Topic 14.3. Management of anxiety in Friedreich ataxia
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.
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.
14.3 Management of anxiety in Friedreich ataxia
Caroline Spencer, Alexandra Durr, George Wilmot, Susan Perlman and Louise Corben
Whilst anxiety has not often been explicitly studied in individuals with FRDA, it appears to not be as common as depression. In a large cohort (n=650) of individuals with FRDA, 20 (3.1%) reported experiencing anxiety (15). Importantly, 18 of those 20 were in the typical onset group, which had a prevalence of 3.3% (15). The clinical experience of the authors indicates there is no difference in response to anti-anxiety medication in individuals with FRDA, compared to those without FRDA. There have not been any studies in FRDA, but if anti-anxiety medication is efficacious as in the general population, then the desirable effect on individuals who are anxious would be large.
It is important to make sure individuals with FRDA are screened and clinicians are alert to the possibility of anxiety. In addition, clinicians should explore what will work best for an individual with FRDA, particularly in the context of other aspects of their lives, such as accessibility requirements of face-to-face counseling and time constraints for work/study/family. The clinician may need to consider treating symptoms of anxiety with both counseling and medication.
Anti-anxiety medication
QUESTION: Should anti-anxiety medication versus none be used for anxiety in individuals with Friedreich ataxia?
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RECOMMENDATION: We conditionally recommend the use of anti-anxiety medication in individuals with Friedreich ataxia who present with symptoms of anxiety.
JUSTIFICATION: Cohort studies indicate the presence of anxiety symptoms in individuals with Friedreich ataxia. Whilst there is no published evidence in Friedreich ataxia, if treatment is efficacious then the desirable effect on individuals who are anxious would be large. Clinical experience of the authors indicates there is no difference in response to medication in individuals with Friedreich ataxia, when they accept the use of medication, compared to those without Friedreich ataxia. It is important to make sure individuals with Friedreich ataxia are screened and clinicians are alert to the possibility of anxiety.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia and symptoms of anxiety and should be implemented according to an assessment of the severity of anxiety. Balance of side-effects versus efficacy needs to be taken into account when considering treatment options.
Evidence to Recommendation Table PDFCounseling for anxiety
QUESTION: Should counseling or therapy versus none or anti-anxiety prescription be used for anxiety in individuals with Friedreich ataxia?
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RECOMMENDATION: We recommend counseling or therapy over no counseling for individuals with Friedreich ataxia who present with symptoms of anxiety.
JUSTIFICATION: While there is no published evidence in Friedreich ataxia, if counseling is efficacious then the desirable effect on individuals who are anxious would be large. It is important clinicians explore what will work best for an individual with Friedreich ataxia, particularly in the context of other aspects of their lives (accessibility requirements of face-to-face counseling, time constraints for work/study/family, etc.). The clinician may need to consider treating symptoms of anxiety with both counseling and medication.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia and symptoms of anxiety. It may be difficult for a person with severe dysarthria to engage in counseling. Consideration should be given to a possible link between visual disturbance and the emergence of anxiety.
Evidence to Recommendation Table PDFLifestyle changes for anxiety
QUESTION: Should lifestyle changes (exercise, diet, social activities) versus none or anti-anxiety treatment be used for anxiety patients with Friedreich ataxia?
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RECOMMENDATION: We conditionally recommend against lifestyle changes as a primary intervention to treat anxiety in individuals with Friedreich ataxia, favoring anti-anxiety medication or counseling prior to or in conjunction with any lifestyle changes.
JUSTIFICATION: There is a lack of research on the effectiveness of lifestyle changes in treating anxiety in individuals with Friedreich ataxia. Medication and/or counseling are likely to be more efficacious.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia and symptoms of anxiety.
Evidence to Recommendation Table PDFLay summary of clinical recommendations for anxiety in Friedreich ataxia
Individuals with Friedreich ataxia often report mental health concerns. These issues can vary in nature throughout the person’s life, and often include anxiety. Mental health concerns can affect physical, emotional, and social wellbeing.
Why these recommendations?
There are various strategies that may help a person with Friedreich ataxia experiencing anxiety.
Medication
Due to a lack of studies, there is no direct evidence showing the benefits of anti-anxiety medication for treating anxiety in individuals with Friedreich ataxia. However, if medication is effective for treating anxiety symptoms, the benefit to the individual would be large. Ways to limit undesirable side effects (such as dizziness or worsening balance), which would likely have a greater impact on individuals with Friedreich ataxia than other people, should be considered.
Counselling
While there is no direct evidence for the benefits of counselling in individuals with Friedreich ataxia who have anxiety, we recommend counselling to treat these conditions. If counselling is effective, the benefit would be large and there are unlikely to be any undesirable effects.
Lifestyle changes
There is little research or clinical experience indicating that lifestyle changes will improve anxiety. Thus, we recommend other interventions, such as medication or counselling, over lifestyle changes for individuals experiencing anxiety.
What does this mean for you as a person living with Friedreich ataxia or caring for someone living with Friedreich ataxia?
It is important to talk to your doctor or other healthcare professional if you feel anxiety may be an issue for you or someone you care for. A health professional will assess whether the use of behavioural management, counselling or medication may be appropriate for you.
Who are these recommendations specifically for?
These recommendations are relevant to all individuals with Friedreich ataxia who have symptoms of anxiety. Consideration should be given to treatments that are best suited to the age of the individual, the specific diagnosis and the severity of symptoms.
Louise Corben, PhD
Principal Research Fellow, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia
Email: louise.corben@mcri.edu.au
Alexandra Durr, MD, PhD
Professor of Neurogenetics, Sorbonne Université, Paris, France
Email: alexandra.durr@icm-institute.org
Susan Perlman, MD
Clinical Professor of Neurology, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
Email: sperlman@ucla.edu
Caroline Spencer, PhD
Postdoctoral Fellow, Boston University, Boston, Massachusetts, USA
Email: cspencer@bu.edu
George Wilmot, MD, PhD
Associate Professor, Department of Neurology, Emory University, Atlanta, Georgia, USA
Email: gwilmot@emory.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.