Chemo #1

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 8

I remember nothing about the week between shunt surgery and returning for the port/chemo day except that I did not eat. I think people brought us meals, a friend brought me a 3 lb bag of M&Ms which I couldn’t open. I did make a prettier cover for the info binder they gave us (see my post about binder trauma from last year at this time). My other kids had no clue what was going on, my Rosie started sleeping with us every night, Andrew was a wreck, too.

I found this one picture from that week.
Behind G is the massive Care Bear someone gave her.
In that first 2 week period I think she got about 2 dozen stuffed animals.
this is also our old kitchen. ergh.  

At some point this week (or thereabouts), I realized that Rosie had café au lait spots too.

Yes.

if a mom's brain implodes inside her skull, will anyone hear it? 

No words. I had zero to even fathom that my baby had this horrible NF thing too…

And Genna was in complete horrifying meltdown.

Apparently when you have significant hydrocephalus and it gets relieved, the change in intercranial pressure can affect behavior significantly. Hypothalamic tumor can also affect behavior, and between the two, Genna was in terrifying OCD type meltdowns several times those first few days.

Aside from that, we had school to get ready for. I had humanity to hide from. Shock covered everything we did, we just sort of fumbled through whatever the doctors told us to do.

And my first NF friend, momma Beth, sent me a long email about chemo and surviving. I still have that letter, printed out, in a binder. My family is STILL grateful that Beth suggested we get more tv channels (we had only basic antenna channels, no cable then), so G could watch kid shows when she didn’t feel good.

Every time we got bad news after that, we got more channels. We now have approximately 749 television channels, indirectly thanks to NF and brain tumors. Sigh. You don’t see THAT in any of the literature, do you?

Here is how I described it on Caringbridge:

Thursday, September 9, 2004 1:53 PM CDT

Hello, again.

A quick update on things here. Genna got her haircut today. (we knew G might lose her hair, this was a pre-emptive strike) While I was pretty emotional about it, it actually looks really cute. Kathy G is a goddess, and even brought treats. I should have brought Genna to her ages ago.

We're trying to determine whether or not Genna can safely start school: she received her chicken pox vaccine years ago, but apparently there were some hold outs in her class. Chicken pox is very dangerous for kids on chemo. I have a call in to the oncologist to make sure G's vaccine will hold up once she starts chemo, or if recently vaccinated kids are a risk. I really hope she can start school; she's so excited.

Which brings up the one other thing I figured I should mention, especially for those folks who see us around. Genna is completely asymptomatic at this point. She seems fine, which is disconcerting, I know. She just went outside to play. While this is great (i.e. thank God the tumor, while quite large, hasn't hurt her much yet), it's made it that much harder to wrap our minds around the reality of diagnosis. So if you see Genna, or call here, don't be surprised if she's as feisty as always.

And thankfully her behavior has levelled out a bit; post-surgery was rough here, which is apparently a common result of a sudden decrease in intercranial pressure. A little less "ferocious diva", a little more our sweet Gennahenna.

We hope to have some family time this weekend (since last weekend's planned getaway didn't happen), and then off to Philly on Sunday evening. We should be home by dinner on Monday if all goes well.

thanks for your continued prayers,

Kristin

Anyway, G started first grade, so happy (although we had to tell her she could NOT keep showing kids her abdominal scar from the shunt. Eek) , and then on September 13, 2004, my dad’s birthday, we showed up at CHOP, ready for a port and chemo.

We were petrified.

G got through surgery fine, a port is not a major procedure. Essentially a little metal and rubber plug (almost like a wine-bottle re-topper you can get) with a tube attached to it was placed in G’s chest. The tube went right into a vein. This way nurses could plug a needle into G’s chest without having to search for a vein; we could numb the skin over the rubber plug and G would have no pain.

this is fancier than the one G had then. Hers was round and blue, not triangly and purple. I wonder if the one she has now is purple, she would like that.

G got rolled back to the day hospital (we learned that is the name of the place kids get chemo), a nurse came in with a tiny vial of clear liquid, and I was kind of agog, I guess I expected chemo to be glowing neon scary smoking green or something. I made a comment about poisoning my child (oh how it hurt my heart to see that medicine put in my G), the nurse got a little irked at me.

The second medicine, the carboplatin, took longer, but G slept through the entire thing.

Sigh. One chemo down, only about 60 more times to go.

Carboplatin /Vincristine is often the first therapy children get for low grade brain tumors. It is given once a week, after 10 weeks you scan to see if it’s working (has the tumor stabilized, or, in my mind, shrunk even a little), and then you do it for a year.

I kept thinking, G will be well into second grade when we are done with this. That is so long, a year.

To our surprise, G did pretty well with chemo. Of course the day AFTER surgery/first dose she spiked a fever, which meant we had to go to our local ER and spell things and NOT let them touch the new port and get admitted and find out G was fine.

The rest of the week G had some stomach aches, but as day passed into day she kept almost all of her mountains of hair, she was tired but still got to school for at least part of each day, yes, she would wake up in the night and come tell me that she just didn’t feel good, AND she woke up every day at 4 a.m. FOR THE DAY, but we were surviving. We were getting into a routine. Each week Dave & I would take G to the hospital. We started to know the nurses, G had a few favorites who would help her through the ugh of port access (just because the needle in the chest doesn’t hurt doesn’t mean a kid is ok with the process. It’s way not cool).

I couldn’t even speak to God. Jesus I was ok with, but God…I had no words for Him. Well, no printable ones. And Teresia Benedicta of the Cross? I was so mad at her. I was just so angry, and so afraid, and so exhausted. And angry.

About a month into things, G’s behavior was getting scary. More and more she was falling into odd OCD rituals, erasing mistakes on her homework 15 and 20 times, until she made holes in the paper. Re-walking pieces of sidewalk until she got it “right”. She had an elaborate 20+ step bedtime routine that had to be followed exactly, or she would become hysterical.


some of the ritual. Everything had to be done in this order, or we had to start over.
The chart is an attempt to limit the growth of the ritual,
a tactic that DID work, eventually.  Well, with medication help,  too.


After Rutgers Homecoming, an attempt at normalcy, G pulled away from me in a crowd of thousands to “re-walk” a piece of sidewalk. I knew we needed help.


 

that day at RU. My poor baby.



trying to have a normal day for Andrew, too.

We had a long conversation with Dr. B about medicine, and we had G start talking to a psychologist. Brain tumors can often cause emotional and behavioral difficulties because the dang tumor squishes the parts of the brain that regulate impulse control and behavior. We had a very painful meeting at G’s school where a teacher asked if G wouldn’t STOP erasing or re-doing work, wasn’t that a character issue? Wasn’t she being disobedient?

I told them if they had a problem with her behavior, to take it up with God who gave her the brain tumor, and then I started to weep and everyone pretended not to notice.

Awkward.

We did decide that Genna needed to start only going to school half days. She was just so tired, so tired all the time, she was napping every day in the nurse’s office after lunch.

On the OCD front, after some tweaking of meds, and work with Merritt (oh , how we miss Dr. J!), G did start to improve. Those meds gave me my child back, and I am grateful.

We were chugging (ok, maybe limping) through induction. Scan day was set for November 29/

In early November I got a call from the school nurse.

Genna had failed a vision test at school, like EPICALLY failed it.  The nurse was concerned.

I felt that feeling again, that sinking, collapsing feeling, like my insides were draining out through my toes.

“G, “ I asked, she was sitting on my bed by where we had our computer, “can you see me?”

“A little!” she said cheerfully. I was maybe 8 feet away.

I started asking her if she could see things in the room, random things.

And she couldn’t. Most of them she could not see.

Somehow she had compensated so well, none of us realized she was going blind.

No inspirational music filled my head. Just despair.

Meetings, Medicine, and Making Sense of the Madness

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.

G went to school today, sleepy (apparently she fell asleep during English), but flush with the good news of a decent MRI yesterday.  She is happy. I have to embrace that and celebrate the hope of each day.

May 7

This one is long too, I am sorry. I’m leaving out so much. . .

The neurosurgeon was freakishly young, and ridiculously zen.

That was all I could think as we met with him, in his office at the Children’s Hospital of Philadelphia. He was SO calm. And so young. Granted, we were so young too, but whatever.

I think he showed us a model of the brain, I think he talked to us about G’s scan, I know for sure that he wasn’t as worried as the pediatrician. We did get to wait a day, not all hydrocephalus kids do, so he was pretty zen about it. He told us we’d have to have surgery the next day, a shunt would be put in to release the fluid, a tube would go from the obstructed ventricle (G had “obstructive hydrocephalus”, ie a blockage caused by tumor), through G’s neck and down into her abdomen.

Yikes.

After we talked to him, he sent us to talk to a neuro-oncologist, a brain tumor doc. G was pretty hungry at this point, but we had no clue if we had time to get her food at the cafeteria, so we tried to hold her off a bit, we promised her a happy meal later.

I don’t remember ANYTHING about getting to Dr. B’s office. Not one thing—except that there was a sign that said “Neurofibromatosis”—I would not have to spell it for anyone! This was a huge comfort (that sign is now gone, but I still don’t have to spell out NF). All I remember is that when we got to her office, Dr. B spent the first 10 minutes of our appointment talking to our hungry and increasingly crabby Genna. She chatted with her, found out what she liked, there may have been jokes told, I’m not sure. But I knew, I knew as this woman who Knew How To Save Kids With Brain Tumors talked NOT to us but my child, the actual Kid With Brain Tumor, that we were in the right place.

She eventually explained G’s tumor to us, what we would be doing in surgery, what was the “gold standard” of chemo for this type of tumor.. . Genna’s tumor was a bilateral optic-chiasmic-hypothalamic glioma. (I spent the rest of the night figuring out how to spell that frontwards and backwards. Literally, I could spell it backwards by morning. Somehow that gave me a sense of control, being able to spell it backwards). This is just a brain geography term, really, telling us the tumor was wrapped around both optic nerves, through the chiasm where the nerves cross, and into the hypothalamus. Translation: massive amounts of tumor. Pardon my language, it was shitloads of tumor in my child’s head. And none of it was operable. To operate would leave Genna blind or worse.

Yes, worse. That’s code for severely disabled or dead.

G was ravenous by the time we finished this meeting, we were sent off to the Pediatric Intensive Care Unit, the PICU, which apparently is standard procedure for shunt patients. We were terrified. Genna was hungry AND scared.

We tried to get settled in the PICU,G was nearly berserk with hunger. A fellow came in, I don’t know who he was. He told us G couldn’t eat, we needed to go get a CT scan for shunt placement sometime that evening, so she couldn’t eat. I told him she needed to eat, it just wasn’t fair to her since we had promised her that she could eat…and I needed at least one promise we had made her about things to be true...since things were NOT going to be ok...

He told me life wasn’t fair.


It is good that after that night I never saw that doctor again, the doctor who put G’s films, her horrible scan up on a light board and gave a pompous lecture to the students trailing him, a lecture about the train wreck of my child’s scan, right in front of but completely ignoring Genna. (I asked Dr. B the next day if we could take that picture down. She did, immediately). He had one of those med students fill out paperwork with me, and I did fine, I did fine until she asked, “does your child have any health problems?” and all I could choke out was “you mean besides the brain tumor?” and then I got hysterical and Dave had to take over.

That man was the ONLY bad person we ever have met at CHOP. In 9 years, hundreds of people, he is the only one. For years I have prayed that I can forgive him. And then I remember that night, and realize THAT prayer has not been answered. I hope he is not still in pediatric medicine.

Genna was distracted by the nurses who played Candyland with her, giggling and cheating wildly.

I actually had to leave to go throw up. I have never had that happen before, my body just went haywire with the stress of the day--I was so afraid they would think I was really sick and make me leave, so I went and found a bathroom down the hall, outside of the PICU.

At some point that night G finally got her CT scan (unsedated—she could have eaten after all), we got her food, and she played more Candyland with the nurses. At one point I saw the nurses reading her scan report, and I saw their faces fall… since G seemed fine. SHE SEEMED FINE, smiling and playing. . .

The night dragged on, I climbed into the bed with G-- she is a bed hog--and Dave stretched out on that weird PICU chair thing they have. G finally fell asleep, and I finally got up at sat by the window that overlooked the atrium. Through the atrium’s glass roof I watched the medevac copters come and go through the night. All I could do was cry, and ask God why He had abandoned us. Why was He punishing my child because of MY sins? Why?

I have panic attacks now when I see a medevac copter. Still.

Ugh.

The next day, she got wheeled off to surgery, and we finally sent home a message for our prayer group to send out: Genna had a brain tumor. Not malignant, but inoperable. Please pray. I was adamant they say it EXACTLY as I had worded it. That mattered to me. She didn’t have the same thing as Mrs. C, and I knew everyone would go right there…heck, I would.

The anesthesiologist (who looked like Santa Claus; I loved that man, he was so kind to us) told us that G did well, as they rolled her away from us she told them she was a little nervous since it was her first time . . .

That was my G.

By the time we got her back my sisters had arrived. G was inconsolable when she came out of surgery, in so much pain and so sad, just crying and crying. It was an agony on top of agony to see her like that.

After another day in the PICU G was stable, we went and saw the special neuro-ophthalmologist and we got moved to an oncology room. I was just too scared to go home, I think Dr. B had mercy and let us stay one more night. I just couldn’t believe we were in a pediatric oncology ward. 4 days earlier we were just a regular family. How could NF have done this to us?

We were like onco zombies, just shocked beyond all get out.

Eventually (there is just too much to say!), we had an “official” meeting with the oncology people, Dr. B, a social worker, some other folks. Hard questions about the future got asked. We must have looked awful, someone commented on how shocked we must be. We learned more about treatment, we learned that the “gold standard” chemo, carboplatin / vincristine would hopefully stabilize the tumor (or shrink it, my mind added), it seemed to be effective in 7 out of 10 kids, more in NF kids. . .but what if we are in the 30% , I asked. What if we are the 3 out of 10?

“We’ll cross that bridge if we get there,” Dr. B answered.

We got a bag with a little angel, a stuffed animal, a journal, a book about brain tumors (which I read in one sitting. Mistake!) , and a binder for our new onco-life.

And then they sent us home with instructions to return on September 13 to get a port put in Genna’s chest so she could start chemo.

I pushed to wait for that day. She needed to start school on the 10th. She needed to start first grade before starting chemotherapy.

Sentences like that are why I hate NF and brain tumors.

this video has an interview with the head of CHOP neuro-oncology (Dr. Phillips, who we hear is great)   AND G's neurosurgeon, Dr. Storm. And this is current, imagine how young and zen he was 9 years ago, he still looks so young...

The First Worst Day Ever, Part II

May 6

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.

As a side note before this hard post, today Genna had an MRI that was stable. Her protein levels were off, so no chemo today, but we got some hope today.  This is the first time we've gotten to the year mark on chemo without progression (or such toxicity that we had to stop). Hope.

the original MRI scheduled. Yes, I am an onco hoarder. Whatever.

“Genna has massive hydrocephalus, fluid on her brain”, the doctor was explaining to me. “She has brain tumors, large brain tumors that are blocking the flow of cerebral spinal fluid. She needs to get to a hospital NOW.”

Um, say WHAT, Willis?

Tumors? What the…I felt like the floor was literally collapsing under my feet, I had run upstairs, away from my noisy children , to hear the doctor better. He explained G had tumor, lots of tumor. Apparently “lesion” said by eye doctor is euphemism for “crazy gigantic tumor but I can’t bear to tell you so call your doctor”.

“Wait, so is this life threatening?” I finally squeaked out.

“Yes,” he answered. “She needs to get to Columbia Presbyterian tonight.”

I just… I had no words, I just , my brain just shut. Down. Completely. I kept asking the doctor to repeat things, then the nurse, then the doctor again, I had to call New York, I was supposed to go to a hospital in Manhattan? Somewhere in Manhattan? I am phobic about driving on the highway or ordering at the deli counter, Manhattan? G seemed FINE, how could this be? I was trying to write words on little pieces of paper.

I still have that paper somewhere, that shocked scribble of words.

patriotic scraps of paper. I found them in my original onco binder. sigh.

Life threatening?

But optic glioma aren’t life threatening, THAT IS WHAT ALL THE NF LITERATURE SAID.

The NF literature neglected to mention that a big enough tumor pushing back along the optic tract can block the ventricles that drain cerebral spinal fluid and cause life threatening hydrocephalus. Details matter, people.

The next few hours were a disaster of confusion, I know I called Dave, I know my friend Jen brought us a chicken divan (she was one of the few folks who knew about G’s NF, she had offered to bring us dinner on MRI day), I whispered to her “PRAY FOR US. PRAY HARD” as I held the phone to my ear, trying to work out details and doctors.

At certain points I remember just weeping and asking the nurse to please just tell us what to do. Just tell us what to do…

…Because the doc at Columbia Presbyterian didn’t take our insurance. A nurse there suggested we come in through the ER with a “headache”, then they would HAVE to treat us, but she had never met Genna, who had never had a headache. Massive tumor, massive hydrocephalus, NOT one headache or bout of nausea EVER. Not one.

Part of G’s ferocious diva-ness was the compulsion to INSIST UPON TRUTHFULNESS. If we went to an ER in another state saying G had a headache, I KNEW, I knew she’d be yelling, “MOM! I DON’T HAVE A HEADACHE! WHY ARE YOU SAYING I HAVE A HEADACHE? I HAVE NO HEADACHE! MOM! MOMMY! MOM! HEY, MOM! I DON’T HAVE A HEADACHE!”. Like that. And it seemed karmically ill-advised to try and get brain surgery under false pretenses.

By now it was 5 o’clock, offices were closing, I was in a panic, I didn’t know what to do. Finally our docs arranged for us to go to the Children’s Hospital of Philadelphia, they set up an appointment with a neurosurgeon (I have his name and where to park on that scrap of paper), they told us it was probably ok to wait until morning. Maybe. Possibly.

I will say that at 10 pm the neurosurgeon from Columbia Presbyterian called to make sure we were taken care of. I have always appreciated that, it wasn’t his fault that they didn’t take our insurance.

The rest of that evening is a blur. We were supposed to start construction at our house the next day, to convert our garage into a playroom for the kids. We were supposed to be getting ready for a lazy Labor Day weekend.

We were not supposed to be wondering if our daughter would live.

That night, Dave and I just laid in bed and cried. We cried and held each other and grieved and panicked and despaired for our little girl, our diva queen goddess princess Genna.

The next morning we packed some bags (we didn’t know what to expect, how do you pack for “drive to random hospital in another state to see what the hell is going on in your little girl’s brain”?), kissed my 9 year old and my 2 year old goodbye, and drove 81 miles southwest, to the City of Brotherly Love…

The First Worst Day Ever, part I

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 5

This one might be a little long. Ok, it IS long. sigh. And I don't think there will be pictures.

G’s eye check on August 3 was really not particularly dramatic.  She did have some issue seeing some of the letters on the chart, but I was not super concerned. I got glasses in kindergarten, I figured maybe she was nearsighted too, even though Dr. C said that G’s problem didn’t seem correctable. The vision issue was slight (not even 20/50), but Dr. C couldn’t fix it with those little lensy things she had, so she said we probably should get an MRI.

You would think that somewhere some warning bell would have gone off in my head, but she didn’t seem worried. Heck, I didn’t feel all that worried, I knew maybe we’d have some kind of optic issue but I wasn’t that worried.

No, I am not the sharpest crayon in the box all the time.

Dave & I were set to go away for a 10^th anniversary trip to Cape Cod the week after G’s eye check, so I set the MRI up for August 30, after we came back but before school started.  I didn’t want G to miss any of the beginning of first grade. 

Our trip was awesome. Just amazing, we loved everything, we talked about bringing the kids to Cape Cod, we talked about what we wanted for our family in the next 5 years or so, we made plans and dreams and joys together in that magical week.

And God laughed, not the nice kind of laugh.

We came home refreshed and full of renewed energy for parenthood and life and mayhem.  I assembled the school supplies the kids would need, got all the uniforms ready, just prepped for fall.

Monday, August 30, 2004, we brought G for her MRI. We had promised her Burger King after the scan, knowing that she had to fast before sedation.  Once again she fought the meds, but then she slipped off to sleep and the techs wheeled her away. 

Dave and I sat in the waiting room, he went to get us coffee while I tried to block out the daytime tv blaring over my head.  I think Bill Cosby was on some talk show, which was entertaining for a moment.  A dad and son came in to get the son’s leg MRI-d after a sport injury.  I wrote more melodramatic poetry about how scary it was to be back in this spot again, waiting for a scan.

Finally G was done, they gave her back to us, she woke up, and we went off to Burger King.

I think now about those techs, those people who saw the scan, and then gave my 6 year old curly haired diva G back to us. I think of those folks, and I think of  how much that day must have sucked for them.  They knew. We didn't, and they knew what was going to happen to us and they couldn't tell us or do anything...I feel so bad for them.

As I type this, G just came over to give me a “homework stress” hug.  I needed a hug right now, too.

We got the requisite happy meal and then Dave dropped us off at home while he went back to work.  I told G when she was less post-sedation we’d get her the promised Care Bear. Hey, bribery should never be underrated.   The kids were playing and watching PBS or something, I don’t even know, I just know it was warm and sunny and I had the windows open.

The phone rang around 2:30 or so, it was the eye doctor.

Wow, that was so fast! I was so relieved, I thought I’d have to wait a day or two for results.

Foolish, foolish mortal. Oh, stupid girl.

 MEDICAL LIFE LESSON ALERT:  Phone calls an hour after getting home from a medical test of any kind ARE ALWAYS BAD. EPICALLY BAD. SUPER BAD.  AWOOOOOGA! BAD IN BIGGER THAN CAPITAL LETTERS BAD.

The eye doc told me that I needed to call our pediatrician, the scan had showed something, a lesion,  there was new medicine called temodar that you could do at home that could take care of things, this mass, but I should call our pediatrician, I needed to call him right away, he knew I’d be calling (ie so I would be able to break through the Great Wall of China that is the pediatrician’s reception staff) and I needed to call right away.

I was so grateful for the call. “Thank you SO MUCH for calling so fast, I really thought I’d have to wait, “ I told her, so relieved. 

I cannot even fathom what she thought, she just told me no problem, make sure I call the pediatrician RIGHT AWAY.

I called Dave first, told him that there was some kind of lesion but I just had to call our pediatrician and he would give me the fuller report.  Dr. C hadn’t made it seem like a big deal, so I would call and let Dave know what the doc said. Dave was cool with that, so was I.

Deep breath.

So I dialed The Pediatric Center, and SO WEIRD, the nurse answered and got all crazy efficient and put me RIGHT THROUGH to the doctor. I couldn’t believe it! Right through!

Yeah.

MEDICAL LIFE LESSON NUMERO DOS: IF THEY PUT YOU RIGHT THROUGH TO THE HEAD OF THE PEDIATRIC PRACTICE INSTANTLY WITHOUT ANY TIME ON HOLD THAT IS NEVER A GOOD THING. EVER. NOT EVER.

“So Dr. C told me to call you…” I said.

And then my world crumbled.  Like, “things fall apart/the centre cannot hold/mere anarchy is loosed upon the world/the blood-dimmed tide is loosed/and everywhere, the ceremony of innocence is drowned” . . .

And that is not melodrama, thank you, Yeats. That was how it was.  :(


as a side note, tomorrow is once again an MRI day for Genna. A sign of hope, nearly 9 years later we still have these days. But oh they are so hard, and the stakes are so high. Please pray for us.

Everything we Need to Know about Brain Tumors We Somehow Missed in Kindergarten

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 4


Kindergarten was a challenge for Genna. She LOVED going. She loved her uniform, seeing her teacher, coloring pictures, but the academic part was challenging. Genna was impulsive, speedy, emotional, she struggled with fine motor skills in a way that her brother had not. Don’t get me wrong, her brother’s first week of kindergarten was marked by a call from his teacher telling me that “he was quite an entertainer”, which is teacher code for “your child is constantly talking, joking, falling out of his desk, making vulgar noises, and generally distracting everyone around him as much as possible” . But my son loved letters and numbers and had no problem with schoolwork when he wasn’t trying to be a 5 year old Rodney Dangerfield.

Genna struggled with letters and numbers and just kind of a lot of things, enough that her teacher and I agreed I should talk to someone. Our local school district would have nothing to do with us since Genna goes to Catholic school, so we went back to the neurologist who sent us for that first MRI. After hours of expensive testing and a ridiculous waiting room wait (a tiny room full of wailing children), the doc said maybe we should have her tested.

You can read it twice, it still makes no sense. I thought that’s what he was DOING, testing her…

I was young, and kind of uh, ok? and frustrated and unsure of what to do, and I didn’t push the issue, except to run around in circles with our school district some more. Something just wasn’t quite right. . .

Easter, 2004 . I went and dug out pictures, and hey, I used to scrapbook things.
 I wish I still did that, I love how these pages look.
G's face is a little pale, now I see that her coloring just wasn't quite right, but then...yeah. I saw nothing

Genna was frequently tired, she had zero energy for her new dance class, I figured she just didn’t like Irish step dancing as much as twirling in a tutu. She was kind of ferocious diva a lot of the time, but I figured that was just a phase she was going through. The doctor had given us no answers, so we just kept slogging on.

At the very end of her kindergarten year, Genna’s teacher got sick. Mrs. C had all sorts of doctor’s appointments, and near the end of May 2004 we found out, to our unspeakable horror, that she had a malignant brain tumor.

You know, 9 years later this is still really hard to talk about.

Genna didn’t really understand, she just knew Mrs. C was sick, and we tried to stay hopeful, but we knew the stats were grim.

We finished out the school year without Mrs. C, and jumped into summer. G’s annual pediatric check was set, as was my son’s (two summer birthdays means we always do all those appointments in the summer), her eye check was set up for August 3, after our annual trip to Lake George.

I remember nothing else about that summer.I have pictures somewhere. But I remember nothing else.

After August 30, everything else disappeared.

Apparently we went on a steam train ride. Look at how pale G is. How did we not know?
Old pictures bring up hard questions.

tomorrow: D-day, aka The First Worst Day Ever

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.

Beginnings

May 2

So in October of 1994 I married that pamphlet toting guy. And at the end of October 1994 I got pregnant (yes, I know, SUPERFAST), and then I realized, in between constant throwing up for months and months and months, that I was terrified about my child having NF. When clutching ye olde porcelain goddess, inspirational music doesn’t appear when the specter of adversity rears its ugly head.

I was obsessed and terrified.

But in July of 1995 I had a perfectly healthy baby boy. He was gorgeous, big blue eyes and brown hair and juicy legs and arms and cheeks. Challenging as all get out (sleep? I don’t need no stinkin’ sleep!), but totally healthy. He had zero signs of NF.

but many, many signs of cuteness.

Yes, there are things to look for to diagnose NF. For a firm diagnosis patients have to meet at least any 2 of a list of criteria. My children were ALL born with ONE of the criteria met: a parent with NF. Remember in that awful long paragraph from yesterday? That “50% chance of passing it on” thing? Yeeks. Having a parent with NF is one of the criteria. The complete list is: (thanks to www.nfcalifornia.org) :

1. Six or more café-au-lait spots 1.5 cm or larger in post-pubertal individuals, 0.5 cm or larger in pre-pubertal individuals (kind of a light brown birthmark)

2. Two or more neurofibromas of any type or one or more plexiform neurofibroma (nerve tumors)

3. Freckling in the axilla (underarm) or groin

4. Optic glioma (tumor of the optic pathway)

5. Two or more Lisch nodules (benign iris hamartomas)(ie marks on eyeballs, only visible with a special lamp the eye doc has)

6. A distinctive bony lesion: dysplasia of the sphenoid bone or dysplasia or thinning of long bone cortex

7. A first-degree relative with NF1



And by 3 months, 6 months, a year, two years, my son had none of the other 6 criteria. Woo! I started to relax. Well, as much as I have ever relaxed about anything in my entire life.

At a certain point we realized firstborn was starting to act like a Crown Prince of the Universe, and that perhaps a sibling would be a good idea. We both come from large families (I am the oldest of 9, Dave is the oldest of 6…and the only one in his family with NF), so more kids was always part of the plan. It took a bit more trying than our first time around, but in October of 1997 we got pregnant with kid #2.
And this time around, in between constant throwing up, I didn’t much worry about NF. We dodged the bullet with firstborn, so…yeah. Maybe I was just so sick (I ended up hospitalized during this pregnancy when I hit the ol’ 103 lb mark. I am 5’7”. I’m not kidding about constantly throwing up), or just overwhelmed with already having another kid at home and still working part time, but I didn’t much worry.

Genna was born exactly on her due date, July 24, 1998, at 8:30 in the evening. (She still refuses to be late for anything). She was a juiceball of deliciousness, dark brown eyes like Daddy, dark hair like both of us, totally Italian looking, totally a diva pretty much from the moment of birth. We were completely smitten immediately, pretty much exactly as we had been with firstborn, which was awesome (and totally normal, but it’s always a bit of a surprise just HOW MUCH you can love each child as they come, so utterly and completely right away).

Juicy Couture, indeed.  Or, Diva In Repose.

G was healthy and developmentally on track, really nothing was amiss at all.

I had two gorgeous, healthy kids. I almost couldn't believe it, because I'm kind of a dork, these kids were so gorgeous...

see? Gorgeous. G was about 3 months old when these were taken, Andrew was 3 and 3 months.

G has always been an all or nothing kind of gal. All smiles, or All Oh No You Did NOT. But mostly smiles. 

 

And then in January 1999, while changing G’s diaper, I noticed the pale brown marks on her little round self. I guess because the weather had been cold and she was so bundled up, and even in the tub she was a slippery moving mass of juicy baby-ness, I hadn’t noticed before…but there was one mark, ok. Then I found another. And another. Not super dark, just light brown birthmarks, mostly a centimeter or more in size…

Yeah, if you look back at that list, café au lait spots are one of those diagnostic criteria.

My baby had NF.

And I knew it , I knew it instantly and I completely unraveled, it was a Friday afternoon, and I called Dave, and I frantically called my pediatrician’s office (we were already scheduled for G’s 6 month well child visit)—they didn’t freak out. I don’t remember Dave’s reaction at all. How weird is that? But I don’t remember.

I only knew that everything was different. And that nobody was going to know that my child had this disorder unless they had to…I didn’t want her judged, I wanted her to be free and joyful and beautiful in all the ways I had not been. . .

I was devastated.  My hope for G's future was shattered.

 

tomorrow: Secret Life with NF.