NHD Issue 149 Dietitian advisor to national society for PKU

A DAY IN THE LIFE OF . . .

Suzanne Ford, Dietary Advisor to NSPKU Suzanne is a Metabolic Dietitian working with Adults at North Bristol NHS Trust and also for the National Society of Phenylketonuria (NSPKU).

REFERENCES Please visit: https://www. nhdmag.com/ references.html

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A DIETITIAN ADVISOR TO NATIONAL SOCIETY FOR PHENYLKETONURIA Working for a small charity called the National Society for Phenylketonuria, I work with a group of people who have one of the most challenging and complex lifelong dietetic treatments I have come across as a dietitian.

This work is both fascinating and rewarding and I have the greatest respect for those who live with this metabolic disorder.

Phenylketonuria (PKU) is an inborn error of metabolism with an incidence in the UK of about 1 in 10,000. so we believe there may be about 6000 people living with PKU in the UK – although probably only about 3000 of them are diagnosed, with fewer than that attending specialist clinics. People with PKU are treated in the UK via a low phenylalanine (Phe) diet, with Phe prescribed in measured exchanges per day, in effect, a very low-protein diet and Phe-free protein substitutes.1 If an individual has PKU, or their child has, then the chances are, they spend a lot of time researching what to eat. When we, the NSPKU, undertook a survey (the biggest PKU patient survey in the world!), more than half of all respondents with PKU were taking less than 10 Phe exchanges daily – equivalent to 10g protein per day.2 This means that patients and families are reliant on lowprotein prescribable foods and they need to find manufactured foods that are lower in protein, such as new coconutbased cheese alternatives, some vegan products, ‘free-from’ manufactured foods, such as puddings, ice cream, baked goods and so on. European guidelines confirmed recently that this diet is for life.3 It is an incredibly complex diet.

Different foods have quite different protein contents and for every gram of protein content, the Phe content might vary too. For example, sweet potatoes are naturally low in Phe and are ‘exchange free’, but standard potatoes need to be weighed and counted at 80g per 1 x

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50mg Phe exchange for boiled or mashed potato and 55g for roast potatoes but only 45g for chips. Sweet potato fries and wedges are usually coated with either wheat or rice flour and so all food labels must be scrutinised and food weighed accordingly (although, if someone makes sweet potato wedges at home without a coating, then they are exchange free.) This is just one part of the diet. Planning how to get the right number of exchanges into a child to ensure that they have enough Phe to grow is important – if a child or adult doesn’t have the right amount of natural protein in a day, then there is risk of deficiency.

This constant, individualised diet research, planning, weighing and recording takes 19 hours/week4 when combined with the time it takes to negotiate the primary care prescription system and local or home delivery of dispensed items. (The PKU ‘community’ or patient population relies heavily on prescribable products.)

MY TYPICAL NSPKU DAY

8am-8.45am: Detailed dietary information via social media The @NSPKU Twitter account (me) tweets three to five foods per day as an information service to the PKU community – so it could be vegan cheese and what its exchange value is, comparisons between different rice or corn-based breakfast cereals and their respective exchange value, coconut ice cream and more. Accurate labelling is vital so that patients can weigh the amount of food that provides one gram

A DAY IN THE LIFE OF . . .

The Bath community members Step Forward for PKU.

Members of the NSPKU’s All-Party Parliamentary Group on PKU meet the Rt Hon Jeremy Hunt.

of protein (equivalent to one Phe exchange) and ensure they have the prescribed number of daily exchanges.

11am-1pm: Helping people with a rare disease overcome social isolation People who live with a long-term condition such as PKU assimilate that condition into their lives in different ways, and the NSPKU magazine aims to demonstrate that. Recording our events in the magazine is important too – the photo top left shows the community in Bath ‘Stepping Forward for PKU’, for International PKU Day (held on 28th June every year).

9am-10am: Dealing with prescription issues via NSPKU helpline I could be supporting someone who is struggling with their GP and not getting the prescriptions they need. Last week saw me (ironically, on ‘World Patient Safety day’), emailing a prescription administration team about a pregnant woman with PKU and delays in her scripts. In a registry of prescription issues undertaken earlier this year, we analysed 252 responses about poor access to prescribable products for PKU; in 47% of the responses the result of prescription delays or obstructions resulted in patients running out of prescribed food.5 Treatment disruption for very young children and pregnant women could have irreversible results on the brain development of the individual patient or their offspring. It’s my job to support patients by raising awareness of these risks.

10am-11am: Enabling low-protein school meals The NSPKU was set up in 1973 and the Medical Advisory Panel was established to inform our resources with a cross section of clinical expertise. We are currently writing a main dietary information booklet, but equally important is a project on resources to support children with PKU in receiving school meals, and a project to support eating out. If children cannot be like their friends and get a cooked meal, if they can’t be part of a family outing to a restaurant, then social isolation can occur, affecting quality of life. The dietitians volunteering their time for the NSPKU Medical Advisory Panel are developing written materials for use by school cooks, catering assistants and teachers, as well as for waitresses, chefs and restaurant managers.

2pm-4pm: PKU awareness work amongst NHS England and Public Health England, as well as the Dept of Health The NSPKU has set up an All-Party Parliamentary Group (APPG) on PKU to explore treatment access and to address the issue of the Soft Drinks Industry Levy and its adverse effects on the PKU community. Aspartame is a Phe ester, so any foods which have had aspartame added since the levy was introduced, are now completely off the menu for people with PKU. Also, the tax may be extended to other manufactured food groups, so raising awareness is vital. The photo (above) shows me explaining to the then Health Secretary the Rt Hon Jeremy Hunt, exactly how restrictive the PKU diet is.

4pm-5pm: Clinical dietetics: supporting Pregnant & Post Partum women with PKU Metabolic dietitians are working to develop practices that support the PKU community, including women after pregnancy. I send email newsletters to all NSPKU members, dietitians and dietetic assistants, providing them with news on new resources and products. People often send me information back which can then be shared. It is really rewarding to be part of such a committed and energetic community, which rises up to meet the challenges faced. www.NHDmag.com November 2019 - Issue 149

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