Amy
 One of the struggles of
having a congenital heart disease is the challenge of finding appropriate care within
the existing health care system. My own story, although
perhaps extreme, is a lesson in how easily congenital
heart patients are poorly served.
What is particularly striking to me is that, when I
set out to get good health care, I had every possible
advantage. I am a well-educated patient with excellent
health insurance options and good financial resources. My
defect was accurately diagnosed early, I have always
followed doctor’s orders, and I consistently sought
out the best quality care. Yet despite these advantages,
I slipped through the cracks repeatedly, and have had
crucial care prevented and delayed. In this story, I have
left out names and some details, using descriptive names
like The National Expert instead. This is not an
indictment of a particular hospital or doctor. The
problem is a health care system that virtually guarantees
poor care for the adult congenital patient.
I was born light blue and with a murmur, and was
immediately referred to one of the country’s top
pediatric cardiologists. My defect, an unusual one, was
diagnosed accurately at age 5, at which time I was a
thriving and vigorous kindergartner. The diagnosis was
presented as good news, in that my family was told that
my health would continue to be excellent. In adolescence,
I myself began to ask the hard questions – would I
deteriorate over time? Could I have a baby? All the
answers were reassuring. I was told that my heart,
although unusual, worked well and there was no reason to
suspect that it would weaken. As for pregnancy, there was
no reason why not.
Like many patients, I kept seeing my pediatric
cardiologist until after college, at which point sitting
in a room with babies became increasingly uncomfortable.
I knew I had questions about birth control, sexuality,
and life-style options which I just couldn’t bring
up with this lovely lady who felt almost like my
once–a-year Grandma. I also had my first job, and my
HMO certainly didn’t cover trips out of town for
cardiac care. I called my pediatric cardiologist and
asked her to recommend someone local, but her
recommendation was also not covered under my plan. In the
end, I made an appointment with the head of cardiology at
a prominent teaching hospital, assuming that this was the
way to get quality care.
This was my strategy throughout my twenties. With
every move and change of health plan, I went to the head
of Cardiology at the most respected medical school
hospital available to me. Each of these physicians were
reassuring to the point of condescension, one going so
far as to interrupt me when I described myself as having
a heart "problem", saying, "you don’t
have a problem – your heart works perfectly".
When I asked about phrases I had overheard in the echo
lab – "1st degree AV block",
"moderate mitral regurgitation", rather than
answers, I was told again that I was fine and I should
just stop worrying.
Now that I have the records from these visits, I can
see that during these years I was deteriorating, and yet
no one told me. At the time I knew that my lack of
knowledge about my condition made me uneasy, but I
assumed that, since I was seeing the city’s
"best" cardiologists, there must not be
anything to worry about. When I got pregnant, I was told
that I needed no special care, and after 2 days of labor,
I delivered my daughter by unplanned c-section.
After the birth of my daughter, I did not see the
Cardiologist for over two years. Nursing and working
full-time, I was struggling to get through my workday,
falling asleep at 7:00 and out-of-breath every time I hit
the stairs. I told myself that this was what having an
infant was like, and chalked up the breathlessness to
being out of shape. But as my daughter grew, I found that
the demands on me were lessening, but my fatigue
wasn’t. My husband and I were also planning our
second, and I knew I did not feel well enough to go into
a pregnancy with confidence. So I went back to my
physician, who ran a bunch of tests, all of which were
negative. Looking at my chart, she saw that I hadn’t
been to the Cardiologist in a while, and suggested a
neighborhood Cardiologist who was particularly caring and
competent.
I often wonder what went through my doctor’s head
as I chatted away about my unusual but benign condition,
while she listened to my now very alarming sounding
heart. I headed into the echo lab, where for the first
time in many years, I was allowed to watch the monitor.
It only took one look at the multi-colored mess on the
screen for me to know that something was now terribly
wrong. My doctor could offer little information or
reassurance, since she was not an expert in my condition,
but she promised to do some research.
Within a week she had found information for me that
stated that pregnancy had been shown to damage my kind of
heart, and that the current median age of survival for
patients with my defect was 38. She advised me to change
my health insurance immediately, and explained that it
was imperative that I see an out-of-state expert on my
condition. Until that could happen, she wanted to send me
downtown to see a prominent echocardiogram expert. Tape
and husband in hand, I headed off, terrified about what I
was going to hear.
When I arrived at The Echo Expert’s office, my
first question was whether he had looked at the echo
tape, and he assured me that he had and he saw nothing to
be concerned about there. He examined me carefully and
declared that I was in excellent health, should enjoy a
normal life span and have no trouble with a second
pregnancy. He then sent us, breathless with relief, to
get a new, better-quality echocardiogram. Two hours later
the doctor walked in again, red-faced and visibly
agitated. I’m terribly sorry, he said, but your
condition is not as good as I thought, and not only
should you put the second baby on hold, but you may need
a transplant.
The question of what surgical options were still
possible for me now became critical. My doctor said that
she wanted to send my records off to a national
congenital heart surgical center, to The National Expert,
and stated that until I heard back, I should not make any
assumptions about my prognosis.
Compiling these records proved to be extraordinarily
difficult. Whereas one call to my pediatric center
resulted in a pile of information dating back to 1965,
the other hospitals had uniformly lost key information.
The echo reports we did find were incomplete and
confusing, often using incorrect terms to refer to my
anatomy. At two hospitals I was nowhere in their records,
and I was told that I could not have ever visited their
facility. Since the question of how quickly I had
deteriorated was crucial in assessing my current
situation I persisted, tracking down a key echo report in
my former GP’s attic. Only with the echo reports in
front of them were the hospitals willing to even look for
my old tapes, and in the end I only succeeded in finding
one. I then went away for the summer, assured by my
doctor that, when I returned in two months, I should have
heard back from the experts.
When I returned, I found that not only was there no
news, but the records had not yet been sent. Apparently,
because my HMO was disputing the need for me to be
referred for a second echocardiogram, The Echo
Expert’s hospital was refusing to release my
records. The next day I was at the records office, where
as is mandated by law, my records were immediately
released to me. Seven months after the need for a
consult became apparent, the information was finally
sent.
In addition to a consult about my condition, my doctor
had also asked The National Expert to recommend a
congenital heart practice which could take over my
routine care, since she knew that my condition demanded
congenital heart specialists. But I needed a new echo
before we heard back, so she sent me to see The
Transplant Doctor. Highly esteemed locally for both her
technical and personal skills, it was clear that, if and
when I needed a transplant, she was the one to do it.
When making the appointment, my doctor emphasized that by
no means did this mean that I did need a transplant, and
that ultimately a congenital heart center, not The
Transplant Doctor, should be overseeing my care.
The Transplant Doctor explained that, like many women,
I had a thinned, dilated heart due to pregnancy. She
stressed that the congenital origin of my illness was
unimportant, and that surgery was no longer an option for
me. When we asked what The National Expert had said, she
held up a letter, and said that she was telling me what
he had said – it was too late for surgery, and
transplant was my only option. When I asked about being
seen by a congenital heart center, she again pointed to
the letter, and said that I already had gotten the
opinion of one of the country’s top congenital
doctors, and there was no need for any more congenital
consults.
For the next six months I was a patient at the
transplant center, convinced that I had pursued every
alternative and worked on making peace with my inevitable
decline. At a routine visit, I asked the nurse to give me
copies of my new records, since I had now become
scrupulous about getting copies of all my health
information. On the top of the pile was the letter from
The National Expert, which I had never seen. As I looked
it over, I could not believe what I saw. The National
Expert had written to say that since echocardiogram
studies for people with my defect were often unreliable,
he needed the results of a cardiac MRI before he could
present my case for consult. My consult had in fact never
been completed.
The Transplant Doctor never was willing to see me or
return my phone calls, choosing instead to send me a
terse certified letter expressing regret that I was
unhappy with my treatment and reiterating her belief that
she had given me excellent care. My physician succeeded
in getting her on the phone, and was told that the reason
she had misrepresented The National Expert’s letter
so dramatically was because she "knew" that a
cardiac MRI was unnecessary and surgery was no longer an
option for me. It was clear that, in her mind, not only
was her expertise about congenital conditions
unquestioned, but the need for truth in dealing with
patients was irrelevant.
Meanwhile I had immediately called up The National
Expert, who was appalled at being so misrepresented and
adamant about the need for me to fly out to have the MRI
and complete the consult. When we finally met, he was
grim. He first stated that he had not yet seen an MRI,
and when this was received he would take my case to
consult. However, looking at my existing information, he
was virtually certain that it was too late for surgical
interventions. When I noted that my symptoms were quite
mild, he said "I think you’re in denial about
your symptoms". He went on to state that I should
expect to deteriorate rapidly, and the new breakthrough
heart failure medicines, so esteemed at the heart failure
center, would not help me. He ended my visit by noting
that breakthroughs were being made daily in transplant
technology, and he thought that the best hope I had was
in pig hearts.
Although a small detail, what stands out most vividly
about this visit is the waiting room dˇcor. As I sat
trying to process this devastating news, above my head
was a jungle mural featuring a little furry tiger that
popped up and said, "BOO!" Seeing adult
patients in a pediatric setting may seem benign, but at
this moment the jolly dˇcor made a mockery of what I was
going through. I went home to weep, imagining a future
where I would not see my daughter graduate from grade
school.
Two weeks later, I got a call from the National Expert
to report on the results of the consult. Prefacing his
remarks with the comment that perhaps he should have
waited to see the MRI results before he spoke to me
previously, he went on to tell me that the MRI showed my
heart to be in much better condition than the echoes had
suggested. Not only did the consulting physicians think
that surgery was possible for me, but they couldn’t
figure out why I had any symptoms at all.
At this point, it was hard to know what to believe.
Although I was elated at the prospect of surgical
intervention, I was also acutely aware that this radical
change of perspective seemed to have come from one test
– the cardiac MRI. I asked the National Expert
whether he thought a second opinion was appropriate, and
he both assured me that it was, and sent me off to the
appropriate place – to the Expert on My Condition.
She pooh-poohed the MRI results, stating that they never
used cardiac MRI at her center. However, she ordered a
new echo, eyeballed the result, and declared that my
ventricle was well above the "cut-off" for
surgery. As for my symptoms, they were due to nothing
more than stress and lack of exercise. She expressed her
certainty that, after my surgery, I would be riding my
bike and back at work in no time.
I had successful surgery three months later, and would
love to report a happy ending, with me back in the
classroom and cross-country skiing. Certainly, despite a
number of uncomfortable post-surgical complications, I
have never felt more taken care of than I did during my
stay in the hospital. Performing complicated life-saving
procedures is their specialty and the quality of the
care, from all the doctors and staff involved, was
superb.
The follow-up, however, was distinctly less
distinguished. My recovery has been a struggle, with many
on-going questions left unanswered nine months later.
Starting two months after my surgery, my local doctor
started contacting the surgical center’s doctors,
stating her concerns and asking for supervision. The
response consisted of assurances that everything was fine
and nothing more needed to be done. When I called, I also
got minimal response, and it was clear that my worsening
breathlessness and fatigue were just not dramatic enough
to attract their attention.
Finally, I got The Expert on My Condition on the
phone. She made clear that whatever the problem was, she
knew it wasn’t serious – certainly not serious
enough for her to have to call me herself.
When I started at this national center, I asked about
local congenital heart care, and was told that my care
could be managed effectively by my local doctor, under
the supervision of the out-of-state experts. What I have
discovered is that this does not work – I cannot fly
out of town for echocardiograms and pacemaker
adjustments, and the experts cannot provide effective
supervision when they are busy and miles away.
What else have I learned? I get, and keep, all of my
medical records, and I bring these records to every
appointment. I never expect doctors to have read my
records in advance, and am prepared to not only explain
my situation, but also to find and show them the data
they need. I no longer let them tell me that their tests
disprove my symptoms – they may be the experts in my
condition, but I am the expert in how I feel.
Perhaps most importantly, I have realized that,
although many doctors are entirely willing to play the
role, I can no longer ask them to be all-knowing. I was
thrilled when I was told that I would soon ride my bike
again, and devastated when I was told that I would be
dying soon. But the reality is that no one knows what the
future holds for me, and any claim otherwise is foolish.
At best, research tells us about probabilities, not
individuals, and for people with rare conditions like
mine, the research itself is scanty and subject to
debate. Guided by research, experience, and our own
common sense, my doctors and I will make our choices and
hope for the best.
What do I wish for in the future? Perhaps most
crucial, but also most unlikely, is a change in the
culture of our health care system. Doctors need time to
read patient’s records, and permission to say
"I don’t know" and "I’m not
sure". My experience tells me that doctors often
feel compelled to be the expert, even when they’re
not, and to give answers immediately, before they’ve
had time to review all the appropriate information. I
always say that the best care I ever got was from the
doctors who said, "I don’t know".
The other crucial changes, which are more likely, are
in the insurance system, and in the existing cardiac care
model. Local adult congenital centers need to be
developed with standards in place to ensure that patients
are neither inappropriately referred for transplant or
being denied crucial interventions. Rather than blocking
access to expertise, HMO's and other health insurance
agencies should be promoting the referral of ACHD
patients to the appropriate centers.
It is imperative that the existing authorities in
cardiac care, such as the American Heart Association and
the American College of Cardiology, begin educating their
constituents on the needs of the adult congenital
patient. The bottom line is that people are dying
prematurely due to the lack of quality care.
I am lucky. It’s been a wild ride, but despite
all the bumps and turns, I was able to have surgery, and
I finally feel that my care is on the right track. But
this is no way to run a railroad.
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