Question:
Female, 40 years old, unknown subtype
My sister has digestive episodes (severe constipation and now vomiting which she didn’t do as child) which usually result in vasovagal episodes and her complaining of side and chest pain. Nothing hospitals can do but how should my parents better manage her diet?
Nurse Lynn’s Response:
Digestive issues, including severe constipation, are common in individuals with Prader-Willi Syndrome (PWS) due to hypotonia, slower gut motility, and challenges with satiety regulation. These symptoms can sometimes trigger vasovagal episodes and associated pain, as you’ve described. Vomiting can occur but this is less common. Vomiting in a person with PWS should always be taken seriously. If it is accompanied by severe pain, bloating, or fever, seek immediate medical care to rule out complications like bowel obstruction or gastric rupture.
- Increase Fiber Intake: Focus on higher-fiber foods, such as vegetables, fruits (in controlled portions due to sugar content), whole grains, and legumes. A sudden increase in fiber intake can lead to bloating and discomfort, so a gradual introduction is recommended. Opt for soft- well cooked vegetables and fruits like mashed bananas, berries and steamed broccoli.
- Adequate Hydration: Ensure she drinks sufficient water throughout the day, as dehydration exacerbates constipation. This is crucial for individuals with PWS, as their natural thirst mechanism might be impaired.
- Monitor for Gastroparesis: Vomiting in PWS could indicate gastroparesis (delayed stomach emptying). A low-fat, low-fiber, and small, frequent meals approach might help if this is a contributing factor. Consult a gastroenterologist for a definitive diagnosis.
- Regular Bowel Regimen: Incorporate natural laxatives (e.g., prune juice) or mild over-the-counter options (e.g., polyethylene glycol) as advised by a healthcare provider. Establishing a consistent toileting schedule can also help.
- Monitor Meal Portions: Even with dietary modifications, portion control is critical for PWS management. Meals should be nutrient-dense and lower-calorie to prevent overeating.
- Limit Problematic Foods: Avoid foods that may exacerbate constipation or slow digestion, such as high fat, fried, or processed foods. Assess her response to dairy, as lactose intolerance can worsen symptoms.
- Incorporate Physical Activity: Gentle physical activities, such as walking, can aid digestion and bowel regularity. Even minor increases in movement can be beneficial.
- Work with a Specialist Dietitian: A dietitian experienced in PWS can provide tailored guidance, ensuring her diet supports optimal digestion while managing PWS-related challenges.
A comprehensive evaluation by a gastroenterologist, possibly including imaging and motility studies, might also uncover underlying issues contributing to these episodes. Keeping a daily record of BM’s using the Bristol stool chart will be helpful (type 3-4 is considered normal).
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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.