The Month of Darkness

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 15

Brain tumors are horrible dastardly beasts.

NF can cause brain tumors. So in our world, NF is also a horrible dastardly beast.

And February 2006 was one of the lowest, darkest portions of our journey.

G’s scan looked horrible. There was this weird area that wrapped around the ventricle…

We have never really spoken of this to G. Now, 8 years later, I think I can speak to it a little, but for many years only a few people knew the truth about that month. But now, knowing that my G is still here, still living and laughing and loving, I can name this truth and let hope come through the darkness.

Without being totally specific, our doctors thought that Genna’s tumor had undergone malignant transformation, that is, it had turned into a fast growing, aggressive, incurable cancer.

We were given a timetable of life that did not get us to 5th grade. 4th grade was questionable.

TPCV was done. We needed to decide what we wanted to do. I just cried and cried and cried, I could taste the pain, the pain of my child’s vanished future, the hole in my family.

Typing this, even as one of our friends is facing this moment, is excruciating.

The few close friends who knew what was going on rallied and gifted us a camcorder to make memories and a laptop for those CHOP stays. We were blown away by this generosity, and by the hats pouring in, and by the people helping us with a concert we had planned to have to raise money for the Children’s Tumor Foundation, an NF organization ( www.ctf.org ) , our first Concert for a Cure. I couldn’t even see straight most days, the fear and grief were paralyzing.

I think I emailed our doctor 57,000 times with frantic despairing questions, and she answered every e-mail.  She stayed by us in that awful time, not just AT CHOP, but through e-mail, as a lifeline.
In the middle of this month we went to see David Bailey in concert; David Bailey was (at that time) a 10+ year glioblastoma survivor who sang of hope. That evening in PA, through snow and dark, was such a lifeline for us. He told us every day we woke up meant God still had something for us to do. This hit me really profoundly. We still had a mission, we still had to hope…even our doctor told us to always hope.

We were not done with treatment, but if the CHOP tumor board’s thoughts were correct, any treatment would simply prolong things. While talking to the docs about our choices, watching G play in the playroom, even Dr. B couldn’t believe we were having this horrible conversation. G just looked like a normal kid (and then she & Rosie started licking Pringles crumbs off the playroom table...ew ew ew! Normal kid ew! )

We had the option of just chugging through random treatments, or having a biopsy to know for sure what we were dealing with. After a lot of soul searching, we opted for biopsy. And of course G, being a smart kid, wondering why I had been talking to Dr. Storm in the playroom, asked if she needed an operation…at that point I wasn’t sure, which is what I told her. We really try to always be honest with G, let her lead what she wants to know, but within that we are honest. Always.

She didn’t need to know that timetable, we didn’t know for sure yet…but if we only had a year or two, it was going to be one hell of a year or two. We needed to know.

So surgery was scheduled for February 21, 2006, 3 days after that Concert.

We told no one except immediate family.

The concert was emotional and amazing, a beautiful evening put together by our friends and family.

And then I had to tell Genna that yes, she WAS having brain surgery. We cried together on her bed, just cried and hugged. I told her we needed to know what kind of medicine would be best, so we had to do an operation.

I know this is choppy. I am sorry. It’s like pulling off steri-strips, typing this one.

I had requested the Santa Claus anesthesiologist again, he was such a comfort to us during surgery, I cried at him, and he didn’t run away, he comforted me. I have never forgotten him. His name is actually Dr. Schwarz (sp?). He is awesome. And after the surgery, when I was in despair and counting the balloons stuck on the CHOP atrium ceiling, Dr. Storm (the neurosurgeon) said to me, “You know, this could just be an NF thing”. A glimmer of hope. He was so zen, still so zen. He answered all my frantic pre-surgery e-mails too...

We were just relieved that she survived the surgery (those are some scary consent forms to sign. terrifying.), this was our CB entry the day after surgery:

February 22, 2006

...I am moved to tears by all the prayers & support, thank you all--if only I could tell you how you carry us...

Genna came through the surgery fine...we had a 6 hour wait (our specialty), then an excruciating wait after 3 pm when they wheeled her so quickly away...I didn't even get to say I love you...but Dr. Schwarz is a gem of a human being (the anesthesiologist)--he kept giving us updates, &; Dr. Storm is meticulous...G came out ok, around 7.

When asked how he thought the incision looked (before we saw G) Dr. Storm said, "Well, I always think they look good!" I guess CONTEXT is everything, but it did make me laugh.

Spent sleepless night in PICU (well, G slept mostly)...

incision is ghastly. from the front of her head (by the part)a long curving line down in front of her right ear.  (I have no pictures. I probably should, but I don't).

Only JUST NOW did she admit to major pain, take some meds. She is very, very quiet...which is, I suppose, an indication of how she really feels (like her mom in that regard).

And then, we just had to wait, and see what our fate would be.

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Today, I ask you to please, please pray for our friends, the Dickey family and beautiful Caitlin.  I am so sad and angry that they are not getting the reprieve we did in 2006. I am heartbroken and angry about what brain tumors are doing to Caitlin and her whole family.  Please pray for strength and comfort and peace for them in this difficult time ahead.  Thank you.

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...