Abstract
Progressive bulbar palsy (PBP) is a form of motoneuron disease and is widely classified as a subtype of amyotrophic lateral sclerosis (ALS) with a shorter time of survival and female predominance. In this retrospective case series of 14 patients with PBP, we focus on challenges in palliative care for this patient cohort, including symptom control, gastrostomy, non-invasive ventilation, and end-of-life phase. We show that rapid physical decline at the end of life is associated with bronchopulmonary infection and excessive oral secretion leading to a high level of symptom burden. Early and regular advance care planning discussions with a focus on oral secretion management with patients and caregivers are crucial.
Highlights
Integration of palliative care is an important task when treating patients with amyotrophic lateral sclerosis (ALS) [1, 2]
Progressive bulbar palsy (PBP) patients may stay independent in self-care and mobility but have many symptoms, and medical interventions can lead to specific challenges
The median time of survival and female predominance was similar to the characteristics defined by others [3, 5]
Summary
Integration of palliative care is an important task when treating patients with amyotrophic lateral sclerosis (ALS) [1, 2]. The rapid progression of the disease and the question of whether or not to include life-prolonging therapies, such as feeding tube placement or ventilatory support, should be a stimulus for early discussion regarding therapeutic limitations and advance care planning (ACP). ALS and motoneuron disease (MND) are generic terms for a range of different phenotypes defined by a varying involvement of spinal and bulbar upper and lower motoneurons [3]. Progressive bulbar palsy (PBP), or bulbar phenotype, is defined as bulbar onset with dysarthria and/or dysphagia, tongue wasting, fasciculations, and no peripheral spinal cord involvement for the first 6 months after symptoms onset [3]. The median survival time in bulbar phenotype patients is shorter than in other subgroups [3, 4]
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