Karin
is our second child and first daughter. She was born in Ann Arbor , Michigan Sandy ’s
dismay, Karin arrived about four weeks later than the due date given her by the
OB/GYN in Gainesville
The
very first day Sandy and I brought Karin home from St. Joseph Mercy
Hospital
As
an added bonus, Karin almost never cried. When she was hungry she would whimper
or protest a little but then was quiet until we fed her. She seldom fussed or
complained for more than a matter of seconds. We would have to forget to change
her diapers for a considerable time before she would protest, and then it was
never a loud, prolonged, or outraged crying. In addition, she was never ill.
Never once in her first year.
None
of our friends with infants of a similar age or slightly older could believe
how sweet natured and content she was. All we had to do was put her in the
pumpkin seat or the wind-up swing and she would be happy for hours. Literally
for hours. She was in so many ways our angel baby, which is what we called her.
On
Halloween 1971, Sandy
Soon
after giving Karin’s name at the reception desk we were called in, right on
time, which was a rarity as the pediatrician, Dr. X, was popular and was
almost always busy and behind schedule. And therefore typically late with all his appointments. I
put Karin on the examination table, removed her top, and sat on a nearby chair.
We had both been through the procedure several times before and she enjoyed it,
like most things in her life. Karin, as always, was in a happy mood and smiled
like she had invented contentment.
As
soon as the doctor came into the examination room he immediately pulled up
Karin’s undershirt and listened to her heart. And listened, and listened.
Usually Dr. X was relaxed and friendly but that day he seemed a bit
short-tempered and brusque. Then he had her sit up and put the stethoscope to
her back and listened, and listened, and listened some more, moving the
instrument around quite a lot, even under her arms. All told, he must have
listened for several very long minutes. It was obvious that he was not
following his regular procedure. I became progressively uneasy but didn’t want
to say anything because he was so intent on listening. After removing the
stethoscope, he turned from the examining table and sat at the small desk against the wall and began
writing in Karin’s medical records. And wrote and wrote, without saying a word , which I thought very strange. Almost in defense I picked Karin up and
held her close as he wrote in silence.
Finally,
not able to stand the tension mounting within, I asked, “Is
something wrong? I mean, you were listening to her heart for quite some time.”
My voice seemed squeaky with apprehension.
Without
turning to look at me, and continuing to write as he spoke, he said, “It’s as I
diagnosed at birth. Karin has congenital heart disease.” He never glanced
at me. Had he done so he would have seen the stunned look on my face.
Congenital heart disease? What the hell was that? I didn’t know what to say or
do. I sat there, totally confused. My perfect baby had a disease? A congenital
heart disease? What the fuck was that? My brain refused to function. I was so shocked I could have
fallen on the floor.
At
last he put his pen down and turned toward me. “Mr. Ernst, it’s just as I
initially diagnosed the day Karin was born. I detected an abnormal heart sound
then but, because that’s not at all unusual at that early age, I said nothing.
Many babies have similar abnormal heart sounds that disappear in the normal
course of growth and development. However, as you can see from my records,” he
gestured to the document on the desk, “I noted the murmur at each succeeding
office visit. At three, six, and nine months.” He gestured for me to come over
to his desk. “Come and see. I made these notes the first time I examined Karin
at the St Joseph
Sure
enough, his scribbled notes read, “Irregular heart sound. Murmur. Check heart
carefully at three months exam.”
“That’s
why I asked to see her at nine months. After six months, I usually see normal
babies at their twelfth month, not nine. But I wanted to keep track of Karin’s
problem. Today, the sound seems louder and more distinct than before.
Consequently, the chances of her growing out of it are almost certainly
non-existent.” He stopped, looking at me expectantly, waiting for a response.
I
had no idea of what to say, what to ask. Had I missed something? A cue,
perhaps? This role, as the parent of a child with a disease, was one I had
never imagined I would be playing. I was out of my depth. But I had to say
something.
“What
does that mean? What, what are the implications?”
“Quite
simply it means we have to watch her very closely for the next nine or ten
years or so. Probably some time after age ten she will require open-heart
surgery to correct the defect. But that probably won’t be until she is between
twelve or thirteen years old. This kind of heart defect is best dealt with at
that age. When her body is nearly full grown. That way the repair will be
permanent. We’ll just have to keep our eye on her to make sure she doesn’t get
really sick and develop pneumonia, because a significant part of her blood is
not circulating through her body. So, in the interim, there’s plenty of time.”
For
what, I wondered. Time for what?
The
rest was a blur, an impenetrable fog bank. I know he told me more about the
type defect she had but I wasn’t able to retain it. It was like I was wrapped
in a thick blanket of insulation. The only thing I remember about the rest of
that morning is driving home and looking at my perfect angel baby, so beautiful
and healthy-looking, never sick a day in her twelve months of life. My
daughter. Thinking that she was not the same as she was a half-hour ago. That
on the trip to the doctor’s office she had been perfectly fine and now she had
congenital heart disease. I had no idea of how I would break the awful news to
San.
* * *
Telling
San was a horrendous chore I dreaded. I sat around the apartment for an hour,
wondering how to do it. San didn’t take bad news well, to put it mildly.
Unexpected bad news always upset the shit out of her and this was about as
unexpected and bad as it came. When she and David arrived, flushed with
excitement from the Halloween party, I felt a tremendous urge not to tell her,
to put it off until tomorrow. But I had no choice. I had to tell her then.
After she settled the kids down for their afternoon nap and we both went to the
kitchen for a sandwich I knew I could not eat. Off-handedly she asked me how
the doctor’s visit had gone. Expecting the usual answer. It went fine. I was
sitting the table and told her to sit down. She immediately got a strange look
on her face and asked me what was wrong.
“Is
Karin sick?” she asked. Her voice held the first inkling of apprehension.
I
took her hands in mine, feeling stupidly dramatic but not knowing what else to
do and said, “No, Karin’s not sick but something is wrong with her. Doctor X
told me that she has congenital heart disease.” Even though I said the words it
was as if they had no meaning.
Her
reaction mirrored mine. “Congenital heart disease? What’s that?” She started to
get up from the chair but stopped half way up and gave me a hard look. “Bob
Ernst, if this is another one of your stupid practical jokes, I going to kill
you.”
“Sit
down, San,” I said. “This is serious. Dr. X said he noticed the problem when
she was born but thought it would go away on its own. A lot of babies are born
with abnormal heart noises that don’t develop into anything. Karin’s problem
didn’t go away and it seems to have gotten worse. She’ll need an operation in
about eleven or twelve years.”
“An
operation? Oh my God!” Her eyes teared up immediately. “My baby has to have a
heart operation?” The tears streamed down her face as I held her and tried as
best I could to comfort her.
The
rest of that day and the next were straight from hell. Neither of us could
believe it. Karin was such a perfect baby in every way the idea of her having a
disease from birth was almost incomprehensible. San couldn’t accept the
diagnosis but refused to talk to the doctor. When I suggested that we make an
appointment for both of us to go see him she refused. It was almost as if she
did not hear the words from his mouth then the situation wasn’t real. I tried
to get her to talk about it but she became angry. So I dropped it.
The
doctor had scheduled Karin’s next check-up for the end of February, when she
would be fifteen months old. When the time arrived I insisted that San and I go
together so we both could ask whatever questions we wanted and hear exactly
what he said. We were both very tense and stressed out by the time we were
called into the examination room. The exam went almost exactly like the
previous one. Dr. X listened and listened to Karin’s chest and back and then
went to his desk and wrote his notes. But afterward the good doctor took a
little more time to explain the problem.
Karin
had a hole between two of the chambers in her heart. As a result, a significant
amount of blood was not circulating through her body. The surgical procedure to
correct the defect was fairly simple, as heart surgery went. But it had to be
postponed until her body had neared the end of the pre-teen growth spurt, when
the heart had reached its maximum size. He said that the murmur remained quite
distinct and might even be a little more pronounced than the last time. He told
us to bring her back in another three months.
That’s
when, out of the blue, I asked if he could refer us to a heart surgeon or a cardiologist so we
could get a second opinion. At that point he became rather testy, saying that I
was jumping the gun by many years and that Karin’s condition simply wasn’t
serious enough to warrant seeing a specialist. When I insisted, he very huffily
wrote two names on a prescription form and left the room, making a sarcastic
remark about over-protective parents that I have long since forgotten.
I
spent the rest of the day trying to convince San I was right in taking Karin to
see another doctor. She agreed with the doctor and thought I was over-reacting.
All she wanted was to put it out of her mind as quickly as possible. To forget
Karin had a problem for ten or twelve years. But I was absolutely determined.
A
very nice couple with two children five or six years older than ours lived
immediately upstairs in the apartment building. The husband’s name was John but
I have forgotten the wife’s name and their last name as well. John was a very
bright guy who I liked a great deal. He and I played chess periodically and
chatted quite a bit in the hallway and outside about our kids and life in
general. More importantly, he was a fourth-year resident in radiology at the
University of Michigan Hospital. That night I went upstairs to talk to him. I
told him the whole story about Karin and Dr. X. I asked him if he knew the
doctors whose names Dr. X had so grudgingly recommended. He looked at the piece
of paper Dr. X had given me and said that he knew both doctors. And that they
both were well regarded cardiologists. From his tone I knew he wanted to say
more so I prodded him, asking what he would do if one of his own children had a
similar heart defect. Without a word he wrote two more names on the paper and
handed it back to me.
“I’d
take my kid to see one of these two guys,” he said without hesitation.
I
looked at the names, not recognizing either. “Who are they?”
“They’re
both thoracic surgeons at the University of Michigan Hospital
After
a little more prodding he admitted that he would call the first name on his
list as soon as possible. Tomorrow, in fact. When I questioned him as to the
reasons for selecting that particular surgeon he told me that he was the most
skilled and the fastest thoracic surgeon in Ann Arbor
And
that’s how, the very next morning, I came to call Dr. Otto Gago, over San’s
strong objections. In fact, she was so upset that she hardly spoke to me all
that day. Two days later the three of us were sitting in Dr. Gago’s office at St. Joseph ’s Mercy
Hospital in Ann Arbor Caracas , Venezuela
After
talking to us for a few minutes about Dr. X’s diagnosis (he had copies of
Karin’s medical records in front of him) he helped Karin to take off her top
and listened to her heart. I know that I was holding my breath and thought San
was as well. Hoping beyond hope.
After less than thirty seconds he removed the stethoscope and placed it
on the table. To my surprise he thumped the examination table rather sharply
with his hand.
“No, no!” he exclaimed sharply but not loudly. “Dr. X is quite
wrong. Karin has a defect but it is not a hole in her heart.” He smiled
reassuringly at us. “You see, many pediatricians have limited exposure to
persistent heart defects. Consequently, they have some difficulty in diagnosing
those conditions correctly. Your little girl has a PDA, which is patent ductus
arteriosus. In Latin, patent means ‘open.’ The ductus arteriosus is a shunt or
a small blood vessel that is connected to the pulmonary artery, the main vessel
leading to the lungs, and to the aorta, the main vessel of the body. You should
know that PDA is present in all babies before birth. In utero, the baby’s lungs
are not used because the fetus gets its oxygen from the mother across the
placenta. The ductus arteriosus is part of the fetal pathway that helps to
distribute oxygen from the mother to the fetal organs and allows the blood flow
to avoid the lungs, which do not need a lot of blood at that time. When the
baby is born, the lungs expand, their blood vessels relax to accept more flow,
and the ductus arteriosus usually closes on its own within the first 15 hours
of life. Which is one reason why Dr. X should be congratulated for detecting
the problem at that first examination. However, as you now know, sometimes the ductus arteriosus does not
close on its own. That is why it is referred to as a patent, or open, ductus
arteriosus. And although this condition is much more often seen in premature babies,
it may also occur in full-term infants. Like Karin.”
“You
mean she doesn’t have congenital heart disease?” San’s eyes pleaded for him to
give us the only answer she wanted to hear.
“Oh,
no. About that Dr. X is absolutely correct. And he should be congratulated on
catching the defect as promptly as he did and for following it so closely.” He
handed his stethoscope to San. “Please, put the tips in your ears and the metal
cone on Karin’s chest and listen carefully. You should be able to hear the
loud, regular lub-dub heart beat. But in the background, if you listen very
carefully, you will hear a soft but distinct swishing sound. That will be the
blood moving from the aorta to the pulmonary artery through the shunt. Can you
hear it?” he asked, leaning forward as if to encourage her.
San’s
face lit up with amazement. “Why yes, I do. Actually you can hear it very
easily.”
My
turn was next. And there was no doubt, the sound was easily detectable, even to
my untrained ears. How strange it was to hear first-hand what previously had only
been a fear-producing phrase. Congenital heart disease. I felt at once relieved
and even more worried. Now our baby’s problem was a concrete reality. But it
was one we had to face squarely. And I was determined to do just that.
“How
many years before Karin will need surgery,” I asked innocently. Wanting but not
wanting to hear his response. Praying that she would never need it. That Dr. X
was wrong about that key point.
“Years,
Mr. Ernst? I’m afraid your daughter will have to have an operation as soon as
possible. This summer is the absolute longest you should wait.”
BOOM!
It
was a bomb exploding in front of our eyes. My stomach dropped away, like I was
ridding an express elevator to Hell. His words not only took my breath away but
I couldn’t breathe. When I could bear to look at San she was as white as
a ghost. For a moment I thought she would faint.
“This
summer! Oh, Bob. What are we going to do?” San quickly turned and looked at the
doctor. “Is there any alternative to surgery?” She looked completely lost and
helpless as Gago shook his head.
A
terrible lump sat in the middle of my throat. It was fairly easy to accept that
Karin would need an operation some time in the far distant future. Ten or
twelve years is an eternity. But four months? That was almost unthinkable.
“Let’s
schedule it for the end of May when your classes are over. Or June,” San said
in an outwardly calm voice. “That way you’ll be on summer break. It’ll be
easier on the both of us if you’re home.” Her eyes gave the lie to her steady
tone.
“What
would you recommend?” I asked Gago, trembling inside, knowing that that awful thing
inside Karin was not going to get better as time passed.
“In
all honesty, I must tell you that in a case like this sooner is much better
than later. We cannot predict how fast the shunt will grow. Or if it will grow
at all. But waiting is not a strategy I would recommend.”
That
did it for me. “Let’s schedule it as soon as possible,” I said firmly, with far
more strength than I felt. “There’s no reason to wait until summer.” I couldn’t
look at San but felt her eyes burning into me. I knew she wanted to put it off
as long as possible and was angry with me.
The
doctor reached for the phone on his desk and told his secretary to contact both
Ann Arbor
Gago
spoke for only a few seconds before placed the phone back in the cradle. “An
operating room is available in the middle of March here in St. Joseph University Hospital
“Let’s
do it in March,” I said without hesitation and as I looked at San.
Dr.
Gago turned to San and asked, “Mrs. Ernst? What do you think?”
She
sighed before answering. “Okay. I don’t like it but March it is.”
“Good,”
he clapped his hands. “That was a hard but wise decision. Now it is my duty to
caution you about the difficulties in this type of thoracic surgery. Actually,
the surgical procedure will not involve the heart directly but only the two
major arteries, the aorta and the pulmonary. In itself, the operation is
relatively short, safe, and simple. But any surgery in the chest cavity is not
to be undertaken lightly. We have to be concerned with many variables. The
child’s reaction to the arteriogram that will precede surgery. The anesthetic
during the operation. The operation itself. Possible post-op infection. And,
most seriously, pulmonary hypertension.”
The
way he paused in what was obviously a well rehearsed and frequently given
speech and looked at us expectantly told me that this was a problem he wanted to avoid.
“What’s pulmonary hypertension and how dangerous is it?” Ever the university
professor, I always had to have maximum information.
“Well,
it can be extremely dangerous. Hypertension occurs after thoracic surgery in approximately
three to five percent of patients. Why it happens is unknown. The causes are
many and complex and have proven difficult to unravel. But the critical fact is
that more than 75 percent of post-operative patients who develop hypertension
die.”
He
pursed his lips into a tight, thin line. “It’s a risk you have to take because
without this operation your little girl will probably not survive three or four
years at most. If that. With a successful operation, her cardiovascular system
becomes normal and the prognosis is that she will have a normal life span.” He
shrugged his shoulders and spread his hands expressively. “As Karin’s parents
it is your decision.”
We
looked at each other for a brief second and simultaneously said, “March.”
“Okay.
Before we leave, can you tell us what’s an arteriorgram,” I asked, feeling lost
in all the unfamiliar medical jargon.
“Of
course. It’s a fairly simple procedure that involves injecting a dye directly
into the coronary arteries so we can see the problem. It’s classified as an
invasive test because it requires passing a tiny catheter from the femoral
artery in the groin up through the aorta to just above the heart where the
coronary arteries start. The test will show blood flow through the coronary
arteries and is the most direct method for detecting the specifics of Karin’s
problem. Seeing the blood flow, the size and the position of the shunt, will
allow your cardiologist, Dr. Paul White, and the thoracic surgeon, which is me,
to identify the best surgical procedure.”
“When
does Karin need to see the cardiologist,” San asked.
“As
soon as my secretary can schedule it. This week I hope. I’ll have her call you
with the time. He’s located here, just down the hall." He smiled at San and patted her arm, “So you can relax,
Mrs. Ernst. Your daughter is in excellent hands.”
[1]
Strange, but for several decades, I remembered Gago as being from Argentina
