Tired/Elated

I was with Elle yesterday when she had to go to the clinic for her Vincristine push. The real downside to this med is that she gets tired. And sure enough, later in the day she was cranky and sleepy. But that passed (somewhat) and she was able to fall asleep rather sweetly and zonked the whole night.

The really good news is that the initial rounds of chemo are doing their job. Her doctor said that the mass on her lung had "noticeably shrunk" from the chemo we have done so far. That doesn't mean it's almost gone. What it means is that the shrinking of the tumor is visible from just looking at it. I couldn't tell you how much or anything like that. But the doctor said he would measure it and let me know next time.

The other really good news is that there has been no spread of her cancer outside of the initial area. As before, this means the chemo drugs are working and the decision to add the Vincristine/Irinotecan combo to the treatment schedule was the right one. In a very real sense, Elle is breaking new ground here. PPB is so rare that there is no set protocol for its treatment. It's really scary...hell, it's terrifying knowing that your little girl has a form of cancer that doctors still don't know a lot about. And yet it's inspiring as well, knowing that Elle's fight and eventual success could lay the groundwork for another child beating PPB.

In so many, many ways, she is a unique and special girl. And I am just so damn proud and humbled at the same time (if that is possible) that I am lucky enough to be her dad. And the way that Davey has treated his sister and has been there for her...we're twice-blessed.

Changes

We have a new diagnosis.

This isn't entirely unexpected. Both Beth and I knew that the rhabdomyosarcoma diagnosis wasn't 100% sure. But we didn't want to tell anyone that because we figured it would be too much to have everything up in the air for that long. The other reason being that the new diagnosis is almost exactly the same as the original diagnosis. In fact, these two cancers can only be differentiated in their basic genetic code. (Update: After reading this, what I said isn't entirely accurate. The two types do share a common genetic pathway. I don't know if that means they're exactly the same.)

Elle's cancer is officially pleuropulminary blastoma. It occurs in and around the lungs. It's also quite rare; only 10-25 cases per year are diagnosed in the United States.

Luckily for us, our doctor at Dana-Farber is the doctor to see for this kind of cancer. As in, out of a global population of six billion, he is the one you want to see your child when they have PPB.

Better yet, he and our doctor at Maine Med know each other quite well and were already working together on Elle's case before our referral. I can honestly say that between our two doctors, I know with 100% certainty that Elle is getting the best treatment possible.

Now, the good news and bad news. Start with the bad...in treating PPB, the best way to have an optimal result is to remove the lung. And not just the lung but the lining and wall around the lung. So regardless of how well she responds to chemo, the lung is being removed. It's just a matter of when.

It sounds bad, but in reality it isn't horrible. She will be able to live a completely normal life with one lung. Short of being an Olympic marathoner or deep sea diving without a oxygen tank, Elle will be able to do anything. We (and she) will just have to be vigilant in avoiding the flu and pneumonia. And she absolutely will not be able to smoke.

Now the good news. The chemo regimen for PPB is not as extensive and toxic as the one for rhabdo. And instead of a 12-month cycle, it's a nine-month cycle. And her hospital stays will be fewer days at a stretch.

Also, it appears (everyone knock on wood) that Elle is responding very well to her vincristine and irinotecan sessions. Her lung apparently sounds better than it has before. And her fluid output is down even more. There is a good chance (knock wood again) her chest tube could come out tomorrow.

Oddly, I almost feel relieved by this. Now we know for sure what we are up against. And Elle has been kicking ass so far and I have no doubt she'll continue to do it.

We will beat this. The diagnosis just gives us a name to dance on when Elle wins.

Funny story: At Dana-Farber, our doctor's assistant has given up potato chips for Lent. Elle was eating some chips and the doctor suggested that she offer his assistant a chip. As a joke, of course.

Elle offered her the chip. When she turned it down and we all chuckled, the doctor said he would take it.

Elle locked him with a look and extended the chip. As he reached for it, Elle closed her hand around the chip, shook her head, and crushed it in her hand. Then she ate the crumbs.

And the doctor fell in love with her. Just another convert falling to the Power of Elle.

Chemo: Round 2

So Elle got her Vincristine today. According to Beth, she tried to do it herself. While her doctors love and encourage her feisty spirit, they don't encourage it that much.

After that it was a visit to her surgeon who thinks Elle may be able to remove her chest tube on Thursday. That would be a very good thing indeed.

My sister and her daughter Maya were over again today. Elle and Maya had a blast today with painting and watching movies. While that was going on I went back to work for the first time in about two weeks. I had been working from the hospital/house. It's odd going back after all that time. But it was nice too; there's something to be said for the regularity of work and how it occupies the mind.

Tomorrow is another day of work and then we bring Elle to Dana-Farber in Boston for her official second opinion on Wednesday.

Attitude

Elle has it in spades. And it has been making a re-appearance over the past two days.

She has ordered around the nurses (with "please"s and "thank you"s, but they're still orders) and has demanded to be rubbed down with cream on her legs because...well, she's Elle and that's what she wants.

She has also charmed the pants off of everyone who has met her. One of the doctors let Elle play with her wedding ring while she rubbed Elle's back. The X-Ray lab let Elle bring a DVD upstairs because she like it so much. Remember that grouchy banker from It's a Wonderful Life, Mr. Potter? Elle could get a loan from him at 0% interest.

All this is great news because it gets her that much closer to coming home. She is also taking short walks, which is impressive anyway and doubly so when you realize she has a drain tube in her chest. How many of us would want to walk with a tube in us? I'd be moping in bed. She is eating more food (including a McD treat last night) which is a big step as well. And last night she slept without the oxygen thing that goes in your nose for the first time and her O2 levels were great, which means that right lung is getting back to normal.

So it has been a good 24 hours. We find out about her bone scan later today and lay out the course of treatment, which will start as soon as tomorrow.

Many of you have inquired as well about whether we would be taking Elle to Boston. Right now both Beth and I are thrilled with the doctors we have here in Portland. They belong to a regional pediatric oncological group, which means they are regularly discussing issues and treatments with doctors from Dana-Farber/Children's Hospital and Mass General in Boston. Our doctors here have also assured us that if they think at any time Elle would be better served in Boston, then we're going to Boston. That said, I have contacted Children's in Boston through a referral from a family friend. They will be getting Elle's information to review and we'll have an appointment down there in the next month or two for a second opinion. It should reassure all of you to know that they are very familiar with Elle's doctor in Portland and speak very highly of him. Trust me when I tell you this; Elle is in great hands right now.