My son Allister was born in December 2009 with classic PKU – a rare condition in which a baby is born without the ability to properly break down an amino acid called phenylalanine (Phe). Phe is found in food that contains protein.
Phenylketonuria (PKU) is inherited, which means it is passed down through families. Both parents must pass on the defective gene in order for a baby to have the condition (For this to happen, both parents have to have the EXACT same defective gene’s – so you can imagine how rare this is).
Babies with PKU are missing an enzyme called phenylalanine hydroxylase, which is needed to break down an essential amino acid called phenylalanine. The substance is found in foods that contain protein.
Without the enzyme, levels of phenylalanine and two closely-related substances build up in the body. These substances are harmful to the central nervous system and cause brain damage.
Because of all this, Allister has to be on a strictly controlled diet (for the rest of his life) – the diet is very restrictive, no animal products, nuts, soy, wheat etc. Everything he can eat has to be precisely measured and calculated for each day’s intake. (sounds nuts I know but you get used to it)
Today I made AJ some Low-pro bread, it smelled soo good mmm check it out: