Further Information
Further information with regards to Optimal Nutrition for People with PWS can be found on the Foundation for Prader-Willi Research website.
View WebsiteDiet and Nutrition for Adults
Control of the food environment and effective behaviour management will set the scene for good dietary habits. As the nutritional needs of people with Prader-Willi Syndrome vary greatly from someone who is trying to maintain a healthy weight.
Optimal Nutrition
The diet of someone with Prader-Willi Syndrome can be very different from the general population who are trying to maintain their weight.
As we are learning more every day about Prader-Willi Syndrome, it is essential that your dietician understands; or is willing to learn about Prader-Willi Syndrome, and is abreast of all the latest research in their respective field of expertise. Foods that were once thought to be beneficial in a PWS diet may not be today.
The University of Queensland publication ‘Need to know nutrition for Children with Prader-Willi Syndrome states:
When we look at diet for an individual with Prader-Willi Syndrome , there are four key aspects important to maintain a healthy weight:
- Remove temptation – access to food should be controlled at all times, other than the food provided for meals and snacks. It is often necessary to lock cupboards, fridges and prevent access to rubbish bins. Controlling the food environment is very important to ease the pressure on the child with Prader-Willi Syndrome and will help your child’s behaviour, reducing temptation, tantrums, guilt and sense of failure.
- Behaviour – you will need to establish good eating routines, offer a wide variety of healthy food choices from an early age, and be consistent. Don’t reward with food, and don’t give in to tantrums.
- Quantity – your child will need to stay on a highly restricted diet with a lower quantity of kilojoules (kJ) than children without the condition.
- Quality – it is best to offer your child high quality foods which are low in fat, but high in nutrients
While low-fat options sound great in theory, it is essential to remember that good fats help support healthy brain development. Often packaged products that are labelled low fat often contain other ingredients that increase the amount of sugars and carbohydrates in the product. It is important to look at the nutritional information on the packaging to ensure that the food item/product fits within your child’s diet. It is vital to seek the guidance of your dietician if in doubt about food.
The right menu for someone living with Prader-Willi Syndrome will include vegetables, proteins, fruits, dairy and good fats, preferably as whole foods. Research has been conducted on a range of different diets and their effect on people with Prader-Willi Syndrome. As with the general population, the truth is that some foods will work for some and not for others. The key to a healthy PWS diet is to work closely with a dietician to create a healthy eating lifestyle that is sustainable, and that works for you and your family
Keeping well hydrated is vital. While water is preferred, strangely, people with Prader-Willi Syndrome do not enjoy drinking water. To ensure hydration is maintained, especially over the summer months, families have become creative in this area and used things such as natural tea infusions to add flavour to the water.
Fruit juice, although natural, is not recommended as a glass could contain as much sugar as a soft drink in the form of fructose, and this is rapidly released into the body unlike a piece of whole fruit. While you would not typically eat several apples as a snack, the equivalent amount can be swallowed easily in a glass of fruit juice and is less satisfying.
The same can be said for dried fruits, with decreased volume and concentrated fruit sugars. Be guided by your dietician before replacing full fat milks, yogurts etc. with low-fat varieties as fat is essential for healthy brain development.
The rule of thumb is to make each bite nutrient-dense. For people with Prader-Willi Syndrome to maintain a healthy weight, restricted kilojoule meals is essential. The kilojoules in some foods are higher than others with highly refined food, sometimes containing a full day’s nutritional requirement.
Nutrient dense foods are foods that are :
- Good for the overall health; and
- Can be consumed in greater amounts and are more satisfying to the body and the mind of the person with Prader-Willi Syndrome.
You are probably aware that these guidelines should apply to us all. Having a family that shares the same eating habits will not only support your own good health but will make it easier when preparing meals and will set an excellent example to support your child on their journey. Teaching your child that every person has different requirements for their unique body can help with the inevitable comparing of meal sizes.
Food Seeking and Hyperphagia
Of all the topics regarding Prader-Willi Syndrome, Hyperphagia, which is the insatiable drive to eat and of never feeling full, is the most well-known, most misunderstood and not necessarily the most significant issue when living with PWS. Like most aspects of Prader-Willi Syndrome, hyperphagia occurs at a widely varying age of onset and intensity.
Somewhere between the ages of eight and adulthood, you may notice that your child’s preoccupation with food has now become obsessive and their constant need for food has become uncontrollable. adults with Prader-Willi Syndrome, the insatioable appetite dimishes and they have the ability to feel full.
The International Prader-Willi Syndrome Organisation (IPSOW) provides information on their website to help us understand the hunger drive in people with PWS. Their website states the following:
“To understand the importance of this hunger drive, try to look at Prader-Willi Syndrome as a ‘starvation’ syndrome 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 keeps telling the stomach, “you’re starving, you need food”, and the drive to find food overrides everything else”
Early implementation of no snacking outside of set meal and snack times is essential, and treats of highly processed foods should not be offered. Rules, such as not helping yourself to food and no second helpings at mealtimes are all good practice. Modelling healthy food choices and eating habits makes it easier for your child also to understand and make good choices and gives a clear message or guideline to extended family.
Because the body composition in individuals born with Prader-Willi Syndrome is imbalanced, with a higher ratio of fat to lean muscle, it is surprisingly easy for individuals to gain weight in a very short period of time. Lower amounts of lean muscle result in less energy being used and low tone can make exercise difficult.
A person with Prader-Willi Syndrome will need to have a well-balanced, low-calorie diet and maintain regular exercise for the rest of their life. Height, weight, and BMI should be monitored every 6 months during the first 10 years of life after infancy and Is regularly monitored throughout their life to make sure a healthy weight is maintained.