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.