Running Update:

1/25/2019	Run		5.0 mi	48:07
1/26/2019	Swim		2100.0 yd
1/27/2019	Run		9.0 mi	1:31:26
1/28/2019	Swim		2100.0 yd
1/30/2019	Run		5.0 mi	48:27
2/6/2019	Run		2.0 mi	18:36
2/8/2019	Run		5.0 mi	49:10
2/10/2019	Run		7.0 mi	1:08:26
2/11/2019	Swim		1500.0 yd
2/15/2019	Swim		2100.0 yd
2/16/2019	Run		5.0 mi	49:44
2/17/2019	Swim		2100.0 yd
2/20/2019	Run		5.0 mi	49:15
2/22/2019	Run		5.0 mi	48:29
2/23/2019	Swim		2100.0 yd

I’m not sure why I’m even posting this month’s stats—February has been a training disaster!  I lost a week and a half to a bad chest cold, then another week and a half to a stupid knee injury.  My knee is still not 100%, but I’m hoping to get a long run in this weekend and get back on track.  Only three weeks until my first race of the season, the NYC Half Marathon, with the Children’s Tumor Foundation.

Jane/NF Update:

Last week Jane and I returned to NIH for her semi-annual check-up.  Thank you for patiently waiting for our report.  It sometimes takes me a few days to process what happened at the visit and to recover from the trauma from our trips, because they can be traumatic!  Even when we get good news, it always come with the price of worry, discomfort, and exhaustion; and knowing that the results are only a temporary respite from the chronic troubles that NF presents.

And we did get mostly good news from this visit!  But that simple accounting belies the hard work we had to do to obtain it.  I thought I would give you the hour-by-hour details of our whirlwind trip so you could get a taste of our experience.  I therefore present:

NIH February 2019: There and Back Again in 36 Hours

This is the itinerary we got from our care team ahead of our trip:

An ambitious schedule (maybe even overly so), but doable as long as we stayed on task.

Here’s how it actually played out:

Monday 2/18/19, 10am.

Jane and I listened and sang to most of the first act of Hamilton during our drive to the airport.  (A pleasure!)  We navigated the airport without any problems and made it to our gate.  Then, just as we were boarding, Jane told me her head was starting to hurt.

Jane is prone to migraines, whether because of her medication or because of NF itself, we’ve never been sure. And her migraines, when they happen, are not just bad headaches. They’re knock-you-down headaches with nausea and vomiting that last for hours and only resolve by sleeping it off.

Jane spent the first half of our flight trying to get comfortable while retching into the airsickness bag.  (She went through two.)  She finally fell asleep on my shoulder and slept for about a half hour.

2pm.

Once we deplaned, we sat quietly in the airport for about 45 minutes before Jane felt well enough to drive to NIH.  Thankfully, we made record time in the cab and once we got to the Children’s Inn at NIH Jane immediately felt better.  (Phew!)  Someone crocheted a zillion scarves for the Children’s Inn residents, so when we arrived, they gave us each one (and even one to take home to Helen).

4pm.

Jane and I found a perfect niche to play Bananagrams!  (I had brought this favorite game of ours along.)  The Inn is still under construction because of roof damage from last summer, so one of the hallways is now a dead end, perfect for a quiet board game.

One of Jane's rounds

6pm.

We joined the communal dinner the Inn provided, then Jane had a chance to do some glass painting with the other children.

Jane's creation has a black rim, in the center, middle row

8pm.

Bed!  We had to be up early the next morning!

Tuesday, 2/19/19 7am.

We started the day at the Pediatric Clinic at the NIH Clinical Center for Jane’s vitals, blood draw, and pre-MRI medication.  FYI:  Jane is now 5’6”.  Five feet six inches at eleven years old!  Everyone we meet exclaims about how much she’s grown when they see her.

8am MRI.

MRI number 26 for Jane—she’s getting pretty good at them.   Jane fell asleep during the second part of the MRI.  I could tell because her legs were twitching like she was dreaming!  It seems the combination of not sleeping well the night before and the 0.5mg of Ativan they gave her did the trick.

9am EKG.

While waiting for the EKG technician we were amused to find that the EKG staff use the sticky EKG leads to tape things to the announcement board.

9:15am Pediatric Clinic again.

A full physical exam with the lovely new APRN on our team.  All went well except that she had trouble seeing Jane’s eardrums, so arranged for Jane to be seen by ENT later that afternoon if we could fit it in!

10am Echocardiogram.

Easily the most relaxing exam.  The exam room is dark and warm and filled with soothing heartbeat sounds.  Jane has the EKG and echocardiogram every visit because her study medication can affect heart function.

While in the waiting room for this test we learned that the DC area was expecting 4-6 inches of snow starting early Wednesday, the following morning.  We were scheduled to fly home at 10am that day.  I started wondering if we could try to fly home Tuesday night, but with such a full schedule at the hospital I didn’t think we’d ever be able to swing it.

10:30am Pediatric Clinic again.

Back to meet with our research nurse to go over medication diaries and other paperwork.  By this time, Jane is getting antsy and starting to pester me that she’s hungry.  Already?  Well, when you’re eleven and you get up an hour earlier than you’re used to spend a day at the hospital, you don’t feel like eating much breakfast.  Thankfully she was able to grab a granola bar and apple juice from the clinic fridge.

11:00 ID renewal. 

Our NIH IDs were due to expire soon.  If we didn’t renew them this trip it would make it very difficult to get back on the NIH campus on our next trip.  Security is crazy strict on the NIH campus.

11:30 Lunch! 

Jane even searched out her favorite lunch lady to give her a hug and to allow her to marvel at how tall Jane is getting :)

(Aside—eleven-year olds should not spend enough time in the hospital to have a favorite lunch lady in the hospital cafeteria.)

Noon Photography. 

Photos taken to compare the outward appearance of Jane’s tumor across visits.  This appointment is usually quick and relatively fun, though lately Jane will only pose for the photographer and not for me!

12:30pm Ophthalmology Clinic. 

Eye Clinic is the appointment that take the longest—typically hours because of the many layers of testing that take place.  These are done because Jane’s medication can affect the cornea and retina, and NF itself can affect the optic nerve.  That day we had a miracle worker from the Eye Clinic staff who was able to complete Jane’s OCT in record time.  We were in and out in a little over an hour!

1:45 Otolaryngology Clinic.   

Jane’s right ear canal has always been vulnerable.  Before starting selumetinib Jane’s plexiform neurofibroma (tumor) had surrounded and collapsed her right ear canal, making it prone to obstruction and infection.  In recent years that ear canal has opened with the shrinking of Jane’s tumor.  However, today it seemed blocked again so our team’s APRN wanted it double-checked by ENT.  She managed to get us in to see them in between Jane’s eye and skin appointments!  They cleaned Jane’s ears and took a good look at her ear canals and drums and pronounced them healthy.

2:00pm Dermatology Clinic. 

One of the longest appointments of the day.  While waiting to be examined Jane was holding up reasonably well.   To pass the time she read, played games on my phone, made balloons out of exam gloves, and we did the New York Times crossword puzzle together.

Jane was scheduled with the Dermatology Clinic in part to get recommendations for the significant eczema that selumetinib has given her (OMG she’s itchy all the time!  Despite oatmeal baths, gobs of moisturizer, Vaseline, steroid creams, and Claritin!) but primarily to help address a particularly nasty rash on her legs Jane has been dealing with for the past month or so.  It’s painful and itchy and we’re not sure what is causing it.  The Dermatology team thought one possibility is a viral skin infection that can occur when the skin is chronically damaged (as Jane’s has been from the eczema); the other is a drug eruption—a reaction to her medication.  The treatment for a drug eruption would be steroid cream, but steroids would make the viral infection worse, so it was important to determine what the cause was.  Unfortunately, that meant a biopsy of one of the spots.  And not just a swab—it meant shaving off one of the spots with a razor.  By now it’s almost 4pm.  While this would not ordinarily be a huge deal to you or me, try explaining that to an eleven-year-old who has already been at the clinical center for 9 hours and who has already gone through all the other testing I’ve described.  Jane was not happy.  There were tears and no small amount of protest initially, but Jane agreed to let the doctors numb up her leg and take a sample.  The procedure itself took only a few seconds, but all the discussion and anticipation ahead of the procedure made it worse, especially coming on top of a long day.

This was the hardest part for both of us.  Jane was quite angry with me from this point on.  She blamed me for making her have a biopsy.  I completely understood her frustration and anger, and knew I shouldn’t take it personally, but that doesn’t make it easier for a mother.

4:15pm Family Meeting.

Last appointment of the day!  When it was time for us to meet with our team, I went to collect Jane from the play room and found her playing a board game with a girl who seemed to have a large tumor in the same spot as Jane.  As we walked away, I said to Jane, “I wonder if she has NF, too.”  Jane said, “She does.  I asked her.  She’s 17.”  Jane never seems to shy away from making new friends—it is one of her greatest strengths.

Finally, we heard some results:  Jane’s tumor is stable, and perhaps even slightly smaller!  The decrease may not be statistically significant, but that’s way better news than “slightly larger”, as it has been on our last several visits.  We are thrilled!  We’re so relieved that the increased dose of medication seems to be having an effect.  There is one very small section of the tumor that is a little more prominent than before, but we’re just going to keep an eye on that for now.  All of Jane’s bloodwork and other testing looked good as well.

Look at that lovely final plot entry!

The radiologist created this comparison for us.  It never ceases to amaze me how much progress we've made.

The plan going forward:

-Jane will continue on same dose of selumetinib.  Her BSA is at the lower end of the range for this dose, so she’ll stay on it for a while, unless she grows significantly.

-Wait for the skin biopsy results to see what can be done for the rash on Jane’s legs.

-Return to NIH again in 6 months.  At that visit Jane will have a whole-body MRI, instead of just a scan of her head and neck.  Our team recommends this be done every few years.  The last time Jane had a whole-body MRI was 5 years ago, as it was required for admission to the clinical trial for Gleevec in which she participated at Indiana University.  At that time, in addition to further analyzing her known facial plexiform (and all its tentacles), we learned that she also has a bony deformity in one of her cranial bones which is causing a small herniation of part of her brain, and multiple 1-2 cm neurofibromas throughout her chest, abdomen, pelvis, arms, and legs.  I don’t like the whole-body MRI because, quite frankly, I would rather not know what other tumors may be lurking in Jane’s body since there is nothing else we can do about them.  For me it will just be another source of worry.  But we trust our care team completely, so we’ll do what they recommend.

5pm Travel Office.

Our appointments may finally be complete, but we were not done with our day yet.  Our next stop was the Travel Office to see if we can fly home that night to avoid being stranded in the DC area with the coming snowstorm.  Miraculously, there was a 10pm flight home available, so I took it.  Jane wanted nothing more to just head back to the Inn to sleep, but with everyone at NIH telling us to get out while we could, I felt we’d be smarter to leave then.

5:15pm Pharmacy.

We must pick up all of Jane’s medication for the coming 6 months as selumetinib is not available by prescription at any local pharmacy, and NIH is not supposed to ship it if at all possible.

This is what six months of chemotherapy looks like.

5:30pm The Children’s Inn.

Packing up and checking out.  OMG.

6pm Shuttle.

Caught the last bus from NIH to BWI airport with time to spare!

7:30pm Diner.

We got to the airport starving but with two hours free time before boarding, so we decided to treat ourselves to a sit-down dinner at the airport diner!  Sigh.

Jane's favorite: an Oreo shake

8:30pm At the gate.

As luck would have it, we ended up taking a seat right next to Jane’s first grade teacher!   She recognized Jane before we even recognized her.  She gave Jane a huge hug :)  She has always been one of Jane’s favorite teachers, so it was a special coincidence to bump into her.  And she was traveling with her puppy!  What a delight for Jane after such a demanding day!  Jane’s teacher told us that she herself was originally scheduled to fly home the following day, but that her flight had already been canceled due to weather.  I think we made a good choice to switch our flights.

9:30pm Boarded!

We don’t end up getting home until after midnight, but I’m so glad we didn’t get stuck in DC!  We both slept well in our own beds that night.

And there you have our hectic 36-hour journey, one of dozens we’ve made in our quest to #endNF.  You can see why we need a few days to recover!  At least now we have six months to prepare for the next trip, though Jane will continue to face the challenges of living with NF every day until then.