Topic 16.3. Considerations for prescribing wheelchair and seating systems
This chapter of the Clinical Management Guidelines for Friedreich Ataxia and the recommendations and best practice statements contained herein were endorsed by the authors and the Friedreich Ataxia Guidelines Panel in 2022.
Topic Contents
16.3 Considerations for prescribing wheelchair and seating systems
16.3.1 Review of literature related to Friedreich ataxia
16.3.2 Review of literature from like conditions
16.3.3 Provision of powered wheelchairs and scooters
16.3.4 Pediatric versus adult individuals with Friedreich ataxia
Disclaimer / Intended Use / Funding
Disclaimer
The Clinical Management Guidelines for Friedreich ataxia (‘Guidelines’) are protected by copyright owned by the authors who contributed to their development or said authors’ assignees.
These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.
Guidelines users must seek out the most recent information that might supersede the diagnostic and treatment recommendations contained within these Guidelines and consider local variations in clinical settings, funding and resources that may impact on the implementation of the recommendations set out in these Guidelines.
The authors of these Guidelines disclaim all liability for the accuracy or completeness of the Guidelines, and disclaim all warranties, express or implied to their incorrect use.
Intended Use
These Guidelines are made available as general information only and do not constitute medical advice. These Guidelines are intended to assist qualified healthcare professionals make informed treatment decisions about the care of individuals with Friedreich ataxia. They are not intended as a sole source of guidance in managing issues related to Friedreich ataxia. Rather, they are designed to assist clinicians by providing an evidence-based framework for decision-making.
These Guidelines are not intended to replace clinical judgment and other approaches to diagnosing and managing problems associated with Friedreich ataxia which may be appropriate in specific circumstances. Ultimately, healthcare professionals must make their own treatment decisions on a case-by-case basis, after consultation with their patients, using their clinical judgment, knowledge and expertise.
Guidelines users must not edit or modify the Guidelines in any way – including removing any branding, acknowledgement, authorship or copyright notice.
Funding
The authors of this document gratefully acknowledge the support of the Friedreich Ataxia Research Alliance (FARA). The views and opinions expressed in the Guidelines are solely those of the authors and do not necessarily reflect the official policy or position of FARA.
16.3 Considerations for prescribing wheelchair and seating systems
Ellen W. Goh, Manuela Corti, Kathrin Reetz, Sub H. Subramony and Louise Corben
16.3.1 Review of literature related to Friedreich ataxia
There is only one published study that specifically examines the prescription of wheelchairs for people with FRDA (2). This prospective two‐year randomized crossover study included 19 male participants, four of whom had been diagnosed with FRDA, although the method of diagnosis was not reported (the study predated genetic diagnosis). This study examined the efficacy of provision of a customized wheelchair seating system on sitting posture, respiration and upper limb function. While having some methodological shortcomings, this study is important in highlighting how critical adaptive seating is to an improved seated posture. There was, however, little immediate effect on respiratory function and only small differences in upper limb function. An important conclusion from this study was the potential to over‐correct musculoskeletal changes, and moreover, in the process of correction for deformity, to further compromise functional capacity. The study findings reinforce that in prescribing a manual wheelchair and seating system, it is essential that functional capacity is not impeded for the sake of an anatomically correct seated posture (2, 16). No new studies specifically relating to prescription of wheelchairs for people with FRDA have been identified for inclusion in the updated clinical management guidelines. However, most clinicians treating individuals with FRDA support this notion.
16.3.2 Review of literature from like conditions
As with FRDA there are few documented studies evaluating the efficacy of wheelchair and customized seating in people with like chronic neurological conditions. There are however, retrospective audits (17), review articles (18, 19) and clinical guidelines (20).
People with FRDA usually present with some degree of scoliosis with associated hypertrophy of back musculature, and pelvic asymmetry. Uncorrected, this can lead to further loss of function, pain, pressure areas and compromised respiratory function (21, 22). Provision of an adjustable contoured seat that has the flexibility to correct pelvic asymmetry by the use of gel or foam inserts can be effective in preventing further deformity (20, 21, 23, 24). Moreover, the use of a contoured padded back support with either or both pelvic and thoracic lateral supports can assist in providing support in a coronal plane (22, 25). Holmes and colleagues (22) identified the efficacy of using four adjustable lateral pads in managing scoliosis related to cerebral palsy (CP). Whilst the mechanisms of scoliosis in people with FRDA have some differences to those with CP, the intervention may prove just as effective. A correctly positioned four-point lap belt can maintain the person in an optimum position despite a tendency to change position as a response to coughing, laughing or sneezing. De Sousa & Frank (24) describe the prescription of tilt-in-space wheelchairs for people with rare diseases to help manage problematic pain and scoliosis. Consideration should be given to the most appropriate type of wheelchair frame (18). Whilst people with FRDA benefit from a lightweight, responsive model (often with a rigid frame), it is essential it has the strength to manage at times poorly controlled, forceful movement (18, 26, 27). Removable footplates are critical to ensure an optimum sit to stand stance during a standing transfer. Likewise, the use of one‐piece lift-off footplates may prove easier to remove and replace as well as achieving optimum positioning for hips, knees and feet.
16.3.3 Provision of powered wheelchairs and scooters
Individuals with FRDA may benefit from provision of either a powered wheelchair or scooter. This equipment is particularly important in facilitating community access for those who find it difficult to wheel distances in a manual wheelchair (28). As discussed previously, careful attention needs to be given to appropriate seating, in addition to the programming, control, stability and storage of the chair and the mode of transfer on and off the chair or scooter (17). In one study (24), there was recognition that whilst some people with FRDA may lose the ability to control their powered wheelchair, many do not and are able to continue to use the wheelchair with standard controls. Modifications to the controls may allow appropriate responsiveness despite a reduction in upper limb co-ordination or strength. With either manual or powered wheelchairs, it is critical that structured training programs are conducted to ensure the user is safe, competent and confident to use the wheelchair (29). It is also important to consider the transportation of a powered wheelchair, particularly if needing to access more distant destinations where modifications to a car would be necessary.
16.3.4 Pediatric versus adult individuals with Friedreich ataxia
It should be noted that the prescription of wheelchairs for children with FRDA raises different issues than those for adults. An earlier age of onset is associated with faster rate of progression to wheelchair use (30, 31). In addition, the repeat size on GAA1 has been associated with the presence of scoliosis, wheelchair dependence, impaired vibration sense and presence of foot deformity (32). The earlier scoliosis is evident, the greater is the likelihood of progression and requirement for surgical intervention (21). Prophylactic management of scoliosis in children is critical even before symptoms are apparent. The goal of seating in both pediatric and adult populations, regardless of evidence of scoliosis, is to maintain the spine in a balanced position in the coronal and sagittal planes over an even pelvis (21). Prescribing a wheelchair for a child has to incorporate requirements for training, growth, postural stability, completion of activities of daily living and school/leisure/home access issues (19).
Assistive technology for mobility
QUESTION: Should customized assistive technology for mobility (e.g., modified vehicles, wheelchair, transfer devices, walkers, adaptive seating and positioning) versus non-use be used for individuals with impaired independent gait/mobility with Friedreich ataxia?
[sg_popup id=”587″ event=”click”]
[/sg_popup]STRENGTH OF RECOMMENDATION: ↑
[sg_popup id=”658″ event=”click”][/sg_popup]LEVEL OF EVIDENCE: ⨁◯◯◯
RECOMMENDATION: For individuals with Friedreich ataxia with impaired independent gait/mobility, we suggest the use of customized assistive technology for mobility (e.g., modified vehicle, wheelchair, transfer devices, walkers, and adaptive seating and positioning) to enhance independence in mobility, quality of life, and social and occupational participation, and to reduce falls.
JUSTIFICATION: Despite the low level of evidence in like conditions, expert clinicians who provide clinical care for individuals with Friedreich ataxia agree that the use of customized assistive technology for mobility can benefit independence in mobility, quality of life, and social and occupational participation, and reduce falls.
SUBGROUP CONSIDERATION: This recommendation is specifically for individuals with Friedreich ataxia with impaired independent gait/mobility. The guideline expert panel considers that all individuals with Friedreich ataxia might benefit from the use of digital and assistive technology.
Evidence to Recommendation Table PDFPersonal care and environmental control
QUESTION: Should customized assistive technology (personal care technology, environmental control [iPAD, home APPs, smart watches], ALEXA/ SIRI) versus non-use be used for individuals with impaired upper limb functionality with Friedreich ataxia?
[sg_popup id=”587″ event=”click”][/sg_popup]STRENGTH OF RECOMMENDATION: ↑
[sg_popup id=”658″ event=”click”][/sg_popup]LEVEL OF EVIDENCE: ⨁◯◯◯
RECOMMENDATION: For individuals with Friedreich ataxia with impaired upper limb functionality, we suggest the use of customized assistive technology for personal care and environmental control (e.g., iPad, home apps, smart watches, Alexa/Siri) to enhance independence in daily activities, quality of life, and social and occupational participation.
JUSTIFICATION: Despite the low level of evidence in only one like condition, expert clinicians who provide clinical care for individuals with Friedreich ataxia agree that customized assistive technology for personal care can benefit independence in daily activities, quality of life, and social and occupational participation in individuals with Friedreich ataxia.
SUBGROUP CONSIDERATION: This recommendation is specifically for individuals with Friedreich ataxia with impaired upper limb functionality. The guideline expert panel considers that all individuals with Friedreich ataxia might benefit from the use of digital and assistive technology.
Evidence to Recommendation Table PDFHealth monitoring
QUESTION: Should health monitoring/alert devices (e.g., monitoring heart rate, steps, sleep, ECG, activity, healthy eating, medication, glucose for diabetes) versus non-use be used for individuals with fatigue, fall risk, poor sleep, diabetes, etc, with Friedreich ataxia?
[sg_popup id=”587″ event=”click”][/sg_popup]STRENGTH OF RECOMMENDATION: ↑
[sg_popup id=”658″ event=”click”][/sg_popup]LEVEL OF EVIDENCE: ⨁◯◯◯
RECOMMENDATION: For individuals with Friedreich ataxia and fatigue, a risk of falls, poor sleep, diabetes and/or cardiomyopathy, we suggest the use of health monitoring/alert devices (e.g., monitoring heart rate, steps, sleep, ECG, activity, healthy eating, medication, glucose for Friedreich ataxia-related diabetes) to enhance independence in daily activities and quality of life and improve medication control.
JUSTIFICATION: Despite the low level of evidence, expert clinicians who provide clinical care for individuals with Friedreich ataxia agree that customized assistive technology for health monitoring can benefit independence in daily activities, quality of life, number of hospitalizations, sleep, diabetes and medication control in individuals with Friedreich ataxia.
SUBGROUP CONSIDERATION: This recommendation is particularly relevant to individuals with Friedreich ataxia who report fatigue or poor sleep, are at risk of falls, and/or have diabetes or cardiomyopathy.
Evidence to Recommendation Table PDFCommunication and workplace assistance
QUESTION: Should customized assistive technology for communication and to expand workplace opportunities (e.g., writing, speech, computer use, tablets, workplace design [adaptive seating and positioning], vision and hearing) versus non-use be used for individuals with impaired communicating ability and workplace difficulties with Friedreich ataxia?
[sg_popup id=”587″ event=”click”][/sg_popup]STRENGTH OF RECOMMENDATION: ↑
[sg_popup id=”658″ event=”click”][/sg_popup]LEVEL OF EVIDENCE: ⨁◯◯◯
RECOMMENDATION: For individuals with Friedreich ataxia with impaired communication and workplace difficulties, we suggest the use of customized assistive technology for communication and the workplace (e.g., writing, speech, computer use, tablets, workplace design [adaptive seating and positioning], vision and hearing) to enhance independence in communication, improve quality of life and increase occupational participation.
JUSTIFICATION: Despite the low level of evidence in like conditions, expert clinicians who provide clinical care for individuals with Friedreich ataxia agree that customized assistive technology for communication can benefit independence in communication, quality of life and occupational participation in individuals with Friedreich ataxia, particularly in the later stage of the disease.
SUBGROUP CONSIDERATION: The provision of customized assistive technology is particularly relevant to individuals with advanced Friedreich ataxia who may have concurrent issues related to upper limb ataxia, visual difficulties and eye movement problems.
Evidence to Recommendation Table PDFCompensatory or remedial interventions with digital and assistive technology may improve independence for individuals with Friedreich ataxia.
Prescription of a manual or powered wheelchair or scooter should be preceded by an assessment of the home/school/work and community environment the equipment will be used in.
A comprehensive prescription of a manual or powered wheelchair or scooter should be completed by a qualified clinician familiar with the specific issues related to Friedreich ataxia.
A validated assessment and evaluation tool for wheelchair and seating prescription may be used to guide the process of prescription and evaluation.
In prescribing a manual wheelchair and seating system, functional capacity should not be impeded for the sake of an anatomically correct seated posture.
Appropriate training should be provided for the safe use of a wheelchair or scooter in the home or community environment.
Suitability of the seating and wheelchair system should be evaluated on an annual basis in adults and biannually in children.
Lay summary of clinical recommendation for digital and assistive technologies in Friedreich ataxia
Why these recommendations?
These recommendations are about the use of digital and assistive technologies such as modified wheelchairs, adaptive seating, environmental controls, apps and smart watches. These technologies may help individuals with Friedreich ataxia with work, leisure, social and daily activities that involve communication, mobility and upper limb function. They may also be useful for individuals for whom it is important to maintain health and well-being, such as for management of diabetes or maintaining healthy eating.
Due to a lack of studies, there is little direct evidence showing the benefits of using digital and assistive technologies in individuals with Friedreich ataxia. However, the clinical experience of the authors and published research in other neurological conditions supports the consideration of using such technologies.
What does this mean for you as a person living with Friedreich ataxia or caring for someone living with Friedreich ataxia?
It might be important for you to talk to your allied health and medical clinicians to see if the use of digital and assistive technologies may be appropriate for you.
Who are these recommendations specifically for?
These recommendations are relevant to all individuals with Friedreich ataxia, particularly those who have lived with the condition for a long time and have many inter-related issues that may interfere with independence, such as speech, mobility and upper limb problems.
Louise Corben, PhD
Principal Research Fellow, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia
Email: louise.corben@mcri.edu.au
Manuela Corti, P.T., PhD
Assistant Professor, University of Florida, Gainesville, Florida, USA
Email: m.corti@peds.ufl.edu
Ellen W. Goh, BAppSc(OccTherapy)
Neurosciences Clinical Lead Occupational Therapist, Monash Health, Melbourne, Victoria, Australia
Email: ellen.goh@monashhealth.org
Kathrin Reetz, MD
Professor for Translational Neurodegeneration, Department of Neurology, RWTH Aachen University, Aachen, NRW, Germany
Email: kreetz@ukaachen.de
Sub H. Subramony, MD
Professor of Neurology and Pediatrics, University of Florida College of Medicine, Gainesville, Florida, USA
Email: s.subramony@neurology.ufl.edu
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These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.
This chapter of the Clinical Management Guidelines for Friedreich Ataxia and the recommendations and best practice statements contained herein were endorsed by the authors and the Friedreich Ataxia Guidelines Panel in 2022.
It is our expectation that going forward individual topics can be updated in real-time in response to new evidence versus a re-evaluation and update of all topics simultaneously.