What Would You Do with More Protein?

My Unexpected Questions After Starting the Sapropterin Trial

Starting the Sapropterin (BH4) trial has brought up some questions and challenges that I hadn’t expected. I embarked on this journey with a low expectation, as I am borderline classic PKU, so I knew my chances were slimmer than others.

My levels hadn’t been below 1000 umol (micromoles per litre is the unit used to measure Phenylalanine (Phe), in the blood) for years now. I had previously been content with being consistent, despite my levels being much too high. The first stage of the trial is to get a baseline. This is done by getting your Phe readings at a stable level, it is then used as a benchmark to evaluate the results of Sapropterin. I began my baseline by focusing on getting my levels down, to 600 umol, (the optimal window being 400 – 600 umol).

Starting on the strictest diet I had done for many years, my levels very quickly dropped to 600, and then further still into the low 500s. Having managed to drop them by over 600 umol the change in how I was feeling was incredible. I had switched from my standard, more negative mindset, into one of pure positivity. I can’t remember when I last felt this alert, or alive. I was feeling so hungry, not for food, but for life.

If I ever contemplated having more protein available in a day, my mind goes straight to being able to eat high protein foods. I don’t know if this is because I spent so much time off the diet in the past, or if this is just the natural response to the idea of having more exchanges (grams of protein). In reality though, this thought isn’t very realistic, because even if I doubled my daily intake of protein, having PKU would still mean high protein foods would be off the table! This left me pondering as to what I would be able to do with my extra allowance, if indeed I were to be a responder to the treatment?

If you told me 10 years ago how challenging this question would’ve been, If I was ever fortunate enough to need to answer it, I would never have believed you.

When I opened that first message on the MyChart App and saw my results and my levels had hit 325 umol, and only after my first week of taking Sapropterin, I was speechless; I had to pinch myself. Reading the message that followed the result to see my exchanges had been increased by 2, was nothing short of joyful! I was amazed at how quick this had all happened.

My choice on how to use the extra 2 exchanges for the first time was easy, a pint to celebrate! It was then to my surprise, I was finding myself with spare exchanges at the end of the day. Initially, I used these to have a coffee and a couple of biscuits in the evening. I was finding this challenging because I didn’t want to have to increase my portion sizes, but I was struggling to find new things that would naturally take my newfound increase in protein, without having to eat more food.

As I have talked about before, I have struggled previously with an overeating disorder, which has been under control for a while now. Starting this trial had given me the bonus of losing two and a half stone in the three months since strictly returning to the diet for the baseline. I am very eager to not go backwards and put it all back on again.

My amazing wife Donna suggested I make a list of the amber protein foods from the PKU traffic light system (rather than spending my time ogling all the red ones!). And it made total sense to me that those should be my initial focus of investigating new options. I started this straight away by introducing peas and sweetcorn into my meals. A simple and easy fix for the extra 2 exchanges, and far healthier than the biscuits I was consuming in the evenings.

Within a week I was being increased again, by another 2 exchanges. This allowed me to introduce a small amount of bread into my diet, after discovering a whole French stick from Asda is 20 exchanges. Armed with a bread knife and several sandwich bags I equally divided it up into 4 pieces. Putting 3 in the freezer, I then loaded the remaining one up with Violife cheese, salad and a drizzle of sweet chilli sauce for lunch that day. That was quite the treat. I also managed a single slice of bread toasted one day that week. The thought of maybe not needing to bake loaves of bread every week, seemed like a lottery win on the horizon. It was hard not to start getting excited now!

My focus has really changed from wanting/being able to eat high protein foods (which would obviously be a dream fulfilled), to making the diet less work and less reliant on prescription foods.

I’m now totally focused on how Sapropterin can make my life easier. Being able to cook and eat more in tune with the rest of my family would be life changing and so much simpler. What could be better than removing the need to cook different meals, stopping the struggle to make food to take out and removing the need to batch cook in the same way, if at all.

Three weeks after I started the trial and I am now on 6 exchanges more than before I started this trial. This has opened the door further; If I am blessed enough that my exchanges increase further, I will start looking at the possibility of adding rice into my diet.

Despite my excitement over responding, I am fully aware that some of our community members will not be responders and I can’t imagine how disappointing that must be. But please, if this is you there is still hope for the future. There are more treatments in the pipeline, with better results than Sapropterin and with much higher response rates.

If you would like to share your experiences, whether relevant to Sapropterin or not, please leave a comment below. I always encourage you to share your stories with me. Your stories motivate me to keep writing this blog and pushing to raise awareness, wherever you live in this world of ours.

Take care of yourself.

Dan

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Life Behind The Blog

My Sapropterin Trial

I have been quiet for a while now, family life has been busy and taken precedence over everything. But it’s great to back behind the keyboard again, doing what I love.

After a sustained period of struggling with the diet, I found myself contemplating once again, how on earth I was going to get myself on a version of the diet, allowing me to start the Sapropterin trial.

The pressure from the thought of preparing for the trial had been huge. I had been on a relaxed form of the diet for a long while and I was a long way from the 13 daily exchanges I should have been adhering to.

During June, I made the decision to just go for it. There was never going to be a perfect time to start, and it didn’t make sense to keep putting it off. So, I emailed my support team at Guys and St. Thomas and by July I was doing 2 weekly blood spots to form my baseline. My Phenylalanine levels started at just over 1000 umol/L and by the end of July they were at just above 400.

During the first week of August, I received my first dosages of Sapropterin through the post along with instructions for starting.

Day 1

19 tablets, crushed into water.

Day 2

Today I had the most horrendous wind. I kid you not, they were worthy of the Guiness Book of Records. I literally felt like I’d been pumped full of air and had to keep sneaking off places to decompress!

I had to take my daughter out in the afternoon, and I found myself making a swift dash for the Gents in Tesco. This is where I made some guy’s day when I let out a fart that had the confidence of a man who had nothing left to lose. Turns out decompressing in public is quite the conversation starter. At least the amused guy in the next cubicle thought so! After we engaged in conversation which included ‘what the hell have you eaten?’, the guy left the rest room still laughing hysterically.

Day 3

After a recommendation from another PKU community member, I made my Sapropterin into a banana and strawberry smoothie. The result was so much better (and no more wind today!).

Day 4

I had the most horrific headache. I don’t know whether this was anything to do with Sapropterin or not, but it was a real deep headache which stayed with me for almost the entire day, rendering me completely useless.

Day 5-6

Everything good (no wind and no headaches).

Day 7

First blood results came back, mid and low 300 umol/L

Discovering I was a responder was beyond exciting. I had been trying so hard to not think about it working, I wanted to be prepared, given the chance of it working was slim, as I am borderline Classic PKU.

Still here I am, 21 days into my trial and my exchanges have been increased to 17 already.

I had a slight set back last week, when I accidently had a drink with Aspartame in. It blew my levels to over 500 umol. Because I am back to 2 weekly blood spots, I am having a painful wait for them to recover back to below 400, so I can hopefully get my next increase.

That’s it for now. I hope you are all keeping well and managing to keep on track yourself. Please get in touch if you want to chat or share anything, I’d love to hear from you.

Stay PKU Strong.

When Restriction Turns Into Excess

What happens when you spend your whole life being told “no” — and then one day, no one is there to say it anymore?

For a long time, I thought my story was about coming off a medical diet.
Looking back now, it feels like something else entirely.

It feels like I didn’t just step away from restriction.
I ran straight into excess.

Growing up, my parents were always preparing me for the moment I would take control of my diet. Everything was structured, measured, controlled. Every portion had a limit. Every choice had a consequence.

But while all of that was happening around me, I had something else in my head entirely.

Freedom.

Not careful, managed freedom — just… freedom.

So when that moment came, I didn’t gradually adjust. I didn’t ease into responsibility.

I let go.

At first, it looked small.

A full bag of chips instead of a weighed portion.
A second helping. Then a third.

But it didn’t feel small.

It felt like I was finally getting something I’d been denied for years.

And once that door opened, I didn’t just walk through it — I kept going.

I started going to every chip shop I could find, comparing portions, chasing the biggest one. I would cycle 40 minutes just to get it. Sometimes I walked.

At the time, it felt like dedication.

Now it feels more like something else.

Like I was trying to prove that no one could limit me anymore.

When you grow up with restriction, you don’t necessarily learn moderation.

You learn longing.

You learn how to wait.
You learn how to want.
But you don’t always learn how to stop.

Over time, the food changed, but the pattern didn’t.

Chips turned into battered sausages.
Then pizzas — breakfast, lunch and dinner.
Then pub meals, five times a week.
Then anything and everything I had never been allowed to fully experience.

Every new food felt like a discovery.
Every meal felt like a reward.

And underneath that, there was a constant feeling I didn’t recognise at the time:

If I don’t have it now, I might not get it again.

I wasn’t just eating because I was hungry.

I was eating for comfort.
For happiness.
For satisfaction.

But more than that, I think I was eating because I didn’t know how else to feel those things.

Eventually, it stopped feeling like freedom.

It became routine. Then dependency.

I would plan what I called “pig outs” just to get through the day.
Sometimes I would call in sick to work so I could eat and then sleep it off.

Looking back, that wasn’t indulgence.

That was numbing.

Eat. Sleep. Reset. Repeat.

There’s a point where excess stops feeling like a choice.

And starts to feel like something you’re stuck inside.

Over the years, it caught up with me.

Financially. Physically. Mentally.

I gained weight.
I lost direction.
I drifted into unhealthy patterns — not just with food, but across my life.

What had once felt like reward started to look a lot more like self-destruction.

And the hardest part is — on some level, I knew.

But knowing doesn’t always stop you.

It took years before I even considered going back.

Because going back didn’t just mean changing how I ate.

It meant giving up the one thing that had come to represent freedom.

Even if that freedom was hurting me.

When I eventually tried to return to structure, it wasn’t simple.

By then, my habits weren’t just habits.
They were coping mechanisms.

And trying to remove them without support, without understanding what they had been doing for me… was never going to work long term.

Looking back now, I don’t just see a story about diet.

I see a story about what happens when restriction and freedom exist without anything in between.

No transition.
No tools.
No understanding of how to handle either.

Just “no” for years… followed by “yes” to everything.

And that’s the part I think we don’t talk about enough.

Not just in PKU, but anywhere people grow up under strict control — whether that’s medical, social, or personal.

If you only ever learn restriction, freedom can feel overwhelming.

And sometimes, it doesn’t look like freedom at all.

Sometimes it looks like excess.
Sometimes it looks like losing control.
Sometimes it looks like self-destruction.

For me, that lifelong restriction came from PKU — a condition that meant controlling my diet from childhood, every day, without exception.

But the pattern?

That feels much bigger than that.

I write about life with PKU, but also about the wider themes it touches — restriction, freedom, food, and identity. If you’d like to understand more about PKU itself, you can start here:

What Is PKU?

PKU Life – Family Dynamics

The dynamics of a family vary hugely from one household to the next. I remember as a child thinking that every family in the world would be a carbon copy of mine. I assumed every child had two loving parents, four loving grandparents, and a generous helping of aunts and uncles who were fun. Not to mention a gaggle of cousins whose ages ranged from younger than myself up and into their late teens.

Although my PKU diet made me different, the family around me was ‘completely normal’. Through my eyes as a child, I truly believed that every family would be set up the same. I mean, I knew they came in different sizes. My parents had six siblings between them and when I was born, I already had fourteen cousins and another five followed my brother (don’t even get me started on the second cousins. I wouldn’t know where to begin!). My family was bigger than anyone else’s I knew growing up.

As that young and naive child, I never thought for a minute any of my school friend’s lives were any different from mine. I mean we all went to school during the week. So, I presumed they all went home to loving parents who cared for them and kept them safe. And like me, they saw their grandparents every weekend, ate a roast dinner every Sunday, and had a set bedtime that would get increased around the time of your birthday, if you’d been good! How wonderful is the innocence of a young child?!

It wasn’t until I was a young adult and started spending more time around other people’s families, that I realised this wasn’t the case. It came as a real surprise to learn that the dynamics of different families differed wildly.

Lying in bed one night, I started thinking about the dynamics of my own unique family. I have been feeling challenged recently, by how circumstances have shaped it. My family has been through a difficult and prolonged season. The three of us had been forced to move a long way from my wifes family to escape their negative and poisonous influences. We just needed to get distance and start afresh. As I lay in thought and reflection, it dawned on me how the actions of others have influenced how my family looks today. Both positively and negatively.

As I look at the dynamics of the family I grew up in and the family I have built with Dee, my wife, I am amazed at how massively different it is in comparison. I never dreamt that would be the case! I never stopped to consider how many factors are involved in creating your family unit.

I always expected to create a family like the one I grew up in. However, I have learned that every family is unique. Like us as individual people, it has its own exclusive DNA. No one person is the same. It makes sense that no one group of people can be either!

My journey with PKU is unique, as is anyone else’s PKU journey. Everyone living with PKU in the world also has their own story. There are several of us, me included, who are fortunate enough to be able to share our stories with the world. But there are so many more who don’t have the same opportunity.

I am becoming increasingly interested in the PKU stories I hear from everywhere around the world. Especially the accounts of those who don’t have a voice. It’s important that those who are lucky enough to be treated for PKU, don’t spend all their energy looking for better treatments. We can never lose sight of how much better off we are compared to so many of our PKU brothers and sisters around the world. Our lives can still be dramatically improved by the advancement of medical research into treatments and chasing cures. But we must recognise that too many don’t have access to the required treatments needed to manage a diet at its most basic level.

It is easy to take daily supplements for granted. I know I have in the past. I have been taking them for over 40 years now and I know I have complained many times about the flavour or how bad the experience has been at times in my life. All this is true, but I haven’t stopped once to consider our fellow PKUers out there who are unseen and unheard who would do anything to get their hands on a regular supply of Lophlex or Sphere. They would tear your arm off for access to Aminogram and Calogen (for those of you old enough to remember.). They shouldn’t be left behind in our search for better tasting treatment, and, an even better life! Life is short and medical progress can take decades. For me, everyone with PKU deserves the same chance to manage their PKU and have a fair shot at life.

We need to shout for all the PKUers in the world who can’t! Because we have been given a chance they haven’t, to be independent and have the opportunity to thrive.

Using the findings of a study I found in the National Library of Medicine, at the time of writing this, there were approximately 337,183 people in the world with PKU. During the one hour I have been sitting here working on my laptop in Costa, the world population has increased by 8,591.

According to my educated calculations, approximately one new PKUer is born somewhere in the world every 3 hours! This equates to 8 a day, 56 a week, 240 a month and 2,920 a year. The global PKU population continues to grow every day! These are only my educated estimations, but it is humbling to see those numbers and know we are far from alone on our journey! My question is what percentage of those are being treated versus untreated? I think it would be interesting to know how many people with PKU there are out there who are in desperate need of help. I need to understand exactly how fortunate I am. Behind every suffering PKUer, a family is suffering with them; desperate to provide the best lives to their loved ones.

Whilst there may be (by my estimation) approximately 337,183 PKUers in the world, the number of people affected by PKU could run into the millions.

One World, One PKU.

Links to sources used in this blog:

National Library of Medicine

Worldometer

Science direct

Worldometer

National Library of Medicine

Science direct

The Last 15 Months Part 2

May 2024

Haven’t Read Part 1 Yet?

It was only a few weeks before we were due to take our daughter and ourselves on a much-needed break to Butlins when a letter arrived on our doorstep. It was a Section 27a from our landlord (otherwise known as an eviction notice). We had a month to find a new home. We were out. No arrears, no issues from our end at all. Chaos and blind panic ensued.

Now I find myself frantically house hunting on top of everything else I have going on (see Part 1), along with packing down the house single handedly. My wife, Dee, was laid up in bed getting over a double infection (on top of her usual complex health conditions), fighting to be fit to go away on holiday.

PKU wasn’t even on my mind at this point. How am I supposed to find time to plan meals in advance, shop and batch cook? I was continually grabbing food on the go, while attending never-ending appointments with counsellors, housing support groups, never ending unplanned meetings with my daughter’s head of year and pastoral support teams, I also had various daily and weekly hospital and doctor’s appointments for my wife; all while squeezing in house viewings in between as I went. Life literally couldn’t have been any more stressful!

It was several days later that I discovered the eviction process took more like six months before it would reach the court. Given this new information, we decided to go ahead with our Butlins break as planned, clear are heads and then return and push on with house hunting.

July 2024

Three days before we were due to head to Butlins, my wife received a text message from her estranged family (a very long and complicated story) to announce the death of her Mum. A vague message with next to no detail other than a date a few days before. It’s hard to put the feelings into words. We had already had the rug pulled out from under us with the eviction notice; this was just too much. We just sat in silence, looking at one another. Aside from the obvious grief of losing her Mum, we both knew that this was going to reignite tensions within the family. The family we had moved hundreds of miles to get away from. To escape abuse and lies that had already devastated our family and forced us to move mid-COVID.

We weren’t wrong in dreading what games and nastiness would be directed at us by the family this time. It started with the withholding of all the information on the death of Dee’s Mum. (Despite this, we decided to still head to Butlins for our daughter’s sake; she was looking forward to it). All further communication (from Dee’s family) after the initial message was cut off. We knew nothing of the circumstances or her Mum’s whereabouts. I set about ringing around hospitals and funeral directors from our chalet in Butlins, for hours, trying to find out any information I could. Everyone I spoke to had been told not to talk to us. We couldn’t find out anything; it was just a wall of silence.

After being helped by a good friend, we managed to obtain the date and time of the funeral. With much deliberation, Dee decided she needed to go. Why should she be stopped from going? She/we had done nothing to deserve the way we were being treated.

It was a very long and sombre drive to the crematorium. When we arrived, we were a few moments behind everyone else, although nothing had started yet. But we were refused entry and had to stand outside in the foyer, my wife distraught and refusing to leave until she had at least had some private time with her Mum. We stood there helplessly while the police were called on our request.

We never got entry to the service and instead had to listen to the whole service through the closed doors from the foyer, while trying to negotiate for my wife to at least get some time with her Mum afterwards. All requests were refused. The service finished and the guests were ushered out a back door to avoid us. They all left without saying a word. And not a word has been said since. It was devastating to see my wife go through this. My daughter didn’t deserve not to be considered by them either. How do you explain this to a teenager who is already battling mental health issues (many caused by this family in the first place)? It was heartbreaking. We could do little more than come home and deal with the issues we still had waiting for us at home.

The 4-week deadline to vacate our home passed and we are no closer to finding somewhere to live, due to our current circumstances. It became obvious that finding a new place to live was going to be a bigger challenge than we’d ever imagined. The rental market was like I’d never seen it before. Rental prices had gone through the roof and much of it was well out of our budget. To my surprise, whatever was within our budget required a guarantor, regardless of whether we could afford the rent or not. I am a 48-year-old man with a family, so who on this earth am I meant to ask to be my guarantor? For the first time in my life, it was looking like I could be made homeless, along with my vulnerable teenage daughter and disabled wife that I care for, not forgetting my PKU diet and other health issues I needed to manage. I was beside myself with anxiety.

I was eventually put in touch with an anti-homelessness charity called Launchpad, who was there to support me in finding a home and/or temporary accommodation. It was through the charity that I discovered temporary accommodation would more than likely be a hotel room and we could be there for months. To manage my PKU, not having a kitchen wasn’t an option. I emailed my PKU team at Guy’s and St. Thomas, who are an amazing support team. They wrote a letter stating the importance of a kitchen for managing my PKU diet, along with other information about PKU. I added this to a growing portfolio of evidence that the charity helped me compile for our case for social housing. I have never had to deal with so much paperwork in my life! I don’t know how I would’ve managed to get it all done without them. Launchpad were fantastic!

August 2024

With our main portfolio of evidence submitted to the council to further our housing application. It was now a waiting game. What would come first, an offer of social housing or the eviction date?

Meanwhile, we tried to continue our private rental search, but it was hard to get a viewing; we just weren’t meeting the criteria to even view places. When we managed to view properties, we were putting in an offer regardless of the condition, but we were declined every time. It was demoralising. Lots of the properties were in pretty poor condition. It is quite an experience to be made to feel not even worthy enough to pay through the nose to live in some over-priced hovel that a landlord has the cheek to even advertise as a home.

The days dragged by as we tentatively waited to find out our fate. The private rental market continued to look futile as we waited for the local council to chew over our application and for the court system to decide our fate with the eviction. What would happen first?

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The Last 15 Months

December 2023

Welcome to Dan’s Diary. Navigating PKU life can be challenging at times, and here, I want to share with you my personal experiences; the ups and downs of life with this rare disorder.

It’s been a long time since I first started writing this blog. It’s the longest it has ever taken me to write one. It’s not because I have had nothing to say, but because life has been throwing a lot in my path.

It started on Christmas Day in 2023 when I developed a burning sensation in my chest. I was forced to abandon cooking our Christmas dinner, leaving it half-cooked, for a trip to the local hospital. After a short wait and being wired up like a Christmas tree, I was given an ECG. My observations came back all clear (all is well with the old beatbox!). Following much back and forth from the medical professionals, I was diagnosed with Shingles. Eventually, with my medication in hand, I returned home (at 7 pm) to try and salvage Christmas dinner.

The medication I received had to be taken every 4 hours. This included during the night. Having to wake up in the night to take medication destroyed my sleep pattern. I am a heavy sleeper and can’t get back to sleep for anything, once I’m awake. In the days that followed, I was like a zombie as my days began at 3 or 4 am and it doesn’t take many nights like this to put life into a wobble!

The lack of sleep and energy affected my ability to manage my PKU diet and left me feeling pretty rubbish over the Christmas period!

As you can imagine, I was so grateful to wake up in 2024. I’m not one for New Year’s resolutions, but I love drawing a line under everything and the feeling of a fresh start. There is nothing better than closing the book on all the ups and downs of the previous year. I find something invigorating about the optimism of a new beginning. That notion that things can be better and anything is possible; it’s almost nostalgic for me. The opportunity to learn from past mistakes and build on any successes I can claim from a year passed. I do love New Year!

I am no stranger to fresh starts. It’s how I approach the PKU diet daily. No matter what happened on the diet yesterday, today is a fresh start. Always a fresh bank of exchanges (my protein allowance) for the day ahead, regardless of a previous good or bad day. My daily focus is taking all four of my supplements and hitting my allowed target of exchanges.

Since returning to my PKU diet, I’ve always found it hard to plan my meals. I like to go with the flow and eat what I am in the mood to eat (I guess I’m clinging to the old habit of being off the diet), but this method contradicts how PKU works, often putting me at the disadvantage of finding myself inappropriately organised. It’s something I need to work on in 2024.

Jan – April 2024

The day arrived when my daughter returned to school following the Christmas holidays. It was time to rediscover our daily, family routine after the festive holiday hiatus. I love the daily routine that term time brings. I thrive on it. I am always up early; a fully paid member of the 5 o’clock club. It’s my most energised time of the day. Nothing is better than seeing the first light in the morning, accompanied by the silence broken by the birds singing. I make the most of this quiet time to get on with work and chores around the house.

My daughter has been suffering from mental health issues caused by bullying at school for a while now. Dee and I have been working hard to support her and set up as much support from outside agencies as possible. This meant weekly, often daily meetings with the school and other healthcare professionals. But truth be told, everything has been painfully slow at getting activated and we were helplessly watching our daughter sliding further into darkness. It was truly heartbreaking. My daughter’s slow spiral had caught me unawares. It was so gradual at first, that I hadn’t noticed the severity of its impact on my life. I thought I had a handle on everything, but I was unaware that things had changed so much.

Life had been becoming more and more about supporting my daughter and I’d had to learn a whole new way of parenting. Everything I had ever learned about being a good Dad, suddenly seemed to count for nothing. The rule book had been ripped up in front of me. This realisation meant my priorities had to change overnight. I already care for my wife who has many health conditions and now I was stepping up as carer for a teenager who was becoming increasingly isolated.

My once vibrant, fun-loving daughter who attended many clubs and sleepovers, had dropped out of all her clubs over a short time. One by one she had lost her friends and became a recluse who never left her bedroom and who cried herself to sleep at night. It was devastating to watch. My daughter, a bright child with good grades and perfect school attendance, became the opposite. Her attendance had started to slide and her grades began to slip.

Despite our daily communications and meetings with the school staff and arranging counseling within the school. Regardless of the many emails and appointments for extra support from outside charities and the local council, things were still skidding in the wrong direction. We were helpless.

During this time, Dee’s (my wife) health was taking a new pattern of decline. It had been in decline for years, but something had been shifting. This change meant she was becoming increasingly frustrated at being unable to do more to help and support us both. This only aggravated her already poor health even more and she was going through a miserable time of her own. The start of this year has seen Dee fighting off infection after infection in addition to the conditions she already has to contend with on a normal day. It has been tough to watch. Seeing someone you love going through this and being unable to wave a magic wand, is unimaginably hard.

Managing my PKU diet around all this had become near impossible. My days had no routine, every morning I got up not knowing what the day would bring, a trip to the hospital with Dee, a meeting in school with my daughter, or both! I was having many bad or non-existent days on the PKU diet. I then followed these days by cutting out protein completely, trying desperately to reset my Phe levels. This high and low trend in my Phe (Phenylalanine) levels continued for months as I tried to do the diet when I could. I stopped sending in bloodspots because I didn’t see the point; I knew I was failing at the diet and why shame myself by letting other people see it? The feeling of guilt, shame and embarrassment for not succeeding with the diet can be epic. It’s something I struggled with daily, and it is exhausting, depressing and a downright lonely place to be.

As we approached May, the meetings and emails from school had peaked, becoming a part of my daily routine. My daughter’s absence had become so bad that she spent almost as much time at home as she did in school. We decided to take a few days away to Butlin’s to get away from all the stress and see if we could reset things. Unbeknown to us though, by the time we returned from Butlins, things would be even more complicated…..

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PKU Chocolate Log

Protein Guide – Entire Chocolate Log is 4 Exchanges. Please note, if you use just Mevalia Chocotino and no dark chocolate the entire Chocolate Log is 1.5 Exchanges.

Ingredients:

For the cake:

6 tsp of PKU egg replacer

200mls of water

170g caster sugar

170g low protein mix

4 tbsp cocoa powder

1 tsp baking powder

For the filling & icing:

25g butter

0.5 tbsp golden syrup

140ml pot Elmlea 100% plant double cream

70g chocolate (I have used half dark chocolate & half Mevalia Chocotino)

100g icing sugar, sifted

icing sugar to decorate

Method:

Step 1

Heat the oven to 200C/180C fan/gas 6. Line a baking tray with baking parchment. Place the egg replacer and water into a bowl and beat together. Then add the caster sugar and whisk together until its mixed.

Step 2

Mix the flour, cocoa powder and baking powder together in a separate bowl, then sift onto the egg replacer mixture. Whisk this together until its completely mixed, then pour into the tin. Tip the tin from side to side to spread the mixture into the corners. Bake for 15 mins.

Step 3

Lay a sheet of baking parchment on a work surface. When the cake is ready, tip it onto the parchment, peel off the lining paper, then roll the cake up along its longest edge with the paper inside. Leave to cool.

Step 4

To make the icing: melt the butter and dark chocolate and/or chocotino together in a bowl over a pan of hot water. Take from the heat and stir in the golden syrup and 3 tbsp double cream. Beat in the icing sugar until smooth.

Step 5

Whisk the remaining double cream until it holds its shape. Unravel the cake, spread the cream over the top, then carefully roll up again into a log.

Step 6

Spread the icing over the log, then use a fork to mark the icing to give the effect of tree bark. Scatter with sifted icing sugar to resemble snow and decorate.

Wishing You A Very Merry PKU Christmas

Christmas was always a busy time in our kitchen. I’m not sure how she did it, but Mum was an absolute machine at Christmas. She baked Christmas cakes for our family and for grandparents. Made both PKU and non-PKU mince pies and chocolate logs. Despite me being on the PKU diet, Mum always ensured I had the best Christmas food possible. Trifles, stuffing rolls, swede and carrot rolls; the list went on. I have never come close to matching the food that Mum used to produce. It really was something special.

The long-standing tradition in our house for Christmas dinner was always to have an enormous variety of vegetables on the plate. It’s something I still do today and has often turned into a bit of competition over the years between all of us. We always try to deliver the greatest number of vegetables for a Christmas roast. The minimum entry level would be six, normally carrot, swede, parsnip, broccoli, sweetcorn, sprouts; sometimes we added peas, cauliflower, leek; whatever we had space to cook. I always double up carrot and swede in the same pan just so I have an extra hob to cook on. Sweetcorn and peas are easy to do in the microwave. This saves another hob. As a child, Christmas dinner was always a real showdown in our house, and to this day, for a PKU’er, Christmas really is the best roast dinner of the year!

Mum and Dad made the traditional Christmas banquet even better, because we repeated on Boxing Day, when we would have upwards of 10 people join us for part two. Grandparents, aunts, uncles and cousins would join us, bringing chairs to sit on. I still don’t know how we fitted everyone in! They were incredibly fun days. Those memories will always be so precious to me.

These days, Christmas is a lot different. It’s much tougher preparing the festive food when you are living with PKU. Christmas is a busy time and managing the diet between working, Christmas shopping, ferrying the kids to extra clubs and parties (all while battling through the dreaded Christmas traffic); it leaves very little energy for the demand expected from the complex PKU diet of an everyday cycle of batch cooking and meal planning. The extra festive cooking requires energy I rarely have in reserve.

Reflecting on past Christmases when I was a young adult and off diet makes me realise, being off diet gets harder as you get older. I didn’t struggle with the same level of fatigue I do now from having high Phe levels (Phenylalanine). The fatigue is so much more intense now, compared to my younger years.

Christmas can be a very testing time for the diet. Temptation is very real with all the extra treats floating about. The will power needed to overcome it is so much more than when you are trying to give up smoking. If you are an ex-smoker, like myself, I can tell anyone who knows what it’s like; when you first give up and you’re sitting in a group watching everyone else smoking. Furiously chomping on a bit of gum to beat the cravings. Now, imagine that craving is hunger and everyone around you is eating your absolute favourite snack. Maybe it’s Doritos or peanuts or maybe even both. But all you can eat is the carrot sticks which are sat on a plate in amongst the bowls of proper snacks. For the duration of a whole evening, tell me, would you be able to fight the temptation?

Everyone knows the consequence of going mad for the Christmas goodies. It will no doubtably result in extra pounds and a New Year’s resolution to be hitting the gym, hard! But, as a PKU’er, going mad for it isn’t an option. Santa doesn’t stuff our stockings with extra exchanges (the units in which we count our protein intake. 1 gram = 1 exchange) at Christmas! We are still having the same restrictions for eating as we do for the rest of the year. This is despite there being four times as much food available, and it’s all the good stuff! Who wouldn’t want to go mad for it! Just remember whilst you are having your second helping of double chocolate cake, someone with PKU will have to be grateful for their having a second helping of fruit salad (like they don’t already live on fruit every other day of the year!)

PKU’ers love Christmas too. Sadly, we can’t just go to the local supermarket and load up our trollies with extra treats for Christmas. PKU doesn’t allow us the privilege of convenient eating. Because of this I wanted to share with you, once again, the Festive Wellington and stuffing rolls recipes. To add to this, I have done a PKU Christmas Chocolate Log, including a PKU swiss roll. I always love to decorate my chocolate log and every year, I add a new figurine, just to keep things exciting!

PKU Chocolate Log

Protein Guide – Entire Chocolate Log is 4 Exchanges. Please note, if you use just Mevalia Chocotino and no dark chocolate the entire Chocolate Log is 1.5 Exchanges.

Full Recipe

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Why Do I Struggle with PKU

The struggles with PKU can be very complex. This is because there is a wide range of challenges that can arise. Some of these are caused by factors out of my control, throwing up unexpected challenges. For example, an issue with my prescription order, causing a delay in one of the many products I rely on daily. Not receiving a delivery of my supplements (of which I must take four times a day) can have an immediate impact on my PKU diet.

The supplement is all-important because it contains all the minerals and goodness I miss from not eating high protein (normal) foods. Alongside this, and important to me, it fills me up. When I am living on a diet fit for a rabbit, I need something that’s going to keep me feeling full and satisfied. Without my supplements for a day, I feel hungrier. This means I need to eat more, and in turn my protein intake increases for the day (which I must closely monitor) raising my Phe (Phenylalanine) levels.

Just as Superman loses his superpowers when Lex Luthor hangs kryptonite around his neck. High Phe levels have a similar effect on me, because I lose my ability to function properly.

Despite the vast difference in my capabilities when my Phe levels are high compared to when they are low, I cannot detect the change as my levels increase. I find this strange because when I was a child, I’m sure I could tell if my levels were high. This is because I felt a little out of control of myself. I would suddenly become aware I was being silly and pushing the boundaries. Like the throttle jamming on a car. I knew I needed to slow the car down, but I didn’t have total control!

As an adult now, I am totally blind to it. Ask me and I will be convinced that I am doing things the same as if my levels were within range. I don’t know when or why this has changed. Maybe it’s from spending many years off the PKU diet? Maybe I’m just numb to it now. I can’t really explain it!

Being organised is the key to surviving on the PKU diet (as I’ve said many times) and it’s something I am still grappling with daily. I’ve tried to work on those areas that are out of my control. So, I asked myself, what could I do differently to help myself combat the difficulties in my supply of prescribed supplements and dietary foods? The main cause of these delays are due to late deliveries, human error or stock issues. The result was, I slowly managed to increase my stock of prescribed foods at home. This has allowed me to create a surplus, a buffer for when things don’t run smoothly. This has been a challenge due to the cost. My average monthly prescription is worth over £2,500. Consequently, my GP has been reluctant to allow me to order extra. I now ensure I have enough stock to carry myself for an extra two weeks. I have also linked up with another PKU family locally, so we can help each other out if we have supply issues.

The state of my mental health has also been an enormous factor in how much I struggle to manage the PKU diet. I take medication for depression and anxiety and have done for some time now. My mental health is something that has always been an issue for me since I was a child. Like many, I didn’t realise I was struggling with my mental health until I was much older (well into my thirties). As a young adult, it plagued me, but I just thought I had to put my big boy pants on and push through it. In the nineties, there was very little focus on mental health. No one ever spoke about it.

Mum and Dad were there every day when I was younger to put a PKU meal on the table. As a child, this meant at least my Phe levels stayed in range regardless of how I felt. As an adult, I must battle the symptoms that PKU throws at me like fatigue, depression, anxiety and severe brain fog; despite this, I must still manage my own Phe levels. Getting a PKU meal on the table when you are tired, depressed and can’t focus on anything can be impossible.

If I hit a low point when I am due to go shopping and the cupboards are bare, or maybe on a day when I’ve planned batch cooking to restock the freezer; things can go south very quickly.

If I physically can’t get off the sofa to get to the shops or prepare a PKU meal, I go into panic mode. If I get hungry, I am going to grab whatever is available in the cupboards. Worse still, it could have me reaching for my phone to get a takeaway delivered. This means my Phe levels will rise above where they should be, and tomorrow, I will be suffering even more. Extra fatigue and feeling yet more depressed will be the order of the day. I will be less likely to follow the diet (especially if I still need to go shopping before I can cook a meal from scratch).

One of my other struggles, and I know many other PKUers struggle with as well, is being judged by others because of weight and apparent laziness. This issue isn’t restricted to just people with PKU. But many people living with a hidden health condition, including those who deal with fatigue, chronic pain, and mental health issues.

I have openly spoken about my struggle with Compulsive Eating Disorder many times. Every time I speak about it, I hope it helps someone to avoid the path I took. It’s a dangerous path, especially for someone with PKU. People who judged me on my weight and laziness helped to feed my disorder. The truth is it used to bother me. It doesn’t anymore, but it’s been a long and painful road getting to this point.

Over the years, I have encountered some ignorant people. A few have been family members who should’ve had a better understanding of PKU. Some are even guilty of making me feel the need to go home and binge on so much food, I would pass out from the protein overload. I did this in part, because they made me feel ashamed for struggling in life and not measuring up.

I’ve worked hard to get past the shame of being overweight and at times unenergetic. I’ve learnt to appreciate the important people in my life who always support me and are there for me, understanding my struggles. My wife, my children, my parents and my brother are the most important people in my life. There are friends and family who have supported me over the years, and whether they take the time to really understand PKU or not, I know I am accepted for who I am. For them, I am also truly thankful.

For those who judged me and called me ‘Fatboy’. I really couldn’t care less anymore! I no longer allow myself to be affected by them. It’s taken me a long time to get here, but I now know my worth! To all of those who understand what I’m talking about, stay strong, be yourself, and surround yourself with people who love and accept you for who you are.

‘But you’d have to walk a thousand miles in my shoes, just to see what it’s like to be me’ – Eminem ‘Beautiful’.

This song really resonated with me during the peak of my struggles to get a handle on PKU again. When I felt no one understood what I was going through. Although it was a dark time for me, I realise now I am not alone. There are plenty of people who care and understand. Some with PKU and some without.

My message to you is don’t suffer on your own like I did. If you give the people closest to you a chance to step up, they won’t disappoint you. If you don’t have anyone close to you, then reach out to the PKU community via a Facebook group. There are many there who are always ready to support anyone in need!

Failing this, here are some support numbers to call. Please, DON’T be alone in your battles!!!

Helpful links:

Samaritans 116123 – UK ONLY

befriendersworldwide – have contacts in 51 countries

The Emotions Surrounding PKU

It’s been quite the experience, unpacking all the emotions I have faced during my journey with PKU. I never thought for one minute that I have navigated my way through twelve different emotions during my lifelong battle with PKU.

I have previously spoken about the struggles I had growing up with the feelings of anger and frustration. I went through a phase of venting out at anyone who gave me a reason to. As an adult, I have learnt to hold my anger in. I never direct it towards anyone, anyone other than myself, that is. Until recently, I did this by binge eating and verbally abusing myself.

Like everyone, I have a limit to the amount of anger I can suppress. The higher my Phe (Phenylalanine) levels are, the quicker I reach boiling point. Then, like an over pressurised steam engine, I’m in danger of exploding. I must seek solitude to enable me to calm and collect myself. I have learnt how to do this by heading to a quiet room to pray or by going out for a walk.

When my Phe levels are in range, I can absorb a lot more of that anger. It’s like a wet sponge versus a dry sponge. Low Phe levels mean my sponge can absorb more Phe than when my sponge is already wet and can’t absorb as much.

Guilt and shame are two more emotions that often come hand in hand. These two emotions have played a huge part in my PKU journey. As a child, I suffered from guilt and shame due to sneak eating food. I was ashamed of pinching food because I was hungry. As I grew older, I struggled with guilt and shame because I spent all my money on food and was always broke. This always affected my ability to socialise because I always had limited finances. I was always that ‘one’ in the group who could never afford to buy a round of drinks. I had some very generous friends, but that didn’t stop me from leaving the night plagued with guilt at not being able to buy ‘my’ round.

In later years, I also fought with the feelings of guilt and shame after I binged vast quantities of food, and again when I started gaining weight.

On returning to the diet, my guilt and shame manifests itself in the effect it inflicts on my family. Returning to diet has had a tremendous impact on my lifestyle and that impact has directly affected them. There has been no avoiding it.

The mental toll of feeling continually guilty and ashamed of yourself is so hard to put into words. Imagine waking up in the morning, feeling like you had spent the entire night discussing physics with Steven Hawking instead of sleeping. Exhausted doesn’t even begin to describe it!

Anxiety and depression have been persistent unwanted friends of mine over the years. I know too well the feelings of desperation. The feelings of darkness that smother you like a thick fog. During these times, I’ve felt lost, and I’ve felt alone. One thing I feared the most was being around people. I just wanted to be on my own. I found the solitude comforting in some strange way.

I have on many previous occasions, found myself pacing around the house talking to myself. I was often worried about something! One day, I remember having to leave the house in a massive panic, for absolutely no reason. I just felt unsafe. It felt like I was in imminent danger! I had to sit in the car for an hour where I felt safe, parked on the road outside. When the feelings subsided, I returned home. Both depression and anxiety can be so debilitating. It comes and goes as it likes; I have no control over it at all. It quickly knocked lumps out of my confidence, not knowing how I was going to feel from one day to the next.

When I was in my twenties, it could sometimes stop me from making plans. Now I am older, my anxiety is worse, especially over the last ten years. I am finding it harder than ever now to make plans. I often catch myself avoiding it. I much prefer to do things on the spur of the moment. It’s more comfortable for me than planning something in advance, when I can’t guarantee how I will be feeling.

Disappointment is something I am no stranger to. So many of the things I have done in my life have ended in disappointment. But I now know why! It is because I have been so inconsistent in almost everything I’ve ever done. The only thing I have been consistent with during my long period off the PKU diet has been making poor decisions. I have struggled to achieve many of my life goals because of it!

To focus on a more positive note, I am turning all these around now; all thanks to returning to the PKU diet. Despite how hard the journey has been so far, it’s been worth every step! I encourage anyone to return to the diet. I deeply regret stubbornly putting it off for all these years and putting the joy of eating ahead of anything else, including my health.

I struggled with embarrassment as a child and through my teens. One thing I remember the most was as a young boy, Mum pulling out a set of scales and weighing out my chips in front of a busy seaside restaurant. I just wanted to slide under the table and disappear. I felt like I was going to burst into flames with embarrassment.

The smell and appearance of my ‘special’ PKU foods was an immense challenge as a child at school. Trying to explain to friends why I was scooping my sandwiches out of my lunchbox with my fingers isn’t exactly one of my better memories. A slice of PKU bread in the eighties didn’t hold together long enough to put butter on! To then expect it to survive being placed in a lunchbox before being swung about on the way to school was a big ask. For it to then be launched across the classroom by my so-called mates, you can only imagine it didn’t resemble a sandwich by lunchtime!

As an adult returning to diet, I can honestly say that embarrassment hasn’t been an issue. In society these days, being different is totally normal. Back in the eighties, you were either ‘normal’ or seen as weird, odd or special! It really wasn’t a great time to be ‘different’.

Apathy is a new emotion I have linked with PKU. The absence of emotion and enthusiasm is something that I have only recently understood, despite having dealt with it for many years. My recent attention to it makes me question whether it is something I am experiencing more frequently now. Apathy is one of three symptoms that has become more sensitive to my inconsistent Phe levels since reaching forty. The second is anxiety which I have already mentioned. My short-term memory is the third thing that is affected more by a higher Phe than it previously used to be.

It is very apparent that I have better control over all my emotions when my Phe levels are in range. I am also more capable and likely to stand up for myself. Thinking quickly because my mind is focused and clear and enables me to respond faster and stay in control of my emotions. Because of this, I feel mentally strong and confident enough to defend myself without losing control.

During my mid-twenties, when I had been off the PKU diet for about 6 years, I battled all these emotions alongside all the classic PKU symptoms ( stress, anxiety, lethargy, poor concentration & focus). I lost several years of my life during this period. It was like living in a giant black hole. I often wonder how I made it through those times. To be honest, I’m not sure how I made it out of the other side, but I did! And here I am, in a place I never imagined was possible. Forging a new life where I have taken back control of PKU.

Thanks for reading. Please can I ask you to share this far and wide! Help me to tell our story to more people outside of our PKU community. It’s where we need to be heard the most!

Take care until next time and stay strong #PKU

Dan