Diet and Nutrition
It is important that food management starts early. It is far easier to start outright than to remove privileges because of weight gain. It is also important that you recognise that food-seeking will be a problem which may result in morbid obesity, and that you take steps to control this before it becomes a life-threatening situation.
Diet and Nutrition in PWS
Children and adults with PWS do not have the expected sensation of how much they need to eat, this is because of the hypothalamic dysfunction (in the brain) . During the first months of life, the baby does not show signs of hunger and later this changes to overeating if there is no control. So the regulation of food intake is always required. When the person’s weight is normal it is tempting to relax and serve sweets and cakes, but it can then be very difficult to go back and “change the rules” again. Therefore it is important that food management starts early and remains consistent.
Food-seeking behaviour can become a demanding problem, especially when the child has access to food, and when there is no plan for a diet, and you perhaps sometimes give in. Without a plan for daily food and medical recommendations around nutrition it can easily result in morbid obesity, a life-threatening situation.
Often family members can have different opinions about how strict you have to be with the diet. You can tell them that it is similar to the situation for children with diabetes or allergy, the child becomes ill when the diet plan is not followed.
Remember how hard or impossible it is for the children to control their eating. They need our help and support, and should never be blamed for not having control themselves. This is to blame them for having PWS. Their environment is key.
To understand the importance of this drive to eat, try to look at PWS as a ‘starvation’ syndrome (behaving as if you were starving) rather than an over-eating one. Because of the dysfunction in the hypothalamus, there is no on/off mechanism that tells the brain, “I’ve eaten enough”. What happens instead is that the brain (hypothalus) keeps telling the body, “you’re starving, you need food”, and the drive to find food overrides everything else.
This is a picture of what happens, but the child is not able to explain this, it just happens: when food is seen it is a great temptation, difficult or impossible to resist.
Good Support and Management
Without support and good management, severe obesity will arise early on and can, within a few years, becoming life-threatening. A paradox when you think about how many hours it took the child just to drink one bottle of milk during the first months. The vast majority of people with PWS will, without support, show excessive eating behaviours, which in some cases can include stealing food or stealing money in order to buy food. They might take food from others in the school. Some can display an extraordinary ability to find food and just when you thought it was safe to leave the room for a few moments, you can return to find something missing! Added to this is an inability to reason between right and wrong when it comes to food-seeking, and you have the makings of some serious behavioural challenges, especially if you blame the child/adult for stealing food, instead of looking at the situation and why it happened. Think about what happens, you cannot demand that the person behaves as if he/she did not have PWS. And no one with PWS sets out to create such difficult and painful situations.
Management also means locks on pantries, fridges, food cupboards – or only have food in a kitchen where you can lock the door. This is not from birth, but when food-seeking becomes apparent. Although this might seem antiquated and unfair, it is incredibly helpful to the person with PWS to know that food is secure and is not a temptation to them. Some children even remind the parents that they have to lock the kitchen.
Thinking about food
Many children and adults with PWS concentrate on thinking about food. Be aware in kindergarden, school and work etc that food is not in the class room, play room, activity room etc.
It will take the child/adult´s attention, and playing, learning and work become difficult or impossible.
When you go to parties, have a plan for what will be served , and prepare the child/ adult with PWS, so that no discussions (or arguments) arise and tell your family that they have to respect the disease, instead of bringing the child into a critical situation.
Gather your network together
You will need to gather around you a good network of professionals; opportunities for visiting occupational therapists, nutritionists, paediatricians, specialists, will be given to you – take advantage of everything to help you, your family, your child. IPWSO is also here to help you. We can put you in touch with other families in your country, send you information, counsel, advocate, or just talk things through. There will be times of despair, but there will also be times of great joy.
Counting the calories
Calories are what the body uses as its source of fuel and energy. Energy from food is calculated in calories to give a quantity we can measure. For example, a boiled egg has 80 calories. Although the correct terminology for calories is Kilocalories (kcals) and, even more confusingly, kiloJoules (kJs), which are more used in some countries as food measurement.
Reading food labels
In many countries foodstuffs are required to carry a label stating, among other things, the energy level (or calories) per 100g. You will find that this will in some countries be stated as Kilojoules or kJs. A rough guide to convert to calories is to multiply by 4. How many calories does a person with PWS need? This will vary, of course, depending on the age and physicality of the person. For an overweight adult (not on growth hormone therapy), a diet of 1,000 calories per day is often recommended. But the needs can be lower, can very a lot, depending how much the person moves and how tall the person is. If weight loss is important the calorie needs can be less than 6-7 kcal/per cm height: this is 1,000 kcal if the person is 150 cm, but less if the person is shorter or moves very little.
On the other hand we see adults being very physically active, being taller because of growth hormone treatment during childhood , and with a normal weight they can have a daily calorie need sometimes above 2,000 kcal.
And if the person put on weight with a prescribed diet there are two possibilities: either the diet is not strictly followed, (or respected by the family, friends etc) or too many calories are prescribed, which can be seen , if the dietician is not familiar with PWS.
Why is the calorie need so low? The case is that the muscle mass in the body is small in people with PWS. Muscle activity burns the calories eaten. If you have small muscles (as you are born with in PWS) and perhaps use the muscles very little, sitting most of the day, the calorie needs to avoid becoming overweight become very small.
Exercise and calories
Exercise – even of the slowest kind – needs a certain amount of energy, and therefore burns calories. It is an extremely important part of managing weight. The only way to lose weight is to burn off more calories than we eat.
For example for an adult without PWS, with normal muscles and a weight of 75 kg:
- Sitting for one hour and not doing much, will use 100 calories
- Doing housework for one hour, will use 180 calories
- Gardening for one hour, will use 220 calories
- Brisk walking for one hour, will use 330 calories, and
- Jogging for one hour, will use up 750 calories
For a person with PWS with smaller muscles, but even higher weight, the calories used during these described activities are much less, perhaps half. Besides this, we have also a basal calorie need to keep our body alive, which for a person without PWS will be around 1,000 kcal per day, but much less for a shorter person with PWS with small muscles.
Try to encourage exercise at all times – even hidden exercise, such as hanging out the clothes, window-shopping, parking further away in the supermarket car-park, climbing the stairs, helping push the supermarket trolley, it is all better than nothing. There are all sorts of exercises designed to strengthen muscles and if your child is under the care of a physiotherapist, make sure you start an early exercise programme and… make it fun! And do it daily at home.
For adults: walking after each meal for 20-30 min is a good way to get the body and calorie burning activated
Growth hormone treatment
Growth Hormone treatment will help strengthen muscles, improve energy levels and enable a much greater level of physical participation. It does not mean that the drive to eat will decrease, or food-seeking will disappear, but it will help with the development of good muscle tone which will improve your child’s ability to exercise.
With or without out Growth Hormone treatment, a regular exercise programme is essential and management of diet is critical. It is much harder for a person to exercise if they are overweight, so try to involve them in your planning so that they can choose things to do that they enjoy.
Updated March 2021
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