Occupational therapy and Duchenne muscular dystrophy - the later stage

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Information on occupational therapy for the later stage of Duchenne muscular dystrophy. The later stage covers the use of indoor/outdoor powered wheelchairs to the terminal stage.

(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 - physical management

Physiotherapy treatment, including stretches to the upper and lower limbs, continues to be important and good liaison between therapists is invaluable to a family. From time to time the issues relating to splinting of the arm to prevent ulnar deviation and wrist flexion contractures may be discussed; although, currently, this practice is not generally used because of the lack of compliance, it may be appropriate in some cases.


Mobility - spinal surgery

A scoliosis often develops in boys who have Duchenne muscular dystrophy, soon after they become unable to walk. Eventually, this results in pelvic obliquity and deformity of the chest, which will restrict the capacity of the lungs. It is important to keep the child walking for as long as possible, and then to consider a spinal support or spinal surgery.

The issues to be considered in spinal surgery are as follows:

  • When is the correct time?
  • What does the operation entail?
  • What are the risks?
  • What may be the results?
  • What are the disadvantages?
  • Who makes the decision? (14)

Prior to surgery, it is important for the occupational therapist working in either the specialist neuromuscular clinic or the orthopaedic clinic to liaise with the community occupational therapist to ensure that the necessary equipment is in place. If equipment has been provided at the appropriate stages this should not present a problem; this underlines the need to plan ahead and thereby to avoid a crisis following surgery, which may culminate in a delay in discharge from hospital.

Spinal surgery results in a number of issues that affect the boy’s function; therefore, without suitable equipment, some independence can be lost. The relevant issues are as follows:

  • As a result of the fixation and rigidity of the spine the boy can no longer compensate for his shoulder weakness by flexing his spine and bringing his head down (for example, for eating); there therefore appears to be a reduction in upper limb function, and a loss of independence.
  • The length of the spine is increased and the boy sits more upright in his wheelchair making height-adjustable equipment vital.
  • Although the initial loss of head control is usually regained within a few weeks, there may be persistent difficulties with head control if the boy is tipped away from a vertical position (for example in a hoist); hence, the need for head support in a sling.
  • The inability to flex forwards and ‘dip’ his head when entering a wheelchair-accessible van will result in the need for greater headroom, in the height both of the door and of the roof of the van. Because a van may be bought several years before spinal surgery is carried out, this need must be anticipated in advance.

The following equipment is needed:

  • A hoist with a sling which provides a stiffened back support and head support;
  • An electric bed;
  • Support on the toilet;
  • A wheelchair (which may need modifying to provide armrests of the correct height) and a head support;
  • A height-adjustable table or work surface that can be adjusted to provide wheelchair accessibility, irrespective of the height of the armrests;
  • A height-adjustable washbasin.


Mobility - mobile arm support

In addition, increasing upper limb weakness can cause difficulties with feeding. Manual dexterity frequently remains good in many children until a fairly late stage. The provision of a mobile arm support to counteract the force of gravity can be useful in ensuring maximum independent upper limb activity for as long as possible.

The recent development of a powered height adjustable mobile arm support (from Neater Solutions), which is attached to the wheelchair, is an exciting concept and should enable even very severely disabled children to feed themselves.

Find out more about mobile arm supports

Mobility - reclining backrest/comfort in wheelchair

Despite the provision of a good postural position in the wheelchair achieved by a suitable seating system, backache and pain associated with increasing deformity make it essential to be able to alter the pressure on the body. At this stage of the condition, the following wheelchair features are necessary:

  • A reclining backrest;
  • A seat and backrest that tilt back from the horizontal;
  • Leg rests that can be raised and lowered independently of each other

All these functions should be controlled electrically, to allow the young man complete independence to move within his chair (3, 15). At present, the supply of sophisticated wheelchairs by the statutory wheelchair service is often limited. Fortunately, however, there are Charities that will help with the funding of mobility equipment. The Muscular Dystrophy Campaign has produced a factsheet justifying the provision of these wheelchairs (15).

School

At this stage the factors to be considered should be divided into those relevant (a) before and (b) after the child reaches the age of 16 years (pre- or post-16).

(a) Pre-16
Technical support. Consideration must be given to the support required for all the technology subjects, including food technology, science, metal/woodwork and IT.

Examinations:
The necessity for extra time and/or a scribe or a separate room to use voice-activated software must be assessed.

(b) Post-16. The opportunities can be categorised as follows:

  • Educational
  • Employment
  • Occupational


School - educational

Opportunities at 16+ will depend on the young man’s academic ability, his level of functional ability and his interests and skills. Options for consideration at the transition stage may include:

  • 6th form at special school
  • 6th form college
  • Further-education college
  • Vocational college
  • Residential college
  • Home-based opportunities
  • University and a place on a degree course

The use of ‘ high tech’ computer equipment, including voice-activated software and on-screen keyboards, will enable young men to compete on equal terms with their peer group. As before, prior to external examinations, it will be necessary for the teachers or lecturers to assess the need for extra time or the use of a scribe.

Careful assessment of all the care needs is important at this stage to ensure an adequate level. Most young men will need 24-hour support if they are residential at college or university, or if they leave home to live independently; furthermore, such support is increasingly necessary for boys who choose to remain living with their parents, to relieve the latter of the caring role.

School - employment

The young person should be referred to the Disability Employment Advisor of the Employment Service for advice about the range of schemes available to assist (even severely) disabled individuals into employment or training. Office-based occupations are likely to provide the best solution; computer activities, graphic design or desktop publishing are particularly suitable and, if necessary (although this is not ideal), the young man can work from home. In this situation it may be possible to obtain further adaptations with a discretionary DFG.

School - occupational

Occupational activities should be considered for boys who do not wish to remain at school beyond the age of 16 or to progress to further education at the age of 18. Options include the following:

  • Home-based activities
  • Day centre
  • Leisure activities, either at home or away from home
  • Contact with disabled and able-bodied peer groups


Home - medical support

Medical support may be necessary for the relief of respiratory distress, with nocturnal ventilation, bowel and bladder care, the relief of pain, and discussion about emergency resuscitation. At this stage it may be necessary to refer to a dietician for appropriate advice to improve nutrition, and food supplements may be recommended. Nasogastric feeding and gastrostomies are used in some centres.

Home - emotional/care support.

In addition to further emotional support, increased care support (day and night), may be needed. The specialist mattress or mattress topper should be re-assessed to increase comfort, to eliminate any pressure problems and to try to reduce the number of times that the young man needs to be turned in the night.

Continuing respite care is important for the young person and his family. It may be necessary to offer pre-bereavement support to the family and an opportunity for the boy to talk about his death, if he wishes. This is a very difficult time for the whole family.

Home - environmental controls

Referral for this equipment should be considered if it was not included when the home adaptations were carried out.


Home - automatic door opener

It is essential that boys are supplied with indoor/outdoor wheelchairs to provide independence, and it is equally important that they can get into and out of the house without having to ask someone to open the door. When the adaptations are carried out there is usually a lack of funding, and the recommendation would be to install the electrical socket for the unit to be supplied at a later date. This can be funded privately, or an application for an additional grant can be made.

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