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Sheffield Teaching Hospitals will be raising awareness of a rare but potentially serious inherited disorder ‘Phenylketonuria’ (PKU) to coincide with international PKU day on the 28th of June.

People with PKU have a deficiency in an enzyme in the liver and are unable to breakdown one of the building blocks of protein. The condition means that the patients suffering from the condition have to follow a low protein diet for the rest of their life. Individuals with PKU are also unable to eat foods such as eggs, dairy products, pasta, rice and potatoes and they rely on low protein foods such as low protein bread, cereals, milk and pasta.

For the week of the 28th of June, the Dietetics team from Sheffield Teaching Hospitals NHS Foundation Trust will be following the strict PKU diet to help raise awareness of the condition, so that they will see implications and struggles of having to follow the strict low protein diet.

Nicola Moran, Deputy Head of Dietetics and Specialist Dietitian for Adult Inherited Metabolic Disorders for Sheffield Teaching Hospitals NHS Foundation Trust, said: “PKU is a rare genetic disorder affecting only 1 in 10,000 people in Europe. A strict low protein diet is the only treatment available in the UK and Sheffield’s Northern General Hospital is one of only a few centres in the UK that treat adults with PKU.”

Sheffield Teaching Hospitals NHS Foundation Trust Dietetics department currently have 120 PKU patients on their case list and get approximately 20 new referrals a year. The Dietetics department also educate patients on their diet, teach patients how to interpret food labels and give advice on eating out. Additionally, the department also works closely with GPs, pharmacists and the CCG regarding any prescriptions for the PKU diet.

Sarah Kimpton is a patient of Rotherham General Hospital who receives advice from STH dietitians to help her follow the strict PKU diet.

She said: “Following the PKU diet is very time consuming. However, it ensures I am eating the correct nutrition for my body.

“The support from the Sheffield Metabolic clinic is amazing. In particular the specialist dietitian always being able to answer any questions and queries I have.

“I am luckily enough to have all my supplements and food items prescribed by the dietitian at STH. I know without this specialist input from the specialist dietitian I would not be able to access all the prescribable food items I require.”

For more information regarding PKU, visit NSPKU | The National Society for Phenylketonuria.