Awareness is the first step of any real change, so let's build an active community who wants to ! Phenylketonuria (PKU) is an inherited metabolic disorder characterised by mutations of the gene that encodes phenylalanine hydroxylase (PAH), the dysfunction of which causes high concentrations in the blood of phenylalanine (Phe). Nowadays, the standard of care for PKU is mainly ba
sed on two essential pillars: a lifelong low protein diet (that limits Phe intake from foods) and regular protein substitute administration (to support physiological protein synthesis). The main objective of the treatment is to maintain Phe levels in the recommended range, and the efficacy of the treatment is strongly influenced by compliance to the prescribed diet. As seen on evidence, compliance becomes increasingly difficult with age due to diverse factors. Bad taste, odour and aftertaste of amino acid-based protein substitutes are still a big issue that often causes adult PKUers to be less compliant to the diet or even leave it completely. Moreover, scientific evidence indicates that significant sub-optimal health outcomes still exist in compliant PKU patients. This is mainly due to the absorption profile of free amino acids (AA), which is very different from that of intact natural proteins, contained in common food. Free amino acids bypass the digestive phase, meaning that amino acids are absorbed quicker than proteins, giving place to plasma levels of amino acids that are higher, peak faster and decrease more quickly. Thus, the diverse kinetic profile of free amino acids has an impact on body metabolism and consequently affects PKUers' health and quality of life. International experts within PKU agree that there is still room for improvement in the PKU treatment to deliver improved taste and odour-free products, with the properties of natural protein, delivering a stable chemical environment associated with optimal physiological function and patient tolerance (https://bit.ly/EvolutionAAmixPKU)
Let's build a community of PKUers from all over the world, who are well aware that is just the beginning to embrace life!
