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

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 96^th 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 4^th 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