Five months after donating a segment of her liver to her 3-year-old daughter, Tiffany Kuzmick, MSN, RN, is loving life as she, husband Kevin, and 18-month-old son, Ben, are all getting reacquainted with their healthy little Emma.
Emma, a sweet-faced blonde, was diagnosed with biliary atresia at 8 weeks old. "She was always a happy girl, but she really didn't know a life without discomfort," Tiffany told ADVANCE in a late-August interview.
But since the successful liver transplant of April 17 at Nemours/Alfred I. duPont Hospital for Children in Wilmington, DE, Emma has finished summer gymnastic camp and has soccer, dance and music classes lined up for the fall - not to mention pre-school.
"We have lived our life in such a bubble, now we want to do it all," said Tiffany, 32, a nurse in the NICU at Lankenau Hospital in suburban Philadelphia. She returned to work in June, 8 weeks after the procedure.
Emma, who after a little prodding demonstrated a perfect somersault in the basement of the family home in Aston, PA, offered a quick, "Good," when asked how she is feeling. She was more interested in cuddling with the family pet, a 6-year-old Puggle named Jake, and leading Ben on an expedition to see exactly where an inquisitive visitor had parked her vehicle.
With both Ben and Jake, Emma couldn't have been more gentle, or polite.
On Angel Wings
It was past Emma's 7-7:30 p.m. bath, which precedes her 8 p.m. medication routine. Though she's slowly being weaned off multiple medications, Emma will take anti-rejection medication for the rest of her life. Additionally, she's down to monthly hospital visits.
When she asks why she was sick, or why she has to take medication, which she does often, Emma's parents answer the questions as best they can for her to understand. Something Emma understands is the special "angel wings" that both she and her mother share. That's what Tiffany calls their incisional scars.
"She is 3 going on 30," her mom said. "She has been forced to grow up quickly."
As they spoke about the donor experience that has forever changed their lives, Tiffany and Kevin, who is employed as a director for Bayada Home Health Care, couldn't help but look at Emma and smile. A typically private couple, they wanted to share their story to increase awareness about the importance of donor programs. Beyond designating themselves as donors on their drivers' licenses, neither Kevin nor Tiffany knew much about it themselves.
Contrary to 31-year-old Kevin, who found encouragement in online support groups, Tiffany admitted she internalized her feelings about the disease, and her daughter's prognosis, at first.
"It took me longer to deal with it than Kevin," she said.
Tiffany recalled her pregnancy with Emma as a normal one, and Emma was born full term, but a little jaundiced. The jaundice went away, but soon returned. Tiffany called the pediatrician and, before long, their infant daughter's medical ordeal had begun.
"All of her liver functions were significantly elevated," Tiffany said. As the condition progressed, Emma's "bile duct was like a string," she noted. A Kasai procedure was performed, though there they knew there were no guarantees it would offset an eventual liver transplant. While some children born with the rare disorder can squeak by without a transplant, most require it.
A Mother First
A nurse for 10 years, Tiffany said she found her professional knowledge helpful in dealing with her daughter's condition; but more than anything, she just wanted to be a mom throughout the experience.
"Sometimes, ignorance is bliss," she said. But at the same time, she felt she was better prepared to take care of her daughter's special needs, including a feeding tube for 8 months.
Since the need for a liver transplant was something that could have happened to Emma at any point in her life, her mom and dad are grateful that it happened to her at a young age when she was most resilient.
On Feb. 17, Emma developed a severe gastrointestinal bleed. It took all night for the healt care team at duPont to get the bleeding under control. It was then they knew Emma needed a transplant.
Tiffany immediately began weeks of testing to determine live compatibility. From the outset, if and when it happened, she wanted to be the living donor.
"I gave the gift of life once," Tiffany said. "I wanted to do it again."
"There are no guarantees, but the odds are generally good a parent is a match," said Kevin, who would have been next on the list for testing.
"Kevin is one of five children, I am one of two, all of them said, "Sign me up," Tiffany said. "We started with me. Fortunately, we didn't have to go any further. I was a perfect match."
At the same time, Emma's name was added to the donor list - for either a cadaver organ or a living donor.
Thanks to unwavering support of family and many friends, Tiffany said she and Kevin were able to be with Emma throughout all of her many hospital stays, 24/7.
Old School Nursing
Pulling out a file with names and dates and other details regarding his wife and daughter's care, Kevin, too, obviously put a few of own professional skills to use. Not that he needed his well-kept notes to retrace their path; he's committed much of the information to memory.
The overall surgery took 10 hours, he said. "Tiffany was first. They took her in at about 8 a.m. They took Emma in at 10. Tiffany was finished at 3 p.m., and Emma was done at 6."
For Kevin, there was much pacing in between, while being surrounded by many family members.
"It was the first nice day of the year and I spent a lot of the time outside," he said. He was grateful for the updates he received almost hourly.
"I was never out of the loop," he said.
Kevin sounded off a list of names, from transplant coordinators Louise Flynn and Dana Mannino to the many nurses who cared for his two favorite girls. He considers all nurses to be saints.
"From the initial diagnosis to the transplant, we are coming up on 100 nights in the hospital," he said. He couldn't say enough about the team at duPont.
"It's a well-oiled machine," he said.
Tiffany, too, praised her and her daughter's entire nursing team.
But it was her overnight nurse, Amy, who left an indelible impression. Describing her as an "old school nurse," Tiffany said she encouraged her to shower and walk the halls when she would rather sleep.
"She really whipped me into shape," Tiffany said.
Looking back, she couldn't be more grateful. She said Amy went above and beyond by not only caring for her, who was part of her patient assignment, but she kept an eye on Emma as well.
Hugs All Around
Amy Whetham, RN, CPN, has worked her entire 30-year nursing career at duPont. She is one of about 30 nurses assigned on 3C South.
"We are the transplant unit," she said. In addition to caring for patients like the Kuzmicks, they care for all urology, orthopedic, kidney and liver disease patients on the 23-bed unit.
"I like taking care of donors, as well as the children," she said. But she couldn't do what she does without the help of dedicated co-workers, including aides.
After 3-6 days in ICU, all the liver and kidney transplant patients are brought to 3C South. In addition to caring for them as they heal, her job is to educate the adult donor.
"We get them immediately from ICU until they are discharged home," she said.
Amy was thrilled to hear about Emma's progress.
"They are such an awesome family," she said. "They went through so much and they were so grateful for everything that was done for them."
Working the 7 p.m. to 7 a.m. shift, Amy said she gets to know her patients and their families. Overnight is when she will walk her patients - whether they want to or not. Though she sometimes feels bad, she knows it is in their best interest.
Working at a children's hospital, Amy sees a lot.
"The best part of my job is my patients get better. We see them walk out the door."
Like the Kuzmicks, she's a firm believer in donor awareness.
"It's such a wonderful thing that they as parents can give a kidney or part of their liver to their children," she said. She wishes more people could witness how it changes lives.
"These kids who are so sick coming in, open their eyes after surgery, give you a hug and ask to go to the playroom," she said.
Amy was about Emma's age when she first started telling people she wanted to be a nurse, like her grandmother.
"I never once changed my mind," she said. "I love what I do."
Rose Quinn is a frequent contributor to ADVANCE.