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Sheyenne Davis was born with a rare and complex array of vascular abnormalities called PHACE. Doctors near Davis' hometown predicted the newborn wouldn’t live until her first birthday. Rather than settle for putting their baby girl in hospice care, as doctors had advised, Sheyenne’s parents instead sought a second opinion with the Hemangioma and Vascular Malformation Center at Cincinnati Children’s Hospital Medical Center.
Sheyenne with her physician, Dr. Denise Adams, in April 2007.
“She’s just seems like a normal 2-year-old, the busy little thing, and just looking at her you’d never know she has a medical issue,” Joy Davis says. “Sheyenne’s story is a true miracle, but it isn’t just about Sheyenne. It’s also about the people put here to help make miracles happen. It wouldn’t have been possible without the care and concern we received at Cincinnati Children’s.”
Sheyenne’s primary physician at Cincinnati Children’s is Denise Adams, MD, medical director of the Hemangioma and Vascular Malformation Center and a physician in the Cancer and Blood Diseases Institute. In directing Sheyenne’s care, and that of other children, Dr. Adams helps lead a multidisciplinary team of surgeons and other caregivers who are building a national reputation for successfully diagnosing and treating vascular anomalies.
PHACE Syndrome (or PHACE) is a group of anomalies that include a rare association of hemangiomas (non-cancerous vascular tumors) of the face and birth defects that affect the brain, heart, eyes or skin. Relatively little is known about PHACE, and this can lead to misdiagnosis, according to Dr. Adams.
“There is some misdiagnosis because PHACE is so poorly misunderstood,” she says. “Sometimes children get the wrong treatment or it may not be known what the disease is. If parents have doubts or concerns about a diagnosis, they can come to a multidisciplinary center like ours.”
In Sheyenne’s case, an intense focus at Cincinnati Children’s on vascular anomalies and conditions like PHACE, as well as a multidisciplinary approach to diagnosis and treatment, made a difference. According to Joy Davis, it helped transform what had been a devastating diagnosis into one of hope, followed by lifesaving treatment and care.
Sheyenne’s parents, who live in Pennsylvania, noticed something about their new daughter the moment she was born. The infant had what looked like a bruise on her face, Joy explains. It covered Sheyenne’s cheeks, lower lip, chin and neck. Initially, it was diagnosed as a birthmark, but a couple of weeks after her birth, a dermatologist confirmed that Sheyenne had a hemangioma, and might have PHACE – a diagnosis that was soon confirmed.
Sheyenne had her first echocardiogram when she was 3 weeks old. It showed a coarctation of her aorta, a severe and likely fatal congenital defect common in PHACE. When Sheyenne was evaluated for corrective surgery, the parents were told their new baby girl was not a candidate. Physicians told the Davises that Sheyenne had suffered a stroke – a risk of PHACE – and, according to Joy Davis, “that her anatomy was something they had never seen before.”
The “something” turned out to be Sheyenne having three of the five anomalies linked to PHACE – aortic coarctation, arterial anomalies (including absence of her left carotid and vertebral arteries) and the hemangiomas. Then, Joy Davis says, the doctors told the Davises something “that no parent wants to hear – that there is no hope for your child. They sent us home on hospice care for our terminally ill newborn baby.”
The Davises found out about the work of Dr. Adams and the team at Cincinnati Children’s when, like so many parents with severely ill kids, Sheyenne’s father, Orvie, went on the internet to search for hope. When they brought their daughter to Cincinnati, only a few weeks had passed since the Davis family had been devastated by Sheyenne’s bleak prognosis. Then, something very different happened, Joy explains. They met Dr. Adams.
“She was the first sign of hope for Sheyenne and our family. Within only a few moments of meeting our daughter, Dr. Adams saved her life for the first of many times,” Joy says.
In short, Sheyenne was very sick, indeed. But there was no sign of stroke, or heart failure, and after Sheyenne was given a little time to get stronger – which included an immediate blood transfusion to boost her hemoglobin – she would be scheduled for corrective cardiac surgery. It was in typical multidisciplinary fashion for Cincinnati Children’s that teams from several different divisions of the medical center helped turn around Sheyenne’s medical condition for the better.
Pediatric surgeon Roshni Dasgupta, MD, found a proliferating and inoperable hemangioma on Sheyenne's spleen from a biopsied growth. Dr. Adams used the chemotherapy drug Vincristine to treat Sheyenne's facial and internal hemangiomas. The treatment was started at Cincinnati Children's and then she was able to resume treatments at her home hospital.
When Sheyenne returned to Cincinnati Children’s for further treatment cardiologist Russel Hirsch, MD, and cardiothoracic surgeons Peter Manning, MD, and Pirooz Eghtesady, MD, began preparations for surgery to address her complicated aortic arch reconstruction. The successful surgery was performed by Dr. Eghtesady. The repair was challenging because of Sheyenne’s unusual anatomy. Her aortic arch combined with the absence of other important arteries were causing inadequate blood supply to her brain.
Sheyenne has been off Vincristine since January of 2008 and is doing well, her mom says. The hemangioma on her spleen is monitored with ultrasound and the latest findings show that it has decreased in size. She also had a hemangioma removed from the left inner side of her lip and she has begun laser treatment on the visible veins on her face. Now back home with her family, Sheyenne is hitting all of her developmental milestones as she enjoys looking at books and being with her three sisters.
Joy Davis knows her family’s journey is not over. Sheyenne’s ongoing health status must be, and is, monitored closely by the team at Cincinnati Children’s, including Joy regularly sending photos of Sheyenne to Dr. Adams’ team by e-mail. But compared to where matters stood a couple of years ago, Joy takes great comfort in her growing family, and knowing that Dr. Adams and others at Cincinnati Children’s are just a phone call, or e-mail, away.
If you have had an experience with the Cancer & Blood Diseases Institute, we invite you to share your story.
Sheyenne enjoys the life of any active 2 year-old, including pumpkin painting.
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