Braces off (!) and more October updates

Running Update:

Less than a month until the Philadelphia Half Marathon.  But the good news is that last week I ran on the road for the first time in two months!

Since August 11, because of my knee injury, I’ve only exercising by elliptical and swimming.  But on October 11 I ran five miles on the road!  It was also two weeks to the day after I had come down with COVID again (before my updated vaccine had a chance to kick in.)  My lungs weren’t quite 100%, but I figured that would keep me from overdoing it with my knee :)  I was slow as heck and wheezy and every joint hurt, but I was SO STUPIDLY HAPPY to be running again!!  Afterwards, I even went on the Philadelphia Marathon website and briefly thought, “Maybe I could actually do the full marathon instead of the half…”

I know some people think it’s crazy, but I truly do love running <3

So now I need you all to do your part:  Please donate to our Children’s Tumor Foundation campaign!  Help us to #endNF!

www.KRath4Jane.com

 

NF Update:

People living with NF often suffer not only from the physical effects of their neurofibromas, but also from the effect of the tumors on their appearance.  This is particularly hard if someone, like Jane, has neurofibromas on their face.  People with facial differences can experience harassment, stigma and prejudice because of how they look.  Other people with facial differences can include those with clefts, craniofacial conditions, and burn survivors.

Enter Face Equality International.

From their website:

The mission of Face Equality International (FEI) is to mobilise the many groups and organisations, big and small, national and international, which support and represent people with facial differences and disfigurements and thereby to create the critical mass and solidarity needed to gain global attention for the campaign for face equality.

 “Together we can create a world where anyone with a disfigurement is free to be unapologetically themselves. In order to celebrate what makes us ‘different’, first we must recognise that it is society that must change, not us. It is the limiting perceptions placed on faces and bodies that do not conform to the ‘norm’ that we must change.”

Phyllida Swift, CEO of FEI

It was around this time last year that I shared Face Equality International’s excellent post about Halloween, reminding people that facial disfigurement is not evil or scary (I’ve also written about this), and so should not be used in costumes as such.

This year I thought I’d share FEI’s new petition calling upon the entertainment industry to include accurate, authentic representation of people with disfigurements and visible differences, to prevent further marginalization of the community. Take a look—it is well written and spot on: www.change.org/p/tell-hollywood-visible-difference-doesn-t-equal-evil

 

Jane Update:

This week Jane got her braces off for good!  It’s been three long years—time to celebrate by chewing gum and eating popcorn again!

While it’s a relief for Jane to get the braces off from a comfort standpoint, it also makes a HUGE difference for our upcoming return to NIH and any future MRIs.  Every time Jane would have an MRI she’d have to have her braces wire and any metal brackets removed a few days before the scan and then replaced afterward (even though we opted for ceramic brackets for this very reason, the anchoring brackets on her molars had to be metal).  It was very inconvenient, and prolonged the amount of time she had to wear the braces.  But that’s in the past now!

Though we’re glad to be done with braces, we do love our orthodontist.  After one of Jane’s recent appointments he came to me in the waiting room to tell me:

Jane just said something that changed my life.  She asked, “Why do you ask people to ‘bite down’?  Shouldn’t you ask them to ‘bite up’?”  She’s changed my whole world view.  I’ll never say “bite down” again.

Typical Jane—changing people’s whole world view :)

Before

After!

The 2023 Dave Parcells Madison Sprint Triathlon

Yesterday was the 2023 Dave Parcells Madison Sprint Triathlon hosted by a group in our hometown, the Madison Jaycees.  The Madison Jaycees is a civic organization that raises money to support local charities and families in our community.  They host several events throughout the year, including a ThanksgivingTurkey Trot (!) and an annual sprint triathlon.  This year was the 37th annual Madison Triathlon, named for a former director, Dave Parcells.  It is a very family-friendly and novice-friendly event, with individuals, teams, first-timers and para-athletes all encouraged to participate.  There’s even a Kids "Try" category designed for 11-16 year old children to participate with an adult. The race includes a .5 mile swim, a 13 mile bike and a 3 mile run. 

About a week ago, my friend Bridget posted that her triathlon relay team had lost its swimmer (to gout!!) and was looking for someone to replace him.  I was already scheduled to work the day of the race (at HAVEN, a student-run clinic at Yale), but since the race started at 7am and I didn’t have to be at work until 9am, I volunteered!

I had done the Madison Sprint Triathlon once before, in 2021 and while I survived it, it was definitely not my best athletic performance.  My hope was to beat my swim time.  I brought along a change of clothes for work and planned to shower at the beach and then drive straight to work after my leg of the race.  Our team name: Well, We Will Tri!

R to L: Sue (our cyclist), me, and Bridget (our runner)

Remember how I said how serene I found it to swim laps at the Lake of the Woods in Canada?  Well,

Solo lake swimming = Serene

Racing in the ocean with 250 other swimmers = Not Serene

It wasn’t my aforementioned fear of sea creatures that made me nervous.  Rather, it was the swimming equivalent of being trampled!  But we got it done!

I parked across a field from the beach so I'd be able to get to work after my leg.
It was foggy at 6am!

The Madison Surf Club was the heart of the event

My mom even showed up to cheer!

The swim course!!  Half a mile around the orange buoys.

They make you write your age on your calf :/

Out of the water!

After the race, Sue with her husband and Bridget with her 15 year old son,
who finished FIRST in his age group in his first ever triathlon!  Amazing!  

Post-swim shower before work :)

Made it to clinic in plenty of time.  Here is my student team!

Our relay team came in second place in the all-female team category!  …OK, out of two teams :)

Thanks to Bridget and her family for including me in the fun!

~ ~ ~

I'm still hoping to run the Philadelphia Half Marathon in November.  If you haven't yet donated to our fall campaign for the Children's Tumor Foundation, please do!

www.KRath4Jane.com

Labor Day Weekend

Running Update:

August 2023
Run                     66.9 mi               10:46:10
Swim                   10.7 mi               2:52:00

This weekend was to be the start of our fall racing season and fund-raising season for the Children’s Tumor Foundation.  The deeply disappointing news is, because of a knee injury, I won’t be running for the next month or so.  That means no New Haven Half Marathon today—the first New Haven Road Race I’ve missed in 14 years.  The first New Haven Road Race I’ve missed since Jane was two.

Even more upsetting—no Berlin Marathon this year. 

My right knee has been giving me trouble ever since I started training for Berlin this summer (for reasons I can't understand.  It was fine for the Flying Pig Marathon in May.)  After a couple of trips to an orthopedic office, physical therapy, and a cortisone shot didn’t help, I got an MRI and learned that, in addition to the expected age-related arthritis, I have a bad meniscus that isn’t amenable to surgical repair.  If I want to continue running in the long term, the orthopedist’s advice was to cut back on my running mileage and to cross train as much as possible.  In his words, “Maybe instead of running two marathons a year, you just run one.  Or just run half marathons or 10Ks.”  Not words that any endurance runner wants to hear!  With the orthopedist’s permission, I initially kept training for Berlin.  However, while we were in Canada visiting Todd’s family in August, I was in enough pain during my long runs that I realized my knee would never hold up for a marathon. 

All summer I'd been thinking I could just push through, given that I'd already got so much invested in this event—I'd already booked time off of work, we'd already bought plane tickets, I'd been fundraising, etc—but I finally made the hard decision to cancel my next few races to give my knee a rest.

I know this is a minor issue compared with the problems some people have (like those with NF!) but I confess I am devastated.  I said as much to our NF Endurance captain, Lydia Vanderloo, and she responded:

“I think you actually captured it in your comment.  Although with endurance fundraising we typically position our fundraising around a specific goal—your 20th marathon at Flying Pig, a big celebration in Berlin, etc—sometimes things happen that are beyond our control (injuries, pandemics, etc).  But that does not mitigate the need to fundraise to end NF.  And the challenges you're facing with your knee are in some ways a parallel to the challenges faced by those living with NF, like Jane.  Who knew her latest challenge would be to remove a tumor from her tongue?  But she managed it (with her family's support, I'm sure!)  You are managing your own challenge right now, uncertain as to when you'll be able to run another race, but with the hopeful target of the Philly Half looming in the distance.  I hope this makes sense.  Your donors are generous because you ask, they care about you and Jane, and what you're doing is inspiring.  None of that has changed with this injury or your pulling out of Berlin.”

This is why Lydia is our team captain! <3

While we were still in Canada, I took advantage of being on the Lake of the Woods and threw myself into swimming.  Swimming at the lake is very serene; peaceful even when the water is choppy.  You’re gliding though cool black waters, surrounded by tall poplars with fluttering green leaves and big blue skies.  From the cabin, the best landmark for swimming is an island in the bay—one lap to its shore and back is about 500 yards.  I’ve swum to the island for years, but this summer I enjoyed it more than I can remember.  I’m finally confident in my spotting, and have managed my irrational fear of encountering a sharp-toothed lake creature.  I enjoyed it so much that when we got back to Connecticut, I searched the web for nearby lakes that allow open water lap swimming.  (Yes, living close to Long Island Sound, I could swim in the ocean.  But remember, I’ve only just managed my irrational fear of sharp-toothed lake creatures—not my less-irrational fear of sharp-toothed ocean creatures.)

A view of the island from our dock in Echo Bay

Just when I was starting to lament that, with the summer ending, I wouldn’t be doing much more open water swimming this year, a friend posted that her sprint triathlon relay team had lost its swimmer and was looking for someone to replace him.  I volunteered!  (“When one door closes…”)

So in the end, I won’t be running a race for a few months, but I will be swimming a race!  (Thank you, Bridget!)  Watch for us in the Madison Sprint Triathlon on Saturday, September 9!

NF Update:

That means we still need your donations to the Children’s Tumor Foundation for our fall campaign!

As you know, this summer Jane underwent another surgery due to neurofibromatosis to remove a tumor from her mouth.  I am happy to say she has recovered fully!  While we have come a long way with NF treatments—like the selumetinib that Jane continues to take, the first drug ever approved for treatment of NF—you can see we still have far to go.  Please help us to #endNF!  Donate here:

www.KRath4Jane.com

 

Jane Update:

Jane and her siblings are all back to school in this past week.  Wishing everyone a very successful school year!

Alec was first back to school, starting his second year at Lafayette College in Easton, PA

Alec showed us one of his favorite places to run, a park right on the Delaware River

Henri liked Alec's raised bed

Jane was next back to school, starting her junior year at Daniel Hand High School.
(She's not happy to be waking before sunrise again!)

Helen was the last to start back to school.  She was welcomed to her dorm with this nametag :)

Starting her SENIOR year at Mount Holyoke College in South Hadley, MA

Another Surgery

Jane Update:

Last week Jane had her second surgery due to NF.  Readers of this blog will remember that our team of doctors wasn’t sure what exactly the mass in Jane’s mouth was—an enlargement of a salivary gland due to her large plexiform neurofibroma, or the neurofibroma itself.  All her pre-op exams and testing, including special MRI sequences, seemed to point to the former.  But—surprise!—it was an extension of that dang plexiform into her tongue after all.  Our stellar surgeon was able to remove the tumor without removing the salivary gland, and Jane is making a quick recovery.  She had a few days of just liquids and soft foods (milkshakes!) but is now back to a regular diet.  Thankfully, the pathology report showed no signs of malignancy.

The fact that Jane still has to have surgeries to remove neurofibromas despite taking the most effective  chemotherapeutic drug available for plexiform neurofibromas daily for the past nine years means we clearly still have more work to do to #endNF.

In good spirits before surgery

Recovering at home with the ultimate therapy dog, Henri <3

A pre-surgery and post-surgery look at Jane's mouth.  (Not for the squeamish!)

Can you believe our stoic girl has been living with this in her mouth for six months?? 

About one week post-op.  Hooray!

Running Update:

July running to date:                     128.9 mi             21:02:38
July swimming to date:                11.3 mi               8:41:00
 
Just a little over a month until our first race of the season to benefit the Children’s Tumor Foundation!  The New Haven Half Marathon is Labor Day, followed just three weeks later by the Berlin Marathon in Germany!  Please consider sponsoring us!  Here is the link to donate:

www.KRath4Jane.com

NIH Update

Running Update:

June running to date:                   68.1 mi               11:22:25
June swimming to date:               5.7 mi                  4:17:00

Happy summer, everyone!  I took a short break from running after the Flying Pig Marathon last month, but now training for the Berlin Marathon in September is already in full swing.  We’ve had beautiful late spring weather here in Connecticut (with the exception of those few smoke filled days courtesy of Quebec).  It’s been perfect for running and being outside in general.

NIH/NF Update:

I delayed in posting my report from our trip to NIH last month until we had all our test results and a formulated plan.  We are still waiting for one more piece of information (which I’ll describe), but I thought I’d finally catch you all up with what we have so far.

As usual, we had an action-packed day at the NIH Clinical Center.  This was our schedule:

8:00am- Labs and urine test- Phlebotomy 1st Floor

8:30am arrival for 9:00am scan- MRI (Right Humerus), Radiology, 1st Floor

9:45am- MRI (Face, sinus, ENT), Radiology 1st Floor

11:00am- Vital signs (3 hts, 3 wts) -Pediatric Clinic 1st Floor

11:15am- Consult with Donna Barnett- Pediatric Clinic 1st Floor

12:00pm- Meet with Maggie RN (pill count/diaries)- Pediatric Clinic 1st Floor

12:15pm- Meet with Dr Margarita Raygada, Genetics- Pediatric Clinic 1st Floor

1:30pm- Physical exam with Anne Dufek- Pediatric Clinic, 1st Floor

2:30pm- EKG, Near Phlebotomy 1st Floor

3:00pm- Echo, 5NE, 5th Floor

I should point out that on this day Jane entered the MRI scanner at 9am and didn’t come out until 10:30am—90 minutes of continuous scan time!  #rockstar

Jane’s lab work was all stable, as were her EKG, echocardiogram.  The MRI of her right humerus was a follow-up to an abnormal bony finding on her full-body MRI last December.  Happily, the irregularity seen in that bone is less prominent this time, so we don’t have to do any more testing for this issue.

More good news: the MRI of Jane’s right-sided plexiform is also stable.

The newest problem:  along with NIH and our hometown pediatric ENT, we’ve been following another NF-related mass under Jane’s tongue for the past 6 months.  In retrospect, it was present on her MRI in December, but Jane didn’t notice it until January when it started getting larger.  Imagine a couple of big marbles attached to the base of the underside of your tongue and you can understand what Jane’s been feeling.  It’s become bothersome enough that we’re finally having it removed surgically next month. 

The area of concern in December 2022 (right) and now (left)

Before any operation could be planned, we had to first determine what the mass was—it was unclear just from physical exam whether it was an extension of the existing right-sided plexiform, a new neurofibroma, or some other type of growth.  After lots of discussion and an MRI dedicated entirely to Jane’s mouth, the thought now is that the mass is actually Jane’s sublingual salivary gland, significantly enlarged because of some effect (pressure? blockage?) by the plexiform tumor.  Thankfully, the gland does not seem to have been invaded by the tumor itself, and so can be excised without worrying about detangling it from the plexiform (which would be a much more complicated procedure).  The plan is to remove the entire salivary gland.  (Our ENT reassures us that we have enough salivary glands in our mouths that removing one doesn’t make a difference.)  We’re not exactly sure of the precise details of the surgery except that we know it will be an outpatient procedure done entirely though the mouth, and that Jane will be sore for several days afterward but that it shouldn’t affect her overall activity.  We will keep you posted!

There is one other development since our last NIH visit in December.  Recently, our team was able to reanalyze the abdominal tumor that Jane had removed in 2020 for newly discovered genetic mutations, a process called genomic profiling.  Genomic profiling may be done on tumor tissue to look for mutations or other genetic changes in a tumor's DNA. This may help doctors understand how different types of tumors form and respond to treatment, which may lead to new ways to diagnose, treat, and prevent other tumors.

The reanalysis of Jane’s tumor of revealed a mutation in the ALK gene.  The ALK (or “anaplastic lymphoma kinase”) gene is a gene that makes a protein that is involved in cell growth.  Mutated (changed) forms of the ALK gene and protein have been found in some types of cancer, including neuroblastoma, non-small cell lung cancer, and anaplastic large cell lymphoma. While in Jane this mutation is most likely limited to the cells of the tumor that was removed (all tumors have changes in their DNA—that’s what makes a tumor different from normal tissue), our team wants to double check that it is not also present in Jane’s normal cells (a so-called “germline mutation”).  It’s unlikely that this will be the case (especially since she already has the NF mutation in all of her cells), but we are now waiting on the results of genetic testing collected from Jane this month to be sure.

As we usually do during our visits to NIH, we spent some time catching up with Dr Brigitte Widemann, our team leader, and her second-in-command, Dr Andrea Gross.  Somehow the topic turned to other NF clinics around the country and our research nurse, Anne Dufek, mused about whether we would ever use a different NF clinic closer to home if there was one available.  Our answer was a resounding “no”, since Jane and I feel like we get the best care in the world through our team at NIH.  Then we joked, “You know that person whose name is on all the NF papers and who is the leader in her field?  Yeah, she just gave me a hug and showed me pictures of her cats.” <3

Incidentally, the day we flew to NIH was May 17, which was also World NF Awareness Day!  My coworkers honored the day by wearing green—I was so touched.

Our team at NIH also celebrated!

Jane Update:

School is out for the summer so Jane is officially a high school junior!  (OMG.)  She got her first job this summer working in a local grocery store, and has already memorized lots of PLU codes.  Bananas, she tells me, are 4011 :)

 

Todd Update:

I’m going to take a bit of this post to celebrate Todd!  This spring he received several honors, including an endowed chair from Yale University and the Gold Medal Award from the International Society for Magnetic Resonance in Medicine.  Meet the Elizabeth Mears and House Jamison Professor of Radiology and Biomedical Imaging and Professor of Neurosurgery, Todd Constable <3

The 2023 Cincinnati Flying Pig Marathon

Running Update:

5/7/23  26.2 mi 4:58:22 (Ugh!)

Oh my, what a weekend!  It had everything!

    The Good--reuniting with friends and meeting new ones, and experiencing Cincinnati in all its Flying Pig glory.

    The Bad—the hills.  Oh, the hills!

    And the Ugly—thunderstorms for almost the entire marathon!

Here’s the recap:

Friday

The weekend was off to a promising start as I was greeted at the airport by a Flying Pig!  (For an explanation of how the marathon got its name, see my post from earlier this year.)

At the Cincinnati Airport!

I got into Cincinnati early enough to make it to the Expo Friday evening.

Lots of pig balloons!

And pig statues!

Every marathoner had their name written on the Flying Pig car

The corner of Fifth and Elm Streets

I bought a souvenir for Jane :)

Saturday

I milled around the Family Fun Festival at Smale Park right on the Ohio River.  Saturday’s events included:

. the 10K race

. the 5K race

. the Flying Piglet for younger kids.  Depending on their age, the kids covered different distances, from 100 yards for 6-7 year olds down to a 15 foot crawl for those under 1!

. the 26th Mile for older kids, who ran one mile to cross the marathon’s actual finish line

. PigAbilities, a one mile walk/run/roll through the finish line for people living with disabilities

. and the Flying Fur, a 2-mile dog-and-human walk/run!

The starting line

The Flying Piglet races

A play structure at Smale Park

Can you beat it?

A view of the Ohio River and the Roebling Suspension Bridge into Kentucky

The half marathon, full marathon, and marathon relay were reserved for Sunday.

 I saw So. Many. Pig. Costumes.

I also met Dr Elliott Robinson.  Dr Robinson is a neurobiologist who runs a research lab at Cincinnati Children’sHospital studying, among other things, cognitive problems in NF1.  He not only manned the Children’s Tumor Foundation information booth at the Family Festival on Saturday, he ran the marathon for CTF on Sunday!

Saturday afternoon I met up with my friend Janet, who lives in Kentucky just across the river from Cincinnati.  I first met Janet while running the Boston Marathon for CTF back in 2013!  We’ve kept in touch ever since, and for years she’s been trying to get me to experience the Flying Pig.  She showed me around Cincinnati and even drove me through the marathon route so I’d know what to expect.  I had known the course was hilly, but seeing the hills in person helped me to mentally prepare for them.

Overlooking the Ohio from the highest point of the marathon route

Pre-race pasta dinner!

Our waitress' Flying Pig makeup :)

Back at my hotel that night, I tried to get a good night's sleep.

Gear check.  Note the rain poncho to the left.

Sunday

Oh, the weather!  Not just pouring rain, but thunder and lightning for 19 of the 26.2 miles!

Todd's screenshot of the weather at the start of the race (in Celcius)

A photo from mile 3 of the race, posted on a local news site.
We had to run 23 more miles from this point!

After just the first mile, I wondered why the race wasn’t being postponed, but I figured it must be safe if the race officials were proceeding.  It turns out they did issue a “shelter in place” recommendation, but those of us who were already on the course didn’t receive it!

From the local newspaper, the Cincinnati Enquirer: www.cincinnati.com/story/sports/2023/05/07/heres-why-the-flying-pig-went-ahead-with-the-race-despite-bad-weather

And from the local TV station, WCPO: www.wcpo.com/news/local-news/despite-weather-flying-pig-marathon-continues 

I can’t say it was fun to run that day.  With each step my feet squished in my shoes and I felt like I was carrying an extra gallon of water in my clothes.  However, as miserable as the conditions were, there were still lots of die-hard spectators to cheer us on.  Each mile of the race route had multiple stations set up by local groups offering support and entertainment.  Pig puns abounded!

. There was a Bacon Station where a community church gave out strips of bacon.

. The Greased Pig Station run by a local Boy Scout troop supplied Vaseline (for our chafing).

. A high school lacrosse team had the Pigskin Station in which marathoners could throw a football into a basket as they ran by.

. The Hogwash Station had intended to provide mists of water to cool down the runners, but because of the conditions instead offered dry towels.

. And there were so many snack offerings!  There was the Hogs and Kisses Station (giving out Hershey’s Kisses), the Girl Snout Cookie Station, the Pork n Beans Station (for jelly beans), the Pigkle Station (for pickle juice!  Some claim it’s better than Gatorade!), and the Pig Newton Station, to name only a few.

By the time I reached the finish line (or rather, the “Finish Swine”) the rain had stopped and the clouds were starting to break up.  What a relief to be done!

The medal

Afterwards I headed straight to the CTF post-race booth to meet up with my teammates who I hadn’t been able to catch before the race.

There I met the Samblanet Family.  Jessica is the vice chair of the CTF Volunteer Leadership Council, of which I’m a member.  Her husband Brett ran the marathon this year!  Their daughter lives with NF.  (In a funny turn, Brett encountered my friend Janet in the starting corral of the race when she recognized and commented on his CTF shirt.)

I also met Caitlin Ottesen who, among many other CTF activities, organizes the Cincinnati Cupid’s Undie Run for CTF, which raised over $50,000 in 2023 alone!  Her son lives with NF.

Myself, Elliott Robinson, and Caitlin Ottesen

Finally, I met Chuck Dolish and his family.  I have known Chuck for many years as a member of the NF Endurance Team, but I had never met him in person.  Chuck himself lives with NF, and runs the Flying Pig Marathon every year, among many other races.  This was his thirtieth marathon for CTF!

Myself and Chuck Dolish on either side of Chuck''s pig-clad nephew!

For myself, this was my 20th marathon.  In 2023 to date we have raised $10,226 for the Children’s Tumor Foundation.  Feel free to keep the donations coming!  

www.KRath4Jane.com

Trying to dry my running gear in the hotel room after the race

Jane Update:

Jane and I head to NIH for her semi-annual check-up in a little over a week.  It always seems to come up so quickly.

 

NF Update:

A sad ending to this post, but it illuminates why I do all that I do in this blog.  Nick Gilbert, 26 year old NF Hero and son of Dan Gilbert, owner of the NBA’s Cleveland Cavaliers, lost his life to complications from NF this week.  The Gilbert Family has donated millions upon millions of dollars towards NF research.  Our heart breaks for this family.  And so I’ll carry on racing for an end to NF.