Information for families with RCDP

Recommended laboratory testing to follow children diagnosed with RCDP includes:

Phytanic acid levels
Level of docohexanoic acid or DHA
Plasmalogen levels
Routine liver function tests, complete blood count, basic chemistry panel, calcium, phosphorus, and Vitamin D levels

Phytanic acid levels could be high in children with RCDP type 1 (PEX7 defect) whose diet is not largely composed of an infant formula. If phytanic acid levels are very high, we recommend dietary changes to reduce these levels.

It is possible that DHA levels might also be low in children with all forms of RCDP, and since DHA can also be supplemented, we recommend that DHA levles be followed.

Plasmalogen levels are obtained at diagnosis however, it is unclear if these levels increase over time, possibly due to recycling of existing plasmalogens. In order to determine this, we suggest obtaining a repeat red blood cell plasmologen level every few years.

When you have this laboratory testing on your children, please be sure to include the physician involved in the care of the child to whom the results should be sent.

Dietary requirements

While most countries do not currently advise parents to avoid foods that are high in Phytanic acid it makes sense to not give children with RCDP something that their bodies cannot deal with. This does not mean that you should avoid it completely as Phytanic is in so many foods it would be unfair to never let a child with RCDP experience the taste of these foods. We would however recommend that their main source of food is either free from Phytanic acid or has as small an ammount as possible. Most children with RCDP are on some form of Paedeatric Milk so it is probably best to liase with your childs dietician to choose a milk that is Phytanic acid free.

However do not fall into the trap of just giving your child boring milk for every feed. Allow them to try new tastes and where possible textures as often as possible. We have found that Callie loves to taste fresh fruit and while she cannot eat the fruit completely she loves the taste no matter if it is sweet or sharp, she loves the feel of it in her mouth and the strong flavour and sucks her lips to get every bit of taste. Allow your child these joys that you or I would take for granted, I've tasted the milk Callie has and it is quite bland and disgusting so give them some fun flavours.