Catherine Noone (Fine Gael) | Oireachtas source

I thank the Minister of State, Deputy Catherine Byrne, for coming to the House on behalf of the Minister, Deputy Harris. I will be very happy to take her response and it is perfectly adequate to receive a response from the Department via the Minister of State.

The matter concerns the PKU issue and the drug Kuvan, on which there was a presentation in the AV room this morning. PKU is a rare blood disease that affects 13 or 14 babies per year in Ireland. It is known to be more prevalent in Ireland than in other countries - it is a Celtic gene, or so it was described this morning in the briefing I attended. The issue is that there is no known cure for this disease. It is diagnosed by a heel prick test and the Irish Government was revolutionary in introducing the heel prick test in the 1960s. As a young lad who suffers from PKU said this morning, we were really ahead of the game back at that time in dealing with PKU but, sadly, since then those affected by PKU have simply been advised to follow a strict no-protein diet. While that gives patients a certain amount of help and relief, if the levels of protein in their blood become too high, it affects them mentally and causes brain damage. We were shown videos this morning of young people who were behaving in a very distressed manner due to brain damage from high levels of protein in their blood.

The quality of life of patients and their families is a huge issue. PKU requires a highly regimented diet and lifestyle. The mother of the child who was at the presentation this morning described how they have to use a powder they buy in the pharmacy to make bread or an omelette. They colour it so the child knows it looks like bread, although the child has never tasted bread, but everything still tastes more or less the same. It is quite difficult for families to live with. The mother described how a sibling was very unhappy eating around the younger child because of the nice smells and so on. It is a difficult dynamic for a family to deal with.

Many Western countries have adopted the drug Kuvan, which has been described as a wonder drug. My question to the Minister of State is why Ireland has not decided that we should keep up with our intentions in the 1960s in being one of the forerunners on this. Kuvan received EU regulatory approval in 2009 and is currently approved for use and reimbursed in Austria, Belgium, Bulgaria, the Czech Republic, Estonia, France, Germany, Hungary, Italy, the Netherlands, Luxembourg, Norway, Portugal, Romania, Russia, the UK, areas of Sweden, Lithuania and Denmark. The Irish PKU community cannot see any justification for the drug not being available here.

While I know everything comes down to budgets, the harsh reality is that even when the gruelling PKU diet is maintained, people managing the condition still suffer with long-term permanent health conditions such as neurocognitive defects, neurological side effects, growth defects, bone mass reduction and obesity. This is very serious. I hope the Minister can find the budget to help the individuals with this disease. The eldest of the people who were diagnosed with this disease in Ireland are only in their 60s, so it is relatively new in terms of science and the medical area. I am very interested to hear the Minister's response.