'A tough little girl': Dickinson baby born with extremely rare heart condition
When now 5-month old Anavey was born Nov. 8, 2018, her doctors didn't know exactly what was wrong with her, just that her heart was different from a typical heart.
During mother Mayra Scarrow's pregnancy, her doctors discovered abnormalities in Anavey's heart and referred Scarrow to cardiologist Justin Horner in Fargo. A couple of days before Anavey was born, Scarrow was transferred to Fargo.
"I was having contractions, and they were really worried. I couldn't have her here; they don't have the resources," Scarrow said.
But even after Anavey was born, they had trouble diagnosing her.
"At that time, they still didn't know what she had. They were taking her and doing the echogram. They were doing all the images and sending them to different people," Scarrow said.
Without a diagnosis, Scarrow and Avaney went home to Dickinson. It took the doctors about a week and a half to figure out what was wrong.
"At that time, she was already in heart failure," Scarrow said. "After the third day home, she wasn't really eating. ... She would just sleep. It started getting worse. I went to go see her doctor in Bismarck. At that point, he was terrified. He's like 'She's not doing good. We need to give her ICU right now in Bismarck. I'm still finding who's the best surgeon for her.'"
That surgeon was James Jaggers of Children's Hospital Colorado. They were flown to Denver, and the day after Christmas, Anavey had her first surgery.
"It was just a small little band that they were going to put (on) the small top artery of her heart to help the blood flow and to slow down the heart a little bit. That gets done all the time. The surgeon was so confident," Scarrow said.
The confidence of the surgeons' team reassured Scarrow. She wasn't worried.
After Anavey's surgery, Jaggers told Scarrow that Anavey's stats were low—and then Anavey died.
"They didn't know what happened. They didn't know what went wrong. They had no idea. It was completely out of their hands. They were all at a loss. When they came out to talk to me, they didn't know what to tell me," Scarrow said.
She recalled waiting for her daughter to be resuscitated.
"They had rushed her back to OR," she said. "(Jaggers) left and I think a few minutes passed by, and they were like 'We're still performing CPR.' Fourteen minutes passed by, and they're like 'She's not coming back. We're pumping steroids in her heart, and the surgeon's pumping her heart.'"
While she was waiting, Scarrow prayed.
"I was just like, 'Please—just—God, let me hold her one more time,'" she said.
Plenty of other people were praying for Anavey as well.
"Everyone was praying. ... I felt it, everytime I wanted to feel sad, I always felt the Holy Spirit just be like 'No, I'm here. Don't fall on your knees. Not yet. I'm still here.' I would feel it and I'm just like 'God, at my lowest, you never let me fall.' ... It was exactly what I needed," Scarrow said.
After 20 minutes passed, Anavey was finally resuscitated. Scarrow was told that Anavey would need to be put on life support because she was already in heart failure and had lost a lot of oxygen.
Anavey was put on extracorporeal membrane oxygenation, or ECMO, which supports a patient's heart and lungs. The doctors didn't think Anavey would be able to get off ECMO, so they told Scarrow to say goodbye.
"I didn't say bye to her that day. I remember I was wiping my tears, but I didn't. I was like, 'You're doing so good; I'm so proud of you; You're still here; You came back, honey," she said.
After a few days, they took Anavey off life support, and she did well, but because her brain was deprived of oxygen for so long, Anavey's doctors warned her mom that Anavey will never be normal.
"She's going to have developmental delays or she's not going to be able to do anything that a normal baby will do," Scarrow said. " ... For me, I feel like she's just like any other baby her age. ... She grabs onto things and now she's trying to sit up."
Back home in Dickinson, Anavey's nurse, Deb Schweitzer, said that she gave Anavey a developmental assessment when she first started seeing her. Anavey was 3 months and 16 days old.
"She was at 3 months in her cognition and her receptive and expressive language and her social/emotional," she said. "Her gross motor and fine motor and adaptive were lower, but part of that ... she doesn't reach with her left hand ... but that was where she had surgery. It's difficult to assess that because of the surgical scars and things she's been through."
Schweitzer works with the KIDS (Key Infant Development Services) Program, which helps families with early intervention.
Anavey was gagging and throwing up a lot when eating, which caused her to lose weight, so she now has to be fed through a feeding tube. When Schweitzer first started seeing Anavey, she was even having trouble with the feeding tube.
"She was projectile vomiting a lot of what she was getting with the feeding tube," Schweitzer said. "I think we problem-solved that away now ... when she would have a tube feeding, it was displacing the food, so we were able to figure out ways to get every bit of air out of her body, cause that can be real difficult with tube feeding."
One of those ways is to massage Anavey's belly before feeding her, which expels the trapped gas through the tube.
Anavey has now been gaining weight, which she needs to do before her next surgery—the surgery that will fix her heart.
"She's a tough little girl, though. She's really a fighter," Schweitzer said.
Anavey's condition is complex. Her cardiologist Justin Horner calls it isolated atrioventricular discordance with ventriculoarterial concordance and describes her heart as being basically backwards.
"The left side of a heart will normally pump blood out to the body, and the right side of our heart will pump blood out to our lungs. In her, the bottom chambers are backwards and so the left lower chamber is on the right side and her right lower chamber is on the left side. She's got a little bit of a backwards heart, if you will," Horner said. "Those ended up pumping out to the right spot. Even though her left lower pumping chamber, which should pump out to the body, is on the right side, it now continues to pump out into the lungs, which is the way it should if it was on the right side."
This condition is very rare.
"One percentage of babies will be born with congenital heart disease, and hers is very small of that one percent. I don't know the exact—I'm not quite sure anybody does because her heart is super rare," Horner said.
He said it is possible that she is the only one in the country with it now.
She also has a large ventricular septal defect , or VSD—a hole between the lower chambers of her heart.
"It has caused her in her first couple months of life to go into what we would say is congestive heart failure. She had too much flow going through that hole and out into her lungs and that caused the significant problem of breathing or gaining weight," Horner said.
The surgery will fix both problems.
"It's going to entail rerouting the blood so it goes in the right direction, more or less. We hope to have the original left lower chamber that's now on the right side pump out to the body, the way it's supposed to," Horner said. "We'll have to do an arterial switch. She will also need a VSD closure. She will need to have an interatrial level switch as well, so her top chambers need to be rerouted ... It will be a very big heart surgery. It will be very complicated."