Saturday, March 31, 2018

NIH visit 3/28/18 - 3/30/18
We don't have any trouble identifying our luggage these days

Jane and I arrived at NIH late Wednesday afternoon, just in time for the Family Dinner at the Children’s Inn.  We were looking forward to seeing another NF family who we knew were visiting NIH at the same time.  Travis and his mom, Kelly, come to NIH all the way from Utah.  I knew Kelly from working with the Children’s Tumor Foundation, and we had met her and Travis in person on a previous trip to NIH (  Like Jane, Travis has a large, troublesome, plexiform neurofibroma (PN), but where Jane's PN involves her head and neck, Travis' occupies the whole length of his leg.  He is the same age as Jane, and in the same clinical trial!

After dinner, we attended a painting activity, then headed to the game room for a few rounds of air hockey.  We documented the rematch between Travis and Jane and compared the pictures to ones from a year and a half ago.

Dinner buddies

Goofing around

Painting fun

Adding her finishing touches

Some of the Inn residents' creations

Jane and Travis at the air hockey table during this visit...

...and back during their first meeting in October 2016!
Jane had a blast—I had to pull her away to come to bed.  We were starting at the hospital at 8am the next morning, so wanted to get a good sleep.


Our busy itinerary for Thursday:

—Vital signs (3 hts, 3 wts) and Labs, Pediatric Clinic, 1st floor
8:30am—Meet with Trish, Pediatric Clinic
9:00am—EKG, Radiology, 1st floor (or anytime during free time)
9:30am—Photography, Room 1N230, 1st floor
10:00am—Echo, 5NE, 5th floor
11:00am—Physical exam with Andrea, Pediatric Clinic, 1st floor

will need Ativan prior *
12:00pm—Unsedated MRI (Face, sinus, ENT), Radiology 1st floor
          Lunch Break
12:45pm—ENT exam, Outpatient Clinic, 3rd floor
2:00pm—Family meeting, Pediatric Clinic
2:30pm—Eye exam, Outpatient Clinic, 10th Floor

We were appointment champions—keeping on time with that crazy schedule and barely stopping for a breath.  We even managed to get our security badges renewed before our Photography appointment!

We learned that Jane is almost 5’4” now!  Despite her height, she is still a 10-year-old at heart.
Her faithful stuffed animal, Puppy, accompanied us the whole day, and Jane and I played Go Fish in the waiting room between doctors’ visits.

Waiting for an EKG

At Photography

Needing a hug!

While Jane was having her echocardiogram, I commented that we could see her “heartstrings”—the chordae tendineae, thin tendons that attach to one’s heart valves.  We noted that dragon heartstrings can be used for wand cores (in the Harry Potter universe!)  The ultrasound technician said his children loved Harry Potter, too, and proceeded to give us a complete anatomy lesson on the chordae tendineae, the heart valves, and the papillary muscles that control them :)

Jane did brilliantly during her MRI, staying perfectly still for the whole scan.  I think she may have even had a little snooze during it. (She is given 0.5 mg of Ativan right before it, so that helps).  Unfortunately, we won’t have MRI results until sometime next week.

One of the dozen or so eye tests Jane endures.  Ugh!
During Jane’s eye exam the Ophthalmologist mentioned that he knew an Ambassador Jane Constable, one of the first and the few female ambassadors from the United States.  I had never heard of her, so we quickly Googled “Ambassador Jane Constable”.  Would you believe the first hit was our Jane’s speech at the NF Gala from November? (Link here.)  We showed it to the doctor, and he was duly impressed.  (It turns out the real ambassador was Elinor Constable, who was the Ambassador to Kenya in the 1980s.)

After eight full hours at the clinical center, we had an afternoon of well-earned fun time at the Inn.  Jane and Travis and a couple new friends spent most of it out-of-doors, enjoying the finally-spring-like temperatures.  One newcomer was a 13-year-old girl from England who was at NIH for treatment of leukemia.  A second was another 10-year-old boy with NF who also has a plexiform neurofibroma and who also is just starting the same trial and Jane and Travis!  While the kids played, we mothers compared notes and took pleasure in the company of other moms who know what it’s like to live with NF.

Jane on the playground! 
Shoes off, hangin' with the other kids, wearing sunglasses
(because she just had her eyes dilated), just being a kid <3

Travis' mom, Kelly, shared with me these fabulous photos of the kids being kids:

Passing the time by playing Go Fish with Travis with tiny cards while waiting for an appointment

Back at the Children's Inn

Petting Zilly, the therapy dog who lives at the Children's Inn

Racing Travis in his motorized wheelchair.
Three out of these four children are 10 year olds with NF on the selumetinib trial!
What good company!

Air hockey champion!

All of Jane’s screening came back normal, though as I mentioned, we don’t yet have her MRI results.  I will update you all as soon as we learn them.  Meanwhile, Jane has restarted the selumetinib at her usual dose.  It may be that her care team will recommend a higher dose going forward since Jane has grown so much, but we will wait until we know the current size of her tumor before making that decision.  For now, we’re done with testing until our next NIH visit in September.

Sunday, March 18, 2018

Running Update:
2200.0 yd

3.2 mi
5.0 mi
3.2 mi
5.0 mi
1500.0 yd

12.0 mi
5.0 mi
5.0 mi
2000.0 yd

9.1 mi
5.0 mi
13.1 mi

Today was my first race of the season, the NYC Half Marathon.  The course was a new one this year, starting in Brooklyn and finishing in Central Park.  It was cold, windy, and uphill, but I still managed to finish in under two hours!  I had missed more than two weeks of training in February due to an evil virus, so I didn’t think I’d make it under the 2-hour mark this time.  In the end, I finished in 1:58:40 which put me 203rd out of 1098 women in my age group.
Another 13.1 miles done to #endNF!

At the pre-race Expo

My name on the wall of runners!

Gear ready the night before

Running through Times Square

Coming up to give a hug to Todd and Alec

After the finish, with Neil, my running partner, Alec and Todd

The Bling

Jane Update:
Poor Jane fell while ice skating a couple of weeks ago and broke both bones in her forearm!  She’s been an extremely good sport about it, and we’re thankful it didn’t happen during soccer season or over the summer.  To be honest, it’s refreshing for me to report on a medical issue of Jane’s that is unrelated to NF.  Breaking a bone is just a Normal Kid thing to do.  Unfortunately, we’ve been instructed by our care team at NIH to stop Jane’s study medication until her arm heals.  Apparently, one child on selumetinib had some delayed bone healing after a break, so out of an abundance of caution, we’re holding the drug.  (That part isn’t a Normal Kid thing to have to consider.)
We return to NIH in less than 2 weeks for Jane’s next check-up and MRI.

Still smiling despite a broken arm!

After replacing the temporary cast with a (thankfully) waterproof one

NF Update:
I was recently reminded that it was four years ago this February that we were coming off a failed trial of Gleevec because Jane’s tumor was growing relentlessly, and we were desperate. We were offered the last spot in a clinical trial, involving only 24 children, for a new drug treatment for inoperable plexiform neurofibromas.  That drug, selumetinib, was just granted Orphan Drug status and has the potential for FDA approval.  How far we’ve come!
This is great news.  Jane (pictured in the article!) started taking selumetinib as part of Phase I of the trial. Jane’s tumor is now 30% smaller thanks to this medication. Phase II of the trial is now nearing completion, and selumetinib then has the potential for FDA approval.
Since plexiform neurofibromas are rare, to date there has not been great financial incentive for pharmaceutical companies to develop treatments for these tumors. However, Orphan Drug status means that the manufacturer of selumetinib (Astra-Zeneca) can get monetary support to continue researching and producing the drug.
And we want selumetinib to be available, not just for Jane, but for all children with NF tumors like hers!
Thank you to everyone—from our clinical team to our donors—who are helping to #endNF in our Jane's lifetime.