Managing the syndrome

While your child is young, there is much you can put in place that will make later management much easier. It is important to set some boundaries (for your child, and for yourselves) that will stand you in good stead as your child grows up. There are many excellent papers, pamphlets, textbooks written on the management of PWS. But that's all they are - textbooks. The management of your son or daughter is what you make of it. If you decide that you want the very best for your child, then you will become that child's chief advocate throughout life.

It's not easy, but there is help, support, advocacy, and good advice. And of course, plenty of written text! Here is a little more...

Two of the most outstanding characteristics of PWS are insatiable appetite and challenging behaviour. It doesn't happen all the time, and it doesn't have to be 24 hours a day, but it will happen.

The most important thing as a parent, member of the family, caregiver, or teacher, that you have to bear in mind is that much of this is caused because there is a tiny deletion in one of the chromosomes (15th) and not because the child is going out of his/her way to make your life a misery. Often, they cannot help it.

Often, life for them becomes a misery of its own - governed by what "everyone else is allowed to do, or eat". Every single day, three times or more, there will be meals provided.

Try to imagine what it would be like if your life revolved around this need to eat. Your major anxiety will be focussed on whether the next meal really is coming, and when, and what it is.

What if someone forgets me? What if midday comes and goes and no one gets me lunch? What if my parents/caregivers decide to go out and forget my tea? Who will feed me? If I have to go out visiting, will the people have food there, and will I be allowed it? If no one makes my dinner, where will I find some money to buy food with? Why can't I have what everyone else has? Why can't I eat at McDonalds? Why can't I have chocolate cake and fizzy drinks for afternoon tea? Why? Why? Why?

And so it goes on. All their lives.

How do you cope with this? First, by recognising the syndrome-driven needs. Second by trying to be one step ahead. Third, by intervening early enough to make it a way of life. Fourth, by educating everyone else around!

The craving for food is complicated by the fact that people with PWS have poor muscle tone so that exercising is often difficult, and so the weight goes on. People with PWS really need to eat only 75% of what their peers would eat.

There is a simple balanced equation that works, no matter what...

Energy input (what you eat) equals Energy output (exercise)

In other words, eating good low-fat meals and balancing this with the right amount of exercise should ensure that the person's weight remains stable.

Behavioural management should:

• be intervention-prevention oriented
• avoid arguments
• anticipate
• plan ahead
• reward

Strategies around weight management:

• need to be individualise
• include weekly weigh-ins
• provide complete control of food intake
• establish secure environment (eg. locking food cupboards, fridges)
• be based on a diet acknowledging fewer calories are required if your child/adult is not on growth hormone treatment

Strategies around food:

• Keep food inaccessible at all times - because they cannot fight their compulsion to seek food, you need to do it for them.
• Use smaller cups and plates, spread the food attractively on the plate and add extra non-fattening items, eg. grated carrot, salad vegetables.
• Lock cupboards and refrigerators.
• Clear food away from benches,
• Keep to routines and systems: people with PWS enjoy knowing what's coming next. 
• Be consistent
• Keep sight of the fact that the hand is quicker than your eye!
• It's not a good idea to use food as a punishment, or as a reward - this can often lead to extreme temper tantrums.
• Try to pre-plan your trips to the food stores and restaurants - set the rules before you leave the car (eg. "if we go into this supermarket, we will buy only groceries for the house. If you want to come with me, you must know that I am not here to buy you treats or presents, ok?")
• The same for restaurants, eg. "We are going to this restaurant as a special treat. Once we are in, I will look at the menu and help you choose. If you don't like my choice, then we will go home without having a meal here. Is this ok?"

Strategies for behaviour

• Praise and recognise good performances - a lot of mileage can be gained by praise.
• Listen when they need to talk.
• Include the person in planning and programming. They need to feel they have some control within their lives. 
• Inform neighbours, relatives, teachers, baby-sitters, alternative carers - in fact, everyone who comes in contact with your child. Keep on informing them!
• Remember that the person with PWS has a very concrete pattern of thinking and reasoning. When they lose their patience, there is no point in your trying to reason logically.
• Remember that it will be difficult to argue and "win". Make the statement, allow the person one more comment, warn that the discussion is now over - and stick to it! 
• Encourage your child to do everything possible for him/herself and don't excuse them from helping out around the house. Make tasks simple.
• When the upsets come, talking it out does not help, it only seems to increase the upset. Don't give in to the demand in order to stop the behaviour. This only reinforces the fact that tantrumming works. Use "time out" (bedroom or some quiet place) until the eipsode is over. (If necessary, remove everyone else from the room if the person refuses to go to their bedroom.) Then forget it. Dealing with outbursts can be exhausting, so give yourself credit and take a break as needed.
• Don't hesitate to ask for help.