7.2 Section
Management of fatigue
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.
Full text
Kathrin Reetz, Jӧrg B. Schulz, Caroline Spencer and Louise Corben
Although many individuals with FRDA report fatigue, there is little reported evidence regarding management strategies. However, the use of behavioral management, pharmacological intervention, alternative therapies and/or a specific diet have been reported in like conditions. These interventions are reviewed below.
7.2.1 Behavioral management
Due to a lack of studies, there is no direct evidence showing the benefits of behavioral management in individuals with FRDA. However, the clinical experience of the authors and published research in other neurological conditions, such as multiple sclerosis and neuromuscular diseases (2-9), favors (with low-level evidence) the consideration of using behavioral management for individuals who report fatigue with FRDA. This is because the overall benefit may be greater than not using behavioral management. Behavioral interventions include mindfulness, energy conservation, use of assistive devices, sleep hygiene, stress reduction, cognitive behavioral therapy, relaxation, and avoiding multitasking.
7.2.2 Physical activity
Due to a lack of studies, there is no direct evidence showing the benefits of physical activity programs in individuals with FRDA experiencing fatigue. However, the clinical experience of the authors and published research in other neurological conditions such as multiple sclerosis (10-15) favors (with moderate certainty of evidence) the consideration of physical activity programs for individuals who report fatigue with FRDA.
7.2.3 Pharmacological intervention
There is very low certainty of evidence demonstrating small beneficial effects of pharmacological intervention (antioxidants, antidepressants, e.g., idebenone, citalopram) for individuals with FRDA reporting fatigue.
7.2.4 Alternative therapies/physical modalities
Due to a lack of studies, there is no direct evidence showing the benefits of alternative therapies/ physical modalities in individuals with FRDA. There is only very limited evidence of beneficial effects from using alternative therapies/physical modalities (i.e., light therapy, cooling therapy, pulsed electromagnetic devices, acupuncture) in published research in other neurological conditions such as multiple sclerosis (16, 17). Based on current research evidence no recommendation for alternative therapies/physical modalities can be made for individuals with FRDA reporting fatigue.
7.2.5 Specific diet
Due to a lack of studies, there is no direct evidence showing the benefits of a specific diet (i.e., vitamins, coffee, Mediterranean, ketogenic) in individuals with FRDA. There is only very low-level evidence of beneficial effects of applying a specific diet in published research in other neurological conditions such as multiple sclerosis (18-20). Therefore, no recommendation for a specific diet can be made at this time.
Recommendations
Please note: Recommendations 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. Best Practice Statements are commonly accepted practices, as such formal rating of the quality of evidence by the GRADE process is not indicated. In addition if recommendations from the 2014 guidelines were deemed still relevant, although unable to undergo the scrutiny from a GRADE framework, they were also included as best practice statements.
7.2.R1 Behavioral management
- Strength of recommendation:
- Conditional for intervention
- Level of evidence:
- Low
We suggest behavioral management (mindfulness, energy conservation, use of assistive devices, sleep hygiene, stress reduction, cognitive behavioral therapy, relaxation, avoiding multitasking) may assist in managing fatigue in individuals with Friedreich ataxia.
Question, justification and subgroups · Evidence to recommendation table (PDF)
7.2.R2 Physical activity
- Strength of recommendation:
- Conditional for intervention
- Level of evidence:
- Moderate
We suggest a physical activity program be used to manage fatigue in individuals with Friedreich ataxia.
Question, justification and subgroups · Evidence to recommendation table (PDF)
7.2.R3 Pharmacological interventions
- Strength of recommendation:
- Conditional for intervention
- Level of evidence:
- Low
We suggest some pharmacological intervention (antioxidants, antidepressants) may have value in managing fatigue related to Friedreich ataxia.
Question, justification and subgroups · Evidence to recommendation table (PDF)
7.2.R4 Alternative therapies/physical modalities
- Strength of recommendation:
- Neither intervention nor comparison
- Level of evidence:
- Very low
We cannot recommend either alternative therapies/physical modalities (i.e., light therapy, cooling therapy, pulsed electromagnetic devices, acupuncture) or no alternative therapies/physical modalities be used for all individuals who report fatigue with Friedreich ataxia.
Question, justification and subgroups · Evidence to recommendation table (PDF)
7.2.R5 Specific diet
- Strength of recommendation:
- Neither intervention nor comparison
- Level of evidence:
- Very low
We cannot recommend any specific diet over usual diet to assist in managing fatigue in individuals with Friedreich ataxia.
Question, justification and subgroups · Evidence to recommendation table (PDF)
Best practice statements
Please note: Recommendations 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. Best Practice Statements are commonly accepted practices, as such formal rating of the quality of evidence by the GRADE process is not indicated. In addition if recommendations from the 2014 guidelines were deemed still relevant, although unable to undergo the scrutiny from a GRADE framework, they were also included as best practice statements.
- 7.2.BPS1 Sometimes it may be difficult for individuals with Friedreich ataxia to identify if they suffer from fatigue. In this case, they should be offered a detailed assessment of fatigue including administration of a standardized assessment such as the Modified Fatigue Impact Scale (21) and enquiry about the impact of fatigue on the capacity to participate in daily activities.
Lay summary
Lay summary of clinical recommendations for fatigue in Friedreich ataxia
Individuals with Friedreich ataxia often experience fatigue, which can have widespread effects on many domains of a person’s life. Fatigue can hamper participating in leisure, society, employment, physical activity and daily activities.
Why these recommendations?
There is a range of strategies that have been suggested as being helpful in managing fatigue, but due to a lack of studies in people with Friedreich ataxia, it is not yet known which strategies give the most benefit.
Behavioral management
Behavioral management techniques to manage fatigue include mindfulness, energy conservation, use of assistive devices, sleep hygiene, stress reduction, cognitive behavioural therapy (CBT), relaxation, and avoiding multitasking. While there is little published evidence that these techniques help to treat fatigue in individuals with Friedreich ataxia, the clinical experience of the authors and published research in other neurological conditions, such as multiple sclerosis and neuromuscular diseases, suggest the use of behavioural management techniques may also give individuals with Friedreich ataxia some relief from fatigue.
Physical activity
Due to a lack of studies, there is no direct evidence showing the benefits of physical activity programs in individuals with Friedreich ataxia experiencing fatigue. However, the clinical experience of the authors and published research in other neurological conditions, such as multiple sclerosis, suggests that physical activity programs for individuals with Friedreich ataxia who report fatigue could be considered.
Medication
Due to the small and diverse nature of current research studies, there is only low-level evidence that medicines (antioxidants, antidepressants) may be of small benefit for managing fatigue in individuals with Friedreich ataxia.
Alternative therapies/physical modalities
Alternative therapies/physical modalities (light therapy, cooling therapy, pulsed electromagnetic devices, acupuncture) have been suggested as helpful for managing fatigue, but no studies have been done in individuals with Friedreich ataxia. There is some low-level evidence that these techniques may be of benefit in other neurological conditions such as multiple sclerosis. Based on current research, we cannot recommend the use of alternative therapies/physical modalities for individuals with Friedreich ataxia experiencing fatigue.
Specific diet
It has been suggested that specific diets (including vitamins, coffee, Mediterranean, ketogenic) may be helpful for fatigue. This has not been studied in individuals with Friedreich ataxia. There is some low-level evidence showing some benefit from specific diets in other neurological conditions such as multiple sclerosis. Based on current research, we cannot recommend specific diets for individuals with Friedreich ataxia experiencing fatigue.
What does this mean for you as a person living with Friedreich ataxia or caring for someone living with Friedreich ataxia?
If you are suffering from fatigue, which is frequent in patients with Friedreich ataxia, it might be important for you to talk to your allied health care provider and doctor to see if behavioural management, physical activity or in some cases, medicines may be appropriate for you.
Who are these recommendations specifically for?
These recommendations are relevant to all individuals with Friedreich ataxia experiencing fatigue.
Authors
References
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Methodology
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.
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.