Carbohydrates are important

These should be included at each meal

 

The brain needs a regular supply of glucose to function. Without a regular supply of glucose, your body will break down its carbohydrate stores and once it has used those, it will break down your fat stores and release phytanic acid from your body fat. It is very important not to follow a low carbohydrate diet.  Carbohydrates can come from starchy foods (bread, potatoes, rice, pasta, breakfast cereal) and sugary foods (fruit, sweets, fruit juice).  Carbohydrates should be distributed throughout the day (included at each meal and in snacks, including a bed time snack), and should not be less than 200g of carbohydrates in total per day. Carbohydrate free meals should be avoided.

 

If you have other health conditions such as diabetes, it is important to talk with a dietitian for specific advice.

 

Ensuring adequate carbohydrates when exercising

It is important to fuel your body with carbohydrates prior to exercise. This could include eating a carbohydrate containing meal or snack before exercising. If exercise lasts longer than 45 minutes, you should take a carbohydrate snack during exercise. For example, if you go on a 90-minute walk you should bring a small snack to eat at 45 minutes (e.g. banana, a couple of dates). If you exercise without adequate carbohydrates, your body will start to break down fat and release phytanic acid. If you are starting a new exercise plan or wanting to increase the amount of exercise you do, it is important to talk with your dietitian. 

 

Good Protein Sources for Adult Refsum Disease

Protein is an important nutrient for growth, development and maintenance of muscle. Protein foods should make up 1/3 of your plate at meals. Some protein foods are very high in phytanic acid (e.g. beef, lamb, cheese, full-fat milk) and need to be avoided. Below is a list of protein foods that can be eaten daily. You will also get some protein from vegetables, cereals and grains. You can use the traffic light lists above for further ideas of protein foods (including those that can be eaten in moderation on the ‘caution’ list).

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• Chicken, turkey, duck breast, lean pork (e.g. pork loin, ham without fat, back bacon without fat)

• Eggs

• Prawns/Shrimp, Scallops, lobster, clams

• Perch (wild), coley, sold

• Quorn

• Tofu

• Kidney beans, black beans, green lentils, butter beans, chick peas

• Whey powder

• Fat-free cow's yoghurt

• Soya yoghurt

• Fat-free milk

• Almonds, Cashew nuts, walnuts, pistachios, hazelnuts, macadamia nuts

• Pumpkin and sunflower seeds

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Eat regular meals and snacks

This means having breakfast, lunch, evening meal and a bedtime snack. If you have a long break between meals (4 hours or more), have a snack. It is particularly important to eat breakfast, so that you break your overnight fast. It is also important that you do not exercise in the morning unless you have eaten breakfast.

 

Dieting to lose weight is not recommended unless under close supervision of your medical and dietitian team. If weight loss is desired, it is important that you work closely with your medical team and dietitian to develop a weight loss plan and regularly monitor your phytanic acid levels.  Any time you lose weight phytanic acid is released from your fat stores; therefore, very slow weight loss would be recommended.

 

Avoid high intakes of caffeine

Consuming large amounts of caffeine from energy drinks, sports drinks, or high intake of coffee is not recommended. If consuming products with caffeine, a moderate caffeine intake is advised. For an adult woman (not intending to become pregnant) this would be 200-350mg a day and for an adult man, 300-450mg a day.  Examples of caffeine content in various products are listed below:

 

Getting enough glucose when unwell

If you are feeling unwell and unable to manage all your usual meals, you will need to consume glucose at regular intervals in order to stop the breakdown of your fat stores and release of phytanic acid into your body. This is called an emergency regime, and an example of one used at Guy’s and St Thomas’ Hospital, London, is included (see Emergency Regimen). This regimen is specific for those who do not have diabetes. If you have diabetes, it is important to contact your medical team and dietitian for specific advice.

 

Medical procedures or surgery that requires fasting

Fasting for a surgical or medical procedure can result in increasing phytanic acid levels. To prevent the increase in phytanic acid levels, glucose solutions (oral and IV) will need to be provided prior, during and post procedure. If you are having a surgery or medical procedure, please contact your medical/dietetic team to make them aware.

 

Ensure you are getting your essential fatty acids

Omega 3 and omega 6 are essential fatty acids that the body cannot synthesise and therefore need to be consumed in the diet. Omega 3 and 6 are found in vegetable oils, and can be a source of essential fatty acids on a low phytanic acid diet. In addition, omega 6 can also be found in chicken, eggs and wholegrain breads. It is important to discuss with your dietitian about ensuring you are getting omega 3 and 6 from your diet.

 

Ensure adequate vitamins and minerals

With all specialist diets it is important to regularly monitor nutritional bloods (for example, vitamin B12, folate, ferritin, vitamin D). These should be monitored annually and vitamin / mineral replacement provided as needed.