October 2016 NIH Update:

Jane and I arrived in Bethesda at the National Institutes of Health on Sunday afternoon.  It was one of our smoother trips.  After six years of traveling to and from NIH for Jane’s treatment, we almost have the trip down pat.  The best way to get to NIH from the airport is via the NIH shuttle; however, this only runs on a certain schedule, on certain days.  If we miss the shuttle, the next best option is to catch a cab at the airport and have it drop us off on West Cedar Lane just outside the heavily-guarded NIH campus at a side entrance by the Children's Inn.  Since we have long-term visitor passes, we can breeze past the security gate there and walk about 100 yards up to the Inn.  In the past I would have the cabs bring us through the main entrance--a process that requires the cab driver to exit the cab, have his vehicle wiped down for dangerous substances, and obtain a temporary pass at the security gate.  This always took forever, and never went over well with the cabbies.  It took me this long to learn the short cut!

For our next trip, I'll be able to use my newly-obtained “Known Traveler Number” from the TSA so we can by-pass the long lines at airport security, and the trip will be positively seamless!

Jane on one of the play structures outside the Children's Inn at NIH

Once at the Inn we took our time settling in.  I was looking forward to connecting with another NF Mom, Kelly, who represents Utah on the Children's Tumor Foundation Volunteer Leadership Council.  I had met Kelly online several years before, but we'd never met in person.   It turns out that her son is in the same clinical trial as Jane, and that Jane and I would overlap with Kelly and her family for a day during our return visit to NIH.

We finally met Kelly, her son, Travis, and the rest of their family at the Inn’s Family Dinner that night.  It helps me so much to be able to talk to and share with other families who are going through the same ordeal as us!  After dinner Jane and Travis together enjoyed petting and walking the therapy dogs that were visiting the Inn, and then had a fierce (and giggly) game of air hockey. Travis is Jane's same age, and has siblings the same ages as Jane's.  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.

Jane walking Lucy, a therapy dog

Jane with Travis and one of his brothers

Air hockey!

Just a couple of NF Heroes :)

Our first day at the Clinical Center was a full one.

All went relatively well.  Jane even had time between two of her appointments to pet a Guinea pig in the main playroom!

Photography is my favorite appointment

Our least favorite, but nonetheless important, appointment is at the Eye Clinic.  The session is usually very long and the series of tests are challenging (not to mention Jane hates having her eyes dilated), but we usually come away reassured.  This time the ophthalmologist noted for the first time that Jane's left eye protrudes a bit more that the right (a condition called proptosis).  The difference between Jane’s eyes is only a few millimeters, and the doctor said it was possible her eyes had always been that way, but just never previously documented.  Jane also has a known deformity of one of the bones forming her eye socket (sphenoid wing dysplasia, a complication of NF) which could be responsible.

Despite this, the eye doctor was concerned because proptosis can sometimes be caused by a tumor behind the eye.  One such tumor, an optic glioma, is a tumor of the optic nerve, and is known to be associated with NF.  Early on in our experience with NF, Jane was thought to have optic gliomas in both eyes, but her optic nerves, while slightly thicker than normal, have stayed stable for many years.  They've been so stable that our care team at NIH has actually skipped the MRI of Jane's orbits over the past year in the interest of keeping her MRIs a tolerable length (since she had stopped having sedated scans).  However, with the new finding of proptosis, it was felt Jane's orbits did need to be scanned again.

We were scheduled for a non-sedated MRI of Jane's face, ear, nose, throat, and sinuses (where most her tumor resides) that evening, but despite all their efforts, our care team could not get an MRI of Jane’s orbits added to that session.  This meant a second MRI session the following morning, and required contrast (which requires an IV).  In other words, Jane would have her 20th and 21st MRI during the same NIH visit. 

Jane did not take the news well.  She had cooperated so well with her MRI that evening.  Staying completely still for that long is so hard when you're nine!  It wasn't fair to have to do it again!

A spooky dinner: mummy dogs, zombie toes, Jack-o'-lanterns, and blood

For dessert: chocolate pudding "dirt" with gummy worms!

We didn't enjoy our Family Dinner that night as much as we might have (“mummy” dogs, “zombie toes”, and “blood”) but Jane’s mood improved tremendously when after dinner she met another little girl playing hide and seek with her mother.  Jane introduced herself, and the two were immediately off playing in all sorts of places around the Inn.  The little girl was Lois, she was Jane's age as well, and had traveled all the way to NIH from London! Lois has a genetic condition which causes tumor to grow in her endocrine glands, and, by this young age, had already had her thyroid removed and had a tracheostomy. After hiding and seeking for a while, Lois and Jane had the opportunity to paint pumpkins!

Jane with Lois in the craft room

The following morning, we were back at clinic bright and early, before any other patients, to have Jane's IV placed.  Jane was still quite upset about having yet another procedure, and, alone with her in the waiting room, I was running out of ways to console her—she rebuffed all of my attempts.  Then, by a stroke of good fortune, Lois and her mother arrived at clinic!  It was like the sun breaking through the clouds--Jane's tears stopped flowing and she greeted Lois with a watery smile.  The girls ended up playing a quick round of bowling before Jane and I had to leave for Radiology.  I thanked Lois for her smile, and thanked her mother for being there for us right when we needed her.

Jane’s MRI ended up being delayed for over two hours (which did not improve our mood).  Despite this, Jane sat still through 67-plus minutes of scan time, longer than she’s ever done without sedation.  Due to the delay and the length of the scan, we were behind schedule to get to the airport.  After leaving Radiology we had to dash to collect our belongings at the Inn and catch our cab (but not before allowing Jane to select a prize from the prize chest in clinic—the promise of which got us through the final minutes of her MRI!)

While on our way home, Jane and I were discussing the long and unexpected MRI.  I told her I was proud of her, and that she was a strong and brave little girl.  I told her, “I don’t know any other kids who can do what you do.”  Immediately, Jane responded, “I do.  Travis.  And Emma.  And all the other kids with NF!”  This is why we call them NF Heroes!

Driving home from the airport that night, we happened to catch up to the bus that was bringing Jane’s sister, Helen, home from her high school soccer game!  Instead of turning for our house, we followed Helen's bus all the way to the school parking lot!  Jane jumped out of the car and ran over to give her big sister a hug as she got off the bus!

We won’t have results on Jane’s MRI for another day or so, but will update you all as soon as we have them.  Thank you all for your love and support!

Addendum:

This just in!!

Email from NIH:

The MRI that J did was excellent, she did a very good job.  J’s plot and table are attached.  Her facial tumor is extremely stable compared to last time and currently has decreased by 29% compared to baseline.  Very good news! 

The orbit MRI was reviewed by our neuro-radiologist and our team and we do not see any optic nerve gliomas, this is very reassuring.

Whew!  I was fearing new growth.  I'm content with "stable" this time. <3 <3 <3