Bridging the Gaps for Patients with Complex Conditions When Children Need Care After Hours CME: Intersex Conditions
Vol. 29.2 Summer 2017
on Pediatrics
An All-new Birth Experience
Focus on Pediatrics is published by Children’s Hospital of Greenville Health System. Medical Editor Joseph L. Maurer, MD Managing Editor Lark Reynolds GHS Photographer AV Services Art Director GHS Creative Services Editorial Board Nichole Bryant, MD Karen Cantu Kristi Coker, PhD, MSN, MHA, RN Jeanine Halva-Neubauer Jennifer Hudson, MD Emily Hughes Eric Nash Terri Negron, MN, RN Janine Sally, MS, CCC-SLP Robert Saul, MD Kerry Sease, MD, MPH If you would like your name added to or removed from our mailing list or have any comments, questions or suggestions, please send the appropriate information to: Marketing Services Greenville Health System 300 E. McBee Ave. Suite 200 Greenville, SC 29601 (864) 797-7544 The information contained in the Focus is for educational purposes only—it should not take the place of medical advice or diagnoses made by healthcare professionals. All facilities and grounds of Greenville Health System are tobacco free. “Greenville Health System” and GHS symbol design are trademarks of Greenville Health System.
© 2017 Greenville Health System 17-0645
FROM THE MEDICAL DIRECTOR
Health Sciences Center at GHS The last issue explained how Greenville Health System (GHS) is dividing itself organizationally into four regions, and I used this column to describe my extra duties outside of pediatrics as Chief Clinical Officer of the Western Region. What a difference a few months make! I have now dropped that regional role and acquired a new one: VP for Development in the Health Sciences Center.
a medical school and nursing school, along with clinical and translational research, in South Carolina’s largest healthcare delivery system. Every year, 5,000+ medical “learners” are on our campus earning credentials as healthcare providers. Children’s Hospital benefits greatly from HSC, as many of our learners return as colleagues already familiar with our mission, vision and values.
GHS’ Health Sciences Center (HSC) is a new LLC whose purpose is to embrace all of the programs of our academic health center. Its five primary areas of focus are to 1) manage and expand the GHS clinical learning environment; 2) sponsor graduate medical education; 3) oversee business and economic development and entrepreneurial opportunities; 4) develop and implement a new model of philanthropy; and 5) promote equity and inclusion.
In keeping with our practice of physicianadministrator dyad leadership, I am paired with HSC’s David Sudduth, VP and COO. Our attention will be directed to focus area No. 4.
The HSC is supported by a nine-member board of managers consisting of two representatives from GHS along with two representatives each from our major academic partner institutions (University of South Carolina, Clemson University and Furman University). It is chaired by GHS President Spence Taylor, MD. HSC is dedicated to helping health professionals of today and tomorrow meet the real-world needs of our community by providing leadership and a clinical environment for over 40 academic, professional and workforce development programs that include
As before, I will serve as chair of Pediatrics and medical director of Children’s Hospital. George Haddad, MD (Clinical Services); Des Kelly, MD (Academic and Community Services); and Robin LaCroix, MD (Quality and Medical Staff Services), will continue in their vice chair roles and provide excellent assistance in running all of our children’s programs.
William F. Schmidt III, MD, PhD
GHS’ Health Sciences Center provides leadership and a clinical environment for over 40 academic, professional and workforce development programs.
CONTENTS
Patewood Preparing to Receive Bundles of Joy 2 Patewood Memorial Hospital will begin offering obstetrics services for low-risk pregnancies in September.
When Illness Strikes After Business Hours
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Children’s Hospital’s two night clinics offer parents an alternative to urgent care and the emergency room when care is needed in evenings and on weekends.
Center Bridges the Gaps for Patients with Complex Needs 23 The Ferlauto Center for Complex Pediatric Care streamlines care for patients with challenging conditions.
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A Collaboration That’s for the Kids 26 The Institute for Child Success advocates for programs that benefit the state’s youngest residents.
Departments What’s New? 5 Small-baby Unit, Changes to Car Seat Rules, Mobility Tool for Toddlers
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Medical Staff Spotlight 9 Meet Our New Physicians
Academic News 11
Congratulations, Residents! Welcome, New Residents!
Quality Counts 13 Molecular Diagnostics
CME 14
Intersex Conditions
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Celebrations 18
Accreditation and Philanthropic News
Case Study 20 Undescended Testis
Clinical Research 29
Research at Bradshaw Institute for Community Child Health & Advocacy
Ask the Faculty 30 Eosinophilic Esophagitis
On the cover: Come autumn, low-risk deliveries will be offered at GHS’ Patewood Memorial Hospital.
26 13 To access this publication online, go to www.ghs.org/publications.
LEAD STORY
A Place for Extra-special Deliveries Low-risk deliveries will be offered at Patewood Memorial Hospital beginning this fall By Lark Reynolds
“We live off Pelham Road in Greenville, so it’s very close,” Payne said. “I’m excited about the fact that it’s less than 10 minutes away. And it’s not as big and busy as GMH.” That size and pace of activity are typical for a tertiary care center like GMH. But PMH is a community hospital, and as such, the pace is slower and ambience more relaxed. “We’re still going to be able to maintain the same standard of care that we deliver at GMH, but the pace by which we have to maintain things there isn’t going to carry over to Patewood,” explained Mary Beck, MD, lead physician at Piedmont OB/ GYN, one of the two GHS practices whose low-risk patients will deliver at PMH (Greenville Ob/Gyn Associates is the other).
Size Matters
Another perk for moms at PMH will be the size of their room. “We have fewer rooms because the patient volume won’t be as high as it is at GMH, so we were able to make the rooms bigger,” Dr. Beck pointed out. Rebecca Wright, MD, observes baby Anna Jane Ellis with mom Brittany Ellis. Dr. Wright will serve as medical director of Newborn Services at Patewood Memorial Hospital when obstetrics services begin in September.
For the last several decades, moms-to-be in Greenville who wanted to have their baby at a Greenville Health System (GHS) hospital have had one option—bustling Greenville Memorial Hospital (GMH), a regional referral center located near downtown. All that is about to change, though. Beginning this fall, women with low-risk pregnancies can deliver at Patewood Memorial Hospital (PMH), a smaller and less hectic facility located in the heart of Greenville’s Eastside community. The projected start date is September 25. Sarah Payne is due to have a girl—her first child—October 2, and is excited that she’ll be delivering her baby at PMH. For one thing, it’s much closer to home than GMH, which makes it more convenient not only for Payne and her husband, but also for many of the friends and family who plan to visit. 2
The rooms and bathrooms reflect more of a home-like environment as opposed to a clinical one. Large windows provide ample natural light, and the bathroom sinks have a glass tile backsplash. Each bathroom has a spacious shower with a bench, big sink and mirror. Each room also is set up to accommodate a tub should a woman desire water therapy during labor.
Delivering High-quality Care
While well-designed rooms and a peaceful setting are valuable on the one hand, the quality of care women receive at PMH will in no way be compromised, Dr. Beck emphasized. Despite not having a neonatal intensive care unit (NICU) at PMH, pediatric hospitalists with specialized training in neonatology and newborn care will be in-house around the clock.
“We do our best to predict when a mother is going to have risk associated with her delivery, but we don’t always know for sure,” stated Rebecca Wright, MD, medical director of Newborn Services at PMH. “The hospitalists we’ll have inhouse at Patewood will spend several intensive weeks at GMH learning newborn care specifically, spending time with the Bryan NICU delivery team, attending deliveries of infants at all risk levels, and that will be an ongoing process. They’ll continue to rotate through with the NICU delivery team during their time at Patewood to keep up that skill set.” In addition, PMH will participate in Delivery Buddy, GHS Children’s Hospital’s telehealth program that brings the expertise of neonatal nurse practitioners into the labor and delivery suite via secure video when needed. This program helps the nurse quickly determine whether the baby needs to be transported to GMH.
GHS’ departments of Pediatrics and OB/GYN have worked closely throughout the process of planning and bringing to fruition the new women’s services at PMH. “This project has been planned with OB/GYN and Pediatrics working hand in hand,” Dr. Beck shared. “We’ve worked closely together, because it is an outlying hospital.”
Left: An artist rendering shows what labor and delivery suites will look like at Patewood Memorial Hospital. Above right: Christa Moormeier, RN, a core nurse for Newborn Services at PMH, and Dr. Wright examine the artist renderings of The Family Birthplace–Patewood. Below: Floor plan of a labor and delivery suite at The Family Birthplace–Patewood. Right: Artist renderings show the bathroom of a labor and delivery suite at The Family Birthplace– Patewood (above) and the family waiting area on that floor (below).
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Dr. Wright hopes FastTrack will be available by 2019. In such cases, the baby would have to be brought back to PMH for an outpatient visit for some services such as newborn metabolic screenings and bilirubin checks. But the family could avoid spending an extra night in the hospital if they wished. “That’s something more and more families are showing an interest in,” Dr. Wright observed.
In the Long Run
Long-range plans for PMH include offering additional services on an outpatient basis that have previously required a trip to GMH, and sometimes even an admission to Children’s Hospital. For instance, babies with jaundice currently must be readmitted to Children’s Hospital to receive phototherapy, but Dr. Wright hopes they will be able to offer that service to parents at PMH without admitting the infant. Lactation consultations and lab draws are other services she envisions providing, along with outpatient circumcisions, for cases where it cannot be performed before discharge for some reason.
Jonathan Shoultz, MD, a doctor with Piedmont OB/GYN, answers a question for Sarah Payne during an office visit. Low-risk patients at Piedmont OB/GYN and Greenville Ob/Gyn Associates will be delivering at Patewood Memorial Hospital.
For example, the pediatric hospitalist will be in the room for every C-section delivery at PMH. At GMH, the pediatrician visits a new mom and baby within 24 hours of a C-section. Dr. Wright said the pediatric doctors also are going to concentrate on maintaining a calm environment for every mom, baby and family. “We’re going to focus on limiting interruptions and keeping it a peaceful space for moms to recover postpartum,” she noted. “We’re going to try to bundle interactions, to do as much as possible together, in single visits, rather than interrupting a number of times for various tests, immunizations and screenings.”
Birth of a Notion
While it won’t be available on Day 1, Dr. Wright explained that long-range plans include adding the opportunity—if medically appropriate—for a mom and baby to be discharged in as little as eight hours after the birth. “We understand that a lot of families want, during the birth process, the safety of being in a medical center, but then they may want to spend their recovery at home,” Dr. Wright added. “If they meet the criteria for the FastTrack discharge, then we would like to offer it.”
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Future plans more relevant to the mom (and the rest of the family) include concierge services to help individualize each woman’s birth experience. Dr. Beck said they intend to offer special packages such as food delivery service for a special meal for mom and dad, or a spa-type package where the mom is pampered with fresh flowers, magazines and a plush keepsake robe. When plans come to fruition, a concierge nurse will meet with patients during prenatal care to discuss how each woman would like to customize her stay. Decisions such as what drinks the mom would like to be stocked in her recovery room refrigerator would be made during these prenatal concierge nurse visits. Also incorporated into each woman’s prenatal care will be parts of the birth plan that relate to the baby’s care, as these decisions often are not considered much during the prenatal period. “A lot of times, the birth plan focuses on the time up until the baby is born, and then there’s nothing after that,” Dr. Wright recounted. “But there are a lot of decisions to make that moms don’t want to have to make in that hour they’re trying to recover from delivery.” It’s another example of the way Pediatrics and OB/GYN have partnered to make Patewood a special place to give birth. “The facility is lovely,” Dr. Wright summarized. “It’s a very peaceful campus, and I think the offerings present a good hybrid between the minimal intervention that families are moving toward more, but also the safety and evidence-based care of a hospital.”
WHAT’S NEW? Children’s Hospital of Greenville Health System (GHS) opens a unit dedicated to very low-birthweight babies, welcomes a new piece of equipment for toddlers with mobility issues and launches a new therapeutic program, among other advances.
New Law Changes Car Seat Rules A child passenger restraint bill, signed into law in May, makes several changes to the previous rules for child passengers. The biggest change is a requirement that infants under 2 years old who still meet the manufacturer’s seat height/weight limits ride in a rear-facing car seat. Although the American Academy of Pediatrics has recommended this practice for years, South Carolina is one of just five states to include it in child passenger safety laws.
New Unit Caters to Tiniest Patients
The SC Department of Safety has summarized all the changes contained in this new child passenger restraint bill. Go to http://www.buckleupsc.com/safety_seat_law.asp to access the summary.
GHS Children’s Hospital’s new Small-baby Unit clusters together those infants born at less than 28 weeks’ gestational age or less than 1,000 grams at birth in an environment that provides specialized care and minimal stimulation. The unit, housed within the Bryan Neonatal Intensive Care Unit (NICU), includes 17 beds and is designed to help these fragile patients develop. The unit features dim lighting and low noise levels. Each family has a small private area around their baby’s isolette. Developmental positioning aids and beds allow minimal handling and movement of these tiny patients. In addition, the Small-baby Unit makes it a priority to use bubble CPAP (continuous positive airway pressure) as an alternative to ventilators whenever possible. Care is provided by a team of specialized nurses who have experience working with very low-birthweight and extremely low-birthweight babies.
Lee Penny (far left), Injury Prevention manager for Children’s Hospital’s Bradshaw Institute for Community Child Health & Advocacy, represented GHS at the signing of the law.
Foodshare Program Launches in West Greenville Children’s Hospital’s Center for Pediatric Medicine–West has launched a foodshare program in the West Greenville community it serves after providers at the clinic observed that there were few opportunities within the community to obtain fresh fruits and vegetables. The program allows community members to purchase a box of fresh produce for $5 using an EBT card. All the produce is donated by local farmers. The program is facilitated through Mill Village Community Ministries, which also is located in West Greenville. 5
Go, Toddlers, Go!
Wonder Center Expanding Children receiving therapy at GHS’ Kidnetics® now have new ways to move, thanks to a donation of two Go-Baby-Go cars retrofitted by occupational therapists at Medical University of South Carolina.
The national Go-Baby-Go program modifies ride-on cars to make them usable for toddlers with mobility challenges. The Go-BabyGo car fills an important gap in mobility tools for children with disabilities. While a typical toddler spends about three hours a day in physical play, exploration and interaction with toys, children with limited mobility have far fewer opportunities to do these activities. There are no commercial ride-on cars tailored for children with these disabilities, and power wheelchairs are not an option until children become older.
The Wonder Center, GHS Children’s Hospital’s day treatment program for medically fragile children located at the Center for Developmental Services, is expanding. At the Wonder Center, children who cannot attend a traditional day care because of significant medical needs are cared for by pediatric nurses and a child development specialist. Demand for the center has historically exceeded its capacity, as it is the only such program for medically fragile children in the state. Construction is expected to be complete by late summer 2017. Much of the funding for the expansion came through philanthropic events held by J.L. Mann High School and Fluor.
The modifications give these children the chance for increased independence away from their caretakers at a younger age, allowing them to reach cognitive, social and motor developmental milestones at a faster pace. The cars are available for use by children with a variety of developmental issues.
Program Focuses on Pelvic Floor Disorders A new pelvic floor specialty program provides treatment for patients with pelvic floor dysfunction. Examples include daytime or nighttime wetting, increased or decreased frequency of voiding, pain with urination, constipation, fecal incontinence and/or fecal withholding. Treatment involves manual therapy, therapeutic exercise for pelvic floor muscle strengthening, relaxation training, biofeedback, neuromuscular re-education of pelvic floor muscles, core strengthening, and patient and family education. These interventions are provided by a physical therapist at Kidnetics (pediatric therapies) trained in pediatric pelvic floor dysfunction.
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Pediatric Psychology Services Offered GHS Children’s Hospital now offers a Division of Pediatric Psychology, led by medical director Anne Kinsman, PhD, to assist with patients’ psychological needs. The division consists of eight psychologists in addition to Dr. Kinsman. These psychologists are embedded in four areas: New Impact (a healthy lifestyle/weight management program), the divisions of Developmental-Behavioral Pediatrics and Pediatric Gastroenterology, and the Supportive Care Team (formerly Palliative Care). This division also includes two master’s-level outpatient therapists. To learn more about the services provided, please call Dr. Kinsman at (864) 454-5115.
FEATURE STORY Children’s Hospital of Greenville Health System (GHS) offers two after-hours pediatric care practices for times when care is needed outside of regular business hours.
When Illness Strikes After Business Hours By Lark Reynolds
Any parent is all-too-familiar with the after-hour illness scenario: A fever in a little one starts to spike right as most doctor offices are closing. While options in such cases once were limited to an emergency room (ER) or a facility like MD360® Convenient Care, now there’s an after-hours alternative with a focus on pediatrics. GHS Children’s Hospital has two such offerings—Children’s Hospital After-hours Care in Greenville and Children’s Hospital Spartanburg Night Clinic. George Haddad, MD, vice chair of Pediatric Clinical Services for Children’s Hospital, was instrumental in developing these resources. “As a pediatrician in Spartanburg for almost 15 years, I would encounter children who had been seen by providers who were not familiar with common pediatric illnesses and conditions,” Dr. Haddad said. “The kids were oftentimes misdiagnosed along with having unnecessary tests performed on them.” Spartanburg Night Clinic began seeing patients in 2013, and Children’s Hospital After-hours Care followed a year later. The two practices are staffed by board-certified pediatricians and nurse practitioners mainly from GHS pediatric offices, so the care provided is on par with what one could expect from a regular daytime visit. “We recognize that the ideal provision of medical care is going to a medical home, seeing the same doctor, the same nurse and the same administrative staff,” acknowledged Perry Earle, MD, medical director of Children’s Hospital After-hours Care and a pediatrician at Pediatric Associates–Simpsonville. “In the real world, sometimes that doesn’t work for the parents.”
Case in Point
Julie Walker of Spartanburg is one of those parents. Her son, Jacob, is 2 years old, but the Walkers also regularly host foster children—they’ve had five in the last two years, mostly infants and toddlers. And when children that young show worrisome symptoms, no matter what time of day, parents often are tempted to panic. “It’s a little bit frustrating and a little bit terrifying all at the same time,” Walker recounted.
Walker said all of the foster children they’ve hosted, as well as her own son, have made visits to Spartanburg Night Clinic. “I wouldn’t know what to do if I didn’t have the night clinic there,” she stated. “I’ve gotten a little bit spoiled being able to take them when I need to and not really having to wait until the doctor’s office opens the next day.” In Walker’s case, the issue often was simply an ear infection that was causing a fever to spike or other minor problem. But once, an 8-week-old foster child was referred from the clinic to the ER, where she received a diagnosis of respiratory syncytial virus (RSV). Walker recalled that the baby was admitted and spent several days in the hospital before her oxygen levels returned to a satisfactory level. According to Nicholas Kelley, MD, medical director of Spartanburg Night Clinic, it’s often the case that even if parents suspect their child may have a more urgent medical issue, they call the clinic first. “People tend to trust us and come to us first, even in cases where the child might eventually need to go to the ER,” observed Dr. Kelley. “It may be because it’s a more personal setting or they’ve seen our doctors before.” 7
Dr. Kelley said his staff sometimes had to send children to the ER for simple labs and chemistry panels, but now that an MD360 has opened in Spartanburg, he’s able to save those families a trip to the ER. “That’s going to be an amazing partnership going forward,” he emphasized.
Clinics Provide a Win-Win
Keeping kids out of the ER when their issue is not a true medical emergency is a win for everyone, Dr. Earle added.
Above: Julie Walker, right, and her spouse, Liz, have turned to Children’s Hospital Spartanburg Night Clinic on numerous occasions for care for their son Jacob (center) and the foster children they’ve hosted in their home. Left: The waiting areas at Children’s Hospital’s after-hours clinics are child friendly, making the visit less stressful and uncomfortable for both parent and child.
Timely, Cost-effective Care
Other factors that bring parents to the clinic before the ER or convenient care setting are cost and efficiency. “If we weren’t offering these services, these children would be going to other medical providers during that time,” Dr. Earle pointed out. “They would be seen in ERs and MD360s and urgent care centers. We recognize that all those areas are excellent providers of medical care, but often more expensive and perhaps less efficient.” At Children’s Hospital’s after-hours clinics, parents are encouraged to call during clinic hours to schedule an appointment. That way, they can do the bulk of their waiting in the comfort of home. “Patients can come in and out within a 30-minute window and be seen for their issue, and it costs less to do that in our facility than it would in an ER or urgent care type of setting,” Dr. Earle said. Dr. Haddad added that long wait times in an ER can actually have a detrimental effect on a child’s health: “When kids have to wait four to five hours in that type of setting before being seen, there is a chance they will pick up other illnesses.”
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“There are more than 20,000 visits to the Children’s Emergency Center (CEC) at Greenville Memorial Hospital each year, and a lot of those we can service and keep out,” he said. “If we can service them more efficiently and affordably, and reserve the CEC for cases that are truly medical emergencies, that is a good thing for everybody.” Another way the after-hours clinics help families is by providing an option for same-day appointments during busy times such as flu season when their regular pediatrician’s office can’t fit them in. And it’s the same child-friendly setting parents are used to at their child’s medical home. Also, the clinics are open to all families—including those whose children aren’t patients at a GHS practice. Not only does this convenience provide extra access to high-quality pediatric care, but it also helps families get connected to a regular doctor if they need one. “The clinics are an avenue for a child who doesn’t have a physician to get established with a pediatrician after being seen for an acute illness,” Dr. Haddad stated. “Overall, we felt like all children should have the option to be seen by qualified pediatric providers and staff in a reasonable time, at a reasonable cost, in a kid-friendly environment.” Children’s Hospital’s Center for Pediatric Medicine (CPM), which serves the Medicaid population, also offers evening appointments Mondays-Thursdays until 8 p.m and Saturday appointments 9 a.m.-1 p.m.
“Keeping kids out of the ER when their issue is not a true medical emergency is a win for everyone.” — Perry Earle, MD
MEDICAL STAFF SPOTLIGHT Children’s Hospital of Greenville Health System welcomes several new physicians to the GHS Medical Staff.
Meet Our New Physicians Developmental-Behavioral Pediatrics
Charles Hatcher III, MD, MPH, earned his medical degree and a master’s degree in public health from Medical University of South Carolina in Charleston, S.C. He completed his pediatric residency at Children’s Hospital of Georgia at Georgia Regents University in Augusta, Ga., and a fellowship in DevelopmentalBehavioral Pediatrics at GHS. Dr. Hatcher can be reached at (864) 454-5115.
General Pediatrics
Kym Do, MD, earned her medical degree from University of Alabama at Birmingham School of Medicine in Birmingham, Ala. She completed her Pediatrics residency at GHS. Dr. Do is working as a pediatrician with the Center for Pediatric Medicine. She can be reached at (864) 220-7270.
Cristina Lopez, MD, earned her medical degree from Florida International University Herbert Wertheim College of Medicine in Miami, Fla. She completed her Pediatrics residency at GHS. Dr. Lopez is working as a pediatrician with the Center for Pediatric Medicine. She can be reached at (864) 220-7270.
New Director of Philanthropy Donna Carver, CFRE, has joined Children’s Hospital as director of Philanthropy. She has spent the last four years as director of development for the College of Architecture, Arts and Humanities at Clemson University. In this role, Carver was responsible for major gift fundraising, development strategy and building relationships with alumni, industry partners, faculty, students and university administration. She can be reached at (864) 797-7746.
New Community Pediatrician Liz Chea, MD, has joined Clemson-Seneca Pediatrics. She can be reached at (864) 654-6034.
Dr. Schmidt Takes New Role at Health Sciences Center William F. Schmidt III, MD, PhD, medical director of Children’s Hospital and chair for the Department of Pediatrics, also now is serving as Vice President for Development for the GHS Health Sciences Center. As a result of this new role, he has transitioned out of the role of Chief Clinical Officer for GHS’ Western Region. In his new role, Dr. Schmidt will lead coordination of a comprehensive plan for philanthropy with the center’s four partners—GHS, Clemson University, Furman University and the University of South Carolina.
New DevelopmentalBehavioral Fellow
in Jacksonville, Fla.
Ryan Baker, MD, has joined Children’s Hospital as a fellow with the Division of Developmental-Behavioral Pediatrics. He earned his medical degree from University of South Florida Health Morsani College of Medicine in Tampa, Fla. Dr. Baker completed his residency training at University of Florida Health Jacksonville
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New Administrator for Children’s Hospital In March, Children’s Hospital welcomed a new administrator in Nikki Stafford, MS, MBA. Stafford has been with GHS since 2010 and had previously been serving as manager of the Neurosciences Clinical Programs and outpatient therapies for Roger C. Peace Rehabilitation Hospital. Her role now includes administrator duties over both Children’s Hospital and Neurosciences. Stafford has more than 17 years of healthcare experience and is board-certified in healthcare management as a Fellow of the American College of Healthcare Executives.
Resident and Faculty Awards The following doctors and caregivers were recognized at Pediatric Residency Program graduation ceremonies: • Liz Colvin, MD: Pediatric Resident Teaching Award (voted on by first- and second-year Pediatric residents) • Kym Do, MD: Pediatric Resident Teaching Award (voted on by medical students); Miracle Maker Award (given by Pediatric faculty for extraordinary care, community service and furtherance of health education) • Matthew Grisham, MD, and Michael Stewart, MD: John P. Matthews Jr., MD, Outstanding Faculty Teaching Award (given by residents to a general pediatrician and to a subspecialist for superb teaching and enthusiasm for resident education) • Kerry Sease, MD, MPH: Paul V. Catalana, MD, Exemplary Character Award (given by the graduating class to a caregiver who exhibits the qualities of honesty, fairness, compassion, altruism and leadership by example) • Pediatric Intensive Care Unit: 2017 Division of the Year Award (chosen by Pediatric residents) • Brent Speer, MD: Margaret L. Wyatt, MD, Outstanding Grand Rounds Award • Chris Graves, MD: Pediatric Resident Journal Club Award
Several pediatric faculty members attended convocation in May for students graduating from University of South Carolina School of Medicine Greenville. Pictured are (l-r) Josh Brownlee, MD; William Schmidt III, MD, PhD; April Buchanan, MD; Des Kelly, MD; and Robert Saul, MD.
• Jeremy Loberger, MD: Medicine-Pediatrics Resident Achievement Award (chosen by Pediatric faculty for teaching and research skills and commitment to education) • Craig Anderson, MD, and Meghan Jordan, MD: Inpatient Care Award (voted on by inpatient Pediatric faculty) • Meghan Jordan, MD: Jill D. Golden, MD, Primary Care Award (for outstanding care in the outpatient setting as voted on by ambulatory Pediatric faculty)
Mark Your Calendars! Mark your calendars for the 26th annual DeLoache Seminar, scheduled for Thursday, Nov. 9, at Embassy Suites Greenville, 670 Verdae Blvd. The featured speaker will be Stan Shulman, a professor in the Division of Pediatric Infectious Disease at Northwestern Medicine’s Lurie Children’s Hospital. Dr. Shulman will be speaking on the topic of Kawasaki disease.
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ACADEMIC NEWS Children’s Hospital of Greenville Health System (GHS) congratulates all of our residents who graduated in May 2017 and welcomes 11 new Pediatric residents, five MedicinePediatrics residents and a new Developmental-Behavioral Pediatrics fellow (see Page 9).
Congratulations, Graduates! Pediatric
Elizabeth Ann Colvin, DO, has joined Neighborhood Outreach Access to Health (NOAH) in Scottsdale, Ariz. Kym Do, MD, has joined GHS Children’s Hospital as co-chief pediatric resident and junior faculty member. Cristina Lopez, MD, has joined GHS Children’s Hospital as cochief pediatric resident and junior faculty member.
Dr. Colvin
Dr. Do
Dr. Lopez
Dr. Reis
Dr. Rutledge
Dr. Speer
Dr. Torni
Dr. Witrick
Dr. Yeh
Dr. Gammon
Dr. Hovland
Dr. Loberger
Dr. Wells
Dr. Wood
Adam Reis, MD, began work at Edgewood Pediatrics in Edgewood, Ky. Shanika Rutledge, MD, began work in private practice at Snellville Pediatrics in Snellville, Ga. Brent Speer, MD, began work in private practice at Cooper Pediatrics in Duluth, Ga. Kyle Torni, MD, has joined San Juan Health Partners Pediatrics in Farmington, N.M. Megan Witrick, MD, has joined AnMed Pediatric Associates in Anderson, S.C. Julie Yeh, MD, is working with Madigan Army Medical Center in Tacoma, Wash.
Medicine-Pediatrics
Nicole Gammon, MD, will begin work at the GHS-owned practice of Heritage Pediatrics & Internal Medicine–Simpsonville. Rebekah Hovland, MD, has joined Koke Mill Medical Associates in Springfield, Ill. Jeremy Loberger, MD, has begun a Pediatric Critical Care fellowship at the University of Alabama at Birmingham School of Medicine. Sarah Wells, MD, will join the GHS-owned practice of Heritage Pediatrics & Internal Medicine–Simpsonville. Bernadette Wood, MD, will join the GHS-owned practice of Heritage Pediatrics & Internal Medicine–Wren.
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Welcome, New Residents! Pediatrics
Caroline Brooks, MD: University of South Carolina School of Medicine Greenville, Greenville, S.C. Anna Brownstein, DO: Campbell University School of Osteopathic Medicine, Lillington, N.C.
Jacqueline Razzaghy, MD: Medical University of South Carolina, Charleston, S.C. David Roper, MD: Medical University of South Carolina, Charleston, S.C.
Angela Chiang, MD: Medical College of Georgia, Augusta, Ga.
Kelly Shymkiw, MD: University of South Carolina School of Medicine, Columbia, S.C.
William Dalkin, MD: Virginia Tech Carilion School of Medicine, Roanoke, Va.
Medicine-Pediatrics
Cassandra Graeff, MD: Geisinger Commonwealth School of Medicine, Scranton, Pa. Zachary Gray, MD: Loma Linda School of Medicine, Loma Linda, Calif. Michelle Matthews, MD: University of South Carolina School of Medicine Greenville, Greenville, S.C. Leon Przybylowski, DO: Edward Via College of Osteopathic Medicine, Blacksburg, Va.
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Justin Holladay, MD: University of South Carolina School of Medicine, Columbia, S.C. Quinn Hunt, MD: University of South Carolina School of Medicine Greenville, Greenville, S.C. Eric Polley, MD: University of South Carolina School of Medicine, Columbia, S.C. Jackie Queen, MD: University of North Carolina School of Medicine, Chapel Hill, N.C. Zachary Wood, MD: Brody School of Medicine, East Carolina University, Greenville, N.C.
Dr. Brooks
Dr. Brownstein
Dr. Chiang
Dr. Dalkin
Dr. Graeff
Dr. Matthews
Dr. Przybylowski
Dr. Razzaghy
Dr. Roper
Dr. Shymkiw
Dr. Holladay
Dr. Hunt
Dr. Polley
Dr. Queen
Dr. Wood
Dr. Gray
GHS Children’s Hospital Physician Directory For admission to Children’s Hospital: (864) 455-0000
Phone Fax Phone Fax 454-5115 241-9205 William F. Schmidt III, MD, PhD 455-8401 455-3884 Tara A. Cancellaro, MD 454-5115 241-9205 Gerald J. Ferlauto, MD Medical Director; Chairman, Department of Pediatrics 454-5115 241-9205 Charles R. Hatcher III, MD Adolescent Medicine Desmond P. Kelly, MD 454-5115 241-9205 Sarah B.G. Hinton, MD 220-7270 241-9211 454-5115 241-9205 Steven H. Ma, MD Allergy, Immunology and Asthma 454-5115 241-9205 John M. Pulcini, MD 675-5000 675-5005 Darla H. McCain, MD Nancy R. Powers, MD 454-5115 241-9205 Ambulatory Pediatrics/Center for Pediatric Medicine (Medicaid) 454-5115 241-9205 Victoria L. Sheppard-LaBrecque, MD J. Blakely Amati, MD 220-7270 241-9211 Emergency Medicine Jessica P. Boyd, MD 220-7270 241-9211 Elizabeth L. Foxworth, MD 455-6016 455-6199 Elizabeth W. Burton, MD 220-7270 241-9211 Jacqueline J. Granger, MD 455-6016 455-6199 Kym N. Do, MD 220-7270 241-9211 455-6016 455-6199 Alison M. Jones, MD Sarah R. Emerson (formerly Ryder), MD 220-7270 241-9211 455-6016 455-6199 Janelle E. Godlewski, MD 220-7270 241-9211 Patrick J. Maloney, MD Matthew B. Neal, MD 455-6016 455-6199 Lochrane Grant, MD 220-7270 241-9211 455-6016 455-6199 Matthew P. Grisham, MD 220-7270 241-9211 Kevin A. Polley, MD Jeremiah D. Smith, MD 455-6016 455-6199 Sarah B.G. Hinton, MD 220-7270 241-9211 455-6016 455-6199 John D. Wilson Jr., MD 220-7270 241-9211 Mark B. Krom, DO Endocrinology 220-7270 241-9211 Cristina M. Lopez, MD James A. Amrhein, MD 454-5100 241-9238 220-7270 241-9211 Dolores P. Mendelow, MD 454-5100 241-9238 Elaine A. Apperson, MD 220-7270 241-9211 Mary A.S. Putnam, MD 454-5100 241-9238 Melissa D. Garganta, MD 220-7270 241-9211 Robert A. Saul, MD 454-5100 241-9238 Bryce A. Nelson, MD, PhD 220-7270 241-9211 Kerry K. Sease, MD, MPH Ferlauto Center for Complex Pediatric Care 220-7270 241-9211 Cady F. Williams, MD 220-8907 241-9211 W. Kent Jones, MD Anesthesiology 220-8907 241-9211 Cady F. Williams, MD Carlos L. Bracale, MD 522-3700 522-3705 Gastroenterology Michael G. Danekas, MD 522-3700 522-3705 Liz D. Dancel, MD 454-5125 241-9201 522-3700 522-3705 Lauren H. Doar, MD Michael J. Dougherty, DO 454-5125 241-9201 522-3700 522-3705 Jake Freely, MD Jonathan E. Markowitz, MD, MSCE 454-5125 241-9201 522-3700 522-3705 John P. Kim, MD 454-5125 241-9201 Richard F. Knox, MD 522-3700 522-3705 Colston F. McEvoy, MD Laura H. Leduc, MD 522-3700 522-3705 Genetics 250-7944 250-9582 Steven W. Samoya, MD 522-3700 522-3705 David B. Everman, MD 250-7944 250-9582 Matthew R. Vana, MD 522-3700 522-3705 R. Curtis Rogers, MD Gynecology Randall D. Wilhoit III, MD 522-3700 522-3705 Dianna T. Gurich, MD 455-1600 455-2805 Bradshaw Institute for Community Child Health & Advocacy Melisa M. Holmes, MD 455-1600 455-2805 Kerry K. Sease, MD, MPH 454-1100 454-1114 455-1600 455-2805 Cardiology Benjie B. Mills, MD Hematology/Oncology (BI-LO Charities Children’s Cancer Center) Benjamin S. Horne III, MD 454-5120 241-9202 455-8898 241-9237 Jon F. Lucas, MD 454-5120 241-9202 Nichole L. Bryant, MD Rebecca P. Cook, MD 455-8898 241-9237 David G. Malpass, MD 454-5120 241-9202 Cristina E. Fernandes, MD 455-8898 241-9237 Manisha S. Patel, MD 454-5120 241-9202 Leslie E. Gilbert, MD, MSCI 455-8898 241-9237 454-5120 241-9202 R. Austin Raunikar, MD Aniket Saha, MD, MSCI, MS 455-8898 241-9237 Child Advocacy Medical Program William F. Schmidt III, MD, PhD 455-8898 241-9237 Mary-Fran R. Crosswell, MD 335-5288 241-9277 Infectious Disease Nancy A. Henderson, MD 335-5288 241-9277 454-5130 241-9202 Critical Care Joshua W. Brownlee, MD 454-5130 241-9202 Michael G. Avant, MD 455-7146 455-5380 Sue J. Jue, MD Robin N. LaCroix, MD 454-5130 241-9202 Eric L. Berning, MD 455-7146 455-5380 Inpatient Pediatrics Christina M. Goben, MD 455-7146 455-5380 Darryl R. Gwyn, MD 455-7146 455-5380 Greenville April O. Buchanan, MD 455-8401 455-3884 Robert S. Seigler, MD 455-7146 455-5380 Gretchen A. Coady, MD 455-4411 455-4480 Developmental-Behavioral Peds/Gardner Center for Developing Minds 455-8401 455-3884 Karen Eastburn, DO, MS Ryan A. Baker, MD 454-5115 241-9205 Jeffrey A. Gerac, MD 455-4411 455-4480 James H. Beard Jr., MD 454-5115 241-9205 Continued on back
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ghschildrens.org
17-0645 Revised 7/17
QUALITY COUNTS
Molecular Diagnostics have an organism attributed to the bacterial growth detected. The gram-positive panel includes rapid identification of Staph aureus, methicillin-resistant Staph aureus, Streptococcus, Enterococcus, vancomycin-resistant enterococcus, Strep pyogenes (group A), Streptococcus agalactiae (group B), Streptococcus pneumoniae and Listeria.
Molecular diagnostics, which detect specific sequences in DNA or RNA that may be associated with disease, hold promise to shorten length of hospital stay, avoid inpatient admission and limit antimicrobial use. Technology now allows for rapid polymerase chain diagnosis of many common conditions affecting children. Polymerase chain reaction (PCR) testing has been simplified and refined for use in hospital laboratories without a defined molecular diagnostic lab. This technology also can be used in the office setting by nursing staff. For example, a respiratory pathogen panel that screens for several common viral illnesses—such as parainfluenza, adenovirus, influenza, metapneumovirus, pertussis, Chlamydophila, respiratory syncytial virus, mycoplasma, rhinovirus and enterovirus—also identifies the viral cause. The result is reduced antimicrobial use. It also helps physicians limit additional testing. The test is performed from nasal secretions and has an approximate two-hour turnaround time. This rapid diagnostic facilitates discharge from the ER and averts admission for many children who might have been hospitalized in the past due to the uncertainty of the cause of their febrile respiratory illness. Also available is a rapid spinal fluid PCR analysis run directly off the spinal fluid with an approximate two-hour turnaround time. The meningitis panel notes the presence of Neisseria meningitidis, Haemophilus influenza, Streptococcus pneumoniae, Escherichia coli K1, Listeria, group B strep, cytomegalovirus, enterovirus, herpes simplex virus 1, herpes simplex virus 2, human herpesvirus 6, Human parechoviruses, varicella zoster virus and Cryptococcus. This information can guide decisions about inpatient admission, antimicrobial use or concerns around antibiotic pretreatment of abnormal spinal fluid.
The gram-negative panel includes Acinetobacter baumanii, Haemophilus influenza, Neisseria meningitidis, Pseudomonas aeruginosa, Enterobacter before meals, Enterobacter cloacae, Escherichia coli, Klebsiella oxytoca, Klebsiella pneumoniae, carbapenem-resistant Klebsiella pneumoniae, Proteus and Serratia marcescens. The technology also can rapidly identify Candida species such as albicans, glabrata, krusei, parapsilosis and tropicalis. Some PCR panels are directed toward GI pathogens: Clostridium difficile A and B, Campylobacter species Plesiomonas, Salmonella species, Vibrio species, Vibrio cholera Yersinia species, Enteroaggregrative Escherichia coli, enteropathogenic Escherichia coli, enterotoxigenic Escherichia coli, Shiga toxin, Escherichia coli 0157, Shigella, enteroinvasive Escherichia coli, cryptosporidium Cyclospora, Entamoeba histolytica, giardia, adenovirus, F 40/41, norovirus, rotavirus and Sapovirus. These tests allow for directed therapy and enhance the ability to diagnose infections to facilitate epidemiologic investigations around outbreaks. As molecular diagnostics become more easily implemented and cost-effective for an office setting, using nucleic acid-based testing to diagnose common illnesses such as group A strep will lead to enhanced sensitivity and eliminate the need for backup throat culture. These tests are CLIA-waived, which stands for Clinical Laboratory Improvement Amendments. Sensitivity and specificity are described as 96%. Current testing modalities and rapid turnaround time can impact several areas of medicine, including antimicrobial stewardship, length of stay, healthcare use and epidemiologic investigations. As technology continues to evolve, additional direct specimen testing from blood and other body fluids will further advance medical care. Article author Robin LaCroix, MD, is vice chair of Quality and Medical Staff Services for Children’s Hospital of Greenville Health System and a pediatric infectious disease physician at GHS.
Another rapid molecular diagnostic test is blood culture PCR identification. This technology allows a positive blood culture to 13
CONTINUING MEDICAL EDUCATION
CME: Disorders of Sex Development
CME Credit Information
To receive possible continuing medical education (CME) credit for this article, please complete the online Q&A that can be accessed on page 17. Both physicians and nurses are eligible to test for the credit. It is the policy of the GHS Continuing Medical Education Committee to ensure balance, independence, objectivity and scientific rigor in all its individually sponsored or jointly sponsored education activities. Article authors James A. Amrhein, MD, and Andrew P. Smith, MSN, CPNP, have disclosed that they have no significant financial interest or relationship with any company that may be considered an actual or potential conflict of interest with this educational activity. The planning committee have listed no duality of interest with regard to potential relevant financial relationships for the FOCUS enduring activity. The CME committee have listed no duality of interest with regard to potential relevant financial relationships for the FOCUS enduring activity with the exception of Sandra Weber, MD (Committee Chair), Grant/Research Support–Eli-Lilly, NIH, and Pfizer and William A. Coleman, MD (OB/GYN), Consultant–Merck. The Greenville Health System (GHS) designates this enduring activity for a maximum of 0.50 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity. The Greenville Health System is accredited by the South Carolina Medical Association to provide continuing medical education for physicians.
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Definition, Frequency and Terminology
“Disorder of sex development” (DSD) refers to a congenital condition in which development of chromosomal, gonadal or anatomical sex is atypical. Genital anomalies can vary from mild hypospadias or clitoromegaly to significant ambiguous genitalia clearly indicating an intersex disorder. In practical terms, a DSD is considered in an infant or child with apparent genital ambiguity/anomaly severe enough such that gender assignment and sex of rearing are not immediately clear. Frequency of genital anomalies varies greatly by degree of severity. However, only approximately 1:4,500 infants are born with a genital anomaly significant enough to lead to uncertainty about gender assignment, according to a study published in 2006. Multiple terms have been used to describe infants or children with these anomalies, including ambiguous genitalia, intersex, pseudohermaphrodite or hermaphrodite. The accepted terminology since the 2006 Consensus Statement is disorder of sex development.
Classification of DSD
Most DSDs can be categorized into one of the following groups: • Virilized 46,XX female • Undervirilized 46,XY male • Chromosomal or gonadal intersex Virilized 46,XX Female The cause of the majority of 46,XX females with virilized genitalia is congenital adrenal hyperplasia (CAH). Nearly all females with CAH exhibit ambiguous genitalia at birth. Degree of virilization can vary from mild clitoromegaly to marked labioscrotal fusion with enlarged clitoris and occasionally a penile urethra. Therefore, it is necessary to evaluate any apparent female infant with even mild virilization of the external genitalia. Undervirilized 46,XY Male Inadequate virilization in a male infant results from either insufficient testosterone production or androgen insensitivity. These individuals generally exhibit micropenis, hypospadias and undescended testes. Growth of the penis, formation of a penile urethra and descent of the testes all are part of the androgen-dependent sexual differentiation of the male fetus. Mild to moderate hypospadias without undescended testes is not usually considered a DSD; therefore, these individuals do not require extensive evaluation. However, infants with more severe degrees of hypospadias, especially when associated with unilateral or bilateral cryptorchidism, will need to undergo workup for DSD.
Figure 1. Normal sexual differentiation of fetus
Chromosomal or Gonadal Intersex These infants exhibit discordance between chromosomal sex and gonadal differentiation. This discord is a very complex group of disorders that includes X/XY mosaicism usually resulting in mixed gonadal dysgenesis (presence of a streak gonad + testis), ovotesticular DSD with both ovarian and testicular tissue present (previously termed true hermaphroditism), XX male syndrome, and XY female. These infants typically present with ambiguous genitalia at birth, and the diagnosis is made only after extensive workup that often includes gonadal biopsy. However, XX males and XY females may be phenotypically normal at birth and only discovered to have this abnormality later in life.
Infants Requiring Evaluation for DSD
Any time a healthcare provider cannot adequately determine the sex of an infant, it is necessary to consider a DSD as the possible cause of the genital abnormalities. The following circumstances all indicate significant likelihood of DSD, and, therefore, require recognition and appropriate evaluation. • Ambiguous genitalia • Apparent female with … - Clitoromegaly - Posterior labial fusion - Palpable inguinal/labial gonad (always represent testes or ovotestes) • Apparent male with … - Bilateral nonpalpable testes - Hypospadias plus bifid scrotum or undescended testis - Micropenis (<2 cm in stretched length at birth— see Figure 2)
Figure 2. Penis growth curve
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Evaluation of Infant/Child with DSD
Consultation with appropriate pediatric subspecialists is mandatory. Many hospitals, including GHS, have a DSD team that includes specialists from pediatric endocrinology, urology, genetics, psychology and gynecology. A pediatric ethicist also is on the team. Whoever is in charge of the diagnostic evaluation needs to quickly and efficiently perform the following studies: Laboratory Studies • Karyotype with FISH for X and Y (SRY) (results in 72 hours) • Electrolytes every two days for first week (if CAH #K+ initially, then $Na+ by end of the 1st week) • 17-Hydroxyprogesterone, testosterone, androstenedione, cortisol and plasma renin activity (may take 3-7 days) • Anti-Müllerian hormone (AMH) (indicates presence of testicular tissue) Imaging Studies • Pelvic ultrasound (often false-negative when uterus present) • MRI pelvis (better, but still not perfect) • Genitogram or retrograde urethrogram (identifies vagina and cervix—preferred but difficult test requiring significant expertise)
Discussion with Family
Presence of a DSD in a newborn infant is very stressful to all involved, but it is important to reassure the parents that they have a normal, healthy infant who exhibits a problem with genital organs that prevents you from determining whether the baby is a boy or a girl. Explain what you are seeing and demonstrate the abnormalities to the parents. Briefly explain how genital abnormalities can develop. Indicate that a rapid evaluation will be performed that will help determine the gender assignment and diagnosis for their child. Ask the parents to delay telling friends and family the sex of the baby. A single team member should be assigned to be the primary communicator with family members to keep them upto-date as the laboratory and imaging studies are completed. Commonly, a diagnosis is made within a few days, but it may take several weeks before a specific diagnosis is available. Once the laboratory and imaging studies are completed, they are presented in a very thoughtful and careful fashion, allowing the family to understand what has caused the DSD in their child. At the same time, a discussion of appropriate gender assignment ensues based on the information available. This information must be repeated on several occasions, accompanied by appropriate educational materials on DSD such as the excellent publication Disorders of Sex Development: A Guide for Parents and Physicians by Drs. Wisniewski, Chernausek and Kropp.
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Treatment Options Gender Assignment Fortunately, the majority of individuals with DSD are easily diagnosed, and gender assignment and treatment are fairly straightforward. However, occasional times ensue when the gender assignment is quite difficult and complex based on the chromosomal pattern, gonads and external genital appearance. In such situations, a gender assignment of either male or female must be considered based on the medical information as well as data on long-term follow-up of similarly affected individuals. Parents ultimately make the decision of sex assignment for their child in consultation with the DSD team based on the best current information available. Surgical Treatment Pros and Cons The surgical choices that parents might consider are based on the type of DSD that affects their child. In general, surgical treatment falls under two categories. Gonadectomy (removal of the testes, ovaries or streak gonad) usually is performed to prevent cancer by removing a cancer-prone gonad before the tumor develops. The second type of surgery is genitoplasty, in which female or male external genitalia are surgically reconstructed. Surgical treatment options must be presented in an open and honest fashion, especially when they involve irreversible procedures such as gonadectomy or removal of a phallus, uterus or vagina. Much controversy surrounds surgical management of DSD individuals. This controversy has arisen for a number of reasons. First, except in limited cases, there is no immediate medical need for or benefit from early surgery. Second, the surgery often is irreversible and may lead to physical complications, scarring, loss of sexual sensation and need for repeat surgeries. Third, many parents do not fully understand their options, and it has been suggested that surgery be delayed until the child is older and can provide his or her own informed decision regarding surgery. Finally, a number of individuals who underwent early surgical correction are unhappy with the results and would have preferred to wait and be involved in the decision at a later age. In spite of these objections, the majority of parents continue to make a decision for early surgery for their children. Thus, a number of issues need to be addressed with the family. There is less controversy about early surgical correction of moderate or severe hypospadias, including masculinizing genitoplasty, but delaying surgery also is an option that needs to be discussed in these cases. Other questions parents may have include things like, “What is the indication to perform gonadectomy in a child before puberty?” or “Should all children with complex DSD be referred to a Center of Excellence for second opinion, evaluation and management?”
To address all questions, it is important to provide adequate support, education, and counseling for patients and families, which includes putting them in touch with appropriate support groups. The DSD Consensus Statements published 10 years apart in 2006 and 2016 greatly assist us—and our families—in addressing a number of these complex issues. A brief summary of some of their conclusions follows: • Medical and surgical decisions by family members should only be made after an evaluation has been made by a multidisciplinary team and adequate time has been given to review appropriate resources and communicate with relevant support groups. • DSD surgery continues to raise unresolved questions and dilemmas regarding indications, timing (in infancy vs. at adolescence) and choice of procedure. Inadequate long-term evidence exists regarding the impact of early surgery vs. delaying surgery as far as medical, sexual or psychological outcomes. • Healthy, functioning gonadal tissue should remain in place until puberty unless significant risk of malignancy is evident. Patients should actively participate in decision-making at the “appropriate age.” • Long-term follow-up data are limited, and our ability to predict outcomes in terms of gender identity, sexual function, fertility and general quality of life are uncertain. • Gender dysphoria and patient-initiated gender change vary from 0-66% across DSD syndromes and greatly depend on the specific diagnosis. Later change in gender is associated with significant emotional, social and financial burdens.
Long-term Outcome of DSD Individuals
Although the initial experience is extremely stressful for family members, it is important to remember that most people affected by DSD grow up to lead happy and healthy lives. Many of these individuals will require one or more surgical
Article Authors James A. Amrhein, MD, is a pediatric endocrinologist at Children’s Hospital of Greenville Health System. He has a longstanding interest in disorders of sexual development following his fellowship at Johns Hopkins Hospital where his primary clinical and research interest focused on patients with these disorders. Andrew P. Smith, MSN, CPNP, is a pediatric nurse practitioner with Children’s Hospital’s Division of Pediatric Endocrinology. His focus and primary interests include type 1 diabetes, problems of growth and development, and disorders of sexual development.
procedures along with lifelong medical care. Sexual satisfaction, gender dysphoria and infertility remain ongoing issues for some. Providing these patients and families with options for psychological counseling and peer support groups may significantly improve quality of life.
Summary
A child with DSD poses particular challenges to healthcare professionals as well as parents, because these disorders are medically and surgically complex. Every person with DSD has aspects of his or her condition that require difficult decisions mandating a team approach to diagnosis, treatment, counseling and follow-up. Care for DSD individuals continues to evolve, although the timing and extent of surgical intervention remain controversial.
References Consulted
• https://patient.info/doctor/ambiguous-genitalia • Hughes, IA; Houk, C; Ahmed, SF; et al. Consensus Statement on Management of Intersex Disorders. Arch Dis Child 91(7): 554-63, July 2006. Epub April 19, 2006. • Lee, P, et al. Consensus Statement on Management of Intersex Disorders. Pediatrics 118: E488-E500, April 2006. • Clinical Guidelines for the Management of Disorders of Sex Development in Childhood. Consortium on the Management of Disorders of Sex Development. Intersex Society of North America/Accord Alliance, 2006. • Lee, P, et al. Global Disorders of Sex Development Update since 2006: Perceptions, Approach and Care. Horm Res Paediatr 85: 158-180, Jan 2016. • Meyer-Bahlburg, Heino, et al. Gender Assignment, Reassignment and Outcome in Disorders of Sex Development: Update of the 2005 Consensus Conference. Horm Res Paediatr 85: 112-118, Jan 2016. • Meyer-Bahlburg, Heino. Gender Monitoring and Gender Reassignment of Children and Adolescents with a Somatic Disorder of Sex Development. Child Adolesc Psychiatric Clin N Amer 20: 639-649, 2011. • Karkazis, K, et al. Genital Surgery for DSD: Implementing a Shared Decision-Making Approach, J Pediatr Endocrinol Metab 8: 789-805, 2010. • Wisniewski, Amy; Chernausek, Steven; and Kropp, Bradley. Disorders of Sex Development: A Guide for Parents and Physicians. Johns Hopkins University Press, 2012.
CME Questions Available Online As a convenience for our audience and to conserve resources, Focus on Pediatrics has transitioned to an online format for the Q&A portion of CME articles. Here is a link and a QR code you can use to access the CME questions online. http://www.ghs.org/PediatricsElectronic 17
CELEBRATIONS
Children’s Hospital of Greenville Health System (GHS) has many reasons to celebrate! Lifetime Achievement Award George Maynard III, recently retired VP of Institutional Advancement at GHS, received the Brian Donnelly Lifetime Achievement Award from Safe Kids™ Upstate in December. Maynard was responsible for generating philanthropic revenue that facilitated the launch of Children’s Hospital’s Bradshaw Institute for Community Child Health & Advocacy, which includes Safe Kids, in fall 2016. The gift from the Bradshaw family to establish the institute was the largest private donor gift ever to a Safe Kids coalition. Maynard retired from GHS in March 2017.
Clement’s Kindness Honors Nurse with Rocking Chair Clement’s Kindness Fund paid tribute to a longtime nurse at the BI-LO Charities Children’s Cancer Center by donating a rocking chair to the center. Ruth Cook spent 50 years as a nurse with GHS, and was known for rocking babies and praying over them at the children’s cancer center. She retired in 2008, but returned to the center for the dedication of the rocking chair. Cook said she still receives letters and phone calls from those she cared for while a nurse. She now serves on the board of advisors for Clement’s Kindness.
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Bryce Nelson, MD, PhD, medical director of the Children’s Hospital’s Division of Pediatric Endocrinology, accepts the Community Champion Award at the JDRF Gala in the TD Convention Center.
Pediatric Endocrinology Honored The Greater Western Carolinas chapter of the Juvenile Diabetes Research Foundation (JDRF) honored Children’s Hospital’s Division of Pediatric Endocrinology as the 2017 Community Champions at the group’s Upstate Gala in Greenville on March 25. The award recognizes the division’s hard work and dedication to the local type 1 diabetes (T1D) community. JDRF is the leading global organization funding T1D research. Its mission is to accelerate life-changing breakthroughs to cure, prevent and treat T1D and its complications. In addition, the division’s research team was honored by the Pediatric Diabetes Consortium with the Outstanding Performance award in recognition of the team’s recruitment and participation in pediatric type 2 diabetes trials. The Pediatric Diabetes Consortium is a multicenter group of leading pediatric diabetes treatment centers in the United States whose long-term objective is to improve the care of children with diabetes through sharing of best practices, collecting outcomes data in a common data repository and advocating for changes in pediatric diabetes care.
CELEBRATIONS
Children’s Hospital Physician, Residents Earn Research Awards Jennifer Hudson, MD, medical director of Newborn Services for GHS Children’s Hospital, earned a first-place award in the poster competition at the GHS Health Science Center’s Research Showcase in March. The poster, of which Dr. Hudson was a co-author, was titled, “Forecasting Longterm Medicaid Savings with Early Treatment for Neonatal Abstinence Syndrome.” Pediatrics residents Meghan Jordan, MD, and Andreea Stoichita, MD, earned a first-place award in the showcase’s Resident Cases in High Value Care Competition for their project, “Early-onset Sepsis Calculator.” Dr. Hudson served as faculty mentor. Dr. Hudson also earned the systemwide GHS Health Sciences Center 2017 Outstanding Faculty Research Award for her manuscript, “Early Treatment for Neonatal Abstinence Syndrome: A Palliative Approach.” The manuscript, which she co-authored with Pediatrics resident Kindal Dankovitch, MD, was published by the American Journal of Perinatology in December 2016. Dr. Dankovitch was the recipient of the GHS Health Sciences Center 2017 Outstanding Resident Research Award for her work on the same manuscript.
Dr. Hudson
Dr. Jordan
Dr. Saul Pens Children’s Book Robert Saul, MD, senior medical director of Pediatric Medicaid Services for GHS Children’s Hospital, wrote a children’s book called All about Children to serve as a companion book to his earlier work, My Children’s Children: Raising Young Citizens in the Age of Columbine. All about Children uses vivid illustrations to highlight activities parents and children can do to improve the lives of others in their communities. The paperback debuted in July and is produced by Archway Publishing.
Dental Practice Donates Pajamas A local dental practice and its patients donated more than 100 pairs of pajamas to GHS Children’s Hospital to help alleviate the discomfort of an emergency trip to the hospital for a child. Children’s Hospital often is in need of pajamas, as during the summer months children may end up in the hospital after an accident at camp or the pool and need a fresh set of clothes. After learning of this need, the staff at ProGrin Dental, which has three locations in the Upstate, requested pajama donations through its Facebook page and radio spots. Patients donated pajamas when coming in for dental appointments, and others in the community visited ProGrin offices just to drop off pajama donations. “We appreciate this gift and the many people who supported ProGrin Dental to make it possible,” said Emily Durham, supervisor of Child Life Services for Children’s Hospital. “These pajamas will help many young patients and take at least one item off of a family’s to-do list.”
Dr. Stoichita
Dr. Dankovitch 19
CASE STUDY
Undescended Testis MJ is now a 4-year-old boy who was born at 36 weeks estimated gestational age. He did well and did not require NICU care. His birth exam was unremarkable with the exception of an undescended left testis. The testis was palpable in the inguinal canal, and there did not appear to be an associated hernia. Phallus was normal and the right testis was easily palpable in the scrotum. He underwent neonatal circumcision uneventfully and was discharged home on day-of-life 2. He did well and his parents faithfully brought him to his well-child checks where his pediatrician dutifully noted the undescended testis. When MJ was 1 year old and well above the 90th percentile for weight, the pediatrician could not palpate the left testis, so he ordered a scrotal/inguinal ultrasound for evaluation. It confirmed the inguinal location of the left testis as well as the normal right testis. Both the pediatrician and MJâ&#x20AC;&#x2122;s parents were reassured with the findings. Observation continued until MJ was 3 years old. During this time, MJâ&#x20AC;&#x2122;s pediatrician saw him regularly for both well-child checks and sick visits, but it is unclear if genital exams were performed as none were recorded in the medical record. 20
Around the time MJ turned 3, the family changed pediatricians because of insurance concerns. The new pediatrician was unable to locate the left testis, so a second ultrasound was ordered. It again confirmed the inguinal location of the left testis with a normal right testis, and MJ was referred for a pediatric urology consultation. On exam by the pediatric urologist, the left testis was palpable in the inguinal canal; outpatient orchidopexy was recommended. At the time of surgery, the testis was identified at the level of the external ring and was noted to be somewhat small. A communicating hydrocele also was found. Orchidopexy was completed along with hydrocelectomy, and MJ did well postop. On exam six months later, the left testis was located in the scrotum, but still was noted to be smaller than the right. Undescended testis (UDT) is the most common genital anomaly in boys with an incidence of nearly 3% in full-term infants. At least two-thirds of these testes will descend spontaneously in the first few months of life and require nothing more than observation. As in MJâ&#x20AC;&#x2122;s case, the diagnosis
is generally straightforward, but the management of UDT (especially in cases of palpable UDT) has evolved and undergone significant changes over the years. As researchers have developed a better understanding of testicular growth and function, recommendations for treatment have changed accordingly.
A Look Back
While it has long been known that a testis located outside the scrotum would exhibit diminished spermatogenesis and increased malignancy potential, the necessity and timing of intervention have been a subject of debate for the past century. In the early 1900s, standard medical practice was to leave an asymptomatic intra-abdominal testis undisturbed. Malignant transformation of the intra-abdominal testis was well described, but prophylactic surgery to reposition or remove the gonad carried an unacceptably high risk of mortality. The inguinal testis could feasibly be addressed surgically in a manner similar to hernia repair, but the prudent surgeon would never operate on a young child for such a benign condition, given the risks associated with infection and anesthesia. The best course of action was considered to be observation until at least puberty to give the testis as much time as possible for natural descent. As surgical and anesthesia techniques advanced, earlier intervention became more reasonable; but as late as the 1970s, the prevailing opinion still was to wait until the child was at least 6 years old before intervening. While it was recognized that spontaneous descent was unlikely beyond 1 year of age, surgeons generally thought the delicate nature of the spermatic cord structures involved in dissection precluded earlier intervention. Over the past several decades, the recommended age for intervention has gradually decreased to the point where it now is recognized that testes should be in their normal scrotal position by the age of 12-18 months to maximize potential for future normal function. Old habits die hard, though, and if you ask a group of contemporary primary care providers when a boy with UDT should be referred for treatment, you likely will receive a wide range of answers. Any time before puberty, 5 or 6 years old, 2 years old and 6 months old all have been recommended at one time or another. This wide range of opinion is reflected in referral patterns. A recent survey of pediatric urology referrals at Greenville Health System for UDT showed the average patient age at time of referral to be just over 3.5 years old, which is well above the current recommendations.
Current Recommendations
What are the current recommendations? Recognizing that wide variation exists in practice patterns, the American Urological Association (AUA) addressed the issue of UDT management and in 2012 undertook the development and publication of
Guideline Statements Diagnosis
1. Providers should obtain gestational history at initial evaluation of boys with suspected cryptorchidism. (Standard; Evidence Strength: Grade B) 2. Primary care providers should palpate testes for quality and position at each recommended well-child visit. (Standard; Evidence Strength: Grade B) 3. Providers should refer infants with a history of cryptorchidism (detected at birth) who do not have spontaneous testicular descent by 6 months (corrected for gestational age) to an appropriate surgical specialist for timely evaluation. (Standard; Evidence Strength: Grade B) 4. Providers should refer boys with the possibility of newly diagnosed (acquired) cryptorchidism after 6 months (corrected for gestational age) to an appropriate surgical specialist. (Standard; Evidence Strength: Grade B) 5. Providers must immediately consult an appropriate specialist for all phenotypic male newborns with bilateral, nonpalpable testes for evaluation of a possible disorder of sex development (DSD). (Standard; Evidence Strength: Grade A) 6. Providers should not perform ultrasound (US) or other imaging modalities in the evaluation of boys with cryptorchidism before referral as these studies rarely assist in decision making. (Standard; Evidence Strength: Grade B) 7. Providers should assess the possibility of a disorder of sex development (DSD) when there is increasing severity of hypospadias with cryptorchidism. (Recommendation; Evidence Strength: Grade C) 8. In boys with bilateral, nonpalpable testes who do not have congenital adrenal hyperplasia (CAH), providers should measure MĂźllerian Inhibiting Substance (MIS or Anti-MĂźllerian Hormone [AMH] level) and consider additional hormone testing, to evaluate for anorchia. (Option; Evidence Strength: Grade C) 9. In boys with retractile testes, providers should monitor the position of the testes at least annually to monitor for secondary ascent. (Standard; Evidence Strength: Grade B) Continued on next page.
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Treatment 10. Providers should not use hormonal therapy to induce testicular descent as evidence shows low response rates and lack of evidence for long-term efficacy. (Standard; Evidence Strength: Grade B) 11. In the absence of spontaneous testicular descent by 6 months (corrected for gestational age), specialists should perform surgery within the next year. (Standard; Evidence Strength: Grade B) 12. In prepubertal boys with palpable, cryptorchid testes, surgical specialists should perform scrotal or inguinal orchidopexy. (Standard; Evidence Strength: Grade B) 13. In prepubertal boys with nonpalpable testes, surgical specialists should perform examination under anesthesia to reassess for palpability of testes. If nonpalpable, surgical exploration and, if indicated, abdominal orchidopexy should be performed. (Standard; Evidence Strength: Grade B) 14. At the time of exploration for a nonpalpable testis in boys, surgical specialists should identify the status of the testicular vessels to help determine the next course of action. (Clinical Principle) 15. In boys with a normal contralateral testis, surgical specialists may perform an orchiectomy (removal of the undescended testis) if a boy has a normal contralateral testis and either very short testicular vessels and vas deferens, dysmorphic or very hypoplastic testis, or postpubertal age. (Clinical Principle) 16. Providers should counsel boys with a history of cryptorchidism and/or monorchidism and their parents regarding potential long-term risks and provide education on infertility and cancer risk. (Clinical Principle) © 2014 by the American Urological Association
In the coming months, the Division of Pediatric Urology will be putting forth new educational efforts in an attempt to lower the age at time of referral for boys with UDT. 22
a practice guideline for managing undescended testis. The AUA convened a panel of experts in the fields of pediatric urology, pediatric endocrinology and general pediatrics to examine current practice and develop guidelines for the optimal management of a child with UDT. After an extensive review of the best available evidence, the AUA committee published a comprehensive review in 2014, which contained 16 specific statements along with a management algorithm. The unabridged version of the report is available at auanet.org/ guidelines/cryptorchidism-(2013-amended-2015). The panel’s statements are given either as standards, recommendations or options based on available evidence. The strength of evidence was rated as A (high), B (moderate) or C (low). In absence of sufficient evidence, statements are presented as Clinical Principles and Expert Opinions. While all statements are useful in the overall management of UDT, statements 1-9 are most applicable to a general pediatrician’s practice. With these recommendations in mind, let’s look again at MJ’s management to see if there is any room for improvement. His condition was identified appropriately at birth, and he was noted to be 4 weeks premature (#1). Since it was an isolated unilateral UDT that was palpable, no further evaluation was necessary for a disorder of sex development (DSD) (#5). He was seen appropriately for his well-child checks; the testis was palpated at each exam (#2). So far so good, but then his management begins to miss the mark. His testis was still undescended at 1 year of age, but ultrasound was ordered rather than surgical referral (#3, #6). In the absence of concern for DSD, ultrasound is not indicated for the evaluation of UDT as it is expensive and adds little to decision-making. Surgical referral should be made after an adjusted age of 6 months if the testis remains undescended. Surgical intervention should follow within the next year for the testis to be located in the scrotum by 18 months of age (#11). Clearly, MJ’s management was suboptimal and illustrates the common scenario of prolonged observation beyond 6 months of age and unnecessary ultrasound imaging. In the coming months, the Division of Pediatric Urology will be putting forth new educational efforts in an attempt to lower the age at time of referral for boys with UDT. We welcome suggestions as how we can work together to better care for these patients. Article author J. Lynn Teague, MD, is a pediatric urologist and medical director of the Division of Pediatric Urology at Children’s Hospital of Greenville Health System.
SPECIAL PROGRAM Children’s Hospital of Greenville Health System’s Ferlauto Center for Complex Pediatric Care works to make medical care simpler for families of children with complex medical conditions.
Ferlauto Center Connects the Dots for Families By Becky Wilhoit
For families in the Upstate with a child suffering from chronic and complex illness, care is a round-the-clock job. Now, these families have new options for comprehensive care at Children’s Hospital of Greenville Health System (GHS). The Ferlauto Center for Complex Pediatric Care at GHS operates under a collaborative model that brings together physicians, staff and parents to provide a life-changing resource for families caring for a child with medical complexity (CMC). The center’s model of care is deeply personal for Thelma Aiken of Easley. After her son, Blake, was born in 2009, he began exhibiting troubling symptoms. He became a patient of Kent Jones, MD, who at the time was practicing in Easley with the Medically Fragile Children’s Program. After Dr. Jones made a diagnosis of Rubenstein-Taybi Syndrome, he began working Cady Williams, MD, and Kent Jones, MD, spearhead the coordinated care provided to families of children with complex with the family to establish a conditions at the Ferlauto Center. care plan for Blake and help recalls one specific instance in which Dr. Jones walked alongside them navigate the world of complex care. For Aiken, having a resource dedicated to understanding her child’s individual needs them to help care for Blake. was a vital part of Blake’s treatment. “When Blake was just 6 months old, he got sick and was in real trouble,” she recounted. “He was not getting enough oxygen, “They’ve been with us through so much,” Aiken said of their turning blue and running a very high fever. We took him to journey with Dr. Jones and some of his longtime staff. She
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the ER at Baptist Easley and Dr. Jones came directly to us, communicated from there with the team at Greenville Memorial Hospital and then followed through the entire process as Blake was transferred there. We were so thankful to have Dr. Jones with us.” According to Dr. Jones, one of the center’s core tenets is engaging multiple parties in a child’s care. The parents— because they are closest to their child’s situation—along with care managers, home health nurses, physicians and a Ferlauto Center dietitian all participate in each visit as part of the center’s team-based care approach.
A Team Approach
“This practice is team-oriented,” emphasized Dr. Jones. “These parents know their child the best, and working together is how we believe we’ll make the best decisions for these children. In a typical visit, it’s not simply me and the parents, but all the other people who are part of that child’s care plan, such as a dietitian or a home health nurse. We all get in those appointments; we’re all together so we can circle up and discuss what’s going on with that child’s care. We believe that approach fosters more effective and complete care.”
Amy Kirk, RN, checks Blake Aiken’s blood pressure during an office visit at the Ferlauto Center.
“The personal care we’ve received, not just for our son, but our family, is astounding. Dr. Jones and his staff have always treated me like I’m just another part of their team who has gathered around Blake through every challenge. I feel like we’re one big family working toward the same goal.” — Thelma Aiken
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Kathy Crytser, RD, LD, has worked with Dr. Jones for 12 years, going back to when he was practicing with the Medically Fragile Children’s Program. “Every one of us has our eyes on a specific area of that child’s care, but together we’re able to cover the whole child,” she stated. “There’s no other way to care for these children but as a team.” Another core to the Ferlauto Center’s approach is continuity of care—having the same doctor see the child every visit so that parents don’t have to re-tell their story each visit. “Parents of medically complex kids frequently complain about seeing a different doctor every time in many other primary care settings, and we offer much more continuity,” Dr. Jones noted. Like Blake, many patients at the Ferlauto Center began seeing Dr. Jones and his staff before the center was launched in 2014. “Being part of GHS has afforded us so much support in creating this team and taking this concept of care forward,” Crytser observed. “To me, the center is really the ultimate beautiful morphing of our mission and what we do to take care of these kids, because this facility is what we were looking toward years ago.”
‘Seams Between Blocks’
Named for Jerry Ferlauto, MD, the first neonatologist in the Upstate, the Ferlauto Center’s future was secured after a $1 million endowment from Dr. Ferlauto and his wife, Natalina, in 2015. Dr. Ferlauto and Dr. Jones share a special connection—Dr. Jones was Dr. Ferlauto’s senior student in 1980. Now in his 34th year of pediatric practice, Dr. Jones has spent the past 15 years focused on CMC patients and is looking forward with a growing team of doctors, staff and specialists.
big difference. Similar programs have seen decreased hospital admission rates for their patients. We are making efforts to improve transitions in and out of Children’s Hospital for those patients who do get admitted.”
Cady Williams, MD, one of the newest additions at the Ferlauto Center, joined Dr. Jones and his team in 2016. For Dr. Williams, the center has a number of advantages for patients and their families, but also for specialty pediatricians dedicated to CMC patient care. She and Dr. Jones are quick to point out that the center functions as one layer within the intricate network of specialists that CMC patients may see for all the parts of their care, with the ultimate goal of facilitating continuity for both patients and the specialists who see them.
Making their care family-centered is a priority for the doctors at the Ferlauto Center. That commitment includes offering the option to have patients’ siblings seen in the office.
“We do not replace other services our patients use,” remarked Dr. Williams. “We are a small part of the resources our patients need. But what we are finding is that small things can make a
Dr. Jones agreed: “Specialists are the building blocks of this type of care, and so what we do is try to be the seams between those blocks.”
“That type of thing saves parents a lot of trouble and running around for multiple appointments, when they can come in here, bring both their CMC and their other children, and handle immunizations or a cold that both kids have,” stated Dr. Jones. “As an example, we see sets of twins where one is a CMC and one is not, because it just makes sense.” It also makes sense to the families who trust Dr. Jones and the staff at the Ferlauto Center.
When Chris Aiken (far right) brings his son Blake to the Ferlauto Center for an office visit, they see not only Dr. Jones (left) but also case manager Renee Moore, BSN, RN (second from left), and dietitian Kathy Crytser, RD (second from right).
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COLLABORATING FOR BETTER CARE The Institute for Child Success represents a collaboration in which Children’s Hospital of Greenville Health System (GHS) plays a key role.
Jamie Moon leads the Institute for Child Success, a collaboration between Children’s Hospital and other upstate entities that takes a systems approach to improving the health of children.
A Vision for Early Childhood Success By Anne Smith
“Our vision is the success of all young children,” said Jamie Moon, director of the Institute for Child Success (ICS). It sounds simple enough, but this lofty goal begs one question—how? In short, ICS aims to help our youngest patients succeed by changing the environments, systems and policies that shape their early years. The nonpartisan think tank proposes smart public policies, grounded in research; advises elected officials, nonprofits and foundations on strategies to improve early childhood outcomes; and shares the knowledge and solutions it develops along the way. Whether the institute is advocating for a bill on the legislative floor or promoting family reading time on a nursery floor, the goal is the same: giving our children the healthiest, most successful outcomes. 26
Filling a Void
Nearly a decade ago, a group of concerned upstate leaders noticed a gap in focused efforts addressing children’s issues with a systems approach, according to Linda Brees, director of GHS Children’s Hospital’s Bradshaw Institute for Community Child Health & Advocacy. In 2010, these community members—including Brees; Desmond Kelly, MD, medical director of the Division of Developmental-Behavioral Pediatrics; and William F. Schmidt III, MD, PhD, chair of the Department of Pediatrics—envisioned an entity to fill that void by examining and influencing pediatric health from a new angle. The solution they formulated became ICS, and the partnership between the Institute for Child Success and Children’s Hospital began.
“Children’s Hospital is a prominent partner in ICS efforts to improve early childhood outcomes,” Brees said. “The Institute for Child Success is its own nonprofit, but we helped found it and help fuel it with board membership, funding and expertise.”
Natural Fit with Children’s Hospital
As a research and policy organization focused on the success of children, ICS has a mission that dovetails seamlessly with that of Children’s Hospital. Historically, Children’s Hospital interventions occur as symptoms are presented by individual patients. Anticipating, avoiding and preventing the onset of childhood illness, disease or injury, however, is a relatively new paradigm that requires collaboration beyond the conventional approach. To substantially improve a community’s health, Brees noted, children’s hospitals must extend their focus to include the social determinants that impact the overall wellness of young people, whether that be education, family and social support, community safety or the law.
ICS Board Chair Linda Brees, left, and Jamie Moon, right, present S.C. Representative Raye Felder with the ICS Champions for Children award in 2015. Standing next to Brees is Susan Shi, PhD, board chair emerita.
“The Institute for Child Success espouses a systems approach, incorporating a lot of public policy work and examining the systems that are addressing children’s issues. They do not provide direct service, but their work affects all children,” Brees said. Pediatric medicine impacts communities one patient at a time, but policy can impact everyone at once. “Good policy empowers pediatric care providers,” Brees pointed out. “It elevates everything we can do in our offices.”
‘Think-do Tank’
“Outside of Washington, D.C., I’m not sure the phrase ‘think tank’ holds a lot of weight,” Moon said. “I view ICS as a ‘thinkdo tank.’ We are pragmatic. While ‘think tank’ can imply a passive organization, we think the big thoughts and then act on them. We talk to the people who can make these policies happen—lawmakers, government officials, foundations, corporations and the business community.” The institute’s think-do tactic has paid off. One current initiative may seem to have little connection to quality of care or good medicine, but it affects child health outcomes, nonetheless. Studies show, Moon said, that families who receive an earned income tax credit (EITC) have improved health and education outcomes. When a separate bill promoting such a tax credit wasn’t moving smoothly through the state legislature, ICS supported the addition of an EITC to South Carolina’s recent gas tax bill. The credit, benefiting families with lower incomes, will help offset the increased cost of transportation the new gas tax represents. Research indicates the results, while not technically medical in nature, will benefit the state’s youngest citizens and their families.
ICS Board member Darnell McPherson welcomes guests at the 2015 ICS Research Symposium.
“The Institute for Child Success blends medical, academic, safety and health issues; we examine anything that impacts young children.” — Jamie Moon, director Institute for Child Success
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He continued, “In a lot of early childhood work, there is a divide between health and education outcomes for children, but they’re interrelated. The health, development and education of a child are all integrated. That is why we rely on the Bradshaw Institute and Children’s Hospital to help us set a policy agenda each year.” ICS and GHS have worked together since 2013 on the Carolinas Research Symposium, a gathering that focuses on child health, development and wellness. The symposium allows academics to present to other academicians as well as policymakers and frontline providers. “It’s rare to have that mix in one room, hearing the same presentation,” Dr. Kelly said. “Collaborating on this symposium allows us to speak the same language for a moment and align our priorities.”
Early Childhood Years Are Key
The driving force behind all this collaboration couldn’t be more clear: the unified desire to have the healthiest children in the nation. ICS CEO Jamie Moon and U.S. Senator Tim Scott co-moderated a forum hosted by Together SC.
“When we started ICS, it was thought it would be local, but we quickly found there was a void at the national level,” Moon recalled. “ICS has a national footprint now; we’re not just a South Carolina or Greenville organization, but one that has become nationally recognized for our work. That helps us get more public funding, develop a road map for similar work across the country and establish national credibility. If we can do it here, they can do it anywhere.” “Policy” can have an abstract connotation, but there’s more to what ICS does than just thinking. The institute’s approach has a demonstrated impact on child and community health, and its partnership with GHS ensures that approach will continue to be the case.
A Blend of Anything That Impacts Children
The Institute for Child Success has several wins under its proverbial belt: last year’s statewide expansion of the public 4K program, this year’s EITC, and an ongoing partnership with leading researchers and subject matter experts who fuel ICS’ work. “The Institute for Child Success blends medical, academic, safety and health issues; we examine anything that impacts young children,” Moon said. “Children’s Hospital has provided us with a critical health expertise component of our work.”
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“Children’s Hospital contributes to ICS’ research and knowledge base around critical issues, but what’s most exciting about it is the institute’s work to unite the health and education worlds,” Dr. Kelly stated. “So often we operate in silos, but ICS is a key bridge.” He added, “For the last four years, the early childhood research symposium has drawn national leaders, but we’ve also seen South Carolina researchers sharing their work around policy from across different fields of health and education. Learning from each other, in turn, helps us understand advocacy and policy priorities. Where do we need to be working with the legislature to boost funding, expand proven services?” Dr. Kelly noted that it was through the efforts of ICS that the Nurse-Family Partnership, a home-visiting and support program for first-time mothers, has expanded. The institute worked with the state in doing a feasibility study, then promoted the funding once it was shown to improve outcomes. “ICS greatly expanded the capacity of that partnership, and our patients will benefit,” Dr. Kelly said. Moon agreed: “There’s a growing recognition that early childhood is key. If you want to avoid costly mitigation down the road, you need to provide high-quality early childhood experiences. They lead to healthier children, better outcomes and a stronger society. It’s the foundation of the rest of a person’s life, those first five years. That’s why we’re all here.”
CLINICAL RESEARCH UPDATE Research studies at Children’s Hospital of Greenville Health System (GHS) are approved by the system’s Institutional Review Board.
Research at Bradshaw Institute The mission of Bradshaw Institute for Community Child Health & Advocacy, part of GHS Children’s Hospital, is to promote child and family wellness and advance child health through education and research. This year, staff at Bradshaw Institute have commenced several exciting research projects with the dual goals of measuring the impact of the organization’s programming on community health outcomes and improving on the way health services are delivered to children at GHS.
Choosy Farm to Belly Program
Bradshaw Institute runs Choosy Farm to Belly, a fresh-produce program offered at SHARE Head Start centers. The 30-week program aims to influence food choices and help preschoolers and their families make healthy food selections, increase the frequency of home-cooked meals in low-income households, and foster movement and learning in the classroom. A research partnership with Clemson University is evaluating the effectiveness of the program and measuring improvements in healthy eating behaviors in this community over time. Results are expected to facilitate an expansion of the program to other schools.
Referral Patterns of GHS Physicians to Help Me Grow South Carolina
Bradshaw Institute is home to Help Me Grow SC, an affiliate of the national Help Me Grow network. The Help Me Grow system promotes developmental-behavioral health, early identification and linkage to community services for children (prenatal through age 5). Bradshaw Institute, with the help of two University of South Carolina School of Medicine Greenville medical students, is in the early stages of a research project analyzing referral patterns to Help Me Grow SC by GHS pediatric primary care practices. Analysis of physician referral patterns to Help Me Grow SC will provide general knowledge about the factors resulting in referral and improve awareness of systemic gaps in use that could enhance early identification and referral of children.
Equity of ED Use for Childhood Injuries
Bradshaw Institute is home to Safe Kids™ Upstate, an injury prevention coalition that provides safety education for schools and communities on topics including pedestrian safety, child passenger safety in vehicles, and bicycle safety. Safe Kids Upstate’s program areas informed this study, which looks at GHS emergency department (ED) visits for falls, bicycle accidents, motor vehicle collisions and violent incidents among upstate children between 2012-15. The study investigates whether the same socioeconomic characteristics that contribute to higher rates of childhood injury at the individual level also contribute to higher injury rates at the community level. Bradshaw Institute aims to use this study to inform effective injury prevention programming. 29
A S K T H E FAC U LT Y
Eosinophilic Esophagitis (EoE) Q: I suspect a patient has eosinophilic esophagitis (EoE). What are recommendations for testing and managing this condition? A: EoE has only been well-described for the last 15-20 years, yet it is a disease that is increasingly identified in both pediatric and adult patients. With this increased identification, awareness of the condition is growing among primary care providers (PCPs). However, it may be difficult for PCPs to know when to be concerned about this condition, what steps to take to expedite evaluation and when to refer to a specialist. EoE may present with different signs and symptoms. Presenting complaints tend to vary based on age. Infants and toddlers may present only with nonspecific complaints such as feeding refusal, irritability, reflux or vomiting. School-age children may complain of more typical reflux symptoms such as regurgitation and heartburn. Adolescents and adults tend to present with reflux symptoms, dysphagia and, in extreme cases, food impaction.
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In the pediatric population, about 10% of patients who present with refractory reflux symptoms ultimately are diagnosed with EoE. PCPs evaluating a patient with reflux symptoms would treat the reflux with acid suppression. If symptoms do not respond well to an appropriate weight-based dose of acid suppression, the index of suspicion for EoE should be increased. At that point, referral to a gastroenterologist is appropriate. While waiting for a patient to be seen by the specialist, using high-dose acid suppression with a proton pump inhibitor (PPI) is appropriate. “High dose” means twice the standard adult dose in adolescents and adults. In younger patients, “high dose” means about twice the usual weight-based treatment dose. The rationale for using high-dose PPI therapy is twofold. First, it may result in improved symptoms among those who are refractory to standard dosing. More important, increasing the PPI dose is an important step in making a correct diagnosis of EoE.
Pediatric Specialty Services
With this increased identification, awareness of the condition is growing among primary care providers (PCPs). We now know EoE comes in two “flavors”: a PPI-responsive form that looks and acts like EoE but resolves on high-dose PPI therapy and the more “traditional” form triggered by allergens in the diet. To differentiate between the two, a trial of highdose PPI is needed (before diagnostic endoscopy, if possible). Because the standard is to treat for approximately eight weeks with high-dose PPIs before endoscopy, a patient who already has started on this therapy frequently can move forward with the definitive diagnostic test soon after visiting the specialist. While some general concerns exist about long-term use of PPIs and potential side effects, the benefit of a several-month course of high-dose PPI significantly outweighs any theoretical risks and may lead to earlier diagnosis, ultimately resulting in less PPI exposure over time. EoE is the most common cause of dysphagia and food impaction in adolescents; therefore, the index of suspicion for EoE should be very high. While it is worth starting high-dose PPI therapy in these patients, additional testing such as barium esophogram and/or expedited endoscopy usually is indicated. A patient unable to spontaneously clear a food impaction should be referred to the emergency department for urgent evaluation. Increased awareness of EoE among PCPs is important. These professionals play a vital role in caring for those with EoE, which includes not just the identification and referral of potential patients, but also starting therapy that may provide early relief of symptoms and expedite the ultimate diagnosis through endoscopy.
Article author Jonathan Markowitz, MD, MSCE, is medical director of GHS Children’s Hospital’s Division of Pediatric Gastroenterology.
William F. Schmidt III, MD, PhD________________________________ (864) 455-8401 Medical Director; Chairman, Department of Pediatrics Matthew P. Grisham, MD___________________________________________ 455-7895 Pediatric Residency Program Director Russ C. Kolarik, MD________________________________________________ 455-7844 Medicine-Pediatrics Residency Program Director Desmond P. Kelly, MD______________________________________________ 454-5115 Developmental-Behavioral Fellowship Program Director Adolescent Pediatrics_______________________________________________ 220-7270 Allergy and Immunology____________________________________________ 675-5000 Ambulatory Pediatrics ______________________________________________ 220-7270 Cardiology ________________________________________________________ 454-5120 Child Advocacy Medical Program____________________________________ 335-5288 Critical Care_______________________________________________________ 455-7146 Developmental-Behavioral Pediatrics__________________________________ 454-5115 Emergency Pediatrics_______________________________________________ 455-6016 Endocrinology_____________________________________________________ 454-5100 Gastroenterology___________________________________________________ 454-5125 Genetics__________________________________________________________ 250-7944 Hematology/Oncology_____________________________________________ 455-8898 Infectious Disease__________________________________________________ 454-5130 Minor Care (Spartanburg Night Clinic)________________________________ 804-6998 Children’s Hospital After-Hours Care_______________________________ 271-3681 Neonatology______________________________________________________ 455-7939 Nephrology & Hypertension_________________________________________ 454-5105 Neurology_________________________________________________________ 454-5110 Neurosurgery______________________________________________________ 797-7440 Newborn Services__________________________________________________ 455-8401 Ophthalmology____________________________________________________ 454-5540 Orthopaedic Oncology______________________________________________ 797-7060 Orthopaedic Surgery________________________________________________ 797-7060 Plastic Surgery_____________________________________________________ 454-4570 Pulmonology______________________________________________________ 454-5530 Radiology_________________________________________________________ 455-7107 Rheumatology_____________________________________________________ 454-5004 Sleep Medicine____________________________________________________ 454-5660 Supportive Care Team______________________________________________ 455-5129 Surgery___________________________________________________________ 797-7400 Urgent Care (Anderson)____________________________________________ 512-6544 Urology___________________________________________________________ 454-5135
Children’s Hospital Programs BI-LO Charities Children’s Cancer Center______________________________ 455-8898 Bradshaw Institute for Community Child Health & Advocacy_____________ 454-1100 Bryan Neonatal Intensive Care Unit___________________________________ 455-7939 Child Life_________________________________________________________ 455-7846 Cystic Fibrosis Clinic________________________________________________ 454-5530 Family Connection_________________________________________________ 331-1340 Ferlauto Center for Complex Pediatric Care____________________________ 220-8907 Gardner Center for Developing Minds_________________________________ 454-5115 Girls on the Run___________________________________________________ 455-4001 Infant Apnea Program______________________________________________ 455-3913 International Adoptee Clinic_________________________________________ 454-5130 Kidnetics® (pediatric therapies)______________________________________ 331-1350 Neonatal Developmental Follow-up Services___________________________ 331-1333 New Impact (weight management)_____________________________ 675-FITT (3488) Office of Philanthropy & Partnership/CMN____________________________ 797-7735 Pediatric HIV Clinic_________________________________________________ 454-5130 Safe Kids™ Upstate________________________________________________ 454-1100 Spiritual Care______________________________________________________ 455-7942 Wonder Center____________________________________________________ 331-1380 Day treatment for medically fragile children
For admission to Children’s Hospital: (864) 455-0000
This number connects you to GHS’ Patient Referral and Transfer Center, which can handle all arrangements for admission. You also may call 455-7000 and ask the operator to page the admitting resident. Neonatal Transport: (864) 455-7165 To reach a Children’s Hospital doctor or program, call 1-800-4RBUDDY. Pediatric Outpatient Service Locations Call the appropriate Greenville number above for an appointment.
Anderson
Cardiology Endocrinology Hematology/Oncology Nephrology & Hypertension Neurosurgery
Greenwood Cardiology Surgery
Spartanburg
(864) 573-8732 Cardiology Developmental-Behavioral Endocrinology Gastroenterology Hematology/Oncology Kidnetics
Nephrology & Hypertension Neurology Neurosurgery Pulmonology Sleep Medicine Urology
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GHS Vision Transform health care for the benefit of the people and communities we serve. GHS Mission Heal compassionately. Teach Innovatively. Improve constantly. GHS Values Together we serve with integrity, respect, trust and openness.
For information about Children’s Hospital giving opportunities, call GHS’ Office of Philanthropy & Partnership at (864) 797-7732 or visit ghsgiving.org.
GHS and Palmetto Health Form Partnership In June, GHS and Palmetto Health announced a plan to create a new, not-for-profit SC health company. Together, the organization will have the scale, scope and resources required to address the serious health issues—including obesity, diabetes, stroke and other diseases—that plague South Carolinians. Leadership of the new company will rest with Mike Riordan and Palmetto Health CEO Chuck Beaman. This will bring some changes, but many things will remain the same. • Most important, this partnership does not change the clinician-patient relationship today. In fact, it is intended to enhance this special relationship over time. • We are not combining our medical staffs or clinically integrated networks. Palmetto Health and GHS will retain local control of their provider groups and will continue to be responsible for their own credentialing, privileging and oversight of clinical quality. And we will continue to enhance the collaborative activities between the Midlands and Upstate affiliates that improve clinical quality, the patient experience and healthcare value. Read more on the new health company at www.SCBetterTogether.org.
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