When it became clear in January that 3-month-old Sadie Hulsizer would need a liver transplant, she and her family began a wait that can be agonizing.
There are many fewer organ donors than there are people on the transplant list. According to the American Liver Foundation, more than 1,500 people die waiting for liver transplants every year.
Because the liver is the only organ in the body that can regenerate, doctors have the option of removing a segment from a living person for transplantation. This is plan B in most cases, because it requires two surgeries, and if the donor is a member of the same family as the patient, this means that two family members would be under the knife, and in recovery, at the same time.
Sadie’s father, Marine Sgt. Scott Hulsizer, knew he might be a good candidate to help his daughter because he was healthy and young. But he could only donate once. His own liver could be reserved as a backup in case Sadie rejected a different liver.
But as the weeks passed, Sadie grew sicker and sicker, said Dr. Megan Butler, a pediatric gastroenterologist at Duke University Hospital and member of Sadie’s medical team. Her skin and eyes were yellow, a result of bile built up in the blood. Because she was having trouble eating, doctors ran a feeding tube down her nose. They also inserted catheters in her abdomen to remove pent-up fluid that was making it difficult for her to breathe.
Plan B becomes plan A
The family’s medical odyssey had begun in Okinawa, Japan, where Sadie was diagnosed, with stops in California before she was admitted to Duke, near Hulsizer’s new post at Camp Lejeune. They had departed Okinawa so quickly – just days after Sadie was first admitted to the hospital – that they had left behind all their belongings and even their dogs.
When Hulsizer finally met his new commander at Camp Lejeune, he immediately asked for a medical leave. The answer came quickly.
“As soon as I walked in they pretty much told me then and there, ‘Go be with your daughter. Do what you have to do,’” Hulsizer said.
On March 6, he began tests at Duke to determine his compatibility with Sadie. Days earlier, Sadie’s grandmother was deemed not an appropriate candidate for a transplant.
A donor has to have a compatible blood type, of course, but the structure of the liver is also important.
It turns out that there are many natural, apparently harmless variations in the internal plumbing of our livers that become important when it comes to transplants. Where the blood vessels are located on the donated liver segment and where they branch mustmatch the the blood vessels in the patient’s abdomen.
Size, too, is important, especially when the patient is so small.
“A lot of the doctors thought my liver would be too big,” Hulsizer said. Doctors don’t take liver tissue from a donor based on volume: They must transplant an entire segment. In an adult’s liver, that segment may be too big for a young child.
But father and daughter were a match in all three ways: blood type, structure and size.
“It was amazing,” he said of learning he was a compatible donor. “There’s really no way to describe how I felt.”
That moment of relief followed months of worry and despair. It began when Sadie’s mother, Abby Hulsizer, took Sadie to the doctor on Dec. 19, for what she thought was a run-of-the-mill ear infection.
More than jaundice
But her doctor noticed that Sadie, who had always had a touch of jaundice, was looking more yellow than ever. Concerned, he sent Sadie to another hospital in Okinawa, where a gastroenterologist immediately put her on a suite of medications to stabilize her.
Scott Hulsizer had just begun his Christmas leave when Sadie was admitted to the hospital in Okinawa. Just days later, the family was medevaced to Naval Medical Center San Diego. Doctors who examined Sadie’s liver biopsy found white scar tissue, a sign of liver damage, Scott Hulsizer recalled.
Liver damage can come from hepatitis, poor diet, drinking and other issues. But baby Sadie’s condition was congenital. She was diagnosed with biliary atresia, a disease affecting 1 in 18,000 infants, according to the National Institute of Diabetes and Digestive and Kidney Diseases.
Babies with biliary atresia are unable to export bile from the liver to the gallbladder and small intestine. For reasons that are poorly understood, the tubes that move bile – called hepatic ducts – become blocked, or are never fully formed. This means that bile builds up in the liver, quickly scarring and destroying the organ from the inside.
Without a healthy liver, the body can’t process and remove toxins from the blood. This includes everything from ammonia, a natural byproduct of digestion, to the medicines we take and the fumes we inhale.
The liver also breaks down food and sends carbohydrates, proteins and fats to the appropriate places; stores vitamins and glycogen; turns cholesterol into steroids; recycles old blood cells; and processes fat, among other services.
Even if doctors could have fixed the ducts in Sadie’s liver, the organ was now so scarred from excess bile that it could no longer function.
On Jan. 3, doctors in San Diego sent the family to UCLA’s pediatric transplant facility, where she was placed on the transplant list. But when her doctor learned that the Hulsizers were from North Carolina, she arranged to have Sadie transferred to Duke, where they would be closer to extended family.
These family members surrounded Abby Hulsizer in the waiting room as she waited for her daughter and husband to emerge from surgery on March 9.
Both surgeries were successful.
“As soon as I woke up, the first thing I asked was ‘Where’s Sadie?’” Scott Hulsizer said.
Doctors immediately reassured him that Sadie was doing great. In fact, before they even finished closing Sadie up, they could see her new liver make and drain bile. Within days, her coloring came back.
“Right now, other than taking an extra medicine to prevent rejection, Sadie’s a pretty normal baby,” said Butler, Sadie’s pediatrician.
A ‘miracle baby’
Because the liver regenerates, Scott Hulsizer’s liver will keep growing in both father and daughter. In fact, a person who donates half his or her liver to another adult can regrow that organ in just a few weeks, said Dr. Carl Berg, medical director of abdominal transplantation at Duke.
“The miraculous part,” Berg said, is that the liver knows when to stop growing, too. So not only will Scott Hulsizer’s liver grow back to adult size in his own body, it will continue to grow just the right amount in Sadie as she grows up.
Sadie is now 9 months old, and aside from weekly checkups, she is out of the hospital and at home with her parents and older brother, facing more typical baby challenges such as rolling over and learning to stand up.
“Sadie’s a little miracle baby,” her mom said.
Donors face risks such as bleeding, hernias and, of course, a significant amount of pain after surgery, said Berg. “It was painful for a little while,” Scott Hulsizer admits.
But his recovery is going well. Three months after the surgery, he is cleared to bike, run, and swim but must avoid core workouts and lifting anything over 20 pounds.
For a devoted dad, though, those inconveniences barely register.
The day Scott Hulsizer woke up from surgery was March 10 – his birthday.
The moment doctors told him Sadie was OK was “the best birthday present I ever got,” he said.