Oxytocin in PWS - a discussion paper
Over the past few years the issue of gastro-intestinal problems in PWS have become more prevalent. There are many issues around this starting with constipation, gastro-enteritis and stomach necrosis each of which are described in the papers cited here. If you suspect some sort of problem with a gastro complaint, it is essential to contact your GP, or even the Emergency Department of your hospital.
It has been observed that in most people with PWS there is an issue with slow-emptying of the stomach. It can take longer for the food to get through the digestive system and cause back-up in the bowel.
Many parents and care providers believe that because their child or adult has a bowel movement every day, this means they don’t have a slow emptying bowel. This is not necessarily true. Even with a regular daily bowel movement the intestinal tract may not empty appropriately. As the colon becomes more
backed up with retained stool, the ability to evacuate stool is less effective. Over a long period of time, continuous, constant hard pushing has resulted in some people with PWS experiencing rectal prolapse. (The feeling of constant fullness and pressure on the anus or itching of the skin from irritation from bile
acids present in the stool can contribute to reasons that some people with PWS insert their fingers into their anus or pick at it.)
A descriptive study of colorectal function in
adults with Prader-Willi Syndrome: high
prevalence of constipation
Louise Kuhlmann1,2, Iben Moeller Joensson3, Jens Broendum Froekjaer4, Klaus Krogh2 and Stense Farholt1*
How do we know if the individual with PWS we’re caring for has gastroparesis or a slow emptying bowel? What are the signs? What are the symptoms? What do we look for? The answers are, unfortunately, that there probably aren’t many easily recognizable signs or symptoms. Because the abdominal core muscles are generally weaker in persons with PWS, the stomach can often appear to be more rounded. If food is not emptied quickly enough, the stomach can look rounded (distended) and feel “too firm” to the touch. On the other hand, for those who are taking growth hormone medication and are therefore leaner, the stomach can already feel “firm” to the touch.
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RETHINKING THE DIET
"Over the past several years, we have learned many new things in the area of gastrointestinal health for persons with Prader-Willi syndrome (PWS). For years, there have been many anecdotal reports of persons with PWS of all ages having problems with swallowing, choking, stomach issues and chronic constipation. Up until recently, there had only been a few research studies on these issues, specific to persons with PWS. Studies have now documented low production of saliva (6); a high risk of choking (7), a high prevalence of constipation in adults with PWS (1) and documentation of slow stomach/gastric emptying and gastric rupture (2, 3, 4). Dr. Roxann Gross, PhD. (5) reported at the PWSA (USA) 2015 conference that many persons with PWS in her study were found to have significant swallowing issues. Food was visualized on x-ray, lodged in throats, but persons with PWS were unaware of it. We know that persons with PWS have low muscle tone. We are learning that this poor muscle tone affects their entire gastrointestinal system – mouth, esophagus, stomach and intestines. All of these findings point to problems with feeding, nutrition and digestive health. With this new knowledge, we now need to rethink the approach to diet and nutrition for the person with PWS."
Barb Dorn, RN, Kate Beaver, MSW, CSW