Wow. I just went back and read the last post to see where we left things. So much has happened since then. So beware, this might be a long post. For the most part, it’s a lot of good news (ugh, hope I didn’t just jinx us).

For the new friends in our lives, we use this blog to keep everyone updated on Scarlett and her challenges with NF. When someone says “how’s Scarlett?”, I’m always so genuinely touched that they care to ask, and then I think “where do I start?” So, the blog is where I get it all out and try to keep it all straight. I’m really not a writer so bear with me as I try to capture the past few months.

The big goal (i.e. my crusade) in 2017 has been getting access to the new drug that’s having great success on plexiform neurofibromas (i.e Scarlett’s tumors- we call them “plexis”). You may recall from earlier posts that Scarlett’s plexis can’t be completely removed. Surgery can remove parts of them (like the surgeries on her airway) but they wrap around so may critical nerves, vessels, and structures that total removal would be impossible. So, her surgeons take pieces out here and there when the plexis impact her breathing or sight (more on that below). The great hope for NF patients is that we find a drug that shrinks the tumors by operating on the protein dysfunctions that lead to the tumors. I’m hoping this new drug is that drug.

2017 has been a roller coaster. YUGE understatement right? 😉 We finally got the NIH team to look at her case (yay!) and then they closed the study (boo). They sent us the paperwork for compassionate care use (double yay!) and then her doc said she couldn’t prescribe it (ugh). Luckily, an amazing friend (thank you Kerrie!) found another trial offered by the drug maker and we started down that road. The trick was that it had to be championed by the physician who’s considered Scarlett’s “care coordinator”. Well, it turned out that even though we see countless docs at Seattle Childrens Hosp., no one was actually sure who her main care coordinator was. So, I’ve spent the last 4 months getting that sorted out. I won’t get into the insurance battle I’ve had except to say that it was beyond frustrating. Onward.

Last week we saw a total of seven different doctors at Childrens. I reiterated to every one of them that we were pursuing the new drug and needed them to determine who’s in charge of her care so we can get moving on this. Finally, this afternoon at my office, I received a call from the Oncology department to say that they’ve identified a doctor to advocate for Scarlett regarding the drug. They are seeing us in 2 days! I was stunned because it usually takes 2-3 months to get in to see her docs. I want to exhale so badly, but think I’ll hold my breath until we get through that visit.

Other good news:

Scarlett has significant hearing loss on her right side (that is NOT the good news 😉). The plexi crushes her ear canal so sound is restricted from getting to the inner ear. For years I’ve wished there was some sort of stent to hold the ear canal open. Well, guess what? There is now such a device and it comes in BLUE GLITTER! (Scarlett said “make sure to tell them about the blue glitter!”) They made a mold of her ear and let her choose the color. We should have it within 2 weeks. I’m sure she’ll put pics of it on her Facebook. And yes, I’m excited about the glitter, but I’m overjoyed that she may get her hearing back.

So that covers hearing, let’s move on to vision. (Scarlett just said “division? Yuck!” Haha. Did I mention the hearing? 😉 Anyhow, her docs have never wanted to operate on the plexi in Scarlett’s eyelid because it didn’t really impact her vision and surgery was risky. Well, it’s grown so much that it’s not only blocking part of her sight but also pressing on her eyeball and changing the shape of it. Fortunately, Childrens now has a surgeon who came here from the East coast, where he did several plexi eyelid reduction surgeries. He thinks he can definitely take out a significant piece of the plexi and improve her vision in that eye. (I mentioned this a bit in the last post, about Scarlett wanting to keep her “special eye”). That surgery will be in early February.

I think that covers most of it for now. I’m a Happy Shan because we have positive medical news.. and because this is the first blog that Scarlett has helped me write. I’ve always marveled at her, but am particularly struck by how much she’s grown up lately. She’s not a little kid anymore. When we see her doctors, she does all the talking and is such an amazing advocate for herself. And she never loses her sense of humor. Ever. She amazes me every day.

If you’ve read this far, thank you. Thank you for caring about my family, and checking in with us, and keeping me laughing when I’m barely keeping it together. I’d be lost without the laughter.

So much love,

Shan