Question:
Male, 7 months old, UPD subtype
We’re currently living in Dubai & doctors have very minimal experience with PWS cases. My son started GH last September at a 0.15mg, increased the dosage to 0.2mg where IGF-1 levels was 75 & after the 3rd sleep study the endocrinologist said it was safe to increase it to 0.3mg. We tested after 3 weeks from the dose increase & his IGF-1 levels dropped to 45! Our current endocrinologist advised not to increase the GH dosage now as she has mentioned that ‘The recommended dose in PWS is 1 mg/m2, his body surface area is 0.33 m2, so increasing the dose to 0.4 mg would exceed that which she wouldn’t recommend given the risk of apnea in his condition.’ He’s not overweight & he’s on BIPAP room air during the night.
Is there something different we should be looking at or are we missing something.
Nurse Lynn’s Response:
Managing growth hormone (GH) therapy in a child with Prader-Willi Syndrome (PWS) requires careful attention to several factors, especially given the complexity of your situation. Unfortunately, there is really no “one size fits all” for GH dosing. The endocrinologists’ recommendations to stay within 1 mg/m²/day aligns with safety guidelines for PWS. Many endocrinologists also look at growth velocity, height curve compared to the rest of the family, body composition, IGF-1/IGFBP-3.
I would also suggest that you contact the International Prader Willi Syndrome Organization (IPWSO), who has an ‘ask a professional’ service where your question will go directly to a member of their Clinical and Scientific Board. They often have contacts in your area and can help advocate for your child. https://ipwso.org/how-we-can-help/ask-a-professional/
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Jennifer Bolander has been serving as a Special Education Specialist for PWSA (USA) since October of 2015. She is a graduate of John Carroll University and lives in Ohio with her husband Brad and daughters Kate (17), and Sophia (13) who was born with PWS.
Perry A. Zirkel has written more than 1,500 publications on various aspects of school law, with an emphasis on legal issues in special education. He writes a regular column for NAESP’s Principal magazine and NASP’s Communiqué newsletter, and he did so previously for Phi Delta Kappan and Teaching Exceptional Children.
Evan has worked with the Prader-Willi Syndrome Association (USA) since 2007 primarily as a Crisis Intervention and Family Support Counselor. Evans works with parents and schools to foster strong collaborative relationships and appropriate educational environments for students with PWS.
Dr. Amy McTighe is the PWS Program Manager and Inpatient Teacher at the Center for Prader-Willi Syndrome at the Children’s Institute of Pittsburgh. She graduated from Duquesne University receiving her Bachelor’s and Master’s degree in Education with a focus on elementary education, special education, and language arts.
Staci Zimmerman works for Prader-Willi Syndrome Association of Colorado as an Individualized Education Program (IEP) consultant. Staci collaborates with the PWS multi-disciplinary clinic at the Children’s Hospital in Denver supporting families and school districts around the United States with their child’s Individual Educational Plan.
Founded in 2001, SDLC is a non-profit legal services organization dedicated to protecting and advancing the legal rights of people with disabilities throughout the South. It partners with the Southern Poverty Law Center, Protection and Advocacy (P&A) programs, Legal Services Corporations (LSC) and disability organizations on major, systemic disability rights issues involving the Individuals with Disabilities Education Act (IDEA), Americans with Disabilities Act (ADA), and the federal Medicaid Act. Recently in November 2014, Jim retired.