We welcomed Edison into the world on May 1st of
2015. At that time we already knew that
he had a couple of rare medical conditions: hypoplastic left heart syndrome
(HLHS) and heterotaxy. If left
untreated, HLHS would be fatal within 24 to 48 hours. The extent of the heterotaxy was unknown, and
potentially benign, but still concerning.
While HLHS occurs in roughly 1 in every 5000 births, heterotaxy only
occurs in about 1 in every 250,000 births; a very rare condition. Being forewarned, Edison was delivered in
close proximity to Riley Children’s Hospital in Indianapolis and was rushed
there shortly after his birth to be stabilized and monitored in their
specialized NICU. There in the NICU he
would await his first of 3 highly invasive surgeries to treat his very serious
heart condition. Though Edison could
potentially lead a normal life after the 3 surgeries he would always be limited
from a cardiac standpoint. The only
alternative to the 3 surgeries was a heart transplant.
Edison experienced his first HLHS surgery (the Norwood
procedure) at 10 days old. Despite his
severely complex heart anatomy (even more so than typical HLHS babies due to
the heterotaxy), the surgery went really well.
After the procedure the surgeon explained that he had only seen 3 other
cases (out of hundreds) that had cardiac
anatomy similar to our son’s. Nothing
about him or the procedure was routine. It
took Edison some time, but he pulled through like a champ and before we knew it
they were starting to wean him off the ventilator in preparation to be
extubated. Around that time the doctors
started to notice that he was looking jaundiced and his bilirubin (liver bile) counts
were a little high. They kept their eyes
on it over the next few days until the counts continued to elevate and were more
concerning. They ordered a scan to see
if the bile created in the liver was passing through to the intestines. The scan showed that it was not, so they
ordered a liver biopsy to confirm what the scan had shown. It did.
Edison was then officially diagnosed with biliary atresia.
Biliary atresia (BA) is a condition where the liver’s bile
ducts are obstructed, not allowing bile to pass from the liver to the
intestines. When bile builds up in the
body it causes jaundice. If bile can’t
exit the liver over an extended period of time cirrhosis sets in, ultimately
leading to liver failure. BA has one
surgical treatment alternative to transplant, though the procedure is really
just meant to buy time until a transplant is required. Transplant is almost always eventually
necessary to correct BA. In the surgery,
referred to as the Kasai procedure, the damaged bile ducts are removed and a portion
of intestine is attached directly to the liver.
If effective (only about 50% of the time), bile flow is restored at
least temporarily. The hope is that it
would allow Edison to get older, bigger and healthier before undergoing a liver
transplant. That was our understanding
at the time.
During all of the BA testing, Edison’s little corrected heart
was ticking like a vintage timepiece. He
was extubated and making steady and impressive progress towards being free of
hospital care. At five weeks the attending
CVICU doctor told us that if it were not for the liver issue, we would have
been be going home. Instead we found
ourselves in the surgery waiting room praying our Edison would make it though
another surgery.
Four hours into the surgery the rounding nurse invited us to
speak with his surgeon in one of the consultation rooms in the hallway –
typically reserved for the delivery of bad news. Edison’s surgeon and hepatologist entered the
room and explained that they were not able to do the Kasai due to a
malformation of arteries and veins (an AVM) that had formed on the portal plate
of his liver, if his liver had a portal plate.
The AVM was in the exact place they needed to attach the tract of intestine
to the liver. Even if the portal plate
did exist, there was no way to operate in the area without causing him to bleed
out. It was the first time in her thirty
years of experience that the surgeon could not perform the Kasai. They then told us that Edison was not a
candidate for a liver transplant because he only had the first stage of the 3 heart
surgeries. At the time we were not
expecting to hear that, understandably so, and the news was completely crushing. In addition, they explained that they were not
sure if he would ever be considered a candidate for a transplant because of the
complexity of his anatomy and collection of medical conditions.
We were heart broken.
Riley made it clear that they could no longer do anything for him. With the help of our doctors we spent the
next two weeks seeking potential alternatives and second opinions. We thought of every possible scenario and question.
We reached out to Lurie Children’s in Chicago and Cincinnati Children’s. In both cases we provided medical records to
elicit potential treatment plans. Neither
institution had answers for us or anyway to help. Our surgeon reached out to a network of BA
specialists (of whom she was one). No
one had seen such a case. Eventually we
began to see the reality of the situation – how very rare and very final it was
that Edison had all three of these conditions.
Medically speaking, he had nothing in his corner. We later understood that his liver bile ducts
had never even formed. His little body
was engineered to fail.
Though thoroughly convinced of the inevitable, the urge to
pursue a solution was strong. It seemed that
if we wanted to we could hop from hospital to hospital looking for some cowboy
surgeon to take another shot at him. With the aid of caring physicians and
prayer, we ultimately chose to bring him home with hospice care. This way he could get to know his older
brothers, his home and ultimately pass while swathed in love.
Some kids with BA can live to be about a year old before
they need a liver transplant. Edison’s
vascular structure results in high blood pressure in his liver, something that
the liver really does not like. This will likely cause cirrhosis to set in
quicker. The medical team did not really
know how long he has, but they estimated that he has anywhere from 3-6 months,
providing that his heart continues to do well.
Heart and liver conditions in conjunction can quickly lead to spiraling
declines.
One of the hardest things right now is that he looks so whole
– with the exception of his surgery scars and jaundiced appearance. It is difficult to grasp that he could be so
sick. For all of modern medicine’s
marvels, one would think (and ignorantly so) that there should be some clear
solution. Surely there will be at some
point, but there isn’t now and that is what matters for Edison. We had never thought since the beginning that
it would be anything other than his heart that would be so life threatening,
but here we are.
We are constantly finding comfort in our faith. Friends and family have been immensely
supportive and caring. We have seen our
prayers answered in so many ways that we cannot even begin to express. We believe that families can be together
forever as part of God’s plan for us. We
know we will be with Edison again someday after he passes. We believe he is one of God’s special spirits
who has been entrusted to us, even if for a short time. While we wonder what our Father in Heaven
intends Edison’s purpose on Earth to be, one thing for certain is that he was
sent here to be part of our family and we have the perfect opportunity to love
him. For as long as we possibly can we
will spend the days and weeks to come loving Edison.
