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.

November 2022

Running Update:

October                            54.6 mi               9:16:41

November (to date)          42.0 mi               6:51:49

2022 (to date)                  1009.0 mi           170:46:43

I’ve not done a lot of running since Chicago.  I had to take some time off—I don’t recover as quickly from these races as I used to!  Specifically, one of my knees was feeling a little abused, so I did not run the Philadelphia Half Marathon last weekend as originally planned.  (I deferred to next year.)

BUT I did hit 1000 miles of running so far this year AND I ran the Madison, CT Turkey Trot on Thanksgiving with Alec!

I hope everyone is enjoying the holiday weekend.

 

NF Update:

I’m a little late for this year, but felt it was still helpful to post.  Last month Face Equality International (https://faceequalityinternational.org/) spent the days leading up to Halloween to remind people that facial disfigurement is not evil or scary (I’ve written about this), and so should not be used in costumes as such.  Here is a nice recap by Elizabeth Russo:

In the vein of face equality, I was happy to see that the producers of the stage version of the novel Wonder are planning to cast a person with a facial difference as Augie, the main character.  (This is something the film version did not do, to my annoyance.)

Jane Update:

Jane and I return to NIH for her semi-annual check-up in a couple of weeks.

We recently attempted to count how many MRIs Jane has had in her lifetime, and it’s not easy to do.

If multiple MRIs done in one session/visit count as ONE, Jane has had:

24 at NIH,

2 at Indiana University, and

8 at Yale New-Haven, for a

TOTAL 34 MRI sessions.

But almost every session includes two or more MRIs.  For example, on the first day of our upcoming NIH visit, Jane has an hour-long full body MRI followed by a more dedicated MRI of her face, sinus, ear, nose, and throat due to the location of her biggest tumor.  The next morning she has a dedicated brain MRI.  By our definition above, all that counts as one MRI session.  So Jane’s probably had over 50 individual MRIs in her 15 year life.

I’ll report back on our trip next month.

The 2022 Chicago Marathon

I was more excited about this marathon than I have been for any other in a while.  I think because the stars seem to be aligned for a good race:  I lucked out and managed to get a hotel right near the start/finish, so no onerous trekking before and after the race.  The course was very flat and neatly divided into three manageable sections: north, west, and south.  And the weather was perfect!  In the final pre-race email from the Chicago Marathon they said, "According to the advance local weather forecast, the weather on race day is projected to be partly to mostly sunny, with low temperatures in the low-40s (degrees F) and high temperatures in the low-60s (degrees F).  Based on this forecast, the Event Alert Status level is Green, which suggests ideal conditions for marathon running."

And it was!

Course Map

I could see the finish area from my hotel room!

At the Expo

Anyone could write down a reason for running and add it to the wall.

View of the city

The Bean!

The Riverwalk

Pre-race gear shot

The Chicago Marathon is the second only to New York as the largest marathon in the US.  40,000 people participated, but it didn’t ever seem like that many.  Most of the course was on relatively narrow, tree-lined streets, so it didn’t even feel like a big city, let alone a big race.  The only way the Chicago Marathon felt big was the number of spectators--they lined every single step of the course, and they were loud!   There were lots of signs, some with the usual chestnuts:

·       “You’re running better than the government!”  (Alternate: “…the metro”)

·       Pain is temporary, pride is forever.

·       Worst parade ever!

·       Your feet hurt ‘cause you’re kicking butt!

·       On a scale of 1-10 you’re a 26.2!

But I saw a few creative signs that were new to me:

"I'll get you into Boston" (with a photo of Lori Loughlin)

·       “Due to inflation you’ll run 27 miles”

·       Several Wordle-themed signs eg “RACES, ROADS, WANTS, MEDAL”

·       A couple of Lord of the Rings-themed ones:  “Fly you fools!” and “A day may come when you cannot run, but it is not this day.”

Some of our NF Endurance Team!

In Chinatown

Photo by our NF Endurance Team leader, Lydia, who was waiting for us at mile 13!

Todd was able to follow me from afar on the Chicago Marathon app

He took several screen shots as I approached the finish line :)

I felt strong until the last few miles, but I finished with my best marathon time since 2017!  And I was 605th out of 1662 women in my age group :)

At the finish line a guy proposed to a girl right in front of me.  He had the ring with him and everything.  Apparently she said yes because they kissed afterwards.  (Risky!)

Nicholas Kristoff and family were there, too!

One more special treat this Chicago weekend—the day after the marathon I got to have breakfast with college friend, Lolan, and his family! So wonderful to reconnect with old friends.

With Lolan's nephew, Samuel

And with Lolan's mom!

I brought Samuel a Chicago Marathon shirt <3

Next: the Philadelphia Half in November! I'm giving myself a couple weeks break first, but I'm still motivated to keep running to #endNF <3 THANK YOU to all who have donated to our campaign so far this year!!

My Dumb Runner calendar has a perfect message for October

The medal rack Todd made for me several years ago.  It's getting pretty full.