16.R4 Recommendation
Communication and workplace assistance
- Strength of recommendation:
- Conditional for intervention
- Level of evidence:
- Very low
How the strength and level of evidence are graded
What the strength of a recommendation means
The strength of a recommendation reflects the extent to which a guideline panel is confident that desirable effects of an intervention outweigh undesirable effects, or vice versa, across the range of patients for whom the recommendation is intended.
Strong for (↑↑) or against (↓↓) intervention
For patients: Most individuals in this situation would want the recommended course of action and only a small proportion would not.
For clinicians: Most individuals should receive the recommended course of action.
Conditional for (↑) or against (↓) intervention
For patients: The majority of individuals in this situation would want the suggested course of action, but many would not.
For clinicians: Recognize that different choices will be appropriate for different patients, and that you must help each patient arrive at a management decision consistent with her or his values and preferences.
Neither intervention nor comparison (—)
The panel did not recommend for or against the intervention.
Source: GRADE Handbook, section 6.1, Table 6.1 (GRADE uses “weak” and “conditional” interchangeably).
For the rating of the strength of the recommendation, in addition to evidence from studies in FRDA, evidence from like conditions, clinical experience and expert consensus are taken into account when published evidence is not available.
The level of evidence is based on published evidence from studies in FRDA. If there is no published evidence in FRDA, evidence from other like conditions or clinical expertise may have been used to make the recommendation – this is graded as ‘very low’ or in some cases ‘low’ level evidence. See the table below for an explanation of the symbols used to grade recommendations.
| Strength of recommendation | Symbol | Level of evidence | Symbol |
| Strong for intervention | ↑↑ | High | ⨁⨁⨁⨁ |
| Conditional for intervention | ↑ | Moderate | ⨁⨁⨁◯ |
| Neither intervention nor comparison | — | Low | ⨁⨁◯◯ |
| Conditional against intervention | ↓ | Very low | ⨁◯◯◯ |
| Strong against intervention | ↓↓ |
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.
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?
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 (PDF)
Part of 16 Digital and assistive technologies in Friedreich ataxia
References (32)
1. Marsden J, Harris C. Cerebellar ataxia: pathophysiology and rehabilitation. Clin Rehabil. 2011;25(3):195-216.
2. Clark J, Shona M, Morrow M. Wheelchair postural support for young people with progressive neuromuscular disorders. International Journal of Therapy and Rehabiliation 2004;11(8):365-73.
3. Wendrich K, van Oirschot P, Martens MB, Heerings M, Jongen PJ, Krabbenborg L. Toward digital self-monitoring of multiple sclerosis: investigating first experiences, needs, and wishes of people with MS. Int J MS Care. 2019;21(6):282-91.
4. Londral A, Pinto A, Pinto S, Azevedo L, De Carvalho M. Quality of life in amyotrophic lateral sclerosis patients and caregivers: Impact of assistive communication from early stages. Muscle Nerve. 2015;52(6):933-41.
5. Broadhurst MJ, Stammers CW. Mechanical feeding aids for patients with ataxia: design considerations. J Biomed Eng. 1990;12(3):209-14.
6. Wyckoff E. The spoon phase is another option for self-feeding. Am J Occup Ther. 1993;47(9):851.
7. Morgan MH. Ataxia and weights. Physiotherapy. 1975;61(11):332-4.
8. Morrice BL, Becker WJ, Hoffer JA, Lee RG. Manual tracking performance in patients with cerebellar incoordination: effects of mechanical loading. Can J Neurol Sci. 1990;17(3):275-85.
9. Dahlin-Webb SR. A weighted wrist cuff. Am J Occup Ther. 1986;40(5):363-4.
10. Sanes JN, LeWitt PA, Mauritz KH. Visual and mechanical control of postural and kinetic tremor in cerebellar system disorders. J Neurol Neurosurg Psychiatry. 1988;51(7):934-43.
11. Bastian AJ. Mechanisms of ataxia. Phys Ther. 1997;77(6):672-5.
12. Gillen G. Improving activities of daily living performance in an adult with ataxia. Am J Occup Ther. 2000;54(1):89-96.
13. Davis AE, Lee RG. EMG biofeedback in patients with motor disorders: an aid for co-ordinating activity in antagonistic muscle groups. Can J Neurol Sci. 1980;7(3):199-206.
14. Guercio J, Chittum R, McMorrow M. Self-management in the treatment of ataxia: a case study in reducing ataxic tremor through relaxation and biofeedback. Brain Inj. 1997;11(5):353-62.
15. Guercio JM, Ferguson KE, McMorrow MJ. Increasing functional communication through relaxation training and neuromuscular feedback. Brain Inj. 2001;15(12):1073-82.
16. Mortenson WB, Miller WC, Miller-Pogar J. Measuring wheelchair intervention outcomes: Development of the wheelchair outcome measure. Disabil rehabilitation: Assist Technol. 2007;2(5):275-85.
17. Richardson M, Frank AO. Electric powered wheelchairs for those with muscular dystrophy: problems of posture, pain and deformity. Disabil Rehabil Assist Technol. 2009;4(3):181-8.
18. Cooper RA, Koontz AM, Ding D, Kelleher A, Rice I, Cooper R. Manual wheeled mobility--current and future developments from the human engineering research laboratories. Disabil Rehabil. 2010;32(26):2210-21.
19. Burgman I. The trunk/spine complex and wheelchair seating for children: A literature review Aust Occup Ther J. 1994;41(3):123-32.
20. Stockton L, Gebhardt KS, Clark M. Seating and pressure ulcers: clinical practice guideline. J Tissue Viability. 2009;18(4):98-108.
21. McCarthy RE. Management of neuromuscular scoliosis. Orthop Clin North Am. 1999;30(3):435-49.
22. Holmes KJ, Michael SM, Thorpe SL, Solomonidis SE. Management of scoliosis with special seating for the non-ambulant spastic cerebral palsy population – a biomechanical study. Clinical Biomechanics. 2003;18:480-7.
23. Ferrarin M, Andreoni G, Pedotti A. Comparative biomechanical evaluation of different wheelchair seat cushions. J Rehabil Res Dev. 2000;37(3):315-24.
24. De Souza LH, Frank AO. Rare diseases: matching wheelchair users with rare metabolic, neuromuscular or neurological disorders to electric powered indoor/outdoor wheelchairs (EPIOCs). Disabil Rehabil. 2016;38(16):1547-56.
25. Parent F, Dansereau J, Lacoste M, Aissaouri R. Evaluation of the new flexible contour backrest for wheelchairs J Rehabil Res Dev. 2000;37(3):325-33.
26. Liu HY, Pearlman J, Cooper R, Hong EK, Wang H, Salatin B, et al. Evaluation of aluminum ultralight rigid wheelchairs versus other ultralight wheelchairs using ANSI/RESNA standards. J Rehabil Res Dev. 2010;47(5):441-55.
27. Freixes O, Fernandez SA, Gatti MA, Crespo MJ, Olmos LE, Rubel IF. Wheelchair axle position effect on start-up propulsion performance of persons with tetraplegia. J Rehabil Res Dev. 2010;47(7):661-8.
28. Salminen AL, Brandt A, Samuelsson K, Toytari O, Malmivaara A. Mobility devices to promote activity and participation: a systematic review. J Rehabil Med. 2009;41(9):697-706.
29. Mountain AD, Kirby RL, Smith C. Skills training: an important component of powered wheelchair use. Clin Rehabil. 2009;23(3):287.
30. Mateo I, Llorca J, Volpini V, Corral J, Berciano J, Combarros O. Expanded GAA repeats and clinical variation in Friedreich's ataxia. Acta Neurol Scand. 2004;109(1):75-8.
31. La Pean A, Jeffries N, Grow C, Ravina B, Di Prospero NA. Predictors of progression in patients with Friedreich ataxia. Mov Disord. 2008;23(14):2026-32.
32. Delatycki MB, Paris DB, Gardner RJ, Nicholson GA, Nassif N, Storey E, et al. Clinical and genetic study of Friedreich ataxia in an Australian population. Am J Med Genet. 1999;87(2):168-74.
Disclaimer, intended use and 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.