She gives new meaning to the phrase one-of-a-kind.
Eleven-month-old Briella Caniparoli was born with three severe heart defects, each so rare that the likelihood of being born with even one of them is the same as being struck by lightning numerous times on separate occasions.
So far, Briella has survived multiple hospitalizations, a blood transfusion, open heart surgery and a major stroke; all before her first birthday.
Briella was born with Hypoplastic Right Heart Syndrome, an extremely uncommon condition in which the right side of the heart is underdeveloped and malformed. She also has Heterotaxy Syndrome, which is another unusually rare birth defect which sometimes occurs with paired organs. When Heterotaxy is combined with Dextrocardia Syndrome, Briella's third heart defect, it typically means that the heart is on the right side of the chest instead of the left.
The three conditions are so unusual that doctors couldn't even diagnose her, much less tell her parents, Chris and Mark Caniparoli of Sultan, if their little girl would even survive her own birth. Physicians knew something was wrong, they just didn't know what it was.
Chris explained that they first learned of a problem during her 20-week ultrasound, when she learned that she was having a girl. The doctors realized that they couldn't see part of the baby's heart.
From there they sent her to a maternal-fetal specialist who gave ominous news. They told Chris and Mark that the problem could be a genetic defect or it could be Down syndrome, but it was likely their child would be severely mentally retarded and have limited quality of life.
They offered Chris the opportunity to terminate, and when she refused, they offered her the opportunity to have an amniocentesis, which is a procedure that can definitively detect things like Down syndrome, cystic fibrosis, muscular dystrophy and other conditions. But the procedure itself carried risk; more risk than Chris was willing to take knowing that her baby had something wrong with her heart.
It didn't really matter to Chris and Mark whether their baby girl had Down syndrome or not.
"We were just going to love her no matter what,GÇ¥ said Chris.
The maternal-fetal specialists in Kirkland referred them to Seattle Children's Hospital for another opinion. The doctor at Seattle Children's didn't
favor the Down syndrome diagnosis, but he confirmed that there was a major issue with the baby's heart.
Essentially, they informed Chris and Mark that it looked like their baby only had half a heart.
"All through my pregnancy we had so many ultrasounds,GÇ¥ said Chris. "We were going to the doctors like three times a week, but she never really had a diagnosis until she was born. They said she had a half a heart; that's all they knew. But they wouldn't specifically give her a diagnosis.GÇ¥
Doctors couldn't even tell them if she would survive being born or not, because there simply wasn't anybody to whom they could compare her. Nonetheless, Chris gave birth to Briella at Evergreen Hospital on September 28, 2013.
"They told me that she would come out blue, not crying and needing surgery immediately,GÇ¥ said Chris. "She came out pink and screaming and I held her right away; she didn't need any immediate intervention.GÇ¥
But they knew she would need corrective surgery on her heart, and she would need it soon. Doctors decided that it was important to delay the surgery for as long as possible so that she could gain both weight and strength. Finally, in February, the situation became critical and the next thing Chris and Mark knew, they were at Children's Hospital and Briella was scheduled for emergency open heart surgery that very night.
Chris explained that her biggest fear had come true.
"My list of questions was at home. I'm meeting the guy; I'm leaving her in his hands, you know; 15 minutes before the surgery and I had no questions to ask him,GÇ¥ said Chris. "I couldn't remember anything. I had nothing. I just said, "Take care of my baby.'GÇ¥
Doctors performed the Glenn operation on Briella, which is a special procedure to help improve the blood flow to her heart. Typically, the Glenn operation is a phased procedure meant to be performed in conjunction with the Fontan procedure. Briella's Fontan surgery will take place at a later time.
Briella came out of the Glenn procedure successfully, and for the most part was doing great, except that she couldn't seem to shake the sedation. Briella, explained Chris, apparently didn't feel like waking up right away.
"She just wasn't waking up,GÇ¥ said Chris. "So I told her, "Briella, do you want pretty toes?' And she'd peek open an eye and give a little smirk. And I'd say "No, you have to wake up if you want pretty toes.GÇ¥'
"Finally, she woke up, so I ended up painting her toenails in there,GÇ¥ remembered Chris.
So far, Briella has overcome just about every obstacle placed in front of her. In March, slightly over a month after her open heart surgery, she suffered a major stroke caused by a blood clot that had formed. A CAT scan revealed that the stroke had been a major one, and that it had affected the whole left side of her brain.
But Briella recovered so quickly that the doctor's began to question their initial diagnosis.
"The stroke happened on a Thursday, and by Saturday, she was smiling again,GÇ¥ said Chris.
They wanted to do an MRI to investigate the matter further, but an MRI would have required sedation and Chris didn't feel comfortable sedating her daughter when it really wasn't necessary.
"They are wrong about a lot with her,GÇ¥ said Chris, explaining that doctors have finally told her that they will stop providing her with statistics. "Because Briella always falls into that "not going to happen' category.GÇ¥
It was also determined around this time that Briella was suffering from seizures, so she is currently on seizure medication which is carefully monitored so that she doesn't outgrow her dose and begin having them again. She's also on blood thinners, as a preventative means for avoiding additional blood clots.
Little Briella has not only defied all the odds, but she has done so with a sweet smile on her face. It would be impossible to fathom all she's gone through just by looking at her. She's a friendly and cheerful little girl, with large, intelligent blue eyes and enviable eyelashes. She's social, outgoing and inherently curious. She likes to explore things like purses and electronic devices.
She took her first steps on August 26.
"They didn't think she would make it to her first birthday,GÇ¥ said Chris. "But I never thought about that. I always think about going and getting our hair and nails done, and wedding dress shopping and prom dress shopping. They don't think she'll be able to have her own babies, but that's minor.GÇ¥
Five-year-old Conner is his sister's greatest supporter. Chris shared that she and Mark are always careful to care for Conner and to take time out for him independent of Briella. They want to make sure that he knows that he is special, as well. They also expect him to contribute.
"He's a big help,GÇ¥ said Chris. "He loves helping and he always gets some sort of reward, like a cookie or whatever.GÇ¥
"He can tell you what's wrong with her heart, and what she's had done and how she's different,GÇ¥ she continued. "He uses medical terms; he's been there every step of the way. And for him, he doesn't know what a "normal' baby is. This is normal. This is our normal.GÇ¥
Briella participated in her first pageant in July, officially becoming royalty as she won the title of Miss Gold Dust in the baby division. In August, she also won the Miss FABU pageant in Tacoma.
Chris, Mark and Conner are looking forward to celebrating Briella's birthday on September 28; a birthday that some thought wouldn't come. Chris is planning a zebra-themed celebration with cake and lots of family and friends. She is amazed at all her daughter has been able to overcome and is looking forward to seeing who she becomes as she grows up.
"She's done so well - she hasn't let anything hold her back,GÇ¥ said Chris. "She's amazing.GÇ¥
Comments
Use the comment form below to begin a discussion about this content.
Sign in to comment