Friends and extended family are in the unique position to have an immediate, positive impact on the life of the person with Prader-Willi syndrome and their family. Do not underestimate how important your support is. In a recent study2 comparing parents with children with a variety of disorders and disabilities, parents of children with PWS reported the highest levels of stress of any other participating parents. But that same study found that parents of children with Prader-Willi syndrome listed other family members and friends as their main supporters. Your support really matters, and there are a few rules of thumb you can follow to be the best friends and family you can be.
What does this mean for extended family? It means that when an individual with PWS is visiting your home, you keep food out of sight in cupboards or in the pantry. It means that before they come over, you’ve already checked your counters to make sure there weren’t any food items out. It also means that you’ll help ensure a low-stress family holiday mealby serving food pre-portioned onto individual plates for everyone, rather than a buffet or a self-serve “family-style” dinner. It means creating helpful distractions for the child with PWS if they are struggling with holiday treats; arts and crafts activities in a separate roomis one fun way to help keep the child with Prader-Willi syndrome pleasantly occupied away from food. When you, as extended family, take the time to learn about behaviors triggered by the syndrome, you enable yourself to give the parents well-needed respite when they stop by for a visit.
What does this mean for a family friend? It means that when you invite them over to your house, you don’t have food on the counter. It means that when you want to spend time together with the family, you look for food-controlled environments. Good places for this include going to the zoo, taking a stroll in a local park, or visiting a museum or historical site. When the family doesn’t have to devote their energy to preventing food-seeking behavior, they can actually relax with you. Time with you, as a family friend, keeps the fun in life and is rejuvenating for the whole family.
As family and family friends, you should also know the following:
–Tantrum behavior is a normal, expected phase of human development. Though other children grow out of the “terrible twos”, people with PWS generally don’t pass beyond that developmental stage, regardless of development of intellectual, language, and motor abilities1. If you see tantrum behavior, it is not because the person has been “spoiled”. However, don’t reinforce the behavior by giving in. Maintaining a cool head and calm voice will do much to help the individual get control of their own emotions again.
–Food seeking behavior ranges in severity, but in all cases the brain acts as though it is in “starvation mode”. Their metabolism is lower, lean muscle mass is down, individuals tend to be less active, and the food drive is heightened3,5. Maintaining a healthy weight requires both a low-calorie diet and regular physical activity. In addition, their brain doesn’t register satiety4. As a result, food seeking is unrelated to hunger in the normal sense: they may food seek even if they’ve just finished Thanksgiving Dinner.
-Stubbornness is a way of saying NO to changes that might cause stress or anxiousness.Many individuals with Prader-Willi syndrome display some level of cognitive rigidity, getting “caught” on one thought or concern. It is not malicious or learned – it is a part of the syndrome. Redirecting the individual who is stuck on a thought (by introducing a new conversational subject, for example) is one way to help the individual around their own rigidity.
–Judgment is impaired. Learning via negative consequences does not work. For example, if you or I decided not to bring umbrella one cloudy day and were rained on, we’d remember the unpleasantness and be more likely to bring an umbrella next time it was cloudy. In the same situation, an individual with PWS may continue to leave the umbrella behind in spite of past experience. Fortunately, people with PWS do respond well to positive incentives and rewards. Your praise means the world to them.
1. Dimitropolous A, Feurer ID, Butler MG, Thompson T. Emergence of compulsive behavior and tantrums in children with Prader-Willi Syndrome. Archives of Disease in childhood.1994;70;58-63.
2. Hodapp R, Dykens E, Masino L. Families of children with Prader-Willi syndrome: stress-support and relations to child characteristics. Journal of Autism and Developmental Disorders. 1997;27(1):11-12.
3. Holland AJ, Treasure J, Coskeran P, Dallow J, Milton N, Hillhouse E. Measurement of excessive appetite and metabolic changes in Prader-Willi syndrome. International Journal of Obesity. 1993;17:527-532.
4 Lindgren AC, Barkeling B, Hagg A, Ritzen EM, Marcus C, Rossner S. Eating behavior in Prader-Willi syndrome, normal weight, and obese control groups. Journal of Pediatrics.200;137(1):50-55.
5. Schoeller DA, Levitsky LL, Bandini LG, Dietz WW, Walczak A. Energy expenditure and body composition in Prader-Willi syndrome. Metabolism. 1988;37(2):115-120.
PWCF maintains online and in-person Support Groups for parents and professionals. Friends and family are welcome too.
PWSA (USA) maintains several Facebook support groups.