17.1.R1 Recommendation
Advance care planning
- Strength of recommendation:
- Conditional for intervention
- Level of evidence:
- Low
How the strength and level of evidence are graded
What the strength of a recommendation means
The strength of a recommendation reflects the extent to which a guideline panel is confident that desirable effects of an intervention outweigh undesirable effects, or vice versa, across the range of patients for whom the recommendation is intended.
Strong for (↑↑) or against (↓↓) intervention
For patients: Most individuals in this situation would want the recommended course of action and only a small proportion would not.
For clinicians: Most individuals should receive the recommended course of action.
Conditional for (↑) or against (↓) intervention
For patients: The majority of individuals in this situation would want the suggested course of action, but many would not.
For clinicians: Recognize that different choices will be appropriate for different patients, and that you must help each patient arrive at a management decision consistent with her or his values and preferences.
Neither intervention nor comparison (—)
The panel did not recommend for or against the intervention.
Source: GRADE Handbook, section 6.1, Table 6.1 (GRADE uses “weak” and “conditional” interchangeably).
For the rating of the strength of the recommendation, in addition to evidence from studies in FRDA, evidence from like conditions, clinical experience and expert consensus are taken into account when published evidence is not available.
The level of evidence is based on published evidence from studies in FRDA. If there is no published evidence in FRDA, evidence from other like conditions or clinical expertise may have been used to make the recommendation – this is graded as ‘very low’ or in some cases ‘low’ level evidence. See the table below for an explanation of the symbols used to grade recommendations.
| Strength of recommendation | Symbol | Level of evidence | Symbol |
| Strong for intervention | ↑↑ | High | ⨁⨁⨁⨁ |
| Conditional for intervention | ↑ | Moderate | ⨁⨁⨁◯ |
| Neither intervention nor comparison | — | Low | ⨁⨁◯◯ |
| Conditional against intervention | ↓ | Very low | ⨁◯◯◯ |
| Strong against intervention | ↓↓ |
We conditionally recommend advance care planning (ACP) for individuals with Friedreich ataxia who have reached adulthood, have major complications such as heart failure, have experienced a significant change in their mobility, have dysphagia or have barriers to communication, bearing in mind that the published literature on ACP is in heart failure. ACP should also address the "future loss of dignity" by putting in place a safeguard that a person’s own values and wishes be respected in their care. This would help to implement a degree of control over a disease which is often out of the control of the person with Friedreich ataxia.
Question
Should advance care planning versus no advance care planning be used for individuals who have developed major complications such as diagnosis with heart failure, difficulty ambulating, dysphagia, or barriers to communication with Friedreich ataxia?
Justification
The Friedreich’s ataxia clinical management guideline patient and parent advisory panel felt this was a serious topic that needed to be addressed, and depending on the person’s circumstances, could be urgent. It has not been studied in those with Friedreich ataxia to date. Studies have demonstrated that an ACP program can be effective in facilitating end-of-life care consistent with patient preferences (1).
Subgroup consideration
Advance care planning is more important in adults with Friedreich ataxia, particularly since the life expectancy is between 40 and 50 years of age. For children and teenagers, if there is any evidence of life-altering or life-limiting illness, ACP should be discussed with them. The parent often makes several decisions for those under the age of consent, but children can often be included in a sensitive way and assent to healthcare decisions, and teenagers can often take a more active role in decision making.
Evidence to recommendation table (PDF)
Part of 17.1 Advance care planning
References (18)
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7. Rogers JG, Patel CB, Mentz RJ, Granger BB, Steinhauser KE, Fiuzat M, et al. Palliative Care in Heart Failure: The PAL-HF Randomized, Controlled Clinical Trial. J Am Coll Cardiol. 2017;70(3):331-41.
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15. Pousset F, Legrand L, Monin ML, Ewenczyk C, Charles P, Komajda M, et al. A 22-year follow-up study of long-term cardiac outcome and predictors of survival in Friedreich ataxia. JAMA Neurol. 2015;72(11):1334-41.
16. De Michele G, Perrone F, Filla A, Mirante E, Giordano M, De Placido S, et al. Age of onset, sex, and cardiomyopathy as predictors of disability and survival in Friedreich's disease: a retrospective study on 119 patients. Neurology. 1996;47(5):1260-4.
17. Lah S, Wilson EL, Beesley S, Sagy I, Orme J, Novack V, et al. Association of hospice utilization and publicly reported outcomes following hospitalization for pneumonia or heart failure: a retrospective cohort study. BMC Health Serv Res. 2018;18(1):12.
18. Yim CK, Barron Y, Moore S, Murtaugh C, Lala A, Aldridge M, et al. Hospice enrollment in patients with advanced heart failure decreases acute medical service utilization. Circ Heart Fail. 2017;10(3):e003335.
Disclaimer, intended use and 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.