Phlliddl

Thursday,- JanurlrT 12: 1978

           ~heclk#:?o'-Th@nql~~,f ED ECUSTEIN, Childreti Ho&al
Stmgeons bend over ,the.?ying dying * separation operation

The, Twins-tD&i@ioti

-&by fys
final crisis

By Don&d C. Drake           It was not the abnormal heart that
h~~irer MedicnI Writer         eventually killed Baby Girl B, but
Baby' Girl B, the Siamese'twin who  liver trouble .and an infection that
had been given a chance to five be  doctors could not stop-not even with
cause of a dramatic operation, died . the strongest antibiotics. This is the
yesterday at Children's Hospital des-  story of the efforts to keep her alive
pite desperate attempts to save  after the operation Oct. 11.

her.

The baby's .dea'tb came 92 days
after surgeons senarated her" from 5
her sister, Baby Girl A, whom sur-
geons purposely let die `during thetop-.'
eration so that Baby girl B might,
live. :

At birth four-months_ ago, the tW0
girls had shared' only lti ..hearts,4
which at best were strong enbugh ta`
`support only one of. them.

All along, doctors had feared that
the"abnorma1 `heart could not survive
surgery. The thought had been that
-the heart might stop the minute they
cut .+e second child from her sister,
Or,, If she. survivg,thatQ part of the
procedure, that ths`hkurt could not:.
continue functioning since it was so
abnormal,

"But the heart did remarkably' well.
   (See TWIN on 4-A) '


                                     PhiladelPhia lncwirer I ROBERT L. MOONEY
A saddqed Dr. Louise Schnaufzr describes the efforts tc save the child


The hospital surgical team had hundreds 6f tools on hand during the lengthy operation to separate the two baby girls

The last crisis of Baby Girl' B


TWIN, From 1-A

Even as recently as a week ago,
Dr. Paul Weinberg, the cardiologist
for Baby Girl B-ail family names
have been withheld to protect their
privacy - had said that .everything
looked fine.

The heart, wth six instead of the
normal four chambers, was beating.
forcefully and in proper rhythm.
Dr. Weinberg said it was impossi-

ble to say how the extra two cham-
bers - from Baby Girl A - were
faring. But the abnormalitv was not
interfering with the functioning of the
heart.

But strong heart or not, Baby Girl
B encountered one crisis after
another. She repeatedly became in-
fected because she had been made so
vulnerable by her weakened andk
tion and the many insertions of tubes
into her body.
At one time she suffered a cardiac
arrest when she breathed in, or asm-
rated, her food, but emergency mea-
sures pulled her through.
8 The clotting factors in hei- blood
periodically dropped t.o low levels.
threatening internal bleeding or a
brain hemorrhage.
The nursing care was intense. At
first a nurse was assigned soiely to
her, around the clock, three shifts a
day.'

Every three hours nurses would
vibrate her chest and use suction
devices to clear her windpipe to keep
it free of fluids. They would also
check the ventilator that was breath-
ing for her and make sure that the
intravenous lines and arterial lines
and feeding tubes were clear.  `:-'
The overhead warmer was m&i-

tored constantly to make sure that
her skin temperature always stayed
at 36.5 degrees centigrade ( 97.7 Fah-
renheit).

Drugs, fluids and other supple-
ments were given to keep in propsr
balance the constantly changing bio-
chemical levels of her body.
A bad sign
She seemed strong and alert, and
for same time it seemed that the
medical team was pulling her
through. But despite everything done
for her, one ominous reading re-
mained dangerously high.      

This was the level of bilirubin, a
biochemical made from worn-out
blood cells that the body normally
disposes of. That she had too much
bilirubin could mean only one thing:
Baby Girl B's liver was not function-
ing properly.
The doctors had not expected pob-
<Iems with the liver. The sisters had
shared a fused liver as well as a
heart, but the livers are compara-
tively easy to divide in Siamese twin
separations. .

On two occasions after the sepa-
ration, the surgeons took biopsies, or
samples, of the liver to see whether
Baby Girl B's was badly diseased or
damaged.

The pathologists were shocked by
what they saw under the microscope
on both occasions.

Looking at samples taken from
three different parts of the liver, Dr.
C. L. Witzleben, chief pathologist,
could find no normal liver cells.
Judging from the samples, the twin's
liver was essentially dead. And with-
out a liver, the child would be dead
within 24 hours.    " 1
The surgeons later determined that
they must have inadvertently taken
samples from the scar site where the
liver had been separated from Baby
Girl A's during the surgery. But in
any event, something was decidedly
wrong.

Dr. Philip G. Holtzapple, a liver
specialist, was summoned.

Functioning

Tests indicated that the liver was
still performing the functions on
which Baby Girl B's life depended. It
was still releasing glucose needed
for energy, it was still making the
substances that prevent internal hem-
orrhages, and it was cleaning the
blood of toxins such as ammonia
from the intestines.
But in liver disease, these functions

are the last to cease. The continued
high bilirubin level indicated that the
liver was being destroyed more
every day.
By last week Dr Holtzapple held
out little hope. The destructive proc-
ess had been going on too long.
At best, Baby Girl B had only a
few years to live. At worst, death
could come suddenly.

One of the questions that had been
raised was whether the physicians
should try to resuscitate the baby if
she had another cardiac arrest.
If she had no future, why subject
her and her parents to the trauma of
life-saving attempts that at best
would put off death by only a few
weeks or months?

But on the (basis of the biopsies, Dr.
Holtzapple could not be absolutely cer-
tain. He could not put a figure on the
child's chances; certainly they were
less than 10 percent, probably much
less.
The doctors therefore felt that they
could do nothing less than everything
to save Baby Girl B should her heart
suddenly stop.

Beginning of the end

The firt sign that the end was be-
ginning for Baby Girl B came Mon-
day when doctors and nurses noticed
a swelling around her eyes.
She became listless and lost inter-
est in the pacifier she had been suck-
ing on. The acidic level on her blood
went up:.
The acidic levels got worse the
next day, and on Tuesday the plate-
lets, clotting factors in her blood,
began to drop.
This was strongly indicative of an
infection coursmr throwh her blood.
Antibiotics were pumped by intrave-
nous line directlv into her blood.
But they were-for naught.   ~~-

The liver disease and the tubes into
her body had left her too vulner&Ie,
and the infection could not be stop-
ped.

Her heart stopped at 2 a.m. yester-
day.

Emergency call

The nurse on duty immediately de-
tected this, and an emergtmcy call
for help was sounded. A dozen doc-
tors and Nrses swarmed into Room
4016 - one of the six pediatric inten-
`sive-care isolation units.
Under the direction of Dr. Colette
O'Keeffe, the anesthesiologist on duty,
and Dr. W. Clark Hargrove, the sur-
geon, the quickly assembled team
struggled to restart the heart.
The drugs au-opine and epinephrine
were injected into her circulation to
make her heart beat faster and with
more force.
Sodium bicarbonate was given to
counteract the acid that was building
up in her blood - a lethal waste
product of oxygen-starved cells that
were dying.

Calcium was given to help the
heart.

Baby Girl B's heart rate, which
should have been 110, slowly climbed
from 50 to 100 as the drugs took ef-
fect.

But then down it went again, to 30
this time.
More atropine, calcium, T e@eph-
rine were injected.


Dr. O'K&fe pressed down hard on
the baby's chest, squeezing the stilled
heart between the bones of the chest
and meclia&lly forcing th6 blood to
circulate..

The heart rate went up again but
only to 90 this time.
This process was repeated again
and again. Each time the heart re-
sponded less effectively.
Dr. C. Everett Koop, chief of sur-
gery at Children's and the baby's pri-
mary physician, was on vacation in
Texas. Rut his assistant, Dr. Louise
Schnaufer, related the anxious devel-
apments by telephone.
His orders to her were to continue
the resuscitation attempt even though
$; responses were becoming negligi-
I&nally, a little before 4, two hours
after the heart had stopped, the doc-
tors could get no response with any
of their drugs.

Baby Girl B wae declared dead at
4:04 a.m.

`)Doctors will never know why the

vg failed, Tbe+arents, Orthodox
ews, refused to permit an autopsy.
Rabbi M. D. Tendler, an authority
on Jewish law, had advised the par-
ents, who called him yesterday morn-
ing, that an autopsy would be per-
missible in such's case as this.

In fact, he said, there was a reli-
gious obligation since such an au-
topsy would not be a "fishing trip"
but something that might help pre-
vent similar deaths in the future.

But Baby Girl B's grandfather,
bimself a rabbi, felt differently, and
the family declined.

Why did Baby Girl B die?
Yteday,  Rabbi Ten&r et-
tempted an answer:

"This big question of Why do the
tests of humility and faith - humility
because we don't have a handle to
analyze it and faith that there is a
master scheme for the world.
"One thing we oan be certain of-it

was not an act of randomness. It was
not an act of arbitrariness. There
must be a scheme to this if you have
any concept of God as being an om-
niscient being . . . a rational being
and not a fellow standing up there
and playing games, amusing himself
at our expense. There must be a
ratio&s to all of this."