Reprieve!

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.

May 16

Well, G had to recover from brain surgery first.
She looked like Rocky Balboa at the END of the movies. She was berserk from steroids, berserk from the entire ordeal. At one point she screamed, “NOBODY EVER DOES ANYTHING NICE TO ME, EVER!” while a nurse was removing the arterial blood pressure monitor, an unpleasant task. But it was so funny how she said it. We laughed. Too much. But it WAS funny.

We had lots of visitors, it was a different world from our previous hospital stay.

After a remarkably short time (4 days!) we were cleared to go home! Of course Dave wasn’t there, but my sister and my friend were visiting…in a teeny tiny sports car. We jammed into that car… (as listed in Caringbridge)

1 big suitcase
1 overstuffed computer bag
1 backpack full of snacks
1 tote full of crafts
1 miscellaneous bag of snacks, crafts, and a small soft cooler
1 bag of stuffed animals given to G since we've been here
1 Hello Kitty bag with Genna's animals from home

into Mark's sporty car.

the five balloons might be tricky.
the four people might be trickier still...

but we're coming home!

It was like a clown car... but Never stand between a Camiolo and the way OUT of the hospital. ;)

Once home, G was still kind of a wreck. We had been together SO much, she had massive separation anxiety. Ergh.

And we waited. 

The day before our scheduled appointment, the nurse practitioner called to reschedule. Our results weren’t back yet.

Um, ok.

And yes, our doctor wondered if I was alive, I had stopped e-mailing every 15 seconds. But really, I had asked all my frantic questions. I could only wait.

Finally, on March 2, 2006, we were set to go to CHOP. It was the 4th anniversary of Dave’s grandmother’s death by brain tumor. I wasn’t sure if this was a good day (ie Grandma would be All Over God about this) or yikes (for obvious reasons). On the way to CHOP we drove by several horrible accidents (sigh) and heard Casting Crowns’ song “Praise You in This Storm”, which is pretty much about “why haven’t you answered our prayers already, God? But ok, You got this”…I tried to hold things together. But we were so afraid. So afraid.

And then we got to CHOP… I did not go into cardiac arrest, even when a social worker was waiting in the exam room for us.  Hospital lesson: Social Worker waiting in exam room for you is often a BAD thing.

Thursday, March 2, 2006 5:26 PM CST

TODAY WE GOT OUR FUTURE BACK!!

Must type quickly, supposed to be at MIL's birthday, with the best present ever.

The ominous areas in G's last scan seem to be a weird NF thing, possibly unique to Genna (unusual to hear that at a major pediatric brain tumor center). But we were given all sorts of much more dire possibilities, saw the words written on every consent form before surgery...can't even believe it. Actually might say "Yay, NF!" (although I told Dr. B we DON'T have a cheer like that!!)

And we will be restarting the TPCV, which kept the known tumors stable...can't even believe it.

THANK YOU ALL FOR ALL YOUR PRAYERS AND SUPPORT!!! We cannot thank you all enough...YOUR prayers carried us through all of this. The road ahead is still long, and rough, but we are still FIGHTING TO WIN...thank you!!

…may add more later, really have to run, but I know folks are waiting...couldn't wait to share this with all of you!!!
peace,

Kristin the AMAZED!!
off to eat pizza!!!

It WAS a weird NF thing.
Dr. Storm was right.
The pathologists were a bit flummoxed, nobody knew quite what to make of it, this weird gliosis-y thing.

again, from Caringbridge:
A NOTE: SATURDAY:

E-mailed Dr. B last night...confirmed that she has NEVER seen this before, the 3 neuropathologists have never seen this before...so that would make this turn of events a ....

miracle? Not sure what to do with that...other than thankyouthankyouthankyouthankyou....

3 Neuropathologists, 1 Neuro-Oncologist, (and a partridge in a pear tree?)---mystified (and happily so, I might add).

onward & upward, and thank you all for praying...

BT lesson: SOMETIMES, VERY RARELY, it is ok to stump the neuro-oncologist. Very Rarely.

But we had time back. And with time could come research and with research we can get a cure.

TIME IS THE MOST PRECIOUS THING NF OR BT OR ANY PARENTS HAVE. Make a note of that.

So back to chemo, back to the routine, back to living in the shadow of NF. But this time, the NF thing was a win. Getting to do chemo was a miracle. Not the miracle we had originally prayed for, but we were so grateful. For years after the radiologists still referenced this area by the scary terms the biopsy proved wrong…but THEY WERE WRONG.

from Caringbridge: So the battle is still long; but now we know G can fight another day, which is colossally huge. I can’t overstate miraculous this is—multiple doctors in varying disciplines prepared us for, well, really not good stuff…strange, so strange how the definition of a miracle changes. Strange how “weird NF thing” is better than alternatives. As my dear friend Susan Amato said, who knows what else God can do? (or something like that, I know I’m squashing that up).

We had our future back. We had our G back.

And less than a month after G’s surgery, 3 weeks after Miracle Thursday, we went on G’s Make a Wish trip, another miracle of love and generosity.

It's pretty surreal...can't believe the day is finally here, can't believe we're doing Make A Wish. Talk about the things you never dreamed you'd do...I had the opportunity recently to express to someone that the price for Make A Wish is so very high. We'd trade the trip to Disney in a heartbeat for health for Genna. Half a heartbeat….

Genna said today that it made her happy & sad that we are going. She had trouble expressing what she was trying to say...finally got it out that it made her so happy people were doing this for us, it made her want to cry.

Cruella met us at the gate to Magic Kingdom..."HElllloooo, Dahling!" she said to Genna as she swept through a crowd, straight to G. G was very concerned because Chip took her little camera.
EVERY character treated G like visiting royalty. It was astounding.

We rode that Amtrak from Newark to Kissimmee, a wondrous adventure (I LOVED it, sleeping on a train is awesome, I felt like I was in a history book), we stayed at Give Kids the World (a place completely built by, run on, and infused with love), we hugged princesses and unnaturally large chipmunks (G was ALL about the characters, she only wanted to hug characters, do crafts, and ride the occasional carousel) . We had the most amazing miracle time ever.



Sea World. I love that Andrew is holding Rosie's hand.

 No characters stepped on Rosie’s little toes (she was very concerned about this), Genna felt like a princess, even Andrew had an amazing time. Aside from a weird sunburn on G’s hands and feet, everyone stayed healthy the whole time, also a miracle, since Genna was back on TPCV again…

Genna singing YMCA--her favorite-- with one of the volunteers at Give Kids the World.
You can just see where the incision was ...to this day, when she swims, you can see the scar.

Things were still tough. I didn’t know whether to laugh or cry or twitch or hide under my bed or climb a mountain. But we had another morning to wake up; our work wasn’t finished yet.

Chemo #3 and a pile of Ughulous Firsts

Part 13 in my series on life with NF and brain tumors. The next few posts are hard, I'm not going to lie...but for context, know that last night Genna got to meet Scotty McCreery, American Idol winner and "major babe" (G's words).  There is hope, even in all this ugh, there is hope.

May 13


These next few posts are really hard…and too long. It’s just such a marathon, such a long story… but with NF and brain tumors, sometimes the things that happen are so unbelievable, they need a voice. But I’m sorry this is so long.

We had failed another chemo. Just when we thought we had reached the finish line, we had failed. New inoperable tumor in G’s brainstem. Brainstem tumors in NF tend to be “indolent” (that is the word the abstract uses), but “tend to be” has far too many exceptions in our experience. And the brainstem is a horrible place to have tumor, even an indolent one.

So now, in October of 2005, Genna had bilateral optic, chiasmic, hypothalamic glioma, a cerebellar glioma, and brainstem glioma.

We were back at the beginning again, with nothing really amazing in the pediatric oncology arsenal to throw at these blasted tumors.

After some discussion, we opted for the protocol we had rejected a year earlier due to risks of secondary malignancy. Brain Tumor Fact: brain tumor growth trumps risk, a lot of times. Sometimes you have to fight with what you have, even if it’s not a great option.

There were some significant communication issues with CHOP, I thought we had flunked out or something, but no, we finally were set up to start Thioguanine, Procarbazine, CCNU, and Vincristine.

G had JUST gotten her reflexes back after her first go around with vincristine. Easy come, easy go…but it bugged me. I wanted her to have reflexes, it just bugged me (ie the doc would whomp her knee with that tiny hammer and G’s leg wouldn’t move). I was also overwhelmed at the prospect of what kind of sticker charts I’d have to make.

Anyway, TPCV is done mostly at home, the T, P, and C are pills, given on a strict schedule over 3 or 4 days (like every 6 hours around the clock for the T, every 12 for the P, day 3 start the C, etc). It was so complicated, I had a John Madden-esque chart listing what happened when. At least G could mostly swallow pills at this point. On days 14 and 28 of the cycle G would go to the hospital to get her counts checked and her vincristine via her port.

Thioguanine. Lots of teeny pills

Procarbazine. A fewlarge capsules

In December of 2005, we began chemo #3.

We did quickly learn that yes, you can sing TPCV to the tune of YMCA, AND do hand motions. G had the onco clinic in a heap of hysteria when she first pulled out THAT particular production number.

Even my 3 year old Rosie liked to sing it around the house.

CCNU: the largest capsules known to man

                                         



Vincristine...the iv push at CHOP.

Christmas came and went, my sweet kids embraced that day, my firstborn dressed up like Santa for his sisters.

He made the beard out of tissue paper. It is good for me to remember these sweet moments, now that he is 17 and can grow his own beard.
Ok, well, he can't, but he tries. ;) 

 Two days after Christmas was a CHOP day, but at least Dave could finally come with us.

And then: G’s port wouldn’t work.

Uh oh.

The nurse could flush it, but couldn’t draw blood. We tried putting G in different positions, we tried medical drano, finally after 6 hours we were sent down to interventional radiology to get a picture and see if something was clogged.

G was not amused, this day was taking too long.

After a bit, we saw Dr. B coming down the hall.

LIFE LESSON: if you see your doctor on the wrong floor of the hospital coming to find you, this is never a good thing, which I pointed out to our doc as she approached us.

Apparently, in a bizarre “we rarely see this!” kind of moment, Genna’s port had broken. Half of the tubing had floated into her pulmonary artery. This was quite an amazing picture, I so wish I had asked for a copy. The port was on one side of her chest, and in totally another spot we saw the tube. We have no clue how this happened.

Being unique is NOT SOMETHING YOU WANT IN ONCO WORLD.

So … we were stuck. G needed to have a catheterization to fish the tube out (ie a little lasso sent up her artery in her leg or something) and then the broken port had to be surgically removed.

Now Genna was REALLY not amused. I had told her you don’t stay at CHOP for a clogged port. And Dave had to bring Rosie home (we had thought it would be good to bring her along), AND he was supposed to take Andrew to a Devils’ hockey game as a Christmas gift…and this was Dave’s first time off after months of extreme overtime… oh, the family wreckage…

We got settled in a room, a nurse practitioner came to explain the procedure, and made it sound WAY more dire than our doctor did. Eventually the neuro-onc on call came to talk me off the ceiling.

The next day we rolled into IR (interventional radiology) and a lovely Irish doctor with a shamrock embroidered on her lead apron came to chat with us. We also had the Santa Claus anesthesiologist from G’s first day, I loved him, and we instantly felt better. We sent G off and waited. The procedure went well (only later did we find out it was that doc’s first time doing this…apparently it’s VERY rare to see this happen, alas). We were set to return about 2 weeks later to get a new port in.

Happy New Year to Us.

By “new port day” the sedation & IR people recognized us, G got her new port and we went home.

To add to everything, during these weeks we lost two brain tumor friends. Two children we had been praying for—Dani-Ella and Justis. My heart was breaking, even as I tried to keep moving. Everything paralyzed me. Grief, exhaustion, stress...

And in the middle of all this, G needed to get glasses. My genetics of near-sightedness finally made itself known…G’s one eye, her better eye, was near-sighted. She was sooooo excited to pick out pink glasses.

A few days later, on a Sunday ,my sister happened to be over. I have half a dozen sisters, but the one who is a nurse was visiting, and I showed her G’s neck, which looked red to me…

Except that when G pulled her shirt over to show Aunt Meg her neck, we saw that around her new port was a giant red circle. Giant. Red. Oh snap.

HOW HAD I MISSED THIS? Bad mom, again…

My sister told me I had to call CHOP stat, so I did, and they told us to come down. Right away.

Genna was irate. Livid. Aghast. We had borrowed the movie Madagascar from friends, and she WAS WATCHING IT AND DID NOT WANT TO GO TO CHOP BECAUSE YOU DO NOT GO TO CHOP ON SUNDAY.

Brain tumor lesson number…ok, I lost count. 527? Brain tumor crises never occur at convenient times. 8 years later Genna STILL talks about missing Madagascar that day.

We went to the ER at CHOP, they gave G an antibiotic, I went to call family…came back, and watched G re-enact Violet Beauregard’s blueberry scene from Willy Wonka…except in red. She literally had a line of red moving up her face. Um, yikes?

Nurses were called, the vancomycin was stopped, Benadryl was started, and “Redman’s Syndrome” as an allergic reaction was added to G’s chart. I think the Redman’s Syndrome namers might be the same guys who came up with UBO’s, those Unidentified Bright Objects NF patients often have in their brains. Ahem.

From CB: G then made up a funny song about how Daddy was red, blue, green, purple...he had the rainbow flu, he needed rainbow medicine...

They asked if G had ever had a transfusion, I said no, Nev-er!

Life Lesson: While at a hospital with an onco kid, NEVER ever say that you haven’t done something. Ever.

Within minutes a bag of blood was being set up, G’s counts were horribly low and she needed her first ever transfusion.

From CB: Somehow this was kind of a threshold moment for me...our first transfusion. Watching someone else's blood go into my child at 1:30 a.m. was sort of not so great.

This day (now night) was not improving.

Add to that, if a child has an infection (G’s port had cellulitis around it, although it seemed the port itself was ok) AND her counts are very low, she is not allowed to leave the hospital.

Oh….ugh.

Our camping trip at Club CHOP had begun.