Our First Month
Today is the one month anniversary of receiving our PPB diagnosis. It has been quite a month. We’ve learned a ton about the disease we are fighting and while it has been a grind emotionally and physically we are increasingly optimistic about our chances. We’ve gotten through many milestones and as I like to say, we haven’t received any bad news since the diagnosis. He seems to be handling the chemo regime fairly well (other than being a little fussy and a bit clingy to mom and dad), he has good energy levels, and very little nausea. His eating patterns have shifted but his intake (and outtake) are all relatively fine (he has stopped liking ketchup - yeah!). Preliminary testing shows that the chemo is reducing the tumor size a bit - his breathing is much better and an X-ray has shown that his trachea has straightened (the large tumor had pushed it and the heart to the left). And the genetic testing we did to determine if this was caused by a mutation in the Dicer1 gene came back negative. This is significant as a Dicer1 mutation is associated with a whole list of other cancers in adolescence and beyond, and 70-80% of PPBs are thought to be caused by this mutation. The genetic test is not fully conclusive, Crash may be too young for it to be showing up in his blood and not having the mutation leads to a lot of other unknowns as fewer than 200 kids have been documented as PPB positive, Dicer1 mutation negative but we, and the medical team, are taking it as a victory.
The chemo protocol is fairly severe (apparently they can give kids higher doses than adults). He will likely not need to worry about teen pregnancy and we will be depleting some of the lifetime limits on some of the heavier drugs but the easy consensus is to deal with this critical issue now and hope science/technology advance to such a place that other options will be available to us later.
Take time to smell the flowers.
We are on a four drug, three day inpatient regime every third week (currently on the weekend) followed by single dose treatments outpatient every Friday in between. The inpatient treatment will last for four cycles and the Friday outpatient will continue for about a year - presuming we don’t need to make adjustments. We do all of this at Yale Children’s Hospital just under an hour away from our home. Ami and I generally sleep at the hospital but a friend has graciously given us her apartment for the summer, which is a few blocks away. This is incredibly helpful as we can stage 4 month old “Budda” Jack (he’s currently 21 lbs with two teeth) there with friends/family or our fabulous nanny Diana - or just use as an escape pod when the hospital becomes too much.
At the end of this chemo cycle we will do another CT scan to see where we are at. These tumors, because they are so aggressive, can also be very responsive to chemo treatments. And the current protocol has shown to be very effective, though admittedly against a small sample size.
There are several things that keep Ami and I so strong (and we’ve come a long way on that front). The first is all of the support that we have gotten. The thing that has been truly humbling is the breadth and genuineness of it. From all walks of our life we’ve received such an outpouring of support that it really makes us both just tear up. So thank you all. Everyone deals with these things in different ways but we’ve felt nothing but supported - and perfectly appropriately so - by everyone. Neither Ami nor I have had much experience in these sorts of things but y’all nailed it and we cannot express our gratitude enough. And for those of you that participated (or are participating) in the “food train” you have really helped to lighten our considerable load. So again, thank you.
The other thing has been how Crash has responded to it all, including the chemo. His manners have returned and he continues to charm the hospital staff, which is ADORABLE. He also gets the drill, and happily participates in all of the vitals testing. In fact, he will perform all of the tests on anyone he can with his doctor kit, which now includes a real stethoscope. His energy level is great and other than being a bit whiney (oh, he is 2 1⁄4) and a bit demanding of snuggles, he’s been very good. He loves the trampoline, the swingset, and fishing with daddy. And while he can’t be fully submerged under water, he loves to wade in the pool and take showers with us now that we have finger paints in the shower (though we have to plastic wrap his “tubies”). He has also embraced being bald, which he now wears with pride - even when other kids ask why he has no hair. (I have a video of him vogueing in front of a mirror but I am forbidden to post given his state of undress - but I’m happy to share upon request). And this is all happening simultaneous with his vocabulary kicking into high gear, which is a total trip for us first time parents in and of itself. There are occasional washes of emotion when he does something so cute that it occasions the thinking of the unthinkable, but there is very little in our daily lives and our experience of him that causes sadness - so that is unbelievably encouraging.
