Ireland is one of the leading countries in Europe for instances of phenylketonuria, PKU. Approximately one in every 4,500 babies born in Ireland has PKU in contrast to the United Kingdom, where the figure is approximately one in every 12,000, and further afield in the United States, where the condition is even rarer, affecting only one in every 15,000 newborns each year. In Ireland we follow diet-for-life, whereby each PKU sufferer is continuously assessed from birth to determine how many grammes of protein they can have on a daily basis. This assessment is determined from blood tests, as well as weight and height measurements and these tests are ongoing for life.

In order to facilitate the healthy growth and development of PKU sufferers to supplement the often very low amount of protein they can have, foods are available on prescription through the long-term illness scheme, for example, pasta, pizza, flour milk and bread. Despite the fact Ireland has one of the highest incidences of PKU in Europe, it has one of the most limited varieties of foods available on the long-term illness scheme in comparison with our European neighbours.

Given all of this, and in order to follow a full low-protein diet, it is necessary to purchase much low-protein food from supermarkets. Unfortunately, many of these foods, for example, Violife vegan cheese, fresh fruit and vegetables, are very expensive. The PKU diet is extremely challenging for PKU patients and their families, and the consequences of not adhering to the diet are very serious. Untreated PKU can lead to brain damage, intellectual disabilities, behavioural symptoms and seizures. Ultimately, facilitating PKU patients to adhere to their diet through the provision of a good range of medical and low-protein foods, as well as innovative medicines, will improve their outcomes, resulting in lower overall costs to the health care system, as well as optimising their ability to contribute to society.

As previously mentioned, we have a very limited list of foods available on the long-term illness scheme. Will the Tánaiste outline what is the long-term illness scheme budget for PKU products each year? How is this list managed and why is the variety of foods available in neighbouring countries not available here? Will it ever be a reality to have a PKU patient or their carer involved in the decision making around the foods available to them? PKU patients, who are the end-users, need to be invited to give their input as to the value or otherwise of foods of which they, after all, are the consumers. It needs to be acknowledged that some of the foods available on the long-term illness scheme are of much poorer quality than those available in other European countries, a prime example of this being the quality of breads, a staple of any diet. We should also note that an increased selection of foods of a better quality does not necessarily equate to increased costs.

I thank the Deputy. As we know, PKU is a very debilitating condition and sufferers must have an extremely low-protein diet to avoid the worst effects of the disease. In Ireland we have a very comprehensive screening programme because, as the Deputy rightly said, there is a very high incidence compared with other countries. What is a needed is a lifetime management process for patients who have this condition, known as diet-for-life, which provides continuous individual monitoring and assessment of patients.

Low protein and technical dietary items for PKU patients are available free of charge under the long-term illness scheme. The Deputy asked about the scale of that availability and about the amount of money allocated at present. The most recent data available show that expenditure on these products for one year from December 2015 to November 2016 was approximately €4.8 million. In 2016, an expert group was formed which was drawn from both hospital and community settings. Revised guidelines on the application for reimbursement of clinical nutritional products was looked at and in November last year, the HSE published those guidelines and suppliers were notified that the applications were being accepted. A number of new applications were received and it is hoped this will broaden the list of the products available. In January 2017 the expert group considered the applications that have been received and the applicants will be notified of the outcome in the coming weeks.

When a product is approved for addition to the reimbursement list, it is seamlessly available to a person with PKU under their long-term illness eligibility. The next opportunity for submitting applications for clinical nutritional products to be added to that reimbursement list is expected in June. As for the drug sapropterin, the National Centre for Pharmacoeconomics is currently considering a new application in that regard which it received in February. The HSE will be considering that and deciding on the reimbursement in regard to it.

The Deputy raises an issue which is of concern to a huge number of people throughout the country. Quite an amount of work has been done on this in terms of the recommendations of the working group being examined by the HSE, and decisions are expected in the next period.

I ask the Minister for Social Protection, Deputy Varadkar, to also listen to this part of my question. Domiciliary care allowance is a monthly payment for a child aged under 16 years with a severe disability who requires ongoing care and attention substantially over and above the care and attention usually required by a child of the same age. It is not means tested. Why do some PKU patients receive domiciliary care allowance while others are refused? What are the evidence-based criteria used by the deciding officers? Why do some families have to go through the appeals process? As all PKU sufferers suffer the same condition with the same restraints and consequences, why is there not a blanket domiciliary care payment for all PKU sufferers from birth to 16 years?

Given the increased costs for PKU sufferers over 16 years, it is likely that having received domiciliary care allowance up to this age, they will be in receipt of disability allowance when they apply for it after the age of 16. Obviously, with growth comes increased appetite, which equals increased costs. What are the evidence-based criteria used by the deciding officers in cases of sufferers aged over 16?

The Deputy should conclude.

I will finish shortly, with the permission of the Ceann Comhairle. PKU is a genetically rare disease. The HSE wants to set up a working group to assess the reimbursement of orphan medicines and technologies, for example, Kuvan, which can greatly improve the quality of life for PKU sufferers. Has this group been put into action? The national rare disease plan recommends the setting up of a national budget to fund such treatments so there is no impact on individual hospital budgets, for example, at Temple Street hospital. Has the HSE made any progress to date? I thank the Ceann Comhairle for his indulgence.

Kuvan is the other name for the drug I mentioned earlier. That was first sought in 2009 and it was refused due to lack of evidence of cost effectiveness at that time. As I said, a new application is currently under way.

The Deputy asked about domiciliary care allowance, which is payable in respect of children under 16 years with a severe disability requiring care and attention. As the Government announced last week, there will now be an automatic medical card for those children receiving domiciliary care allowance, which is a very important support to parents in that position.

In response to the question about children with PKU, the approach is that no specific disability automatically qualifies a child for domiciliary care allowance. Eligibility is based on the care needs, not on the disability per se. Of course, the reality with young children and adults who have PKU is that the condition can vary quite substantially. For example, the various food products and interventions can be very successful for one child but not for another.

When seeing the child, the medical assessor has to take all of this into account. It is medical assessors, who are qualified and experienced doctors, who examine the application and the supporting medical evidence and make the decision. The deciding officer reviews the medical evidence and the details of the child's needs as outlined in the application form and assesses the care needs of the child. It is a very individual assessment based on the individual care needs of the child, as opposed to a category like PKU, or indeed any other disability.