Question:
Female, 2 years old, Deletion subtype
Our 2-year-old daughter is having her tonsils and adenoids removed. This will be her 5th surgery. What are some things we need to mention to our medical team to help make sure her care is up to par with our PWS concerns?
Nurse Lynn’s Response:
Because your daughter has PWS, it’s very important that every person on the surgery team (ENT surgeon, anesthesia doctor, nurses, and recovery staff) clearly knows she has PWS before the tonsil and adenoid surgery. Kids with PWS can have breathing challenges that are not always obvious during a regular exam, including obstructive sleep apnea and central sleep apnea, low breathing drive, and low muscle tone that can make it harder to breathe well after anesthesia.
One of the biggest things to tell the anesthesia team is that many people with PWS can be more sensitive to sedating medicines, especially opioids and some medicines that relax muscles. This can lead to more sleepiness, slower wake-up, and breathing suppression after surgery.
Two other PWS “must-mentions” are temperature regulation and pain signals. People with PWS can have unusual low or high temperatures, including around anesthesia, and serious illness can happen without a typical fever. Also, many individuals with PWS have a high pain threshold, so they may not show pain the usual way. That means the team should not wait for big complaints before checking hydration, breathing, bleeding, infection, or other concerns, your parent instincts and any subtle behavior changes should be taken seriously.
Finally, remind the team about stomach and digestion risks in PWS. Vomiting is often uncommon in PWS, and stomach bloating/distention can be dangerous and needs quick attention. PWSA also notes that GI motility can be slow, and feeding after anesthesia should be restarted carefully with good observation. This is especially helpful after tonsil/adenoid surgery, when kids may drink or eat less.
Resources:
Anesthesia-and-PWS-Loker-2022-1.pdf
Anesthesia-complications-study-2022-1.pdf
MedicalAlertsBooklet-GIChart-2022.pdf
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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.
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.
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.
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.
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.