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|
|CCNU: the largest capsules known to man|
|Vincristine...the iv push at CHOP.|
|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. ;)
And then: G’s port wouldn’t work.
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.
Our camping trip at Club CHOP had begun.