NIH/DC Update:

Jane and I had an uneventful flight to Bethesda on Sunday.  We spent the afternoon resting at the Children's Inn across from the Clinical Center.

On Monday Jane had a full day of testing, beginning at 8:30am.  The day started with a blood draw, for which Jane never flinches.  Next was Eye Clinic, where the battery of testing and exams took 2 hours (relatively quick for the black hole that is the NIH Eye Clinic.)  Next came her physical exam with our nurse practitioner.  Since our arrival at NIH we'd had about twenty people tell Jane how tall she's gotten, and now we know exactly how much:  she's grown 2-1/2 inches in the past 6 months :)  We followed her physical exam with an EKG.  Then, right before our lunch break, we even managed to get to the Security Desk to have our hospital badges renewed.

The afternoon began with an echocardiogram.  The room was so dark and quiet that Jane fell asleep during the hour-long exam!  The technologist was so gentle, and Jane so tired, that she didn't wake up when he shifted positions or changed angles.  I was glad, thinking it might help give her the energy to make it through the rest of the day and her ever-important MRI.

The last appointment before her MRI was my favorite, Photography.

By this time, Jane was starting to flag.  I gave her a pep talk telling her she had to hang in there, that the MRI is the most critical test of our visit...  In the end, Jane stayed SO STILL for her MRI!  I think it was the best one she's ever done without sedation.

Back at the Inn after 8 hours of testing at the Clinical Center, we had dinner and an early bedtime in anticipation of receiving the results of MRI #22 in the morning.

Tuesday morning, we were back at the Clinical Center again at 8:30 am, this time for pictures with our care team to be used by NIH for NF Awareness Month in May.

With our research nurse, Trish, Dr Brigitte Widemann, and our nurse practitioner, Andrea

After the photo shoot, we got the news we'd been anxious to hear:  Jane's tumor appears stable!

It is currently 30.7% smaller than when she started the trial 3 years ago.  We bottomed out at 33% smaller about a year ago, and it's remained within that margin of error ever since.  Of course, we are thrilled!

A few more issues we discussed Jane's visit:

Medication dose.  Jane is currently taking only 10mg of selumetinib twice a day.  The typical dose of children in the study at this point is more than twice that.  Jane's dose had been reduced early on due to a severe rash that she developed about a month after starting treatment.  At the time, less than two dozen children had ever taken the medication, so to be cautious our care team kept her at the lower dose.  However, they have since found that several children developed similar rashes early on in this treatment, and that the rash usually resolves without reducing the medication dose.  Jane's care team is again considering returning her dose back to what it would have been had she never had the reduction.

The problem is that the study protocol requires that the medication be given in two equal doses daily; and as luck would have it, the only capsules manufactured by the pharmaceutical company (Astra Zeneca) are 10mg or 25 mg--so there is no option to increase the dose gradually, say by 5mg at a time.  The only way to increase Jane’s dose would be to jump immediately to 20mg or 25mg twice a day, and such a jump would invariably cause more side effects.

However, Jane’s doctor, the wonderful Dr Brigitte Widemann, did hint that it may be possible to alter the study protocol to remove the restriction of equal doses twice a day, thus allowing Jane to take, for example, 10mg in the morning and 20mg in the evening.  This would be a more manageable dose increase.  The team will need to review this possibility and get back to us. 

On the one hand, we are content to see Jane’s tumor is stable, and don’t feel the need to excessively press our luck with a higher dose of selumetinib, especially when she has been feeling so well.  On the other hand, since Jane has already been subjected to the risks of selumetinib, we’d like her to derive the maximum benefit that she can from the medication while it is available, in case there are ever issues with obtaining the drug in the future (eg if it doesn’t get FDA approval or if the company decides it’s not worth their while to continue manufacturing it).

We also discussed with Jane’s team her facial structure. This subject came up because recent dental x-rays had highlighted the bony abnormalities of Jane’s face.  It turns out her face may never be symmetrical, even if her tumor completely disappeared today.  I’m embarrassed to say this never occurred to me before, but it makes sense.  The tumor occupies a large space in Jane’s head and neck, and it has disrupted the growth of the bones in her face, causing a defect in her mandible (jaw bone) and an asymmetry in her mouth.  This is unlikely to change even if the tumor went away.

~ ~ ~

With our NIH appointments complete, Jane and I turned our attention to another adventure:  a visit to Capitol Hill!  In my last post, I described how our Representative from Connecticut, Congressman Joe Courtney, invited Jane and me to meet with him in his Washington office to share our experience with the National Institutes of Health and thereby illustrate the importance of its funding.

Ready to conquer the Capitol 

Jane and I took the Metro from the Medical Center all the way to Judicial Square and walked from there to the Rayburn House Office Building next to the Capitol.  I can’t tell you how intimidating it was walking into this building!  We were literally within the Halls of Congress!  Congressman Courtney and his staff could not have been more gracious and welcoming.  The Congressman spent a good half hour speaking with us, asking questions about Jane and her treatments, and listening to our story.  We had just heard the good news that Congress had agreed to a $2 billion spending increase to the NIH for the remainder of this fiscal year, and Congressman Courtney vowed to fight for continued financial support in the budget for NIH in the upcoming year.

We gave the Congressman a “I kNow a Fighter” shirt, a copy of the New England Journal of Medicine that featured selumetinib, and a copy of the “MEK Makes a Difference” flyer from the Children’s Tumor Foundation that features Jane (more on this in my next post).

Outside the Congressman's office

With Representative Joe Courtney!

Congressman Courtney also presented Jane with his Challenge Coin from the House of Representatives in honor of her visit!

A copy of the Affordable Care Act from 2010 hangs on the Congressman's wall <3

After our meeting, one of the Congressman’s staff members offered to take us on a personal tour of the Capitol Building!  Our guide, Brendan, gave us an entertaining, informed, and often Hamilton-themed (Jane, being a fan of the musical, looked for Alexander Hamilton in every statue and painting we saw) inside view of the Capitol.  He took us through the House office buildings, into the underground tunnel that connects them to the Capitol Building itself, and into the Visitor’s Center.  We saw Emancipation Hall, the Crypt beneath the Rotunda, the Old Supreme Court Chamber, the Old Senate Chamber, the National Statuary Hall, and the Rotunda itself. 

The Statue of Freedom, a replica of the one that sits atop the Capitol Building

A statue of Helen Keller in Emancipation Hall

We spent the most time in the Rotunda, with The Apotheosis of Washington fresco in the oculus of the dome, and with its huge scenes from the American Revolution, painted by John Trumbull.

Declaration of Independence

Jane and Brendan admire the Surrender of General Burgoyne

Alexander Hamilton!

General George Washington Resigning his Commission

Surrender of Lord Cornwallis behind the Portrait Monument, a statue of three pioneering suffragists

While we were in the Rotunda, Brendan noticed that the Capitol Police seemed to be ushering tourists to one side of the room, which made him suspect that someone notable was about to arrive.  He thought maybe House Minority Leader Nancy Pelosi (Yay!) or House Speaker Paul Ryan (Boo!)  We hung around hoping to catch a glimpse of whomever it was.  Jane started to get restless, asking, "Can we go now?" but I asked her to be patient for a few more minutes.  One of the police officers leaned over to us and whispered, "I'll let you know: it's the Vice President."  At this point Jane asked, "Can we really go now?"  (That's my girl!)  But I had the morbid urge to see him in person, and vowed if he came to shake our hands that I would let him know the reason we were in DC and ask him to support the NIH and reasonable healthcare in general.  As it turned out, he shook hands with folks on the opposite side of the barrier from us, so I didn't have the chance to speak my mind.

Jane's silent protest of the interruption by the Vice President

The most exciting part of our tour (I thought) was visiting the House Gallery where we watched the House of Representatives voting on a bill.  It was House Resolution 1679, “FEMA Accountability, Modernization and Transparency Act of 2017”.  Not the most gripping of legislations, it passed 419-0.  Still, I was enthralled.  We could see our super Representatives from Connecticut:  Joe Courtney, John Larson, Elizabeth Esty, Rosa DeLauro, and Jim Himes.  I think I squealed like a schoolgirl when Brendan pointed out Maxine Waters!  I confessed that, at this stage in my life, I no longer follow Hollywood celebrities, but I get excited to see famous politicians :)

With our tour done we said goodbye to Brendan and made our way back to the Metro, back to NIH, where we caught a shuttle to the airport and flew home.  We slept well that evening, thinking of our good news from NIH and our exciting time on Capitol Hill.