Product review of medical foods for PKU - Low Protein Diet

Dear Simon  Harris,

We are Petitioning you as Misister for Health. We are Adults with (or Family/Friends) with PKU (Phenylketonuria) (or Allied disorders), a rare metabolic disorder which require a very low protein diet. We are under the care of Temple Street / Matter hospitals and will be for life. The diet requires the exclusion of protein sources such as meat, fish, eggs, dairy, nuts, pulses and regular baked goods such as bread, crispbread, biscuits.

For us prescribed low protein foods are a key part of the diet due to its limited nature. These prescribed food provide nutrition and satiety. They also contribute greatly to the compliance with the diet, which can be challenging for both children and adults.

Compliance with the diet is paramount as the effects of raised levels of certain amino acids, for example phenylalanine in PKU, will cause neurological damage to the brain. This includes brain damage in babies and young children, reduced cognitive ability in older children and adolescents and there is evidence to show neurological changes also occur in adults with this condition. There is more research taking place globally on the effects of low protein diets and the products available and this indicates that the use of prescribed low protein foods are beneficial for us, both in the short and long term. In addition to the low protein diet, We require protein substitute products which are also prescribed. They are essential for providing the nutrition missing from the usual protein sources in the diet. They also contribute to satiety and to controlling the amino acid levels in the blood, ensuring levels are kept lowered.

Unlike coeliac patients who require a gluten free diet, who can access these products in the local shops, low protein patients do not have this option.

It is Our understanding that no new low protein products have been added to the long term illness card list in approximately 6-7 years. However, the current low protein food providers have discontinued making some of their products in recent times. As we are sure you can imagine this leaves us in a very difficult and frustrating situation. There are a number of companies who are very keen to provide a new range of low protein products to Ireland, however to date, they are not getting any response from the HSE.

One company called Mevalia have products which are of a much higher quality than the current products available on prescription. In particular, their bread. The mevalia bread (unlike other breads available on the long term illness card which are like cardboard and must be toasted-impractical for school,college,work) looks and tastes similar to regular shop bought bread and stays fresher for much longer. We are sure you can understand how a staple food like bread would make a huge difference in the daily lives of so many PKU patients in this country. This range is available in almost all other European and Western world countries so it beggars belief how Ireland is so far behind in terms of updating low protein product ranges.

The ESPKU (European Society) held their annual conference in Dublin a fortnight ago and there is a lot of frustration and anger with Irish patients at the lack of suitable products on the market in Ireland. They are available in almost every other European country. It is very unfair that Irish PKU patients have to put up with substandard foods; bread that looks and tastes like cardboard, when our European neighbours are free to enjoy a wide choice of quality foods, all simply down to government cutbacks. We are referring to basic food products like bread, pasta and rice. These additions would change lives. We are sure you can understand our frustrations.
Some progress has been made of late in relation to the issue by committee members of the PKU Association of Ireland. They are in contact with Kate Mulvenna, Head of Pharmacy in the HSE. She has stated that the Clinical Nutritional Expert Group have been convened and they expect to be calling for new applications for addition to the Reimbursement List before the end of the year. This is promising for patients, however after such a long wait, we as PKUAI members feel we need the support and immediate action of TD's like yourself to push this along and ensure it happens.
We look forward to hearing from you and thank you very much for your time.
Yours Sincerely,

The undersigned