Question:
Male, 34 years old, UPD subtype
Our son has never taken GH. One day, his endocrinologist noticed he had high hemoglobin levels, the highest being 18. Because of this, doctor referred him to a hematology specialist. Doctors checked his lungs, kidneys, and liver, and everything was fine. Seeing that everything was okay, they are now checking if he has any bone marrow disease. Doctors are planning to perform a lumbar puncture and take a biopsy, but there was also the option of drawing blood and using a reagent to test for bone marrow abnormalities. We are wondering if high hemoglobin is common in PWS (unfortunately, doctors here are not familiar with this condition) and what recommendations you have?
Nurse Lynn’s Response:
High hemoglobin is not a typical feature of PWS, but disordered sleep is. These sleep-related breathing problems can directly influence hemoglobin levels. Many individuals with PWS experience obstructive or central sleep apnea, shallow breathing during sleep, or low oxygen levels at night. When the body senses low oxygen, it increases the production of red blood cells in order to carry more oxygen. This natural response can cause hemoglobin to rise. Has your son had a recent sleep study or overnight oximetry?
There are also other possible causes of high hemoglobin in people with PWS. These include dehydration (common during illness, hot weather, or limited fluid intake), certain medications, or underlying heart or lung conditions. High hemoglobin can make the heart work harder and may increase the risk of symptoms such as headaches, dizziness, or blood clots.
Resources:
I have attached two articles that may be helpful for both you and your medical team:
https://pubmed.ncbi.nlm.nih.gov/35172921/
https://www.ncbi.nlm.nih.gov/books/NBK1330/
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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.