Mikaela S. – Failing Liver | Liver Transplant Story

Makaela LDLT

A Decade-Long Journey from Diagnosis to Double Transplant

Heather Sienkiewicz of Burlington, Vt., will never forget when her daughter, Mikaela, mastered tying her shoes.

“She’d lace them up and then take off running,” she laughs.

That same “spunky independence” sustained Mikaela and her family during more than 10 years of illness and repeated hospitalizations.

The cause? Liver and kidney failure.

The Challenge: A Failing Liver and Kidney

“During a wellness check when I was four, my pediatrician noticed that my liver was enlarged,” says Mikaela, now 19. “He wasn’t overly worried, because organs can sometimes grow too fast. But after testing, he diagnosed me with autosomal recessive polycystic kidney disease (ARPKD).”

The condition had been present since birth.

ARPKD is a genetic kidney disease that can affect the liver, as in Mikaela’s case. The family learned that some congenital hepatic fibrosis, — which affects the bile ducts of the liver — also had begun.

“Looking at Mikaela, you’d have never known it. She was as active and playful as her older brother, Luke,” says Heather.

But, a year after her diagnosis — the night before her kindergarten dance recital — Mikaela began complaining of stomach pain and nausea.

Blood was no longer flowing through her liver. Instead, it was backing up, enlarging her spleen, and causing bleeding in her esophagus — a condition called bleeding esophageal varices.

That day, doctors told Heather and her husband, Mike, to start planning for a liver and kidney transplant for their little girl.

The Path to Children’s Hospital of Pittsburgh of UPMC

The Sienkiewicz’s received several recommendations for hospitals as transplant options. Heather’s mom, an emergency room nurse, urged them to go to Children’s Hospital of Pittsburgh of UPMC.

With family close by — Mikaela’s aunt lives 90 minutes from the city — they knew Pittsburgh would be the best place for her.

In July, the family left Vermont for an intense evaluation at Children’s Hospital.

“But the doctors determined that Mikaela’s illness hadn’t progressed to the point where they could place her on the transplant list,” remembers Heather. “We came back home to wait and watch.”

Days became weeks — then months and years.

Mikaela’s health slowly declined, marked by repeated hospital stays and even life flights to Pittsburgh.

It wasn’t until November 2010 — when Mikaela was in 7th grade — that she would return to Pittsburgh to begin a nearly yearlong stay at Children’s.

By then, the 14-year-old’s weight had dropped to 75 pounds. She had a badly distended belly and her skin and eyes had turned yellow. She would nap for hours each day.

“Things were shutting down,” says Mikaela simply. “I had to start on dialysis to live.”

The Solution: A Liver and Kidney Transplant

“The surgeons always planned to use a deceased donor for both organs,” says Heather. “But it was clear that we now needed living donors — quickly.”

The Sienkiewicz’s sent an email to family and friends explaining the dire situation and asking for possible donors.

Round 1: A new liver

Mikaela’s father, Mike, remembers giving blood in high school. But, he says getting out of class to donate blood was likely more motivation than altruism.

“I knew about organ donation, but I never seriously considered doing it,” he says.

All that changed when he had the chance to be a living donor for his daughter.

“It was a no-brainer,” says Mike. “Heather was always at the frontline of Mikaela’s care. She’d been by her side virtually 24/7 for months. My only prayer was that I could be a good and healthy match for her.”

The initial plan was to perform both transplants together. But, Mikaela’s failing health prompted the transplant team to start with the liver on May 23, 2011.

Internal bleeding and frail health kept Mikaela from a quick recovery. As she gradually gained strength, she began getting ready for her second transplant.

Mike left the hospital five days after liver donor surgery. He rested for a few weeks before returning to his physically demanding job as a concrete supervisor.

“But otherwise, I was back to work as usual pretty quickly.”

Round 2: A new kidney

A close family friend — Monica Jacobs of Queensbury, New York — was among those who responded to the family’s plea for transplant volunteers. Both she and Mike’s sister were potential kidney matches for Mikaela.

“It broke my heart that Mikaela had been sick for so long, waiting months for transplants,” says Monica. “I actually had researched the subject of living donors before we got the family’s email. My husband and Mike have been best friends since fourth grade. Mikaela grew up calling me Aunt Monica. I wanted to be there for her.”

Monica was determined to be the best match for Mikaela. The living-donor transplant process was thoughtful and thorough, she says.

“They check you for everything — from heart problems to skin cancer. They even want to be sure that you’re mentally prepared,” says Monica. “I was continually advised that I could change my mind. Although this is a singular journey, I never felt alone.”

Mikaela received Monica’s kidney on August 8, 2011. She soon began producing healthy urine on her own.

Monica's stomach muscles hurt right after surgery, but she was able to manage the discomfort with pain medications.

“My mom came to watch our two children so I could take it easy for the first week or two after surgery. It did take a few months to feel fully myself again. But that was a small price to pay for Mikaela’s health,” Monica says.

Looking back, Monica says:

“I would do it all over again in a heartbeat. The operation didn’t affect my health in any significant way. I just have a little inch-long scar to prove it happened.”

Kyle Soltys, MD, pediatric transplant surgeon at Children’s Hospital, comments:

“I'm in awe of the commitment and selflessness of Mikaela’s donors and by Mikaela’s strength during her recovery. I recall her determination to return home after her surgeries and the love and support she got from all of those who were here for her.”

The Outcome: A Healthy, Bright College Student

Mikaela celebrated her five-year transplant anniversary in 2016. People describe her today as “luminescent,” reports her dad.

“She’s my hero, who triumphed over every adversity with grace and strength,” adds Monica.

After spending nearly all of 7th grade at Children’s Hospital — where she kept pace with her schoolwork through tutoring and studying — Mikaela returned home to Burlington in early October 2011. By the time she graduated from high school, she was an honors student and a student leader.

Today, Mike and Heather are proud of their children and their accomplishments.

Their son Luke is an Army medic and Mikaela is a freshman at Carlow University, just a few blocks from Children’s Hospital.

“It’s too early to be certain,” she says, “but I’m considering pediatric nursing.”

Proof of how small the world can be, one of her professors is a nurse who cared for her at Children’s.

Heather remains an active ambassador for Donate Life Vermont. She speaks at the local Department of Motor Vehicles each month encouraging people to become organ donors when renewing their driver’s license.

“There are so many people we’ll always be indebted to for their support, time, and generosity — from our families, friends, and neighbors to Mike’s company and community groups,” she says. “We want people to know what a difference they can make to families like ours.”

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