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A child’s eye view of living with GSD1a.

By Dr Gabriella Clark
Hello Everyone  My name is George and I am 9 years old. I live with my mum, dad and sister Charlotte and I love to play fo...
I have something called Glycogen  Glyku-jun  Storage Disease, there are lots of different GSDs for the liver and for muscl...
When I was born I was a strong and healthy baby but I soon started getting very poorly. I was being sick all the time and ...
Lots of people ask me what my GSD 1a actually is, and this is what it means to me... So that we can run around and do stuf...
This is where the liver is, circled in green. The liver does loads of different things actually. But I   m just going to t...
I think of my liver as a kitchen cupboard. We store food in our kitchen cupboards and take it out to give us energy when w...
I am Glucose  I am the energy you get straight away from most foods you eat  If you are hungry    it   s because your body...
When there is spare glucose from a meal, it goes to the liver and the muscles. This means we can have energy ready for whe...
I am Glycogen  I am glucose that rests in the liver. I have a different name because your body needs to know I am locked a...
GLY  GLU GLU  2. The door of the liver locks.  GLU  1. You have spare glucose energy in your tummy from a big lunch  So of...
3. You have been running round loads, and start to feel hungry    But you can   t eat lunch until later   .  4. So, you ge...
Because I have my GSD 1a, my body can   t use energy like other people. Something is missing in my body   s kitchen cupboa...
I am Glucose   I am Glucose   I am Glucose   I am Glucose  I am Glucose
BUT, once the glucose has gone into my liver, it gets stuck. This is because I don   t have that key I showed you earlier ...
THERE IS NO KEY     WE ARE STUCK     GLY  GLY  GLY  GLY  GEORGE IS RUNNING OUT OF ENERGY     MAKE ROOM FOR ME
This means I have to eat special food that doesn t need to be stored away in my liver. Also, because I don   t have spare ...
So you are probably thinking there isn   t much I can eat...  WRONG    I can still eat LOADS of different things
The food I have the most, about five times a day, is called uncooked cornflour. You mix it with water and give it a really...
Cornflour is special because the spare energy it gives me doesn   t go into my liver like other foods. Instead it stays in...
After I have my cornflour I tell everyone I have corn powers because it gives me loads of energy
Some people with GSD don   t like drinking cornflour at all. They don   t like the taste or they don   t like to swallow i...
Some foods are bad because they have too much glucose that loves hiding in my liver. I usually know a food is bad for me i...
Foods that are good for me are things like bread, meat, fish, vegetables and my cornflour meals. I quite like salty foods ...
Sometimes it is difficult having my GSD. It makes me feel sad or a bit angry when I can   t eat birthday cake or have a fi...
My mum and dad also have to prick my finger a couple of times a day to check my energy levels in my blood. This is called ...
At night time, my GSD doesn   t go to sleep like me  Even though you are asleep, your body is still working away, making s...
When I go to bed my dad puts a small tube on the Mickey button which connects to a bag of special night time glucose energ...
When the bag of glucose energy is attached to my Mickey button safely, I am ready to go to bed  The special glucose then s...
When I first had the Mickey put in, I went to hospital. They gave me medicines that made me sleep, and when I woke up it w...
I sometimes have to go to the hospital to meet my doctor or a nurse who tells me what I need to eat to stay healthy. I hav...
Not many people have GSD1a, but I know a few  Since I was young my mum and dad have been taking me to a fun weekend away i...
Some of my friends have Mickey buttons just like me, and some also have a naso-gastric tube. It is great seeing lots of pe...
Living with my GSD can be hard sometimes. Because I don   t look poorly to other people it is sometimes difficult to expla...
This is really helpful, but it is more for adults and my friends get confused if I show it to them. So instead I will show...
It is important to remember that not everyone with GSD 1a is the same. We all have the same condition, but our bodies deal...
I hope my story has helped you to understand a little bit more about my GSD and Me    The author, Dr Gabriella Clark, is a...
There are many different types of Glycogen Storage Disease. To find out more about GSD, you can always contact us or visit...