Occupational therapy and Duchenne muscular dystrophy - the early stages

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Information on occupational therapy for the early stages of Duchenne muscular dystrophy.

(Numbers shown in brackets refer to the source of the information. Download this reference document to look at the publications list: OT_DMD_References.doc (23 kb) [doc])

Contents:


The importance of exercise

The most effective way of delaying deterioration in muscle strength is active exercise although care must be taken to ensure that the child is not over-fatigued. He should be encouraged to walk for as long as possible to maintain muscle tone; however, his walking may be laboured. As mobility decreases it is important that a low-fat diet is encouraged to prevent excess weight gain, which would increase the management difficulties.


Provision of bike/buggy/transit and/or lightweight self-propelled wheelchair

A low-geared tricycle may be useful as an interim mobility aid provided that the child is not too large and still has trunk support. However, some children never achieve the ability to ride a tricycle. A powered tricycle, used under supervision, may be helpful for enjoyment and to provide both active and passive exercise. In this respect, it may be particularly satisfactory used in the school playground as an alternative to unsuitable PE activities.

In view of the early mobility problems, a boy with Duchenne muscular dystrophy may use a baby buggy for much longer than his unaffected peers. However, the appropriately timed supply of a properly assessed, lightweight active-user manual wheelchair from the local statutory wheelchair service is a more suitable option. This will enable him to propel the chair himself during such activities as playing outside with friends, in the school playground or shopping. In addition, there will be the psychological advantage of independence, better support and positioning, and the opportunity of playing safely while actively exercising his arms (3).

Passive exercises and night splints

From the time of diagnosis, passive stretching exercises are taught by the physiotherapist to minimise the development of contractures in upper and lower limbs, which can cause functional problems. At every stage, liaison with the physiotherapist is very important, particularly as the physical management will influence the provision of equipment. The wearing of night splints or ankle–foot orthoses in conjunction with passive stretching is the most effective way of reducing the development of contractures (4). It is essential that the night splints fit properly, and are comfortable and lightweight. Early introduction is likely to result in greater compliance.

School - Statement of special educational need.

“Up to a third of boys with Duchenne muscular dystrophy have some degree of intellectual impairment … this intellectual impairment is not related to muscle weakness and is not progressive” (5).

Some affected boys may show challenging behaviour. All affected boys have progressive muscle weakness causing increasing functional and mobility problems.

In view of these difficulties it is important that the procedure for providing a statement of special educational need is in place, preferably before the boy starts school, so that the appropriate level of support is available. The statementing process is as follows:

  • pre-statement stage;
  • preparation of statement;
  • agreement of statement;
  • annual reviews

The practical issues to consider are access to buildings, in order for the child to partake in the curriculum and extra-curricular activities, in addition to the provision of a classroom learning support assistant and specialised equipment. The child should receive physiotherapy treatment at school, monitored by a physiotherapist. Suitable transport in which the child can travel to and from school in his wheelchair will be necessary.

Other issues to consider are the child’s and his parents’ concerns and wishes, liaison with the school staff and other professionals, acknowledgement of changing needs, focus on achievement, and disability awareness. These issues will inevitably result in discussion in relation to mainstream versus special education. The Muscular Dystrophy Campaign has a fact sheet on ‘Children with muscular dystrophy in mainstream schools’(6) and is publishing Guidelines for Inclusive Education(7) in June 2004. This guidance covers all aspects of provision, from adaptations to psychological issues, and includes resources for both parents and teachers.

School - planning and provision of adaptations

If at all possible, the child should remain with his peer group so that he has their support during the transition from walking to wheelchair use(8). Since the implementation of the 1981 Education Act and the most recent 1993 Education Act, this will often mean planning within the context of the child’s local mainstream school.

School - angled writing surface and suitable pens and pencils

The proximal weakness of the shoulder girdle and the deterioration of posture, both affect handwriting, leading to fatigue and reduced control. Consideration should be given to spreading throughout the day those activities that require manipulation and handwriting skills, to minimise fatigue(8).

There is evidence to suggest that use of a sloping desktop can improve posture for writing in the general primary school population(9). Children with Duchenne muscular dystrophy are at risk of developing asymmetries and spinal deformity; therefore, introduction of an angled writing surface can help to promote good posture for writing at an early stage. A sloping desktop can also assist in overcoming the early effects of shoulder girdle weakness, as it raises the working surface slightly and assists in giving proximal stability. Several different types of angled writing surfaces are commercially available.

Consideration should be given to the provision of either a fatter pen or pencil, or a grip to enable the child to hold and control it more easily.

School - seating

Suitable seating should be provided in school from an early stage. The school chair should be supportive and fully adjustable, providing adequate back, arm and foot support. When sitting, it is important that the ankle is kept at a right angle to prevent plantar-foot deformities; where necessary, this can be achieved by introducing a simple wooden block. In the context of writing, attention to seating is important on the basis that if the pelvis, lower limbs and trunk are not supported, upper limb function will be compromised.

In addition, there is the previously mentioned risk of developing asymmetry and spinal deformities; although good seating will never prevent these problems, the age of their onset and their severity can be influenced.

School - introduction of keyboard skills

Difficulties with handwriting arise when deterioration in proximal stability at the shoulder girdle begins to influence motor control, affecting the quality and legibility of written work. Proximal muscle weakness in the upper limb may also lead to fatigue where sustained handwriting is required. Specific learning difficulties can sometimes be an additional factor.

The occupational therapy treatment plan should include preparation of the child and his family for decrease in motor ability and provision of a means of recording on paper that requires a minimum of motor effort before that provision becomes essential. It is advisable to learn keyboard skills while handwriting is still a useful skill (10). Ideally, introduction to keyboard skills should be initiated as part of the child’s individual education plan, with liaison between teaching staff and the occupational therapist. There is an ever-increasing selection of computer software available, ranging from keyboard familiarisation games to touch-typing programmes, which allow the child to progress at his own pace. Children generally find this style of learning more acceptable and enjoyable than formal typing tuition. It can be easily accommodated in most schools and carry-over into the home is often possible.

School - provision of IT support

Initially at primary level, and later at middle school level, additional use of the existing classroom/school computers may meet the child’s needs. However, the work surface must be at a suitable height, and the keyboard must be positioned to allow the child to stabilise his forearms on the work surface, or his ability to function will be severely compromised. This cannot always be readily achieved, as school computers are frequently positioned on standard computer trolleys, which are often not adjustable in height and do not allow for wheelchair access. Where environmental factors, such as physical location or class size, preclude use of the existing school computers, or where the degree of need or volume of work dictate, provision of a personal alternative to handwriting should be considered: ideally, this should be a laptop, which has the convenience of being easy to move between classrooms.

Each Local Education Authority will have its own procedure for undertaking assessment. Independent assessment at a specialist centre may be recommended if one exists locally. A multi-agency assessment is advisable, with advice from the occupational therapist on the child’s physical and (possibly) perceptual needs, advice from the school’s Special Educational Needs Co-ordinator on the child’s educational requirements and subsequent recommendations from an IT specialist with regard to the most appropriate hardware and software to meet these needs. The Local Education Authority should then consider provision of the equipment.

If an application is made to the Joseph Patrick Trust (JPT), which is the grant-funding arm of the Muscular Dystrophy Campaign, complementary equipment can be supplied for home use. JPT supplies some funding, and then refer the application to the Aidis Trust, who subsequently consider provision of the equipment.

School - difficulties and solutions

In the early stages, difficulties within the school environment usually present as follows:

  • unsteady gait;
  • frequent falls and problems in rising from the floor;
  • tiredness on moving from one part of the school to another (e.g. classroom to school canteen);
  • problems in rising from the toilet;
  • inability to carry a lunch tray;
  • difficulty in dressing/undressing (including at a later stage dealing with orthoses such as calipers and spinal jackets);
  • difficulty in participating in PE activities


Initially these difficulties may be overcome in the following ways:

  • providing a classroom learning support assistant to help with mobility and personal care (e.g. when using the toilet or at lunch times);
  • where possible planning the school timetable to limit the need to move between rooms and avoiding rooms approached by steps or stairs;
  • providing handrails adjacent to the toilet, or the use of a toilet that has already been adapted for disabled people;
  • allowing extra time to move between rooms, thus enabling the child to leave before his peers, in order to avoid busy corridors and the risk of being knocked off balance;
  • adapting the child’s role in PE activities so that he does not feel excluded (e.g. using him as a scorekeeper or referee).


Home - emotional support

The stress caused by the diagnosis of Duchenne muscular dystrophy and the subsequent progression of the condition can cause widespread problems in the family. There may be feelings of guilt, particularly in the mother if she is a carrier. The initial shock of the diagnosis may be followed by feelings of anger, anxiety and depression.

Problems may develop within the marital relationship, with parents finding it difficult to express and share their emotions. In addition, the effects on the siblings must be considered (11).

Parents may attempt to compensate the child for their feelings, by over-protection and indulgence; consequently, the child may be restricted in his emotional development and become timid and hesitant. In some children this over-protection only adds to the frustrations caused by the physical disabilities and their behaviour may become aggressive or uncooperative. The parents should be encouraged to express their problems openly and discuss their feelings. Contact with other families may be helpful to some parents and referral to a counsellor may be appropriate at this stage. In addition, the Muscular Dystrophy Campaign employs Family Care Officers to provide support and to refer children and their families to other appropriate professionals and sources of help. Contact can be made through the Muscular Dystrophy Campaign office in London. Paediatric occupational therapists must be aware of the importance of developing their own counselling skills and, where possible, in arranging to attend an accredited course.

Home - Benefits advice

The Disability Living Allowance is divided into two components - the care component and the mobility component. The care component may be awarded at low, middle or high rate, depending on the amount of help needed; the mobility component may be awarded at low or high rate, depending on the level of mobility.

Eligibility is based on the child having more difficulty and needing more assistance than an unaffected child of the same age. Parents may apply for the care component as soon as the diagnosis is confirmed. The child must be three years old to be eligible for the mobility component. It may be useful for parents to ask a professional who has knowledge of Duchenne muscular dystrophy to help complete the application form.

Planning and provision of major adaptations

Adaptations should be planned sufficiently early for the facilities to be available by the time that the child cannot climb the stairs. Usually, this will mean that a referral should be made to the Social Services Department when he is six years old. It is not too pessimistic to anticipate that the whole process will take an average of two years. The aim should be to plan for the long term, as piecemeal adaptations tend to be more expensive than when all the work is carried out at the same time; they also subject the family to recurrent upheaval in their home and accentuate the deterioration of the child. In addition, the initial building work may make the optimum future solutions impossible structurally. The aim should be to have the adaptations ready by the time that they are needed. However, if there is a delay, it may be helpful to provide the following three items in the interim period:

Additional banister rail
The child will find it easier to lean on a rail with downward pressure than to reach up, and the optimum height should be determined for each individual child. He will climb and descend with one foot at a time, placing both feet on each tread; without an additional banister, when he goes down the stairs he will lean on the wall for support.

Bath aid
Getting in and out of the bath independently will become impossible without help and, if he is heavy to lift (particularly out of the bath), a means of getting him up from the bottom of the bath will be a great help. There is an extensive choice of elevating bath seats, but it is usually adequate to use the model that the Social Services Department holds in stock.

Toilet rail
This should be horizontally and vertically adjustable, so that the rail can be positioned as close as the child needs it and at the optimum height. However, the rail is unlikely to be needed in the long term and eventually will restrict circulation space around the toilet.

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