Emotions were running high on Rakell Weber’s first Mother’s Day as a new mom four years ago. After a stressful third trimester of her pregnancy, on April 22, 2019, Rakell gave birth to fraternal twins with birth defects. Her son Skyler was born with a partial cleft lip and daughter Evanley was born with a cleft lip and palate.
“I got stressed out and I cried for weeks I think.” Emotions were running high on Rakell Weber’s first Mother’s Day as a new mom four years ago. After a stressful third trimester of her pregnancy, on April 22, 2019, Rakell gave birth to fraternal twins with birth defects. Her son Skyler was born with a partial cleft lip and daughter Evanley was born with a cleft lip and palate.
For Rakell, a young single woman, the first days and months of motherhood took her and her mother Ember Weber through uncharted medical and emotional territory. They leaned heavily on one another and on the support of the Cleft Palate and Craniofacial team at University of Utah Health.
Dana Johns, MD, a board-certified plastic surgeon on the cleft team, has worked closely with the Webers beginning in the late stages of Rakell’s pregnancy through this Spring 2023 as the four-year-old twins grow and thrive.
From the beginning, Rakell and Ember stepped in and played a huge part to take care of the twins. They were just so on top of the situation and so willing to listen to what we had to say and work with our advice. Having newborn babies is tough, and then we are telling you to do all these additional things.
Some of the additional responsibilities that mother and grandmother took on involved feeding the babies. Skyler had an incomplete cleft lip but was still able to breastfeed. “It was incredible, and I was like, ‘Good job Rakell and good job Skyler,’” Ember said. However, Evanley struggled to eat and had a much harder time gaining weight.
Ember described how she and her daughter handled the challenge of getting Evanley to eat. “When Evanley left the Newborn Intensive Care Unit (NICU), she was still on a feeding tube and so they taught us to place the feeding tube in case she pulled it out when we brought her home,” Ember said. “She was on a feeding tube until she was about seven weeks old, and then we were able to get her completely bottle-fed. It was every three hours for seven weeks. We turned our living room into a nursery, with a recliner and a couch, and we took turns sleeping.”
Of course, making sure the babies were nourished and building up strength for the surgeries that lay ahead was only the beginning of the process to repair the twins’ cleft lips and palate. The Webers, who live in St. George, were often commuting up to Provo or Salt Lake City for appointments with the cleft team.
“Looking back, it was a lot of work, we were tired, we were sleep-deprived, but we had each other, and we drove to Salt Lake multiple times,” Ember said. “In the first four months of their lives, we had 80 doctors’ appointments.”
Skyler had successful surgery to repair his partial cleft lip when he was three months old in July 2019. Meanwhile, Rakell and Ember were strictly following the pre-surgical molding protocols for Evanley’s surgeries.
“(Rakell and Ember) did an outstanding job of lip taping, nose shaping, and placing the NAM device so that the difference between what Evanley looked like when she was born and what she looked like the day of surgery was substantially better,” Johns said. “The lip segments were much closer together and the gum lines were rotated into the position that they needed to be in. The nose was much more shaped up and the cartilage had been formed better because they followed that molding and pre-surgical treatment.”
Rakell and Ember believe the encouragement and trust Johns placed in them helped bring about positive surgical outcomes. “Dr. Johns was so good about giving us positive feedback,” Ember said. “She said, ‘You guys did such a good job of keeping the molding in,’ and then at eight months, she did the soft palate surgery. Then when Evanley was two years old, Dr. Johns did the bone graft surgery, which is quite early considering some kids have to wait until they are 10 or 12 to get the bone graft surgery because they are getting grafts from their own hips.”
Johns cited advancements in bone grafting over the past decade that allow for the use of a bony material that has been decellularized, and then doctors add a bone-growing protein into it. “The bony matrix is like honeycomb, and we add a stimulant that makes the patient grow their own honey into the comb, and they fill it in with their own bone,” Johns said.
So far, the results are nothing short of remarkable, according to Rakell and Ember.
Aesthetically, Skyler and Evanley look normal. It’s a miracle we can get this type of treatment from these multi-disciplinary experts who work with our children to make sure they have the best outcomes.
Thanks to the care the twins received from their mother, grandmother, and doctors, Johns sees excellent results in very different cleft cases. “Evanley’s care made a huge difference,” Johns said. “At this point, I think she probably has a comparable result from an aesthetic appearance to her brother, which is pretty outstanding.”
Today, at age four, the twins are in what Johns calls the watching stage, where the team keeps an eye on their growth, speech development, and appearance as they get their adult faces. It’s all part of what makes her job so rewarding. “If we are completing most of their cleft care before they turn age three, it just makes for a completely different childhood than kids who are undergoing these surgeries every three years during that super formative time of their life,” Johns said. “Our hope is that we just get to come and say hi and hang out with them once a year for the next 11 years or so.”
The Webers themselves could not be more optimistic and hopeful about the twins’ future. They are thankful for the guidance, support, and medical care that has given Skyler and Evanley such a bright future. “This was one of the most challenging things that I have ever walked with somebody through,” Ember said of her daughter Rakell. “I cannot express enough gratitude for the people that walked with us through this. They paved the way and made it a little easier because it is one of the hardest things you can go through in life.”
Note: The 2nd Annual Hirsche Smiles Charity Golf Tournament to benefit a Utah nonprofit that takes local plastic surgery and dental teams to underprivileged areas in Guatemala is set for Friday, June 2 at The Links at Sleepy Ridge in Orem. For more information, visit www.hirschesmiles.org.
Written by: Sandy Olney