Dawn James of Knoxville, Tennessee, was 16 weeks pregnant when a 3-D ultrasound determined that her unborn baby had severe spina bifida that left almost his entire spinal cord exposed. The baby was given zero chance of survival.
“We spoke with 10 specialists during my pregnancy, trying to see if we could find somebody who would give us hope or help. It was completely unanimous; every one of them turned us down. There had never been a child, in documented history, who had survived with the size lesion he had,” she said.
Devastated and full of fear, but with a strong sense of faith, Dawn and her husband, Will, decided to proceed with the pregnancy. The day that Dawn went into labor, she and her family were shown the private room where they would be able to say goodbye to their baby. Elijah (Eli) James was born at 37 weeks gestation on Dec. 16, 2010.
“We were told there was absolutely no way he could function, that he wouldn’t be able to breathe or swallow; he’d be a quadriplegic with no quality of life,” Dawn recalls.
Despite the tremendous odds stacked against him, Eli not only survived, but recently celebrated his fifth birthday.
Lessons in Love and Laughter
Eli has rachischisis, sometimes known as complete spina bifida, and as a result, has 29 additional diagnoses including hydrocephalus, chronic lung disease and scoliosis. Shortly after his birth, he had surgery to close the gaping hole in his spine and has undergone 34 additional surgeries in his short life, most of them at Monroe Carell Jr. Children’s Hospital at Vanderbilt.
Among those surgeries have been 10 revisions to the shunt in Eli’s brain to drain fluid caused by his hydrocephalus; major airway reconstruction surgeries for breathing issues; hiatal hernia repair, and feeding tube placement, which Dawn resisted for a while until physician assistant Matt Greiner explained to her that it would be the easiest procedure Eli would have to endure.
“It’s neat to watch his gift of changing the mood in a room. We’re so used to being in pre-op and in hospitals, so he’s not nervous when we’re in there. He puts other kids at ease. Every kid in there and all the parents laugh and talk with him,” Dawn said. “Eli is our miracle. He is pure joy to be around and always has a smile for us. Against all odds he is thriving.”
Caring for a child with such severe health issues is not easy, but fortunately, the James family has the support, expertise and friendship of a team of health care providers at Children’s Hospital. They sought out this team when Eli was 2 years old, when it became evident he would benefit from a nationally recognized children’s hospital staffed with pediatric neurosurgeons and a complex care program.
Early on, Eli was cared for by Noel Tulipan, M.D. In the 1990s, Tulipan, who died in 2015, began a groundbreaking journey that put Vanderbilt on the map in neurosurgery and fetal surgery. He questioned if a better way existed to treat babies born with open, neural tube defects, specifically spina bifida (myelomeningocele), the most common birth defect in the central nervous system.
Tulipan performed the first in utero repair of spina bifida in 1997, which subsequently led to the seven-year, landmark Management of Myelomeningocele Study (MOMS). Unfortunately, Eli did not qualify for the surgery because of the large size of his lesion, but the James family had at last found a medical home and is continuing under the care of Robert Naftel, M.D.
“We started coming to Vanderbilt for the pediatric neurosurgery group because there was only one pediatric neurosurgeon in Knoxville. Adult neurosurgeons, who sometimes covered pediatrics, were scared of Eli; his chart is intense. They didn’t know what to do with him,” Dawn said. “We decided that Vanderbilt was a much better option for us to care for how complex he has gotten.”
For the past three years, typically every two months, Dawn and Eli head west on I-40 for the three-hour drive to Children’s Hospital, which Eli refers to as “home.”
When he arrives, he greets everyone he meets with a smile and a hug. Amy Fleming, M.D., who cared for him during a hospital stay over a year ago, is often on the receiving end of his affection. Although she is now associate dean for medical student affairs at Vanderbilt University School of Medicine, she makes time to visit with the family when they arrive.
“There’s something about Eli that’s really special; there’s something about his mom that’s really special. Working with them is an incredible experience,” Fleming said. “Dawn is really educated about her son’s illnesses and congenital abnormalities. She is amazingly articulate and thoughtful and wants to listen and participate in the care of her child. It feels like a team effort, which is what I’m always going for with families, our nursing staff, residents and students.
“There is something amazing about this child and his family that makes all that come together.”
Eli is a quadriplegic and operates a powered wheelchair painted with the Superman logo. He is small for his age, weighing in at just 26 pounds. His downy soft blond hair is styled in a Mohawk, which is frequently dyed purple or blue.
After years of speech therapy, his verbal skills are age appropriate. He is still in a special needs preschool program, but the plan is for him to attend mainstream kindergarten next year, his mom said.
“He’s quite the character. His nicknames are the Mayor and the Dictator. He’s a complete social butterfly. He’ll wheel up to you and ask your name. He is the life of the party and has quite the sense of humor,” Dawn said.
A sense of humor goes a long way when caring for a child with as many complications as Eli.
“The first couple years of his life he would quit breathing frequently because he has severe laryngotracheomalacia. His trachea would collapse and he would go non responsive and turn blue,” Dawn said.
“I’ve done CPR on him many times. Since he’s gotten older, he’s figured out how to compensate for it and keep his airway open. I haven’t had to do CPR in almost three years now.”
Eli’s sister, Sky, 12, plays a big role in caring for him. Dawn refers to her as her assistant and says Sky knows as much about Eli’s needs as she does.
“She knows how to provide all of his care. She is the one who, at 7 years old, had the difficult task of sitting with him while I showered so if he quit breathing she could get me.
“She can catheterize him, run his feeding pump and give him a water bolus. She is my best assistant and the most amazing big sister.”
A Special Place for a Special Patient
At Children’s Hospital, Eli’s care is coordinated by the Program for Children with Medically Complex Needs, led by David Hall, M.D., professor of Clinical Pediatrics.
Hall and his team oversee this program for children with multisystem chronic disease. Like Eli, these children typically see many subspecialists and may be dependent on medical technology such as feeding tubes, tracheostomy tubes or ventilators. Subspecialty clinics are often not organized to provide the necessary comprehensive care, and primary care physicians can find it difficult to provide the time needed or to have easy access to the resources these children and their families require.
“As the region’s tertiary referral center for children with complex and coexisting medical issues, we want to be able to offer these children and their families more coordinated care that spans the inpatient and outpatient divide,” said Steven Webber, MBChB, MRCP, the James C. Overall Professor and chair of the Department of Pediatrics. “Providing our medically complex patients this service should improve the quality of care that they receive through improved coordination of services, reduction in return trips to our hospital and clinics, and shorter lengths of stay.”
Hall’s goal for his team, comprised of himself, two nurse practitioners, a scheduling coordinator, a nurse, a part-time social worker and a nutritionist, is to partner with patients’ primary care providers to coordinate their care and help families navigate what can be a complicated medical system. The team focuses on children who do not fit well into an established program.
“Their multisystem illnesses place them at risk for becoming ‘medical orphans’ with no place to go to unify their care,” Hall said. “Parents may go from specialist to specialist and sometimes receive confusing and conflicting advice.”
Medical advances have led to the survival of children who would not have survived in years past. In many cases their survival leads to a chronic condition that requires ongoing management rather than a complete cure. As a result, children with medical complexity occupy an increasing number of beds in children’s hospitals nationally, and have higher readmission rates and longer lengths of stay than typical children. The most complex account for 0.4-0.7 percent of all children, but 15-33 percent of total pediatric health care costs, Hall said.
“In our program we try to make decisions with families, not for families. Eli’s family displays a resilience I find admirable. It’s a privilege to be a part of their lives.”
To create a children’s hospital with such comprehensive resources took decades of vision, enthusiasm and persistence that continue today with the Growing to New Heights Campaign, a $40 million effort that will expand physical space of the hospital while strengthening its commitment to providing the highest quality care for children.
Patients and families, joined by community supporters and Vanderbilt leaders, gathered in September 2015 to celebrate the hospital’s continued commitment to fulfill the growing health care needs of children in Tennessee and throughout the region. Approximately 300 people attended festivities to sign a 10-foot white beam to mark the expansion milestone and signify the solid infrastructure of community support.
The expansion will allow Children’s Hospital and clinics, which already serve about 1,400 children and families each day, to advance the size and scope of their missions to provide world-class health care, train future generations of pediatricians and pediatric specialists and conduct groundbreaking research that leads to new treatments and cures.
“The outpouring of support from our community is making it possible to expand Monroe Carell Jr. Children’s Hospital at Vanderbilt,” said Vanderbilt University Chancellor Nicholas S. Zeppos. “Led by the Carell family and with enthusiastic participation by many individuals and organizations in our region and beyond, this campaign is truly a community effort on behalf of the children and families who need specialized care. I am grateful to everyone who has joined us so far.”
The James family is grateful for the care that Children’s provides to Eli, who continues to battle frequent health care setbacks and challenges. In early January he was airlifted to the hospital when his blood sugar level, blood pressure, body temperature and energy plummeted. Eli, naturally, enjoyed the helicopter ride, something he’d always wanted to do.
“We keep teasing Eli that he faked all of this just so he could ride in the helicopter,” Dawn said. “After we arrived at Vandy, he told me that we needed to go to another hospital so he could ride in a purple helicopter…yup, that’s my boy.”