Emily L Paprocki, DO
Assistant Professor of Pediatrics, University of Missouri-Kansas City School of Medicine
Full Biography
Emily Paprocki, DO, Endocrinology, received a one-year, $22,140 award from the Foundation for Prader-Willi Research for the retrospective study, “Exploring Relationships between Growth Hormone Therapy and Sleep Disturbances in Infants and Young Children with Prader-Willi Syndrome: Multi-Site Polysomnogram Findings and Clinician Survey Insights.”
Prader-Willi syndrome (PWS) is a genetic disorder that can cause intellectual disabilities, behavioral problems, sleep abnormalities, and obesity due to extreme hunger. In infancy, signs and symptoms include low muscle tone, feeding difficulties, poor growth, and delayed development. Children with PWS are typically treated with growth hormone (GH) therapy soon after diagnosis. GH therapy can help improve growth, strength, and development.
GH therapy in children with PWS has known risks, including respiratory complications. Obstructive sleep apnea (OSA) is thought to be one of those risks. Thus, current guidelines recommend a baseline polysomnogram (sleep study) before starting GH therapy. This recommendation is being challenged for several reasons. First, OSA is reportedly rare in infants and toddlers with PWS under 24 months of age. Polysomnograms are also time-intensive, resource-heavy, and costly procedures. Additionally, delays in testing can lead to delays in GH initiation, which then may lead to delays in achieving infant milestones.
The study’s primary aims are:
- Retrospectively assess whether baseline polysomnograms in the first 24 months of life prior to initiation of GH therapy resulted in any intervention other than supplemental oxygen therapy. Additionally, assess how often worsening of sleep apnea was noted on follow-up polysomnograms in children up to 36 months of age within the year following growth hormone initiation.
- Internationally survey clinician practices related to sleep assessments and growth hormone therapy in infants with PWS.
“This multi-site study will provide data on whether we need baseline polysomnograms before beginning GH therapy in young children with PWS, which may ultimately impact time to treatment with GH. We also hope to use this data, along with the expert survey results, to inform new consensus guidelines,” said Dr. Paprocki.
Co-investigators include Kelsee Halpin, MD, MPH, Endocrinology, and David Ingram, MD, MHPE, Sleep Center. In addition to Dr. Paprocki’s award, researchers at Cook Children’s Medical Center, Seattle Children’s Hospital, and University of Utah Health also received grants funds for this project. The grant total was $53,455.