Acute Gastrointestinal Warnings to Parents & Providers

It has been painfully clear with the number of deaths reported that parents and providers need to remain vigilant in providing a safe, food secured environment not only for obesity related issues surrounding a person diagnosed with Prader-Willi syndrome, but also for those who have achieved relatively positive weight management.
Recently the PWSA-USA office issued a very clear warning to all parents who have their adult children in placement, stating that while an occasional “goodie” may be allowed, a note of caution must be sounded. They go on to say that without exception, adults in residential care have their daily meals carefully managed in order to meet the special dietary requirements associated with having Prader-Willi syndrome. This usually means smaller portions and a reduced overall daily caloric allowance. It has become clear that adults who have had solid and consistent dietary management for some time are at risk for serious health consequences if they are then allowed to eat quantities that exceed what they are used to. Several deaths have been reported to have occurred following holiday visits home where food intake was not carefully monitored. As a result, these individuals consumed both individual portions and an overall quantity of food that exceeded their usual daily intake at the group home. These deaths usually occur because the stomach is unable to accommodate and process the increased quantity of food, resulting in gastric dilatation and even perforation. Some have also involved food going down the airway and into the lungs. We want to caution you that dietary monitoring while home that is consistent with the usual daily intake of the residential placement is critical for long term health, but more importantly to prevent short term, possibly life threatening, health consequences from increased intake. If your adult complains of any symptoms such as his/her stomach hurting or cramping, or has nausea and vomiting, or refuses food and appears ill, he/she should be immediately taken to an emergency room where the staff need to be told about the abdominal concerns associated with PWS. They should go to the PWSA-USA web site at www.pwsausa.org and then go to the site titled Medical Alert. The staff of PWSA (USA) and the members of the Clinical and Scientific Advisory Boards are available to assist Emergency Room personnel when needed.
We know that it is next to impossible to prevent every food theft situation, nor can we completely avoid the anxiety surrounding the fact that they are hungry and secretly hoping someone will leave something out, however we can all use a reminder of how serious this syndrome can be even when they are doing well.