Wednesday, December 21, 2016

Running Update:

13.2 mi

7.6 mi

5.0 mi

9.2 mi

5.0 mi

5.0 mi

13.2 mi

3.2 mi

On November 27th, I passed 1000 miles of running for 2016! It's my 4th year in a row running 1000 miles, but the first time I've done it before December.  At this point I’m at 1053.7 miles, and may have to leave it at that.  I had planned to add quite a few more runs before the end of the year, but alas I've succumbed a nasty chest cold and have not run in a week.  I had hoped to try a little run today, but then I started coughing again and decided I'd better wait another day.   I hate not running!  I feel like my muscles are already atrophying.  At least it's not right before a race.  Hopefully will have a good rest over the holidays and I'll jump right back into training in January.

NF Update:
This is Akae, a 13-year-old girl in 1835 in Canton (now Guangzhou), China.
(Lam Qua, 1835, Portrait No. 01, oil painting, 61x47cm, 
Harvey Cushing/John Hay Whitney Medical Library, New Haven.)

 I came across an interesting piece of medical history recently.  Another NF Mom had called my attention to a collection of paintings by the 19th century Chinese artist Lam Qua.  The paintings were commissioned by Dr. Peter Parker, and American surgeon who studied at Yale and who was a medical missionary to China in the 1830’s. The portraits are of his patients before surgery.  As the collection is housed at the Yale Medical School Library, I decided to investigate.

Here is a link to the collection:
I was so intrigued by these paintings!  In particular, I was taken by the little girl in Portrait No. 01, above, because of her facial tumor.  (Here is the link.)

I wrote to the Medical History Department at the library, and, amazingly, she provided me with the case history for the little girl.  (A word of caution: the report contains graphic descriptions of surgery.  You can skip this report and continue with my blog post, which will describe the important parts of the history.)

First Report Quarterly Report, from the 4th of November, 1835 to the Fourth of February 1836, Chinese Repository 1835-36, Vol. 4, p. 467-469: “Sarcomatous tumor. Akae, a little girl, aged 13. As I was closing the business of the day, I observed a Chinese timidly advancing into the hospital leading his little daughter, who, at first sight, appeared to have two heads. A sarcomatous tumor projecting from her right temple and extending down to the cheek as low as her mouth sadly disfigured her face. It overhung the right eye, and so depressed the lid as to exclude light. The parotid and also its accessory gland were very much enlarged. This large tumor was surrounded by several small and well-defined ones, the principal of which lay over the buccinators muscle. Slight prominences on other parts of the body indicated a predisposition to tumors, which I have learned, is hereditary. The mother presents a most singular appearance, being covered from birth with small tumors, some of the size of large warts, and others hanging pendent, in shape and size like the finger. Akae is the only one of her four children thus afflicted. Her general health was somewhat deranged, the tongue foul, pulse frequent and feeble, and the heat of the tumor above the natural temperature of the system. The blood vessels passing over it were much enlarged. The weight much accelerated its growth, and occasioned pain at night in the integuments around its base. The child complained of vertigo, and habitually inclined her head to the left side. According to the statement of parents, the tumor was excited into action by the small-pox, which the child had four years since, but within the last four months had attained three fourths of its present magnitude. The child was put under medical treatment for a month, during which her health decidedly improved. From the first, it appeared to me possible to remove it, yet the possibility of an unfortunate result, or even of the child’s “dying under the knife”, and the operation of the hospital being thereby interrupted or broken up, did not escape any thoughts. On the other hand, however, it was a case presented in divine providence, and it was evident that, left to itself, the tumor might terminate the life of the child, and that, from the accompanying symptoms, before a great length of time. The surgical gentlemen whose counsel I was so happy as to enjoy were all agreed as to the expediency of its removal, yet with all its circumstances, they regarded it a formidable case. Though in a Christian and enlightened land the surgeon might have undertaken it without embarrassment, it was not so here. Having often, in secret as well as in concert with others, commended the child to the great Physician, I resolved upon the undertaking, with the precaution of procuring a written instrument, signed by both parents, stating that the operation was undertaken at their desire, and that they would exculpate me from censure of the child should die in consequence of the attempt. Even the burial of the corpse was a subject of forethought and agreement with the father. On the nineteen of January, with the signal blessing of God, the operation was performed. The serenity of the sky after several days of continued rain, the presence and kind assistance several surgical gentlemen, and the fortitude of a heroine, with which the child endured the operation, call for my most heartfelt gratitude to the Giver of all mercies. A few days previous to the extirpation an evaporating lotion was applied to the tumor. An opiate was given fifteen minutes before, and wine water during the operation. The patient cheerfully submitted to be blindfolded and to have her hands and feet confined. The extirpation was affected in eight minutes. Another small tumor of the size of a filbert was also removed from under the eyebrow. The loss of blood was estimated to be about ten or twelve ounces. Not an artery required to be taken up. She vomited, but did not faint. The tumor weighed one pound and a quarter. The circumference at its base was sixteen inches and three quarters, and the length of the incision from the top to the head to the cheek ten inches. On opening it I found portions of it becoming black and two or three drachms of sanious blood of a dark chocolate color, indicating that it had already taken on a diseased action. After a nap the child awoke cheerful as usual; in the evening her pulse was accelerated and she complained of nausea, but ever afterwards uniformly said that she had no pain. No inflammation supervened, and the wound healed by the first intention. Three days after the operation, in several places of an inch or more in length, it had completely healed, and it fourteen days the whole, except a spot the fourth of an inch, was entirely healed. In eighteen days the patient was discharged.”
|Continuation of the case in the Third Quarterly Report for the term ending on the 4th of August 1836, The Chinese Repository 1836-1837, Vol. 5, p. 188:|“Akae is mentioned in the first report under date of December 27th, 1835. About three months subsequent to the removal of the original tumor, as she was walking by the river side, a coolie, carelessly passing by, thrust the end of the bamboo, with which he carried his burden, against the superciliary ridge of the right temples from which the tumor had been removed. When she came to the hospital a month after the accident, there was considerable tumefacation above the eye. It being the close of that term, she was directed to remain at home until the first of June; at which time the tumor had attained the magnitude of the former one though not exactly the same shape and others previously on the side of her face were enlarged. The new one was altogether of a different character from the former. It had the appearance of a spongy mass, (…) the general health was affected and death seemed probable and that speedily, unless its progress could be arrested by a surgical operation, while the heat of midsummer not a little increased the hazard of such a measure. (…) On the first of July the operation was performed. On the first incision being made a large quantity of greenish fluid gushed out from cells of disorganized matter. The tumors above the ear were all removed (…) There was a loss of sixteen ounces of blood. (…) The constitution suffered much more than in the former instance, but she has very much regained her strength and the flesh she had lost, and now looks forward to the prospect of returning home in a few days, with the hope of enjoying a happy reprieve from the grave.

I confess I gasped aloud when I read the details of the case—I am convinced this little girl had NF!  

Neurofibromatosis (NF) is a hereditary condition that leads to, among many other things, the development of both benign skin tumors called neurofibromas, and larger benign tumors of nerves called plexiform neurofibromas.  NF wasn’t described until Friedrich von Recklinghausen did so in the late 1800s.  However, the description of this girl in the case history, the description of her mother, and her appearance all seem to indicate NF to me.  First, the location of her tumor seems characteristic for a facial plexiform neurofibroma, the same type of NF tumor that Jane has on her cheek.  Parker also comments that "the large tumor was surrounded by several small and well-defined ones".  He also mentions that "parts of the body indicated a predisposition to tumors, which I have learned, is hereditary."  The kicker for me is his description of her mother: “The mother presents a most singular appearance, being covered from birth with small tumors, some of the size of large warts, and others hanging pendent, in shape and size like the finger.”

In Parker’s time, any soft tissue tumor could be referred to as a “sarcoma”. This does not rule out the possibility that Akae’s tumor was a neurofibroma, since it was not until after the development of cellular pathology during the middle of the 19th century that sarcomas were separated from other tumors based on their tissues of origin. (Peltier LF, “Historical Note on Bone and Soft Tissue Sarcoma”, J Surg Oncol. 1985 Dec;30(4):201-5.)

Parker did operate on the poor girl, and sadly, within a year later "the tumor had attained the magnitude of the of the former one".  This is often the case with plexiform neurofibromas, which is why surgical excision is not the preferred treatment option.

I myself suspect the regrowth of the tumor has nothing to do with the trauma that Parker blames—if the recurrence was due to trauma, I would expect the contents to be fluid, not "spongiform".  If it were an infectious process, I would have expected the girl to have a fever, or even to have died after 6 months. (She originally presented in December, with her re-operation in July of the following year.)

Akae has captured my heart, and perhaps my imagination.  I am clearly projecting my feelings for my own daughter onto her.  However, even if this little girl did not have NF, I feel a connection to her because of her tumor.  It amazes me that almost 200 years ago, in another country half way around the globe a little girl like my daughter was experiencing the same pains, limitations, perhaps prejudices.  It makes history and the world seem a little smaller.  And the emotions the portrait evokes in me is a testament to the skill of the artist.

I’d like to mention that the NF Mom who brought my attention to the collection is Rachel Mindrup.  She is herself an artist in the Midwest has a son with NF.  In addition to her regular artwork, she frequently paints portraits of individuals with NF.  Please visit her website:

Jane and Family Update:

We will spend Christmas in Connecticut with my mother, aunt, sister and brother, then will head to Canada to ring in the New Year with Todd’s family.  Have a safe and wonderful holiday, everyone!  May 2017 bring many good things.

Friday, November 25, 2016

Running Update:
5.0 mi
5.0 mi
3.2 mi
3.2 mi
5.0 mi
7.6 mi
7.6 mi
5.0 mi
9.1 mi
3.2 mi
7.6 mi
5.0 mi
13.1 mi
7.6 mi
5.0 mi
9.1 mi
5.0 mi

I haven’t updated running log since the Hartford Marathon, for various reasons, but it’s time to report in:  I’m at 992.5 miles for 2016, closing in on 1000.  This is the first time I’ll (hopefully) reach 1000 miles before December.

Our fundraising total for 2016 has increased since I last wrote, too:  were now at $16,714 for the Children’s Tumor Foundation ($24,270 for the Children’s Tumor Foundation and NF Northeast combined).  Thank you, everyone!  After Black Friday and Cyber Monday, if you’re looking to participate in #GivingTuesday, look no further than our fund-raising site:

Thanksgiving Update:
We had our annual pre-Thanksgiving pasta dinner Wednesday night with our dear friends, Neil and Marcia.  Readers of this blog know that Neil, in addition to being one of our oldest friends, is my virtual running partner.  For the past 8 years Neil and Marcia have come to our house the evening before Thanksgiving and the kids help them prepare a vegetarian dinner. We have sweet potato, kale, pine nuts, and sage over egg noodles.  (Comfort food!)  Thursday morning Neil and I run in the Madison Turkey Trot (we both set PRs this year!) with the others cheering us on.  After lunch Neil and Marcia head home, and the kids, Todd and I head to my Mom's where we have a traditional Thanksgiving dinner with her and her sister, Anne.

Today we took the kids to see Fantastic Beasts and Where to Find Them, since we're all Harry Potter geeks :)

All in all, a lovely few days.

Wednesday night dinner preparations.
Neil and Alec tackle the kale...

...while Helen's got the sweet potato...

...and Jane manages the pine nuts.

Jane washing greens with Marcia

Thanksgiving morning, Neil and Kristy trying to stay warm before the Turkey Trot. 
Good friends <3

At the start

There go the trotters!

Waiting patiently for the runners

Kristy at the finish

Mother and son

With the mild weather these past few days, the kids have been enjoying the outdoors.  Helen spent quite a bit of time (and used her considerable talent) decorating our driveway before our guests’ arrival!

Wednesday, October 26, 2016

October 2016 NIH Update:
Jane and I arrived in Bethesda at the National Institutes of Health on Sunday afternoon.  It was one of our smoother trips.  After six years of traveling to and from NIH for Jane’s treatment, we almost have the trip down pat.  The best way to get to NIH from the airport is via the NIH shuttle; however, this only runs on a certain schedule, on certain days.  If we miss the shuttle, the next best option is to catch a cab at the airport and have it drop us off on West Cedar Lane just outside the heavily-guarded NIH campus at a side entrance by the Children's Inn.  Since we have long-term visitor passes, we can breeze past the security gate there and walk about 100 yards up to the Inn.  In the past I would have the cabs bring us through the main entrance--a process that requires the cab driver to exit the cab, have his vehicle wiped down for dangerous substances, and obtain a temporary pass at the security gate.  This always took forever, and never went over well with the cabbies.  It took me this long to learn the short cut!

For our next trip, I'll be able to use my newly-obtained “Known Traveler Number” from the TSA so we can by-pass the long lines at airport security, and the trip will be positively seamless!

Jane on one of the play structures outside the Children's Inn at NIH
Once at the Inn we took our time settling in.  I was looking forward to connecting with another NF Mom, Kelly, who represents Utah on the Children's Tumor Foundation Volunteer Leadership Council.  I had met Kelly online several years before, but we'd never met in person.   It turns out that her son is in the same clinical trial as Jane, and that Jane and I would overlap with Kelly and her family for a day during our return visit to NIH.

We finally met Kelly, her son, Travis, and the rest of their family at the Inn’s Family Dinner that night.  It helps me so much to be able to talk to and share with other families who are going through the same ordeal as us!  After dinner Jane and Travis together enjoyed petting and walking the therapy dogs that were visiting the Inn, and then had a fierce (and giggly) game of air hockey. Travis is Jane's same age, and has siblings the same ages as Jane's.  Like Jane, Travis has a large, troublesome, plexiform neurofibroma (PN), but where Jane's PN involves her head and neck, Travis' occupies the whole length of his leg.

Jane walking Lucy, a therapy dog

Jane with Travis and one of his brothers

Air hockey!

Just a couple of NF Heroes :)

Our first day at the Clinical Center was a full one.

All went relatively well.  Jane even had time between two of her appointments to pet a Guinea pig in the main playroom!

Photography is my favorite appointment

Our least favorite, but nonetheless important, appointment is at the Eye Clinic.  The session is usually very long and the series of tests are challenging (not to mention Jane hates having her eyes dilated), but we usually come away reassured.  This time the ophthalmologist noted for the first time that Jane's left eye protrudes a bit more that the right (a condition called proptosis).  The difference between Jane’s eyes is only a few millimeters, and the doctor said it was possible her eyes had always been that way, but just never previously documented.  Jane also has a known deformity of one of the bones forming her eye socket (sphenoid wing dysplasia, a complication of NF) which could be responsible.

Despite this, the eye doctor was concerned because proptosis can sometimes be caused by a tumor behind the eye.  One such tumor, an optic glioma, is a tumor of the optic nerve, and is known to be associated with NF.  Early on in our experience with NF, Jane was thought to have optic gliomas in both eyes, but her optic nerves, while slightly thicker than normal, have stayed stable for many years.  They've been so stable that our care team at NIH has actually skipped the MRI of Jane's orbits over the past year in the interest of keeping her MRIs a tolerable length (since she had stopped having sedated scans).  However, with the new finding of proptosis, it was felt Jane's orbits did need to be scanned again.

We were scheduled for a non-sedated MRI of Jane's face, ear, nose, throat, and sinuses (where most her tumor resides) that evening, but despite all their efforts, our care team could not get an MRI of Jane’s orbits added to that session.  This meant a second MRI session the following morning, and required contrast (which requires an IV).  In other words, Jane would have her 20th and 21st MRI during the same NIH visit. 

Jane did not take the news well.  She had cooperated so well with her MRI that evening.  Staying completely still for that long is so hard when you're nine!  It wasn't fair to have to do it again!

A spooky dinner: mummy dogs, zombie toes, Jack-o'-lanterns, and blood

For dessert: chocolate pudding "dirt" with gummy worms!

We didn't enjoy our Family Dinner that night as much as we might have (“mummy” dogs, “zombie toes”, and “blood”) but Jane’s mood improved tremendously when after dinner she met another little girl playing hide and seek with her mother.
  Jane introduced herself, and the two were immediately off playing in all sorts of places around the Inn.  The little girl was Lois, she was Jane's age as well, and had traveled all the way to NIH from London! Lois has a genetic condition which causes tumor to grow in her endocrine glands, and, by this young age, had already had her thyroid removed and had a tracheostomy. After hiding and seeking for a while, Lois and Jane had the opportunity to paint pumpkins!

Jane with Lois in the craft room
The following morning, we were back at clinic bright and early, before any other patients, to have Jane's IV placed.  Jane was still quite upset about having yet another procedure, and, alone with her in the waiting room, I was running out of ways to console her—she rebuffed all of my attempts.  Then, by a stroke of good fortune, Lois and her mother arrived at clinic!  It was like the sun breaking through the clouds--Jane's tears stopped flowing and she greeted Lois with a watery smile.  The girls ended up playing a quick round of bowling before Jane and I had to leave for Radiology.  I thanked Lois for her smile, and thanked her mother for being there for us right when we needed her.

Jane’s MRI ended up being delayed for over two hours (which did not improve our mood).  Despite this, Jane sat still through 67-plus minutes of scan time, longer than she’s ever done without sedation.  Due to the delay and the length of the scan, we were behind schedule to get to the airport.  After leaving Radiology we had to dash to collect our belongings at the Inn and catch our cab (but not before allowing Jane to select a prize from the prize chest in clinic—the promise of which got us through the final minutes of her MRI!)

While on our way home, Jane and I were discussing the long and unexpected MRI.  I told her I was proud of her, and that she was a strong and brave little girl.  I told her, “I don’t know any other kids who can do what you do.”  Immediately, Jane responded, “I do.  Travis.  And Emma.  And all the other kids with NF!”  This is why we call them NF Heroes!

Driving home from the airport that night, we happened to catch up to the bus that was bringing Jane’s sister, Helen, home from her high school soccer game!  Instead of turning for our house, we followed Helen's bus all the way to the school parking lot!  Jane jumped out of the car and ran over to give her big sister a hug as she got off the bus!

We won’t have results on Jane’s MRI for another day or so, but will update you all as soon as we have them.  Thank you all for your love and support!

This just in!!

Email from NIH:

The MRI that J did was excellent, she did a very good job.  J’s plot and table are attached.  Her facial tumor is extremely stable compared to last time and currently has decreased by 29% compared to baseline.  Very good news! 

The orbit MRI was reviewed by our neuro-radiologist and our team and we do not see any optic nerve gliomas, this is very reassuring.

Whew!  I was fearing new growth.  I'm content with "stable" this time. <3 <3 <3

Monday, October 10, 2016

Running Update:
10/8/16  26.2 miles in 4:28:25
Another race done!  Yesterday was the Eversource Hartford Marathon.  We had a cool, overcast day, but the rain held off, so it was good weather for running.  I wasn’t my best time, but it also wasn’t my worst, and I am pleased to be able to say now that I have run 10 marathons in order to help #EndNF!

At mile 6, still fairly fresh

Running through Soldiers and Sailors Memorial Arch to the finish line

Finish line!

All done!

The Family

Marcia and Neil, my running partner

The Bling

This post, courtesy of Women’ Running Magazine, sums up how I feel about racing:

     pre-race: why did I sign up for this? 
     mid-race: why did I sign up for this? 
     post-race: omg so happy I signed up for this! 

Given that, I’ve signed up for the Guilford Half Marathon on November 13 :)

NF Update:
In 2016 so far, Jane and I have fundraised
for the Children’s Tumor Foundation!  Add to that the $7556 we solicited for NF Northeast for the Boston Marathon this spring and you can see we have raised $22,337 for NF charities in 2016!  Thank you to all of our wonderful and generous donors!

If you haven’t donated yet this, you still can!  (You could be the one to put us over $15,000!) Our site will be open until the end of the year:

Jane Update:
Jane and I return to NIH in less than two weeks for her next check-up and MRI.  We will, of course, keep you posted here.