Every couple of months I go to my gp’s clinic where I have a blood test which is sent to a laboratory where it is tested to see the levels of phenylalanine in my blood. This makes it harder to cheat and have things I’m not allowed as it will show up here. Twice a year I see my doctor and my dietician together. We discuss things like my general health and whether I have any problems. My height, weight, and blood pressure are also measured and we discuss my progress. I really enjoy these meetings as they make me feel like I’m in charge of my own diet instead of my mum doing everything for me.

PKU doesn’t stop me doing things I like, I have been on several holidays, both with my family, and with my school. I take my own food and have no difficulty finding something I can eat and enjoy. Most places where I have stayed have been very helpful when I explain my problem.

I used to get picked on by other children for my condition and had to sit by the teachers at lunch so they could keep an eye on what I was having, this made the other kids think I was a teachers pet who wanted to sit with the teachers all the time. AS IF!!! Also when I was younger, there was a time when I wasn’t invited to a friends birthday party. This was because her mum didn’t fully understand my condition and didn’t want to invite me and end up giving me the wrong thing. Eventually my mother went into school and gave a talk on my condition and its effects where the other parents were invited to come along and listen. Since then I’ve actually made more friends because they know I’m not a teachers pet and just want to be treated like everyone else. When a new girl started at our school who is diabetic, I was the first to make friends with her.

One thing I must always look out for is changes in products, especially drinks. This is because manufacturers are always changing the ingredients, adding some or taking them away. This means they can end up on what I call the “red list”.

I do meet other children with PKU. There are special events organized by the NSPKU where can all get together and just have a good time. I find it exciting meeting others with my problems. It makes me feel like there is so much out there I can do, especially when I meet adults with PKU who have families and jobs and don’t let this one thing hold them back, especially as I will have this condition for ever.

PKU has not stopped me having a normal life. Although at times it is a bit inconvenient, I can still enjoy normal activities and my food, within the limitations of my diet.

As a kid, I was eating mainly carbs and fats and lots of fruit, but very small amounts. I was a tearaway with ADHD so I was burning through energy like no tomorrow. As a result, I was a pretty lean kid and my dieticians expressed concern that while I was a tall child, I was pretty light.

As a result, I was put given Calogen supplements which I took in the form of a milkshake with a beautiful zero-protein fruit ice cream called Nectacream. While the Calogen suppliments had their desired effect (I went from a lean skinny lanky kid to a reasonably solid and well-padded youngster) the company that made Nectacream folded, and that was the end of that. It saddens me that Nectacream doesn’t exist today – I’m sure it would have made a killing as a PKU ice-cream if it had gotten the global exp

osure the internet may have been able to afford it. Rest in cold, creamy peace, my long-lost friend.

Anyway, I was around 73 kilos going into college and struggling with my diet. I was taking my Aminogran supplement less and less because the morning doses made my breath smell horrible and I quickly spun out of the habit. In a bid to teach me some responsibility, my mother made me make it for myself. It backfired and I just stopped.

So I went off diet for a while – approximately five years. During that time I found college extremely difficult, and my life was somewhat turbulent. Needless to say, I made it through college and into university. My first year was typical – wild parties, lots of alcohol and lots of bar food – I developed a ‘healthy’ taste for garlic bread and fries, and spent many a sunny afternoon with my textbooks, beer and snack food. After a fun season during my final year of college, I went back to rugby and played in an Under 85 kilo weight grade. Due to my lifestyle and my regular dinner (pork crackling + mash potato + oven fries) I put on a lot of weight, and soon found myself weighing out of my games. After some problems with my then on-again off-again girlfriend and being forced to watch most of my team’s game from the sideline, I became pretty down. The fact I was not getting anywhere near what my body needed in terms of nutrition was no doubt a contributing factor.

Things culminated when a series of high blood test levels and my regular appointment at our national metabolic clinic saw me faced with the very real possibility that I was doing myself serious damage, and my B12 levels were a cause for concern. I knew I had to take my Amingran more seriously, but I could not get myself into the habit of making it. Thankfully, I was introduced to Easiphen, which our national pharmalogical body was contemplating funding.

In the four years that have followed, I have gone from being disorganised, irresponsible, clueless, incapable and dangerously incompetent with my diet to having a really good handle on it. My extra weight, which was putting me at risk of a series of other problems such as high cholesterol and diabetes is now firmly under control. I have regained a svelter figure through an aggressive low-cal diet with rigid Easiphen intake and have re-registered for the Under 85 team I had to effectively walk away from in 2005. I’ve gone from 103 kilos to around 81kgs, and there’s more to come off. I’m also trying to build some muscle mass, although the low protein diet is proving to be a bit of a stumbling block in this regard. If anyone knows of any good suppliments for this purpose, please message me! I love to cook and while I am getting very good at making breads and pizzas, I also enjoy some treats like popcorn in grapeseed oil (absolutely beautiful!).

I’m proud to have PKU. It’s no longer a stumbling bloc, it’s something that I know know a lot about and have read many things on. Understanding my condition has helped me take care of myself and given me something to be proud of.

I’m sorry for how long this is, but there is a silver lining to going back on the diet. If you’re on it now as a teen, for the love of all things awesome, stay on it! Learn to cook for yourself using things like LoProfin and discover the small wonderful things about your condition that other people don’t get to experience. Most other people will rarely smell bread fresh out of an oven – PKU lets you do that all the time! You can also make scones, pizzas and all sorts of wonderful goodies with relative impunity! I love the taste of my baking, I look forward to mucking around with recipes and it’s pretty much the most rewarding skill I’ve ever learned. And there’s nothing wrong with learning to cook – I’m a 22 year old rugby-playing New Zealand university student, and I consider myself to be every bit as rugged and articulate as the next Tom, Dick and Harry.

While I was lucky to have a seemingly mild case of PKU and to not have done too much damage during my more care-free years, I can assure you that life with PKU is far more enlightening and interesting – and ultimately more rewarding – if you have tale the time to understand the science behind your condition, how it works, what you can eat and how it can affect how you live your life.

Best Wishes

Dan

James doesn’t have food allergies, though. He suffers from PKU, which drastically limits his diet for the rest of his life. PKU means James’ body can’t metabolize phenylalanine, one of the building blocks of protein.

A highly restricted diet keeps him healthy and experts hope dramatic improvements in treatments could become a reality in his lifetime.

James loves football and bowling. He has the trophies and medals to prove it. In practically all respects, he’s a healthy, happy little boy. His strength is an inspiration to me and I’m his mum!!

His favorite food is burgers and chips and nuggets, special meatless versions of course. In all his six years, James has never eaten meat. And every bite of food he eats is extremely low in protein. Every bite is carefully weighed and measured.

I am very stringent, I know I am but you don’t cheat; you really have to stick to the diet every day to maintain his blood levels and healthy functioning all around.

Many of his friends think he has food allergies. Many ask why he needs to weigh everything he eats.

He tells them we weigh them because he’s on a special diet called PKU. ‘What’s PKU?’” is the standard response to this.

Unchecked, PKU could cause severe mental retardation and seizures. A special formula provides all the protein he needs without the one he doesn’t. Of course, this is a bit hard for a six-year-old to explain to other kids!!

There are always events and fundraising functions for people like James, so please get involved if you get the chance and who knows what the future holds!

James isn’t too concerned about the future though. He’s content with the present.

I met Jill on a night out, and it was about a week or two before PKU was a topic of conversation. I remember Jill came round one day for a BBQ and to meet the parents (there wasn’t any Ben Stiller style comic shenanigans…unfortunately). After the initial meet ‘n’ greet, we moved through to the garden where the Lord of the BBQ (my dad) got to work. The chat was moving along until the inevitable question came, “What would you like Jill? Hamburger? Sausage? Chicken? Ribs?” Jill replied and started to explain about phenylketonuria and that she would not be able to have any of the food my dad had lovingly prepared. My mum, ever the parent stepped in and offered a breadroll to make a chip butty only to be told that the bread had protein in too so she’d just stick to a jacket potato and salad. I think my dad thought she was on one of these new age diets and even said “you need some meat on your bones” slightly missing the point (bless!)

I remember thinking, “what can you eat?” and being completely puzzled by the fact that in the 25 years of my life I had never heard of this fenny-kettle-yourear thingy. I spent hours that night looking on the internet but the first problem I discovered was that I did not know how to spell it!! Google is a great tool when you know what you’re looking for, less so when you don’t! Typical! The next time I saw Jill I told her about my attempt to find out more information and my (frankly, schoolboy) spelling, which she found highly entertaining. Jill said it is often just abbreviated to PKU, which I was fairly confident I could spell!!

Back to the internet, PKU spelt correctly. Once I’d realized that it didn’t stand for PeKing University, I found quite a lot of information about this mystery condition (or at least a mystery to me!)The first time I cooked with PKU foods, I thought I would be a mans man and make a manly batch of….fairy cakes, as Jill could not go to the shops and just buy them like I do (not all the time, but they are a nice treat!) Jill’s mum gave me the prescription ingredients, (Low protein flour, egg replacer) and lent me some low protein cookery books. Making the mixture I felt quite confident, thinking “this is not too bad”, “looks like normal cake mixture” and I had a taste out of curiosity! It tasted like normal cake mixture! I put the mixture into little fairy cases and put them into the oven for 15 mins. After 15 mins they looked really pale so not knowing much about what they should look like, I put them back into the oven to brown. I removed them from the oven not long after, and they had turned brown, but were as solid as rocks! I had completely burnt them! I cut the top off of them and inside did not seem too bad as they looked spongy but slightly lighter in colour to what I was use to. I was so determined to make them so I had another go. This time I removed them from the oven when they were still pale but they were firm to touch. This attempt was much better, they tasted not dissimilar to the cakes I normally have. The moment of truth was when Jill tasted one, she was very impressed and ate the lot! Gordon Ramsey watch out! She was only off work sick for a week too (only joking!)

Three years on our relationship was going strong and we decided to purchase a house, so after traipsing through many properties we managed to purchase one. This was a voyage of discovery in many ways as it would be the first time we had lived together and it would be our first attempt of cooking meals together. This was the first time I really worried about Jill and PKU as I had no idea on cooking main meals for myself let alone a PKU diet. PKU cakes and biscuits were fine but a main meal? What would I cook? I did not understand the exchange system or how to calculate protein in food: it was a minefield! Jill was really reassuring and taught me lots though

The best thing though is that Jill is actually quite a talented chef herself, not to my levels you must understand, my beans on toast have a Michelin star, but she’s more than happy to cook up a meal when she gets home as it’s usually a good two hours before me. She’ll do 2 versions, one for herself and protein-i-fied one for me. Usually this means doing a veggie sauce and frying a bit of chicken for me to add to it. I ain’t complaining. Beggars can’t be choosers

I expect my experience will be familiar to some of you and for people that have just become familiar with PKU it will become a second nature to check ingredients. Some of my ex-girlfriends used to count the calories on packaging this one just counts the protein!

in 2001 my mum passed away, my great support towards the end of 2007, i was getting angry till one day i grew so angry that i could not control it at all. It reminded me of the days when my count was to high and it was a wake up call. so i found a PKU clinic in Perth western Australia saw the the doc and he said i was back on the diet for life and this blog is of me learning and training my self back on to the diet.

Record

You should work to keep track of how much Phe you consume every day. A registered dietitian should be consulted and can give you a daily number of exchanges (In the UK, one exchange = 50mg of Phe, but this varies from country to country. In the US, one exchange = 15mg of Phe and in Germany they count every mg!! Now that’s dedication!!). This number gives you your Phe tolerance. Everything you eat in a day should be counted toward the number of allowable exchanges. So the best thing I’ve found is to keep a food diary of everything I’ve eaten so I know how much Phe I’ve consumed.

Restrict

I soon realised that there are so many foods that have too much Phe to be included in my diet, including meat, eggs, and dairy. Because of their high Phe content, they must be avoided entirely. There are a variety of substitute foods, the recipe section on this site has quite a lot of good ideas for cooking when on the low-pro diet:

http://blog.nutricia.com/lowproteinliving/recipes/

Replace

I was told that protein is an essential part of my diet, even though too much can harm me, I must consume some protein. Therefore, an absolutely essential part of my PKU diet are special low-Phe or Phe-free protein supplements. I’ve found the effects, convenience, and most importantly, taste, vary wildly from product to product and company to company but I’ve found a diet I’m happy with and adherence isn’t thie issue it was only 5 years ago, never mind ten years ago.

So that’s my little tutorial, back to my day job (unfortunatley) and good luck to anyone out there on following and the diet..and just remeber your 3 R’s!!

My name is Kate. I’m 25 and have classical PKU. I’m Australian, and grew up on the edge of the outback in Queensland, but I live in Newbury near Reading in England with my fiancé and our two puppies. I’m a bit of a Jill of all trades – my day job is PR, but I am also the managing director of a company that provides translation and interpreting services, which I run with two friends.

I guess in a way I see the opportunity to write this blog a bit like therapy, as well as a way to help others avoid the mistakes I have made and regret. In recent years I’ve really struggled with my diet, and I think to be able to write things down and vent my feelings will be really helpful, especially as I plunge headlong into starting a family.

As a child, I never really thought about my diet. It was just a part of me, and who I was as a person. I was never ashamed of it and I didn’t hide it. I was never teased and my parents made sure I knew what I could and couldn’t eat. I had a fairly normal, and as an only child, perhaps even a privileged upbringing – I attended a good school, travelled overseas, rode horses and I had lots of friends. It wasn’t until I was about 16 that things really started to go downhill, and by the time I was 18, I was pretty much completely off diet.

My PKU demise started with formula. The necessity to drink my formula has always been the bane of my life. I could handle PKU if it weren’t for that. As a child I would kick and scream when it came to formula time, and my mother and father on several occasions had to hold me down, hold my nose and pour it down my throat. I feel really sorry for them now, and I feel bad for having behaved like that – I realise now that I must have put them through emotional hell and back. The problem is, I think I have developed a lifelong aversion to formula. As a child, I was on Aminogran and it was truly foul stuff. Even now, I can’t say ‘that word’ – the A word – without involuntarily gagging and pulling a truly ugly face. When I took it I spent most of my day feeling bloated and nauseous, only to then have to take it again just as I was feeling better. Sometimes it would even make me throw up.

When I was in my teens my doctor told me about Maxamum XP. It was still foul stuff but I could get it down and didn’t have the usual four hour long bout of nausea after it. Still, I hated it, and after a while I started pouring it down the sink when mum and dad weren’t looking. Then, when I was 16, I went to Japan for 6 weeks on a school trip. I think in the whole six weeks I tried to take my formula once, and ended up throwing out the tins and telling mum I’d taken it all. It wasn’t until I was 17, however, that I really started to resent my diet. I had just finished high school and went away for a week with some friends to celebrate. When we all went food shopping some of the others started picking on me and my diet, and complaining when I picked up items I needed. I ended up paying for part of the shopping, but not actually having anything much I could eat. This led to a huge argument where one girl said to me that she didn’t believe there was anything wrong with me and that my diet was “attention seeking bull(fill in the blanks)”. I was devastated, and I couldn’t wait to go home. This was closely followed by several bad experiences in restaurants, including one waiter in Brisbane who literally yelled at me and reduced me to tears because I asked him for something that wasn’t a dish on the menu.

I didn’t realise it at the time, but from that point onwards, my diet got steadily worse. I refused to touch formula, take blood tests or go to clinic. I started eating whatever I wanted, and tried all manner of things that I know I shouldn’t have. I deeply regret this now, and if I could turn back time I would have done things so differently. Now I have a taste for things that I know I can’t have but crave desperately most days. I have tried everything I decided I was missing out on, and unfortunately for me I liked the vast majority of it. Thankfully I also didn’t like a lot of stuff – I discovered that beef is disgusting, I love the smell of smoked mackerel but not the taste, and I hate McDonald’s burgers. However, there’s lots of other really bad stuff I do like too, and this is a battle with myself that I fear I will now have to live with for the rest of my life.

I made a couple of feeble attempts to return to the diet over the years, but nothing ever really stuck. The level of medical guidance available to PKU adults in Australia is truly appalling, and I never felt that I was properly equipped to deal with my condition as an adult. I had to travel 8 hours to see a doctor, who I don’t think actually specialised in PKU. I had to wait weeks on end to get blood test results back, and I had limited contact with a dietician. They changed the way I counted protein from the units system I had used my whole life, and I felt confused and bewildered by it all. They also encouraged me to use more low protein products, but the range in Australia is somewhat limited in comparison to what’s on offer in the UK, and I didn’t like most of it, or I couldn’t get good result with it when baking.

So, I slid off diet again. It wasn’t until I came to England for what was meant to be a working holiday in 2006 (at the age of 22) that I decided it was time to get on track again. I found the medical system here pretty tough to get my head around at first, but I eventually got a referral to a PKU clinic for adults in London. I went and met with the team there and I broke down in tears with relief at how kind and understanding they were – I had been absolutely terrified of going back to a PKU clinic. They organised for me to spend a day in the metabolic kitchen where one of the dieticians let me sample all of the low protein products, gave me loads of information and recipes and taught me how to cook some basic recipes. I left for home that afternoon with two lasagnes I had made, two gorgeous loaves of bread and a great big smile!

Since then I’ve been up and down, but I’m trying hard to stick to my diet. Not long after I went back to the PKU clinic I met my soon to be husband, Ed. I was worried about telling him about my PKU, but I had no reason to be. He was very understanding and early on in our relationship took it upon himself to learn about PKU so he could help me. On 9 June 2007 he took me to Greece and proposed. We are to be married in Australia on September 7 this year (not long to go now!), and soon after we hope to try for a baby. I’m booked in for a clinic appointment in the first week of October, at which time I will be put on the preconception diet to get my levels down for pregnancy. Ed is being amazingly supportive about the whole thing – he is my rock, especially on my bad days when I find it tough going. He motivates me to keep going, encourages me and gives me the kick up the behind I sometimes need when I start to let things slide. No matter what, I know he’s there for me every step of the way. He’s even learning to cook PKU friendly meals, and we’re planning to spend another day in the metabolic kitchen, together this time, at the hospital in London so he can learn about the different products, how to cook them and how to count exchanges and manage the diet so we can work together through our journey that is to come.

Even when I was at home and bad on my diet, I was always trying to do things for the greater PKU good. I helped out on a few government lobbies to get financial help for people with PKU in Australia, wrote press releases, did lots of fundraising and supervised camps for kids with PKU. I’m hoping that I’ll be able to get that involved in the PKU community here in England too!

So that’s my PKU journey to date! Every day is still an uphill battle, but I WILL do this, because I know that if I don’t it will affect my whole life and I’ll only have myself to blame.