Phenylketonuria: Treatment and Support

Stephen Morgan Excerpts

Tuesday 26th June 2018 Westminster Hall

[Stephen Morgan (Portsmouth South) (Lab)]

It is a pleasure to serve under your chairmanship, Mr Robertson. I must admit that I had not heard of PKU before my election to the House, let alone of some of the medical terms, which I still cannot pronounce. I think that the same is true for many across our country. In fact, after hearing that it meant a fault with enzymes, leading to an inability to break down the amino acid phenylalanine—PHE—I was not much the wiser.

However, after hearing my constituent Holly Mae’s story and what the condition meant for her everyday life and tasting the various concoctions that replace typical meals for PKU patients, I was left in no doubt as to the potential debilitating impact of the disease. That is why I will begin with the impact on everyday life and what that means in practice for people with PKU.

As has been mentioned, people with PKU have to eat a diet with virtually no protein, meaning that they must take chemical supplements to avoid malnutrition. The briefing I was sent by the excellent NSPKU describes the protein replacements as “unpalatable”. I have tasted those replacements and can assure hon. Members that the NSPKU is being polite; they are absolutely rank. However, they do not just taste disgusting. They form part of an incredibly prescriptive and restrictive diet that not only consumes a huge amount of time—approximately 19 hours a week—but makes living a normal life difficult and social activities intolerably difficult.

Hollie Mae’s mum, Tara, says they hardly ever eat out. When they do, they have to bring separate food. It is the same at friends’ houses. It is inevitably a difficult diet to manage, and because no young person likes to stand out as different, PKU makes sensitivity and insecurity around food and eating particularly pernicious among its teenage victims. These young people just want to live normal, happy lives, but PKU often exacerbates teenagers’ vulnerability to eating disorders and so also becomes a mental health issue. I therefore urge the Minister to do all he can to improve access to psychological support for people with PKU. Clearly, the pressure of PKU and the diet it necessitates puts patients in immense difficulty and can be overwhelming.

We have just celebrated national carers week, and it is important to consider the burden that falls on families too, as we have heard. Half of parents stop working or reduce their hours to accommodate the extra work of caring for a child with PKU. What is more, the burden inevitably falls on women, with 81% of respondents to an NSPKU survey saying that it was the mothers who did most of the PKU-related work. I pay tribute to Tara and all the other parents and families who care for those with PKU. However, they do not want warm words, they need action.

I hope the Minister agrees that the opportunity to offer those with PKU and their families hope of a better life cannot be missed. There are practical things that can be done. I suggest that that means meeting with the manufacturers of a tablet can make a massive difference. It means making life a little bit easier for families and PKU patients by fixing the fragmented service on offer and smoothing and simplifying the chain from specialist metabolic clinic to GP to pharmacist to courier, complications in which constantly cause grief for patients. It definitely means ending the exclusion of PKU treatments from the prescription charge exemption.

The fact that PKU is a rare disease should not mean that it deserves any less of our attention. On behalf of my constituents and Hollie Mae, I urge the Minister to implement these changes.