Topic 5.2. Strategies for fluid and operative management – fluids
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
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.
5.2 Strategies for fluid and operative management – fluids
Roger Peverill, David Lynch and Ronald Mark Payne are acknowledged for the content of this section from the previous version of the guidelines (2014).
Individuals with FRDA are at increased risk of cardiovascular instability and complications during major surgery particularly if major fluid shifts are part of the surgical procedure.
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.
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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.