Development and Testing of a Decision Aid on Goals of Care for Advanced Dementia

OBJECTIVES: Decision aids are effective to improve decision-making, yet they are rarely tested in nursing homes (NHs). Study objectives were to 1) examine the feasibility of a Goals of Care (GOC) decision aid for surrogate decision-makers (SDMs)of persons with dementia; and 2) test its effect on quality of communication and decision-making. DESIGN: Pre-post intervention to test a GOC decision aid intervention for SDMs for persons with dementia in NHs. Investigators collected data from reviews of resident health records and interviews with SDMs at baseline and 3-month follow up. SETTING: Two NHs in North Carolina. PARTICIPANTS: 18 residents who were over 65 years of age, had moderate to severe dementia on the Global Deterioration Scale (GDS=5,6,7), and an English-speaking surrogate decision-maker. INTERVENTION: 1) GOC Decision Aid video viewed by the SDM, and 2) a structured care plan meeting between the SDM and interdisciplinary NH team MEASUREMENTS: Surrogate knowledge, quality of communication with health care providers, surrogate-provider concordance on goals of care, and palliative care domains addressed in the care plan. RESULTS: 89% of the SDMs thought the decision aid was relevant to their needs. After viewing the video decision aid, SDMs increased the number of correct responses on knowledge-based questions (12.5 vs 14.2, P<.001). At 3 months they reported improved quality of communication scores (6.1 vs 6.8, P=.01) and improved concordance on primary goal of care with nursing home team (50% vs 78%, P=.003). The number of palliative care domains addressed in the care plan increased (1.8 vs 4.3, P<.001). CONCLUSION: The decision-support intervention piloted in this study was feasible and relevant for surrogate decision-makers of persons with advanced dementia in nursing homes, and it improved quality of communication between SDM and NH providers. A larger randomized clinical trial is underway to provide further evidence of the effects of this decision aid intervention

Current treatments in familial dysautonomia

INTRODUCTION: Familial dysautonomia (FD) is a rare hereditary sensory and autonomic neuropathy (type III). The disease is caused by a point mutation in the IKBKAP gene that affects the splicing of the elongator-1 protein (also known as IKAP). Patients have dramatic blood pressure instability due to baroreflex failure, chronic kidney disease, and impaired swallowing leading to recurrent aspiration pneumonia, which results in chronic lung disease. Diminished pain and temperature perception results in neuropathic joints and thermal injuries. Impaired proprioception leads to gait ataxia. Optic neuropathy and corneal opacities lead to progressive visual loss. AREAS COVERED: This article reviews current therapeutic strategies for the symptomatic treatment of FD, as well as the potential of new gene modifying agents. EXPERT OPINION: Therapeutic focus on FD is centered on reducing the catecholamine surges caused by baroreflex failure. Managing neurogenic dysphagia with effective protection of the airway passages and prompt treatment of aspiration pneumonias is necessary to prevent respiratory failure. Sedative medications should be used cautiously due to risk of respiratory depression. Non-invasive ventilation during sleep effectively manages apneas and prevents hypercapnia. Clinical trials of compounds that increase levels of IKAP (ELP-1) are underway and will determine whether they can reverse or slow disease progression