DIGESTIVE HEALTH INSTITUTE
THE END OF THE NIGHT
THE ABOVE-AVERAGE LIFE OF A TEEN WITH A DEADLY DISEASE
Meet Alexa
Renae Malara does her best to maintain her body weight near
her daughter Alexa’s, which can’t be easy when Alexa is 19 and
Renae is, well, not 19. But it needs to stay pretty close to the same
for Renae to be a good donor match; if Alexa’s liver ever failed,
Renae would donate half of her own. That day hasn’t come yet,
but there’s about a 40 percent chance that one day it will.
Alexa Malara and her mom, Renae,
show off a photo of Alexa as a baby,
very jaundiced (and very cute).
Born deeply jaundiced, Alexa was
quickly diagnosed in the Pediatric
Liver Center at Children’s Hospital
Colorado with biliary atresia, a rare
and deadly condition in which the
immune system attacks the ducts
that transmit bile from the liver to
the intestines. An untreated infant
would be unlikely to make it to
2 years old.
Alexa’s case was aggressive:
scarring had clogged her main
bile duct and within days of her
diagnosis, Children’s Colorado's
Fritz Karrer, M.D., now Chair of
Pediatric Surgery, removed and
replaced it with a section of her
small intestine, a surgery known
as the Kasai procedure. “Dr. Karrer
canceled his vacation to do the
Kasai,” Renae recalls.
Though rare, biliary atresia accounts
for nearly 50 percent of pediatric
liver transplants in the United States.
In the years before Alexa was born,
the Kasai was basically the only
treatment.
“In the early 1980s, care was
fragmented, very siloed,” recalls
Michael Narkewicz, M.D., Alexa’s
pediatric hepatologist at Children’s
Colorado for the last 19 years. “Kids
came, they saw the surgeon, the
44
surgeon would operate, and that
was it.” Infants often underwent
multiple operations, and when the
operations stopped working, liver
transplantation was the only option.
“We were one of the first centers
to realize that care for biliary
atresia requires a multidisciplinary
approach,” says Dr. Narkewicz. The
Pediatric Liver Center began in 1985
as one of the first multidisciplinary
centers focused on children with
liver disease. For Alexa, while Dr.
Karrer performed the Kasai, Dr.
Narkewicz and a team of pediatric
hepatologists, nutritionists, clinical
nurse specialists, social workers, and
others coordinated every aspect of
her care, from her operation to her
medication to her diet. “Prior to the
formation of this center,” says Dr.
Narkewicz, “nobody was doing it
that way.”
Central to the model of care was
identifying new ways to treat
not just the scarring of biliary
atresia, but the many associated
complications: cholestasis, vitamin
deficiency, and infection. Working
with Ronald Sokol, M.D., another
longtime pediatric hepatologist
at Children’s Colorado, Dr.
Narkewicz was among the first
“We were one of
the first centers to
realize that care for
biliary atresia requires
a multidisciplinary
approach.”
to apply research suggesting
ursodeoxycholic acid, a compound
that debuted in the late 80s to treat
gallstones in adults, could improve
bile flow in kids with biliary atresia.
Alexa was one of the first kids to be
treated with the drug. She’s been
taking it nearly her whole life.
It’s since become a treatment
standard. “Every kid with biliary
atresia takes urso now,” says Dr.
Narkewicz. “Of course, not all of
them have the outcome Alexa’s had.”
Still, at least in part thanks to a
lifetime of comprehensive care,
Alexa’s outcome has been very, very
good. She’s enjoyed a remarkably
normal 19 years of life, even a love
of playing sports: swimming, tennis,
and field hockey, her favorite. Now
an undergrad at Colorado State
University, she’s aiming for an MBA
and a career in sports marketing.
She’s on a good track. The mass of
college credits she earned via an
accelerated high school program
will allow her to graduate a year
ahead of schedule. “She’s very
disciplined,” her mother says.
Aside from being ahead of the
game, she’s had a pretty standard
college career. She haunts a coffee
shop in an alley near campus. She
joined a sorority. And like all college
kids, she goes to parties — although
she can never drink. “I’m always the
DD,” she jokes.
“You’re not missing much,” says Renae.
Alexa laughs. “That’s what I find out
at the end of the night.”
45