A PWS Friendly Birthday Cake

We’re celebrating a very special birthday in our house this week, our darling Jude is ONE!

I’m often sent cake recipes and asked my thoughts on whether I think this or that is appropriate for a PWS birthday and for the longest time I’ve wanted to come up with my own. So here is the first one.

This cake is wheat free and has no added sugars, it is not sweet at all yet still packed full of flavour from the berries, yoghurt and lemon. This recipe is actually based off my Berry Yoghurt Muffins which is in my ‘Finger Foods for kids with Prader-Willi syndrome’ e-guide and it was a client who made the muffins for her daughter’ birthday which gave me the idea to make it into a cake for Jude’s first birthday and I was so happy with how it turned out. Jude loved it too!

If you make this recipe, be sure to tag me on Instagram, you can find me at @catefoxdietitian

PWS FRIENDLY BIRTHDAY CAKE

cook time 40mins

Ingredients

1 ¾ cup almond meal

2 tsp baking powder

½ tsp baking soda

⅓ cup coconut oil, melted

2 large eggs

1 cup plain Greek yogurt

2 tsp vanilla extract

zest from 1 lemon

juice from ½ lemon

1 cup berries

Method

1. Preheat oven to 180C and grease a cake tin. For Jude’s cake, I used two 10x4.5cm (4x2”) round cake tins which gave me a little of the batter left over to make into muffins. Alternatively, you would have enough batter to make 3 cakes.

2. In a large mixing bowl, combine almond meal, baking powder, baking soda and lemon rind.

3. In a separate bowl, whisk eggs, add melted coconut oil, yoghurt, vanilla and lemon juice and stir until combined.

4. Add the wet ingredients to the dry mix and with a wooden spoon mix until well combined.

5. Gently fold the berries into the batter. I used frozen berries above. If you choose to use frozen berries make sure they are still frozen when mixing, this way the colour will not run.

6. Pour the batter into the prepared baking tins and bake for 40mins.

7. Remove from oven and let cool completely before turning cakes onto a wire cooling rack.

8. Stack the cakes on top of each other and cover with your choice of icing (frosting). For the cake pictured above, I used fresh cream placing a layer of cream between the two cakes and covering the outside of the cakes with a thin covering of cream. Alternatively, you could you a cream cheese frosting. Note: if using fresh cream don’t add this until you are ready to serve.

The Mediterranean diet for PWS

Although there is no one diet for PWS, many health professionals recommend following a Mediterranean style diet.

So what exactly is the Mediterranean diet?

Not a traditional “diet” where calories are counted and fat loss is intended, the Mediterraenean diet is a dietary pattern traditionally eaten by the people living in the Mediterreanean, countries such as Greece, Spain and Italy.

It’s a dietary pattern that focus’ heavily on fresh fruits and vegetables, whole grains, legumes, beans and lentils and healthy fats from nuts, seeds and olive oil (Extra virgin always). Foods such as fish and other seafood, dairy foods such as yoghurt and cheese and white meats such as chicken are eaten in smaller amounts and red meats, sweets and processed foods are only included on occasions.

It’s a dietary pattern that focus’ on social engagement, where families come together to share a meal, taking time to enjoy each others company and the food they are eating.

Although not typically a low carb diet and definitely not a low fat diet, it’s a diet that is typically low in saturated fat (unhealthy fats) and rich in monounsaturated fats (healthy fats). Fats are essential in the diet as they assist with brain development and fat soluble vitamin metabolism. With a focus on fresh, whole foods it offers a variety of fibre sources from fruits, vegetables, whole grains, legumes, nuts and seeds.

So how does it benefit those with PWS?

Studies looking at the Mediterranean diet have proven to support a wide range of health conditions from improving cardiovascular health, and fertility and lowering risk of diabetes and some cancers. Although there are few studies looking specifically at the Mediterranean diet and PWS, a study by Dr Miller et al (2012) saw greater improvements in body fat mass and weight reduction in children aged 2-10years who were following a diet that draws comparisons to the Mediterranean diet. People with PWS still need to follow a calorie reduced diet, however Miller et al. found that when kids were following a reduced calorie diet with total energy being made up of roughly 45% carbohydrates, 30% fat and 25% protein better improvements in body composition were seen.

How do you incorporate the Mediterranean diet into a PWS meal plan?

When building a PWS friendly meal, start with vegetables - the more colour the better. Ensure variety day to day to avoid food fatigue and change up the ways you offer these vegetables for example, some days salad, some days roasted in EVOO, some days steamed. Include a protein source and try to include “meat free” days each week. For this you will need to focus on things such as lentils, beans and chickpeas to ensure protein needs are met. Aim for fish 2+ times per week, poultry 1-2 times per week and red meats <2 times a week. Add in a small serve of complex carbohydrates such as brown rice, quinoa, oats or ancient grains such as Freekah. If using bread, opt for well made sourdough. The fermentation process which occurs in sourdough lowers the glycemic index and slows down the speed at which sugar enters the blood stream. Add a healthy fat such as avocado, olive oil, nuts and seeds (walnuts are an excellent source of omega-3s) or olives. And lastly use a small amount of fruit - whether at a meal or as a snack, that’s entirely up to you.

I have complied a brief list of foods which shows you which food group certain foods lie. For example what a carb, fat or protein is. If you haven’t already, you can download this FREE resource HERE.

Another important component in managing PWS is setting consistent food rules. These will look different for every family and may change as your child ages however I strongly believe that one food rule should be a non-negotiable (and is the same advice I’d give all families whether PWS was apart of their life or not). My one non-negotiable is that where ever possible, meals should be eaten together at the table, no devices, no distractions just family conversation on any topic as long as its not food. This sense of connection has so many benefits and is after all, what the Mediterranean diet is founded on - togetherness, inclusion and community.

PWS or not, following a diet that focus’ on fresh fruits and vegetables, whole grains, lean protein such as fish and legumes along with encouraging good use of healthy fats such as omega-3s which have proven benefits for everything from eye, brain, heart and joint health is definitely an eating pattern that gets the tick of approval from me.

Davis et al. (2015) Definition of the Mediterranean diet: A literature review. Nutrients. 7(11) 9139-9153.

Miller et al. (2013) A reduced-energy intake, well-balanced diet improves weight control in children with Prader-Willi syndrome. J Hum Nutr Diet. 26, 2-9

Nutritional Considerations in Prader-Willi Syndrome

Prader Willi Syndrome is thought to affect 1 in every 15000 births. It is a rare, non-inherited genetic disorder where several genes on chromosome 15 are either deleted or not expressed. To date, there is no cure.

PWS affects the hypothalamus, the area of the brain responsible for regulating many of the body’s systems including metabolism, the development of muscle tone, regulating temperature control, blood pressure, heartbeat and sleep/wake cycles, the expression of emotions and many other body functions including controlling hunger. Due to this, a person with PWS exhibits low muscle tone, small statue, a slower metabolism, intellectual disabilities, anxiety and an insatiable appetite.

A Dietitian can play a significant role in a person with PWS’s life. PWS is the number 1 genetic cause of childhood obesity with issues with appetite being a component in PWS that significantly affects an individual and is quite contradictory in its presentation across their lifespan. There are 7 main stages of appetite in PWS (1).

- stage 0 - in utero, less growth in weight and length than their unaffected siblings

- stage 1a - from birth, no interest in feeding, may need to be tube fed

- stage 1b - feeding normalises and infant grows steadily along the growth curve

- stage 2a - weight increases without change in calorie intake

- stage 2b - weight gain associated with an increase interest in food

- stage 3 - hyperphagia, food seeking and lack of satiety (this generally occurs around 8yrs of age)

- stage 4 - rare cases of some adults no longer hyperphagic and are able to feel full

So what are the nutritional considerations for someone with PWS?

There is a high risk of obesity in individuals with PWS due to individuals having a slower metabolism, being placed on restrictive diets and who are preoccupied with or obsessive about food. In order to maintain a healthy weight once an individual becomes hyperphagic, an individual often requires dietary restriction of 50% of the estimated energy requirements for a person of the same age without PWS. In order to achieve this strict dietary regime, cupboards and fridges are typically locked to prevent anxiety and temptation around food and to keep the individual safe. A person with PWS could quickly consume a large volume of food resulting in choking or a perforation to the lining of the stomach which in severe cases can lead to death.

What is the current dietary advice for PWS?

There is no specific diet for PWS. Different dietary approaches work for different families. What is typically recommended however is to aim for a Mediterranean style diet that focuses on good quality lean proteins, healthy fats, fresh fruits and vegetables and complex carbohydrates.

The level of calorie restriction is very individualised with a ‘no one size fits all’ approach and although a reduced calorie diet is effective in controlling weight in children with PWS, studies have found that calorie restriction alone does not control the amount of body fat as well as a carbohydrate controlled diet (2). Research suggests that a calorie controlled diet providing ~45% carbohydrates, 30% fat and 25% protein with at least 20g fibre works best for weight control and improved body composition (2).

Sweet foods, sugar and sweeteners should be avoided in those with PWS. Apart from typically being higher calorie, nutrient poor foods, sweet foods when they hit our tongue, activate reward sensors in our brain signalling to want more and more. The sweet taste, whether it comes from natural sources or artificial sweeteners promotes an increased desire for sweet foods and this mechanism is more pronounced in those with PWS.

Micronutrient supplementation should be individualised and based off consultation with a doctor and dietitian. Depending on the type of diet the family follows will determine what vitamins and minerals may be of concern.

What are the common dietary management strategies?

Having a food and eating schedule can be particularly important for people with PWS as it helps the individual to understand that food will be offered at certain times and removes the uncertainty about what exactly will be offered. This helps to remove any DOUBT that food will be offered again and the HOPE (or chance) of getting more food that isn’t planned which helps to reduce stress and anxiety. Lastly, there will be no DISAPPOINTMENT as the child has received the food they could expect and at the time they were expecting (3).

Setting rules around eating such as only eating while seated at the table or only allowed to eat from their plate/lunch box can also help to establish positive behaviours. Never using food as a reward nor giving into tantrums are also ways in which some families manage this aspect of the condition. That being said, food should never be restricted as a form of punishment either nor should other family members eat “treat” foods or restricted foods in front of the family member who has PWS.

Exercise is another weight management strategy that should be encouraged from an early age. Physical activity is recommended for people with PWS as it improves mobility and prevents unwanted weight gain. A recent study (2019) found that long term (>6 months), consistent physical activity saw improvements in cardiovascular fitness, body weight and composition and glucose metabolism in adults with PWS and T2DM(4).

With the right support from medical professionals and having friends, family and other care givers on the same page as to what is best for the child, families can manage their child’s dietary pattern and weight and redefine their child’s future.

(1) Miller et al. (2011) Nutritional Phases in Prader-Willi Syndrome. Am J of Med Genet. 9999, 1-10

(2) Miller et al. (2013) A reduced-energy intake, well-balanced diet improves weight control in children with Prader-Willi syndrome. J Hum Nutr Diet. 26, 2-9

(3) New Concepts in Nutrition: PWS Nutrition Revised - Linda M. Goulash, MD, Pittsburgh Partnership, 2017

(4) Morales et al. (2019) Physical Exercise and Prader-Willi Syndrome: A systematic review. Clin Endo. 90, 649-661

PWS New Zealand, Dietary Management, www.pws.org.nz