Hyperphagia and obesity are responsible for most of the morbidity and mortality in patients with PWS. There is currently no medication effective against hyperphagia (Heymsfield et al 2014). It is important that both the food seeking behavior and insatiability be controlled to prevent obesity. Obesity is generally curbed by strictly-controlled diet and limited access to food. Hyperphagia is generally managed via development of food security in patients. In certain cases, surgical intervention might also be helpful in weight loss.
Dietary Restriction
Patients with PWS have a predictable pattern of weight gain, with obesity beginning in childhood and worsening as hyperphagia develops. Strict dietary control and long …show more content…
Physical food security menas a physical barrier to food access. This consists of locking up refrigerators and cabinets as well as placing food in places not reachable by the patients (McAllister and Whittington 2011). As patients grow older, access to money should also be controlled to prevent patients from purchasing food (Miller 2012). Controlling food access alone does not manage the preoccupation with food and the food seeking behavior of PWS. The psychological aspect of food security is also important in managing hyperphagia. This can be summarized in the mantra of “No Doubt, No Hope, No Disappointment”(Stotland and Larocque 2012). There is “no doubt” about when meals will occur and what foods will be served. This involves having predictable daily routines where snacks and meals are scheduled (Heymsfield et al 2014). Menus including the type of food and the amount are given to patients for every meal and snack so they know exactly what and how much they’ll be eating (McAllister and Whittington 2011). Food is never taken away from patients as a punishment, but there is also “no hope” for food outside of what’s already planned (Heymsfield et al 2014). This consists of both physical barriers to food, but also an avoidance of situations where there might be excessive presence of food. These situations include celebrations such as birthday parties, going to …show more content…
It is usually divided into three types, malabsorptive (eg. Biliopancreatic Diversion), restrictive (eg. Vertical Banded gastrophoplasty and gastric balloons) and hybrid procedures (eg. Roux-en-Y gastric bypass)(Bingham et al 2013). The success in weight loss achieved by bariatric surgery makes it an attractive option for patients with PWS. Bariatric surgeries have been performed in PWS patients but with mixed outcomes (Heymsfield et al 2014). With Gastric Bypass, there is a weight loss of 6.5% after 1 year but only 2 % after 2 years. This is significantly less than the 35% loss seen in those without PWS. For Vertical Banded gastrophoplasty, there iss actually a weight gain after 5 years. Biliopancratic Diversion leads to a weight loss of 27.6%, but the weight is regained by half of the patients after 2 years (Scheimann et al 2008). BioEntrics Ingastric Balloon (BIB) is a restrictive procedure that reduces stomach volume via the insertion of a balloon. PWS patients treated with BIB show a significant decrease in BMI as well as fat tissues. The procedure is also well tolerated and can be repeated more than once in patients (De Peppo et al 2008). Mini-bypass surgery is a modification on the Roux-en-Y Gastric Bypass. When performed on patients with PWS, there is excess weight loss of 79% after 2 years and no complications (Musella et al 2014). This suggests that bariatric surgery could be a