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.

NF Management 101 , or, Hey, Maybe things WILL be Alright.

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 3


After the shock of realizing G had NF, I started to search the internet. We only had dial up then, and a big clunky computer, but I managed to find the Children’s Tumor Foundation website and a bulletin board of parents of NF kids. I bleated out my pitiful tale of new diagnosis, and a mom reached out to me, talked me off the ledge, gave me hope. I am forever grateful to momma Beth for those e-mails, those words of encouragement. Her daughter was on chemo for brain tumors (ooooh yikes), but she seemed to be doing ok. I tried to wrap my brain around what NF would be for G. . . but it was too scary.

Bizarrely, (in retrospect) I was mostly scared about cosmetic stuff for G, the bumps that can come with NF (ie fibromas, small tumors on the skin). I knew kids had been HORRIBLE to Dave when he was a young teen, all because of his bumps…I knew that I might venture into uncharted levels of fierce of anyone messed with G. Well, maybe not “might venture”. I had always felt hideous as a teen, NF could not make my beautiful girl feel like that.

(and this was before we knew about TLC and programs such as "My 200 lb tumor", which generally is about NF patients. sigh).

Our pediatrician wasn’t super concerned, he did suggest that we see a neurologist and eventually get a baseline MRI for Genna, and that as soon as she was old enough we should start getting her eyes checked every year by a pediatric ophthalmologist. (ophthalmologist is quite possibly one of the hardest words to spell Ever. It took me twice to get it right here, which annoys me.)

We only told a few close friends and family. . .and then we tried to go on with life. Genna was a roly poly ball of fun and attitude, her big brother was a strong willed but curious and energetic kid, we had our hands full WITHOUT worrying about NF.

And really, things were ok.

At 14 months old we did get G that MRI, it was a nightmare trying to sedate her, she had to drink this awful purple sedative (maybe versed?) and she kept fighting the sedation, staggering around like a drunk sailor (we kept trying to hold her, but she kept fighting to walk around). The tech told us, “she is a feisty one!”. Yes. Yes she is. I sat in the waiting room and wrote melodramatic poetry about my baby girl. Like with references to “khaki clad doctors munching chips” (they were) and “tigress claws protrude”. Good stuff. Don’t know WHY I’m not poet laureate yet.

To our great relief, the MRI came back clear. The report noted that G had some UBOs (Unidentified Bright Objects. I’m not kidding. That’s what science came up with to identify these marks in the brains of NF patients. Seriously. The coffeemaker in the lab must have been broken that day or something). Otherwise, her brain was healthy. No sign of tumors.

I was so relieved, I had prayed and prayed particularly to a certain saint (it’s a Catholic thing; we figure folks who are in heaven have time and a mission to pray for those of us still down here. It’s kind of this cosmic solidarity thing that I frankly really appreciate about being Catholic). ..this answer to prayer seemed a direct result of those novenas I had prayed and prayed. St. Teresia Benedicta of the Cross was my NF optic glioma go-to saint…because that is what the MRI was looking for, the optic nerve tumors that list references, and we had scanned on Yom Kippur, which had relevance to St. TBotC. I figured that was a Sign from God. (I say it with capitals, that’s just how it comes out, I was so relieved).

(as a weird side note, optic glioma as a diagnostic criteria was only recently added to the list, based on research. Yay, science. Woo. Glad to help with that.)

Genna’s eye checks each August were also fine. A nightmare (if your kid has had to go to an eye doctor as a preschooler you know what I’m talking about), but the doc was good and G’s vision was perfect. Phew!

Eventually I even stopped being on the CTF board so much, the stories of families in really dire NF predicaments were freaking me out. Our life was full.

When G was 3, we decided I could try to get through another pregnancy, and we found out we were expecting just after the attack on the World Trade Center. 9/11 was intensely painful where we live (NJ), and after that I just had this horrible feeling of impending doom (great thing when you are trying to have another baby, right?) . I just felt like other bad things were coming. No joke, I knew something terrible was going to happen again. Sigh. (cue menacing music).

Just after Christmas, Dave’s grandmother became very ill. After a lot of confusion, doctors finally diagnosed her with a malignant brain tumor. Grandma was past 80 years old, she decided not to endure harsh treatments that would do little to extend her life, and within 2 months of diagnosis she passed away.

This was our first, horribly painful moment of personal brain tumor awareness.

Our grief over losing Grandma was somewhat assuaged by our Rosie’s birth in May of 2002. Rosie was peachy and fair, the opposite of Genna, and she was also round and healthy and perfect. Yup, smitten again!



I was in a hurry, and having a ptsd fest over looking at old pictures, so I just picked this one.
She looks like Andrew, right?


Aside from grief and that feeling of doom, things were good. Firstborn was in second grade, G was going to start preschool, we had a beautiful baby. . .

Genna was way into the baby thing, she would set her dolls up right by Rosie.

I miss those days. I hardly remember them, even writing this I have to think hard to remember what life was like then. We camped, we hiked, (Genna hated both activities even then, admittedly), we went to all of firstborn’s tee-ball games, Genna did ballet (which pretty much meant she twirled around to the beat of her own personal drum), we picnicked with friends and family, we were very involved in a prayer group and my son’s school.

But when G started kindergarten in 2003, things started to seem not quite right. NF was about to make its true self known.