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Please note: This is a basic revision aid, not a comprehensive and reliable statement of medical fact. These notes should not be used to guide treatment. For reliable information and advice, consult a qualified practitioner.
Last updated: 17 February 2001
Motor Neurone Disease is an umbrella term covering three main sub-types of the condition: Amyotrophic Lateral Sclerosis (ALS) Progressive Bulbar Palsy (PBP) and Progressive Muscular Atrophy (PMA). PMA accounts for about 8% of cases. Less common forms include Primary Lateral Sclerosis (PLS)
There are two types of motor neurone. Upper motor neurones run from the brain (cerebral cortex) through the spine. In the spine, they link with the lower motor neurones which connect to the bodily muscles. In Amyotrophic Lateral Sclerosis (ALS), the most common form of MND, both upper and lower motor neurones degenerate, resulting in muscle weakness, fasciculations (twitching) and stiffness. Progressive Bulbar Palsy (PBP) degenerates the nerves that control speech and swallowing. Progressive Muscular Atrophy (PMA) affects mainly the lower motor neurones, while Primary Lateral Sclerosis (PLS) affects primarily the upper motor neurones, but there is much overlap, and other types of motor neurone may be affected.
MND is sometimes known as Lou Gehrig's Disease in the US, where a famous baseball player died of the disease in 1941.
Incidence of 1:100000, prevalence of 5:100000. Males are more at risk, with a ratio of 1.5:1
Usually over 50 years, peaking at 70.
Idiopathic (cause unknown) in most cases. Familial in about 5-10% of cases, with autosomal dominant inheritance, and earlier age of onset.
Degeneration of anterior horn cells in spinal cord, motor cortex neurones and lower cranial nerve nuclei (depending on the type of MND).
Muscle atrophy and weakness in PMA and ALS, speech and swallowing difficulties in PBP. Muscle wasting and weakness may affect upper or lower limbs first. Fasciculation (muscle twitching) can be present, often beginning in the shoulder girdle. Respiratory and postural muscles may be affected later.
Average course of 2-5 years, but there is great variation. Respiratory failure secondary to upper respiratory tract infection (often caused by aspiration of food and liquid into the lungs) is the most common cause of death.
Symptomatic treatment only at present.
Multidisciplinary input will be essential as the person is likely to need home care, physiotherapy, speech and language therapy and nursing care as well as occupational therapy. This needs to be co-ordinated well, and a named keyworker should be designated early on. The relative rarity of the condition can lead to frustration for the person due to professionals' lack of awareness and understanding of the condition. The Motor Neurone Disease Society have a specialist Care Co-ordinator, whose input can be extremely valuable. Likewise, the local palliative care services should be involved early on, to provide valuable specialised support and advice. The tendency toward rapid progression of the disease necessitates regular review and reassessment, and makes planning for the future essential in any adaptations to the home. Liaison with home care workers is important, to ensure they are aware of the implications of the condition.
Kitchen tasks are likely to be problematic early in the course of the disease, with difficulties in lifting, carrying, opening jars and reaching higher shelves. Equipment such as vegetable draining baskets, jar openers, tin openers and trolleys may help, as may a rearrangement of the kitchen surfaces to facilitate sliding objects from one area to another instead of lifting. Upper limb difficulties may also necessitate use of modified cutlery, cups and plates, non-slip mats, cup holders and arm supports.
Operating locks, door handles and light switches may become difficult and may require special modification. Hands free telephones can alleviate problems with the weight of a telephone receiver. Environmental control systems should be considered earlier rather than later, as they can involve a lengthy wait -- additional elements (TV, lights, door openers, etc.) can be added in at a later date. Book rests, page turners and talking books can prove valuable.
With reduced use of arms and hands, toileting can be a problem, and it is worth considering an automatic toilet early on, but ensure that alternative switching methods are available i.e. a foot switch. Clothing can also be difficult to manage. Velcro can overcome problems with zips and buttons, but devices such as dressing sticks and sock aids may be difficult due to their weight. Bathing is another likely problem. The solution needs to take into account the likely progression. If an adaptation to the bathroom is being considered, a level access shower will allow for longer term needs whilst an overbath shower is likely to serve only in the short term. Commodes and shower chairs can be useful (but be aware of potential conflicts between shower chairs and automatic toilets -- the shower chair may raise the user too high above the toilet for the washing spray to be effective).
A community alarm can provide security, reassurance and fast access to help, but special attention needs to be paid to the ease of operation of the device, with the options for alternative means of control.
In the case of lower limb problems, transfers can become difficult and consideration should be given to seating height, possible use of riser-recliner chairs (but bear in mind difficulties in operating the controls) and postural support in the event of deterioration of the muscles of the trunk. Appropriate postural positioning may also relieve dyspnoea (breathing difficulties) due to impaired respiratory muscles. Mobility aids and wheelchairs may be necessary, but consider the likely problems with self-propelling chairs due to upper limb involvement. Early referral for a powered chair should be considered, as the wait for provision can be lengthy. Ensure fast track procedures are used when necessary.
With increased difficulty in transferring, moving and handling equipment may be useful -- such as slide sheets, mattress variators (again bear in mind the difficulty operating manual controls) and hoists. An adjustable height bed can help reduce risk to carers if it is necessary to provide personal care in bed. Increasing problems with mobility bring increased risk of pressure sores, and regular formal assessment of risk is essential. Special cushions and mattresses may be necessary. When providing hoist slings, seating, shower chairs, mattresses and pressure relief cushions, consideration should be given to pain experienced by the person, often caused by unstable joints, weight loss exposing bony prominences, muscle cramps and spasticity. Pain is subjective, and often the only way to determine an item's suitability is to try it out.
Accepting any adaptive equipment can be difficult for the person, as the rapidity of the progression of the disease allows little time for adjustment. The psychological impact of having to rely on a hoist can be devastating, so the issue needs to be approached carefully, and with a positive emphasis on maintaining independence. The psychological consequences of the disease require special attention from diagnosis onward. Rapid progression of the disease also means that equipment and adaptations should be provided with all possible speed. People are rightly very frustrated if a delay in provision means that the item arrives when the person can no longer use it. The disease can impart a sense of urgency, so dithering over clinical decisions can be very damaging. Make use of the opinions of other professionals throughout.
Communication problems can include writing, using phones and speaking. Some people need to use speech synthesisers or other communication equipment. Consideration should be given to how the communication proceeds -- pre-empting someone who is communicating with a light-writer is similar to interrupting someone mid-sentence, but is done all too frequently. Dysphagia is another difficulty to be aware of, due to damage to some of the cranial nerves (hypoglossal, accessory, vagus, trigeminal, facial, glossopharyngeal). Referral and re-referral to Speech and Language Therapy may be necessary at various points.
Families of people with motor neurone disease should be considered from diagnosis onward. Carer strain and carer stress should be monitored and independent carer's assessments carried out where appropriate. People with MND and their carers should be kept well informed of the availability of support services such as home care, home respite, institutional respite, palliative care services and hospice care.
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Dave (not his real name) was in his fifties and had been diagnosed with MND (Primary Lateral Sclerosis type) about 3 months prior to referral to his local social services department. The referral came from the GP and raised a number of concerns including personal care, difficulty with door locks and other appliances, kitchen tasks and difficulty with toileting.
Dave lived alone but had supportive family living in the same city. His home culture was not English, and he often visited a compatriot who worked in a local shop. He lived in a ground floor flat which had level access and was level throughout but would not easily accommodate wheelchair use due to narrow doorways and lack of circulation space.
Dave's initial muscle weakness was centred on the upper limbs, and he had difficulty with kitchen tasks, particularly lifting pans and kettles. We experimented with kettle tippers and small, lightweight jug kettles and settled on the jug kettle option, mainly because it looked more normal and took up less room.
There was no significant lower limb involvement at this stage and Dave was able to mobilise both within the flat and without.
The door lock was lowered to about waist level (funded by social services), to remove the need to lift the arms. A key turner handle was attached to the door key, to give greater leverage. This was adequate at the time, but the door would later be fitted with a door entry system connected to an environmental controls system.
Social services funded the lowering of the light switches to about waist level as Dave was finding it increasingly difficult to lift his arms to reach them.
As muscle weakness progressed, Dave found it difficult to hold and lift a telephone receiver. At this point, the OT provided a hands free phone and referred to the regional environmental controls centre.
The home care assistants were not familiar with Dave's background and were not able to provide culturally appropriate meals. The OT tried to find a care agency that had a worker of the same cultural background, but with little success. Dave knew of a compatriot who worked in auxiliary care, and suggested that this would be the best person to deliver care. The OT gave Dave details of a reliable local care agency and suggested that the care worker register with the agency in order that he could be commissioned as part of Dave's care plan. This all worked out fairly well, and Dave was able to get culturally appropriate meals.
Eating meals was becoming more of a challenge, and the best method found was proximal stabilisation. Social services technicians raised the table slightly to allow Dave to rest his elbows on the table whilst eating, so that only the distal part of the arm needed to be moved. Mobile arm supports had been trialled in hospital but had proved less satisfactory. Some assistance was still occasionally needed. Lightweight plastic cups and beakers were adequate for drinking initially, but drinking straws had to be used later. Ultra lightweight cutlery was used to assist eating and reduce the amount of weight to be lifted.
Psychological considerations were important, given the severe nature of the diagnosis. Dave was fully aware of the prognosis and was open in discussing his feelings about the future. He was particularly afraid of losing the use of his legs. He had considered going home for the remainder of his life, but no firm decision on this was reached. The OT also got involved in supporting an application by one of Dave's children to come to the country and assist in care, but it is unclear if this made any difference (the application was still pending when the OT's intervention ceased.) Dave seemed to be coping reasonably well with the psychological impact of the diagnosis. There was no sign of major depressive or anxiety symptoms. Dave said he was helped by the feeling that support was timely and appropriate -- both OT intervention and home care support were regularly reviewed, and Dave had been referred early to the Motor Neurone Disease Association's Regional Care Co-ordinator, who had been able to provide considerable support in terms of discussion of the future and information about the disease and the services available. A supportive family helped considerably. Dave also attended a day care unit at the local palliative care centre. There was a high level of multidisciplinary co-ordination and liaison between all the services involved.
Because of the relatively mild lower limb involvement, Dave was independent in all transfers except bath transfers. A bath lift proved adequate at this stage, as Dave was able to swing his legs over the bath sides without assistance, but needed some assistance with bathing and with operating the bath lift. A lightweight control handset did not help, as Dave would have had difficulty reaching and picking it up if dropped. Consideration was given to a level access shower, but it was unclear if it would be useful. The reasoning behind this was that if and when Dave reached a stage when he was unable to transfer onto a bath lift, wheelchair and hoist use might have rendered the home unliveable given the lack of circulation space for a wheelchair.
In terms of domestic tasks, Dave needed assistance in housework, shopping and laundry given the difficulties with carrying and lifting. Home care assistance was provided for these tasks.
Dave had significant muscle wastage throughout the arms, hands and shoulders and this was causing some pain in bed, particularly at the shoulders, along with difficulties in adjusting the bed clothes. The district nurses provided a Propad mattress (castellated foam) to reduce the shoulder pain while the OT arranged for technicians to fix a fabric loop to one corner of the duvet and a safe, rounded hook to the wall so that the top corner of the duvet could be anchored to the wall to prevent slippage. This was quite successful, and still allowed Dave to get up at night for toileting.
Using the toilet had become another challenge. Dave's upper limb strength had become inadequate for self-cleaning after toileting. We tried a lightweight wiper as an interim solution whilst planning an automatic toilet installation. The wiper wasn't very successful. They can be useful if the main problem is shoulder mobility, but the additional weight wasn't helpful for Dave. For the automatic toilet, one which has a cleaning spray and a blow dry facility, we had the option of a specially installed toilet, such as a Clos-o-mat or Geberit shower-toilet, or an add-on device that looked like a raised toilet seat with a large side box, housing all the mechanisms. We found one made by Medic-bath. We decided on this option, as it would be quicker than going through all the bureaucracy of an adaptation and could be in place much sooner than the other options. The device had a water spray for cleaning and a warm air dryer and could be operated by a foot pressure pad.
The environmental controls assessment, conducted by an OT and rehab engineer from the regional environmental controls centre resulted in provision of a unit that was operated by a foot switch and linked to the door opener, television, radio and light switches, with the possibility to add more device links as necessary. The unit would cycle sequentially through the different options on the control panel until the operation of the foot switch selected the device currently highlighted.
My involvement ceased at this point as I moved to work with another employer. At this stage, Dave was still living at home, able to transfer, mobilise and use the toilet independently and receiving three or four home care visits daily. Two years on, I was pleased to hear from an ex-colleague that Dave is still at home.
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Last updated: 04 October 2002
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