Exchanges.
Travelling and Socialising.
Morning Sickness.
The first Scan.

Click here to view the embedded video.

Her exchanges went from 2 to 44 during the pregnancy. She explains that babies receive food and nutrients from their mothers. The baby is connected to the placenta by an umbilical cord. This carries the food and nutrients to the baby. Protein is the key building block needed to support a baby’s growth. This is why Angela’s exchanges went up so rapidly.

Being pregnant with PKU does not have to restrict your life. Despite the need to follow such a strict diet, Angela gives an account of how she travelled to Turkey for her sisters wedding.

Morning sickness was a bit of a pain as it sometimes meant having to take a second batch of protein supplements!

The first scan came as a bit of a shock because she discovered she had twins!!!

To see part 1 of Angela’s blog please click here.

Click here to view the embedded video.

An important aspect of the pre-conception diet was the time she spent with her dietitian: revisiting the importance of weighing food properly and being organised. Angela also talks about how her mental alertness improved once she started the diet.

Some useful ideas (relevant to anyone with PKU):

• Keep a food diary.
• Re-think your food shopping – the need for “free” or low protein foods to fill you up.
• Sign up for a home delivery service.
• Fizzy drinks are you friend (especially Lucozade).
• Look for low protein recipes that you can easily make.
• Buy a digital cooking scales – it’s easier and more accurate.

For clarification the term “ pre-conception diet” refers to the need to go on diet prior to conception.

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

There were times when they would use it as a hold over me, particularly when we were fighting. They would chant “we can eat chocolate but Julie can’t…”. I can still picture them doing it. I would get angry, although I don’t think it was because I couldn’t eat chocolate, it was more because they were picking on me. I have to admit I did try chocolate at an early age but I never liked the taste. They were good to me overall and they always looked out for me, some times too much.

Eating out was fun, we would get some funny looks from people as we brought out the scales to weigh the fries and other stuff. Overall this was rare: we did have a game to come up with the best reason for doing it. Once we convinced this old couple that I was a space cadet from NASA getting ready for my first flight!!

As time went on I did start to rebel, this was mainly in my late teen years. I got a real liking for McDonalds! I think I did suffer though… my grades went down and I put on weight.

Now that I am older I am back on my special diet: I would like to start a family in the near future and its real important for the pregnancy phase.

Well that ends my little blog, I hope it was of some interest to you. Just remember, even though you have PKU you can still live your life.

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!

When Luke had his heal pricked I had no idea that the results would be so devastating (devastating at the time but in hindsight it could have been so much worse). My main message to all of you out there who find out that your child has PKU is, “DON’T WORRY”.  I wish I had someone to speak to at the time, I think it would have made life so much easier. I am not saying that the help we got from the visiting Midwife and Dietitian was no good: you just wanted to speak to someone who had been through it all.

I remember being very depressed and wondering how I would cope. Jeff was strong and he found out a lot about PKU at the various libraries he visited. We were then given a life line, an introduction to a family who had two children with PKU not very far from us. Cheryl was great she reassured me that there are few differences to bring up a child without PKU: her first child did not have the disease.

Gradually I got use to the whole idea myself, and I got into a routine of preparing the formulas: I even got use to the smell! Life continued and little Luke grew bigger and bigger. We got involved in with mother and toddler groups and formed a good relationship with a number of other PKU families: we couldn’t meet that often because we were al spread out. As far as I could see Luke was growing up to be a normal little boy. I on the other hand had to learn to be more organised.

Then not long after Luke’s second birthday I fell pregnant again. It wasn’t planned although Jeff and I always wanted more children. We were a bit apprehensive at first but having had the experience with Luke we knew it wouldn’t be the end of the world if number two was PKU too.

And so it was, Matthew was born on a cold December morning. He seemed healthy enough, but the screening was to show that he had PKU. Did we freak out this time, no we didn’t. In many ways I was much happier that Matthew had PKU because I knew meal times would be easier. Luke was as proud as punch that he now had a little brother.

I thought they would be similar but they were very different in character. Matthew was more demanding, and it was nearly a year before he slept through the night. He seemed to be into everything: very mischievous but he always gave me a great big cheeky smile. He had to learn quickly to stand up for himself when his bigger brother decided that it was his turn to play with a given set of toys. After speaking to mothers whose children did not have PKU it was obvious that he was just like any other child: looking to develop his own character.

After 4 years of having a house full of boys I convinced Jeff that we should go for another, this time I wanted a girl. The question of PKU never even crossed our minds, and as luck would have it, I gave birth to a beautiful bonnie girl just over a year later.

In terms of children, we have had PKU, PKU, PKU. They have all grown healthy and they are doing well at school. They are even keen on sports. It has not been easy, but having spoken to many friends I can say that all the problems we have had are the ones experienced by any family. Remember giving birth to a child with PKU is not the end of the world: they will turn out to be just like any other.

The next big thing, and the most relevant to this blog, is that I have started the pre-conception diet. I had my clinic appointment on 1 October – just four days after landing back in the UK from our wedding and honeymoon. I spent the morning seeing doctors, having blood tests and learning about what the preconception diet involves, then spent the afternoon in the hospital’s metabolic kitchen sampling the latest new products. We decided not to actually start the preconception diet until after the weekend, so I could have time to be adequately prepared.

So, on Monday I started the preconception diet with a baseline blood level of around 1400 (well, the proper blood test said 1400, but the blood spot said 1300). After an initial chat with my doctors and dietician it was decided that I would start the diet on only 8 exchanges per day – a cut of 7 exchanges! This was the bit that worried me the most, because I’ve just spent a month travelling and not following the diet all that much (mostly due to lack of access to products etc).

Well, I’m glad to say that three days in I’m going strong and I feel quite happy about things! It’s no-where near as hard as I expected, which is rather nice, I must say! I spent part of my weekend cooking and freezing foods – I baked raspberry muffins, cooked a ratatouille and a sweet potato curry and baked some low protein bread – and I’ve decided that the key to being successful on such a restricted diet is preparation. I have to say, knowing that doing this properly means we will be able to try for a baby once my levels are below 300 is great motivation, too!

I will admit it’s hard work – for the first time in my life I have a food diary which I am using religiously, I’m carrying scales around with me for the first time since I was a little kid, and I’m weighing and counting every single little thing that goes into my mouth. But, to be honest, the food isn’t bad and I’m not going hungry. I was always convinced that I’d pretty much starve on the pre-conception diet, but there are some great products out there that make adhering to such a strict diet so much easier!

At the moment I’m still waiting on a lot of products to arrive, so hopefully I’ll be able to expand what I eat a bit more soon. On week one, day three, on the pre-conception diet, this is a typical day for me:

Breakfast: 1 weetabix with a carton of Sno Pro, five raspberries and five strawberries. One pouch of Lophlex LQ and a glass of juice.

Snack: Three Loprofin chocolate chip cookies

Lunch: 2 slices of PKU bread with free roasted vegetables and salad OR sweet potato curry with 2 exchanges of flour tortilla (about half of one), a can of Sainsbury’s diet cola and a pouch of Lophlex LQ

Snack: One packet of walkers crisps OR a raspberry muffin

Dinner: A selection of free vegetables with my remaining exchanges used up in potato and a pouch of lophlex LQ.

I also snack on free fruit throughout the day if I get hungry.

So really, it’s not too bad! I have to do my first blood test tomorrow morning, and for the first time in my life I’m actually excited about doing one! I can’t wait to see how much my level has dropped and the only thing that stopped me doing a test today is that I know that if I wait 24 hours I should get an even better result.

I’m not really feeling the benefits of being on such a restricted diet yet – then again, to be fair, we’re not sure if 8 exchanges is going to be right for me! I am feeling very tired and extremely emotional – a bit like a rollercoaster actually – but then again, I always feel like that for two days when I tighten up on my diet.

I will continue to document my experiences of the pre-conception diet and throughout pregnancy, so watch this space…

In Northern Ireland, Nutricia held three Home Delivery Birthday Party Cookery Sessions in Belfast, Omagh and Derry, and one Home Delivery Birthday Party Activity Day in Derry. At the Low Protein Living Cookery Sessions, the Nutricia chefs ran through some practical quick recipe ideas such as Low Protein Bread, Low Protein Cakes, Lasagne, Quick and Easy Pizzas, Bruchetta, Garlic bread and Pasta Salads.

Then for the hands on part of the evening, everyone in the audience got a chance to make up their own dishes to take home with them. At the end of the evening everyone got to taste a selection of all the dishes that were demonstrated. The Low Protein Activity Days were a big hit! All of the younger attendees got to play in the activity centres and then afterwards Nutricia provided a party for all the children. This went down really well as all of the party food was suitable for those following a low protein diet. Nutricia also made up low protein cakes and biscuits that the children got to decorate and take home with them.

We must not under estimate the importance of these Low Protein Cookery days. Many people have lost the art of cooking. We now live in a world that has no little or no time to enjoy the finer points of life. Convenience foods have become the norm. Being PKU means we now need to revisit skills that were an every day affair for our grandparents. Baking is the classic example: there is more than likely to be a higher than average proportion of people baking from the PKU community than any other.

It does bring other benefits too, the sweet smell of dough flowing through the house is a joy and the involvement of the children in then cooking. These Low Protein cooking sessions are empowering children and making them more independent.

Low Protein Cooking

With the help of Mona Taylor, a Food Nutritional Advisor, and feed back from the participants, the sessions are always focused on a particular topic. Last night the subject was “Bread Revisited”. The participants were allowed to build on their experience of baking basic bread (the first ever cooking session). This time Mona showed everyone how to infuse flavour into the bread, how interesting shapes could be created and how to pipe the dough.

Everyone who attends finds the experience a great benefit. It also allows the child to get involved and promotes greater independence.

Earlier this year Barbara also organised a training session for dietitians at the Nick Nairn School of Cookery. Here a team of professional chefs looked at what could be done to make Low Protein foods more interesting. It also provided the dietitians with “hands on” experience in making simple Low Protein recipes.

This year was also the 100th anniversary of the introduction of the concept of Inborn Errors of Metabolism by Sir Archibald Garrod. He presented Inborn Errors of Metabolism to the Royal College of Physicians in 1908. It was over 50 years later before his work was taken seriously.

Nutricia gave the delegates an insight into the New Improved Anamix range. This will provide nutrition from infancy through to adulthood. The new extended Anamix family will also cover conditions such as Tyrosinaemia and MSUD. This will be the most complete range of protein substitutes containing EFAs, to promote healthy growth and maximise potential.

They also presented the new Anamix Junior LQ: the first ready to drink protein substitute containing omega 3 in Berry and Orange flavours.