Learning to be Thankful for PKU

Today is ‘International PKU Day’ and I want to start by wishing everyone in our community well, wherever you are on your PKU journey. I have thought hard about what I could write about to contribute to this significant day. Today is important to all of us because our lives have been and continue to depend so much on the hard work and dedication of others.

It’s a day to celebrate the legacy left by important figures within PKU. Dr Horst Bickel, Dr Robert Guthrie, Sheila Jones and her mother Mary are just some of the names from our PKU history that you may recognise. But there are many more whose dedicated efforts are ongoing. The entire team at NSPKU has built a 50-year legacy. Raising awareness, fund raising, advancing treatments and more; along with endless help and support to anyone who has needed it. Still, they strive to improve life for us, and so the NSPKU’s legacy continues to be built.  

Our battles are daily. The challenges we face can be relentless. The fight we fight can be excruciatingly exhausting. We have much in common, but individually the war we fight differs for each of us. Yet we have so much to be thankful for.

If I have learnt anything from connecting with the global PKU community over the last 18 months, it is an understanding of the vast contrast in support and care. The access to basic treatments and funding for our fellow PKUers differs immensely around the world.

Here in the UK, it has been easy to lose sight (especially during our long battles in recent years for access to new treatments) of just how lucky we are here. We are so blessed with the consistency that the NHS brings us regarding the availability and funding of our treatments.

Consistency is so important for us to manage PKU and is something even the United States falls short on. This is due to complications created by relying on insurances to fund their treatments. The refusal of insurances or lapses in insurance cover can cause breaks in treatment.

Anyone with PKU will understand running out of dietary supplements and the other items we rely on daily can cause total chaos. Leading to a complete breakdown in one’s capability of coping with everyday life. It is impossible for non-PKU people to understand how quickly our lives can unravel during a period without treatment.

At the opposite end of the treatment spectrum, we have countries like Morocco. I have been in contact with Driss, a father of 3 daughters, all with PKU. The care, support and treatment they receive there is non-existent. To put this into a greater perspective, the support and care received from the NHS in the 1960s, was better than this poor family receive today, in Morocco.

Worryingly, Morocco as a country isn’t alone in its inability to care for anyone with PKU. According to the World Health Organization’s ‘Healthcare Index’, Morocco was ranked 96th out of 167 countries in 2020. When you consider the care received by PKU patients in Morocco, it really begs the question, what care (if any) is being received for those living with PKU in the 71 countries below Morocco on this list? That doesn’t include the poor health care given in countries who are ranked higher than Morocco. For me, learning this has been a huge wake up call!

Despite my personal journey with PKU (Coming Off the Diet ), it’s easy to forget where I would be now had I not been diagnosed with PKU at birth.

Had I not been born in the UK when I was, my story would be very different. I was blessed with not only incredible parents who sacrificed so much for me. But I was also blessed to be born in a location where I had access to one of the best healthcare systems in the world. The NHS allowed me to receive the best support and treatments available without the burden of its extremely high cost.

I have been unaware that so many people with PKU must overcome the impossible obstacle of funding. All while dealing with this already complex and life-changing condition.

I have spoken many times with Driss about his battles to obtain treatment for his three PKU daughters. Salma is 17 years old; Maryam is 15 and Fadwa 5. They all have untreated PKU. Driss cannot gain access to any support. Support that would enable him to provide the expensive supplements and low protein foods for his daughters.

As anyone within the PKU community will understand well, the results of not being treated for PKU from birth are catastrophic. I was deeply humbled when Driss explained his story to me and the severity of his daughters’ disabilities. It made me aware that I should be extremely thankful for my experience of PKU. In one of our many conversations, I recall how he told me none of his daughters talk. As a father, I can only imagine how devastated and helpless he must feel.

It’s difficult to comprehend the fact, had I not been fortunate enough to have received this life-changing care, I too would be severely disabled now. Writing this blog would not have been possible without the care I have received. My ‘alternative’ life is unimaginable. The life I have today wouldn’t resemble itself in the slightest. This realisation has had such an enormous impact on me. Despite the issues I do face now, it pales in insignificance in comparison.

My PKU experience has made me so thankful. Thankful that I can be writing this today. Something has been burning away inside me for some time now and this has compounded it for me. I feel like it is my duty to do what I can to help, to give something back. To raise awareness and be the voice for others who cannot speak out for themselves (especially those who have not had the chance in life they deserve). Everybody deserves the same opportunity for treatment and the right for a chance to live a normal life.

That said, I am also acutely aware there is so much we need to celebrate within PKU. The generation born with PKU twenty years after me are going to university now and coming away with degrees. The progress in treatment is tearing down walls and PKUers are achieving things now that were not possible for my generation and generations before. We need to celebrate PKUers who are graduating with medical degrees and others with degrees in Law, Engineering and in some cases, even PhD’s. Today must be about celebrating these incredible successes too!

As we progress to brighter futures and better treatments, we also need to work out how we can make all treatments accessible to PKUers all around the world. It proved an excellent example of what we can all achieve last year, when our community pulled together to help our PKU family in Ukraine.

We are just a small community representing PKU here in the UK. The same as there are only small PKU communities in Morocco, Canada, Spain and every other country in the world. But we all have one thing in common, we know life with PKU! Together we could make a sizeable community and have a big voice with access to better resources. The question is, how do we become one big global community?

Dedicated to the NSPKU for 50 years of incredible work and support

Please help me support NSPKU by donating to my 50 (500 piece) puzzle challenge.

I aim to complete by the 30th Sept 2023

Donate Here

Dan’s Journal February

A phone call from the appeal board got February off to a great start. Just a couple of days into the month, I learnt that we had won our case and secured our daughter a place at the best school in our area. So much excitement was to ensue. All our hard work, tears and stress had paid off; now as a family, we would be able to start rebuilding our lives.

It wasn’t the start of the month I had expected. The results of our appeal had come in, in a matter of days, not weeks, like we had been told. I was really flying high and smashing the diet in the process. With such a weight lifted off my shoulders, I turned my focus more towards ‘Truth About Life With PKU’. In the pages and pages of notes I’ve made over the last couple of years, I had long abbreviated the name (Truth About Life With PKU) to TALWPKU. I sat at my desk one morning with a coffee in hand. On the wall in front of me was a post-it note with the heading ‘TALWPKU’ on it, and it dawned on me, if I removed the ‘W’ and replaced it with a ‘K’, it became ‘TALKPKU’.

TALKPKU is the name of a new project I have started working on this month. It has been in the back of my mind for about 18 months, and I felt now was the perfect time to start moving forward with it. The TALKPKU project will be aimed at raising awareness for PKU. I do have a long-term goal, but as I am keenly aware, achieving this will only be possible if I stick to my PKU diet. I know I need to keep the pressure I put myself under to a minimum, and it’s the key to me succeeding. I am so excited about this project that it is so hard not to tell all now! But a step at a time, I will shape TALKPKU over the coming months. I ask you, please keep following Dan’s Journal to find out more and see how the project is progressing.

When the PKU diet and I are working in tandem, my productivity levels are 10 fold. There is no stopping me. I can multi-task and manage my time so much better; these two things are non-existent off diet. Now the school pressure is off, I plan to capitalise on it!

The 28th of February was Rare Disease Day and my birthday! Sadly, all my birthday plans had to be postponed after I managed to catch Covid. So, with a headache that felt like someone was ringing ‘Big Ben’ in my head, I spent my birthday feeling rather sorry for myself!

It can take nothing more than a dose of Covid to throw the PKU diet in the air. Feeling as rough as a bit of sandpaper, I ended up grazing on whatever I could find lying around the cupboards and fridge. Fortunately, I had just baked a fresh loaf of PKU bread (which helped). One good thing about Covid is losing your taste. It isn’t such a bad thing when you are on the PKU diet. I managed to use up a few of those PKU prescription products that have been kicking around in the back of the cupboard.

Despite the bumpy end to the month, February has been kind to me. Plenty of positives to take away and build on in March. The birth of TALKPKU, along with my daughter starting a new school has been some of the highlights for me. Along with another milestone passed this month was Dee and I celebrating our 4th wedding anniversary! I don’t know where that time has gone. I’d like to take this opportunity to thank my amazing wife for all her love and support. Having support without being judged is incredibly important when negotiating life with PKU. Maybe we should consider a day to give thanks for all the support and dedication our hard-working parents, families, friends and carers have given / give us? Who is your PKU rock?

Image provided by Engin Akyurt

Fight or Flight PKU

One of the biggest challenges I find with an invisible disorder like PKU is nobody ever sees the inner turmoil. My battle with PKU is completely down to me. Some days it feels like I’m floating in space, alone. This journey is mine to take, and I take it solo. Some days I feel like I’m at war; me against the world. I must plan, fight and defend continually, just to stay in control of my destiny. It’s exhausting, but I can’t stop. Yet on the outside, I appear like any other ‘regular’ guy going about his daily life.

PKU is like a ‘monster’ that lives inside of me. It’s like having a personal devil willing me to fail, forever in the background chipping away at me. I can keep ‘him’ quiet and locked away in a cage, but only if I eat the right foods and stick to the PKU diet. If I deviate from the diet, even the slightest bit, his voice starts to get louder. ‘He’ uses distracting tactics, stopping me from creating my meal plans or batch cooking; making me reach for the wrong foods. Eventually, escaping from his cage, ‘he’ takes control. Once he is in control, I am out of control and at the bottom of what I call the ‘PKU spiral’.

This is the lowest point that PKU brings me down to and it’s a lonely place to be. Sometimes I have the strength to react quickly and fight back straight away. If I eat nothing but low protein foods for 24 – 48 hours, I can block him out. But if I can’t, it takes a huge amount of energy, especially when I am already struggling with fatigue. If you can imagine a Nasa space shuttle launch, that is how I would describe the energy needed to shove ‘him’ back in his cage.

The ‘monster’ has been in charge of the steering wheel for more of my life than I have! And ‘he’ believes he should still be driving. ‘He’ doesn’t want me to succeed and ‘his’ voice is in my ear, tempting me when I am at my weakest. Once you have been off diet, he always has leverage against you and ‘he’ will use it against you!

Only in recent years have I understood how much PKU has had control over me. More than I had ever realised. The impact on my life has been far greater than I had ever given it credit for. I have spent much of my adult life ignoring the fact I have PKU. I had just put it in a box in the back of my mind and shut it away. I have only recently realised this ‘monster’ has been running things for most of my adult years. Now at 46, I am starting to regain control of my life.

It has been about a year since I discovered I hadn’t been in the driving seat all this time. The truth was ‘he’ had been out of his cage for two decades now, and this meant it was going to take a lot of effort to get him back in again and even more to keep him there!

Life with high Phe (Phenylalanine) levels, combined with stress and high-pressured situations are dangerous territory for a PKUer to walk in. In these situations, I find I am prone to acting irrationally, leading to very poor decision making. Worse still, if I need to make big decisions quickly I will run for the hills and hide because I can’t cope with the situation.

High Phe levels seem to cause a complete breakdown in my capability to quickly assess and deal with intense situations. My natural reaction is to go into fight-or-flight mode. Looking back through my life, the fight-or-flight mode explains so many of my struggles.

As an upper junior (year 5-6 now), I was an angry child. The real me was terrible at standing up for myself. I was hopeless at standing my ground. However, during this period of primary school, I was always fighting. Often over the silliest things. As I reflect now, I can see that I had zero tolerance. Someone simply calling me names would very quickly have me wildly swinging for them!I would often find myself looking for an excuse to fight. If I was feeling irritable, I would scour the playground looking for somebody who was picking on someone else, and then I would start on them. It was as if I had no control over my emotions. The anger just came out of its own accord. I do question now, if this was the first time I had experienced ‘the monster’ taking the wheel off me?

One day during my final year in primary, I had gotten myself into a blind rage with a kid who was always deliberately winding me up. I snapped and got into a big fight with him. Not only did I hurt him, but I accidentally hit the teacher when he was trying to break us up. Fortunately, the teacher understood that I had caught him purely by accident, but the incident really did scare the hell out of me!

It was at this point, I realised I couldn’t go through my life fighting. I had become frightened of myself and so I suppressed my anger, making flight my new default reaction. I went the other way and just absorbed anything that was thrown at me. I managed to get through secondary school without getting into a single fight. I did take a few hits from kids at school, but I just sucked it up and walked away! I guess you could say I grew myself a thick skin.

As my Phe levels get higher, I’ve learnt the thick skin I’ve developed seems to get thinner. When it can’t absorb anymore, I go into flight mode. I learnt to control which way I would go; to fight or to fly, because I’d become scared of what damage I could do as I grew bigger and stronger; hence, I always chose to fly. Sometimes, flying instead of fighting your corner is the wrong thing to do. Sometimes you need to stand up for yourself. But I can’t.

If you ask anyone who knows me, they will tell you I am a kind, caring and gentle person. When my PKU isn’t under control, it smothers me and my ability to think rationally. It will heighten my sensitivity to my surroundings, noises, conversations and distract me; keeping me from focusing on what I am trying to achieve. When it is not under control, it influences my decisions and stops me from being the real me. I have no choice but to fight my war, for every hour of every day, for the rest of my life. There are people working hard to provide us with new treatments; who knows, maybe even in the future, a cure! A cure would banish ‘the monster’ from my life forever, and that would be amazing. But every new treatment will at least make the cage stronger. It will make his voice quieter. Increasing the chances of me being in control of my destiny and reaching my full potential in life. This is my dream and no doubt a dream I share with everyone who struggles to live with PKU.

Images courtesy of Specna Arms, Lil Artsy & Rodnae Productions

Dan’s Journal January

I have been battling with hunger recently. Some nights I have gone to bed early just to try to get past it. I lay in bed at night, still hungry, my stomach rumbling in protest. In fact, I think it may be engaging in deep conversation with itself, as the gurgles respond to the rumbles. In the morning I wake up so hungry it’s not even funny!

I’ve been starting the days with larger portions of low protein foods, but I’m still feeling fatigued. My head is clear and I’m ready to get stuck into the day, but my energy levels are low. I have found myself taking extra time for breakfast. First, I’ve been taking my supplement/formula as well as eating Weetabix, then an hour later, having protein free toast and a coffee.

I manage to get going, eventually; it’s just taking me longer than normal each day. I’m not used to this. Normally, I am an early bird. I get all my best work done at the beginning of the day. I love nothing more than to see the sun rise each morning. It sets me up!

After months of hard work, my daughter is finally getting more help and support. It’s clearly making an enormous difference. Enabling us all to focus more on our own well-being and importantly for me, my PKU diet. Having a chance to take stock, get organised and catch up with some batch cooking has been invaluable.

As January progresses, I’m feeling mentally stronger and determined every day. This is having a positive knock-on effect, as I’m getting more organised as time goes on. I have been eating more regularly and consistently, not having to starve myself in a bid to control my Phe (Phenylalanine) levels. This has also really helped me combat my recent lack of energy.

I have been working hard alongside my wife Dee, desperately trying to get our daughter a new school placement since November. It’s been a hard road so far, and that same road has only grown longer this month. After hearing we’d been turned down for all our applications, we had now been forced into entering the appeals process. It’s an endless battle, but a battle I can cope with now I have less on my plate!

As I have continued to consider what I want to achieve to improve PKU life for everyone, I have never felt such a sense of pride about my PKU. PKU hasn’t just happened to me, it is me! For the first time in my life, I feel like I want to start a conversation about it with all the people I meet. I have a new confidence. I don’t want to shy away or hide from it anymore. Ever since I was a teenager, I have deliberately hidden PKU from everyone possible. I have been hiding a part of myself from the world! Now I want to share it with the world!

Raising awareness is something that is becoming ever more important to me, and I know it is part of what I want to be involved in moving forward. I am a writer and I especially love writing about PKU. The burning question in my head now is, what can I do with it?

Banana Bread

0 Exchanges

Ingredients:

3 very ripe medium bananas

3 tbsp of PKU egg replacer

100g of soft light brown sugar

150ml of sunflower or vegetable oil

275g of low protein all-purpose mix

1tsp ground mixed spice

1tsp baking powder

Method:

Step 1

Preheat the oven to 180C / 160C Fan Asisted / Gas 4 and grease a 900g/ 2lb loaf tin. Peel the bananas and mash with a fork. Tip into a large mixing bowl.

Step 2

Add the PKU replacer into a small bowl and add enough water to mix it into the same consistency as mayonnaise. Then add the egg replacer, oil and sugar into the large mixing bowl and use a fork or whisk to combine. Add the flour, mixed spice and baking powder,then whisk together until thoroughly combined.

Step 3

Pour into the prepared tin and bake for 30mins. Remove from oven and cover with tin foil to stop the top burning and cook for a further 30 mins. Check by inserting a skewer into the centre of the bread comes out clean to ensure it has cooked through. Cool in the tin for 10 mins, then turn out onto a wire rack serve warm or cold.

Notes:

It does dry out after a couple of days like PKU bread, but will work great as pudding by adding custard over the top to give it added moisture.

PKU No ‘Sausage’ Rolls

Less than 0.2g exchanges each

Ingredients:

170g sachet of Paxo stuffing (made up as per instructions on the box)

400g of low protein all-purpose mix

200g of Marg

Water

Method:

Step 1

Preheat the oven to 180C/ 160C Fan/ Gas 4. Take a baking tray and line it with grease proof paper.

Step 2

Add the low protein mix and Marg into a mixing bowl. Using your fingertips, rub the flour into the Marg until it forms breadcrumbs. Next, add water to the breadcrumbs a tablespoon at a time, mixing into your breadcrumbs until it forms your desired texture (I personally prefer it very slightly tacky as it holds together better after being cooked).

Step 3

Split the pastry into to 2 halves (just for ease). Take the first half and roll it out on a piece of grease proof paper. Sprinkle some low protein mix on top as you roll. Then trim the edges so you have a neat rectangle of pastry. Take a handful of the stuffing mix and roll into a sausage in your hand. Place the stuffing sausage along the longest edge of the pastry rectangle. You may need to join a few stuffing sausages together to do this. Using the greaseproof paper, roll the pastry around the sausage inside the greaseproof paper and then peel away the paper at the last moment. Gently firm the join on the stuffing roll and then roll back on to the greaseproof paper before transferring to the baking tray and rolling it off. Repeat this until you have used all the pastry and stuffing mix.

Step 4

Brush the pastry with oat milk before placing the baking tray in the oven and cooking for 20 mins. Once removed from the oven, allow to cool before cutting with a sharp knife to desired size.

Notes:

Please note that Paxo stuffing has the lowest amount of protein content. I cut mine into ‘party sausage roll’ size and this recipe made thirty-six rolls. The protein content for the stuffing is 6.5g and so worked out at less than 0.2g of protein each.

I would recommend you take the protein content of the stuffing you use and divide it by the number of rolls you divide your batch into at the end, so you know the protein content of your own individual rolls.

Dan’s Journal November

I have heard nothing back from my gene test yet. I’ve been talking to others around the UK, and it seems the waiting time is extremely variable. Some people have waited a few weeks, while others have waited months; one person has reported waiting 6 months! So, my wait must continue.

Despite my best efforts to stay on the diet this month, it’s been a bumpy ride, to say the very least. I keep saying this (only because I know it’s the truth!), but the key to cracking the diet and getting the best out of myself relies on me achieving the PKU diet consistently, every day. Getting access to the Sapropterin trial really means the world to me. The chance of getting real help to achieve a stable diet is forever on my mind. I know that there’s only a small chance I will respond, but I must hold on to that hope. I have to cling on to it because currently, I can’t achieve the consistency I need to be ‘normal’ everyday. I’m feeling fed up with my Phe (Phenylalanine) levels bouncing around like a pinball machine and all because ‘life’ impedes managing my PKU diet properly.  

This month my wife and I had to remove our daughter from school due to continued bullying. The school were failing to protect our daughter and had left us with no other alternative. The stress and upheaval this month has challenged my dedication to the PKU diet and tested me at every turn. November has seen many emotional conversations at home between the three of us. Many more conversations with strangers that have pushed me right out of my comfort zone. But I have held fast on the diet as best I could.

I haven’t binged on food this month, which is a huge result considering the tension and stress I have been under. I am proud to have weathered the storm that both October and November have brought me. Now I move into December feeling strangely optimistic. Although my journey is long, I know I have made significant progress in understanding myself and PKU of late. Every month I understand a little more about how PKU affects my daily life and the reasons I am not being consistent with my Phe levels. Another month further along my PKU journey and yet another lesson I have learnt about myself.

Inside, the feeling of wanting to do more for PKU continues to grow within me. I want to dedicate more of my time to help improve the lives of everyone living with PKU. The urge to do something positive helps me to feel more at peace with the journey I still have ahead of me.

One morning, I sat with a coffee and a notepad, quietly brainstorming ideas. How can I be more useful to the PKU community? How do I become a bigger part of the fight for change?

As I sat writing a list of everything that came into my head, I focused on all my knowledge. Recalling the many conversations I’d had with people all over the world directly affected by PKU. It made me realise there is so much work to do to ease the suffering of PKU, on many frontiers.

I realised my struggles with PKU paled into insignificance compared to some plights PKU families are finding themselves in, all around the world. In some countries, PKUers don’t have access to any formulas/supplements or low protein products at all. In other countries, people struggle to get the funding they need for vital PKU treatments.

It has been extremely humbling for me to hear the stories of other PKUers from across the globe. Despite my current struggles, I recognise that because I had treatment as a PKU child, I got through the most destructive and dangerous years of PKU life. Because of the access I had to treatments, no matter how early those treatments were, or experimental, I had a chance of a ‘normal’ life and consequently I am better off than so many of our PKU brothers and sisters.

I’m still figuring out my place in this giant jigsaw puzzle that is Phenylketonuria, but I know I want (and need) to have a bigger part to play in easing the suffering of PKU.

Dan’s Journal October

October kicked off with weeklong celebrations for my non-PKU brother’s fortieth birthday. My parents joined us from Spain, where they have lived now for almost twenty years, so it’s a rarity to have the whole family spending a weekend together. My brother chose to do this on a canal boat, and it couldn’t have been a better weekend.

On day one and being the early riser, I took the early morning shift, sitting at the rear of the boat navigating the sixty-foot-long barge through the winding canals of Hampshire. The scenery was just beautiful.

I always find PKU easy around my family. I just slip back into the old habits. Mum always clicks into PKU mode so effortlessly. She is enviably highly skilled at adapting meals to fit around PKU, often without even a pause for thought. Back at her home, I am always intrigued by how her cupboards are always stocked with everything she needs. On the rare occasion that an ingredient is missing, she always amazes me by producing some random item from the back of the cupboard that will perfectly replace it! Sadly, I don’t have the same knack that my mum displays in the kitchen. But I so wish I had!

It was a fun weekend despite embarrassing myself in front of a pub garden full of people. It was our first evening, and we had moored up for the night outside a lovely riverside pub. Despite being chilly and the sun setting, every table in the pub garden was occupied. Stepping on to the bank with the rope in hand, I reached down to tie the boat up; when my jeans split from front to back with the loudest rip I’ve ever heard in my life! This was followed by an enormous cheer from the pub garden. So, with nothing else I could do, I turned and gave a little bow to my delighted audience, and then quickly disappeared back on to the boat.

My food highlight for the weekend was when I discovered that vegan applewood cheese (from Asda) tastes very much like you’re eating bacon. It was another cold crisp morning and as I steered the boat towards our last destination; A family member brought me out a toasted mushroom and smoked cheese sandwich with tomato chutney. It was a real treat!

Overall, my PKU diet had been on a far more positive track this month. There have been a few difficulties, but it has once again, been an extremely stressful month as my wife and I tried to support our daughter (and each other) through what was an incredibly challenging time.

It’s been hard when I have frequently felt like I’m failing as a father. It stings somewhat. I have felt that all my hard work and sacrifices have been for nothing. It has been hard to digest and move past. This was how I hit my lowest point this month, and I cracked under the strain reaching out for something to make me feel better.  

Once again, I fell into old habits. I found myself sat in the car at the local McDonald’s drive-thru where I tried to find some refuge by binging out. The few minutes of euphoria were abruptly quashed by the feeling of disgrace, disappointment and dread. What had I done? I’d let myself down, and I’d let my family down BIG-TIME! What good was I going to be to them now for the next 24-48 hrs? I was so cross with myself; I forced myself to sit and work out just how much protein I had devoured in less than twenty minutes.

I sat for a moment astonished as I quickly double checked the math again. I had just eaten 78g of protein in one sitting (what on earth was I thinking?!), this is happens when I feel sorry for myself. This is what a moment of weakness can do, in what had been probably one of my most stable PKU diet months of the year. I had to make sure this was just a blip, and I went back on diet immediately. I would not let this stop me from supporting my family, ruin plans or set me back; like it has repeatedly done in the past.

After a couple of days of a ‘super strict’ PKU diet, I was back to feeling ‘normal’ and once again able to sit here at my desk, not only to continue journalling but also to deal with the bumpy road of life I am currently travelling along. Keeping PKU at the centre of what I do is becoming a forever bigger part of my routine and journey. Spreading awareness and sharing my story is growing evermore important to me, and as I move forward, I would like to do more, and be more involved in helping to change PKU for all of us.  

Dan’s Journal September

My aim for this month had been to rebuild my plans and push forward with the progress of the personal goals I had set myself. My diet is the key to achieving. I know I can achieve anything if I can get the diet right. PKU should undoubtedly be the most important thing in my life. It affects every aspect. This was the pep talk I gave myself at the beginning of the month, knowing I had just come through a tough month. I was starting September on a good footing. PKU was, and would continue to be, top of my priority list.

I spent the first weekend of the month in Swansea to help my son move house which was great. Now he is all grown up I don’t see him so often, which is hard. These days he is very busy working on his PhD, and although we talk every week, it’s not the same as spending proper time together, even if it is humping boxes about!

I finally started my journey in pursuit of a Sapropterin trial (another brand of Kuvan), after my PKU gene test dropped through the letterbox, courtesy of our cheerful local postie! Once I had negotiated the boredom of being twenty-seventh in the queue, calling my doctor’s surgery, I booked myself the required blood test. A few days later I had the blood test done and with the relevant paperwork completed, I immediately dropped it in the post box at the end of my road – so it could begin its trip to Bristol (so I am told) where it will be processed. Now I just have to wait for the results!

The latter half of the month was to be far more challenging. My daughter disclosed something to me and my wife that pulled the rug out from under both of us. We were both completely unprepared and shocked by what we had learnt. I’m not going into any details for obvious reasons, but anyone who has received out of the blue ‘life changing’ news, knows, that from that very moment, it puts you and your family unit into a bubble, and within that bubble, everything from the outside world ceases to exist. PKU went from the top of my priority list to the bottom. I had to step up and take control of the situation. My daughter and wife jumped to the top of that list, and PKU became the lesser priority.

Over the next few days, I got to know the inside of our local police station better than I had ever imagined. Reporting a crime to the police is something I have experienced before, but never have I had to report a crime on behalf of someone else; the fact that person was my twelve-year-old daughter, was heart-breaking.

The following days were consumed by endless phone calls, emails and face-to-face meetings. With my diet out of the window because of no meal planning, shopping etc; followed by the comfort eating, which is my main coping mechanism (when under stress), I couldn’t have been further out of my comfort zone talking to and meeting complete strangers.

I sat in my office one morning at the crack of dawn, my head was a complete mess. Many PKUers refer to it as ‘PKU fog’ and I had a severe case of it! I knew I had to be there for my family. They needed me, this wasn’t the time to breakdown and go into self-destruct mode. I took the day to rest up and starved myself of protein. The next day, I was already feeling a little more in control. Whilst I started to re-posture myself to better support my family, I also recognised that I had to keep reducing my Phe levels to cope.

I finished September ‘PKU strong’ because I had to. The journey continues….

A PKU Christmas

Christmas has always been a challenge for me since returning to my PKU diet,especially given my long history off the diet. Add to that my track record of sneak eating food at Christmas as a child and it’s not surprising. It’s not helpful that I’ve spent more time away from my PKU diet during my life than I have spent on it. Something I will always live to regret! During this time, I’ve tasted many foods I should never have tasted, and sadly, I can’t turn the clock back and forget all those incredible tastes and textures. Despite the PKU flavours improving over the years, the textures remain as boring as ever! If I could go back in time and give advice to my younger self, I would say ‘Just don’t try it, I promise you, you are so much better off not knowing!’.

The hardest thing about PKU is that you can’t ever escape food. It is everywhere, all the time! Walking through my city centre now compared to when I was a child (and it was just a town centre back then), I would have had to contend with the smell coming from a bakery or an old coffee shop. Now the high streets are crammed with fast-food places and restaurants with tempting aromas from cuisines all over the world. And they just keep coming, opening one after the other.

It is a normal everyday experience to see food on the tv all the time, to smell it in the street every day, but, at this time of year, it is on another level! The Christmas adverts for food on tv come thick and fast and are even more tempting than ever! And then walking through the high street, it’s just food stand, after food stand, after food stand; one stall has the biggest hot dogs I have ever seen in my life, another with roasted chestnuts, one selling roast turkey rolls and then one selling fresh donuts. When it’s dark, cold and you’re out Christmas shopping; there is this whole Christmas atmosphere, with Christmas trees, Christmas lights, Mariah Carey blaring through speakers hidden under a Christmas tree and then the amazing smell from all those food stands. How am I not supposed to be tempted by all that food?! Especially because a few Christmases ago, I wouldn’t of thought twice about bagging myself a hotdog at the very least!

Still here I am, and I know better than anyone that I am a hundred times better on the PKU diet than I am off it! It’s very hard for anyone who doesn’t have PKU to understand how hard it is to fight that moment of temptation. I gave up smoking several years ago after many failed attempts. I finally achieved it by disassociating myself with anything involving smoking. But you can’t do this with food.

I can often find myself watching total strangers as they purchase the food I crave. I stand there, glued to the spot, watching them eat it; the temptation building inside me. It’s even harder if you are with friends or family, looking on and watching them stand and order at those Christmas food stalls, soaking up the Christmas atmosphere and getting in the ‘Christmas spirit’. I never feel fully in the moment like everybody else. It’s like I’m on my own, looking through a window, feeling detached from the fun and the atmosphere. This, for me, is the moment where I have a choice, a choice to either be weak or strong. If I’m strong and don’t give in to the temptation, the moment of isolation generally passes quickly. But If I’m weak, then I’m likely to step out from behind the window and get something I shouldn’t, just so I can re-join everyone else.

Every year I am getting PKU stronger, and this year is no different. I continue to learn more about the way I think and how I approach food. It’s a continual learning curve to see how far I can push myself to be more PKU and less ‘normal’.  

I haven’t had turkey at Christmas now for a few years, but the pigs in blankets have always been far harder for me to resist. This year I am going to be saying no to them. It is time to break those chains, to enable me to focus on PKU this Christmas. I have been busy planning my own PKU Christmas dinner over these past few months. After many hours in the kitchen, I have finally come up with something that I can look forward to every year. I’m looking forward to this as my new PKU tradition for future Christmases.

PKU Festive Wellington

Full Recipe Here

A massive thank you to you all for the support this year! I hope you all have a fantastic Christmas, be safe, have fun and I’ll see you all in the new year!