(A reminder; I write this “conversationally” so forgive me for grammar, spelling, etc)

Wow. It’s been a year. Sunday marks the first anniversary of Scarlett starting treatment with Mekinist (the oral chemotherapy drug she takes; see earlier posts for that story). You may recall that we fought for almost a year before that to get her access to the med. The drug had been used for melanoma and lung cancer, and recently proven effective at reduction of NF related tumors. When we finally found a doctor who would prescribe it, we were thrilled. I’m still trying to link the video of her announcing that we got the drug; it’s one of my favorites.

Post eyelid surgery

It’s been quite a year. Anyone who hasn’t seen Scarlett in awhile is surprised at the difference. The plexiform neurofibroma (aka “plexi) on the right side of her face is significantly reduced; you can really notice the change around her neck. I’m most amazed at the clarity of her speech; she has a plexi that had enlarged her tongue and pushed it over to one side. The speech therapists at Children’s remarked that it was pretty amazing she spoke as well as she did, given the tongue issue. Well, clearly the meds have reduced that plexi as well. She left me a voice message a few days ago and it really hit me just how clearly she is speaking. She was also able to have reduction surgery on the plexi in her eyelid and that’s been amazing. It still startles me sometimes when she looks up at me and I can see her right eyelid so “open”. You may recall from prior posts that when you cut into plexiforms, they tend to come back pretty quickly (at one point we were having surgery on her airway plexis every spring). Our hope is that the Mekinist slows or delays the post-surgical growth so she doesn’t need surgery again so soon.

As always, Scarlett is a trooper. She goes in for blood work every 2 months (she pointed out that it’s not nearly as bad as the weekly bloodwork for the first trial drug we tried). She also has EKGs done bimonthly and MRIs every 4 months. Her next MRI will be in January and I’m hoping we see significant reduction of the plexi that’s constricting one of the arteries to her brain. We’ve been working with the oncology team at Children’s to monitor the drug and of course, they’ve been phenomenal.

Scar is tolerating the drug remarkably well, but it hasn’t been easy. When she first started, she had bad skin reactions and painful rashes. Her doc was able to adjust the dosage and the issue resolved. She’s been lucky and has mostly avoided nausea but she’s had to be very disciplined about when she can take it (only certain times of day and only with a specific eating schedule). It’s been a lot to handle, but she always does it with a sense of humor. She also really seems to understand how fortunate she is to have this drug when thousands of kids with NF do not yet have access to it.

So yup, she’s a trooper. We have a tradition that whenever she has something “big” done (surgery, MRI, etc) at Children’s she gets to pick something from the gift shop. When she was prescribed the Mekinist last November, the doctor said that if she tolerated it, she could stay on it for at least a year. Scar looked right at me, with a knowing smile, then half jokingly said “if I can do this for a year, there better be something huge in the gift shop when I’m done”. And I ruefully thought to myself “there’s nothing in the gift shop that could ever reward the strength it’s going to take for a year of chemo”. (Wow, typing that just made me cry. I’m so used to this all being our “norm”, but sometimes when I look back at things, I think “jeez, that was really tough at the time”.)

Okay, back to celebrating a year. When Scarlett started the meds, I had toyed with the idea of taking a trip to celebrate making it to a year. If you have followed Scarlett since she was little, you may remember the book I made for her to explain the first drug trial when she was 6. (The full book is here). There’s a part in the book when I tell her we have to fly to Indianapolis and she says “if I am good on the plane, can I go to Paris?” and I said “yes honey, one day we will go to Paris”.  So, today we leave for London and next week we’ll take the train to Paris for a couple of days. We’re beyond excited and I’ve never thought anything was as well deserved as this trip. Her cousins, Kel, and Digda are coming too and it’ll a week long celebration.

One last thing before I sign off. Because we’ve been preparing for the trip, the one year anniversary of having the drug has really been on my mind. I will take some credit for researching the drug studies and fighting tooth and nail for access (it’s what moms do, right?) Yet there’s always been a part of me that felt painfully guilty about having access to it, knowing how many kids are living with NF, often with life threatening complications, and don’t have it. But the world can work in marvelous ways and last week I received a pretty extraordinary phone call. It was Scarlett’s surgeon, who’s been with us since her first surgery at 3, and he wanted me to know that they are now able to offer it to all NF patients at Children’s as a treatment option. I’ll tell you this… all the trips in the world wouldn’t mean as much to me as that phone call. Life is good.

So much love,
Shannon

PS – if you were a Facebook friend of Scarlett’s and now can’t see her page, don’t worry. Facebook must think she’s a bot or something because they keep disabling her page. So, we’re using this page for updates again 🙂

Follow up post-trip:

When we went to London, the kids’ top attraction was the Harry Potter studios. I thought this was pretty cute.