At only 8-weeks old, Adelyn “Addie” Hughes, now 4, had her first open heart surgery. She was born with a congenital heart defect, which caused her to have a damaged mitral valve. Before she was 8-months old, she had undergone five open heart surgeries. Last summer, her family learned that her heart wasn’t working and she needed a transplant.
“A year ago when we were presented with the options it was either do the surgery and put her on the ventricular assist device (VAD) or they were going to send her home on hospice,” dad Allen Hughes, 38, of Valparaiso, Indiana, told TODAY. “It was a life or death kind of thing.”
For 10 months, Addie waited in Lurie Children’s Hospital of Chicago for a heart. A few days before her 4th birthday, she received a gift her family will be forever grateful for — a new heart.
“I have the most heartfelt concerns for people who are going through a difficult situation when they are losing a child and they have an option to do an organ donation,” Hughes said. “They’re facing such a difficult choice … The ultimate outcome of that on the back end is affecting multiple people’s lives with these organs that are really hard to come by and get — especially for little kids who need them to save their lives.”
Birth defect and heart failure
When Addie was born, doctors suspected the newborn had heart problems and performed an echocardiogram. That’s when they noticed her mitral valve hadn’t developed properly. At 8-weeks old, she underwent surgery to help. Over the next months, she received several mechanical valves to help before eventually receiving a Melody valve.
“That worked well for her for about a year and a half,” Hughes said. “Last summer, she started having issues again and we ended up back in Lurie, thinking they could just replace the valve again. But she had pulmonary hypertension.”
Doctors feared that Addie wasn’t healthy enough for another open heart surgery.
“Her heart was too sick,” Hughes said. “Really the only option we had was a transplant and going onto the VAD … to help support her heart function, while she waited.”
The VAD helps her heart pump blood and doctors also worked to control her pulmonary hypertension. After about a month, Addie was added to the heart transplant list. Still, she needed to stay in the hospital.
“She can be a normal kid with the exception that she’s connected to a machine. She could play in her room and go for walks twice a day, around the floor in the hospital,” Hughes said. “Other than that she was limited to her room.”
While Addie was in the hospital, either Hughes or his wife, Rebecca, 37, would spend the day with her. Hughes was still working full time so he often went to visit on weekends. The couple has two older daughters, Kaliyah, 7, and Isabel, 5, and sometimes they could visit with their sister, too. For the most part, Addie was the “spunky” Disney-loving toddler she always was. But sometimes she felt frustrated or sad.
“She asked a lot to go outside. She’d want to go swimming or take a shower, when she was connected to that machine she couldn’t take a regular shower,” Hughes said. “She definitely missed out.”
On September 15, they received news that there might be a heart that was a “good match.” But after some time passed, doctors thought the donor heart wasn’t healthy enough.
“They were nervous about putting it into Addie,” he said. “Ultimately they decided not to go forward with that heart transplant.”
While the family was disappointed, a few days later they learned there was another heart that matched. On September 20, she had surgery for her new heart, a fitting present for her birthday, which was October 2.
“Thankfully that one worked out,” he said. “Her surgery took about eight hours. It very nerve-wracking.”
But every hour a nurse reported to the family on Addie’s progress. After an eight hour surgery, Addie started the recovery process. While she did well, she again had pulmonary hypertension. But doctors were able to control that after the first week.
“She’s back to her normal self. She does get tired more easily, just walking around and doing normal stuff,” he said. “We got all the IVs and all those holes and everything out.”
Life after transplant
The family just learned she can go home today — after a year in the hospital and 10 months on the transplant list.
“Everybody’s super excited. Her sisters have really struggled with this in different areas, having their baby sister not being home and having everybody split apart,” Hughes said. “They are probably the most excited to have her back home and have mom and dad back home and everybody under the same roof.”
Therapists don’t think Addie will need to continue physical or occupational therapy after she leaves the hospital because she’s "where she should be." Though for the first three months, she’ll have to stay isolated from other people because her risk of infection will be so great.
“Life should be pretty normal. She will have to be on medications for the rest of her life, anti-rejection so her body does reject her heart,” Hughes said. “Hopefully, she has a good quality of life.”
The Hughes family hopes that Addie’s story encourages people to become organ donors.
“We were ecstatic that we finally did get her a heart and she’s doing so good,” he said. “The big thing is the importance of organ donation especially for young kids.”