Topic 9.3. Management strategies for foot deformity 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.
Topic Contents
9.3 Management strategies for foot deformity in Friedreich ataxia
9.3.1 Surgery
9.3.2 Ankle foot orthotics
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.
9.3 Management strategies for foot deformity in Friedreich ataxia
John Flynn, David Lynch and Louise Corben
9.3.1 Surgery
There is little evidence to support the efficacy of orthopedic surgery for individuals with foot issues with Friedreich ataxia; however, expert opinion suggests surgery may be considered if it would assist in foot alignment to facilitate mobility and/or safe and effective transfers. In weighing up the advantages and disadvantages of foot surgery, the risk of infection, pain associated with the procedure, risk of loss of mobility in the post-operative recovery phase, sensory issues in managing post-operative casts/immobilization (if indicated), and the risk of intraoperative complications such as fluid loss/overload and associated cardiac issues should be considered.
9.3.2 Ankle foot orthotics
Previous guidelines indicate that ankle foot orthotics (AFOs) may be appropriate for people with FRDA to provide mediolateral stability at the ankle in stance phase, facilitate toe clearance in swing phase, promote heel strike at initial contact, prevent foot deformity, support normal joint alignment and biomechanics, improve range of motion and to facilitate function (25, 26).
See Chapters 3.2 and 3.3 for more details.
There are no studies specifically looking at orthotic prescription for people with FRDA; however, there are multiple studies describing the benefits of AFO prescription in improving postural security and movement, increasing weight-bearing percentage, improving knee control, reducing toe clawing, improving gait parameters, and improving standing and balance in individuals with other neurological conditions, such as stroke and spastic cerebral palsy (25-30).
Surgery for foot deformity
QUESTION: Should orthopedic surgery versus nothing be used for individuals with foot issues 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 conditionally recommend orthopedic surgery for individuals with foot issues with Friedreich ataxia if it is considered such surgery would assist in foot alignment to facilitate mobility and/or safe and effective transfers.
JUSTIFICATION: There is little evidence to support the efficacy of orthopedic surgery for individuals with foot issues with Friedreich ataxia; however, expert opinion suggests surgery may be considered if it would assist in foot alignment to facilitate mobility and/or safe and effective transfers. In weighing up the advantages and disadvantages of foot surgery, the risk of infection, pain associated with the procedure, risk of loss of mobility in the post-operative recovery phase, sensory issues in managing post-operative casts/immobilization (if indicated), and the risk of intraoperative complications such as fluid loss/overload and associated cardiac issues should be considered.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia with foot deformity.
Evidence to Recommendation Table PDFOrthotics for foot deformity
QUESTION: Should orthotics (including ankle-foot-orthotics, ankle braces and in-shoe orthotics) versus nothing be used for individuals with foot issues 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 conditionally recommend the use of orthotics (including ankle-foot orthotics, ankle braces and in-shoe orthotics) for individuals with foot issues with Friedreich ataxia.
JUSTIFICATION: Ankle-foot orthotics (AFOs) may be appropriate for people with Friedreich ataxia to provide mediolateral stability at the ankle in stance phase, facilitate toe clearance in swing phase, promote heel strike at initial contact, prevent foot deformity, support normal joint alignment and biomechanics, improve range of motion, and to facilitate function (25, 26). There are no studies specifically looking at orthotic prescription for people with Friedreich ataxia; however, there are multiple studies describing the benefits of AFO prescription in improving postural security and movement, increasing weight-bearing percentage, improving knee control, reducing toe clawing, improving gait parameters, and improving standing and balance in individuals with other neurological conditions, such as stroke and spastic cerebral palsy (25-30).
SUBGROUP CONSIDERATION: This recommendation is the Individuals with Friedreich ataxia with foot issues.
Evidence to Recommendation Table PDFPhysiotherapy for foot deformity
QUESTION: Should physiotherapy (including stretching, soft-tissue work) versus nothing be used for individuals with foot issues 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 physiotherapy (including stretching, soft-tissue work) or no physiotherapy for individuals with foot issues with Friedreich ataxia.
JUSTIFICATION: There is insufficient evidence to support the use of physiotherapy for foot issues with Friedreich ataxia.
SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia with foot issues.
Evidence to Recommendation Table PDFLay summary of clinical recommendations for foot deformity in Friedreich ataxia
Why these recommendations?
Individuals with Friedreich ataxia can have foot problems that make it difficult to stand, walk or transfer. There are some treatments for foot problems that might help to improve or maintain mobility.
There have not been many studies of the effect of surgery for foot issues in Friedreich ataxia. However, the opinion of healthcare experts who work with people with Friedreich ataxia is that surgery may be considered if it would benefit foot alignment which could help with walking and/or safety and independence in transfers.
Before considering surgery, it is important that alternatives such as ankle-foot orthotics (AFOs) have been tried. AFOs can help to stabilize the ankle when walking, prevent foot deformity, improve posture and standing, and improve walking. In particular, early and regular use of AFOs may mean a person with Friedreich ataxia and foot issues may not need surgery to correct foot deformity.
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 speak with your healthcare provider about Friedreich ataxia and foot deformity and what it means for you. When your healthcare provider is talking to you about the option of foot surgery and how it might help, it is important to also consider the risks before going ahead. These include the risk of infection, pain associated with surgery, possible loss of mobility in the recovery phase after surgery, managing casts/immobilization if that is needed, and the risk of complications during surgery (e.g., bleeding, heart issues).
Who are these recommendations specifically for?
These recommendations are specifically for individuals with Friedreich ataxia with foot deformity.
Louise Corben, PhD
Principal Research Fellow, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia
Email: louise.corben@mcri.edu.au
John M. (Jack) Flynn, MD
Richard M. Armstrong, Jr., Endowed Chair, Chief of Orthopaedic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
David Lynch, MD, PhD
Professor of Neurology and Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
Email: lynchd@mail.med.upenn.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.