these tumors to grow, drug options, patient care - all of that has improved significantly.

I’m also a good patient. I’m resourceful, confident in my abilities and coping skills, resilient yet open-minded. (I’m just talking medicine! Not politics.) I expect a lot from my doctors, and I want them to expect a lot from me. All this leads to my ultimate goal - to live a happy and productive life. How to do that while carrying this heavy load? Here’s my short list.

Get Good Advice
In 1983, while living in NC and after a CT scan at Duke Medical Center revealing acoustic neuromas, I sought a second opinion from Dr. Allen, then practicing neurosurgery at Johns Hopkins. I knew him from previous consultation in the late 1970s and trusted him completely. His words are still guideposts for me.

“You face a lifetime of surgery.” He thought I could do better than the young neurosurgeon at Duke who wanted to operate. I did not like the surgeon at Duke so was receptive to Dr. Allen’s advice.

“You need someone who is highly, highly skilled, who has excellent judgement, and whose philosophy works with yours.” - Dr. George Allen

This affirmed my instinct that NF2 tumors are not for your “neighborhood neurosurgeon.” I’ve seen this happen in my own family and with others who have recently been diagnosed and don’t fully understand what’s at stake yet. Get referrals and travel if you must to find the right doctor (neurosurgeon or neurologist, depending on your case) and continue with him/her for as long as you are pleased with their qualifications and care. As Dr. Allen advised, don’t make yourself crazy going all over the country interviewing various surgeons every time you need treatment. There are more doctors treating NF2 patients now, but Dr. Allen’s advice has served me well. Continuity of care is important.

I also met with the pastor of a small church I attended in Henderson, NC. Rev Jones had multiple sclerosis. He was a fine man and very helpful to me - a frightened 27 year old. “Remember ownership” he said. “You have NF. It doesn’t have you.”

“Instead of asking what if? Learn to say so what?” also from Rev Jones, who knew where of he spoke.

Some Observations
A life with NF2 is indeed a journey. You’ll come to know yourself well. You’ll also develop your own philosophy about illness and develop practices for dealing with the inevitable disabilities and discrimination. I cannot imagine doing any of this without strong spiritual beliefs.

Over the years, I’ve learned the importance of being adaptable, the need to think creatively to roll with the inevitable changes that come with the disabilities caused by your tumors and treatment.

Be prepared for major changes in your lifestyle and activities. For example, hearing loss in lesser degrees changes some things. Complete deafness changes many more things - and will likely have a profound impact on relationships and career options.

Loss of all my hearing was of course a big deal, but it was not devastating in the ways I feared. With the support of good employers, family and friends, I continued to work, had an active social life, married a good man, began painting, traveled, and relocated to the coast of Maine. I also had an amazing hearing service dog - “Doc” - who added much to the quality of my life.

Loss of balance and peripheral neuropathy cramped my style in bigger ways. A wheelchair became a necessity in 2015, so there are many places I simply cannot go now. The neuropathy is weakening my hands. No more painting or cooking. I miss painting a lot, but if anything I have more appreciation of art by others. I miss cooking too, but I can still eat! Now my vision is at risk. Yikes! Creativity won’t compensate for blindness - if it happens - but spiritually grows. I’ve found that some Buddhist philosophies are helpful with loss too.

Build good working relationships with your doctors. The right doctor will respect you and listen to you. Remember that you are a diagnostic tool as well. Information about how you function in your day-to-day life is important.

If you find that your doctor is not listening to you, respectfully request referral to another physician.

Emotional loss comes with disabilities. Know that and get help for depression if you are hurting. Yet also understand that with disability you can also find creativity - new ways of doing things you enjoyed before, and new activities to try.

Be careful about people who treat you as “less”, who put you down. They may not understand what your daily life is like and can damage your self-esteem (which may already be fragile after the surgeries and ensuing disabilities). This has happened to me even with close friends. It has been sad, but I wonder if those who have known me longer have a harder time dealing with the “new” me?

Keep up with your appointments. Don’t skip MRI scans or other diagnostic tests. Try to follow the doctor’s plan for you. After all, that’s what you’re paying for, and timing is crucial for treatment. If you’re having problems with their directions, it’s your job to communicate that to the doctor. Hint: use good judgement as to how and when you complain!

Having a good sense of humor is helpful for relieving stress. Just don’t go overboard. Being able to laugh at myself is probably the best tool I’ve got.

That’s it for today. I hope you’ve found my list interesting and helpful. Personally, I think I have a truly fascinating life. It’s slower than it used to be, but I like the wisdom that comes with age. And patience? Still working on that.

Muddy Wet Wookie Fur

So, I had this dream, see.

I was outside a small house, apparently mine. Instead of sitting next to a cozy bay (as in real life), it was set at the edge of a vast marsh.

The day was bleakly dark and rainy.

A ways off, in the tall grass, I saw someone working at something at a tall table. Looked like they were chopping? Slicing? Readying ingredients for a meal? Something to do with big shiny knives.

I walked over, my sneakers squelching in the rain drenched ground. Who did I find?

Waddya know, it was Donald Trump Junior AKA Beavis (or is he Butthead?), AKA Daddy’s Least Loved Spawn, AKA Junior Meth. I was about to ask what the fuck he was doing and WHY was he doing it in MY marsh. At that very moment though, I caught a rumbling movement in the soaked earth to my right. 

The soil began to pull apart, revealing a drenched, mud coated, fully armored-up malevolent seeming Wookiee. Dude def looked like he was entertaining some violent death thoughts. I was pretty damn stunned and hadn’t yet put my flight response in full gear when another fissure opened up and, yes, here was another spear wielding, murderous (probably murderous) Wookiee.

Damn! I jumped and made a serious beeline for the house—I’d be safe from giant hostile Wookiees inside my cottage, right? They can only enter if invited, right? Oh wait, I think that's vampires.

Before zipping too far off, I stopped—I should go back and help Coke Boy escape, right? That’d be the good, kind, responsible, adult thing to do, right? I gave it a full second’s pondering before coming to my decision. NOPE, junior was on his own. I skedaddled.

Ya know, maybe I was wrong about these Wookiees. Possibly they weren’t death dealing monsters. Maybe they were just cranky after laying in the cold, sodden earth? Could be they just wanted a nice hot shower, a dry martini and some clam chowder after their mud bath. I bet they’d appreciate some roasted Brussels sprouts too. After running Junior Meth off the marsh I expect they’d have an appetite.

It’d be unneighborly of me to turn away a cold, hungry Wookiee in need.

Right?

Of course, I could be mistaken. Maybe, instead of Wookiees, these were Honey Island Swamp monsters. I hear tell they’re distant cousins of Bigfoot and Bigfoot is sort of Wookiee-esque. But wait, doesn’t he make his home in the forests of Minnesota or Montana or someplace like that? Yep, def not a marsh dweller. Also, Sasquatch don’t exhibit Wookiee levels of hirsuteness. Okay…similarly, the Honey Island Swamp monster is native to the swamps of Louisiana. Sure, marshes are sorta, kinda swamp-like BUT the water up here is damn cold compared to, say, Lake Pontchartrain.

I guess these were in fact Wookiees in my dream.  Maybe, in tonight’s Dream Land I’ll get a sequel and find out the important deets like:

• Do Wookiees like Brussels sprouts or would they prefer grilled asparagus?
• Will they clean the hair out of the shower drain after bathing. I know—they’re guests—they shouldn’t have to. That’s a LOT of hair though!
• Martini or Hot Toddy?
• Did they let Junior know, with extreme prejudice, that he wasn’t welcome on my land?

Two To Go

This morning's sunrise, taken by Ten

I’m nearly across the finish line. Today and tomorrow are my last days of radiation (for now anyway). IF this protoning has worked (in freezing the growth of the monster on my spine. I've a 90-95% chance of success), I should be good—safe from losing all mobility and feeling from the tits down—for ten years.

A decade from now, if I last that long, I’ll be 73.

Like every living thing its prime characteristic is a blind, unreasoned instinct to survive.
~ Robert A. Heinlein, Stranger in a Strange Land

“Don't you have a religion?" Dorolow asked Horza.

"Yes," he replied, not taking his eyes away from the screen on the wall above the end of the main mess-room table. "My survival.”
~ Iain M. Banks, Consider Phlebas

Life tries to crush her, but only succeeded in making a diamond.
~ John Green

I cannot defeat cancer. Nobody defeats cancer. There is no winning or losing. There is no surviving or not surviving.

There are only coin flips: heads or tails; benign or malignant; weight loss or bloating; morphine or oxycodone; extreme rescue efforts or Do Not Resuscitate; live or die.
~ Sherman Alexie, You Don't Have to Say You Love Me

While my zoo full of tumors is benign, the lot of them are most def not harmless, slight or merciful. AT ALL. They’re assholes from the lowest depths of Dante’s Inferno. I can’t conquer neurofibromatosis type 2 BUT I will continue to fight it, learn to live with my losses and have some damn fun too.

The secret of survival is a defective imagination.
~ John Banville, The Infinities

I’ve certainly got that covered.

Proton radiation ends tomorrow—the battle continues.

Morning

WEEKEND!

We’re home, thank Bast. Better yet, I have the weekend off. I still need to do my rehab exercising BUT at least I don’t need to get in the car OR face the maskless hordes.

Astoundingly, while in the proton waiting room yesterday, there were two patients who seemed to feel it was just fine and ducky to pull down their masks. HELLo assholes, YOU may not give a fuck about your own odds of surviving COVID but I care about mine. I haven’t struggled through the last few hard-ass years of heartache and thousand natural shocks just to catch COVID and croak because some lazy-ass, fellow-immunocompromised shitheel in the radiation waiting room has a death wish.

No, I didn’t walk over and tell either of them to mask up or face the burning rage of yurs truly. If these two dolts were so bloody cavalier about their own health, they’re probably plague rats and cool with taking others out with them. I couldn't chance proximity.

Nearly all of the counties in Massachusetts now have a high risk for COVID-19 transmission as the number of new coronavirus cases continue to climb.
~~~
On Thursday, the Massachusetts Department of Public Health reported 5,576 new COVID-19 cases and 16 deaths. The state’s seven-day positivity has also increased to 8.24 percent. (source)

Yesterday, in my county alone, there were 491 new cases.

Also yesterday, shortly after pulling up to Valhalla, Ten and I took a lovely long-ish (for me) seawall walk. The air smells SO damn good—the bay at high tide, freshly cut grass, trees! Oh yeah and no bilious car/truck exhaust.

While sitting in waiting rooms (and waiting around in hotel rooms) over the past week, I found myself spending WAY too much time on Twitter. Not terribly good for the old mental health.

I know it’s time to get off the bird app when I'm sorely tempted to:

• begin tippy tapping corrections to spelling (that tiny annoying flying bug is spelled gnat not knat), punctuation (please do not be sluttish in your use of apostrophes) and grammar (ENOUGH with the sentence fragments already!).
• respond to longwinded Tweets with editing suggestions or, at least, general advice on how to concisely and clearly make a point.
• scream tweet, GIVE US A CREDIBLE LINK TO THIS HORRIFIC NEW ATROCITY THING YOU’RE BEING ALL OUTRAGED AND INDIGNANT ABOUT. As Stonekettle admonishes ‘show your work.’
• engage in well actually-ing (AKA persnickety pedanticism and, if I was a Penis American, mansplaining).
• respond to trolls. No, I don’t. Sometimes the wish is nearly overwhelming though. Okay, okay, I did reply but just once and I was restrained! All I said was Bless your heart and Aren’t you special. Undoubtedly pointless but I couldn’t resist.
  

My goals, now that I’m home, with the weekend off? No doomscrolling, a lot of walking, reading (halfway through Matt Ruff’s 88 Names), cat rescue site browsing and maybe some ice cream. I believe these are all laudable objectives.

I Sing the Body Eclectic

I saw Plotkin, my neurologist, yesterday and the good word is that I get the summer OFF. Okay, I’m off until the end of August anyway. I’ll take it!

The big motherfucker (AKA the goddamned meningioma) chillin’ on my frontal lobe has either not grown at all OR it’s dressing strategically to conceal the extra added weight.

Bottom line? There’s no rush too get me back into the OR. YIPPEE!

This means that I can continue rebuilding strength and stamina, begin the process of getting my two broken teeth removed and replaced and maybe even take a real holiday away somewhere.

And speaking of neuro shit (which we totally were!)…

1) Neuroscientists have released the most detailed 3D map of the mammalian brain ever made, created from an animal whose brain architecture is very similar to our own—the mouse. (source)

2) Did you know, bird and human brains share similar memory limits?

The working memory is the brain’s ability to process information for a short period of time in a retrievable state. It is essential for performing complex cognitive tasks, such as thinking, planning, following instructions or solving problems….The scientists found that birds and monkeys – despite their different brain architecture – share the same central mechanisms and limits of working memory. (source)

3) Neurons communicate with each other via electrical impulses, which are produced by ion channels that control the flow of ions such as potassium and sodium. In a surprising new finding, MIT neuroscientists have shown that human neurons have a much smaller number of these channels than expected, compared to the neurons of other mammals.

The researchers hypothesize that this reduction in channel density may have helped the human brain evolve to operate more efficiently, allowing it to divert resources to other energy-intensive processes that are required to perform complex cognitive tasks. (source) 

Nice speculation science researcher dudes and dudettes but this in no fucking way explains the Republi/Fascist party and all their dimwitted, drooling, gun toting, micro dicked and criminally callous adherents.

4) Apparently good ol’ Walt Whitman was on to something.
New findings reveal distinctive electrical properties of human neurons that may give us a boost in computing power. (source)

Frankly, Walt’s poem’s title, I sing the body electric, also the first line, is as good as it gets. It’s honestly now, all downhill from there.

Just look at the next line:

The armies of those I love engirth me and I engirth them,

Engirth? Please, that word has no fucking rhythm or beauty. It means to surround, encircle; encompass. Any of those would be preferable to engirth which sounds like something a giant, sweaty, Olympic Sumo wrestler would do.

Today, Ten and I will return to Valhalla. I only mention it but my sweet Princess Coco is still (two weeks on) dead. She’s not there waiting for me and this breaks my ridiculously tiny heart.

Last night I dreamed that my girl was still alive—incredibly weak but alive. She was living in Jen’s basement where Jen was nursing her back to good health. I just needed to give everyone space—stay outta the way—and Coco would heal and come home with me again (much easier imagined than done).

No pressure, Jen!

Day 22

I thought it’d be nice to go for a little drive around Boston after my proton session yesterday morning. I figured we could look at some cool trees. Yes, this was a good idea BUT between NO available parking in the Back Bay, me not being physically stable enough to manage walking on uneven ground (a park in Brookline with awesome trees) AND the fact that I’ve forgotten how to drive around Boston’s wonk-ass roads, we ended up lost. Mind you, Boston’s a small city—how lost could we get? 

Yesterday along Comm Ave

I'll tell you—we somehow ended up a couple towns north in Revere (which, by the by, is most def not a cute New England beach town). Let me just tell you, we managed to hit every pothole in existence between here, there and back again. Spine jarringly nasty? Bien sûr times ten, good buddies!

Ya know what though? It was still less painful and annoying than the daily slog from Valhalla to MGH. Next time I gotta go through this, I’m seriously gonna consider getting a short term rental for the full six or so weeks. Once I’ve built up my walking stamina (where I can do more than half a mile a day), a month+ in town would be okay—maybe even fun. Ten and I could wander around the North and South Ends, do a tour of Cambridge bookstores, stroll Newbury Street and lay about in the Public Garden.

Having said that—in just these few days away from home—I seriously miss my patch of ocean, Jen, Oni. Skitter, all the visitor cats AND my own goddamn rain room. FWIW, after this morning's session I have a piddling THREE to go. Next Tuesday is my last, for now, protonization.

This afternoon I’ve an appointment with my good neurologist, Doc Plotkin. I’ll get the results of Tuesday’s CT scan. Is it time to slice out or irradiate the fat, asshole meningioma that’s obnoxiously parked over my frontal lobe? If so, how soon? Can I have a little post-proton fucking down time? Mebbe enjoy a summer off and continue (or even finish) the fucking rehab from that mobility thieving surgery of March ’21 (which was interrupted by October ’21’s spine op)? Gee, a break would be wicked swell, eh?

It’s overcast and foggy here in Cambridge this morning. If the rain holds off, maybe—post-protoning, Ten and I can go for a wee walk down Charles Street, poke into an antique shop or two. Later, depending on Plotkin’s afternoon edict, I’ll be needing some quality, fortifying pizza and vino. Hell, even if the news is good, I’ll want ‘za and the grape. Good news means it’ll be a celebratory versus soothing indulgence.

A gorgeous, giant tree in Brookline

Good News/Bad News

Our lovely view

Traffic into town yesterday was so miserable that, despite allotting 90 minutes to crawl 14 miles into MGH, I almost missed my proton appointment. The one good bit was that, in the sea of ugly grey, black and white SUVs , I spotted a bright purple coupe. So then, slow, homely commute but, in the mundane mob, it was nice to see there’s one person with a bit of style.

We checked into the Holiday Inn in East Cambridge where we have a brilliant view of the now defunct Sav-Mor Discount Liquors mart, the ratty Lechmere green line stop and a decrepit do-it-yourself car wash.

Slow down, Joe, I'm a rock-and-roll man
I've twiddled my thumbs in a dozen odd bands
And you ain't seen nothing 'til you've been
In a motel, baby, like the Holiday Inn
Holiday Inn—Elton John (lyrics by Bernie Taupin)

On top of that, their complementary “continental” breakfast was pretty damn dreadful. Note to self, continental breakfast is defined (by Merriam-Webster) as a light breakfast (as of rolls or toast and coffee). Cool, cool and Ten said his cinnamon roll was good. The coffee wasn’t wonderful but better than gas station brew.

I made the obscene mistake of trying the “cheese” omelet. Yes, omelets are not generally part of a continental breakfast spread so maybe I should have anticipated that it would be cold, flat and made with pasteurized process cheese product versus actual, real cheese. If cheese omelets were sold in vending machines THIS is what they'd be like. NEVER again.

Oh and no one’s wearing a mask either. //shudder//

I’m just guessing here but I suspect Mr. Bernie Taupin was being a bit sarcastic when he wrote the last couple lines of his ode to this hotel chain.

Okay, the sunrise view was definitely more attractive

The good news here is that, this morning, our drive into MGH will be about 10 minutes versus 90. IF I was up to it, which I’m sadly not, we could even walk there. The hospital is just a mile and a half away.

After yesterday’s protoning and before my late afternoon CT scan, I made the mistake of doomscrolling which sparked a head full of rage.

Just FYI, I want all the MAGAt/Tea Bagger/Republi/Fascist idiots, their donors, advisors, reps and senators to suffer the loss and pain they vote for and legislate on the rest of us. THEN I want this benighted earth salted with their oh-so-seriously deserved tears. Maybe a forest of giant redwoods will spring up in their stead. FINALLY something good would come from these infinitesimal, cruel, ugly hypocritical hearts.

Yes. I know. I’m always looking on the bright side aren’t I? I’m just kittens, sunshine and daffodils 24/7. I know.

I don’t know what we’ll do today. I’m keen on avoiding the maskless hordes so, most likely, we’ll go lounge and read in a nice quiet park. Thrilling, huh? I can’t wait until I’m able to walk around more!