Wednesday, December 21, 2016

Running Update:
11/27/2016

13.2 mi
2:06:53
11/30/2016

7.6 mi
1:12:39
12/2/2016

5.0 mi
46:04
12/4/2016

9.2 mi
1:28:29
12/7/2016

5.0 mi
47:01
12/9/2016

5.0 mi
46:49
12/11/2016

13.2 mi
2:09:47
12/14/2016

3.2 mi
27:08

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: http://library.medicine.yale.edu/find/peter-parker
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: http://www.rmindrup.com/.

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.