For Occupational Therapists

This section aims to provide occupational therapists working with ataxia patients with information about how common impairments impact on typical activities and occupations; and provide advice on interventions. In the absence of specific research, a philosophical approach, expert opinion and relevant progressive neurological conditions research will be drawn on.

This section has been reviewed by the College of Occupational Therapists Specialist Section – Neurological Practice, Long-term Conditions Forum and is endorsed by the College of Occupational Therapists.

Occupational therapy (OT) is widely recognised to be a valuable part of the multi-disciplinary care team in ataxia although primary research evidence is limited^{220,254–256}. Occupational therapy is an important intervention for people with progressive neurological conditions in maintaining independence and quality of life^222,257,258 enabling them to participate in self-care, work and leisure activities that they want or need to perform²⁵⁹. When it is no longer possible to maintain usual activities, occupational therapists should support people to adapt their relationship with their physical and social environment to develop new valued activities and roles²⁶⁰.

The focus of occupational therapists on ‘engagement’ in activity, rather than the disorder is important in progressive conditions. Occupational therapy intervention should focus on functional goals that support the person and their carers, to address occupational needs thereby adding to quality of life²⁵⁸. Occupational therapists should draw on their core skills to assess and understand the impact of the illness on occupational engagement, utilising problem solving and clinical reasoning skills to provide effective intervention. This will likely require flexible use of different frames of reference depending on the stage of disease progression. For example, a rehabilitation and educative approach could support occupational engagement in the early stages, while compensation will likely be needed as the disease progresses. Use of a client centred model of practice can support this process, guiding understanding of the individual’s key issues in a holistic manner.

Occupational therapists should use assessment and outcome tools that focus on occupational engagement and/or measure the person’s satisfaction with the performance of an activity; as use of tools that measure impairment would not always demonstrate the effectiveness of OT intervention. Appropriate tools include, but are not limited to, Assessment of Motor and Process Skills (AMPS), Goal Attainment Scale (GAS), Canadian Occupational Performance Measure (COPM), self-efficacy tools and quality of life measures^261,262.

It must be emphasised that the guidance given here is mostly based on practice consensus, not research. A literature review only identified a small number of case studies and case series designs focused on OT intervention in ataxia.^{220,254–256}

Due to the similarity of treatment approaches, to supplement this, wider evidence has been used from other progressive neurological conditions where more evidence is available, for example Multiple Sclerosis.^{222,259,260,263,264} Therefore the current evidence base is insufficient to make strong recommendations to clinical practice with the methodological quality of the articles being weak (SIGN 2001), and further research is needed in this area. In addition, most of the studies reviewed describe multidisciplinary intervention and it is therefore difficult to separate the effects of OT specifically.

A compensatory model of practical and physically focussed OT in the management of SCA3 showed positive and statistically significant changes in depression in a small study (although other outcome measures did not show a statistically significant change)²⁵⁶. This intervention was provided via 6-month individually tailored programmes focussed by client-centred goals addressing everyday difficulties, including feeding, work and social interaction. A combination approach to OT for the management of ataxia tremor has been suggested as useful. It is important to keep in mind the following domains when providing OT intervention to this population to ensure occupational engagement and wellbeing: promoting normal posture and movement, equipment provision and advice on activities of daily living, improving proximal stability and automatic equilibrium, and dampening/weighting²²². A comprehensive overview of strategies relating to specific tasks advocated a combination of compensatory techniques including postural stability, splinting and assistive technology, using client-centred goals and a task-orientated approach^220,257. Findings supported use of compensatory equipment and techniques that limit degrees of movement and dampen tremor in specific tasks.

Key findings from literature review:

• Client-centred, individually tailored OT programmes can have a positive effect on mood scales
• A short course of multidisciplinary rehabilitation, which includes OT, is beneficial compared to no treatment
• Client-centred goals along with a theoretical task-orientated approach may be useful to aid clinical reasoning
• Compensatory equipment and techniques that limit the degree of movement and dampened the tremor within specific functional tasks may improve occupational engagement for the individual
• Specialist seating can have positive and negative affects on posture but may improve comfort
• Further research is required

Table 9: General considerations for Occupational Therapy intervention

Using The Occupational Therapy Process, Creek, 2003 (GPP)²⁶⁵

The effects on a person’s occupational performance are not predictable and will depend on the types of activities that the person needs and wants to participate in. It is important to take a person-centred approach to analysing the area of occupational need within performance of daily activities and roles.

Evidence suggests that people with ataxia may have a lower quality of life in the early and end stages of the condition²⁶⁶. It is therefore important to recognise that even at the early stage of the condition difficulties with roles and occupational engagement may benefit from support. As most ataxias are progressive an important consideration is proactive planning for future needs²⁵. This can be a difficult situation to deal with in a sensitive manner, and occupational therapists must respect the individuals in their own journey of acceptance of this condition. If appropriate, occupational therapists should broach the expectation of future decline in occupational engagement when considering any major adaptations.

Common interventions and practical advice collected from clinical experience are outlined below (these are listed in alphabetical order).

I. Computer use

There are many aids to compensate for ataxia when using a computer. This may require joint assessment with a speech and language therapist in considering whether voice-activated software may be appropriate for overcoming problems with using a keyboard to enter information; however, dysarthria may prevent voice-activated software from being useful. Importantly, Information Technology is a constantly changing area with new devices and solutions becoming available all the time. If the person is still at work, funding for a computer assessment should be gained through an Access to Work referral (AtW). Alternatively, occupational therapists should investigate charitable organisations that may provide funding to access these services and equipment.

Practical suggestions

• A referral to IT solutions experts (such as AbilityNet) is strongly advised
• The AbilityNet website has free advice about IT adaptations for people with ataxia (www.abilitynet.org.uk)
• Keyboard and mouse modifications can be made to adjust the sensitivity and speed of response
• Alternatives to a standard mouse, such as a tracker ball, can be helpful
• Smaller keyboards or keyguards may help
• Consider the layout and location of equipment for ease of access
• Consider the impact of seating and ergonomic set up of the computer workstation

II. Control of the indoor environment

Control of the indoor environment can quickly become difficult for people with ataxia due to tremor and reduced coordination, for example use of electrical equipment with small switches or buttons. When assessing the person’s control of their indoor environment, consider priority activities they wish to participate in. Remember that especially in the palliative stage the focus on meaningful activity can provide immense satisfaction and comfort to the person with ataxia and their family.

As the condition progresses, occupational therapists should consider a referral to their Regional Environmental Control Service. These services can provide electronic assistive technology to severely disabled people to enable them to live more independently at home. This may include an environmental control system which enables the person with ataxia to call for carer assistance or emergency help, manage door entry and access, use the telephone, control the television and other media devices and control the lights and electrical appliances.

Practical suggestions

• Consider the use of ‘big button’ telephones and phones with autodial numbers or voice activation
• Consider use of a telephone with two way record to save conversations for replay later and to help keep messages
• Many telephone providers have inclusion phone services policies including a communications solutions guide obtainable online
• Light switches should be simple and easily reached from a standing or wheelchair position suitable to the person
• Appliance sockets are safest when located off the ground at waist level to avoid complex bending, squatting and reaching

III. Driving

If they are a driver, the newly diagnosed person with progressive ataxia is legally obliged to inform the DVLA and their insurance company of their diagnosis as soon as it is confirmed. Reporting the diagnosis may not mean cessation of driving. The DVLA will request information from the person and their medical team and may request attendance at a driving assessment centre before making a decision.

Some people will require driving adaptations to allow safe driving to be completed. Specialist centres provide assessment for suitable adaptations as well as driving ability (www.drivingmobility.org.uk). Where appropriate, the Motability scheme can assist people who receive the higher mobility rate of personal independence payment with minor adjustments, lessons or funding a vehicle (www.motability.co.uk). For more information on driving see www.gov.uk/health-conditions-and-driving.

There are some cases when the condition causes such difficulty with driving that it is unsafe for the person to continue with this role. When this is true, occupational therapists should explore alternative community mobility (see section on outdoor and community mobility below).

Practical suggestions (to assist with car transfers):

• Educate the person and carer on allowing the car door to be opened fully and to consider the height of the transfer being undertaken
• Ensure the person sits their bottom down first before moving their legs into the car
• Try inserting a swivel transfer mat and if the car seat is particularly low a firm foam cushion or blanket in a pillowcase
• Choose a model of car that optimises transfers, door access and storage space

IV. Eating and drinking

Feeding needs to be considered due to multiple impairments impacting on safe and effective eating and drinking. Before commencing any feeding assessment, standard practice would be to ensure that the need for a speech and language therapy assessment is considered (see section Speech and language therapy). Joint working may therefore be appropriate. Feeding solutions may be different depending on contextual factors, and solutions for eating at home may be different for social events. Altering positioning and/or seating will maximise posture and support core stability, thus reducing the impact of excessive limb movement²⁶⁷.

Practical suggestions

• Organise work spaces and utensils to reduce clutter and optimise performance
• Ensure individuals have appropriate postural control, use of a lumbar support will assist with optimal eating and drinking posture
• Non-slip matting can be used as a placemat to limit movement of the plate or cup, eg: Dycem® or similar
• Plate guards can be useful to reduce the need to co-ordinate two movements, and rocker knives may make cutting food easier by limiting the degree of movement needed
• Weighted cutlery may be beneficial
• Use of lidded/insulated cups or cups with straws for drinking can be helpful, especially with hot liquids
• Cups with anti-tremor insert devices can help as can Hotjo Mugs or similar products (www.neater.co.uk) which have a narrow neck and top to limit spills and a large non slip base which makes it easier to place on a work surface with uncontrolled movements
• For people with severe ataxia a sports bottle or camel pack may be helpful
• Use of the Neater-eater® or similar device with a dampening hydraulic mechanism can be very effective in aiding independent spoon or fork feeding (www.neater.co.uk)

V. Falls management

Falls may occur in any area that a person mobilises. Occupational therapists should consider joint assessment with, or referral to, a physiotherapist and referral to a falls management programme or group locally.

Practical suggestions:

• Advice on clothing not being too long and shoes being well fitted should be given, to try and prevent falls
• Non-slip flooring is helpful in fall prevention
• The person should be taught fall recovery techniques and if there is a family member or carer involved, the occupational therapist should consider the safety of the carer
• Where appropriate, consider the use of community care alarms, Telecare and techniques to avoid further injury e.g. pressure sores while waiting for help to arrive

VI. Food preparation

Preparing food is a common concern in the early stages due to its obvious risks. Occupational therapists should carry out an activity analysis of food preparation tasks and suggest a variety of methods and aids that may compensate for difficult or unsafe aspects. This may include completing food preparation tasks in a seated position, having someone else do aspects of the task for them (but not the whole task), such as cutting hard vegetables; or use of devices to aid grip and maximise safety. Again, find out what is important to the person and offer individual assessment of these areas.

Practical suggestions

• Kettle tipper devices or hot water dispensers can help making hot drinks safer
• Using a travel mug with a lid can sometimes assist with carrying a drink
• Waist-height ovens; use of full-length oven gloves; sliding food to a level surface (or level trolley) rather than lifting are all useful suggestions
• A microwave oven can provide a safer alternative to standard ovens
• Chopping boards with an attached cutting blade can be safer than a separate knife
• A food processor can help with slicing or chopping vegetables
• Ergonomic grip knives can be useful to limit the degree of movement needed when chopping food

VII. Hand function

Individuals with Friedreich’s ataxia often experience intrinsic muscle wasting in their hands, impacting on grip and dexterity and leading to a ‘claw’ deformity in the longer term. Assessment and education should be provided to manage this, with splinting explored as a method to maintain range of movement and improve occupational engagement as able²⁶⁸.

VIII. Handwriting

This can be an area of particular difficulty for someone with progressive ataxia. If the person is still at school or at university, it is important to work within the provisions of special educational supports such as those provided through support workers. For someone at work, consider a referral to AtW (www.gov.uk) for a full assessment. This may include an AbilityNet assessment for suggestions of alternative technological solutions for handwriting problems. Activity analysis may reveal the need for adaptations such as alternative positioning and/or seating, ergonomic desks and different pens.

Practical suggestions

• Ensure work spaces and seating are set-up to provide maximum support and optimise posture
• Dictaphones or voice-activated computer software can be used to compensate for problems with handwriting
• Use of weighted pens and thick barrelled pens may help but there is limited supporting evidence
• Consider the type of pen nib and the pressure applied as some people experience fatigue affecting sustained pen grip

IX. Household management

It is important to identify the household management priorities of the person and to recognise the cognitive and physical elements of these tasks. Most people with ataxia will continue to be able to cognitively manage the home but may have difficulty physically carrying out heavy housework such as vacuuming or heavy laundry. Occupational therapists may wish to discuss the impact of fatigue in order to help balance continued involvement in activities whilst recognising what is a priority to them.

Practical suggestions

• Hiring a cleaner to assist with heavy household tasks can be beneficial to save energy for more enjoyable tasks

Indoor mobility should ideally be assessed in the environments that the person frequently uses. For example, a person with progressive ataxia may walk well at home using walls and rails, but be unable to walk independently in a hospital, work or community setting. Mobility should be assessed jointly with physiotherapy colleagues if possible and consideration made of the use of walking aids.

Occupational therapists should consider the interaction of the person with their environment and the tasks that they want to perform once they have walked somewhere, as well as how the person plans to carry any items while walking. Use of walking frames may need to be reconsidered in very small areas and a combination of devices suggested in order to move from one area to another, eg: use of a walking frame to the bathroom and then hand rails once inside the bathroom.

When wheelchairs are required, close liaison with the local wheelchair service is recommended (also see section on outdoor mobility). Consider the environment including access, door widths and interaction with furniture to ensure that the person can access areas they want and/or need to. Major home modifications may be required, if not in the first instance, as the condition progresses. This should be considered earlier rather than later, with sensitive respect given to the person’s psychological adjustment process.

Some people may choose what is considered an unconventional solution to help them navigate their home such as crawling. A compromise between safety, risk management and patient choice may be required.

Practical suggestions

• Bags worn close to the body may be the most efficient and cause the least impact on balance. Later, it is advisable to avoid carrying items while walking
• Trolleys may help to transport items, especially food, drinks and heavy items at work or in the home and should be discussed and assessed if thought beneficial
• One-handed trays such as the Handitray® or similar products can help transportation of items
• Advise removal of items such as scatter rugs and loose electrical cables that may present as risks to mobility in the home environment
• Good lighting will help optimise performance of tasks and ensure that potential hazards in the home are avoided

X. Leisure

Occupational therapists should bear in mind that if there is loss of other occupational roles, leisure could be an area that helps to redress this loss in a different capacity. It is an important area to consider during intervention, as participating in leisure activities can help to maintain physical and psychological wellbeing. Enjoying leisure time with family and friends should be encouraged, albeit in modified ways. For example, use of accessible holiday homes, use of a wheelchair when visiting outdoor areas such as parks and galleries, and ensuring social contact continues in the home or other spaces.

If the person has lost leisure roles such as participation in sport, consider that they may be able to continue their involvement in the activity with alternative roles such as score keeping, participation on committees, or attending social events at their local club. For hobbies such as horticulture, consider adaptations that can be made to maintain participation, for example visiting local garden centres, planning planting or maintaining raised beds.

Reading can present particular difficulty for people with ataxia due to difficulty holding a book and/or visual problems. Electronic books such as ‘Kindles’ can help with this as they are easier to hold, you can adjust the size of the text to suit and use talking text options.

Practical suggestions

• Bookstands can be used to hold books
• Use of elastic bands around the loose pages of books can limit frustration caused by rustling pages where tremor exists
• Use of a rubber thimble can be useful to help turn pages where fine motor coordination is a problem
• Books may be downloaded online and use of zoom text can help where vision is a problem
• Talking books are available if preferred. The RNIB can be a useful support service in this area (see www.rnib.org.uk)
• Electronic page-turners can be purchased but are costly and take up space

XI. Outdoor and community mobility

Mobilising outdoors can often present particular difficulty for the person with ataxia, as it may be an unfamiliar environment. Educate the carer and the person with ataxia about resting regularly whilst walking outdoors. Consider what and how the person plans to carry while walking (see indoor mobility above). A wheelchair for outdoor use can help to reduce fatigue and/or maximise safety.

Practical suggestions

• Shop-mobility, taxi card schemes, mobility buses and dial a ride services can be helpful
• Public transport and rail providers offer subsidised fares for people with a disability and can provide a meet and greet service/access assistance for customers
• Outdoor motorised scooters or wheelchairs can maximise independence, but consideration should be made of transfer safety

XII. Posture and seating

An assessment for optimal posture and seating can be useful even in the early stages, and is essential in the later stages of ataxia. Occupational therapists should consider a referral to local wheelchair services for expert assessment. Consideration should be given to stable cushions and back supports, as canvas backs/seats in standard wheelchairs do not encourage good posture, which may impact on function.

A study using a randomised crossover trial design examined the effects of an individually adapted wheelchair support compared with a standard wheelchair for young people with progressive neuromuscular disorders, including FA. Their findings demonstrated improved postural alignment, but not improved respiratory or upper limb function; however fatigue could have influenced the findings²⁶⁷. Therefore, a compromise between optimising occupational engagement and providing adequate support is important.

xiii. Self-care and toileting

Aims of treatment for self-care and toileting include minimising the impact of excessive movement and helping the person to optimise their independence where possible. Prioritisation of tasks may mean that the person is happy to accept assistance with dressing if it allows conservation of energy that can be used for other, higher priority activities such as leisure or work. It is important to discuss this with the person and to anticipate for the future. For example, early referral for level access showers may be appropriate for the person with progressive ataxia.

Toileting is often an area that people with ataxia report as difficult and stressful. Rails can help the person to fix their arms and provide greater stability during transfers. Other problems encountered include dressing and undressing in the toilet, and managing perineal hygiene. Remember that assessment should be completed and aids trialled, as each person is unique.

Practical suggestions

• Encourage sitting to bathe or shower and consider providing seating with support for the back and arms
• Use of thermo-regulation devices on taps can be an important safety consideration
• Lever taps may be easier to use than standard taps
• Level access showers can be a useful consideration if bath transfers become unsafe or dangerous
• Small aids such as ‘zip pulls’ and button hooks and replacing fastenings with Velcro can be beneficial to increase independence with dressing
• An add-on bidet or an automatic washing/drying toilet such as a Closo-mat® or similar device may help with perineal hygiene
• Rails around the toilet may be of benefit and wherever possible these should be fixed to minimise risk of accidents
• Consider the height of the toilet seat and adapt this where required
• Consider the use of hygiene wipes and alcohol gel to maintain hygiene when away from home
• Register with RADAR for key access to their public toilets

XIV. Specialised equipment

Organisations such as the Disabled Living Foundation or Remap provide information and advice on equipment for disabled people and can be a useful resource for occupational therapists.

XV. Transfers (from bed, chair and toilet)

As with most client groups, ensure that the height of the chair is correct for the person to transfer easily on and off, with the hip and knee angle at 90 degrees and feet flat on the floor. Armrests greatly enhance the ease of chair transfers; ensure the chair is stable and that armrests are at a suitable height and position to enable the patient to push up. Educate the patient and carer on sit to stand techniques and always consider the carer’s safety within this.

Some people will need hoist provision for transfers and occupational therapists should ensure that they and their carers are adequately trained to perform this, with training being undertaken by a relevant team member. In particular, where full body tremor presents slings need to provide the maximum support available for safety reasons.

Practical suggestions

• Consider the height of the bed, chair and toilet and location within the room to ensure the most efficient and safest transfers
• A bed lever can be beneficial to aid rolling and rising in bed
• Mattress variators or profiling beds may be of benefit
• Firmer mattresses can aid bed mobility
• Pressure care needs should be considered if bed mobility is severely restricted

XVI. Work

Occupational therapists should consider maintenance of working roles for as long as the person wants it to continue and for as long as that is possible. When work is no longer possible consider rebalancing the loss of working roles with other activities or help to access relevant benefits.

It is important to provide education on the person’s rights and responsibilities under the Equality Act (2010). Support the individual regarding the disclosure of their diagnosis to others and their employers, if this is a concern. It should be remembered that intervention should allow the person to develop skills to manage ongoing liaison with the employer where possible. Occupational therapists may directly intervene by assessing and advising on reasonable adjustments to support people with ataxia to maintain their working role. This may include adapting work hours, environmental adjustments, advising assistance with specific tasks or travel and managing fatigue at work.

A work site visit may be required and this could be completed by the occupational therapist or via a referral to AtW if appropriate. AtW will help considerably with costs of aids required such as motorised wheelchairs, ergonomically appropriate seating and desks, IT devices, support workers and with taxi travel to/from work.

Fatigue can be a significant problem for people with ataxia and the impact of this on occupational engagement should be addressed within OT intervention. As with other progressive neurological conditions, using the principles of fatigue management can help people to maintain a consistent level of activity and engagement in tasks that are a priority for them. The basic principles of fatigue management are taking regular rest breaks, prioritising and pacing activities, maintaining a healthy lifestyle, organising work area and tools and maintaining exercise tolerance. It is useful to provide information on fatigue and discuss strategies, using activity analysis to help people look at alternative ways of completing tasks in a more energy efficient way. The need for support with fatigue, attention and accessing information was identified in a study exploring the physiotherapy experiences of people with cerebellar ataxia²⁵². Although current research does not demonstrate that occupational therapists are providing such interventions for people with hereditary ataxia, it would not be unrealistic to assume that these are already part of practice. Fatigue management, cognitive strategies and providing support to access services and information are common OT practices for people with other neurological conditions and should be explored for people with ataxia.

The psychological impact of having a long-term condition such as ataxia can be significant, especially for teenagers transitioning into adulthood while coping with increasing disability. Individuals may not be ready to accept advice or equipment and emotional support and anxiety management may be required within this process²⁵⁸.

For all people with ataxia, it is important for occupational therapists to assess whether anxiety and/or depression are impacting on occupational engagement. If anxiety and depression are identified as areas of difficulty, occupational therapists should look to address this within their intervention to support people to manage their occupational needs. Tools such as a Wellness Recovery Action Plan (WRAP) or Personal Wellbeing Plan can be helpful, although consideration may need to be made of specific costs related to this. Referrals should be made onto appropriate psychological services such as counselling or CBT as appropriate. If possible, individuals with ataxia should be given the opportunity to re-access services when they feel ready to make changes.

It is important to consider that some people with ataxia may have cognitive impairment, requiring more tailored interventions (see section Cognition). Additional adaptations may therefore be required, eg: timetables to help with fatigue management, visual prompts to remember risks, and additional instructions may be needed about the use of equipment.

Despite the limited primary evidence of specific OT intervention, expert opinion highlights involvement of occupational therapists in the multi-disciplinary management of people with progressive ataxias. The above examples provide a guide to suggested OT intervention in this group based on the consensus of occupational therapists working in this area. Future research is recommended into OT intervention, within the context of a multi-disciplinary team, for people with progressive ataxia.