Occupational therapy and Duchenne muscular dystrophy - the middle stages

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Information on occupational therapy for the middle stage of Duchenne muscular dystrophy. The middle stage covers difficulty in walking and the use of powered wheelchair out of doors.

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

Contents:


Mobility - physiotherapy

There should be an emphasis on the need for ongoing stretches to both upper and lower limbs. If a growth spurt occurs, there should be a regular review of splints.

Mobility - Ischial-bearing calipers

At the stage where the child is finding it difficult to walk, walking and standing may be prolonged for periods of up to 2 years or more, by the use of ischial-bearing calipers, which stabilise the lower limb. This can delay the development of contractures and scoliosis, maintain independence and be of psychological benefit.

In addition, assisted transfers between a chair, toilet or bed, are easier if the knee joint is stable. However, the use of calipers will increase the risk of falling, particularly in the school environment, and supervision will be essential. Alternatives, such as dynamic elastic bracing, may be considered. Sometimes a small surgical procedure is required to release the achilles tendon (heelcord) prior to rehabilitation in KAFOs (knee-ankle-foot orthoses).

Mobility - standing frame

The use of a standing frame should be introduced before a child is no longer able to walk, and is an integral part of the management of Duchenne muscular dystrophy. It is important that the frame promotes symmetry and, as several different types of standing frames are available, advice should be sought from a physiotherapist experienced in the treatment of children with neuromuscular conditions. Provision of an adjustable table for use with the standing frame is recommended.

Mobility - means of getting the child into a standing frame

Children are encouraged to use their standing frames at home. To avoid the need to lift the frame from the floor manually, consideration should be given to the provision of an electric standing frame or a powered tilt table. The latter option has implications for adaptations: there must be sufficient space in the child’s bedroom to use the ceiling hoist to lift him on to the tilt table, and also a suitable place for storage when not in use.

Mobility - powered indoor/outdoor wheelchair with postural support/pressure relief

At this stage the child should be referred to the appropriate wheelchair service for the assessment of an indoor/outdoor powered wheelchair for independent mobility. This chair will be needed in addition to his manual chair, because it is likely that he will be finding it difficult to propel himself very far indoors, and virtually impossible to do so when outdoors. In addition, a seating assessment should be carried out and an appropriate seating system, (which includes a seat and backrest), provided, so that a good postural position can be achieved while ensuring comfort and maintaining function.

Mobility - transport

It will become increasingly difficult to lift the child into and out of the car, or for him to travel in comfort. Many families will consider the need for a wheelchair-accessible vehicle in which the child can travel in his wheelchair; the headroom available, particularly when entering the van, will be crucial. A wheelchair ramp or a tail lift will be required, together with a safety harness and suitable floor-fixing clamps for the chair. A headrest on the wheelchair is essential to prevent a whiplash injury in the event of an accident.

If the child is receiving the high rate of the mobility component of the Disability Living Allowance, this may be used towards the provision of a vehicle through the Motability Scheme. Financial help may also be available, via a Motability grant, to adapt the vehicle.

School - adaptations

In some situations it will be necessary for adaptations to be carried out in a primary and/or secondary school when the parents have chosen to retain their child within mainstream education. The nature and course of the condition make forward planning and preparation for wheelchair use around the school essential(8). Planning takes time, and the cost may have to be built into the budgets. It is important that the school environment is made suitable before the child becomes dependent upon a wheelchair for mobility.

Planning for major adaptations to a school should take a collaborative approach. The ideal solution is to set up a planning team consisting of the young person, his parents, school staff, special needs co-ordinator/advisor, representation from the Education Department (preferably from the Buildings Department), the school Medical Officer, a moving and handling assessor and the occupational therapist. The role of the occupational therapist will be to advise on facilities required by the wheelchair user and on the adaptation of the environment to accommodate a wheelchair. Consideration may need to be given to the following:

  • Wheelchair access
  • Lift or stair climber
  • Disabled toilet facility
  • Hoist
    • Height-adjustable table/table raisers


School - adaptations - wheelchair access

Ideally, wheelchair access should be provided to all areas of the school. This may involve provision of ramps, levelling of floors, widening of doorways, and a lift. In order for a child to attend the local school, families often accept limited routes of access and a restricted curriculum, but this is not recommended. Semi-permanent ramps can sometimes solve the problems posed by single steps into school buildings or classrooms. It is important that the child has access to canteen and playground facilities alongside his peers, as the social aspects of the school day are an important part of the child’s life.

School - adaptations - lift or stair climber

Provision of a ramp, short-rise lift or through-floor lift are the optimum solutions to overcome the physical barrier of steps or a flight of stairs. In schools where one lift will allow access to otherwise inaccessible areas, it will be the most appropriate choice. However, in some cases this is not practical, either because the layout of the building is not suitable or because more than one lift is needed, making the cost prohibitive (12). A stair-climbing wheelchair may be an alternative.

There are three types of stair-climbing wheelchairs:

1. A chair is incorporated into the unit, which is operated by a helper. This type is unsuitable for long-term use by boys with Duchenne muscular dystrophy as it involves transferring the child between the chair and his wheelchair, and carrying his powered wheelchair up the stairs.
2. A self-propelled or small powered wheelchair is attached to a climbing mechanism, which is operated by a helper. If the powered wheelchair used by the boy is suitable, this option may prove satisfactory.
3. One type of powered wheelchair has the additional ability to climb stairs. This type provides independent powered movement to and from the stairs and is suitable for someone unable to propel a manual wheelchair; however, it must incorporate a suitable seating system, and its main disadvantage is the cost.

Any stair-climbing chair can present a potential hazard; the features and safety of the individual products must, therefore, be assessed carefully by potential users, their families and professional advisors. Where equipment is operated by a helper, it is important to consider the ratio between the size and ability of the helper and the size and weight of the user.(12). If such a chair is used independently, it is recommended that this should be with supervision.

Most stair climbers operate slowly and are not suitable for use in an emergency. Where lifts or stair climbers are in use, provision of suitable evacuation equipment should be considered and an emergency evacuation procedure must be put in place.

A secure area will also need to be provided for charging and storage of stair-climbing equipment.

School - adaptations - disabled-toilet facility

Disabled-toilet facility. Long-term provision for a child with Duchenne muscular dystrophy will need to incorporate sufficient space for the following:

  • the child’s wheelchair and a carer;
  • a mobile or overhead tracking hoist;
  • a changing plinth/table (for adjusting clothing);
  • wall-mounted/drop-down rail adjacent to toilet;
  • a height-adjustable washbasin with lever taps.

Many existing disabled-toilet facilities in schools are designed for independent wheelchair users and do not take into account the additional requirements of a child with Duchenne muscular dystrophy. As a result, many boys have a tendency to restrict their fluid intake, which may eventually lead to medical problems including dehydration. If a school purports to have a disabled toilet, it will be necessary to check that this will meet the child’s needs. Ideally, facilities should be planned for multi-use to cater for any level of disability, by using a wall rail that allows the fittings to be moved laterally and with height adjustment (13).


School - adaptations - hoist

A mobile hoist should be considered if the child needs to transfer to and from his wheelchair in more than one area of the school. Where space is restricted and/or transfers are confined to a specific area of the school, an overhead-tracking hoist may be a better option. Models that can be user operated by a hand-held remote control, allow the child to maintain some independence in the transfer.

A sling that, if necessary, includes head support, will be needed. A secure area must be provided for charging and/or storage of the hoist.

School - adaptations - height-adjustable table/table raisers

In the primary school, where lessons tend to be confined to one classroom, a height-adjustable table will be an advantage. It allows wheelchair access, provides arm support at the correct height, and also can enable the child to use a standing frame while accessing curriculum activities.

In a secondary school, where the curriculum may be spread throughout a number of classrooms, use of the child’s wheelchair tray or less-expensive table raisers (which can be applied to existing classroom tables), is recommended to provide a working surface of appropriate height. As muscle weakness progresses to the trunk and pectoral girdle, table height will become increasingly important in assisting functional activities.

Home

Major adaptations should be carried out at this stage. The priority is to assess the needs and to establish eligibility for a Disabled Facilities Grant (DFG) or Home Improvement Grant. It is important that therapists are aware of the sources of funding and are able to support the family through the adaptation process, which can be very traumatic.

Home - wheelchair access

A non-slip ramp with a safety flange will be needed for the wheelchair and a gradient of 1:15 will be suitable for the powered model. Doors will need to be widened to provide a clear opening of 900mm, although it may be satisfactory for existing external doors to be narrower if the approach is in a direct line without the necessity to turn the wheelchair.

When boys get older they need wheelchairs that have sophisticated features—including reclining backrests, elevating legrests and tilt-in space seat and backrests—and these chairs are usually large; it is, therefore, important to plan for a 1700mm turning circle in order to provide adequate space(13).

Home - ground-floor bedroom/bathroom extension or through-floor lift

There are many factors to be considered and these are discussed in the “Lift vs Extension” chapter of the Muscular Dystrophy Campaign's “Adaptations Manual”(13). Essentially, the decision will depend on the size of the existing house and its facilities. The issues to be considered can be identified by clarifying the priorities: these are for the child to have an en-suite bedroom and bathroom, use of a ground-floor toilet, an easily accessible room on the ground floor in which he can use his computer and entertain his friends, in addition to adequate space within the house for the circulation of his wheelchair.

If the existing house is sufficiently spacious for the floor area taken up by the lift not to be missed, and if the priorities discussed above are either available or can be provided with additional building work, then a lift may be the best option. The reality is that not many houses are able to accommodate these facilities, but they will be available with the building of a ground-floor extension, with the added advantage of increasing the space within the house. The only disadvantages of an extension are:

  • that it may take up space in the garden that the family would prefer not to lose
  • if the child needs help in the night his parents will have to go downstairs
  • he will not have access to the rooms upstairs

However, many families have found an extension to be a very successful solution and the only possible drawback is that if he is timid, he might feel isolated when he is alone downstairs at night.

Home - hoisting (bedroom/bathroom)

A hoist will be needed to lift the child in and out of bed and to transfer him from one wheelchair to another and if necessary to a bath chair on a chassis or to a shower/toilet chair. Unless the hoist is needed in other rooms in the house, a ceiling hoist is likely to be more satisfactory than a mobile hoist, because it does not require space in which to be manoeuvred, is easier for the carer to use and places less strain on their back.

If a shower toilet (previously known as a combined WC/bidet) is needed, it is more satisfactory for the child to sit directly on the seat and the tracking for the hoist should, therefore, extend into the bathroom. In these circumstances, the track should be supplied with a ceiling turntable to allow the bathroom fittings to be placed in the optimum position and the hoist used for both the shower toilet and the specialist bath. The track should pass from front to back over the centre of the seat to allow the child’s position to be adjusted.

Home - level-access shower or bath

A level-access shower is unlikely to be the optimum solution for anyone with muscular dystrophy who cannot shower independently, because it is difficult for a carer to remain dry when helping, and the use of a shower screen makes it impossible to get down to wash the child’s feet. In addition, a shower does not allow the child to soak in the water and relax his muscles. However, it is important that the child and his family consider both options and the factors to be considered are discussed in the chapter “Bath vs Shower” (13).

For a bath to be satisfactory for a child with Duchenne muscular dystrophy, there must be a method of support. Specialist baths are available with an electrically-operated integral seat that allows the child to recline with good head and body support and with supportive chair arms in front and to the side. In addition, the base of the bath is contoured to provide support behind the knees; this will be important in the later stages, if he has knee contractures. The bath must have either an integral shower or a wall-mounted shower for hair washing or for a quick shower over the bath in the summer. The boys love the spa facility; if the DFG, Home Improvement Grant or charitable funds can cover the cost, this should be included in the bath specification when it is ordered.


Home - height-adjustable washbasin

In order to maximise good hand function it is important for a child to be able to get right under the basin until his chest is up against the front edge. To make this possible (because he cannot bend down to lift up the footrests on his wheelchair) the basin must measure 600mm front to back.

The child needs the basin low enough to be able to get his hands into the water and high enough to be able to rinse out his mouth when cleaning his teeth, without leaning forwards which would make it difficult for him to get back into his wheelchair. This can be achieved only with a basin that is height adjustable, and for independence this must be controlled electrically; this also enables the basin to be used as a mobile arm support to bring the child’s arms up to the level of his face for cleaning his teeth, washing his face etc. Because of his difficulty in reaching, the basin should be supplied with remote-control electronic taps operated by a touch-sensitive switch and automatic control of the strip light over the mirror.

Home - shower toilet

This combined toilet and wash/dry bidet will be necessary when the child can no longer clean himself and he wants privacy and independence. There is the option of plinths to raise the pan, but these are not usually necessary, as a standard height will enable him to get his feet squarely on the floor to help to stabilise himself. Shaped supportive toilet seats that will help with balancing are available and the operating switch must be touch-sensitive.

In order for a shower toilet to be effective, the user must sit directly on the seat; if a chair is superimposed, the washing and drying action will be less effective. It is important, therefore, that the unit should be used with a ceiling hoist in conjunction with a special frame to provide the support needed. As previously mentioned, the hoist should pass over the pan from front to back to allow the child’s sitting position to be altered as his body shape changes, to ensure maximum washing and drying efficiency.

Home - height-adjustable work surfaces.

A wheelchair-accessible work surface should be installed in the bedroom. If this work surface is L-shaped it allows the child to sit close into the right angle and support his forearms on the two adjacent surfaces; this will be particularly important when he is using his computer keyboard. The height adjustment will ensure that the optimum height can be achieved, irrespective of the height of the wheelchair seat and armrests.

It will be essential for a ‘standing’ surface 600mm or 800mm wide to be included, to allow the child access to purposeful activities while he is using a standing frame.

Home - electric light switches and sockets.

The height and position of light switches is crucial to enable them to be reached by a boy with Duchenne muscular dystrophy. The Muscular Dystrophy Campaign's Adaptations Manual (13) includes a wide range of information relating to all the issues around adaptations.

Home - referral for environmental control

The purpose of an environmental control for a child with Duchenne muscular dystrophy is to ensure that he maintains maximum independence. The equipment allows him to unlock the front or back door, switch on lights, use the telephone, and it also provides an intercom within the house and to the front and/or back door.

Criteria for supply from the NHS depend on the local arrangements; a designated medical consultant assesses the need. Occupational therapists should make a referral when a child is no longer able to carry out these functions and when it is felt that he needs the independence and will be sufficiently motivated to use the control.

Home - intercom

During the years before a child with Duchenne muscular dystrophy will qualify for an environmental control, it will be necessary for his parents to buy an intercom to be plugged into 13-amp sockets between his bedroom and their bedroom and the sitting room. This will be particularly essential if he needs to call for help during the night.

Home - electrically-operated bed

An electric bed will be needed when a child cannot sit up in bed or when he finds it difficult to get out of bed (13). The elevating backrest will raise him into an upright sitting position; if the bed is height adjustable, this will help him to stand up from the edge of the mattress. This height adjustment will be invaluable also for the carers, so that each one can work at the optimum back height when dressing him in bed and working at the bedside. Beds should be supplied through the Health Authority (to fulfil a nursing need) or, on occasions, by Social Services (to increase independence).

Specific models are recommended for boys with Duchenne muscular dystrophy to provide the optimum head and leg action on the bed, and reference should be made to the information provided by the Muscular Dystrophy Campaign (13).

Home - sleep systems and replacement mattress or mattress overlay

Many boys who are unable to turn over or move in bed need frequent attention in the night. The number of times that a parent or carer is disturbed can be influenced by the surface on which the boy is sleeping and by his level of comfort.

A night time postural system may help here; this is a system of pads and wedges that attach to the mattress and provide postural support during the night. In addition there is a wide range of alternative mattress overlays or replacement mattresses, ranging from simple foam or polyester low-pressure mattress overlays to sophisticated alternating air-pressure mattresses.

Home - supportive easy chair

Some children like a change from sitting in their wheelchair at home, but ordinary furniture is usually unsuitable because it does not provide adequate trunk support. The provision of a wheeled, adjustable and supportive easy chair in which a variety of positions can be achieved is recommended; for independent use, this should be controlled electrically.

Home - provision of small aids

As upper limb strength and hand function begin to deteriorate, provision of a few basic small aids to daily living can assist the child to participate in practical subjects at school and may be useful at home also. These may include:

  • non-slip mats;
  • bean-bag tray (the advantage is that it enables him to work on his lap without the need to raise his arms and the beans allow the surface to be tilted to the most convenient angle);
  • spike boards/buttering boards (for food preparation);
  • easy-grip peeler;
  • large-handled and lightweight cutlery;
  • L-shaped knives;
  • spring-assisted or easy-grip scissors.


Continuing emotional support for the boy and his family is important as the condition progresses. Consideration should be given to facilitating supportive counselling for the child and his family.

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