I just got back from my clinic visit in Detroit and they are going to start me on Kuvan to see if I respond. Anybody have any input?
I went on the kuvan and it did not work on me and the drug you have take a lof of pills that one bad thing about it and some people works on some it does not.
I've been on Kuvan for 2 1/2 years now. I take ten pills a day, which is not that bad, because the pills are relatively small. Keep your diet consistent while taking Kuvan, because that is how your doctor will know if you are a responder or not. They look to see if your phe levels drop even though you're eating the same amount of phe.
Like Breanna, I have been on Kuvan for 2 and a half years as well. I take 9 pills a day. Since taking the Kuvan, I feel much better overall. Best of luck and keep us posted!
My daughter (9) went on Kuvan in August. It was a bit of a roller coaster ride as her levels went up and down and we tried to determine whether or not she was a responder and if so by how much. It was a lot of work – meticulous diet records and frequent blood draws for a few months, but it was well worth the effort. Her phe allowance did not increase tremendously (280 to 420) but every bit counts and it allows her to eat more of the foods she likes. We do not regret our decision at all.
I'm on Kuvan, and have been for about 2 years.
My levels before Kuvan were around 11-12 while eating 30 grams of natural protein (protein from food) and supplementing with 3 servings, 30 grams of protein equivalent of formula.
On Kuvan, my levels are around 4 while eating 45 grams of natural protein (protein from food) and supplementing with 1.5 servings, 15 grams of protein equivalent of formula.
I do have to take a number of pills, and as dosage is based on weight, more than the others posting here – I take 19 pills a day. I find it not a major problem for me at all.
I am from the UK with Classical PKU. I have recently approached my dietician to ask if I could take Kuvan. The first reaction I got was Kuvan was for a very different type of pku- milder form. So this has continued with an argument against using the drug.
I have also been told by my dietician that there are serious side effects and evidence to support using the drug is very limited. From what I've heard which has been positive, I should at least have a go to see if I respond and seems a relatively safe drug.
I hope to hear views from anybody who has tried Kuvan or having difficulties getting to use Kuvan. Would be good to hear from anyone with Classical PKU who has tried Kuvan and has lowered their phe levels.
Although I can't offer much information from a scientific standpoint, I can tell you that I have been on Kuvan for 2 1/2 years now, and my experience has been nothing but positive. When I began Kuvan I was also told that the chances that I would respond were slim. However, my dietitian was more than happy to give me a chance to try Kuvan, because she knew we would not know for sure until after I gave it a shot. As it turned out I responded amazingly, and I have not regretted my decision.
Did your dietitian mention what serious side effects she has heard of? As far as I am concerned I have not experienced any side effects due to Kuvan.
Check out http://www.kuvan.com for more information.
Breanna 18 CPKU
Thanks for your reply Breanna. I have only had 1 conversation with her on the phone about this and have received 2 emails.
In her first email she told me that since it is a milder form of pku and very different in terms of body chemistry she wouldn't allow me to try it. She also mentioned since I have good levels anyway, she couldn't understand why I feel the need to have it. I disagree with this.
There seems to be some sort of underlying issue here maybe the cost I don't know but she came across as not knowing much about it. It was me who also approached her in the first instance. I am still pursuing this and hope I get to try it.
In the email she just sent me she told me that the evidence to support use is very limited and therefore hard to draw conclusions from. Also she said that in 1/3 of patients using it have reported serious side effects but has'nt told me what they are. The only side effects I have heard about is minor ones like headache nausea which passes quickly as long as tabs are taken with some kind of food. It has been very general and no way at all specific. I do feel I need some solid evidence as to why I shouldn't as I have been listening to lots of talks on patient power series on pku which has been very positive. I don't understand completely at the moment why I can't try it.
Have you Classical PKU? Your dietician seems very helpful and I expect quite knowledgeable.
What do you think I should do? Can I have your email address please so can discuss this further?
Yes I do have classical PKU, and if you would like to email me it's email@example.com. Keep discussing this with your dietitian, because if there really is a solid reason she doesn't want you on Kuvan then she should be able to give you specific information.
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!
Thanks very much. I will make a note of it. I will keep trying with my dietician.
My daughter has been taking Kuvan for 2 months now and is reponding well. She take 3 pills disolved in 4oz of apple juice and doesn't have any complaints or side effects. I hope it works for you she seems much happier and energetic then before.