Running Update:
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11/27/2016
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13.2 mi
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2:06:53
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11/30/2016
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7.6 mi
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1:12:39
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12/2/2016
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5.0 mi
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46:04
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12/4/2016
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9.2 mi
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1:28:29
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12/7/2016
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5.0 mi
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47:01
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12/9/2016
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5.0 mi
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46:49
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12/11/2016
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13.2 mi
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2:09:47
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12/14/2016
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3.2 mi
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27:08
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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
and the portraits: http://cushing.med.yale.edu/gsdl/collect/ppdcdot/
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.
