General information about mitochondrial disease
Everything that the body does, from staying warm, to moving and thinking needs energy. The energy that we use comes from the proteins, carbohydrates and fats in our food. We are unable to use these directly, but instead need to convert them in to a form of energy that cells can use called ATP. You can think of this like a battery. This converts chemical energy into a different form (in this case electrical) that is easier to use. For our bodies the conversion from food energy to ATP happens in mitochondria. If your mitochondria are not working properly then you are less able to convert food into ATP. For cells that require a lot of ATP, for example your muscles, this is a problem and they may become weaker and get tired faster.
Alcohol
There is no reason why patients should avoid alcohol, but as with all things moderation is advisable. Excess alcohol can damage both the muscles and the nervous system and it important not to add to any damage caused by the mitochondrial disease itself.
Diagnosis
If you or your child is suspected of having mitochondrial disease the doctors will undertake a number of investigations. These will determine the nature and also the extent of the mitochondrial disease.
Diet
Food energy into ATP is a crucial function of mitochondria. You might think that because affected mitochondria are less efficient at making ATP that you could overcome this by eating more. Unfortunately this is not the case and all that this achieves is that you get heavier. Nor does it seem that one type of food is easier to convert into ATP than another, and so high carbohydrate or high fat diets are not of benefit.
Driving
It is important to stress that whilst we can offer advice as to your suitability to drive the final decision lies with the DVLA. Furthermore it is up to you the patient to inform the DVLA of any medical conditions that may affect driving. The DVLA will then send you a questionnaire to fill out and may contact your specialist for an opinion.
Drug Information
In general there are no specific drugs that you should not take, but there are some that should be used with caution. The only exception is an anti-eplieptic drug called sodium valproate. Sodium valproate can interact with mitochondria and is best avoided in other forms of mitochondrial disease where possible.
Education
Some children with mitochondrial disease will attend mainstream school, while others (or their parents) will find the environment of a special school more appealing. In either case, the class teacher and head teacher should be made aware of any physical problems (epilepsy, postural abnormalities, feeding or mobility problems) or learning difficulties the child has acquired as a result of mitochondrial disease.
Employment
Patients with mitochondrial disease have the same employment rights as any other employee although it is likely that their employer will be less familiar with the particular problems encountered in this illness. Clearly individual circumstances differ, but your specialist should be able to advise your employer on your ‘fitness to work’.
Exercise
Because in mitochondrial disease the cells cannot make ATP in sufficient quantity, anything that requires a lot of ATP such as exercising a muscle will mean that the body will not be able to keep up with demand. This has two main effects; firstly the muscle will become tired sooner than normal and secondly the muscle makes other compounds such as lactic acid in an attempt to keep up with energy demand.
Holidays
In general there are no activities that patients should avoid, but clearly a sensible approach is needed when choosing a holiday. For patients with severe weakness and fatigue a hiking trip to the Himalayas is probably not a good idea. Having said that, we always encourage patients to stay as active as possible and are often amazed by what people can achieve.
Monitoring
Mitochondria are found in every tissue in the body and any of these can potentially be affected in mitochondrial disease. By no means all patients will develop complications of their disease and the fact that we sometimes ask for tests does not mean that we think that you have a particular problem. Nevertheless, we feel it is prudent to look for complications that we know can happen because a lot of them can be effectively treated if caught early enough.
Operations
There is no specific risk in having a general anaesthetic. However, it is important that the anaesthetist knows that you have mitochondrial disease as sometimes patients require additional assessments of breathing, swallowing or cardiac function. The anaesthetist can then make a better judgement of any potential risk and advise you accordingly.