When Lori Howell, MS, RN, first entered the field of fetal surgery, after nearly 15 years of clinical nursing experience in pediatric surgery, any mention of fetal surgery and its potential drew mostly skepticism. "You can't operate on a mother before a baby is born!" cried the naysayers. Insurance companies wouldn't hear of covering it.
That was then. Today, Howell is executive director for the largest fetal surgery center in the world, based at the Children's Hospital of Philadelphia (CHOP). And she has co-authored dramatic new research that has established fetal surgery as one effective treatment of myelomeningocele, the severest form of spina bifida.
Two decades in the making, this randomized clinical trial found that surgically repairing spina bifida in utero rather than after delivery, as is traditionally done, minimizes the birth defect's neurological damage. So convincing were the benefits of prenatal surgery, in fact, that the NIH, which funded the study, stopped it early.
"If it's the right thing to do, you keep fighting," said Howell, recalling how "you could have heard a pin drop" when the long-awaited results of the Management of Myelomeningocele Study were announced at the Society for Maternal-Fetal Medicine annual conference. "As a nurse, I have always been a patient advocate. I think that's what keeps me going."
MOTHER/BABY ADVOCATES: Joanna Horst, MSN, RNC, NEA-BC, (left) Susan Miesnik, MSN, CRNP, and Lori Howell, MS, RN, play leading roles in the fetal surgery program at Children's Hospital of Philadelphia.
Mia Capuano is just the kind of patient who can keep an advocate like Howell going. In 1999, Mia's mom, Giovanna Capuano, of Cape May County, NJ, learned via high-resolution ultrasound that the child she was carrying had spina bifida.
In this most common of all CNS birth defects, the spinal column of the fetus fails to close properly. Part of the spinal cord and surrounding nerves, usually encased in a sac, push through the vertebrae and protrude. Thus exposed, the fragile cord and nerves are continuously bathed in amniotic fluid over the course of gestation and become severely damaged.
Capuano's caregivers listed the dire possibilities awaiting Mia: paralysis, a feeding tube, cognitive delays, impaired bowel and bladder function, and hydrocephalus, a dangerous buildup of cerebrospinal fluid in the brain requiring surgical placement of a shunt to divert the fluid into the abdominal cavity. Mia's prognosis was especially grim: her spina bifida lesion was high up on her spine. Generally, the higher placed the defect, the more severe the neurologic consequences.
"They insisted on us terminating the pregnancy," Capuano explained, glancing at her daughter as the two sat in CHOP's surgical waiting area. "In fact, they tried to convince us before we walked out of the place to sign a consent to do so."
Instead, Giovanna and her husband, Louis, sought an evaluation at CHOP's Center for Fetal Diagnosis and Treatment after Giovanna's brother saw a spot on the local TV news about the hospital's fetal surgery program. Upon meeting certain medical and emotional criteria, Giovanna was approved for prenatal spina bifida surgery.
She had found another option besides termination - albeit a highly experimental one.
Closing the Defect
The repair - done when Giovanna was only 24 weeks into her pregnancy - went well.
At the risk of oversimplifying a complex operation, basically, surgeons made an incision across Giovanna's abdomen, opened her uterus, rotated the fetus to bring the spine into view, removed the protruding spina bifida sac, then closed and sealed the surrounding tissue and skin over the defect.
FAMILY AFFAIR: Giovanna and Louis Capuano, along with their daughter, Mia, visit with Lori Howell, MS, RN. They participated in fetal surgery when Giovanna was 24 weeks into her pregnancy.
During these procedures at CHOP, Susan Miesnik, MSN, CRNP, holds down several responsibilities as a frontline obstetrical care provider. Among other safety measures, she infuses the mother's uterus with a sterile physiologic solution to maintain uterine volume so the placenta doesn't detach prematurely from sudden decompression of the uterus. "I do a lot of hand-holding and talking to these moms," Miesnik said, "anything to help lessen the patient's anxiety because these families are scared to death. They have chosen to undertake a really complex surgery to hopefully improve the life of their child."
Premature birth is a risk for many mothers undergoing fetal surgery, but Giovanna's uterus never contracted in the 3 months prior to delivery. Her cesarean section, however, was problematic. She bled profusely and was in some danger. Surgeons couldn't deliver Mia for an hour while efforts focused on stopping Giovanna's blood loss. When she finally arrived, Mia seemed unscathed, weighing 7 pounds and posting two perfect 10 Apgar scores. Giovanna's difficult delivery highlights the other key piece of CHOP's fetal surgery program: a special labor and delivery unit. Overseeing this unit is a nurse who commutes 4 hours a day for the privilege.Special Delivery Unit
From the time she began her career in neonatology, Joanna Horst, MSN, RNC, NEA-BC, had a nagging feeling about the critically ill neonates brought to her from other hospitals. No matter how skillfully she cared for them, no matter how much love she doted on them, each baby seemed to be searching. "In my heart," Horst recalls thinking, "I knew that baby was searching for its mother." But the mother was lying in a hospital bed a county or city away. To Horst, this separation wasn't respectful to mother or baby.
Years later, while working in maternal child nursing at a community hospital, Horst saw a DVD of CHOP's new special delivery unit, an 8-room labor, delivery, recovery and postpartum unit. Thanks to this unit, mother and child could now remain at CHOP, together, throughout the entire birth experience. Horst was entranced. "As I watched the DVD, I thought, 'Here it is. This is the answer,'" she said. "This is what my mind and my heart have been working on for decades." Now she leads the very unit she first loved from afar.
Howell also feels especially proud of the Garbose Family Special Delivery Unit. "It gives us the best environment to perfect prenatal surveillance, delivery and resuscitation techniques for babies with birth defects, all the while keeping mom and baby together in the same institution so that if the baby does not survive they have precious time together," she said. The special delivery unit is the only unit of its kind in the world, designed exclusively for mothers carrying babies with birth defects. It is staffed by more than 30 experienced nursing professionals, including staff nurses, certified nurse midwives, perinatal advanced practice nurses, and labor and delivery RN staff.
Standard of Care
On the strength of the aforementioned trial, published Feb. 9 on the New England Journal of Medicine's website, fetal surgery has graduated from an experimental treatment to a standard of care for spina bifida in institutions with experience in the procedure. Advocates hope this enhanced status will convince insurers to pay for it. National Blue Cross and Blue Shield, for one, has already amended its policy. "To watch this process evolve from something that was strictly experimental to something that is now proven science, a standard of care, has really been exciting," Miesnik said.
It's not a panacea, and not for everyone. "Fetal surgery entails risks such as the incision on the uterus thinning and opening prior to delivery, as well as risks of infection, the need for a blood transfusion at delivery, and risk of premature birth," Howell cautioned. "It requires a center equipped 24/7 to handle these situations."
On a lobby wall at CHOP hang photos taken from annual reunions of all families referred to the Center for Fetal Diagnosis and Treatment. Among those faces is Miesnik's first patient, who underwent the surgery in September 2001.
"That patient and I remain in touch today," she said. "Her daughter will be 10 in November and is taking ballet lessons and behaving like a normal 10-year-old, which has been a thrill to see."
Passion for Dancing
As for Mia, she is now in sixth grade, loves science and music, and rides horses. Almost 12 years old, Mia has avidly pursued jazz dancing and "acro" dancing since age 3. "She's very determined," said Louis, her father. "She would go out in the yard every day and practice cartwheels. When she did her recital that first year and did her cartwheel across the stage, the place just went nuts."
Mia has full bowel and bladder control and never developed hydrocephalus. She did experience excruciating back pain and a loss of stamina in 2010 and returned to CHOP for surgery to "un-tether" her spinal cord. A child's spinal cord is meant to slide up and down freely within the spinal column as the child moves. In most children with spina bifida, however, the spinal cord is held in place by surrounding tissue or "tethered."
Aside from un-tethering surgery, and some upcoming botox treatments to relieve contractures behind her knees, she has never looked back. Mia politely refused ADVANCE's entreaties to show off her cart-wheeling abilities. She dutifully answered all questions but seemed to be thinking: what's all the fuss about? She just wanted to get home to Cape May, back to her busy life.
Michael Gibbons is an editor at ADVANCE.