Topic 5.4. Strategies for fluid and operative management – anesthesia
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.
5.4 Strategies for fluid and operative management – anesthesia
Ludger Schöls, Kimberley Y. Lin, Ronald Mark Payne, Lisa Montenegro and David Lynch
Kelly Sullivan and Theresa Zesiewicz are acknowledged for some content taken from the previous version of the guidelines (2014).
Peri-operative pain management is a concern in managing people with FRDA. Surgeries and associated anesthesia may be required to treat cardiac, orthopedic and obstetric issues. However, diabetes mellitus, cardiomyopathy and compromised pulmonary function make anesthetic management challenging.
While practice guidelines have not been established, there are case reports of successful anesthesia using remifentanil and propofol (7), alfentanil and propofol (8), and isoflurane (3) in individuals with FRDA. Use of muscle relaxants has been reported with mixed results in individuals with FRDA and there has been one report of hypersensitivity to tubocurarine (9). A more recent report by Schmitt et al (10) described the response of two adolescent girls with FRDA to rocuronium. In particular, the clinical duration of rocuronium for both girls was comparable to children without neuromuscular disease, indicating successful anesthetic management (10). Other reports describe a normal or near normal response to various nondepolarizing neuromuscular blocking agents, such as tubocurarine, atracurium and vecuronium (11, 12). Schmitt and colleagues (10) recommend accurate assessment of neuromuscular block throughout anesthesia for individuals with FRDA. In the case of obstetrics, successful use of fentanyl and bupivacaine has been reported in a case of a vaginal delivery (13) and a caesarean section (14). More recently, Ozgul and colleagues reported the successful anesthetic management of a person who underwent posterior spinal fusion (15).
A related review of the effects of anesthesia in individuals with a variety of muscular dystrophies (which did not include people with FRDA) reports cardiac complications associated with inhaled anesthetics and post-operative rhabdomyolysis (16). The authors recommend avoiding use of succinylcholine which has been associated with life-threatening hyperkalemia (16). This recommendation may also be applicable to individuals with FRDA.
Finally, changes to cardiac function in people with FRDA indicate the capacity to tolerate lower blood pressure and large fluid shift as may be associated with surgery and anesthesia may be compromised. It is important to carefully monitor fluid loss and cardiovascular function during extensive surgery in FRDA (17).
Propofol has been used in a large number of individuals with Friedreich ataxia and there is no published evidence that documents adverse events related to the use of propofol in this population. This suggests that theoretical concerns of possible mitochondrial toxicity with propofol are not likely to be clinically meaningful.
Lay summary of clinical recommendations/best practice statements for surgical and anesthetic considerations in Friedreich ataxia
Why these recommendations/best practice statements?
Friedreich ataxia is often associated with heart problems, scoliosis, diabetes and reduced lung function. This means individuals with Friedreich ataxia may be at greater risk of complications while undergoing surgery than other people.
Particular types of anesthetics and careful monitoring of heart function and fluids may be needed when an individual with Friedreich ataxia has surgery. In addition, surgery and the reduction in activity after surgery may mean a person with Friedreich ataxia will quickly lose physical condition while they recover from the surgery.
What does this mean for you as a person living with Friedreich ataxia or caring for someone living with Friedreich ataxia?
If you are considering surgery it might be important for you to speak with your healthcare professional about Friedreich ataxia and surgery and what it means for you.
We suggest that individuals with Friedreich ataxia considering surgery should be seen by a cardiologist before surgery. In addition, monitoring of heart function should occur during surgery, particularly if there is significant loss of blood or extra fluid is required. Ideally surgery should be done in a center with cardiac intensive care facilities. It is best if mobilization (getting out of bed) commences as soon as possible after surgery.
Health providers may consider a range of anesthetic options for you, including spinal nerve blocks or anesthetics given intravenously.
Who are these recommendations/best practice statements specifically for?
These recommendations and best practice statements are for all individuals with Friedreich ataxia who may be considering or are undergoing surgery.
Associate Professor, 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
Lisa Montenegro
Assistant Professor/Anesthesia, Perelman School of Medicine, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
Email: lisa.montenegro@gmail.com
Ronald Mark Payne, MD
Professor of Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana, USA
Email: rpayne@iu.edu
Ludger Schöls, MD
Professor of Neurology, Eberhard-Karls University, Tübingen, Germany
Email: Ludger.Schoels@uni-tuebingen.de
Kelly Sullivan and Theresa Zesiewicz are acknowledged for some content taken from the previous version of the guidelines (2014).
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7. Pancaro C, Renz D. Anesthetic management in Friedreich’s ataxia. Paediatr Anaesth. 2005;15(5):433-4.
8. Levent K, Yavuz G, Kamil T. Anaesthesia for Friedreich’s ataxia. Case report. Minerva Anestesiol. 2000;66(9):657-60.
9. Kume M, Zin T, Oyama T. [Anesthetic experience with a patient with Friedreich’s ataxia]. Masui – Japanese Journal of Anesthesiology. 1976;25(9):877-80.
10. Schmitt HJ, Wick S, Munster T. Rocuronium for muscle relaxation in two children with Friedreich’s ataxia. Br J Anaesth. 2004;92(4):592-6.
11. Mouloudi H, Katsanoulas C, Frantzeskos G. Requirements for muscle relaxation in Friedreich’s ataxia. Anaesthesia. 1998;53(2):177-80.
12. Bell CF, Kelly JM, Jones RS. Anaesthesia for Friedreich’s ataxia. Case report and review of the literature. Anaesthesia. 1986;41(3):296-301.
13. Wyatt S, Brighouse D. Anaesthetic management of vaginal delivery in a woman with Friedreich’s ataxia complicated by cardiomyopathy and scoliosis. Int J Obstet Anesth. 1998;7(3):185-8.
14. Harmon D. Anaesthesia for caesarean section in a parturient with Friedreich’s ataxia. Int J Obstet Anesth. 2001;10(2):147-8.
15. Ozgul UME, Aydogan M, Korkmaz M, Nakir H. Anesthethic management on a patient with Friedreich’s ataxia: Case report. Medicine Science. 2013;2(4):928-38.
16. Gurnaney H, Brown A, Litman RS. Malignant hyperthermia and muscular dystrophies. Anesth Analg. 2009;109(4):1043-8.
17. Payne RM, Wagner GR. Cardiomyopathy in Friedreich ataxia: clinical findings and research. J Child Neurol. 2012;27(9):1179-86.
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.