Follow-up to NIH visit

NF Update:

This week I had a follow-up conversation with Dr Andrea Gross, one of our team leaders at NIH.

Jane’s right-sided plexiform is stable—always a good result to get!  And a happy way to enter the holiday season.

A friend and mother of another child taking selumetinib for a plexiform neurofibroma recently posted online, “Friends often ask, why can’t they just surgically remove the tumor? Here is the latest radiology report to explain what would be at stake…”  She then posted the radiologist’s description of her son’s plexiform, which is a benign but locally invasive type of tumor.

I will follow suit and present part of Jane’s report:

“Again noted is a large plexiform neurofibroma involving the right side of the face including right masticator, infratemporal fossa and parotid spaces with extension into retromandibular space and involving the floor of mouth.”

Translation: it involves pretty much her entire mandibular nerve (a branch of the largest cranial nerve); her external carotid artery, facial nerve, and parotid gland (in the “parotid space’); plus other important nerves and blood vessels entering and leaving her skull (in the ‘infratemporal fossa’). 

Not something you want to cut into.  Thus, we carry on with selumetinib!

Gray’s Anatomy, Plate 784

As I mentioned in my last post, the full-body MRI also showed an irregularity in one of the bones of Jane’s right shoulder.  Our team thinks it is a “benign fibrous cortical defect” or “non-ossifying fibroma”, both of which are associated with NF, and neither of which would require any treatment.  However, our team would like us to get an x-ray of Jane’s shoulder at home sometime in the next few weeks/months just to fully evaluate.  As you all have witnessed, there is always something to chase with NF.

 

Running Update:

This year marks 10 years of running an average of 1000 miles a year for NF!

My yearly running totals:

2013:  1010.4 mi
2014:  1010.2 mi

2015:  1010.2 mi
2016:  1056.9 mi
2017:  1084.5 mi
2018:  966 mi
2019:  1002.2 mi
2020:  950.1 mi
2021:  1140.1 mi
2022:  1056.7 mi*

*I’d like to add another run or two before we head to Canada at the end of the year, but I’ve been down for several days with some sort of bug (not COVID) so I’m not sure if I’ll be able to.

I’m also almost at 10 years of blogging about NF, but I’ll talk about that more next year!

 

Jane and Family Update:

Helen and Alec got home from college this week!  It’s wonderful to be all together again <3

We’ll head to Canada after Christmas and will celebrate the New Year there, as usual.

I hope everyone has a healthy and restful holiday! 

December 2022 NIH visit

December 2022 NIH Update:

We visit the NIH for check-ups every 24 weeks (every sixth 4-week cycle).  Because the interval is a little less than 6 months, visits happen a month earlier from one year to the next.  Add to that some changes in our visit schedule due to surgeries and breaks from the study medication due to side effects, we haven’t ended up at NIH around the holidays since 2015.  Things were a lot different then!  Jane was a lot shorter, I was a lot less gray, and even though the NIH campus has always been strict about infection control and security, there were a lot fewer restrictions on getting out and about at NIH in 2015.

Jane helping to decorate the tree in the Ophthalmology Department in 2015

Same tree, same skirt, same department, 2022!

One event we were particularly looking forward to was the annual NIH Gingerbread House competition, in which different departments at the clinical center go to town building elaborate gingerbread houses, castles, robots, games, and holiday scenes.  All are put on display in the main atrium to be enjoyed, and ultimately judged, by the public.  Jane and I were disappointed to learn that the competition has not been held since the pandemic started.

COVID has particularly impacted the experience at the Children’s Inn, where they have to be very careful to protect some really sick kids who are especially vulnerable to the virus.  Despite requiring each resident to have two negative COVID tests (one within 72 hours before our trip and another once we arrive at the Inn) there still are no groups meals or big group activities like the Inn has had in the past. (Though there were astronauts—see below.)  I miss getting to meet the other Inn families from around the world and talking with them about their rare and complex medical conditions.  It made us feel not so alone.

Our schedule

Our first day at the Clinical Center started at 7am with a 90 minute full body MRI and dedicated face MRI.  We both fell asleep at points—Jane in the MRI scanner and me in a non-metallic chair at her side.  I guess we were tired!  The day also included blood work, EKG, physical exams, an eye exam, and a meeting with our team.  We headed back to our room after 7-1/2 hours at the Clinical Center.  When we got to the Inn, we discovered some astronauts from the International Space Station (Expedition 66) were giving a presentation!  Jane wasn’t up for joining it—her last appointment of the day had been with ophthalmology, so her eyes were dilated and she couldn’t see anything—but they did save some swag for her :)

Autographed photos!

A model spacecraft to build!

My favorite :)

The second day at the Clinical Center also started at 7am with an MRI, this time a brain MRI which required IV contrast.  (No sleeping this time--I had just downed a large coffee!)  After the hour-long scan, we went directly to her echocardiogram and managed to be out of the Clinical Center by 10am.  We had a seamless trip to the airport, and after quick flight we arrived home before dinner.

Todd and Henri had been home alone during our trip.  That hasn’t ever happened before!  The last time Jane and I were at NIH, Alec hadn’t yet left for college.  Henri did not understand :(  Todd sent us several pictures of Henri watching the front door, waiting for us to come home.

Wednesday night vigil

Thursday morning vigil

We got a quick report from our team before we left NIH on Friday.  So far we know that Jane’s blood work is mostly normal, and her eyes and heart are normal.  We don’t have a formal reading on the MRIs yet, but a quick glance at the scans by our team seems to show:

1- No major change in her facial plexiform neurofibroma.

2- No recurrence of the abdominal tumor that was removed last March.

3- No sign of the new psoas muscle tumor that had first been spotted in June of this year! Yay!

4- A new bony abnormality in the right humerus in her shoulder.  This particular finding is apparently not unusual in NF and probably has no consequences.

Hopefully we’ll have final results before the holiday and know the plan for the next six months.  I’ll update when we do.