Just a quick post to let you know we have the all clear and Scarlett can head home this afternoon. The chest tube is out, and although there’s still some pain from the surgery, she’s feeling (almost) great.

Thanks so much for the many notes and messages. I’ve shared them all with her. We’re sorry we haven’t had a chance to respond to all of them, but we’re so grateful for your kind words and good wishes.

Much love,

Team Scarlett

Whew. What a day. Scarlett is out of surgery and resting. Unfortunately she is in a lot of pain. She’s on heavy pain meds but is awake. The surgeons said surgery went well and they’re hopeful the lung will stay open. The removal of the top part of the lung went fairly quickly, but they decided to abrade the outside of the lung to cause inflammation, which should help it adhere to the chest wall. The surgeon warned us in advance that it would be severely painful for her so we’re all focused on staying on top of her pain meds. They’re going to keep her several more days for observation so the soonest we would be home (best case scenario) is Tuesday.
I need to get some sleep so this will be a short post but I did want to share a happy Scarlett story. When we checked into the hospital, her dad brought her a book with a bunch of stickers in the front. Every single person who has worked with her has picked a sticker (she insists on it). It’s pretty cute to see surgeons and nurses all walking around with unicorns and ice cream cones stuck on their face shields. Anyhow, when she was finally waking from surgery, a pair of new nurses rolled her into her room. I could tell she was trying to say something but her voice was so hoarse it was hard to hear. I leaned over closer and she croaked “get.. the.. sticker.. book”. Heavily sedated or not, she made sure those two nurses were getting their stickers! 😂.

Short version: Scarlett is in the hospital because one of her lungs collapsed this past Sunday. Surgery will be Friday, and we’ll be at Children’s Hospital for awhile longer.

Long version with Shan’s rambling commentary (forgive the spelling, we’re writing this on an iPhone): You know how our family always says “if you don’t laugh, you’ll cry” about the hard stuff? Well, we’re trying to laugh, but it’s getting harder. Here’s the latest: We’ve been at Seattle Children’s hospital since Sunday (today is Thursday). On Sunday morning, Scarlett woke up with chest pain and said it was difficult to breathe. She was also extremely lethargic. Of course, I immediately worried about Covid so we got her to Urgent Care at the Seattle Childrens’ satellite clinic in Everett. If you haven’t been to a clinic or hospital lately, it’s quite an experience. Covid has changed everything; docs and nurses are wearing special sealed helmets with their own air systems and everyone is constantly changing in and out of PPE. They took a Covid test (Scarlett calls it the “brain jab” swab version) and the doctor up there suggested they take an X-ray to make sure it wasn’t pneumonia. They’re seeing a spike in teenagers with severe pneumonia (the teens aren’t testing positive for Covid but the timing is pretty unusual).

They take the X-ray and a few minutes later the doc walks in, races across the room towards me, and very dramatically says “we need to talk!” I’m not used to docs being so animated so I actually thought maybe she was kidding (she wasn’t). The last urgent care doc we saw, who was unfamiliar with Scar’s unique anatomy, saw an X-ray and flipped out about her heart being on the wrong side and plexiform tumors on her chest and airways.  We assured him that was her “normal”, and so I asked the new doc if perhaps that was the case. She looked at me and paused (like she was counting to 10 in her head so she didn’t hit me) and said “see this dark space on the X-ray? That’s where her lung should be”. She suspected a total collapse of the right lung. We had been at the main hospital a week before for an appointment at Orthopedics so I asked if she could pull up the X-ray from that visit. She did and sure enough, the lung was fine the week prior and completely collapsed on the new X-ray. The doc immediately sent us down to the ER at the main Seattle Children’s campus.

We get to the ER and had to chuckle because the check-in nurse immediately picks up the phone and says “the pneumothorax has arrived. I repeat, the pneumothorax has arrived”. Scarlett and I laughed because we thought it sounded like “the Lorax has arrived”. (Also, Pneumothorax basically means “collapsed lung”). They immediately get us back into a room and a doc and nurse come see us. Surprisingly, they were just wearing regular masks and scrubs. About 5 minutes later, another nurse sticks her head in the door and quietly says to them “low fever, test not back yet”. No kidding, the doc who was talking stopped mid-sentence and they all immediately raced out of the room (I know, there are a lot of racing docs in this story). I guess they thought she’d already tested negative. Oops. About 5 minutes later they come back in, decked out in the SuperHelmets and head to toe PPE. (Good news: she was negative for Covid although we didn’t find out until much later that night).

They do another X-ray and confirm the collapsed lung. No one has any idea what caused it; these are often caused by some sort of injury to the chest but she just woke up with it collapsed. The most important thing was getting the lung “up” again, so a surgeon came down and placed a tube in her chest about an hour later. They attached the tube to a machine the suctions all the air out of her chest cavity and gives the lung space to inflate. She’s been on that machine since the tube went in. They admitted her to the hospital (by now it was almost 2 am) for observation and diagnosis of what caused the collapse. The next few days are sort of blurry for us (not much sleep) but involved X-rays every few hours to see how the lung was holding up. They actually had to insert a larger tube two days later because she needed more pressure to keep the lung open.

Unfortunately, the lung still wasn’t holding up well and needed more and more pressure from the machine. That meant there was a hole somewhere in the lung. (Plot twist, there were several). They did a CT scan to locate the holes. Children’s is really amazing about ensuring patients and parents see all the images from X-rays and scans. They bring up the CT scan and show us several “holes” at the upper quadrant of her right lung. The holes are called “blebs” and are like blisters that pop open on the wall of the lung. As I mentioned, she has several. The docs stressed that it’s really good news the blebs were only in one lung and we try to be happy about that. The surgeons and pulmonologists discussed her case and determined that the blebs haven’t shown any sign of closing up and she’s going to need surgery to remove the part of the lung with all the blebs. The surgery is Friday morning. After removing the bleb section, they’ll also insert a substance into her chest cavity that will stick the lung to the chest wall and help the lung stay open for future “bleb attacks”.  What caused the blebs? We don’t know for sure. There have been some documented cases of adults with NF and lung disease (blebs) but only one case of a kid with NF and the disease. That’s our Scarlett, always blazing news trails.

After the surgery, they’ll keep her here (hospital) for several days to observe and make sure they can remove the chest tube. We’re hoping to go home on Monday or Tuesday. We’re all hanging in there. Jon and I are switching off at the hospital. Luke wrote and recorded an amazingly sweet (and hilarious) song for Scarlett. Kel and the boys FaceTime her often. Tax week (this week) is the busiest time of the year for me at work but I’m blessed with the world’s greatest paralegal. And finally, Jon and I would be lost without David and Jessica. We are endlessly grateful for our village.

When she’s awake, Scarlett is staying busy with lots of art projects. Childrens is amazing; they have team members who bring her paints and canvases. She’s also working on her comedy routine. She has a giant stuffed bear in her room and every time a new doc or nurse comes in, she tells the story:

My mom and I were here last week for another appointment. We stopped by the gift shop on the way out and I saw that bear and loved him so much. My mom said “he’s huge; that’s more of a post surgery reward. Too bad you don’t have any surgeries in the near future”.

And I said to myself “lungs, let’s do this!”

I’ll try to get another update posted after the weekend.

Move love, Shan

PS: One last thing – Every decision we make now is made in light of Covid.  If Jon or I get Covid, we won’t be allowed to be with Scarlett in the hospital and I can’t bear that excruciating possibility. Needless to say, a kid recovering from lung surgery is also at high risk for complications from Covid. We’ll likely be isolating even more hyper cautiously now. Thanks for understanding (and please wear your masks❤️)

(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.

I know I’ve gone overboard on updates lately; so much has happened in the last few months. Thank you for bearing with me.

Hopefully, this will be the last update for awhile. Yesterday, I got a voicemail that I’ll probably save forever. I consider it the Exhale Message. It was the Kaiser Pharmacy calling to let us know that insurance approved Scarlett’s meds and I’ll have them in hand on Tuesday. It took everything I had not to cry (happy cry) when I heard it. I’d prepped my inner Xena for a months long battle with the insurance company, but they just approved it without question. Exhale. Full credit goes to the fantastic Oncology docs at Childrens who lobbied for us.

The medication is a tablet she takes once a day (it’s a form of oral chemotherapy). She goes in for one more MRI so we have a baseline at the start of the medication this week. The MRI will probably take a couple of hours, but she’s a pro. Childrens Hospital makes everything easier for kids, so they have movie players in the MRI machines. She’s thrilled because she gets to watch Star Wars. Again. The Force is strong in that kid and I’m beyond lucky to be her mom.

So much love,

Shan

Sometimes written words just don’t convey adequately, so Scarlett can tell you directly:

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Darn. Looks like video link isn’t working. In short, a fantastic Oncology doc at Childrens is helping us get the meds. We’re estastic.

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

I’m feeling less than creative this Monday morning, so I’m going with a somewhat meta title. (D’oh! it’s now changed)

Following up on last post: we’ve received word that Scarlett won’t be in the drug trial. It’s close to completion and all spots were filled. I actually found out about 10 days ago but needed some time to sort out my thoughts/emotions before posting. As always, some time in the mountains and a few long drives were very good for me.

If you know me, you know I always find the upside/silver lining, so here it is. Even though the trial was closed, the folks at NIH were kind enough to look at Scarlett’s scans and records very closely. They determined the drug could potentially and significantly help her (average tumor reduction in the study to date was approximately 31%). So, they suggested we apply for access to the drug via a compassionate care exception. They put together a huge packet for us and I’ve spent the last week working with Scarlett’s docs to figure out how to coordinate the application. It’s up to the drug company whether to approve it and then I’ll likely have to battle the insurance company. Thank goodness I took health care law in law school. Haha; see what I did there? Found a silver lining despite my hatred of law school 😉

So, I suppose I’m still hopeful that we might have access to the meds without the NIH trial. This seems silly in the grand scope of things, but I’m a bit bummed Scarlett won’t get to see D.C. (NIH is in Maryland so I’d hoped for a few days away from hospitals to show her the Capital, etc.). I feel strongly about reminding my kids how truly extraordinary our country can be, and to me, D.C. is so inspiring. Many of you know that I value travel far more than things, and travel has been great therapy for my family. I’ve also tried to teach my kids that having something to look forward to often makes things a bit more bearable in the present. In any case, travel is up in the air for now. (My puns are so bad that I crack myself up).

Wow, sorry about that – I got way off track there with the travel tangent. Not the first time.

So, that’s where we are. Scarlett is feeling great and the kids are enjoying their summer. We try to up the playfulness a bit more in summer. Last night, we dyed the bottom of her hair flaming red (as she says: “the color is not just red, it’s Scarlet Red”). I’m trying to spend more time in sunshine, lakes, and lovely gardens. Simple pleasures. Which reminds me, thanks to all of you who keep me laughing. It’s truly the greatest gift and you can’t imagine what it means to me.

So much love,
Shan

This is going to be my motivation when I have to argue with insurance companies
😂< img src=”https://scarlettscott.com/wp-content/uploads/2017/07/img_3964.jpg”>

Wow, it’s been awhile. I suppose no news on the blog is good news.  But honestly, so much has happened in our lives and family in the past couple of years that I wouldn’t know where to start. (Don’t worry – I’m not about to belt out my best Eva Peron. But still, no tears needed).  Please remember that I’m not a writer so this will probably have no structure and ramble a bit.

Dressed up as Wonder Woman on Nov 9th 2016 to cheer mom up 😉

Scarlett is 10 now (going on 25).  She just finished 4th grade and I’ve returned to the blog because many of you have asked what’s going on lately, and also because I think she’ll be helping me write it soon. She really is her own best advocate,  and she’s an extraordinary kid. I’ve never heard a “why me?” from her.  Her sense of humor is marvelous (I’ll see if I can upload the stand up routine she did for the Camp Korey Gala last year).

Anyhow, we have a motto in the family to always be Kind + Courageous. Both my kids take courage to new levels.  In Scarlett’s case, it’s dealing with the uncertainty and challenges of NF while never being defined by it. She just rocks it.

So, where are we medically?  Some of you may remember the rough time we had last year when Scar was getting dizzy all the time and having fainting episodes. We spent about 10 days living at Children’s trying to get answers. Finally, the neurology team determined that one of the two main arteries into her brain (!) was crushed by the neurofibroma on one side of her neck. Yup, Big Time Scary.   But the body is an amazing thing, and it regulates and adjusts blood flow to the brain as needed. In Scarlett’s case, the other artery picked up the flow.  It turned out she was having a variation on migraines due to this “unique” arrangement (thanks NF!)  and instead of pain, it caused severe dizziness. We’ve been able to control it with migraine meds and she went back to her happy, non-dizzy self.   So, we’ve been stable for awhile. Some of you may remember that “stable” is my favorite word (besides “euphemism”)   😉

I suppose the biggest potential news is that we are trying to get into a drug study that has produced pretty significant results on plexiform neurofibromas.  I hate to make this about me in any way, but this is a tough one for me.  I was shattered after the 2012/2013 drug trial, and I don’t shatter easily.  About 6 months ago, I was sitting in a New York coffee shop with my best friend and she asked what the worst part of this experience with NF has been. And it was one of those “oh sh*t, here comes the sob” moments.  Spontaneous sob. In public. In New York.  I stress the NY factor because there are LOTS of people in NY and I HATE crying in front of other people (but I’m working on that one).  Because, at the end of the last drug trial, when I sat in a doctor’s office and heard him say “the drug didn’t do what we needed it to”… for me, that was the end of Hope for awhile. And if you know me, you know I am ALWAYS Glass Half Full.  But at that moment, I wasn’t even holding a glass anymore.  And you know what was harder?  Sitting in an airport waiting to come home and having to explain it to a 6 year old.   Brutal.  Do you remember the book I made for her about participating in the trial and how she could help other NF kids around the world?  I still can’t look at it. But you know who said “okay, well.. at least we tried” and then moved on? Scarlett did, because that’s who she is. She has a huge, beautiful life to live, and jokes to tell, and people to love.  She just keeps truckin’  (ha ha, who’s picturing mud flaps right now?)

So yes, I’m frightened of the disappointment again.  But it’s reality check time and it’s not about me. The reality is that we will do whatever it takes to blunt the impacts of NF on her life. And a drug with the potential to shrink the tumors would certainly do that.  So, however it is you request help from God or the Universe (prayer, dance, etc) please send one out/up there for us about the drug trial.

Of course, if you know me, you know I always hope for the best but still plan for the.. not best. It’s the  romantic tragedist in me. (If tragedist isn’t a word, I’m claiming it right now.)  So, if we don’t get into the drug trial, the surgeons at Children’s want to do another airway surgery and possibly surgery on her eyelid. It’ll be the first eye surgery (all the rest have been in her throat/airway).  You guys will love this: when we first saw the eye doctor about possibly lifting the eyelid, Scar didn’t say a word. Finally, after more silence in the car on the way home (super unusual for her) I asked if she was scared about the surgery. Even more silence. We were stopped at a light and I looked over at her and saw a huge tear roll down her cheek. And do you know what she finally says?  “Why do they have to fix my special eye? I like that it’s special”.  That’s so Scarlett. So, we agreed that they won’t try to make it look like everybody else’s eye, but will lift it enough to help her vision.

One other thing, and I mention it to thank some extraordinary people. Unfortunately, besides the medical issues, NF causes some pretty tough learning challenges.  And I’m convinced that if angels exist, they are the teachers helping our kids. Scarlett has been blessed with unbelievable teachers and there are no words to express my gratitude. If you’re reading this and are one of her teachers, please know how priceless you are to my family.

So, that’s pretty much where things are right now. Thanks for reading this far.  I admit that one of things I struggle with on this blog is tone.  It always sounds so Debbie Downer, but if you know me and the kids, you know that we spend 90% of our time laughing and cracking each other up. There is no misery.  Right now we are waiting for the sun to set so we can watch fireworks, Boomer is sedated, and I’m hoping the neighbors’ arsenal doesn’t burn the house down.  So, in the words of Carl Spackler, I got that going for me 😉

So much love to all of you- Shan

Adorable kid and sedated puppy – Happy 4th of July!

me with Wonder Woman (x2)