Saturday, July 7, 2018


Running Update:
6/6/2018
Run

5.0 mi
47:58
6/9/2018
Swim

2050.0 yd

6/10/2018
Run

5.0 mi
48:59
6/12/2018
Swim

1500.0 yd

6/13/2018
Run

5.0 mi
48:23
6/17/2018
Run

3.3 mi
32:03
6/19/2018
Swim

1500.0 yd

6/20/2018
Run

3.2 mi
30:21
6/22/2018
Run

4.8 mi
45:17
6/24/2018
Run

5.6 mi
51:45
6/26/2018
Bike

11.3 mi

6/26/2018
Run

5.3 mi
47:37
6/27/2018
Bike

21.3 mi

6/28/2018
Run

5.9 mi
56:26
6/29/2018
Bike

11.1 mi

6/29/2018
Run

3.1 mi
29:03
7/1/2018
Run

4.5 mi
45:34
7/4/2018
Run

4.5 mi
43:13

I took the month of June off and didn’t do much running after the Vermont City Marathon.  It was nice to have a little break, but training for October’s Marine Corps Marathon starts next week!

NF Update:
Jane has almost completed 3 cycles of her new dose of selumetinib (20mg twice a day) and she seems to be tolerating it well.  We haven’t noticed any significant side effects, except for a little bit of acne.  Apparently, in adolescents, selumetinib can exacerbate acne!  Not ideal, but so far Jane’s is minimal :)
In two weeks the dose will increase again to 25mg twice a day.  We’re hopeful she’ll continue to do well with it.

Family Update:
Our summer is off to a great start!
The summer began with Alec’s graduation from middle school.

Walking up to receive his diploma

Shaking the principal's hand


With our dear friend, Glennda

Father and son

With Grandma, too

Proud parents

With his buddies


Then we spent a week in Cape Cod, spending some time with our friends, Neil and Marcia.

Our deck at sunset

National Seashore

Swimming off our deck, bayside, in high waves!

The Fam

Cycling to the ocean side with Neil and Marcia


Anything abbreviated "NF" catches my eye!

This week the kids started a month at Deer Lake Camp.  This is Helen’s final year as a Counselor-in-Training—she’s hoping to work as a counselor next summer.  Alec will be attending the Wilderness School all month, camping out several nights a week, and Jane is a day camper.

Finally, Helen turned 16 this week, and today she got her driver’s permit!  Watch out, world!

Opening presents


Personalized M&Ms!

Outside the DMV, after passing her permit test :)


Sunday, June 3, 2018


Running Update:
5/8/2018
Swim

2100.0 yd

5/9/2018
Run

5.0 mi
45:26
5/11/2018
Run

5.0 mi
46:02
5/12/2018
Swim

2100.0 yd

5/13/2018
Run

13.1 mi
2:11:11
5/15/2018
Swim

2100.0 yd

5/16/2018
Run

5.0 mi
48:29
5/18/2018
Run

5.0 mi
46:32
5/19/2018
Swim

1700.0 yd

5/20/2018
Run

5.0 mi
48:37
5/23/2018
Run

3.2 mi
30:35
5/27/2018
Run

26.2 mi
4:57:31
6/3/2018
Run

3.2 mi
32:05

Another marathon done!  I ran the Vermont City Marathon over the Memorial Day weekend.  It was not my best race, but still we’ve raised over $5000 towards our 2018 campaign for the Children’s Tumor Foundation.

The weekend started with the excitement of meeting Meb Keflezighi.  Meb is a famous long-distance runner who has won the New York City Marathon, the Boston Marathon, and won a silver medal for the Olympic Marathon, among many other achievements.  He was participating in the Vermont City Marathon for its 30th anniversary, and he visited with fans in the days leading up to the race.  I brought him a Children’s Tumor Foundation “I Know a Fighter” t-shirt (like the one I’m wearing in the photo) and told him about Jane.





I have to say, I was a little disappointed in my performance in the race itself.  I finished in just under 5 hours, which is 45 minutes slower than I ran the same exact race last year!

I don't know what happened.  I felt like I’d prepared better than any of my recent races.  My 22-mile training run three weeks before the marathon was at a 10:15 min/mile pace, yet I ran the marathon itself more than a full mile-per-minute slower (11:20 min/mile)!  What the heck?  Maybe too much excitement the day before?  Had I been on my feet to much?  Maybe what I ate for dinner?   It was a little warmer and more humid the day of the race than I would have liked, but certainly not excessively so.  I do think I went out too fast, and that may have been my downfall.  One would think after 13 marathons I should know better!   Though, I wouldn’t have thought that would make me tank so hard before the halfway mark.  I can’t blame the hills because I ran the exact same route last year. 

I am glad I was able to finish (because there were moments during the race that I didn't think I could!)  I’ll just have to prepare that much smarter and hope for better luck next time.  Now at least I get to rest for a bit.

Lining up at the start

Getting a high five from Alec on the hill at mile 15

The finish!

With the family (my support crew!) at the finish

The Vermont City Marathon is unique in providing participants with professional race photos free of charge.  Some of my shots were better than others...

Here I am walking and looking defeated as the 4:45 pace group passes me :(

I like this one better :)

The medal

We did have a very nice weekend.  The weather cooperated, and the city of Burlington is quite lovely.  The race is ideally situated next to our hotel (the start and finish and a key moment in the race all are right in front of it) so it's easier on Todd and the kids than some of the other races I've done.  We all enjoyed swimming in the pool after the race and had a nice dinner out last night in a pedestrian-friendly part of the city.  We even stopped on the way home to tour the Ben and Jerry's ice cream factory.  All in all, a good family weekend.


Jane Update:
Jane was featured as an NF Champion on the NF Northeast Facebook page during NF Awareness Month!  Take a look:



(Here's a link to the NF Northeast Facebook post.)


NF Update:
May and NF Awareness Month may be over, but we’ll keep on working to find a cure for NF!  You can always donate to our campaign for the Children’s Tumor Foundation at www.KRath4Jane.com.



Sunday, May 6, 2018

Running Update:
4/24/2018
Swim

2100.0 yd

4/25/2018
Run

10.2 mi
1:39:22
4/27/2018
Run

5.0 mi
47:43
4/28/2018
Swim

2100.0 yd

4/30/2018
Run

6.1 mi
55:32
5/2/2018
Run

7.3 mi
1:11:35
5/4/2018
Run

7.2 mi
1:10:43
5/6/2018
Run

22.3 mi
3:48:37

Three weeks until I run the Vermont City Marathon.  I’ve completed two 20+ mile runs in the past two weeks—now it’s time to taper, thank goodness.  As always, I am running for the Children’s Tumor Foundation in a quest to #EndNF.  This will be marathon number thirteen!

NF Update:
May is NF Awareness Month and, as is my habit, I am posting NF facts daily on social media.  Here is the full list:

Thirty-one facts about Neurofibromatosis for the 31 days in May, 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 autosomal dominant genetic disorders--they can be inherited from just one parent. 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.

The fact I listed under May 6 and 7 describe a bit of the genetics of NF.  Jane’s brother Alec was studying genetics in his science class recently and asked me to help him review a Punnett square assignment he had completed.  (Remember Punnett squares from Biology class?)  It had questions like: “A pea shape gene has two alleles, round or wrinkled, and round is dominant.  If a heterozygous pea plant was crossed with a homozygous recessive pea plant, what would be the genotype ratio of the offspring?” It also had questions involving Sickle-Cell Disease and Cystic Fibrosis.  Both these diseases are caused by genetic mutations and both are inherited in an autosomal recessive pattern. To have an autosomal recessive disorder, you inherit two mutated genes, one from each parent. These disorders are usually passed on by two carriers. The carriers’ health is rarely affected, but they have one mutated gene (recessive gene) and one normal gene (dominant gene) for the condition.

I mentioned to Alec that NF is inherited in an autosomal dominant fashion.  You need only one mutated gene to be affected by this type of disorder.  Together we calculated that Jane’s children will have at least a 50% chance of inheriting NF from her.  If Jane has children with another person with NF (watch out Renie, Kelly, and Seddra!), three out of four of their children will have NF.  Alec commented, “Whoa.  That sucks.”  I agreed.

Another (somewhat more positive, in Jane’s case) characteristic of NF inheritance is that not all offspring have the disease to the same severity as their parents.  The child of a person with severe NF manifestations might have a child with mild manifestations, and vice versa.

Jane Update:
Jane is doing her part to celebrate NF Awareness Month.  The National Cancer Institutes at NIH featured Jane again this year for their NF Awareness Month campaign.  They tweeted the following on the first day of May!



If you look closely at the NCI Research Center’s Twitter page, you’ll also see Jane’s friend Travis in the banner at the top.  He’s playing air hockey, just like he does with Jane