Vermont City Marathon update:

5/28/17  26.2 miles  4:14:55

After a week of feeling old (this past week marked 20 years since my med school graduation, my 20th wedding anniversary, and my 25th Yale reunion!) Sunday's race made me feel like I'm not over the hill yet.  Despite the hills and the heat, I finished 27th in my division, out of 123 women.  I'm quite pleased with my time—not a PR, but it's my best time since 2014.

The visit to Burlington got off to a rocky start.  Our hotel had inadvertently canceled our reservations and had given our room away.  We spent about a half hour thinking that we had nowhere to stay (all other rooms in that hotel and in all other hotels in Burlington were full on account of the marathon) but someone else canceled last minute, so they gave us that room.  Whew.

(The view from the new room ended up being beautiful!)

View of Lake Champlain from our hotel

Then the hotel restaurant ran out of pasta the evening before the race.  OUT OF PASTA!!  How does that happen??

Family selfie during our almost pasta-free dinner before the race.
(They managed to find some linguini for us!)

Despite these hiccups, race day was great.  Burlington is an impossibly lovely city on the banks of Lake Champlain in northern Vermont.  The marathon route wound in and out of the city in a clover-leaf fashion, treating us to gorgeous scenery along the waterfront and then returning us often to downtown.  The VCM advertises that it is "not hilly".  This is not true!  While I agree that there were only two big climbs (at mile 9 and again at mile 15), there seemed to be about a dozen moderate ones.  Still, the last 10 miles was mostly downhill, which was welcome.  I had been anticipating this fact, so much so that when I turned onto Battery Street for the last big climb of the race (the “Assault on Battery”), I almost wept to see the cheering crowds there.  (The Burlington Taiko drummers were especially inspirational!)

Before the start, posing by the lake

At the starting line

A view of the climb up Battery Street, from our hotel window

Half-way up Battery Street

Collecting some water from Alec!

And I'm off again!

Looking tired in the chute before the finish...

...until I see Todd and the kids!

Done!

Here are more pictures of the day from the Burlington Free Press.

The City of Burlington turned out tons of spectators.  One particularly nice feature of this marathon was that our race bibs displayed not just our numbers, but also our names, so that I would hear random spectators cheering “Go, Kristy!”  It was a nice touch.

Viewing the spectator signs at any race are always entertaining, but at the VCM had a few I’d never seen before:

~ Run like United wants your seat!

~ (Seen around mile 22) Mexico’s not gonna pay for the wall you’re hitting!

~ You think running a marathon is hard, try standing with these kids (Todd’s favorite)
~ Maple syrup shots ahead (And there were!)

Here is my Vermont City Marathon medal. Because I had run the Hartford Marathon within the previous year, I got an extra medal for completing the "New England Double"!  In the words of my running partner, Neil: “Golly—that’s SWAG.”

Time for a little rest now.  My next race isn’t until the New Haven Road Race in September, so I’ll go easy this next month.  Even though I’m resting, you can always donate to our annual campaign for the Children’s Tumor Foundation here:  www.krath4jane.com :)

Jane Update:

On Friday Jane started an increased dose of selumetinib: 20mg in the morning and 10mg in the evening (up from 10mg twice a day).  Hopefully this is a small enough increase that she won’t develop any new side effects, but large enough that the medicine will continue to control her tumor.

NF Update:

It was a busy week on social media for Jane and me! 

During our most recent to visit to NIH, we were asked if we would like to be involved with NF Awareness month at NIH.  Of course, we agreed!  We were photographed with our care team, and interviewed by the Communications Department, and the result was the following series of Twitter and Facebook posts.

First, the NIH National Cancer Institute’s Cancer Research Center introduced NF Awareness Month with a photo of Jane at age 3.  (https://twitter.com/nciresearchctr/status/864091817888944129)

Here's an expanded view.  (I love this picture!)

In fact, they used that photo as the banner on their Twitter home page!  (https://twitter.com/NCIResearchCtr)

 Next, they tweeted a picture of Jane and I together.  (https://twitter.com/nciresearchctr/status/864560917580845057)

  

Finally, they posted a link to our interview!  (https://twitter.com/nciresearchctr/status/864916639707525121)

The Children’s Inn, our home-away-from-home while we visit NIH, even picked up on the post.

Here is the full article, posted on the Cancer Research Center.  (https://ccr.cancer.gov/news/article/a-conversation-with-kristy-and-jane)

A Conversation with Kristy and Jane

Kristy and Jane travel from Connecticut to the Clinical Center a few times a year for follow-up appointments and tests for the clinical trial she is enrolled on. Photo credit: Brooke Bready

Jane has been coming to the NIH Clinical Center for treatment for neurofibromatosis type 1 (NF1) since she was three years old. NF1 is a chronic, genetic disease that one in 3,000 people can develop. Some NF1 patients develop plexiform neurofibromas, which are typically benign tumors that grow on nerves throughout the body. These tumors are usually inoperable because of how and where they grow, so many NF1 patients participate in clinical trials that study different therapies to shrink or slow the growth of these tumors.

Jane has a plexiform neurofibroma on the right side of her face, jaw and neck. She is currently enrolled in a trial with Brigitte Widemann, M.D., that tests Selumetinib, a MEK inhibitor, and her tumor is now 30.7 percent smaller than when she first started this trial three years ago. Her diagnosis has changed the lives of her family but has also given them new passions and perseverance.

How old was Jane when she was diagnosed?

Kristy: Jane was 3 months old when our pediatrician suggested the diagnosis. She was 6 months old when it was confirmed.

How did you find out about Dr. Widemann and NIH?

Kristy: Jane was seeing Dr. Scott Plotkin at Massachusetts General Hospital for her NF.  By 18 months, her plexiform neurofibroma was growing at such a pace that he referred us to Dr. Widemann.

How has CCR’s NF team and clinical trial program impacted Jane’s treatment?

Kristy: It has been life-changing. Jane first participated in a clinical trial with Dr. Widemann from ages three to six. The medication, Pegintron, temporarily slowed the growth of Jane's tumor. When her tumor grew so much that she no longer qualified for that trial, Dr. Widemann helped us to find another trial in Indiana with the drug Gleevec, which she thought was promising. Finally, Jane's current trial, Selumetinib, has actually decreased the size of Jane's tumor.

If we had not had Dr. Widemann's advice and guidance these past 7 years, Jane's tumor would be double or triple the size that it is now. She would likely be hearing impaired, might have impaired vision and a compromised airway and would likely have had major surgery by now.

What is the best part about coming to NIH?

Jane: Spending time with mom and the Children's Inn. It's fun.

Kristy: I know that she particularly looks forward to opening her mailbox at the Inn (to find a prize) every morning we are there.

What do you like to do for fun?

Jane: Read, play outside and soccer.

As a parent and caregiver, how has Jane's journey impacted/inspired you?

Kristy: Jane's journey has impacted me and our whole family. In part because of the need for Jane and me to travel to NIH regularly, I have had to tailor my career to one which allows more flexible work hours. I started my career as a full-time obstetrician-gynecologist but have had to cut my hours and drop obstetrics in order to manage the needs of Jane and our family.

It has affected my husband and I from an emotional standpoint. We worry about Jane's condition and what the future might hold for her because of NF. It is painful to watch one's child suffer without being able to help.

Jane's journey has inspired me to become an advocate for NF. This has helped me combat the feeling of helplessness! I have been a runner all my life but joined the NF Endurance team, part of the Children's Tumor Foundation, about a year after Jane's diagnosis. Since then I have run twelve half marathons and ten full marathons. I also maintain a blog about our NF Journey and my running for a cure.

~ ~ ~

One week until the Vermont City Marathon!  As always, I’ll be running for the Children’s Tumor Foundation.  You can donate here:  www.KRath4Jane.com

Running Update:

4/26/2017	Run		9.1 mi	1:29:39
4/27/2017	Swim		1700.0 m
4/28/2017	Run		10.0 mi	1:37:09
5/3/2017	Run		7.6 mi	1:11:05
5/5/2017	Run		5.0 mi	47:56
5/6/2017	Run		12.7 mi	2:07:19
5/7/2017	Run		5.0 mi	46:53
5/8/2017	Swim		2000.0 mi
5/10/2017	Run		7.6 mi	1:10:26
5/13/2017	Run		22.3 mi	3:42:02
5/15/2017	Swim		1850.0 m
5/16/2017	Run		3.2 mi	28:08
5/17/2017	Run		5.0 mi	47:56

Only a week and a half until the Vermont City Marathon!  I’m in taper mode now.  Whew.

Jane Update:

As readers of the blog know, Jane has been participating in a clinical trial over the past three years which is proving to be groundbreaking for the treatment of plexiform neurofibromas.  The medication part of a family of drugs called MEK-inhibitors, which inhibit a specific enzyme in the cell-signaling pathway.  Because of its success, the Children’s Tumor Foundation produced an informational flyer to educate the NF community about this work.  I am pleased to say that Jane is one of the children featured in the flyer, to be released this month!

NF Update:

Every year during the month of May I post facts about NF daily on social media in honor of Neurofibromatosis Awareness Month to help fulfill the education goal of the campaign.  For those of you not on Facebook, here they are all in one place!  To read more about the events of NF Awareness Month, check out the Children’s Tumor Foundation website: http://www.ctf.org/get-involved/nf-awareness-month

1	May is Neurofibromatosis Month!
2	Neurofibromatosis encompasses a set of distinct genetic disorders that causes tumors to grow along various types of nerves.
3	NF can also affect the development of non-nervous tissues such as bones and skin. Neurofibromatosis causes tumors to grow anywhere on or in the body.
4	There are three forms of neurofibromatosis:  NF1, NF2, and schwannomatosis, each cause tumors to grow on nerve endings in or on the body.
5	1 in 3000 people are affected by Neurofibromatosis type 1, 1 in 25,000 are affected by NF type 2, and about 1 in 40,000 are affected by schwannomatosis.
6	NF1 and NF2 are called autosomal dominant genetic disorders. Half of all cases are inherited from a parent who has NF1 or NF2; half of all cases are not inherited but the result of a new or spontaneous mutation.
7	Each child of an affected parent has a 50% chance of inheriting the gene and developing NF. The type of NF inherited by the child is always the same as that of the affected parent, although the severity of the manifestations may differ from person to person within a family.
8	The severity and physical signs of NF1 can vary widely from patient to patient. People who have NF1 may have very few neurofibromas (tumors) or they may have thousands of them throughout their body.
9	Although most cases of NF1 are mild to moderate, NF1 can lead to disfigurement; blindness; skeletal abnormalities; dermal, brain, and spinal tumors; loss of limbs; malignancies; and learning disabilities.
10	NF1 also has a connection to developmental problems, especially learning disabilities, which are five times more common in the NF1 population than in the general population.
11	NF1 can result in disfigurement in a number of ways. Skin neurofibromas may develop on the face or on exposed areas of the arms or legs. The larger and deeper plexiform neurofibromas may grow around the eye or eyelid, or affect growth of one side of the face. Scoliosis, or curvature of the spine, can affect appearance when it is severe. Rarely, an overgrowth of skin or bone causes enlargement of an arm or leg.
12	Some people with NF suffer from a bony defect called tibial dysplasia, in which the leg bones are curved.
13	Another complication of NF is pseudarthrosis, in which a bone breaks, typically a long bone such as the femur, and does not fully heal, causing a "false joint".
14	People with NF are at increased risk of high blood pressure and renal artery stenosis.
15	NF can also affect the cardiovascular system causing congenital heart defects. The most common heart defects seen in NF are those affecting the heart valves, particularly the pulmonary valve.
16	Café-au-lait spots, the most common sign of NF, are the flat, pigmented spots on the skin, which are called by the French term for coffee (café) with milk (lait) because of their light tan color. In darker-skinned people, café-au-lait spots appear darker in color than surrounding skin. People with NF almost always have six or more café-au-lait spots.
17	May 17 is World NF Awareness Day.
18	5% of NF1 patients have a bone-related issue called sphenoid wing dysplasia, in which the skull and eye orbit bony areas erode away, causing possible craniofacial abnormalities, loss of the eye, and enlargement of the eye orbit cavity.
19	About 10% of people with NF will develop scoliosis, or a lateral curvature of the spine. In most cases it is mild, but more severe cases may require surgery.
20	Approximately 15% of patients with NF will develop an optic glioma with the peak age of onset between age 3-4 years old. An optic glioma is a tumor of the optic nerve in the brain which controls the vision
21	Many people with NF1 suffer from frequent headaches, particularly migraine headaches.
22	May 22 is NF2 Awareness Day.
23	The distinguishing feature of NF2 is tumors that grow on the eighth cranial nerve in both ears, commonly causing deafness and severe balance problems.
24	NF2 brings on increased risk of other types of nervous system tumors as well.
25	NF2 can also cause severe vision problems, including cataracts, retinal abnormalities and orbital tumors.
26	NF is not the "Elephant Man's Disease," although it was at one time believed to be. Scientists now believe that Joseph Merrick, the so-called "Elephant Man," had Proteus Syndrome, an entirely different disorder.
27	The tumors in NF are usually noncancerous (benign), but in some cases these tumors become cancerous (malignant) tumors.
28	NF related malignancy is estimated to occur in 7-12% of affected individuals. People with NF are at increased risk for MPNST (malignant peripheral nerve sheath tumor), brain tumors, and leukemia, as well as several other forms of cancer.
29	NF can cause itching of the skin.
30	NF is worldwide in distribution, affects both sexes equally and has no particular racial, geographic or ethnic distribution. Therefore, NF can appear in any family.
31	The Neurofibromatoses are genetically-determined disorders which affect more than 2 million people worldwide; this makes NF more prevalent than cystic fibrosis, Duchenne muscular dystrophy, and Huntington's Disease combined.

NIH/DC Update:

Jane and I had an uneventful flight to Bethesda on Sunday.  We spent the afternoon resting at the Children's Inn across from the Clinical Center.

On Monday Jane had a full day of testing, beginning at 8:30am.  The day started with a blood draw, for which Jane never flinches.  Next was Eye Clinic, where the battery of testing and exams took 2 hours (relatively quick for the black hole that is the NIH Eye Clinic.)  Next came her physical exam with our nurse practitioner.  Since our arrival at NIH we'd had about twenty people tell Jane how tall she's gotten, and now we know exactly how much:  she's grown 2-1/2 inches in the past 6 months :)  We followed her physical exam with an EKG.  Then, right before our lunch break, we even managed to get to the Security Desk to have our hospital badges renewed.

The afternoon began with an echocardiogram.  The room was so dark and quiet that Jane fell asleep during the hour-long exam!  The technologist was so gentle, and Jane so tired, that she didn't wake up when he shifted positions or changed angles.  I was glad, thinking it might help give her the energy to make it through the rest of the day and her ever-important MRI.

The last appointment before her MRI was my favorite, Photography.

By this time, Jane was starting to flag.  I gave her a pep talk telling her she had to hang in there, that the MRI is the most critical test of our visit...  In the end, Jane stayed SO STILL for her MRI!  I think it was the best one she's ever done without sedation.

Back at the Inn after 8 hours of testing at the Clinical Center, we had dinner and an early bedtime in anticipation of receiving the results of MRI #22 in the morning.

Tuesday morning, we were back at the Clinical Center again at 8:30 am, this time for pictures with our care team to be used by NIH for NF Awareness Month in May.

With our research nurse, Trish, Dr Brigitte Widemann, and our nurse practitioner, Andrea

After the photo shoot, we got the news we'd been anxious to hear:  Jane's tumor appears stable!

It is currently 30.7% smaller than when she started the trial 3 years ago.  We bottomed out at 33% smaller about a year ago, and it's remained within that margin of error ever since.  Of course, we are thrilled!

A few more issues we discussed Jane's visit:

Medication dose.  Jane is currently taking only 10mg of selumetinib twice a day.  The typical dose of children in the study at this point is more than twice that.  Jane's dose had been reduced early on due to a severe rash that she developed about a month after starting treatment.  At the time, less than two dozen children had ever taken the medication, so to be cautious our care team kept her at the lower dose.  However, they have since found that several children developed similar rashes early on in this treatment, and that the rash usually resolves without reducing the medication dose.  Jane's care team is again considering returning her dose back to what it would have been had she never had the reduction.

The problem is that the study protocol requires that the medication be given in two equal doses daily; and as luck would have it, the only capsules manufactured by the pharmaceutical company (Astra Zeneca) are 10mg or 25 mg--so there is no option to increase the dose gradually, say by 5mg at a time.  The only way to increase Jane’s dose would be to jump immediately to 20mg or 25mg twice a day, and such a jump would invariably cause more side effects.

However, Jane’s doctor, the wonderful Dr Brigitte Widemann, did hint that it may be possible to alter the study protocol to remove the restriction of equal doses twice a day, thus allowing Jane to take, for example, 10mg in the morning and 20mg in the evening.  This would be a more manageable dose increase.  The team will need to review this possibility and get back to us. 

On the one hand, we are content to see Jane’s tumor is stable, and don’t feel the need to excessively press our luck with a higher dose of selumetinib, especially when she has been feeling so well.  On the other hand, since Jane has already been subjected to the risks of selumetinib, we’d like her to derive the maximum benefit that she can from the medication while it is available, in case there are ever issues with obtaining the drug in the future (eg if it doesn’t get FDA approval or if the company decides it’s not worth their while to continue manufacturing it).

We also discussed with Jane’s team her facial structure. This subject came up because recent dental x-rays had highlighted the bony abnormalities of Jane’s face.  It turns out her face may never be symmetrical, even if her tumor completely disappeared today.  I’m embarrassed to say this never occurred to me before, but it makes sense.  The tumor occupies a large space in Jane’s head and neck, and it has disrupted the growth of the bones in her face, causing a defect in her mandible (jaw bone) and an asymmetry in her mouth.  This is unlikely to change even if the tumor went away.

~ ~ ~

With our NIH appointments complete, Jane and I turned our attention to another adventure:  a visit to Capitol Hill!  In my last post, I described how our Representative from Connecticut, Congressman Joe Courtney, invited Jane and me to meet with him in his Washington office to share our experience with the National Institutes of Health and thereby illustrate the importance of its funding.

Ready to conquer the Capitol 

Jane and I took the Metro from the Medical Center all the way to Judicial Square and walked from there to the Rayburn House Office Building next to the Capitol.  I can’t tell you how intimidating it was walking into this building!  We were literally within the Halls of Congress!  Congressman Courtney and his staff could not have been more gracious and welcoming.  The Congressman spent a good half hour speaking with us, asking questions about Jane and her treatments, and listening to our story.  We had just heard the good news that Congress had agreed to a $2 billion spending increase to the NIH for the remainder of this fiscal year, and Congressman Courtney vowed to fight for continued financial support in the budget for NIH in the upcoming year.

We gave the Congressman a “I kNow a Fighter” shirt, a copy of the New England Journal of Medicine that featured selumetinib, and a copy of the “MEK Makes a Difference” flyer from the Children’s Tumor Foundation that features Jane (more on this in my next post).

Outside the Congressman's office

With Representative Joe Courtney!

Congressman Courtney also presented Jane with his Challenge Coin from the House of Representatives in honor of her visit!

A copy of the Affordable Care Act from 2010 hangs on the Congressman's wall <3

After our meeting, one of the Congressman’s staff members offered to take us on a personal tour of the Capitol Building!  Our guide, Brendan, gave us an entertaining, informed, and often Hamilton-themed (Jane, being a fan of the musical, looked for Alexander Hamilton in every statue and painting we saw) inside view of the Capitol.  He took us through the House office buildings, into the underground tunnel that connects them to the Capitol Building itself, and into the Visitor’s Center.  We saw Emancipation Hall, the Crypt beneath the Rotunda, the Old Supreme Court Chamber, the Old Senate Chamber, the National Statuary Hall, and the Rotunda itself. 

The Statue of Freedom, a replica of the one that sits atop the Capitol Building

A statue of Helen Keller in Emancipation Hall

We spent the most time in the Rotunda, with The Apotheosis of Washington fresco in the oculus of the dome, and with its huge scenes from the American Revolution, painted by John Trumbull.

Declaration of Independence

Jane and Brendan admire the Surrender of General Burgoyne

Alexander Hamilton!

General George Washington Resigning his Commission

Surrender of Lord Cornwallis behind the Portrait Monument, a statue of three pioneering suffragists

While we were in the Rotunda, Brendan noticed that the Capitol Police seemed to be ushering tourists to one side of the room, which made him suspect that someone notable was about to arrive.  He thought maybe House Minority Leader Nancy Pelosi (Yay!) or House Speaker Paul Ryan (Boo!)  We hung around hoping to catch a glimpse of whomever it was.  Jane started to get restless, asking, "Can we go now?" but I asked her to be patient for a few more minutes.  One of the police officers leaned over to us and whispered, "I'll let you know: it's the Vice President."  At this point Jane asked, "Can we really go now?"  (That's my girl!)  But I had the morbid urge to see him in person, and vowed if he came to shake our hands that I would let him know the reason we were in DC and ask him to support the NIH and reasonable healthcare in general.  As it turned out, he shook hands with folks on the opposite side of the barrier from us, so I didn't have the chance to speak my mind.

Jane's silent protest of the interruption by the Vice President

The most exciting part of our tour (I thought) was visiting the House Gallery where we watched the House of Representatives voting on a bill.  It was House Resolution 1679, “FEMA Accountability, Modernization and Transparency Act of 2017”.  Not the most gripping of legislations, it passed 419-0.  Still, I was enthralled.  We could see our super Representatives from Connecticut:  Joe Courtney, John Larson, Elizabeth Esty, Rosa DeLauro, and Jim Himes.  I think I squealed like a schoolgirl when Brendan pointed out Maxine Waters!  I confessed that, at this stage in my life, I no longer follow Hollywood celebrities, but I get excited to see famous politicians :)

With our tour done we said goodbye to Brendan and made our way back to the Metro, back to NIH, where we caught a shuttle to the airport and flew home.  We slept well that evening, thinking of our good news from NIH and our exciting time on Capitol Hill.