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Care and mobility considerations

Duchenne Muscular Dystrophy

Needs are summarised and described in the text and disabling effects of the Duchene Muscular Dystrophy table (see the link at the end of this section). This can be used to identify what stage the Duchenne Muscular Dystrophy has reached and the typical needs present at this stage.

From diagnosis at age 4- 6 years

Care

In the first few years after diagnosis extra care will be required. The parent or carer will need to help with physiotherapy stretching exercises each day. Most children need to do these exercises and typically they take 20 minutes each day to do them. Over time physical help with some aspects of mobility may be required due to muscle weakness and may include helping the child to climb into a bath if climbing is required or help or support climbing up stairs. These children tend to struggle on the stairs and getting up from the floor. They fall down more than other children and are more easily knocked over by other children in the playground – this is because they do not have the strength in their postural muscles to regain their balance.

If Achilles tendon contractures develop, they will need to apply splints to be worn over night. These are not walking splints and parents may need to help their child to the toilet during the night. The speed and distance the child can walk will reduce over time and they are likely to start using a wheelchair some of the time.

This age group may start steroid treatment to prolong walking ability. The steroids are taken in high doses and may cause behavioural problems. A side effect of steroid treatment is ravenous hunger and children will need to be restrained from eating to avoid significant obesity. Managing the diet to avoid obesity particularly if there are behavioural problems, enhanced or caused by steroid use, may be a significant aspect of care given in some families.

At some stage when the child is aged between 8 and 11 parents may need to help them rehabilitate and learn to walk again wearing long leg callipers (KAFO) as walking aids. This is because their child cannot support their own weight to walk around and walking distance has reduced to a few steps.

Rehabilitation of walking ability when independent walking ability is lost age 8-12

Mobility

Fitting the child with callipers or knee-ankle-foot orthoses (KAFO) may involve surgery to release the Achilles tendon contractures (shortening) and a process of learning to walk again. With the walking aids useful walking ability will rarely be achieved and children will only be able to mobilise short distances. They will need help rising to standing in the aids and negotiating steps is not possible. They will require help to put the aids on and with transferring at other times. They will use a wheelchair some of the time and will need help with all transfers. They should be considered as virtually unable to walk once these walking aids are necessary. Some children will not try callipers or walking aids and will go straight into a wheelchair. Over time, walking with the aids will become more and more difficult and a wheelchair will be used more frequently. An electric wheelchair is the best choice because it offers the capacity for moving around independently. The weak upper limb muscles make an ordinary wheelchair unsuitable. Confinement to the wheelchair almost always occurs by the age of 12.

Care

Care will be required related to the mobility aids, transferring whenever that is required and helping with therapy. Any walking aids or splints will need to be put on in the morning and taken off for washing, going to bed at night and with activities such as swimming. Help with therapy exercises to prevent contractures (shortening) may still be required on a daily basis. Mobilising around the bathroom to wash will not be possible independently and the parent or carer will have to help with this. Children are likely to need help with putting on trousers and shoes but they may still be able to put on their upper garments.

Parents may report difficulties with the layout of the home particularly if they are using a stair lift and problems with transferring their child and their wheelchair to their car to go out. If obesity related to steroid treatment is present this will be more difficult and adaptations to the home such as a hoist are more likely to be required. Upper limb mobility is maintained but the arms are weak and some help with washing and drying may be required at this stage.

A proportion of children may need turning at night from this stage because they are unable to roll over in bed or adjust their position. At this stage they will also require assistance at night to pass urine. This is because they haven’t the physical strength to manoeuvre a urine bottle. Continence aids such as catheters are rarely used by children with muscular dystrophy.

After independent mobility is lost age 8-12

Care

Weakness affects all muscles including the upper limbs. Help will be needed with all transfers because of the effect of weakness of the upper limbs. Parents are likely to have a hoist at home to assist with this. Assistance will be required with transferring, dressing and some aspects of washing and dressing.

Boys will require help at night whenever they want to pass urine because upper limbs are weak and they are unable to lift a full urine bottle. They are likely to require help turning in bed at night and readjusting their position to get comfortable because of muscular weakness.

Spinal surgery may be performed at this time. There may be a step change increase in care needs at this point because the fused spine limits the range of movement of the upper limbs – for example reaching to wash and to get dressed will be limited.

Once the Forced Vital Capacity (FVC) is reduced, night time respiratory support will be used. This is a form of non-invasive ventilation using a tight fitting face or nose mask. The parent or carer will have to fix this on at night and will need to be available to make adjustments through the night if necessary. Some individuals may have ventilation via tracheostomy at a later stage when ventilation is required for night time and the majority of the daytime. It is common for weight loss to occur in the later stages of the condition and supplemental feeding may be required. Eating becomes more effortful over time and they develop swallowing problems that can lead to choking episodes. Choking risks and difficulties with swallowing may mean that they physically cannot consume enough calories to support themselves and extra nutritional support is required. This commonly includes gastrostomy feeding. Care required includes cleaning and monitoring the gastrostomy site on a daily basis. Attaching the pump feed to the gastrostomy tube as necessary for feeding and keeping the gastrostomy tube flushed and clear between feeds.

See table - Disabling effects of Duchenne Muscular Dystrophy related to age

Becker Muscular Dystrophy

This condition is less likely to be diagnosed in early childhood unless it is known to run in the family or is severe. There are unlikely to be any symptoms beyond cramps and limb pains in the early years. Motor milestones may be delayed in some children. The principle effects of the condition will be difficulty running, climbing stairs and getting up hills as easily or as quickly as other children, because of relative muscle weakness. There is likely to be some limitation of long walking distance. Postural problems do not develop and normal walking ability is maintained throughout childhood in all but the most severe cases. Regular exercise is encouraged as this can increase muscle strength in the early years. A common problem for these boys in the teenage years is muscle cramps after exercise and this may be relieved by intermittent compression therapy boots (flotrons). These are pain relieving and can be worn as required. A teenager could be expected to decide when to use them and to put them on himself.

Reduced walking ability may be present in a severe case and walking aids, typically a scooter, for longer distances may be used.

Limb Girdle Muscular Dystrophy

Needs are very variable ranging from children with severe disability who were diagnosed in infancy who will never walk or independently self care due to muscle weakness, to children with no or minimal disability who have a normal childhood and no care or mobility needs.

Facioscapulohumeral muscular dystrophy

Childhood onset or Infantile Facioscapulohumeral muscular dystrophy is a severe progressive condition with care and mobility needs very similar to those seen in Duchenne Muscular Dystrophy with the exception that cardiomyopathy is rare in this condition. Care and mobility needs related to profound and progressive muscle weakness seen in this condition are described in detail in the Duchenne Muscular dystrophy Care and mobility section.

Adult onset or mild form of Facioscapulohumeral muscular dystrophy - no care or mobility needs would be anticipated during childhood.

Emery-Dreifuss Muscular Dystrophy

Needs are very variable ranging from children with severe disability who were diagnosed in infancy who will never walk or independently self care due to muscle weakness, to children with no or minimal disability who have a normal childhood and no care or mobility needs.

Congenital Muscular dystrophy (CMD) with protein laminin-A2 (LAMA2) deficiency – the condition is also known as MDC1A, classic CMD or merosin deficient CMD

Care required from diagnosis until age 5 is likely to consist of extra care and attention with feeding to overcome any feeding or swallowing problems and may include anything from special feeding techniques to gastrostomy feeding. Physiotherapy exercises involving muscle stretching to maintain or encourage normal posture. Splints or braces may be used and the parent will have to fit these on a daily basis.

Less severely affected children may learn to walk but may not achieve normal walking ability, for example they may be standing and taking a few steps but not walking a useful distance so may be in the virtually unable to walk category. Children that learn to walk a reasonable distance and maintain walking ability are less likely to develop scoliosis and respiratory complications and would have a moderate rather than a severe condition. These children are still likely to require significant care in the form of physiotherapy exercises and help with braces or splints. Children who do not learn to walk or at least stand by around 7 are likely to require help with all aspects of care and may develop scoliosis requiring surgical treatment, respiratory failure requiring ventilation at night or cardiomyopathy.

Ullrich Congenital Muscular Dystrophy

Care required from diagnosis until age 5 is likely to consist of extra care and attention to prevent contractures (shortening) and support normal development despite muscle weakness. Motor development is likely to be severely delayed. Night time respiratory support may be required as early as the 1st decade of life.

Physiotherapy exercises involving muscle stretching to maintain or encourage normal posture will be done daily. Splints or braces may be used and the parent will have to fit these on a daily basis.

Most children will not learn to walk. Less severely affected children may learn to walk a useful distance but will never achieve normal walking ability and require extra care related to their reduced walking distance and difficulties climbing stairs. Walking ability will be lost 2-10 years later and once lost, considerable care and help with activities of daily living is likely including:

Spinal surgery may be required – these children are likely to have care needs broadly similar to children with Duchenne muscular dystrophy.

Walker- Warburg Syndrome (a form of congenital muscular dystrophy)

Care required from diagnosis until age 5 is likely to consist of extra care and attention to feeding to overcome any feeding or swallowing problems and may include anything from special feeding techniques to gastrostomy feeding. Care may be required related to visual impairment and learning disabilities. Physiotherapy exercises involving muscle stretching are necessary to maintain or encourage normal posture. Splints or braces may be used and the parent will have to fit these on a daily basis. Less severely affected children may learn to walk but may not achieve normal walking ability, for example they may be standing and taking a few steps but not walking a useful distance so may be in the virtually unable to walk category.

Children who learn to walk a reasonable distance and maintain walking ability are less likely to develop scoliosis and respiratory complications. These children are still likely to require significant care in the form of physiotherapy exercises and help with braces or splints. They are likely to require extra supervision related to their learning disability or are likely to require extra help and supervision if they have visual impairment.

Children who do not learn to walk or at least stand by around 7 are likely to require help with all aspects of care and may develop scoliosis requiring surgical treatment, respiratory failure requiring ventilation at night or cardiomyopathy.

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