Tuesday, November 20, 2012

Visions of Sugar Plums - A Warning for People with PWS During the Holidays

This is one of those topics where, to someone not intimately acquainted with PWS, it can look like an invasion of the crazy parents who don't let their kids have any sense of freedom and childhood wonderment when it comes to the holidays. But friends, it's complicated. If nothing else, please read the concluding paragraph as we head into the holidays....

By: Janalee Heinemann
Director of Research & Medical Affairs, PWSA(USA)

It is OK to have visions of sugar plums dance in the dreams of your child with Prader-Willi syndrome – but how do you keep them out of their mouths? For the holidays, with food being so plentiful, it is important to plan ahead to prevent problems. 

Thanksgiving, Christmas or Hanukkah 
• If you will be with relatives, carefully plan ahead of time and communicate the importance of food control with all involved. Make sure all attending know the “rules of engagement” and agree to cooperate.

• It is okay to request that Grandma and other relatives tuck away tempting items during your visit and to discreetly check with you prior to offering your child a treat. 

• If your child is old enough, rehearse the “rules” before that day and come to a mutual agreement on what your child will be allowed to eat. You can barter, i.e. “Do you want a little extra turkey and dressing, or do you want a piece of pie as your special treat?” 

• Make sure you know what everyone is bringing, so there are no surprises on what the choices will be. 

• See that someone at all times is clearly in charge of your child with PWS. In fairness to mom, you should switch off, each taking an hour, and clearly defining when you are changing guards. As Dr Linda Gourash states, “When everyone is in charge – no one is in charge.” 

• Grandpa and Grandma, or aunt and uncle may want to bring a special gift toy to compensate for the food they have to deny your child. 

• Go over with the hostess or your family on how to contain the accessibility of food. See to it that where your child is sitting there will not be a lot of bowls of food, rolls, or condiments nearby (Many people do not consider how many calories our children can consume with the extras – sugar, butter, catsup, etc.) 

• After eating, when people are just visiting, see to it that if the food cannot all be put away, someone is responsible for guarding it. 

• Your child must have the security of knowing you will be strong in your commitment to keep them protected from food – in spite of themselves. Giving in, even once, means several battles ahead. I know you get tired of hearing it, but consistency is the key. 

Of course, each family must judge their own situation based on their child’s food drive and their own regulations on treats. Some families are raising their children to never have any sweets – no exceptions. Others (like ours) just go by calories and the weight of the child, trying to keep the diet less in quantity yet similar to others in variety. Often, the most important thing is to prevent food sneaking or food demands. There is a large variance in the food drive of children with PWS. Some will ask or beg for more food, but make no significant attempts to sneak food. On the other hand, some will go to great extremes to get food, and are incredibly cleaver at doing so. 

Remember, it is also your holiday – Take special time away from your children. Tell your relatives what you would like for a gift is a day, night, or weekend away from your children. Then, you can come back refreshed to help make it a happy holiday for all. 



A couple of holiday seasons ago, three of our teens and adults with Prader-Willi syndrome (and a fourth unconfirmed) had unexpected deaths due to food binging episodes that led to necrosis of the stomach wall and a perforation (tear) in the stomach. In three of the four deaths, the person with PWS was slim, so there was no great concern about weight gain. Since then, we have been putting out alerts during the holiday season. In this last year, two more deaths occurred in a similar way. Keep in mind that a person with PWS, who is slim, does not mean they have total food control. Add too many temptations around, the lack of feeling full, the high pain threshold, and a weak vomiting reflex -- and you have the potential of filling the stomach dangerously full. Because there are many food binging episodes of our children and adults with PWS, most not having such disastrous results, we think there are probably other factors that play into this life-threatening situation that have not been identified to date. Please see that all provide the safety and security that your child deserves.

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