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Bella when she was sick

Preserving brain function by purposely inducing strokes

Bella when she was sick

Born to young parents, no prenatal testing had suggested any problems with Bella’s brain. But just a few hours after birth, Bella suffered her first seizure – one of many that would follow in the ensuing days. After brain imaging, her doctors in Iowa diagnosed her with hemimegalencephaly.

Strokes are neurologically devastating events, cutting off life-sustaining oxygen to regions of the brain. If these brain tissues are deprived of oxygen long enough, they die, leading to critical loss of function – and sometimes loss of life.

“As physicians, we’re taught to prevent or treat stroke. We’re never taught to inflict it,” says Taeun Chang, M.D., director of the Neonatal Neurology and Neonatal Neurocritical Care Program at Children’s National Hospital.

That’s why a treatment developed at Children’s National for a rare brain condition called hemimegalencephaly is so surprising, Dr. Chang explains. By inflicting controlled, targeted strokes, Children’s National physician-researchers have treated five newborns born with intractable seizures due to hemimegalencephaly before they’re eligible for epilepsy surgery, the standard of care. In the four surviving infants, the procedures drastically reduced or completely relieved the infants of hemimegalencephaly’s characteristic, uncontrollable seizures.

The most recent patient to receive this life-changing procedure is Bella, a 13-month-old from Iowa whose treatment at Children’s National began within her second week of life. Born to young parents, no prenatal testing had suggested any problems with Bella’s brain. But just a few hours after birth, Bella suffered her first seizure – one of many that would follow in the ensuing days. After brain imaging, her doctors in Iowa diagnosed her with hemimegalencephaly.

A congenital condition occurring in just a handful of children born worldwide each year, hemimegalencephaly is marked by one brain hemisphere growing strikingly larger and dysplastic than the other, Dr. Chang explains. This abnormal half of the brain is highly vascularized, rippled with blood vessels needed to support the seizing brain. The most conspicuous symptoms of hemimegalencephaly are the numerous seizures that it causes, sometimes several in the course of an hour, which also may prevent the normal half of the brain from developing and learning.

Prior studies suggest early surgery achieves better developmental outcomes with one study reporting as much as a drop of 10-20 IQ points with every month delay in epilepsy surgery.

The standard treatment for unilateral megalencephaly is a dramatic procedure called a hemispherectomy, in which surgeons remove and disconnect the affected half of the brain, allowing the remaining half to take over its neurological duties. However, Dr. Chang says, implementing this procedure in infants younger than 3 months of age is highly dangerous.  Excessive, potentially fatal blood loss is likely in infants younger than 3 months who have a highly vascularized brain in the setting of an immature coagulation system. That leaves their doctors with no choice but to wait until these infants are at least 3 months old, when they are more likely to survive the surgery.

However, five years ago, Dr. Chang and her colleagues came up with a different idea when a newborn continued to have several seizures per hour despite multiple IV seizure medications: Because strokes cause irreversible tissue death, it might be possible to effectively incapacitate the enlarged hemisphere from within by inflicting a stroke on purpose. At the very least, this “functional embolization” might buy time for a traditional hemispherectomy, and slow or halt ongoing brain damage until the infants are able to withstand surgery. Ideally, this procedure may be all some children need, knocking out the offending hemisphere completely so they’d never need a hemispherectomy, which has late complications, such as hydrocephalus.

A pediatrician friend of Bella’s paternal grandparents read a story on Children’s National website about Darcy, another baby who’d received functional embolization a year earlier and was doing well. She contacted Dr. Chang to see if the procedure would be appropriate for Bella.

Within days, Bella and her family headed to Washington, D.C., to prepare for functional embolization herself. Within the first weeks of life, Bella underwent three separate procedures, each three to four hours long. Under real-time fluoroscopic and angiographic guidance, interventional neuroradiologist Monica Pearl, M.D., threaded a micro-catheter up from the baby’s femoral artery through the complex network of blood vessels all the way to her brain. There, in targeted branches of her cerebral arteries, Dr. Pearl strategically placed liquid embolic agent to obstruct blood flow to the abnormal half of Bella’s brain.

Immediately after the first procedure, the team had to contend with the same consequences that come after any stroke: brain swelling that can cause bleeding and herniation, complicated further by the already enlarged hemisphere of Bella’s brain. Using neuroprotective strategies learned from treating hundreds of brain-injured newborns, the neonatal neurocritical care team and the neonatal intensive care unit (NICU) minimized the brain swelling and protected the normal half of the brain by tightly controlling the brain temperature, her sugar and electrolyte levels, her blood pressure and coagulation system.

As the brain tissue in the oversized hemisphere died, so did the seizures that had plagued Bella since birth. She has not had a seizure since she left Children’s National more than one year ago. Her adoptive parents report that Bella is hitting many of the typical developmental milestones for her age: She’s getting ready to walk, blowing kisses and saying a few words. Physical, speech and occupational therapy will keep her moving in the right direction, Dr. Chang says.

“We believe that Children’s National is the only place in the world that’s treating newborns in this way to preserve their futures,” Dr. Chang says. “We’re privileged to be able to care for Bella and other kids with this rare condition.”

Bella’s transfer and successful procedures required the support and collective efforts of many within the hospital organization including William D. Gaillard, M.D., and his surgical epilepsy team; interventional neuroradiology with Dr. Monica Pearl; Neurosurgery; Neonatology and the NICU; social work; and even approval from Robin Steinhorn, M.D., senior vice president of the Center for Hospital-Based Specialties, and David Wessel, M.D., executive vice president and Chief Medical Officer.

“While obvious credit goes to the medical team who saved Bella’s future and the neonatal intensive care nurses who provided exceptional, intensive, one-on-one care, Bella’s team of supporters extend to all levels within our hospital,” Dr. Chang adds.

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Robin Steinhorn in the NICU

Coming together as a team for the good of the baby

Robin Steinhorn in the NICU

Children’s National has a new program to care for children who have severe bronchopulmonary dysplasia, a serious complication of preterm birth.

Around the 1-year-old’s crib is a tight circle of smiling adults, and at the foot of his bed is a menagerie of plush animals, each a different color and texture and shape to spark his curiosity and sharpen his intellect.

Gone are the days a newborn with extremely complex medical needs like Elijah would transfer from the neonatal intensive care unit (NICU) to the pediatric intensive care unit and transition through a couple of other hospital units by the time he was discharged. Gone are the days when he’d see a variety of new physician faces at every stop. And gone are the days he’d be confined to his room, divorced from the sights and sounds and scents of the outside world, stimulation that helps little baby’s neural networks grow stronger.

Children’s National has a new program designed to meet the unique needs of children like Elijah who have severe bronchopulmonary dysplasia (BPD), a common complication of preterm birth.

“It’s more forward-thinking – and I mean thinking for the future of each individual baby, and it’s allowing the baby to have one team and one location to take advantage of a deep knowledge of and relationship with that baby and family,” says Robin Steinhorn, M.D. Dr. Steinhorn is senior vice president of the Center for Hospital-Based Specialties and one of Children’s multidisciplinary team members who visited Elijah’s bed twice weekly during his lengthy hospitalization and who continues to see him regularly during outpatient visits.

“The pulmonologist, the neonatologist, the respiratory therapist, the physical therapist, the dietitian, the cardiologist – we all come as a team to work together for the good of the baby,” Dr. Steinhorn adds. “We stick with these babies through thick and thin. We will stick with that baby with this team and this location until they are ready to go home – and beyond.”

BPD, a serious lung condition, mostly affects extremely low birthweight preterm babies whose lungs were designed to continue developing inside the womb until the pregnancy reaches full term. Often born months before their due dates, these extremely vulnerable newborns have immature organs, including the lungs, which are not ready for the task of breathing air. Children’s program targets infants who experience respiratory failure from BPD. The respiratory support required for these infants ranges from oxygen delivered through a nasal cannula to mechanical ventilators.

Robin Steinhorn and Colleague

“It’s more forward-thinking – and I mean thinking for the future of each individual baby, and it’s allowing the baby to have one team and one location to take advantage of a deep knowledge of and relationship with that baby and family,” says Robin Steinhorn, M.D.

About 1 percent of all preterm births are extremely low birthweight, or less than 1,500 grams. Within that group, up to 40 percent will develop BPD. While they represent a small percentage of overall births, these very sick babies need comprehensive, focused care for the first few years of their lives. And some infants with severe BPD also have pulmonary hypertension which, at Children’s National, is co-managed by cardiology and pulmonary specialists.

Children’s BPD team not only focuses on the child’s survival and medical care, they focus on the neurodevelopmental and social care that a baby needs to thrive. From enhanced nutrition to occupational and physical therapy to a regular sleep cycle, the goal is to help these babies achieve their full potential.

“These babies are at tremendous risk for long-term developmental issues. Everything we do is geared to alleviate that,” adds John T. Berger III, M.D., director of Children’s Pulmonary Hypertension Program.

“Our NICU care is more focused, comprehensive and consistent,” agrees Mariam Said, M.D., a neonatologist on the team. “We’re also optimizing the timing of care and diagnostic testing that will directly impact health outcomes.”

Leaving no detail overlooked, the team also ensures that infants have age-appropriate developmental stimuli, like toys, and push for early mobility by getting children up and out of bed and into a chair or riding in a wagon.

“The standard approach is to keep the baby in a room with limited physical or occupational therapy and a lack of appropriate stimulation,” says Geovanny Perez, M.D., a pulmonologist on the team. “A normal baby interacts with their environment inside the home and outside the home. We aim to mimic that within the hospital environment.”

Dr. Steinhorn, who had long dreamed of creating this comprehensive team care approach adds that “it’s been so gratifying to see it adopted and embraced so quickly by Children’s NICU caregivers.”

Robin Steinhorn

Children’s National senior vice president elected to American Pediatric Society leadership

Robin Steinhorn

Robin Steinhorn, M.D., Senior Vice President of Center for Hospital-Based Specialties at Children’s National Health System, was elected by her peers to become vice president and president-elect of the American Pediatric Society (APS) beginning May 2018 at the annual Pediatric Societies Meeting in Toronto, Canada. Dr. Steinhorn will serve in this role for one year and will then become the Society’s president in May 2019 for a one-year term.

Dr. Steinhorn is a globally recognized physician-leader, researcher and clinician in the fields of neonatal perinatal medicine and fetal pulmonary development. She was elected to the APS Council in 2015 and currently holds a seat on the American Board of Pediatrics’ Board of Directors.

“Dr. Steinhorn has devoted her professional career to advancing the field of pediatrics through exemplary leadership in related societies, as well as editorial oversight of cutting-edge research,” says David Wessel, M.D., executive vice president and chief medical officer of Hospital and Specialty Services at Children’s National. “This elevated role with the APS will enable her to further share her expertise to benefit children on a national and international level.”

Dr. Steinhorn serves as associate editor of the Journal of Pediatrics and is also a contributing editor for NEJM Journal Watch’s Pediatric and Adolescent Medicine.  Additionally, she sits on the editorial boards of Pediatric Critical Care Medicine and Pulmonary Circulation. Dr. Steinhorn is an elected fellow of the American Heart Association and a member of both the Perinatal Research Society and the American Thorasic Society.

Founded in 1888, the American Pediatric Society is the oldest and most prestigious academic pediatric organization in North America. Members are elected to APS based on their accomplishments as academic leaders in pediatrics and goal to shape the future of academic pediatrics. Mark L. Batshaw, M.D., physician-in-chief and chief academic officer of Children’s National preceded Dr. Steinhorn as APS President from 2016-2017.

“This is a tremendous honor, and it is a special privilege to follow Dr. Batshaw’s sound leadership. I look forward to leveraging the collective leadership and research accomplishments by our members to improve the health of infants and children throughout the U.S.,” said Dr. Steinhorn.

Dr. Steinhorn joined Children’s National in 2015 after a successful tenure as professor and chair of the department of pediatrics at the University of California, Davis (UCD) School of Medicine and as physician-in-chief, UCD Children’s Hospital. Previously, she was vice chair of the department of pediatrics and chief of the division of neonatology at Northwestern University and the Ann & Robert H. Lurie Children’s Hospital of Chicago.

Dr. Steinhorn’s clinical and academic interests have focused primarily on fetal and neonatal pulmonary vascular development. Her translational work has spanned from in vitro studies, to experimental models and clinical trials. In addition to her own translational research program, she has participated in numerous multicenter trials that have helped define the clinical treatment of pulmonary hypertension during the neonatal period. Her clinical research work also has addressed other topics, such as harmonization of electronic health records for clinical research and telemedicine support of neonatal care in small rural hospitals.

Additionally, Dr. Steinhorn is particularly passionate about mentoring faculty and supporting the growth and career development of young neonatologists and scientists, with several having developed their own research laboratories and assumed division leadership positions. She was selected as a “Top Doctor” by Northern Virginia  Magazine in 2018.