May was PKU awareness month, and this year we have had a considerable boost from the story line on the BBC programme Call The Midwife. It was a sobering reminder of how far we’ve come. And how far we have to go.
The development of a restrictive diet therapy at Birmingham Children’s hospital in the 1950s allowed thousands of people around the world to avoid serious cognitive degradation and lead nearly normal lives.
However, seventy years later, PKU is still treated predominantly by diet therapy in the UK. Given the rarity of the disease, PKU was neglected for years. New therapies have been developed, and some are available now. But not on the NHS.
The NSPKU are continuing to fight for access to treatments. The next NICE consultation is on, and you can sign up to watch it on the NSPKU website.
PKU Awareness month has been difficult for me. My adherence to diet has not been great. It is odd to admit that I’m over 40 and still find myself sneaking food. As a teenager, cheese was my vice. I would sneak it from the fridge thinking it was the perfect crime. I still sneak cheese, though more of a danger recently has been the dark chocolate buttons I bought for baking. No baking has happened and yet: ‘Umm, the level of the chocolate buttons is dropping?’
Guilt and shame flood in.
‘Yes, I have been eating the chocolate buttons… and also your cheese.’
‘There is PKU cheese in the fridge.’
‘I know, but I wanted your cheese.’
Breaking the habit
How did I get into this habit in the first place? I need to know why I sneak food so I can break the cycle. Working from home over the last fifteen months has been a problem as the kitchen is right there! The fridge and cupboards filled with tasty little treats. When I got peckish in the office, I had PKU cereal and biscuits in my desk drawer and nothing else. Now all I have to do is walk downstairs and there is normal cheese, cereal, nuts, chocolate… So will power is definitely needed. And I need to remind myself:
Don’t be a dolphin
Dolphins do not drink, they get all of their liquid requirements from their food. In some cases, a dolphin in captivity will drink from a fresh water hose. But will then not eat for days because they cannot distinguish between hunger and thirst.
After I submitted a food diary, one dietician pointed out that I tend to eat within an hour of a meal. At that time, I am probably thirsty rather than hungry. This is because you do need to take water after most PKU supplements, and liquids are required in the digestion of food.
So, I started drinking more during and after meals. Sure enough, I was making fewer trips to the cookie jar within an hour or two of eating. Now, when I start feeling peckish, I’ll remind myself not to be a dolphin and try a drink first. If I’m still hungry after that, then I will snack.
My other snack tigger is temperature. If I’m cold, I’ll head to the kitchen for hot toast & a cuppa. If that isn’t enough, then I start looking elsewhere for the quicker snacks, regardless of their Phe level.
This is amplified by the menstrual cycle. I know that my non-PKU friends have trouble regulating their body temperature and get the munchies just as their period arrives. It is true for me, but the comfort food is forbidden. Unless I bake it. This month I decided to make cookies, and then ate half the dough before it even saw an oven.
How do you work out the protein for a dozen spoonfuls of raw cookie dough? Add in a couple of salted cashews, some of the husbands far tastier cereal…It adds up over the days and the end of the month has been a bit harder. I have more brain fog, headaches and general grumps.
It’s just one thing after another
The thing about the PKU diet therapy is that you just have to keep doing it. There is no respite. I’ve had a bad month. But I just have to keep going. Until we get other treatments for PKU, there is no choice.
We just have to dust ourselves off and try again. And it is punishing and relentless. But it is that or suffer more.
Dragging yourself out of a downward spiral or back onto the diet therapy is not easy. But for me, and everyone I have talked to, it is worth the effort to discover who you can be.
It is also why PKU needs a treatment which isn’t 70 years old.
Get more treatments on the NHS
This is why the NSPKU and many others are campaigning to get Kuvan and other treatments through the NHS. The next meeting is on the 8th of June and you can register as an observer through the NSPKU website. Please join us!