From patient to advocate: Trinity’s journey with cleft lip and palate

After a normal pregnancy and healthy delivery, Trinity was diagnosed at birth with cleft lip and palate, birth defects that occur when a baby’s lip or mouth do not form properly during pregnancy.

Her parents, Krisha and Jason, didn’t know much about these conditions at the time. They were quickly referred to the CHOC Children’s Cleft and Craniofacial Program, which includes a team of multidisciplinary specialists including plastic surgeons, otolaryngologists (ear, nose and throat), dentists, and more. Krisha and Jason relied on their CHOC team for help, education and resources regarding their daughter’s diagnosis.

Trinity underwent her first in a long series of surgeries when she was just 10 weeks old. Although it’s been a decade since that day, her parents still remember waiting for Trinity to emerge from that first surgery.

“In that first surgery, minutes felt like hours,” says Krisha. “Having to give up your 10-week-old baby knowing that they’re going to look different when they come out—it was a scary situation.”

Although Krisha and Jason knew their little girl was in good hands at CHOC, they were understandably worried about her surgery. What helped was memories of Jason’s own experience as a CHOC patient when he was a child.

“It’s been over 30 years since I was a CHOC patient, but I still remember that CHOC took really good care of me,” he says. “Even then, I remember CHOC being a really neat place. It’s so family-oriented.”

trinity-at-choc
Trinity at CHOC Children’s Hospital, before a surgery.

Over the last ten years, Trinity has returned to CHOC for more surgeries, check-ups and other care. This has included surgeries to improve the appearance and function of her lip and palate, multiple ear tube procedures, and a palate expander. She’s also undergone multiple bone grafts where bone is taken from her hip and grafted into her mouth and gum line. She has had multiple ear tube procedures, a palate expanded etc. Future surgeries include jaw surgery, nasal surgery, orthodontia, and potentially additional lip and palate repairs.

“People assume that cleft lip and palate is just a cosmetic deformity, but it’s so much more than that,” Krisha says. “Trinity sees a wide variety of specialists, including a craniofacial plastic surgeon, a craniofacial orthodontist, an otolaryngologist (ENT), a speech pathologist, an audiologist, and more.”

Becoming part of her own care team

As Trinity has gotten older, she’s taken a more active role in her care—especially when interacting with Dr. Daniel Jaffurs, medical director of plastic surgery and craniofacial services at CHOC.

“Dr. Jaffurs acknowledges Trinity first when he walks in the room, before he speaks to us as parents. He explains everything on her terms, and then he will address us as parents.”

This confirms to Krisha that Dr. Jaffurs knows that Trinity is the most important person in the room and has helped foster the bond between doctor and patient.

“Dr. Jaffurs is my favorite doctor,” Trinity says. “He’s really nice. When I was little, I was afraid of doctors, but I’m not afraid anymore.”

A lifelong love of music

Children with cleft lip and palate often have trouble speaking clearly because the structure of their mouth and nasal cavity makes it hard to produce certain sounds. Although Trinity underwent speech therapy when she was younger to normalize her communication abilities, anytime she sang, it always came out clear. She also taught herself to play piano when she was just 8 years old.

“Whenever I have surgery and I don’t feel good right after, I put on music and then I start to feel like my normal self again,” Trinity says.

Always the performer, Trinity also participates in school musicals and dreams of being a professional musician someday.

A budding advocate

Trinity has become an advocate for other craniofacial patients.

“She’s always made her story known,” says Krisha. “She’s had situations in school where other kids ask questions about how she looks, and instead of getting mad, she brings in her baby book and says, ‘I’m going to tell you why I’m different.’”

Before performing a song at an annual picnic that brings together craniofacial patients and families, she gave an impromptu speech about confidence to the 400-person crowd.

“If someone makes fun of you because of how you look, get back up again and don’t let them bother you anymore,” she told the crowd. “You are special, and you were born exactly who you were supposed to be.”

This message was in line with what her parents have been telling her since she was born.

“I never wanted her to forget where she came from,” Krisha says. “Even though she was undergoing all these surgeries, I wanted her to know that she was beautiful and perfect the way she was born.”

Trinity continues to increase craniofacial awareness in her community. She often shares her story with her classmates and once even spoke to her school board about her surgeries.

trinity-and-family
Trinity with her family.

Trinity and her family find value in connecting with other craniofacial families. Their decade-long relationship with CHOC’s craniofacial team has given them an inside look on how CHOC and the department have evolved.

“The craniofacial program at CHOC has grown so much in the past 10 years. It’s incredible to see what they can do,” says Krisha. “This program has changed our lives for the better.”

The importance of community support

Support from their community means the world to Trinity and her family.

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Trinity paints a picture frame in a playroom at CHOC Children’s while recovering from surgery.

“Donors have a big role. They help CHOC be up to date on everything,” says Jason. “CHOC is constantly evolving and in terms of the latest technology and procedures. It’s neat to see everybody coming together to make all this accessible for families like us.”

To Trinity’s family, CHOC is an essential part of the community.

“It changes families’ lives,” says Krisha. “It definitely does. It changed our lives.”

Trinity plans to keep on singing, inspiring others and returning to CHOC to continue her treatments.

“Hands down, CHOC makes everything better,” she says. “CHOC is the friend you never knew you had.”

Learn more about CHOC's Cleft and Craniofacial Program

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CHOC Children’s Expands Plastic Surgery Services

A pediatric plastic surgeon specializing in reconstructive plastic surgery has joined CHOC Children’s. Dr. Raj Vyas sees patients with a variety of complex conditions including clefts and craniofacial anomalies.

plastic surgery
Dr. Raj Vyas

“Care at CHOC is delivered in such a multidisciplinary fashion, allowing for complex higher-level discussions and nuanced treatment planning in both standard and exceptional circumstances. There is also an emphasis on safety and process improvement, both critical for a state-of-the-art facility serving a complex and diverse patient population,”Dr. Vyas says.

During his third year of medical school at UCLA David Geffen School of Medicine, the  Southern California native discovered that plastic surgery was his newfound passion.

“I unexpectedly fell in love with plastic surgery, particularly craniofacial surgery,” Dr. Vyas explains.  “My appreciation for the depth and breath of plastic surgery grew from my experiences during this rotation in medical school. I remember seeing a child with hypertelorism undergo a facial bipartition and I was fascinated.”

Following medical school, he completed a plastic surgery residency in Boston at the Harvard Combined Plastic Surgery Residency Program. He then completed a craniofacial surgery fellowship at New York University Medical Center, where he treated children and adults undergoing facial reconstruction for congenital anomalies or after suffering trauma and cancer. He also gained expertise in microvascular surgery and virtual surgical planning.

Dr. Vyas is working with the team at CHOC to build a multidisciplinary program in facial reanimation. By combining craniofacial surgery with microsurgery, he hopes to restore facial form and function in children and teens with facial paralysis.

“We have a great team at CHOC. Dr. Daniel Jaffurs has been an incredible mentor,” Dr. Vyas says. “My goal is to help make the program the best in Southern California and beyond.”

Dr. Vyas enjoys working with virtual scanning and 3D modeling technology available at CHOC, as it helps him plan and ensure optimal safety and precision before a procedure.

“Today, we are able to use cone beam CTs to significantly minimize radiation exposure while still obtaining high quality 3D imaging. This allows for virtual surgical planning that is both precise and extremely effective in reducing uncertainty in the OR,” Dr. Vyas explains. “In neonates with upper airway obstruction, for instance, who are scheduled to undergo mandibular distraction, we are able to customize bony osteotomies in a way that prevents injury to dental structures and nerves, while optimizing distraction vectors that improve the airway and maintain cosmetic considerations. We can also virtually anticipate the extent of facial movements and design custom splints that facilitate orthognathic (upper and lower jaw) surgery. The benefit of these types of pre-planned procedures are expanding as surgeons utilize this technology in a variety of craniofacial reconstructions.”

When this dedicated physician is not busy helping patients at the hospital, he volunteers with Global Smile Foundation, a nonprofit organization that treats children with facial congenital deformities in underserved countries. Most recently he went to Trujillo, Peru, where he helped over 100 patients.

“It’s so rewarding to work with children,” he says. “Their reconstructions have a long-life impact.”

Dr.Vyas is board certified in plastic surgery and a member of the American Society of Plastic Surgeons, American Society of Craniofacial Surgery, American Cleft Palate-Craniofacial Association and International Cleft Lip and Palate Foundation, among other professional organizations.

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New Laser Treats Children with Vascular Anomalies

Many babies are born with small blemishes—a little patch of redness here, a birthmark there. These typically aren’t cause for concern, and most go away on their own. But what if they don’t?

Casey at birth and at two months old
Casey at birth and at two months old.

When Casey Lang was born, she had two small marks on her body: one on her left cheek, and one on her abdomen. “As the doctors were coming in to check on her, we kept asking if it was normal. They said it was a stork bite and that it would go away,” mom Michelle says. “A couple of weeks went by and it started to get darker and larger. I kept telling my husband, this is not right.”

By the time Casey was two months old, the blemish on her face had become blotchy and was encroaching on her eye, and the growth on her abdomen had grown to the size of a lime. Her parents were getting increasingly concerned.

Getting Answers

Michelle took Casey to her pediatrician, who referred her to CHOC Children’s plastic surgeon Dr. Daniel Jaffurs. Immediately upon seeing Casey, Dr. Jaffurs diagnosed the marks as infantile hemangiomas and consulted with the rest of the team from the CHOC Children’s Vascular Anomalies Center. They recommended that Casey be admitted to the hospital that day for comprehensive testing, to determine the severity of the hemangiomas.

“They started her on propranolol in the hospital and the journey started from there,” Michelle says. “It was a year on the medication, and we came to CHOC every single month. The medication was remarkable. It brought down the hemangioma on her face and opened up her eye.”

The growth on Casey’s stomach did not respond as well to the medication and was surgically removed by Dr. Jaffurs. What remained of the hemangioma on Casey’s face, however, could be treated with a simple procedure that had just become available at CHOC.

No Surgery, No Scar

CHOC’s new pulsed dye laser (PDL) is a minimally invasive treatment for hemangiomas, port-wine stains and post-surgical scarring anywhere on the body. The laser delivers very quick pulses of energy at a specific wavelength that is absorbed into the skin, destroying the abnormal blood vessels just below the surface. CHOC uses the latest PDL model—the Vbeam Perfecta— because of its level of precision, which is especially important when lasering sensitive areas like near the eye.

“With this new laser, we sometimes can avoid an operation which leaves a lasting scar,” Dr. Jaffurs says. “And, you can see the results immediately.”

Casey after her first laser treatment, and after her second laser treatment
Casey after her first laser treatment, and after her second laser treatment.

CHOC’s pediatrics-trained anesthesiologists give patients a small amount of anesthesia using a mask, to relax them and minimize movement during the procedure. Patients are sent home the same day; side effects are very minimal and may include slight pain or bruising. The number of treatments needed depends on the location and size of the vascular anomaly.

Casey was one of the first patients at CHOC to be treated with the pulsed dye laser and after just two treatments, the hemangioma on her face is nearly gone. Most patients require three to five treatments depending on the severity of the malformation.

“I want other parents to know that if their child has this, there is a cure for them,” Michelle says. “The team they have at CHOC, it’s just amazing, and if you go there, you’re going to get answers.”

The CHOC Children’s Vascular Anomalies Center brings together pediatric specialists in hematology, plastic surgery, head and neck surgery (ENT), cardiology and more to assess and treat all forms of vascular anomalies and malformations in children. For more information, call 714-509-3313.

CHOC’s new pulsed dye laser is generously supported by the Nora and Charles Hester Endowment for Craniofacial Care.

Genetic Syndrome Causes Cleft Lip, Palate in Sisters

CHOC Children’s Cleft and Craniofacial Program Just like most sisters, Tristyn and Raigyn Snyder will share toys, clothes and friends throughout their lives. They also share something unique: a genetic condition called Van der Woude syndrome.

In the Genes

When Cindy Snyder was 12 weeks pregnant with Tristyn, a prenatal ultrasound showed that the baby had a bilateral cleft lip and palate. After Tristyn was born, specialists at the CHOC Children’s Cleft and Craniofacial Program noticed she also had small indentations on her bottom lip, or “lip pits,” a key marker of Van der Woude syndrome (VWS).

Van der Woude syndrome is the most common single-cause of cleft lip and palate. Individuals with VWS may have a cleft lip with or without a cleft palate, a submucous cleft palate, lip pits along the lower lip, or any combination of these features. Tristyn’s dad, Matt, also has Van der Woude syndrome and had a unilateral cleft lip as a child.

“If a child’s mother or father has Van der Woude syndrome, each child would have a 50 percent chance of also having the condition,” according to Dr. Neda Zadeh, a CHOC Children’s genetic specialist. “VWS can present differently in each person. For instance, a mom may have had lip pits, but her child could have a cleft palate and no evidence of lip pits.”

Shortly after Tristyn’s birth in 2011, she had a nasoalveolar molding (NAM) device fitted inside her mouth to partially close her cleft. Plastic surgeon Dr. Michael Sundine performed surgery at CHOC to repair Tristyn’s lip when she was seven months old, and he did a second surgery when she was 10 months old to finish her palate repair and remove the lip pits.

Now 3 years old, Tristyn barely shows any physical evidence of her condition. Other than a need for speech therapy, “No one even knows anything happened to her,” Cindy says.

Baby Raigyn after her surgery.
Baby Raigyn after her surgery.

The Decision to Expand Their Family

“We wanted to have another child, regardless,” Cindy says. “Some people are like, how could you have another one? Tristyn is so happy and healthy. How could we not have another one?”

In 2014, Cindy and Matt were thrilled to learn they were expecting another girl, Raigyn. An ultrasound showed that Raigyn’s lip was not forming properly, a sign that she likely had Van der Woude syndrome. A genetic test could have confirmed it, but the Snyders opted out.

“We didn’t really need to know either way, and we felt like the cleft was enough to know,” Cindy says.

The Snyders were prepared when Raigyn was born with a bilateral cleft lip and palate. She wore a NAM device and recently had surgery to repair her lip. In a few months, she’ll have another procedure to repair her palate. Cindy is confident that Raigyn will continue to develop normally, just like her big sister.

“They are perfectly healthy children,” Cindy says. “Everyone’s born with different things, and this is just what they were born with.”

Learn more about cleft and craniofacial services at CHOC.
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Understanding Pierre Robin Sequence in Babies

CHOC Children’s is the only hospital in Orange County to offer comprehensive treatment and surgery for mandibular_distractionpatients with a facial condition that can limit breathing in infants.

Pierre Robin Sequence is a birth condition wherein a baby has a small jaw and a small, receding chin. As a result, the tongue tends to be displaced back toward the throat, where it can fall back, block the airway and cause difficulty breathing. Most of these infants will also have a cleft palate. If left untreated, Pierre Robin Sequence can be a fatal condition.

To correct this condition, CHOC plastic surgeons perform an operation within the baby’s first few weeks of life called Mandibular Distraction. This procedure moves the jaw forward and reorients the tongue so it doesn’t block the airway.

Mandibular Distraction is a unique surgery.  This operation can be done on the smallest of the small. Babies can’t breathe well because their tongue is blocking their airway.

Performing this surgery on babies with Pierre Robin Sequence reduces or eliminates the chance the baby will need a tracheostomy, or a hole cut into the windpipe, to breathe.

It’s important for parents to know that the condition can be treated successfully when diagnosed.

Sometimes children are born with this and it’s not recognized or diagnosed. Babies are sent home and parents notice their baby is not breathing well or – even worse – turning blue.

Children who also have a cleft palate are later treated with surgery at the optimal age of 9 to 12 months.

Learn more about CHOC’s craniofacial program.

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