The NSPKU is the National Society for Phenylketonuria. Phenylketonuria (PKU) is a rare metabolic disorder and people with PKU cannot metabolise phenylalanine, an amino acid found in protein foods. This builds up to levels that are toxic to the brain.
PKU is diagnosed by the ‘heel prick’ in the new-born baby screening test, however, PKU is a treatable condition. Treatment is by a restricted diet and has been shown to be effective. People born with PKU who receive the correct treatment can now have the same educational and career goals as people without PKU.
The standard treatment for PKU is a low phenylalanine diet. The PKU diet involves removing almost all-natural protein from the diet and utilising prescribed medical dietary products to ensure adequate nutrition. The PKU diet is complex and very restrictive. Many patients find it difficult to adhere to dietary treatment. If PKU is not correctly treated, sadly, it can result in severe and irreversible brain damage. Drug treatments for PKU are already licensed but are not yet available in the UK on the NHS. Research into PKU and new treatments is still ongoing.
Lawrence Alston, Treasurer of the West Lancashire Masonic Clay Pigeon Shooting Society (WLMCPSS) said: “I was personally made aware of this condition when my son’s partner Sarah Derbyshire was about to undertake the Wainwright coast to coast walk of some 200 miles with her brother Adam in aid of PKU. Adam’s partner Robyn is affected by PKU. I met her for the first time at a family gathering and found out the details of her treatment and the amount of medication she was forced to take on a daily basis. It was great to see both Adam and Sarah set off on the long 200-mile walk, both with minimal training and some monies posted on the PKU JustGiving page.
I sought support from our West Lancashire Masonic Clay Pigeon Society at its AGM and proposed that this charity was definitely worthy of some support. The committee membership agreed unanimously and offered a generous £1,000. We know that this donation will be used for the benefit of PKU research and hopefully will lead to a more effective treatment and ultimately a cure for phenylketonuria.”
Report and picture by Lawrence Alston, edited by John Topping.