Information for patients, their families and helpers
Diet: Why diet is important
Since 1963, researchers have known that adult Refsum's disease patients accumulate phytanic acid in their body tissues and serum. This fatty acid comes into the body in foods, and adult Refsum's disease patients lack an enzyme which is needed for metabolising and eliminating it. So if phytanic acid can be excluded from the diet - stopping it coming in to the body in the first place - the condition of patients can be substantially improved.
At first little was known about the amount of phytanic acid in foods. It had been found in fats from cows, sheep and fish, and possibly other fats, so at first an overall low fat diet was prescribed. All green vegetables and some fruits were also forbidden, in case the phytol they contained could turn into phytanic acid in the body. The resulting diet was very restricted and unpalatable, and lacking in vitamins, so supplements had to be taken.
Much more is now known about phytanic acid in foods. A systematic analysis of foods from all food groups has identified the foods which are significant sources of phytanic acid, and which adult Refsum's disease patients need to avoid.
We have used information from the most recent analysis to draw up a list of which foods to avoid, which to enjoy freely, and which to eat occasionally and sparingly.
Next: Phytanic acid and phytol
Page last updated 26 June 2006