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.