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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 football

I have something called Glycogen Glyku jun Storage Disease there are lots of different GSDs for the liver and for muscles My GSD is a liver type called Type 1a It is a long and confusing name so I just call it my GSD Not many people in the world have GSD 1a so people don t know much about it I have had my GSD forever so I thought it would be cool if I could explain what it is to other kids with it or anyone else who wants to know more about it I hope you enjoy my story

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 my tummy got really big The doctors and nurses got really worried because they didn t know what was wrong with me One day I started to shake a lot and an ambulance took me to a big hospital There were lots of machines and doctors but I don t remember any of it That is when they found out I had GSD 1a

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 stuff our body needs energy every single second of the day We get this energy when we eat food But we can t eat little bits every second of the day or we wouldn t have time to go to school or play So when we have a meal our body puts a bit of energy in a safe place to use when we are not eating This safe place is called the liver

This is where the liver is circled in green The liver does loads of different things actually But I m just going to talk about how it takes care of our energy

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 we need it This is just what our liver does too we just don t feel it But did you know that food gives us different types of energy The important type of energy in food to remember is a special sugar called glucose

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 wants more glucose

When there is spare glucose from a meal it goes to the liver and the muscles This means we can have energy ready for when we are not eating such as between breakfast and lunch My GSD is about the liver not the muscles But now it gets a little bit confusing When glucose goes to the liver it changes its name to Glycogen Why does it do this

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 away for safe keeping If you need to use this energy you need a key Turn over and see how this happens you just need to start at GO

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 off it goes to your liver to be glycogen 6 Now you have some energy you carry on playing until its time for lunch Then it all starts again

3 You have been running round loads and start to feel hungry But you can t eat lunch until later 4 So you get your key out to unlock the door of the liver GLY 5 The door opens GLY Glucose You use me as energy To let out

Because I have my GSD 1a my body can t use energy like other people Something is missing in my body s kitchen cupboard This is not my fault I was just born like that So if I have spare glucose it goes into my liver just fine The door is always open for me to store spare energy You can see this over here

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 that is needed to let any glucose out It s just like someone has put all their food shopping in their kitchen cupboard locked it for safe keeping and lost the key This means that 1 Once glucose energy is in my liver I can t use it so I quickly run out of energy 2 If I kept putting glucose in my liver would keep getting bigger and bigger with glycogen and start to break

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 energy in my body I need to eat more regularly If I don t do these things I start to feel low When I am low I feel weak and wobbly and I need to eat something quickly or else I would get poorly

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 good shake before drinking it It looks just like milk and I don t think it tastes bad

Cornflour is special because the spare energy it gives me doesn t go into my liver like other foods Instead it stays in my tummy and is let out slowly for me to use It doesn t make my GSD go away but it helps keep me going until my next meal

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 it They can have a tube in their nose that goes to their tummy called a naso gastric tube You then put the cornflour down this tube instead of drinking it I had one when I was a toddler Look

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 if it is sweet so stuff like cake fizzy drinks and fruit Milk and yoghurt isn t good either because it has lots of a sugar like glucose that goes straight to the liver If I eat foods like this my body can panic and make me go really low

Foods that are good for me are things like bread meat fish vegetables and my cornflour meals I quite like salty foods like olives too Because I can t really eat certain things everyday I take a multivitamin to keep me fit and strong

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 fizzy drink at the cinema But when I really think about it it is horrible feeling poorly and being ill can stop me doing other fun stuff If I do want something sweet my mum adds some lemon to my food which my liver doesn t mind

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 my blood glucose level Sometimes I do it myself If the number is below 4 I need a snack or some cornflour quickly Not everyone with my GSD needs to check their blood glucose levels it just depends on you and what your doctor says

At night time my GSD doesn t go to sleep like me Even though you are asleep your body is still working away making sure it is ready for the next day But because you don t eat when you are asleep your spare energy from your liver is VERY important This means I need to have my extra energy like I do in the day time It would be really annoying if I had to get up lots in the night to drink cornflour So my doctor gave me a little button on my tummy called a Mickey

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 energy that I hang on a stand next to my bed

When the bag of glucose energy is attached to my Mickey button safely I am ready to go to bed The special glucose then slowly drips through the tube and into my tummy through the Mickey It goes all night so I don t go low on energy I don t feel it going in but I know it is because I wake up feeling just fine The reason I don t have cornflour running through is because it is quite thick and might get stuck

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 was in But now my mum or dad can change it at home every 6 months I always get scared but they make sure it doesn t hurt by putting some magic cream on They also have to clean it every day and cover it with a plaster to make sure no bugs get in

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 have blood tests to make sure my liver is happy and that everything else is healthy I get weighed and measured to make sure I m growing well and sometimes I even have a tummy scan

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 in different places each year They call it a conference Here I get to meet lots of other children and adults with GSD

Some of my friends have Mickey buttons just like me and some also have a naso gastric tube It is great seeing lots of people around me drinking cornflour because it makes me realise I am not alone There are also lots of doctors there from around the world who want to make people with GSD better forever

Living with my GSD can be hard sometimes Because I don t look poorly to other people it is sometimes difficult to explain how important it is for me to eat the right foods at the right time My doctor has given me a sheet of information to give to people like my teachers which explains what it means to have GSD and what I need to do every day to stay well

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 them this story I read it with my mum dad and Charlotte

It is important to remember that not everyone with GSD 1a is the same We all have the same condition but our bodies deal with it differently This means we all need to have slightly different treatments which are unique to us For me I find that if I eat the right things at the right time and go to see my doctors I can do everything that my friends do I play football everyday at school and when I grow up I want to be a doctor to help other children just like me

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 junior doctor at the Royal Cornwall hospital She has had a long standing interest in paediatric endocrinology since being diagnosed with type 1 diabetes She was also greatly inspired by a friend who had GSD 1a and who s courage never let the disease hold her back

There are many different types of Glycogen Storage Disease To find out more about GSD you can always contact us or visit the charity website Phone 0300 123 2790 Email info agsd org uk Website www agsd org uk