Sorafatastrophe, or the 4th Worst Day Ever

For the month of May, in honor of brain tumor and NF awareness month (so convenient to have them at the same time!), I am trying to tell our family's story of living through both. I hope that by the end folks have a sense what brain tumors and NF can do to a family, why and how we can work for better treatments (and maybe a cure!), and why we should always, always hope.

We've almost made it! thank God, this has been way less fun than I anticipated.

May 28

From Caringbridge (and sorry, these recent ones mostly are. I just have nothing in me to elaborate much further).


Hey, all.

Tumor Battle 2012 has commenced

At 6 a.m. G swallowed her 3 pills and started smiting her tumors. She was pretty upbeat about it once she realized she could swallow all 3 at once. Then she & Dave went out to get First Day of Chemo Bagels.

This led to a cheerful bopping about singing of “first day of chemo bagel…la la la la la …wooo!”

Sigh.

Please pray that the side effects are too yikes. I am going to stay at school after my classes until the end of the day just in case she needs me. This is my plan for the near future. I’m also hoping I can then keep most of my schoolwork to school, and leave home to home.

At least she was really perky when she left today.

There is some relief in starting, I guess.

I didn’t sleep at all, I knew I had to be up by 6, so…I kept waking up and waking up and waking up all night. Then one of the pills dropped on the floor when I was opening the bottle, and the dang thing rolled…the sight of me & Dave crawling around the kitchen floor searching for the tiny investigational drug that was so hard to get and likely is not supposed to be dropped onto a kitchen floor and lost ON THE FIRST DAY! Ack. Dave eventually found it…

Dose 2 will be around 5:30 today…G can’t eat for 2 hours before or 1 hour after, so that might be tricky, but …Game Face is Now On.

Here we go…

Peace,

k

Game Face On...Tumor Battle 2012 Begins
and if this teaching thing doesn't work out,
I am going to start a motivational t-shirt business

And so it was.

We got into the routine of it pretty quickly, this Sorafenib thing. I got pretty good at explaining it to people, this trial drug. We bought G all sorts of soft shoes since Sorafenib can be hard on the hands and feet. Weird, right? G hated the fast after snack in the afternoon, the odd timing of meals AROUND chemo. I felt constantly on edge of “don’t forget!!”

Rosie had a stable scan, we had many epic trips to Philly…and G slogged through. She tolerated the medicine really well,mostly. She was so tired, but generally ok.

Until the President’s Day session at Camp Sunshine.

She just wasn’t herself, she was refusing food, falling asleep randomly (like to the point where she was scaring the other bt moms.)

BT NOTE: If you are freaking out other brain tumor parents with your brain tumor kid, you know you are in Deep, Deep Yikes.

We nearly went to CHOP over it, but then G perked up a bit. But generally she was so tired. Her feet hurt. Her limbs cramped. She was exhausted, and just sad. She would lie in bed and worry in the mornings, instead of getting up to go chat with Grandma next door.

Reading through these entries on Caringbridge hurts my brain so badly.

G is still struggling with fatigue—I have come to understand that weekends are just sleepy times for her, she pretty much lay in a heap all day; the last 2 days her feet have been more sore…I can’t SEE why, I believe that they hurt more, but there’s no new blisters I can see. Her ears are a mess…I didn’t realize how bad they were because of her hair, but eek…they are like peely reptile ears. My poor G. Lotion does seem to help. Sorafenibed again!

That said, we were talking about NF the other night, a little friend from our slide show above just started chemo this week, the same one G started with back in ’04…so the girls had questions, and I apparently have some level of PTSD. Urp. But Rosie wanted to know about NF1 and NF2, which was worse…trying to explain genetic variability to a 9 year old is a bit tricky…finally I got to “some people have NF badly and some have it not so badly”. G piped up, “I don’t have a very bad case.” Rosie jumped right in, “I hardly have it at all!”

Genna has multifocal diffuse inoperable brain tumors, a vp shunt, vision impairment, learning challenges, and she’s on her 4th chemo protocol. Even my sweet Rosie, who has not needed treatment, has 2 tumors in her head.

You can’t really have only a little NF.

While I managed to just kind of smile and nod, I was struck by how that moment reflects who my G is, particularly. She is so mighty. She just takes whatever comes…sometimes with fear, reluctance, and sorrow, but she takes it and keeps on going. She is so mighty…and Rosie wants to be mighty too, and I can see she is growing into that, and I pray she is spared what G has had to endure…

And then it was Holy Thursday, and time to scan…and we left for CHOP so early, and got G in…

And everything, every single hope died that day--not for the first time, but anew.

Thursday, April 5, 2012 8:59 PM CDT

Hi, all.

Today’s final score: tumors 10 billion, sorafenib zero.

G’s scan was a lot worse.

Like, even WE were kind of shocked…

So we regroup, we plan, we start again. And we beg God. We beg for mercy. We beg for what so many other parents have begged for their bt kids.

G said to me today, before we saw the doc, “Mom, I told God whatever He wanted to do was ok, if I have to stay over, or start another chemo, it is what it is”. And I took her hand and told her she was so brave…that I knew she was scared, but she was doing what she had to do, and that meant she was so brave. I told her I was so proud of her.

We have to wait 4 weeks for the Sorafenib to get out of her system…and then we will begin again, if I have my way it will be on the day that marks 4 weeks or just after…the beast has to know that we are not going down without a fight.

G took it pretty well, MUCH better than in December. She was pleased to not have to do chemo at dinnertime anymore, she asked if she could just get a port and get it over with…but no. She knows we will have a plan, likely some cocktail involving avastin.

As we drove to CHOP at 5 am, we marveled at how gigantic the moon was, so big and white…then gradually yellowing, until it slipped beyond the horizon…and I realized that song is right. The darkest hour really IS just before dawn.

All I can do now is pray for dawn.

Thank you all for praying for us. Thanks for all who texted G with jokes, pictures, random chitchat…that makes her smile. And come hell or high water, I need her to smile. She was smiling at the end of the day, our long talk with Dr. B got G some one on one time with the child life specialist, Megan. They were playing cards when I retrieved her. “That was so fun, mom,” she said…

I may or may not go on facebook tonight, to see everyone so sad is like knives in my heart, and my heart is already broken today. But thank you. I also learned I get wickedly carsick if I text in the passenger seat for 2.5 hours. Ergh. And tomorrow I can’t eat, so this should be interesting. Good Friday my fat fanny.

I knew this was coming, but it doesn’t make it any easier.

We were devastated. Our priest referred to the “economy of words” this past Sunday, and on Holy Thursday, 2012, the economy of words simply could not describe our brokenness, our fear, our despair. Sorafenib didn’t just fail. It was a catastrophe. We have never seen that speed of tumor growth, and I hope we never do again. It was horrifying, those scans.  Horrifying doesn't begin to describe it.

Our poor G…

Back in the Saddle Again

I'm sorry, this one is SUPER long. I am running out of month!
Don't worry, in this house EVERY month is brain tumor and nf awareness month...

May 27


I know, as I go through our story, that it becomes almost ridiculously repetitive…statistically improbable bad stuff happens repeatedly! Stability achieved! Scary scan—rescan in 6 weeks! Stability achieved! Lather, rinse, repeat…

But you know, that right there is pretty much HOW life with NF and low grade brain tumors can be. When our doc talked to us in the very beginning about what was going on, she referred to G’s situation as a chronic illness, and it is…just a chronic illness that can take a catastrophic turn in a heartbeat.

So I apologize for the redundancy. Makes a bit more sense now why I am a tad psycho, no?

But in the fall of 2011, we had once AGAIN dodged a bullet. G was stable after growth. We always had a very hard time with these 6 weeks after scare scans…the area of concern was never SMALLER. How can we be zenlike if nothing is smaller? But weird low grade brain tumor factoid: generally you only treat if there is growth or symptoms. G’s tumor growth had stopped…and she was asymptomatic. So we waited. Twitching and increasingly neurotic, we waited.

bullet dodging day

(and that wasn’t just me. Dave was aghast at the “growth but then stopped is still ok” thing).

So 9 days after scan day, I had to run my first half marathon, that thing which had seemed like SUCH A GREAT IDEA right after the bad scan. My knees hurt, and I was TERRIFIED. But race day dawned bright and cool, and really, the entire day was one of the most empowering of my life. Don’t get me wrong, it was hard. At mile 5 I saw my girls, hugged them, got the oomph to head into Fairmont Park…mid-park an announcer saw my neon singlet and announced “NF IS IN THE HOUSE” so I cheered back at him…By about mile 8 I was like “are we EVER going to start aiming back towards Philly again?”, and by mile 9 my knees were starting to really hate me…but at mile 10 I was all “WE GOT THIS!” to anyone around me, because ANYONE can do a 5k, and that was all I had left! At mile 12 I pulled out my Team Sandy/G-foRce! keychain, clutched it in my fist, and knew that Sandra was there cheering me on and telling me not to stop…at mile 13 I saw my family, and I was overcome with emotion and sprinted to high five them…and then realized I still had .1 to go, and my legs were DONE. Oops. ;) But I made it, and I was SO PROUD OF ME.

More phobias overcome in 2 hours 17 minutes than I thought possible. And I whomped some NF while I was at it.

These races are so important. Our NF friends on the team in 2011 meant so much to us, and continue to (Kris Whalen, who told me I COULD go 13.1 miles in all one day, Trish Budlong and Steve Kendra…these people mean so much to us).

And then…back to the race of regular life.

School was so hard for G. Just so hard. And the more scares we had, oncology wise, the scarier I became. My fuse for any kind of anything towards G was at an epic low. (That, unfortunately, has not improved). I felt like moving forward became harder each time we had a yikesish scan.

But in October Genna got to meet her other favorite singer, Matthew West. He called her up on stage during his concert and sang Strong Enough and Survivor and all the songs that mean so much to her. She was glowing with delight.

Dave ran his first full marathon in Philly at the end of November, it was a logistical yikes of getting hither and yon along the race course to give him the specific hydration things he needs (we are so careful about that after his weird collapse), and of course Rosie had the stomach bug the morning of the race (my brave girl managed to walk all over Philly to help Daddy). But once again, Dave NFE’d for our girls.

And then, right after Thanksgiving, we were due to scan again. Both Genna and Rosie were due to perform in the Nutcracker through their dance studio…my girls have been blessed for years to dance at AP Motion in Flemington, where Miss Joanna always found a way for my visually impaired, galumphy, vaguely unbalanced child to feel like a dancing queen. So G was due to be an unlikely ballerina the weekend after her scan.

December 5, 2011, is our own day that will live in infamy.

This one is so hard. Five years was just not long enough. But here it is, from Caringbridge, December 5, 2011.

"we're ba-ack!"...

at CHOP.

Inpatient.

Facing chemo.

After shunt surgery scheduled for tomorrow.

Every single ounce of my being hurts right now. We knew it was bad as soon as we saw our doctor...

I just can't believe we are here again.

I can't believe 5 years has come and gone and what do we have to show for it?

Walking back onto the onco floor was like stepping into the Twilight Zone, albeit a TZ with newer beds.

G is so upset...thanks to everyone who has texted her or called her, that is literally carrying her, when she is texting or chatting she forgets...now she is watching the CMA's (that would be COUNTRY MUSIC awards? hello?), she just had a doctor ordered bath pre-surgery and I have to do some weird anti-bacterial wipe thing later. We met the anesthesia guy, I have papers about 3 different chemos to choose from (and I know of 2 or 3 others I don't have in my bag). My head is throbbing, and I am eating a salad...I know I have to eat.

I was shaking so badly today when talking to our doc...completely held it together until I had to tell G. She had left the room...

Dear God, I cannot believe we are here again. I cannot believe she will be starting chemo again. She asked if she was going to lose her hair...

As G left our house at 4:40 this morning, she was singing in a deep bass voice, "I hate scan day! I hate scan day!"...

I hate scan day too.

please pray for us, we are just so broken...and my other kids are at home, and I don't know what to do for them, and I have no work for my classes...and how will I keep working?

unprintable moment coming, or tears....

I HATE NF AND BRAIN TUMORS WITH EVERY FIBER OF MY BEING, AND I WILL NEVER UNDERSTAND WHY THIS HAPPENS TO INNOCENT CHILDREN. I just don't.

please storm heaven for my poor G...and my other kids, too...and for me & Dave as we try to pick our poison literally for G.

peace from 3 South room 21...oh my God, I cannot believe we are here...

k


We finally had met a bullet we couldn’t dodge.

At least I had packed a just in case bag. Dave didn’t.

Her surgery went well, and started early (less fasting!), and Dr. Storm was super fast, fast enough that I was terrified to see him (his response when I said “why are you here already?”…”what, am I not that good?” , which struck me as kind of an awesome answer, honestly.

Back in the beginning, in 2004, when I read the book about brain tumors all in one sitting, people said in a very late chapter that relapse is often harder than the original diagnosis. I feel this might be true, because we KNEW, we knew what chemo was like…to be back at the beginning again was crushing.  And G was so much older, she KNEW, she understood...

this is how she really felt. Sigh. :(

See, NF optic glioma tend to go dormant at puberty, the ONE good NF thing ever. Non-NF optic glioma tend to fuss about destructively much longer. G’s optic glioma were still stable. But OTHER NF brain tumors don’t have this handy rule. So the area of concern was in G’s hypothalamus and brainstem, and it was obvious and ugly and terrifying and we weren’t sure if the shunt was just clogged or if tumor had blocked it again.

Thankfully, it was just normal clogged. We were home by Wednesday.

And as I struggled to pull myself together (we had so many decisions to make, and since we had blown through three chemos already, we were at the bottom of the barrel chemos now—or, as I said on Caringbridge, “Highly craptastic choices from a melange of sucky alternatives”), G got ready to dance in the Nutcracker on Saturday, December 10.

so freaking mighty. 5 days after freaking brain surgery.

.  

That day was a miracle. G gutted it out, she was so beautiful. She was so mighty.

Ultimately, Dave and I decided (with our doc’s help) to enroll G in a Phase II clinical trial of a drug called Sorafenib, ironically a drug I had researched for plexiforms after Rosie’s diagnosis. This drug was an oral chemo, so fewer hospital trips for G and no port…quality of life was a huge issue for us, now that G was a teen she had a say in what we did, and she really didn’t want to go to CHOP a lot. But to do this chemo G had to scan again at the end of the month (literally 12/31) and then wait until we could get approvals and such…she had to be a month post surgery…

So we were given Christmas.

Hard to deck them halls and falalalala in these circumstances, but we tried. G told me about 5 times a day that she didn’t want to start chemo. I couldn’t focus on anything, our shock over starting again was so pervasive…

Realizing too that in less than 2 weeks we are starting again is kind of hitting me. I think in the first week or so of ugh we were able to kind of focus on the YIKES, we are starting…but in kind of a theoretical way, like, we’ll be going to CHOP more, what a pain. G will need a treat post each cycle, better think of that. How will I ever get lesson plans or substitutes for my classes? Oh, is chicken on sale, better stock up…in the last few days there’s been a mental shift to dear God, this is a TRIAL drug. We don’t even know if it will work, and G’s tumors are so obnoxious in the face of treatment…we failed 2 last time before one worked…her tumors could very likely GROW while we’re on this…old feelings long buried. We have been here before. We know what chemo failure is, it is NOT theoretical for G. And this drug has so little info, but once you blast through 3 options …well, the statistics on anything aren’t super great.

And we know too many NF teens who battled long and hard. Past tense. We have so many bt friends in the thick of the battle right now, and NF friends too…it’s brutal in our community right now.  Ugh.

And Genna is scared, and not sleeping well, and…sigh.

This has not been a festive week in that corner of my brain. And I know, I know we need to be hopeful, and we will get our game faces on, and we will march forward bravely into the night, but still, it’s freaking terrifying. I think some part of me hoped and secretly thought G had gotten to some magic stable place. She had been off treatment 5 years, and at least 3 of those were mostly drama free-ish. We were the hopeful story.

And maybe we still can be. I am just saying it like it is. The reality is, this is serious. Low grade brain tumors are serious, and I have TWO children with brain tumors. Both of my little girls. This is weighing on me this week. I say it here, so when I see people in real life I can speak without falling apart, I can joke about G’s mighty attitude, I can talk about shoes, or whatever, and NOT fall apart. So far so good. But truly, this is so not good.

Genna is mostly nervous about kids making fun of her (that won’t happen), missing school, feeling weak in her legs, missing school, losing her hair, missing school, etc. It’s a lot for a kid to process, and it is so not what I hoped for my daughter.

But once again, people showered us with love and kindness. We are so grateful for those people.

G’s New Year’s Eve scan was stableish,

scan day 12/31/11

we were safe to start Sorafenib, and after ridiculous delays, 18 pages of consent forms, and bopping back and forth to Philadelphia, we finally had those blasted pills in hand.

And G began her Tumor Battle 2012,  tiny pills in the morning,  tiny pills at night.

Back in the saddle again…