Question:
Female, 21, Deletion
My daughter with PWS (deletion) is wondering how common it is for females to be told by an endocrinologist to take estrogen and progesterone for the rest of their lives. She has already developed a blood clot (now resolved) and was told by another doctor that hormonal therapy could be a contributing factor. She is also concerned that it could lead to cancer if she takes it for life. Finally, is it common for people with PWS to also have Turners syndrome? Our daughter has swollen varices in her colon that occasionally bleed–a condition that can be common in Turners syndrome. Please advise!
Nurse Lynn’s Response:
Thank you for your questions. I have asked one of our experts for their input.
Most physicians will say estrogen and progesterone is for life for women with PWS. However, this should be discussed with your physician as he/she would know more about family history, risk factors etc.
The physician is correct about estrogen and progesterone being pro-clotting medications. I would strongly advise to do a risk/benefit analysis seeing that she has already had a blood clot before continued/prolonged use.
No significant risk of cancers from doing this with current formulations of hormones, per OB/Gyn .
It would be highly unusual to have Turner and PWS. There are many individuals with PWS and other genetic conditions. I would ask the geneticist to review the findings.
Varices are also common in PWS due to chronic constipation and straining. I would continue to monitor her BM’s daily and adjust medications as needed.
Do you have a question for Nurse Lynn? Submit your question here:
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.