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HARRISON FORD IN GSD FILM Extraordinary Measures starring Harrison Ford, based on the book "The Cure" it tells the story of John Crowley and his part in funding the development of an enzyme-replacement therapy for people with Pompe Disease. Two of John's children suffer from Pompe (GSD Type II). UK release 26th February.
MCARDLE HANDBOOK PROJECT Thanks to the Vodafone Foundation Kathryn Wright is preparing a Handbook for people with McArdle's. More details here.
The human body is very complex and we are all different. However, some general points can be made and the following advice should be useful to the majority. This page is written by Andrew Wakelin, the AGSD Type V representative, who himself has McArdle disease.
Pain killers There are two phases of pain experienced in McArdle disease. Firstly, the pain experienced on exercise. Secondly, the pain which can be experienced after damaging the muscle through execessive exercise and experiencing a fixed contracture (cramp). During activity or exercise it is very necessary to experience the pain as this gives feedback about how the muscles are coping. The pain starts in a gentle way and gradually increases. It can be managed by lessening the level of exercise, for example by slowing down when walking. Or if the pain starts to build up quickly it is necessary to stop and rest until the pain eases off, then start again. If pain killing drugs are taken there is a significant danger that they will interfere with this vital feedback process, which could lead to excessive activity and damaging muscle cramps. In the event of an activity being, or becoming, anaerobic and causing a muscle cramp there will be pain, possibly severe pain, for some hours or days thereafter. In these circumstances pain killing drugs would be effective and, once activity has finished, there is no concern about masking any feedback.
Summary
Tell your friends and colleagues It is best to have the understanding of your family, friends and work colleagues. And, of course, they can't understand if you don't explain your condition to them. You may feel a bit awkward at first, but you will soon be able to explain in just a few sentences and you won't think anything of it. You need to avoid doing anaerobic activity such as lifting or pushing heavy things. Some very ordinary activities are anaerobic - like standing on tiptoes to put something into a high cupboard. Do that for more than a few seconds and you can cause a muscle cramp. So get someone else to do these things for you! If you have explained your condition to friends in the past it is then much easier to ask for their help when the occasion arises. Aerobic activity like walking is very good for you. But just a little too fast, up too much of a hill, or carrying a little too much, and the activity turns anaerobic and can cause you pain and muscle damage. If you are walking on your own it is easy to slow down or stop and rest to avoid damage. But if you are with friends or work colleagues who do not understand your McArdle disease, it is tempting to say nothing and keep pressing on. If they are in a rush you can end up in severe pain and holding them up anyway. So much better to have told them beforehand, then when you need a rest just say 'McArdle's' and stop. They will soon get so used to it that they won't even comment.
Explaining about your condition When you tell someone about your McArdle's it is a good idea to describe it as a muscle condition rather than a muscle disease. That way they won't think that they are going to catch it. If you explain that it is a genetic condition they will understand that it is just bad luck that you got it at conception, rather than it being in any way down to anything you have done or not done. If people want to know more you can explain that the main symptom is pain on exercise and in everyday activities, but that you can do pretty well if you go at you own pace and avoid things like lifting heavy objects and running. Say that if you overdo it you can get very painful muscle cramps which last for hours or days. If they are severe you can experience muscle breakdown with an associated risk of damage to the kidneys. If they still want to know more, suggest that they read the McArdle's section of this web site.
General anaesthetic If you need to have a general anaesthetic, for instance in order to have surgery, you should tell the surgeon and the anaesthetist that you have McArdle disease. This is because with McArdle disease you have an increased risk of a problem called 'malignant hyperthermia'. The risk is incredibly small but it makes sense to be take care. Malignant hyperthermia is a sudden and severe condition in which your body temperature rises and keeps rising. It can be life threatening. However, given prior warning the anaesthetist can choose the most appropriate anaesthetic with a lower risk, monitor you carefully for any signs and be ready to take appropriate action if necessary. In some instances it might be possible for the anaesthetist to choose an alternative type of anaesthesia such as an epidural. With local anaesthetics there is very little risk of malignant hyperthermia, but there is some thinking that certain local anaesthetics such as lignocaine (lidocaine) are best avoided. The most common reason for many of us to need a local anaesthetic is for dental work - so also tell your dentist about your McArdle disease.