Monday, June 19, 2017

Running Update:

5.0 mi

5.0 mi

3.3 mi

7.6 mi

3.2 mi

5.0 mi

8.3 mi

15.3 mi

I’ve been taking it easy since the marathon, though I did just hit over 500 miles for 2017—halfway to 1000 again!  I’ve added a new sport to my training for this week: cycling!  It will be short-lived.  We are on a family vacation to Cape Cod, where we bike almost every day.  Jane and I ride a tandem bike, so I get an extra work out :)

Jane Update:
Jane’s class held a Living Wax Museum during the last week of school.  Students researched a historical figure, wrote a biography, and then became the person they had studied in a presentation to parents.  Jane chose to be Amelia Earhart!  She chose Earhart because, in Jane’s words, “She was an accomplished woman, like I want to be.”

We also recently got the following email from NIH:
“AstraZeneca (the company that makes the Selumetinib) is having an internal meeting to discuss plans for seeking FDA approval for the study drug. They have asked if we have any patient testimonials that could be used during this meeting and the research team thought of the story that we did on Jane recently. (Here is the link.)  The pictures and write-up would be presented to a small group of AstraZeneca staff.”

My daughter, Jane—helping to get life-saving medicine approved :)

NF Update:
The New York Times published an Op Ed piece on June 7th as part of their weekly series of essays, art and opinion by and about people living with disabilities.  The author, Alex Hubbard, is a reporter at The Tennessean, the local paper for Nashville, TN.  He shared his own story about living with neurofibromatosis, type 2, with the New York Times:
"I am not just a blind person who developed a severe speech impediment but didn’t give up on his career dream. I am a 26-year-old man with a quick wit, who is fluent in hockey, is widely read and can name every United States president in order, backward."  Here is the link to the full article.  Give it a read—this is NF.

Wednesday, May 31, 2017

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!


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: :)

Saturday, May 20, 2017

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.  (

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

In fact, they used that photo as the banner on their Twitter home page!  (

 Next, they tweeted a picture of Jane and I together.  (

Finally, they posted a link to our interview!  (

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.  (

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:

Wednesday, May 17, 2017

Running Update:

9.1 mi

1700.0 m


10.0 mi

7.6 mi

5.0 mi

12.7 mi

5.0 mi

2000.0 mi


7.6 mi

22.3 mi

1850.0 m


3.2 mi

5.0 mi

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:

May is Neurofibromatosis Month!
Neurofibromatosis encompasses a set of distinct genetic disorders that causes tumors to grow along various types of nerves.
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.
There are three forms of neurofibromatosis:  NF1, NF2, and schwannomatosis, each cause tumors to grow on nerve endings in or on the body.
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.
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.
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.
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.
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.
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.
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.
Some people with NF suffer from a bony defect called tibial dysplasia, in which the leg bones are curved.
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".
People with NF are at increased risk of high blood pressure and renal artery stenosis.
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.
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.
May 17 is World NF Awareness Day.
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.
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.
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
Many people with NF1 suffer from frequent headaches, particularly migraine headaches.
May 22 is NF2 Awareness Day.
The distinguishing feature of NF2 is tumors that grow on the eighth cranial nerve in both ears, commonly causing deafness and severe balance problems.
NF2 brings on increased risk of other types of nervous system tumors as well.
NF2 can also cause severe vision problems, including cataracts, retinal abnormalities and orbital tumors.
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.
The tumors in NF are usually noncancerous (benign), but in some cases these tumors become cancerous (malignant) tumors.
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.
NF can cause itching of the skin.
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.
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.