Managing your GSD

If you have any of the liver GSDs, the main aims of management are to prevent you experiencing hypoglycaemia (low blood glucose level) and to help you achieve normal growth and a healthy lifestyle. In a lot of cases, all of this can be successfully achieved through careful dietary control. In some cases, you may need medications or other medical treatments alongside your dietary management. Some centres recommend monitoring of your blood glucose levels although others think it is better to monitor symptoms and behaviour as a guide to your blood glucose levels.

A key thing to remember is that the management plan for you will be individualised to meet your needs and lifestyle.

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Regular eating

The most important aspect of managing a liver GSD is to avoid long gaps between meals and snacks. If you have a liver GSD, your Specialist Metabolic Team will advise you on the longest gap allowed between meals. In addition to regular meals and snacks during the day, it may be necessary for you to eat cornstarch. During the night, an overnight feed or some overnight starch may be required. 


Cornstarch is another name for the cornflour you find in supermarkets. When eaten in its raw, uncooked state, it is slowly digested. This leads to a slow, steady release of glucose into the blood. This means it can keep blood glucose levels within safe limits for longer than eating other foods would. In the last ten years, Glycosade, a physically altered cornstarch has become available. The Specialist Metabolic Dietitian and Doctor will advise on which starch is best for you and how much to use.

The number one rule with cornstarch usage in GSD is that you must not heat it, as this will reduce its effectiveness. 

The simplest way to consume cornstarch is to mix it with cold water and drink it immediately. Some people prefer to mix it with different foods or drinks to make it easier to take. If you have a liver GSD, you should always check with your Dietitian before adding it to anything -  the food or drink you want to add it to may not be suitable for you.

Take a look at this page if you're already using Glycosade.


Already using Glycosade?

Tube Feeding

It may be the case that a feeding tube is required by individuals with GSD to help them maintain their blood glucose levels within the safe range. This will stop the body producing lactates and urates.

If you have any questions about tube feeding, please speak to the Specialist Metabolic Team.

Managing illness

 If you have a liver GSD and become unwell, it becomes harder to keep your blood glucose levels within the safe range. If you are vomiting and/or have diarrhoea, this is especially true. If you are unwell, an emergency regimen or ER is required.

ERs are glucose polymer drinks. Glucose is a sugar and is therefore very sweet. Glucose polymers are lots of individual glucose units joined up together. Glucose polymers give you all the energy that you find in sugar but without the sweet taste.

Normally you would make an ER by mixing a glucose polymer powder with water. The Specialist Metabolic Team will provide you with more information on what an ER is and how it should be used. You should keep the recipe for your ER that your Specialist Metabolic Team provides in a safe place that is easy to find when an emergency arises.

ERs are individualised and they will change as you get older. When a recipe for an ER is updated, discard the old recipe. 

If an individual with GSD cannot drink their ER or if they are vomiting after taking it, they need to go to hospital and be given an intravenous dextrose infusion (sometimes called a ‘drip’) to keep their blood glucose levels within the safe range. The ‘drip’ will be stopped when the person is eating and drinking normally again.

Some healthcare professionals will have little or no experience of GSD so, if you are taking a sick child with GSD to a hospital, it helps to bring all your paperwork to help inform the Doctors and Nurses there. This will typically include written explanations of GSD and contact numbers for your Metabolic Team, so that the ‘drip’ is started without delay. This can be particularly useful if your child, in the case of an emergency, needs to be taken to a smaller, local hospital who are not familiar with your child.

Recognising hypoglycaemia

If you have a liver GSD, your friends and family may easily recognise when you are becoming hypoglycaemic (low blood sugar levels). Your behaviour may be different to normal and you may also become sweaty or pale. There can be different reasons for hypoglycaemia:


Using blood glucose monitors at home is not recommend by some centres as they are unreliable when blood glucose levels are low. In hospital your child will be monitored on machines that are more accurate.

If a hypo is suspected, food or, in the case of illness, an emergency regimen (ER) should be given without delay. If hypos are occurring regularly, the Specialist Metabolic Team should be contacted.