Topic 7.2. 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.
Topic Contents
7.2 Management of fatigue
7.2.1 Behavioral management
7.2.2 Physical activity
7.2.3 Pharmacological intervention
7.2.4 Alternative therapies/physical modalities
7.2.5 Specific diet
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.
7.2 Management of fatigue
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.
Behavioral management
QUESTION: Should behavioral management (mindfulness, energy conservation, use of assistive devices, sleep hygiene, stress reduction, CBT, relaxation, avoiding multitasking) versus no behavioral management be used for all individuals who report fatigue 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: 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.
JUSTIFICATION: There is low-level evidence indicating benefits in other diseases with similar characteristics to Friedreich ataxia.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia who report fatigue.
Evidence to Recommendation Table PDFPhysical activity
QUESTION: Should physical activity program (exercise such as aerobic exercise, strengthening exercise, yoga, tai chi, dancing) versus no activity program be used for all individuals who report fatigue 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: We suggest a physical activity program be used to manage fatigue in individuals with Friedreich ataxia.
JUSTIFICATION: Most of the evidence reviewed for this recommendation was based on studies in multiple sclerosis. These studies indicate a favorable effect of physical activity programs on fatigue. The majority of expert authors surveyed consider a physical activity program to be of benefit for managing fatigue in individuals with Friedreich ataxia.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia who have no medical contraindications to participating in a physical activity program. Those individuals with other medical comorbidities/contraindications for exercise should work with their healthcare team to carefully consider the risks and benefits of an exercise program before deciding to begin one.
Evidence to Recommendation Table PDFPharmacological interventions
QUESTION: Should pharmacological intervention (antioxidants, antidepressants, e.g., idebenone, citalopram) versus no pharmacological intervention be used for people 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: We suggest some pharmacological intervention (antioxidants, antidepressants) may have value in managing fatigue related to Friedreich ataxia.
JUSTIFICATION: : Based on current small studies, there is only low certainty of evidence of small desirable effects of pharmacological intervention (antioxidants, antidepressants) for individuals with Friedreich ataxia reporting fatigue; thus, only a conditional recommendation can be made.
Most studies of pharmacological interventions use clinical scales such as the ICARS/mFARS/SARA as outcome measures. Expert authors do not consider these scales to be sensitive enough to capture the extent and impact of fatigue. Indeed, in as yet unpublished evidence, one expert author found several patients reported that when they started using a wheelchair or a walker, their fatigue actually improved. So, although their ataxia severity score was likely to have got worse (reflected by moving to a wheelchair), their fatigue improved. Also, there is a lot of day to day fluctuation in fatigue, whereas there is unlikely to be comparable daily fluctuation in the ataxia severity score.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia who report fatigue.
Evidence to Recommendation Table PDFAlternative therapies/physical modalities
QUESTION: Should alternative therapies/physical modalities (i.e., light therapy, cooling therapy, pulsed electromagnetic devices, acupuncture) versus no alternative therapies/physical modalities be used for all individuals who report fatigue 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: 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.
JUSTIFICATION: There is little evidence supporting the benefits of alternative therapies on fatigue in Friedreich ataxia. Undesirable effects are unclear; hence, we cannot make a recommendation either way.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia who report fatigue.
Evidence to Recommendation Table PDFSpecific diet
QUESTION: Should a specific diet (i.e., vitamins, coffee, Mediterranean, ketogenic) versus usual diet be used for all individuals who report fatigue 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: We cannot recommend any specific diet over usual diet to assist in managing fatigue in individuals with Friedreich ataxia.
JUSTIFICATION: There is insufficient evidence and clinical experience to support the use of specific diets to manage fatigue in individuals with Friedreich ataxia.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia who report fatigue.
Evidence to Recommendation Table PDFLay 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.
Principal Research Fellow, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia
Email: louise.corben@mcri.edu.au
Kathrin Reetz, MD
Professor for Translational Neurodegeneration, Department of Neurology, RWTH Aachen University, Aachen, NRW, Germany
Email: kreetz@ukaachen.de
Jӧrg B. Schulz, MD
Chair of Neurology, Department of Neurology, RWTH Aachen University, Aachen, NRW, Germany
Caroline Spencer, PhD
Postdoctoral Fellow, Boston University, Boston, Massachusetts, USA
Email: cspencer@bu.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.