STRIVE-ing for Comprehensive Care for DSD in One Program
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To better care for children and youth with differences in sexual development (DSD)/intersex, variations in sex characteristics (VSC) and other conditions that may affect genitourinary (GU) development or reproduction, Children’s Hospital of Philadelphia (CHOP) created the STRIVE for Kids Program. STRIVE, which stands for the Supportive Team approach to ReproductIVE (STRIVE) variations, brings together experts from endocrinology, urology, genetics and psychology in one clinic.
“Children and families can see all these specialists in one day,” says pediatric endocrinologist Maria G. Vogiatzi, MD, who co-leads the STRIVE Program with Chief of the Division of Urology Thomas Kolon, MD. “But more important than the convenience for families, every member of our team has special expertise caring for patients with these rare conditions. That’s the biggest benefit of having a dedicated team. Because of our experience, we can manage the patients in the most effective way.”
The team reviews each case ahead of a child’s appointment, so team members have a good idea what patients may be facing before the clinic day. Then they gather near the end of the clinic day to collaborate on a treatment plan, so families leave knowing the next steps in their child’s care.
Depending on the patient’s specific diagnosis and needs, additional subspecialists knowledgeable in DSD, VSC and reproductive issues from oncology, cardiology, pathology, gynecology, radiology, neonatology and medical ethics are ready to consult.
For example, patients with intra-abdominal gonads who are at risk for germ cell cancers, such as gonadoblastoma or dysgerminoma, will also be seen by the team’s oncologists (Vandana Batra, MD, and Suzanne P. MacFarland, MD) and pathologist (Rebecca L. Linn, MD).
Growing body of knowledge
The STRIVE Program team holds monthly case conferences with the broader team to review cases and share what has been learned.
“It’s a wonderful opportunity to pick each other’s brains,” Vogiatzi says. “It serves as an educational forum because everyone is an expert is one part of care — like Tom is an expert in surgery. We all learn from each other, and that prepares us for future patients.”
These cases are also discussed further at a weekly Urology/Radiology/pre-op conference to expand the conversation more from a surgical evaluation point of view. “This quickly gives each patient a dozen more Urology 'second opinions'," Kolon says.
The STRIVE Program is among the largest programs in the country dedicated to serving children with DSD/VSC. “Because we see such a high volume of patients, we have experience with even the rarest, most challenging diagnoses,” Vogiatzi says.
Follow-up frequency
Each child’s treatment path is determined by their presentation and diagnosis. Typically, a baby with genitalia differences is seen frequently during the first year as the STRIVE team leads the family through genetic testing, biopsies, decision-making and potential surgery. Follow-up is generally less frequent until adolescence, when the team guides the patient and family through puberty, with the help of hormone replacement therapy, if needed.
“We are always reassessing and making sure the child doesn’t have any other needs, beyond reproductive issues,” Vogiatzi says. “Some of the genetic conditions in this population come with involvement of other organs. Some conditions are associated with kidney problems, for example. We want to stay on top of everything that could be involved in the patient’s overall health.”
The STRIVE Program team also works closely with patients’ local physicians to share testing results and treatment plans.
Psychosocial support
A key component of treatment is ongoing support from STRIVE Program psychologist Leela Morrow, PsyD. She works with families at all stages — from coming to terms with a challenging diagnosis to checking on the child’s psychosocial adjustment to helping families learn to recognize signs of gender development.
“When the child has genital atypia, the team discerns the gender assignment that we think will be the most successful in the long run, given the patient’s genetics and physical presentation, but we always want to make sure the family is adjusting and the patient’s gender is developing as expected,” Vogiatzi says.
Knowing how challenging decisions surrounding gender assignment can be for families, the STRIVE team includes a certified medical ethics consultant, Kimberly J. Mason, MSN, CNS, PCNS-BC, HEC-C, Associate Director of the Department of Medical Ethics.
Referrals
The STRIVE Program has received patient referrals from endocrinologists, urologists and geneticists from across the country and internationally. Some patients already have a diagnosis, but many families are still searching for definitive answers when they come to CHOP.
The STRIVE Program team is seeing an increasing number of families seeking prenatal counseling when NIPD results don’t align with sonogram imaging around gender.
On the other end of the spectrum, the STRIVE Program also cares for patients for whom DSD isn’t suspected until the adolescent fails to progress through puberty.
Care for additional related conditions at CHOP
CHOP also has dedicated programs within the Division of Endocrinology and Diabetes to provide exceptional care to children with related hormonal and reproductive challenges who may have more homogeneous conditions.