Better News

 Running Update:

12/18/2020	Run	5.0 mi	49:03
12/20/2020	Run	13.2 mi	2:12:40
12/21/2020	Run	4.4 mi	42:52
12/23/2020	Run	5.0 mi	47:53
12/24/2020	Run	5.0 mi	47:30

Every year for the past seven I’ve run 1000 miles per year.  This year I lost several weeks with a broken heel, but since I swam almost 60 miles this year, I figure I only have to make it to 940 miles running to keep the streak going.  I’m at 923.3 miles as of today—only 17.1 to go before the end of the year.  Barring any surprises I should make it!

 

Jane Update:

Two days before Christmas Jane had a PET scan to further evaluate her abdominal tumor, which had grown more than expected in the past 6 months.

We arrived at the hospital just after 9 am.  Jane had been instructed not to eat or drink anything the morning of the test.  Once we were in an exam room, the first thing that happened was Jane had a radioactive tracer infused into a vein in her arm.  The tracer is a chemical similar to glucose (a type of sugar) with a radioactive bit attached.  Since tumor and cancer cells are more active than most cells, they use up more glucose than regular cells (in medical terms, they are more “metabolically active”).  Tumor and cancer cells will also take up more of the tracer than regular cells, so by looking for areas of the body where the tracer is concentrated, a PET scan can identify cancers.

Jane's radioactive now, just like Spider-Man

Jane and I joked that the radioactivity made her like Spider-Man.  The technologist tending to Jane said she couldn’t promise that Jane would develop any superpowers, but we agreed that she does have a certain glow about her :)

After the infusion we had to wait an hour for the tracer to be absorbed by her body.  During that time Jane was given a large cup of oral contrast to drink (to help the radiologists see her intestines).  Since the contrast is not particularly palatable, the technologist said she mixes in some Crystal Light powder to make it taste better!  Jane was allowed to choose from various flavors and picked Peach Tea.

Finally came the scan, which itself took about a half hour.  The PET scanner is not noisy like an MRI, so it was a bit more peaceful.

 

Puppy by her side, as always

While we haven’t yet been able to speak with NIH regarding the results, we were given the preliminary report.  All of Jane’s tumors lit up, as expected, since most tumors are somewhat metabolically active.  NIH had said they would be concerned if the metabolic activity (“SUV”) of Jane’s abdominal tumor was a value over 5.  (Normal is zero.)  I am happy to say the maximum SUV was 3.8!

So now we wait for the interpretation from NIH.  This scan was to determine if Jane needed surgery ASAP because of a malignancy, which thankfully does not yet appear to be the case.  We will be meeting (virtually) with the NIH team neurosurgeon and urologist in early January to discuss timing of the surgery.  Ideally, we would plan surgery during a school break in either the spring or summer, but also have to take into account the status of the pandemic.  The tumor is pressing on her kidney and the bigger it gets the more complicated the surgery will be.  (If it hadn’t been for the pandemic, Jane would have had this darn thing removed 6 months ago!) 

 

NF Update:

While this week brought good news for our family, it was not so for another NF family.

Jeff Hanson was a brilliant artist, philanthropist, and NF Hero. I’ve sent many of you notecards with his brightly colored artwork.  He passed away this week due to complications of neurofibromatosis. 

NF is Not Fair.

Here are some links to learn more about Jeff and his art:

www.ctf.org/news/remembering-jeff-hanson

jeffhansonart.com

More Uplifting Stories While We Wait

 Running Update:

12/7/2020	Run	5.0 mi	46:46
12/9/2020	Run	5.0 mi	47:37
12/11/2020	Run	5.0 mi	49:03
12/13/2020	Run	10.3 mi	1:43:24
12/14/2020	Run	5.0 mi	48:38
12/16/2020	Run	7.6 mi	1:15:01

Tonight I was honored to attend the virtual NF Endurance Team End of Year Celebration & Awards Ceremony and was recognized as the CTF Top Fundraiser in 2020 for the NYC Marathon!  Thank you to everyone who donated to our campaign <3  

You can always donate at www.KRath4Jane.com!

Jane Update:

Earlier this week Jane had a follow-up (in person!) appointment with our wonderful local Dermatologist, Dr Antaya.  The selumetinib-induced aceiform rash on her face and trunk is improved since the summer but still significant.  We are going to stay on the higher dose of doxycycline for now, but we’re going to change our topical regimen.  We had tried triamcinolone cream to her trunk 2-weeks-on, 2-weeks-off, but that didn't seem to make a big difference.  He suggested changing to pimecrolimus cream which has a similar effect to the steroid but can be used continuously, and can be used on her face.  We will see him back in 2 months.

Dr Antaya let us know that the case report he and his student had written about the regimen we used to treat Jane’s paronychia last year was published in the Journal of Pediatric Dermatology!  (Treatment of MEK inhibitor‐induced paronychia with doxycycline.)  Jane’s toes are famous :)  This is the second time Jane’s case has been published in a medical journal.  (A link to the first is here.)

Towards the end of the visit Dr Antaya told us the story of an older boy who recently came to him with a large tumor on his side.  The boy was about to undergo surgery with another doctor for what was thought to be a lymphatic malformation.  Dr Antaya set them straight and diagnosed it as a plexiform neurofibroma!  The surgery (which would have been dangerous on a plexiform neurofibroma) was canceled and child is now being seen by an NF specialist. He will be treated with selumetinib, which, as Dr Antaya put it, is thanks to Jane and the other children who participated in the clinical trial which led to FDA approval!  She's helped make a difference in others' lives <3

NF Update:

The Children’s Tumour Foundation Australia recently posted an awesome explainer about different tumor types in NF.  Check it out.

The text accompanying the post read:

It can be challenging trying to understand all of the different tumour types and how any of them may impact your health.

There are many different types of tumours seen in NF1, but what does it mean to have a neurofibroma versus a plexiform neurofibroma or a schwannoma? This image provides a visual representation of their differences.

Schwannomas can be removed without disturbing nerves, whereas the removal of neurofibromas will cause damage to the nerve on which it is wrapped around. A plexiform neurofibroma envelopes multiple nerves meaning they can be difficult to remove completely, which is why doctors often speak of "debulking" a plexiform neurofibroma with surgery.

If you would like to learn more about the different tumour types, symptoms and treatment options, head to our website at www.ctf.org.au

#conquernf

Henri Update:

We had the first major snowstorm of the season last night and ended up with over a foot of snow today!  It was Henri’s first big snow.  He was hesitant to explore it at first, but ended up having a blast, and even playfully chased our young neighbor as he sledded down the hill in our backyard.

An Uplifting Story and Some Upsetting News

 

An example of Lisch nodules

This past week Jane finished up the last of her restaging appointments: ophthalmology and a virtual meeting with our team at NIH.

Jane’s ophthalmologist, Dr Howard, works at a teaching hospital, which means she is usually accompanied by several trainees.  Before Dr Howard joined us in the exam room, we were first greeted by an ophthalmology resident and a visiting fellow from the pediatric emergency department who was observing.  The resident began the exam, and at one point he explained to the fellow about Lisch nodules, tiny growths that can be found on the irises of many people with NF and that can only be seen with a special camera, which he was using.  As he talked, I could see Jane getting uncomfortable because no one had ever made a big deal about her Lisch nodules before. I tried to reassure her that they were harmless, but the tension was broken when the fellow looked through the camera and she gasped, “Oh! They’re beautiful!”

Never, with all of the hundreds of evaluations she’s had in her short life, has anyone ever described any component of Jane’s NF as “beautiful”. Her comment moved me to tears. As Jane’s mother, of course, I am biased and think every part of her is beautiful, but usually our interactions with health care providers focus on the complications of her NF, not the rare beautiful things.

I decided to write a letter to the fellow and to the head of her department to praise her.  I told the story above and commented that I had no doubt the fellow was competent in her knowledge of pediatric emergency medicine, but that what stood out to me was her kind, compassionate heart.  I got this lovely letter from the department head in return:

That afternoon we had a virtual meeting with Jane’s pediatric oncology team from NIH to review all of the recent testing she has had. The good news is that her facial plexiform neurofibroma appears stable when compared to her last MRI in May.  The bad news is that her abdominal tumor, which had previously been stable, has increased in size by 30%. The rapid growth makes the team a little concerned for a possible developing malignancy.  (Neurofibromas have about a 10% chance of becoming cancerous in a person's lifetime.)  The team wants us to do a PET scan to help decide if the tumor needs to be removed immediately or if surgery can wait until the pandemic lessens and the school year is over. The PET scan is now scheduled for just before the holidays and we will meet virtually with the NIH neurosurgeon and urologist (the tumor is next to her kidney) right after the holidays.

How do you tell a child they have to have a test to check if their tumor is cancerous?

Jane is always present for our visits with our team and was listening in on this one.  I asked her later what she understood from the conversation.  She said, “So I might have to get the tumor out sooner rather than later?”  I said yes, but that there was one more test we could do to see how much sooner—whether we had to have it done immediately or whether we could still wait for a school break.  I said the test was a PET scan.  She said she’d heard of a PET scan before but didn’t know what it was.  I told her that the PET machine looked kind of like an MRI, but that it can tell which cells in the body are most active or growing fast and which cells aren’t.  The kidneys, I told her, for example, have lots of cells that are very active, so the kidneys light up on a PET scan.  Jane said, “So if the tumor lights up that means it’s growing quickly and we’ll have to have it out sooner?”  Exactly.  I told her she’ll have to have an IV for it for a type of contrast—she was OK with that.  Then I braced myself and said, “There’s one more thing.  The contrast is radioactive.” 

She broke into a wide grin!  “So you mean I’ll be radioactive??”  I smiled with relief.  “Yes, but only a teeny, tiny, bit for a short period of time.  Not enough to be harmful.”  She said, “Kind of like bananas!  Did you know that enough natural potassium is radioactive that if you ate 10 million bananas at once you’d get radiation poisoning?  Of course, you’d die of other reasons—your stomach would probably explode.”

And that’s how you tell a child they have to have a PET scan <3  Jane and I later found this graphic about radioactive bananas :)

All kidding aside, in my opinion, children should not need to have PET scans :(

Running Update:

11/23/2020	Run		3.2 mi	30:47
11/25/2020	Run		7.1 mi	1:08:45
11/26/2020	Run		5.0 mi	43:51
11/27/2020	Run		3.3 mi	31:02
11/29/2020	Run		13.2 mi	2:12:05
11/30/2020	Run		3.2 mi	30:57
12/2/2020	Run		5.0 mi	48:03
12/4/2020	Run		5.0 mi	46:18
12/6/2020	Run		5.0 mi	48:44

Still going. 

Last month Alec and I spontaneously decided to run our town’s Turkey Trot route, even though the race was virtual this year. Todd was our excellent support staff and photographer (as usual).

My little boy!

Always wearing CTF gear

With my broken heel in this summer I lost a month and a half of training so I knew I wouldn’t hit my annual 1000 miles of running this year.  But today I figured out that I swam 59.6 miles this year, so if I add running and swimming miles, 1000 is still within reach!  It would mean running another 87.4 miles before December 31—doable, but no room for slacking.

Jane Update:

Within the next week we have a rescheduled ophthalmology appointment and a follow up dermatology appointment—both of these in-person visits had been postponed due to Jane’s quarantine.  Then we’ll (virtually) meet with our team at NIH to hear their analysis of Jane’s MRI and the plan for her treatment going forward.

NF Update:

I love JRR Tolkien’s works, especially The Lord of the Rings.  I’ve read the books over and over (even The Silmarillion!)  I’ve watched the movies dozens of times.  If I were stranded on a desert island and could have only one set of books (or one set of movies) it would be The Lord of the Rings trilogy.  It has its problems—for one, too few decent female characters—but I’m willing to forgive them for the joy they bring me.  But there is one thing in the movies that make me cringe.  This guy:

Gothmog, Lieutenant of Morgul, the Orc commander at the Battle of the Pelennor Fields, as portrayed in Peter Jackson's The Return of the King.

He’s among the worst of the worst in Middle Earth, one of its most evil characters.  He leads an army of vicious creatures bent on destroying the world of humans.  But here’s the thing—he could have NF.  Look at his unilateral facial tumors that render his eye shut and his jaw asymmetrical.  His drooping cheek and mouth.  His hypertrophied, bulbous hand.  The growth on his shoulder.  He’s got plexiform neurofibromas, as I see it.  And every time I see him I wonder:  Why is “disfigured” representative of evil? 

Legolas:  Good or Evil?

Gorbag:  Good or Evil?

Saruman:  Good or Evil?
(trick question!)

The New York Times touched on this issue in a recent article “Scary Is How You Act, Not Look, Disability Advocates Tell Filmmakers” by Cara Buckley.  It was written in response to the upcoming release of the movie The Witches, based on the Roald Dahl book, and to the villains in the newest James Bond film.  The article doesn’t answer the “why”—and that discussion is beyond the scope of this blog—but it enumerates the damage that is done by perpetuating this trope. 

"For as long as there have been stages and screens, disability and disfigurement have been used as visual shorthand for evildoing — a nod to the audience that a character was a baddie to be feared. But disability rights advocates say this amounts not just to lazy storytelling but stereotyping, further marginalizing an already stigmatized community that is rarely represented onscreen."  Even when disabled characters are featured in film, “critics say disabled characters still fall too often into predictable buckets, among them the villain or the victim that provides uplift for all, which some have nicknamed ‘inspiration porn.’”  (I’ve written about this phenomenon before regarding the novels Wonder and American Dirt.)  Not only that, but many times the disabled character is played by an able-bodied actor whose appearance is altered by make-up of special effects rather than by a disabled actor. 

Having more disabled people in the entertainment industry—not just actors, but writers, directors, producers—would help combat the stereotypes and avoid casting failures.  Representation matters!