UK Rare Diseases Framework

Part of the debate – in Westminster Hall at 5:12 pm on 24th March 2021.

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Photo of Christina Rees Christina Rees Labour/Co-operative, Neath 5:12 pm, 24th March 2021

It is a pleasure to serve under you as Chair, Mrs Miller. I was not aware of the rare metabolic disorder phenylketonuria, or PKU, until my constituents with PKU explained that it prevents them from metabolising phenylalanine, or PHE, which is an amino acid in protein foods. The standard treatment is a low-PHE diet, removing almost all natural protein and replacing it with prescribed medical dietary proteins to ensure adequate nutrition.

The PKU dietary regime is very complex, very restrictive and very difficult to manage. I joined the all-party parliamentary group on phenylketonuria, which was formed by my hon. Friend Liz Twist, and became vice-chair. I congratulate her on securing another PKU debate today. The National Society for Phenylketonuria, a charity set up in 1973, is remarkable. It has no premises and no full-time staff, but is run by wonderful volunteers with personal experience of PKU.

Managing PKU is extremely demanding. Every meal, snack and drink must be planned in advance. People with PKU and their families spend on average 19 hours every week preparing their diet. Many of them have applied for personal independence payment, which is assessed on the basis of how much help is needed with ordinary daily living activities, one of which is managing therapy or monitoring a health condition.

The Department for Work and Pensions has not accepted that the PKU diet is a therapy, so many people, including my constituents, have been denied the daily living activities component of PIP, even though they need hours of help from relatives every week to manage their diet. However, in 2020 a tribunal decided that the PKU diet qualifies as a therapy, following a legal challenge by a 21-year-old man whose PIP application had been refused by the DWP. He appealed to the first-tier tribunal, but it agreed with the DWP that his PKU diet was not a therapy. He appealed again to the upper tribunal, which found that the first-tier tribunal should re-examine his case, because the reasons it gave for reaching its decision were not adequate. The case re-examination found that his PKU diet was a therapy under PIP criteria, because he needed more than 14 hours of help per week and therefore met the criteria to qualify for PIP, and should receive £87.65 per week. That is good news, but it remains to be seen whether this will govern future DWP decisions about PKU. I sincerely hope that it will, to help the brave PKU sufferers who struggle every minute of every day to live with such a challenging rare metabolic disorder.