Hi Patrick – I don't have PKU but my (almost ) 3 year old daughter does. We live in the UK and go to the Evelina Children's Hospital (part of Guys and St Thomas' in London).
Every time I go to clinic I ask our consultant (Dr Champion) abut Kuvan. He is less negatvie than your dietician, and last time we visited he gave us the following information:
1. He, and various other professionals, would like every PKU patient in the UK (classical or mild) to be able to try Kuvan to see if they respond. He concedes that some patients with classical PKU (as my daughter has) have responded to Kuvan. 2. However, because PKU affects so few people in the UK, although the cost of Kuvan per patient is very expensive the total cost over the whole population is marginal. This means the issue is too small for NICE (the National Institute of Clinical Evidence) to consider. I believe our doctor and other metabolic professionals are going to another institution (I can't remember what they are called) with their proposal, though I have no idea of timescales. 3. If this team of healthcare professional is successful, and every PKU patient is allowed a Kuvan trial, then only those who respond significantly will be offered the treatment on the NHS, in our consultant's opinion. He did not define what significant means. 4. In the UK only one person has been given Kuvan so far – a lady who was pregnant and not on diet, so they decided it would be better for the health of her baby for her to have Kuvan than nothing. 5. One other person in the UK has self-funded and gets Kuvan from the states. Sadly not an option for us unless we win the Euro millions!
It annoys me that in the UK we seem to be told that if the levels are low on diet then no other (possibly better) treatments will be made available. We all know the diet is not a perfect solution, and I would like to see the doctors and dieticians try the low protein diet, or try giving it to their children. I get very frustrated with this attitude and I hope you get further with your dietician! Which hospital do you go to? I have to say ours is very good but it is always us who ask about new treatments. Last time we went we were simply told by a registrar but the diet is the only treatment.. at which point I demanded to see the consultant!