One Year and a Frightful Day to Scan

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 12

We hit the one year mark.

Part of me couldn’t believe it had been a year. I struggled to wrap my brain around how our life had changed, how so many people had been SO kind to us…we had never been on the receiving end of that kind of support, we were completely overwhelmed by the support and kindness of so many people. I wrote a long (surprise!) post reflecting on how many people had helped us, how I had suddenly learned to be assertive to protect my G…and how I would NEVER forget the Just In Case bag again.

The love and kindness we experienced are two of the beautiful things amidst the wasteland of our bt/nf journey, and continue to be so to this day. And I still bring a bag every time we go to CHOP.

G and her goddess doc (and me), September 2005.
 Her doc and I matched that day, which was kind of funny. We still do, rather often. Strange.

Anyway, summer wound down, and over Labor Day we decided to bring G to a priest who had a healing ministry.

I had refused EVERY such thing like this before this date. I knew G believed with all her heart that God would make her better. I figured if He wanted to we didn’t need to visit a limb of a medieval saint or something. It made me angry, it just seemed presumptuous or something; I love a lot of devotional stuff, but this made me mad.

But this priest from New Zealand was coming to our area, and I agreed that we could go see him. Following so close on the heels of our one year smite-aversary, this was a tough call, but G was totally into it (she LOVED praying stuff), so we went.

I still had trouble with God. But Fr. Rea was pretty awesome, so completely humble and normal. He simply prayed with us. A simple, simple, quiet set of words that only we could hear, asking God for healing for G. And while G did not get healed there (understatement of century), MY heart got healed that night. I know that may sound crazy to people, but I stand by it. After that night, for the first time in a year, I knew it wasn’t my fault that G was sick. Or maybe, more accurately, I finally believed that G’s illness was not God’s punishment on me for failing and failing. I knew it wasn’t my fault. The freedom in that moment, a moment which grew in the weeks following Fr. Rea’s visit…it’s hard to describe unless you have felt crushed by cosmic smite.

And G started second grade, replete with new challenges (sigh. I have some blazingly ranting posts about the trials of second grade social life, challenges with teachers, and the perils of the bus --brain tumor and nf school issues can be pretty epic, regardless of tumor type, from what I’ve observed, G isn’t alone in this), and we cruised on. She had been stable since that bad scan in November of 2004, if she was still stable at our Halloween scan, we would stop chemo.

We were all just so tired at this point. Some rounds G did better than others, sometimes she decided She Was Done With Chemo (problematic when you um, aren’t done), we were just tired.

G was growing alarmingly fast, some of that seemed to be tumor related, but her counts were good and she loved telling everyone about her upcoming Make A Wish trip…which we finally got on the calendar for March of 2006. We figured by then G would be feeling better, if in fact we ended chemo in October.

the last temozolomide chart

Sunday, October 16, 2005 9:21 PM CDT

Hi, all.

Round 12 is done.

I never thought I’d say that. We made it through 12 rounds of temozolomide.

Genna was a superstar this round. She gulped the pills so fast some nights I had to do the “God bless Justis, Dakota, James, Ryan, Kyle, Jed, Jordan…” for her (girls were on the other one—so Hadley and Hailey and Caden got prayers, too!). It’s our cutest chart yet (a pumpkin patch with trick-or-treaters), and Genna had no real side effects except CRABBINESS and tiredness— …

So this round is done. Who knows what the future will bring?

Yesterday I got to Andrew’s baseball game, and I could see Dave’s face…the “Oh, no, I’m trying to explain the Story of Genna” face. Apparently Genna was telling all the moms at practice about her Make a Wish trip…and someone asked Dave. Genna had said "Make a Wish came to my house, but I don't know what for." Funny, right? For as talkative as I am online, I don’t chat about this stuff to random everybody. Dave was very relieved when I showed up…and the moms were so sweet about everything. They are really lovely people. The one poor mom (the coach’s wife) was shaking after I gave her details…Genna plays with her little girls each game, our boys were on the same team when I was pregnant with Rosie...I felt bad. I’m used to saying the clinical stuff…but I’m getting weepy now, I don’t know why…

I think my pre-MRI psychosis is beginning…



As a note for brain tumor awareness, all but one of the children in G’s chemo list of prayers have passed away. All. But. One.

There are no words for that.

I felt bad about this post a day later (long school vent included in it), so the next day I related this story…which kind of captures G’s way of the world:

Genna had "writing center" at school (an activity center in the classroom) so she made cards for all of us...well, wrote notes on donated cards. So I got a "thinking of you" card, Andrew & Rosie got festive pictures/blank inside cards...and she wrote Dave a sympathy card. "Earth has no sorrow heaven can not heal" on the outside, "thinking of you at this difficult time" on the inside...and a note from G about how she's sorry work is so hard for Daddy right now. We pretty much fell apart laughing. You know Daddy's job schedule is bad when the 7 year old happily chooses a sympathy card...

And then, on Halloween, we scanned. The day before the scan we brought the kids to the Phillies/CHOP Halloween party, they had a great time meeting the Philly Phanatic and playing silly games, then we sent Andrew home and did scan day on Monday.

Life Lesson: Don’t Scan on Halloween. You have to know that's not going to end well.

Monday, October 31, 2005 8:20 PM CST

Hi, all.

No miracle for us today.

For the record, the tumors are stable. Genna knows the tumors are stable. She is satisfied with this. And growth hormone levels are ok…on the high side of normal, but still normal. She’s not off the charts or anything, so apparently I’m supposed to stop worrying. Genna knows her tumors are stable. This is what she and Andrew know, please remember this if you continue reading.

But…

Don’t you love the “but”? It’s like the scary music in a movie, just before the clueless coed walks through the door of doom. This is sort of the abandon all hope ye who read this warning…well, not all hope, that’s me being melodramatic, but let’s just say when your neuro-oncologist says after talking to the head radiologist that there are some areas of concern, this isn’t reason to sing happy songs.

Simply, some “flecks” show up on the scan that have not appeared before. Is this because the post-shunt-revision ventricles are so much smaller now we can see these previously obscured enhanced areas? Perhaps. Is it a weird NF enhancement thing? Maybe. Is it an area of new (inoperable) growth within the brainstem? Well…

It gave Dr. B pause. Enough said.

So the tumor board will confer about what this may/could mean on Wednesday, and Dr. B will call me. …

Not doing so hot right now, so please forgive me if I hide a bit. I feel a little bit sucker punched…but I knew I wasn’t worried enough this time. I SWEAR, results almost directly conversely correlate to my level of worry.

I did mention to Dr. B that "benign brain tumor" is a complete misnomer. There is NOTHING benign about this except the rate of growth. Which is a big deal, but benign doesn't mean no problemo. Benign doesn't mean we can ignore it. Benign stinks too. Dr. B agreed, kind of sadly.

When Dr. B called, we learned that yes, those spots were new tumor in Genna’s brainstem. And just like that, we were back at square one again.

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.

Our Perpetual May

May is Neurofibromatosis Month.

May is also Brain Tumor awareness month.

I suppose May is an official month for lots of other things, too. It’s the month I was born, it’s the month of first communions and graduations and dance recitals and the official insanity of things running down before the respite of summer.

But in our house, the dual NF/Brain Tumor awarenesses become all –encompassing in May.

Spreading awareness for us means helping people KNOW about NF & brain tumors, begin to glimpse the reality of what LIVING with these two things can mean, and then hopefully help us push forward research for a cure.

Thanks to the officialness of the Children’s Tumor Foundation website, www.ctf.org , I can tell you through the magic of paraphrasing that Neurofibromatosis (NF) is a genetic neurological disorder which affects more than 2 million people worldwide. It can be classified into 3 distinct types, NF1, NF2 and schwannomatosis. They are caused by different genes on different chromosomes. NF1 is the most common neurological disorder caused by a single gene; occuring in one in every 3,000 children born. NF can be inherited from a parent who has NF or may be the result of a new or "spontaneous mutation". Parents with NF have a 50% chance of passing the disorder on to their children. Children who inherit NF from their parents get the same TYPE of NF, but the severity of the disorder can vary wildly within a family. NF doesn’t discriminate based on gender, race, geography, or anything. NF is totally egalitarian about who it smites. CTF’s website asserts that “Although most cases of NF1 are mild to moderate, NF1 can lead to disfigurement; blindness; skeletal abnormalities; dermal, brain, and spinal tumors; loss of limbs; malignancies; and learning disabilities. NF1 also has a connection to developmental problems, especially learning disabilities, which are five times more common in the NF1 population than in the general population.”

If you made it through that entire paragraph without breaking out in hives, CONGRATS.

How did I know this BEFORE www.ctf.org even existed?

Well, in 1993, at the end of a very nice date with my boyfriend, Dave, he stood at the end of my walkway and kind of cleared his throat. We were pretty serious at that point, and thoughts of the future had crossed both our minds, I guessed. He pulled a pamphlet out of his pocket, and said, “um, I have this thing, it’s called NF…”

Well, then. Some gals get a good-night kiss to end a date, I got a medical pamphlet.

Ok, that’s not fully accurate, I am sure there was a good-night kiss, too. We were young then, you know. Ahem.

I read the pamphlet, and felt concerned and a bit confused, but I was in love, and sooooo young, and I knew that if I ever had a child who was blind because of NF (which the pamphlet told me could happen, even though Dave was fine, he just had some bumps on his skin, but whatever,), I would go all Annie Sullivan and teach the child Braille and we would Triumph Over Adversity.

Yes. I always choose melodrama. I could almost hear the inspirational music. But Dave was perfectly healthy, so the inspirational music and corresponding adversity were mostly theoretical.

Brain tumors, well, I was aware of those too, one of my classmates lost her mom to a brain tumor when we were in eighth grade, I only remember her mom near the end, on steroids and chemo and unable to really talk to us much when we brought a meal or something to their house…and I remember the day things got really bad and my classmate got called out of class, and we all knew, we just knew…

Pediatric brain tumors I knew nothing about. Low grade brain tumors, even less, although I knew my sister’s allergy doctor had had a brain tumor for years and years. . . I’m guessing now that that was a low grade tumor.

He still died.

There are many, many different types of pediatric brain tumors, in different parts of the brain, comprised of different types of cells. Some tumors are highly malignant and deadly, some are malignant but potentially beatable. Some tumors are slow growing, so called “benign” tumors because they tend not to metastasize to other parts of the body. The treatments for brain tumors include surgery, chemotherapy, and radiation, depending on the type of tumor, the location of tumor, and the age of the child. Scientists keep learning more and more about hidden variations within even pathologically similar types of tumors, variations that hopefully can be targeted in developing therapies.

Brain tumors and the treatments to cure them (oh, double edged sword!) can cause motor, speech, and cognitive difficulties, vision and hearing impairments, paralysis, and death. Brain tumors are horrible, dastardly beasts, low grade or high grade, brain tumors are horrible, dastardly beasts. And while many brain tumors are random acts of cellular yikes, brought on by no act of the afflicted, some brain tumors are caused by underlying conditions like tuberous sclerosis or… wait for it… Neurofibromatosis.

The brain tumors caused by NF tend to be low grade brain tumors—such as optic glioma, those tumors that can blind a child--which again simply means they grow slowly.

According to the Children’s Brain Tumor Foundation, www.cbtf.org (yes, the two addresses are weirdly close, I know) , 11 families each day find out their child has a brain or spinal cord tumor.

On two separate days, with two of my children, my family was one of those 11.

Twice.

Because of NF.

So May means a lot to our family, not that we are unaware the other 11 months of the year (I wish!), but in this month our NF and BT awareness reach epic overloading proportions. And this year, in an attempt to clear my own mind or make sense of the nonsensical or something, I am going to try and tell some version of our story, our family journey through NF and brain tumors, our never-ending May.

I don’t know, honestly, if I can manage it. We hit the 9 year mark this coming August, so I have a LOT of years to cover in 31 days, and I am committed to NOT writing too much more than 800 words or so each day. (stop laughing, really, I’m going to try. I know, I’m at 1300 on this one already, FAIL). MRI day is in less than a week, so…yeah. I don’t know. There is so much, so much to this journey. I don’t even know if it’s helpful for people to know. . .

But NF is so common. And treatments for low grade brain tumors (including the kinds caused by NF) haven’t much changed in decades.

DECADES.

So for May, I hope to spread a little NF 1 awareness (the variant our family has, the more common variant), a little low grade brain tumor awareness, and maybe a little hope. Because even though much of our story is really hard, really, really hard. . . we do have hope.

May is NF awareness month—and brain tumor awareness month. And I know that low grade tumors are only a subset of the pediatric brain tumor world, which is a subset of the larger brain tumor community. So I apologize for the limited scope of what I can say. For a long time I wasn’t sure we even really should have a voice about brain tumors, because my kid only had low grade tumors. That almost wasn’t sick, right?

Sigh. I don’t think that anymore.

I hope this story serves some purpose. I hope the struggles we've had can help other people who are struggling, too. I hope people understand more about these dual disasters by the end of May. And I hope that people feel some hope at the end of it all.



*****************************

For a really amazing and heartwrenching account of a family’s journey through a high grade brain tumor diagnosis, please, please read Donna’s Story, over on the Mary Tyler Mom blog, written for September, Pediatric Cancer Awareness Month. Donna's Cancer Story

Don’t read it all in one sitting. Don’t . Really. Just don’t. But do read the whole thing. It’s so important that Donna’s story be heard. And thanks to Mary Tyler Mom for inspiring this attempt at telling our story. You don’t even know me, but you are one of my heroes.