NIH visit Sept-Oct 2019

Sunday:
We arrived at the Children’s Inn around 4pm.  Just enough time to settle in before the Family Dinner served by the Inn.  We had tacos!  There seemed to be fewer residents than I would usually expect.  Part of the Inn is still under renovation after structural damage was discovered last year.

Jane getting in a little painting before dinner

Monday:

Our itinerary

We didn’t have to be at the Clinical Center until 9am (later than usual!) but we had a full day. 

Started with vitals and labs.

Nurse Clarisa and Jane--using Puppy to make a fist for her blood draw

Then meetings and a complete check-up with our team’s Nurse Practitioner, Anne.

In the exam room where Jane had her check-up, there was an old globe. Jane glanced at it quickly and said it must have been made before 1997 because the Congo was still labeled “Zaire”.

She went on to determine that since Myanmar was labeled Burma, Yugoslavia was one country, the USSR existed, Sudan was one country, Germany was split East/West, and Yemen was two countries, the globe must have been made sometime between 1967 and 1990.

ENT

In the past this appointment just involved checking Jane’s ears because her tumor compresses her ear canal.  This time she experienced something new—a laryngoscopy!  After spraying a numbing solution in Jane’s nose, the doctor used a small camera to look through her nose into her throat.  Jane said it was like a colonoscopy but for the nose!  She did not enjoy it.  We could see her vocal cords working and could happily see that her tumor was not obstructing her airway.

After ENT we added in an unscheduled ID renewal and pharmacy stop, too.

Eye exam

We had a brilliant Ophthalmology check-up. Jane had been holding her beloved stuffed animal, Puppy, and the doctor commented how well loved he looked.  She said that still has the stuffed horse she carried around as a child.  The horse had lost an eye at one point, so the doctor renamed him Odin. Then the conversation went something like this:

Jane: You should have named him Sleipnir. That’s Odin’s horse.

Doctor: Oh, yes! Odin’s eight-legged horse!

J: The horse was Loki’s son.

Doc: Right! From the time Loki shape-shifted into a mare...

They spent the whole visit discussing various mythologies—Norse, Roman, Greek (Jane argued that Greek was her favorite)  The doctor admitted she was a mythology buff. We asked if she had read the Percy Jackson series, and she asked Jane if she had read the Golden Compass series.  It turns out that the doctor was such a fan of the Golden Compass that she named her daughter Lira!  The exam was over before we knew it, but Jane hardly noticed because of the conversation.

Time for a brief stop in the Main Playroom before reporting to MRI.

Painting a ceramic

And a landscape

MRI

Jane’s 26th.  Every six months Jane has an MRI of her facial tumor, but this visit she was also due for a full-body MRI.  Our team recommends this be done every few years.  As I have written previously, the last time Jane had a whole-body MRI was five 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, 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 much we can do about them.  For me it will just be another source of worry.  But we trust our care team completely, so we do what they recommend.

Jane spent one and a half hours in the MRI scanner, wrapped up like a burrito, encased in sensors and coils.  I have no other words to describe her except: Rock Star.

~~~

After spending eight and a half hours at the Clinical Center, we got back to the Children’s Inn just in time for Jane to catch the end of the Inn’s #CakeItLikeAKid challenge. They were letting the children stick cupcakes into the faces of the Inn’s staff in honor of Pediatric Cancer Awareness Month.  Jane got the last smash!

Look at that smile!

The Family Dinner that evening was “Breakfast for Dinner” (sausage, eggs, and hash browns) with special cupcakes for dessert.

Mocha fudge cupcake for me, chocolate vanilla for Jane

Tuesday:

We were back at the Clinical Center by 8am this morning.  The day was scheduled to be a little bit shorter (ending at 3pm), though it proved to be more emotionally taxing.

Echo

I ask you: what other child ends up singing Led Zeppelin with the sonographer during her echocardiogram?  

It started when Jane noticed a poster in the sonographer’s exam room of the Beatles from a concert in France in 1965. The sonographer was a fan, and I asked if he’d attended the concert.  He said no, he was too little, but he still remembers not being allowed to stay up to watch the Beatles on the Ed Sullivan Show.  We started taking about musical groups and somehow landed on Led Zeppelin. Jane commented that Led Zeppelin had a song featured in the Marvel movie, Thor Ragnarok. The sonographer identified it as the Immigrant’s Song, and Jane and he started singing it together!

We come from the land of the ice and snow

From the midnight sun, where the hot springs flow...

During this silliness, I received a call from Anne, our Nurse Practitioner.  They had seen a new mass near Jane’s left kidney on the MRI and wanted to add an abdominal ultrasound to our morning schedule to take a closer look at it.  Ugh.

Forty-five minutes later we were in the Ultrasound Department (having squeezed in an EKG along the way).  This scan was not as much fun, as both Jane and I were apprehensive.  I could see the area they were studying on the screen, a 3-4cm solid mass to the left of her abdominal aorta.  I’m not gonna lie, I saw Jane’s life flash before my eyes.  People with NF are at increased risk of several different types of cancers.  But as I talked myself down, I tried to reassure Jane: the mass is nice and smooth and uniform, which made it likely to be just a neurofibroma.  (I spoke these words with confidence to her, although I didn’t feel as confident as I pretended.)

Photography

This is usually an easy visit, but Jane and I were both preoccupied.  Jane’s old enough now to understand what’s going on and she was nervous.  She kept asking me what the mass might be.  I tried to reassure her as much as I could.

Jane is not amused by my picture-taking

Family Meeting

Mercifully, this meeting with our team was moved to earlier in the day so we could get some answers.  We were joined by Dr Andrea Gross, another pediatric neuro-oncologist on our team, since our usual team leader, Dr Brigitte Widemann, was not available.  She put our minds are rest right away but telling us that the mass did not look like cancer, but instead was likely one of several types neurofibroma.  We were told one possible type, a distinct nodular neurofibroma, does have a higher chance of becoming cancerous, so we will watch this tumor very closely.  It might need to be removed, eventually, depending on how much and how fast it grows.  We’ve no idea how long it’s been there since we haven’t looked in Jane’s abdomen for five years.  It could have developed over just a few months, or it could have been growing for several years.

Jane asked appropriate questions, including, “Did this tumor grow because I was off the MEK inhibitor?” Answer: No. This kind of neurofibroma not affected by MEK inhibitor.

I was lightheaded with relief, but THIS IS LIFE WITH NF.  You manage to get one tumor or complication or side effect under control, and another one pops up.  This is the way it will be for the rest of Jane’s life.  This is why we need to find a cure!

Cross section of Jane's abdomen on MRIs 2014 (left) and 2019 (right)

Seriously?  WTF?

We don’t yet have volumes on Jane’s facial tumor—those will come next week—but the team said it looks like it has only grown a small amount since Jane has been off the study medication these three months.  We are planning to restart the selumetinib this week.  It will be a slightly lower dose than Jane has been on in order to avoid all the dermatologic side effects she’s had.

When we were wrapping up the meeting, Anne told Dr Gross about Jane’s investigation of the exam room globe.  Dr Gross asked if Jane knew about the Geo Bee. We said of course! Jane represented her school in a Madison last year.  Dr Gross told us that her brother once represented Connecticut in the National Geo Bee!  We marveled at the coincidence.

Believe it or not, we still had another clinic appointment before we could head home!  We had a little bit of time before our Dermatology visit, so we stopped in the Main Playroom to pick up Jane’s now-dry artwork from the day before.  When we got there, we discovered the playroom Guinea pigs were out to play!  Jane got to pet Twirl (whose sister is Swirl was also there).

Getting some therapeutic Guinea pig time before our final appointment

(Volume up to hear Guinea pig chatter!)

Dermatology

This appointment was a bit anticlimactic after the stresses of the morning!  The Dermatologist recommended starting a daily low-dose antibiotic when restarting the selumetinib in order to prevent the nailbed infections and acneiform rash associated with selumetinib from recurring.  While we were waiting in the exam room, a familiar face popped up on the computer’s screen saver.  It was our friend, Travis!

Our friend Travis encouraging you to wash your hands

~~~

So, the plan from this visit is:

-Restart selumetinib this week and slightly reduced dose.

-Start a low dose antibiotic to keep dermatologic side effects at bay.

-Repeat an abdominal MRI at home in 3 months to check on the new tumor.

-Meanwhile, our team will consult with NIH Neurosurgery about the possibility of removing the tumor.

-Follow up at NIH in 6 months.

Lots to do and think about.