Not So Easy

Yesterday Elle went to Maine Med for her round of chemo involving Doxorubicin and Cisplatin. These two chemo drugs are heavy hitters; they can make you feel awful and lay you out. They both present their own dangers if not flushed out of the system; Doxorubicin can affect your heart and Cisplatin can damage your kidneys. Both can cause some nasty vomiting. And the doxorubicin is red...the stuff just looks alien and evil in the IV bag.

So this is not going to be an easy one for Elle. There are some good things, though. First, she has a double line in her chest. That means they can give her the chemo and flush her system with an IV drip at the same time. Usually, they have to interchange between the chemo drug and the IV flush. This way, both happen at once and it keeps the levels of the two drugs from getting too high and it shortens the overall time. Second, she slept through the first part last night, so that will cut down on her overall "feeling bad time." And third, they gave her an anti-nausea medicine that is steroid-based but completely safe (believe me, I asked!) It makes her hungry and a bit moody, but really seems to be controlling her nausea.

So right now she is in her room. I left her with Beth, eating a cup of Jello and watching television. Which is about as good as she can be doing at this point. She'll be there all day and tonight to keep flushing the chemo drugs out of her system, and then she is supposed to come home tomorrow.

Reading what I wrote at the top...these two drugs are rough, but they are also very effective. Doxorubicin has been around almost 30 years and is the genesis for a new class of anthracycline antibiotic-related drugs that may be useful in cancer treatment. Cisplatin has been around almost as long and is a cornerstone drug in treating numerous types of cancer. It absolutely sucks that we have to pump this toxic stuff into Elle...but it gives her the best chance to beat this.

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.

Flushing The Lines

Elle has something in her chest called a central line. It's a semi-permanent fixture (it'll be there until we're done) that allows the doctors to deliver IV drugs (including chemo) and draw blood samples through one of two lines. The base (where it enters the chest) and a majority of the two lines is covered with a waterproof dressing that is changed once a week (more if she takes a bath/shower/goes swimming in a pool). It looks like a clear piece of Saran Wrap.

But these lines need to be cleared, or flushed, every day. This is so blood doesn't clot and block the lines, which would render them useless. So every day each line has to have a sterile saline solution injected into it, followed by an injection of heparin. The heparin is an anti-clotting agent that flushes any particle of blood out of the line but breaks down before it hits Elle's bloodstream, so it's perfectly safe (ie she won't become a bleeder like a member of the Russian aristocracy.)

The visiting nurses, despite their name, don't actually visit every day. Care to guess who has to learn to do this?

Beth and I each took a line today while the nurse watched us. Beth did a great job. I forgot to de-clamp my line and tried for five seconds to push the saline through before I realized what had happened.

It's an intricate process. You have to:

• Wash your hands
  

  
  

• Wipe the cap on the end of the line clean for a minimum of 10 seconds with an alcohol wipe
  

  
  

• Do everything in your power not to let that cap touch anything else that occupies a point in space and time. Because if you do you have to clean it again.
  

  
  

• While holding the cap at its base in one hand, attempt to de-cap a syringe filled with saline solution in your other hand. Oh, and it's threaded, so good luck.
  

  
  

• Once you figure out how de-cap it without dropping the cap on the line, you take the syringe and screw it onto the end of the cap.
  

  
  

• Then you unscrew it and draw some air into the syringe to break the seal and then push a little water through the top to get rid of the air.
  

  
  

• Rescrew the syringe onto the cap and start to push the saline solution into the line.
  

  
  

• Realize that the water isn't going through because you forgot to de-clamp the line.
  

  
  

• Undo the clamp on the line and try to inject the saline solution again.
  

  
  

• Unscrew the syringe from the line because by trying to push the saline through with the clamp on, you create a vacuum that you have to break. And for God's sake, do not let that cap on the end of the line touch anything!
  

  
  

• Screw the saline syringe on for a third time and finally push the solution through the line.
  

  
  

• Look away as your daughter, who drew her own blood the other day, glares at you with thinly-veiled disgust.
  

  
  

• Repeat with the heparin.
  

On the bright side, I only have to do this 200+ more times over the next year. I have to get good at it at some point.

Elle had a good eating day today. Right now the goal is getting calories in her. So while I was able to get her to drink an Ensure shake made with ice cream and milk, she also had soup, vanilla wafers, Goldfish and french fries to go with multiple cups of juice. It's easily the most she has had to eat in two weeks. And she didn't have any nausea today.

She also sat on the couch watching her numerous Barbie DVDs (Barbie and the Magic Pegasus, Barbie as the Princess and the Pauper, Barbie Explains How to Sell and Buy Credit Default Swaps) and coloring with those great Crayola markers that only show up on their special paper. Her diarrhea lessened today and her fluid production was down slightly. So it was yet another good day. And that's all you can ask for right now.