Patient-centered Decision Making in Amyotrophic Lateral Sclerosis: Where are we?

Abstract

Practice pointsr Amyotrophic lateral sclerosis patients are supported by models of patient-centered decision making delivered through specialized multidisciplinary clinics. r Advances in research have led to changes in service delivery, with new members added to the multidisciplinary team. r Developments in genetics research have increased the complexity of patients' choices for genetic testing. r User-designed decision support tools could improve communication between patients' families and clinicians, to negotiate this complex and ever-changing care.Developments in amyotrophic lateral sclerosis research and care delivery have created new arenas, and new dilemmas, for patients' decision making. This review explores three aspects of amyotrophic lateral sclerosis patient-centered care and decision making: patient-centered service delivery through the expanding multidisciplinary team; decision making for genetic testing and the implications of undergoing testing; and development of user-designed decision support tools to help patients and families make decisions as their choices become more complex. Until a cure is found, well-timed and effective decision making will rely on patient and family preferences to guide them through an increasingly complicated disease landscape. Amyotrophic lateral sclerosis (ALS) is a disease in search of a cure, or at least a highly effective treatment that will alleviate symptoms and slow disease progression. Until this can be achieved, people living with ALS, and their families, rely on healthcare services to help them manage their symptoms and maximize their quality of life as their needs increase. Best practice for people with ALS adopts a multidisciplinary approach of specialized care [1] that draws on patient-(or person-) centered principles and practices for cohesive and collaborative care [2].Patient-centered care, and the decision making that underpins it, have been defined, conceptualized and modeled many times [3][4][5][6][7]. In short, four principles [5] apply: affording people dignity, respect and compassion; offering coordinated care; offering personalized care; and enabling people to develop their unique range of capabilities to participate in their own care. Shared decision making, where patients and health professionals collaborate to arrive at a care decision, is considered best practice for patient-centered healthcare delivery [8]. Although preferences for sharing decision making vary with age, cultural background and disease setting [9][10][11], a majority of patients prefer to make their decisions using this approach [9]. Decision making for ALS care is often more complicated than for other degenerative conditions, as continual change in the patient's condition and circumstances mean that decision making is ongoing throughout the, frequently brief, course of the disease. Time frames for choosing and implementing a treatment may be short; and the number of health professionals to consult may be extensive; with multiple decisions under consideration at one time.

Giving and receiving a diagnosis of a progressive neurological condition: A scoping review of doctors’ and patients’ perspectives

“…Effective information provision and patient education are considered two of the pillars of patient-centred care, a model of care considered appropriate for individuals with chronic conditions and complex health care needs such as MND [75], MS [76] and PD [77]. However, patients in the studies included in this review often expressed their dissatisfaction with both the amount and nature of information they received (or did not receive) during the delivery of their diagnosis.…”

“…Qualitative studies and qualitative comments in quantitative studies sometimes highlighted the need for doctors to show more empathy. Patients often felt that their doctors did not approach such a serious diagnosis in a caring and sensitive way and were described as 'detached', 'very clinical' and 'insensitive' [31,38]. Similarly, Pavey et al [34] described that patients considered that doctors were unwilling to be personally involved and offer emotional support; a participant in the Hughes et al [33] study also reported feeling 'dehumanised'.…”

Current practices and barriers in gastrostomy indication in amyotrophic lateral sclerosis: a survey of ALS care teams in The Netherlands

“…Additionally, it seems important to explore motivations and emotions underpinning patient preferences, but also possible cognitive deficits (30) and low health literacy which can negatively influence patient decision-making. Decision aids have been proven effective in supporting decision-making ( 33) and can combine patient information with questions prompting patients to reflect on their preferences to better prepare them to discuss the decision with HCP's (34). Finally, all respondents in our survey included the family of the patient in the decisionmaking process and provided patients and their families with relevant information.…”

“…Third, ideally, decision aids and other information should be developed together with patients to provide patient information and support patients in exploring their preferences, which can help physicians to better explore patient readiness and tailor decision-making to individual patient needs. This is not an easy process, but the research group of Hogden and colleagues in Australia provide a useful development pathway that could provide guidance (18,26,34,36). Fourth, prospective studies on gastrostomy in ALS should be conducted aimed at providing conclusive evidence on efficacy on survival, weight, and quality of life, and on optimal timing.…”

Genetisk utredning ved amyotrofisk lateral sklerose

“…Disse må forholde seg til at deres risiko for å utvikle sykdommen kan vaere betydelig økt og må ta stilling til om de ønsker prediktiv testing, som etter norsk lov krever genetisk veiledning. Usikkerheten rundt betydningen av både positive og negative funn gjør at denne verken kan gi friske slektninger et tydelig beslutningsgrunnlag før testing eller sikker avklaring av egen risiko når testresultatet foreligger (18). Dette skiller seg fra Huntingtons sykdom, der en negativ gentest utelukker sykdommen og et positivt funn også gir viktig informasjon om forventet prognose (19).…”