making muscle wasting history

THE USE OF STEROIDS
One option for maintaining and prolonging mobility is to consider the use of steroids. In some studies steroids have been shown to help maintain muscle strength and prolong ambulation but they are not without side effects. A large scale study of the use of steroids in children with Duchenne is now in progress. Steroids can be used in quite young boys with Duchenne. If the use of steroids is of interest to you talk to your child’s consultant about it. The Muscular Dystrophy Campaign publishes a factsheet on steroids and on some of the questions you might like to ask your consultant about their use. Prior to starting steroids certain tests and checks must be completed. Children on steroids should always be monitored regularly by the medical team and side effects noted and discussed. There is some evidence that steroids may aid heart and lung function too.

ORTHOTICS (SPLINTS)

To try to prevent contractures at the ankles your consultant or physiotherapist might suggest that your son wears night splints.These are lightweight splints made by an orthotist and are designed to be worn only at night.They hold the ankle in a neutral position (that is, at 90 degrees or as close as possible) and support the foot and calf areas. Some children find night splints uncomfortable, especially in hot weather. If your child cannot tolerate wearing them at night you could compromise by suggesting he wears them in the evening when watching television, or on one leg one night and the other leg the next night. Night splints are not designed to be walked in. A little later, when even walking around the house becomes a struggle, the use of long leg callipers might be discussed with you. These are sometimes known as KAFOs (knee ankle foot orthoses).They support the legs and can help prolong walking for up to 18 months. Callipers are worn under trousers and extend from the foot to the hip. They are fairly lightweight. The child’s style of walking will be different in callipers – he will walk with straight legs and swing from the hip, perhaps holding onto someone’s hand for extra support. Callipers are designed for walking short distances, mostly indoors. If a child cannot get their foot flat on the floor it may be necessary to do a small operation under a general anaesthetic to release their Achilles (ankle) tendons before supplying callipers. This will entail a stay in hospital and intensive physiotherapy. Remember that using callipers is an option for some children but it is not a “must do”. Callipers are used most successfully in children who are able to comply with a programme of physiotherapy and who have a strong desire to continue walking.

STANDING FRAMES
Once walking becomes difficult it is important to continue to encourage standing if possible. Standing helps maintain range of movement in the joints and good posture. It is also beneficial to the function of the bladder, the circulatory, digestive and respiratory systems. If a child can still take weight through their legs, having the ability to stand also makes transfers easier. Standing frames and tilt tables (supplied on the advice of your physiotherapist) are available to support a child in a standing position. Some people may make use of a sophisticated wheelchair with a built in standing facility. Ensure that you take expert advice prior to purchasing specialised equipment like this as contractures at the ankles, knees or hips may mean that such a facility cannot be used safely.

PAIN
Pain is not usually a very significant feature of Duchenne, even in much later stages of the condition. Some boys of primary school age will occasionally complain of muscle cramps in their legs.There is no specific treatment – warm baths and gentle massages generally help. Discomfort can be felt if joints become stiff – stretching exercises (as shown by a physiotherapist) should help prevent this happening.

VACCINATIONS
We are keen to prevent boys with Duchenne developing chest infections. For this reason the consultant may recommend an annual flu vaccination and a pneumococcal vaccination (you will need to check with your child’s consultant about when, or if, this needs repeating). If your child is being started on steroids and he has not had chickenpox, he may need a blood test, and if he is not immune,may need chickenpox vaccine before starting the steroids.

CHEST INFECTIONS
Chest infections usually need to be treated aggressively with early antibiotics and chest physiotherapy.Your son’s GP should be made aware of this by the consultant. On occasions, admission to hospital (especially for older boys) may be necessary if the chest infection becomes very troublesome or does not respond to the antibiotics.

VENTILATION
As chest wall muscles become weaker, in order to aid breathing which is shallower (hypoventilation),many teenagers (and occasionally boys a little younger than this) may need to use non-invasive ventilation at night.This involves the use of a ventilator and facial mask. It does not involve any sort of surgery and is not painful. Non-invasive ventilation can
be enormously helpful in maintaining good respiratory function and in fighting off chest infections. Lung function is usually monitored by your son’s neuromuscular consultant and if there are concerns, a sleep study (done overnight) may be recommended. If this shows that your son would benefit from using a ventilator, further discussion will be arranged with the relevant respiratory consultant. A sleep study involves monitoring oxygen levels in the blood by using a clip on the finger – it is not painful.

ANAESTHETICS
Children with Duchenne are likely to have reduced respiratory function, and where possible general anaesthetics should be avoided. If a child does need a general anaesthetic the anaesthetist must have advance knowledge of the child’s medical condition. Lung function should be tested. Ideally, the child’s neuromuscular consultant should be informed. The Muscular Dystrophy Campaign publishes a factsheet on anaesthetics which gives general advice.

HEART MONITORING
The heart is a muscle and it is important that its function is monitored over the years. Weakening of heart muscles occurs in boys with Duchenne but usually only late in the course of the condition. Most children with Duchenne will not be aware of any problem with their heart but if a problem is identified advice can be sought and medication may be offered. In some clinics boys may be offered early medication to boost the pumping function of the heart, even before there are any symptoms of weakening heart muscle function.

CONTINENCE

Most children with Duchenne do not experience problems with bladder or bowel incontinence. There is usually no loss of sensation in Duchenne and children will generally be aware of when they need the toilet. Some children (especially if they have learning difficulties) may be late in achieving continence and a few will have problems in the longer term. As a child becomes less mobile, accessing the toilet facilities quickly enough can be an issue, particularly in school. The best way round this is to establish a regular pattern of using the toilet facilities during break and lunchtimes .Do not encourage your child to restrict his liquid intake or he may become dehydrated. Constipation is a frequent problem for children who are wheelchair dependent. This difficulty can be managed by encouraging a diet rich in fibre (found in foods like bran, brown bread, fruit and vegetables).Doctors can advise on the use of medication to help if necessary. Constipation can be painful and parents should seek help sooner rather than later. Children may be embarrassed about needing assistance to use the toilet. Try to ensure that their privacy and dignity are respected – close doors, only provide the help needed/requested and ask permission before touching them. Remind a child that his body is private and nobody has the right to touch him without his permission. Let him know that if he feels he is being touched inappropriately whilst being assisted to use the toilet, he should share this information with a trusted adult.