Hope Is All We Need

A few months ago I stared at an image of my brain splayed across a computer screen. As we all know by now, tumors congest every orifice and ventricle to be found. On that day I asked my neurologist to send me to a neuro oncologist to discuss what possible medicinal options I may have. (and I do not mean smoking weed!) In October 2011 I met Dr. Barth , who has treated patients in tandem with my current neurologist many times. At our consultation he had already reviewed my case and explained that he wanted to retrieve samples of tumors I had removed years ago from Hoag Hospital, and run tests on them at Clarient Laboratory to see if we could pinpoint a specific medication that may best target my own personal tumor biology.

There are many different types of tumors, derived from different types of cells, and relying on different pathways to feed themselves (from my elementary understanding!) Every person with NF2 has their own individual tumor chemistry. That is why while Gamma Knife has successfully stagnated every one of my 13 treated tumors, I have met people who did not have any response at all to the same treatment. There are over 400 different types of drugs that have the potential to stop the proliferation of tumor cells, and Dr. Barth likened trying random drugs to finding a needle in a haystack. He postulated that mapping my tumor DNA to decipher my unique tumor genetic code could lead us to a tailored medicinal assault to cut off my tumors' blood supply and theoretically starve all of them out. Now there are different types of tumors just in my own body, but Dr. Barth said it is likely that they all rely on the same pathways.

So this week I found myself in Dr. Barth's office waiting to see if he has had any luck in this ridiculously modern endeavor. Dr. Barth came in with a warm hug and then informed me that what he had to share was incredibly cutting edge, that nobody is doing this yet, that it is brand spanking new, and that I am literally his first patient and one of a handful ever to have this testing done. He then told me that the testing successfully told him which types of receptors my tumors are expressing, and with this information he has identified which medication has the best chance of potentially slowing or stopping the growth and development of tumors in my body.

Can we just have a moment of silence as we all absorb that information?

Theoretically, he has identified a medication that could SLOW THE PROGRESSION OF MY TUMOR GROWTH!!!!!!!!!

He then said he would understand if I am unwilling to try, since this is all so new, and I may not want to be a guinea pig. Are you kidding me? I was born to be a guinea pig! Einstein said that "Insanity is doing the same thing over and over and expecting different results."


My grandmother, uncle, Mom, and numerous friends and amazing people I have known have been born with NF2 and been hacked and radiated to death all in the pursuit of a tumor-free existence. They all basically became deaf, blind, paralyzed and alone, trapped in disease ridden bodies that gave out before their lives had even had a chance to start. I am willing to try absolutely anything different. Dr. Barth could tell me I may grow a third arm and I would do it!

So, the next obvious step was to ask, "What is it???" It is called Pazopanib, also known as Votrient. You all have google, so I'll let you research for yourself, but the only significantly reported side effect is diarrhea. Dr. Barth said this apologetically, to which I raised my hands in the air as to mimic the scales of justice and asked, "Brain tumors? Or diarrhea?" We all smiled in response as though the three of us in that room knew something amazing, and we did.

There is one caveat, Pazopanib is FDA approved for kidney cancer, not NF2, and insurance does not cover off-label usage of medication. I casually asked how much the medication costs out of pocket and was told $9,000 a month. A MONTH. Dr. Barth said I need to be prepared for a fight with the insurance company, and I told him I will win by any means necessary. I will write letters, appeal, file whatever papers need to be filed, and if Medicare will not approve me I will write GlaxoSmithKline ten letters a day with pictures of my children attached until they underwrite the medication.

I will get the medication, and it will slow these tumors. Bossy, aren't I?

Now, I feel the need to be realistic because a lifetime of reality has taught me that there is no such thing as an answer. Consider that: no answer is ever absolute, nothing is ever forever, and life by nature is chaos. Fairness does not exist. It is why I am not Christian, I cannot abide by a belief in judgement. Life is not fair, why would death be? Most people believe that prayers and maybe a drop of karma should make a difference, I believe that if that were so entire countries would not be famished and angry in this world. None of us know. Know what? Anything. We know nothing.

So please do not assure me this will work out. I really think that given my current situation in life I will be able to start treatment in due time. There is a chance I will not be able to, because there is always a chance. I may take the drug, have a new MRI, and find that my tumors are shriveling up and screaming a little as they melt. I will believe it when I see it. In the meantime I am haunted by that image of my brain, and every time I close my eyes I wonder if they will open again. I wonder if they will open to darkness. I wonder if I will live, and when I will die. The difference is that now I really and truly have hope. A good man once famously said that love is all we need, but I say he was a bit off. Hope is all we need.

Film

I read my own blog and forget I am watching myself. My eyes glaze as memories play like a movie skipping through my mind. Without these words or pictures to prod my remembrance the stories are thin, and evaporate one droplet at a time. I console myself with promises to think only of the future. How easily one forgets the past once lay before us, and time does not exist.

A summer that is done seems to be yet to happen, should I still save this dress for a midsummer dream? Spring has sprung without ever ending, I vaguely remember a chilly fall day, and strain to recall smiling my way through the social season.

Clarity returns like a burst of actuality. Happiness blooms in spite of oncoming frost. How much was lost? You cannot miss what was never to be. You should not miss what you only expected. Expectations are meant to disappoint.

Now comes the poison that promises to extend life. Are promises meant to be broken? Practitioners smile and escape out the back door, out of practice and devoid of care. No way to explain that what people expect is not as tragic as they imagine and nowhere near as heartbreaking as one could suppose. The pain lies not in the nerves but in the heart when you wonder who will comb your daughter's hair. The pain cuts ever deeper as you wonder if your boy will have need for another mother to hold his hand as he dreams the same dream.

Days lie in waste when you consider their rarity, toil seems ridiculously uncouth in a life with such altered prospects. Nothing to conjecture but the end of time itself, no need to plan for a life not to be lived, and not possible to prepare for what I will not be here to oversee. Rueful smiles, unsolicited clemency, smothering hugs and empty offers of empathy haunt my days. I inspire nothing but take credit for everything, my accomplishments are mundane yet solicit high praise. I rush through the prosaic, seeking out the moments of purity, completely aware I only march closer to the ending credits. As they roll I will regret nothing, for it will be too late to suppose and too early to accept their finality. Life will live itself and require nothing of just one, there wont even be a blink of an eye.

Its in the Past

One year ago today is in the past. One year ago I ran a half marathon at Surf City in 2:05. I vividly remember the course, counting the miles down as I ran my heart out in my bright yellow singlet. I weighed more but felt stronger. My legs carried me without protest, they were trained to complete that race. Hours of time running in circles and praying toward the horizon had prepared me for each mile. People passed me, but I owned it, they flew by and were gone to run their own race. The pain, the happiness, the freedom of flying along by my own will. The views of that expansive blue we call an ocean. Sea air filling my lungs and a crisp breeze propelling me forward, then slipping out as I gasped for the next breath. The creeping pain building under my rib cage as I burned through more and more of my stamina. I knew I was running on empty, but soon the finish line loomed ahead, and I surged forward.

Running what I could never know would be my last race.

One year ago today is in the past, and all I can do is look forward. Surge ahead in a new race. Forge a new future without succumbing to the immense sense of loss I feel for that course. For every course.

State of the Brain Address

On Thursday Paul and I made the drive to Newport Beach to see my neurologist Dr. Duma. He has been my primary neurologist since I was a teenager, and I don't foresee that changing at any time. I trust him, we have a good rapport, and he has used Gamma Knife to treat over a dozen of my brain tumors without any major deficits. Tumor number 12 to be obliterated by Dr. Duma was my first treated tumor to have caused such serious problems as I have had over the past year. I consider that a damn good run. My concern now is that after my last MRI with him I was told the swelling was stable, when in fact #12's evil twin Lucky 13 was swollen and angry at the left side of my brain. For those of you keeping track that would be the opposite of stable. I wanted to see Dr. Duma myself for an updated opinion, a state of the brain address if you will. It was overdue, I mean I am a bit attached to my brain and like to make sure it isn't going to just explode or melt one day.

So I hobbled into the office... oh did I forget to mention I had an adverse affect to the medication that drains the fluid from around my optic nerves? Yes, it also depleted the fluid cushioning my joints apparently. Good to know, I found out the hard way when I sprained both my ankle and knee in one weekend. Ok carry on... so I hobbled in, we sat down with Dr. Duma, and his immediate concern was for my weight loss. The last time I saw him I mentioned food was low on my list of priorities these days and he told me I didn't look like I was hurting for food. Since then I have gone from 123 to 104 pounds. My family keeps saying I am too small, my girlfriends can't stop telling me how great I look, and I am just happy to be back in a size 2. Dr. Duma was only slightly amused, ordered another abdomen MRI and told me to eat more. I told him about my concerns regarding the imprudent use of the term stable when referring to brain damage. His response was that I seem to be doing fabulous lately, and that the swelling is just going to be a permanent irregularity we see on every MRI. He doesn't foresee any serious problems stemming from it. I said that may be so, but humor me, if this huge burn spreading across my brain does perchance become a problem, what are my options? He said that if necessary he would go in and surgically resect the offending tumors, that the option is there if necessary one day. He also pointed out that all of my other zillion tumors seem to be behaving right now, and as I had told him myself I am still seeing clearly even without the crazy fluid draining/ankle spraining medication that I relied on to see just a few months ago. He gave me a Medrol pack to help my joints recover, a prescription for physical therapy and of course the abdomen x-ray. At that point I was released back into the wild, free to live my life for another 6 months.

So now I am in a terrific mood. I am as healthy as I can be at this point, and on March 1st our little family of four is moving into a house! Yes, finally, we are moving on up. Life is good, fabulous really.

Non-Results?

So every 6 months or so I get all morose and assume I am dying when I have my MRI's and am forced to see the images of my atypical brain and spine splayed across a huge computer screen at NIH. Sometimes I look and want to get upset, but it seems I would be overreacting given the general apathetic professionalism the doctors maintain. "Look, here is your brain, we can't count the tumors so just be careful mmmmkay? If you die, please be sure to let us know so we can replace you immediately. Now time is money, keep it moving." I ask what a spot is and they look at me patronizingly and tell me that is just more edema, don't worry, if it gets too bad we'll shave your head, saw it open and cut everything out. Now buh-bye, oh and make sure the nurse has your current payment information."

I seriously doubt any doctor actually knows the meaning of the word "stable." 3 months ago I had a brain MRI here near home just to check the "possible edema," you know that massive cloud of debris smashing up a huge chunk of my brain? I was told I was stable, I looked at the screen and remarked it did not look stable to me, but was promptly shooed away by a tech and told not to strain myself looking at the pretty pictures. Fast forward to this week at NIH where I was told the edema has now spread to both sides of my brain. This news was delivered with a practiced straight face, and I took it as such that I was meant to simply nod. At this point they tried to put me back on steroids and I started laughing. I told them oh we have done that, repeatedly, different types at different doses. They made me manic and unable to get through the most basic of tasks. Yet here we sit, with more swelling, so no I would rather not continue the crazy medication that is not working at all. I told him the medication I take for my eyes has helped tremendously over the last few months, and not that he had asked but I was able to see much better on it which to me signified less pressure. He nodded his assent and we shook hands and said good bye. At this point he went back to his life of delivering news to patients with an unreadable face, and me to mine in the corner of my couch.

I am home on the same medications I left with, with a most likely shortened prognosis, not that I was offered a prognosis or options beyond steroids. It seems odd to me that while the simplest of tasks can frustrate me to the point I want to cry and can't speak, going through the motions of neurological exams and results feels completely ordinary to me. So the doctor and I have that in common at least.

Change

Before my eyes even open I smell the remains of a fire burning out and hear the roar of the sea nearby. Warm water laps at my toes and beneath is something as smooth as silk. I slowly look and see exactly what I expected, a vast expanse of black sand so smooth it cannot stick to the warmest of skin, running along a deep blue ocean without waves as far as I can see. The horizon is lined in pink and a soft sun you can look directly into warms me to the core. A deep breath draws in air, thick and sweet, without a hint of the ash the fire should have caused. The water begins to recede and an empty day settles on my chest. There are no numbers to count or time to pass here, no people who speak and nothing to need. Here I exist and nowhere else does. I remember things from before of course, but they seem of no consequence now. I do not ruminate or miss, I have left everything behind and live in a seclusion so thick that change seems both inevitable and impossible. A sigh escapes my lips and rather then explore all of the things I have seen before I simply lie back down and continue to exist. No dreams disturb me, for there is nothing to desire. No nightmares either, for there is nothing to fear. Each moment lies undefined and every step lacks depth. Finally, I place my finger on my lip and without further thought I bite myself as hard as I can. Warm blood trickles down and I realize I have not seen red since arriving wherever I am. I have not felt pain. Until now. Change has come.

Sleep & Run

If I could describe the last month in one word it would be, "Sleep." I fall asleep early, wake up and hustle the kids to school, then fall back asleep until it is time to pick them up. I grab them back from school and try to catch up on the insidious pile of laundry tumbling out of the hamper, do a round of dishes, wipe the bathroom down and rearrange some pillows. Then I lie down exhausted and try to answer homework questions from my Hello Kitty cave before throwing together a dinner. Every day I feel a little better. Every doctor's visit I get news, some good, some bad. Nothing tragic and nothing life changing. Sometimes when I am at the store and JT is helping me find what I need I stop and look around and for the millionth time I wonder what it would be like to be them. The same answer generally bounces back at me, and it says I wouldn't want to be. I like myself, oddly enough I love my life. Besides if I wasn't me I wouldn't have the family I have, and I don't know anyone with a family I would rather have. That may be the irrationality of human nature speaking, but I am comfortable with that.

In fact I am generally very comfortable lately. People have disappeared, as I knew they would, and I just can't muster the empathy to care. I've had the same best friends for a very long time and they aren't going anywhere. My family has proven themselves, not that they needed to, but time and again they do so anyway. I am surrounded by people I trust and love. People who handle everything because they want me to just rest so I can get better. What more can a girl ask for? Probably a lot of things, but no matter what you get there is always something else to want, so I choose to just be happy as I am.

I was definitely happy last weekend at the Long Beach Marathon with the NF Endurance Team!! My fave weekend of the year (right up there with Christmas, don't judge, I love fudge and presents.) So our entire team kicked butt. JT did the kids' run with Haley, and smashed the mile in 9mn! Paul's little brother Thomas kicked the 5K's ass, I mean he is like 14 and finished in 21:30. I wanted to adopt him and train him I swear. Uncle Franky and I stuck together for the 5K, for my safety and his motivation. I yelled at him the entire last quarter mile, then we ran into Sarah Johnson, and I grabbed JT from the cheerleaders, and we all ran in as well as we could for a big finish. It felt wonderful and I was proud of everyone involved!

Now its back to life as usual, whatever that may be for me, it works.