3011OL Palliative Care Case Overview

Describe the pathophysiology of Motor Neurone disease and identify if this disease could be classified as a ‘life-limiting illnesses.

Motor Neurone disease is a condition that affects the brain and the nerves leading to weakness that exacerbates over time (NHS, 2023). Where the pathology of Amyotrophic Lateral Sclerosis (ALS), is the degeneration of anterior horn cells in the spinal cord which occurs due to retrograde axonal loss, and of the pyramidal Betz cells in the motor cortex, and the lower cranial motor nuclei of the brainstem. The lost neurons are replaced by Gliosis which is a fibrous proliferation of glial cells, found in the injured areas of the CNS. This can be seen in affected muscles and spinal cord atrophies which eventually leads to paralysis. The pathophysiology of ALS can be a combination of genetic, environmental, and biochemical factors, where the most prevalent form is sporadic which accounts for 90-95% of the cases while the rest of the cases are familial forms. There are several intracellular inclusions in degenerating neurons. The pathophysiology can be due to the modification of gene expression, any trigger factor or propagating channels such as protein misfolding, neuroinflammation, mitochondrial dysfunction, oxidative stress etc. (Brotman et al., 2024). Due to excessive glutamate levels, the motor neurons are vulnerable to excitotoxicity that leads to cell death. For every individual the progression of the disease is different, reducing the quality of life and leading to respiratory failure.

Thus, ALS can be characterized as a life-limiting illness, due to its progressive nature, where the quality of life significantly decreases as the disease advances and the lack of research leading to its cure. The prognosis for ALS is detrimental as neurons are unable to send signals to the brain, and the brain loses its ability to initiate and control voluntary movements such as standing or even breathing thus most of the patients can only live 3 to 5 years after the diagnosis (NIH, 2024).

Identify which illness trajectory is the best model for this illness and justify your choice.

The illness trajectory is the course of disease progression and its treatment, which includes various phases and directions it may take which include remission or end-of-life care. The suitable illness trajectory model for this case will be the “chronic illness trajectory” model which follows consecutive phases of pre-trajectory, trajectory onset, stable, unstable, acute, crisis, comeback, downward, and dying. As for the unstable and acute conditions, symptoms of the disease become more prevalent or are unrelieved, and are less under control than in the stable phase (Ben-Assuli and Padman, 2020). The ALS is characterized by gradual degradation of the patient combined with acute exacerbations followed by some period of stability. This model is thus appropriate as it elaborates on the nature of the disease where the patient experiences a gradual decline in the condition rather than a rapid one (Vandenbogaerde et al., 2024).

Discuss the impact of this disease on Sharon including current symptoms and losses she will face as the illness progresses.

Since Sharon is the patient of this disease, ALS will impact her profoundly bringing her many physical and emotional challenges. The existing symptoms of speech distortion, difficulties while swallowing and breathing difficulties have made it difficult for her to communicate and perform her day-to-day tasks which has made her lose her sense of autonomy and independence. As the progression of the disease happens Sharon is likely to face further losses and will ultimately depend on others for mobility and personal care. She is most likely to lose her ability to communicate, and even breathe. Accepting this painful truth of losing her identity as a teacher, she will face professional loss and social connections he made as a teacher. She will not be able to a parent or spouse will cause grief, helplessness, frustration and depression. She will probably not be able to witness or process Tom’s graduation or the birth of her grandchild. Everything will bring a huge psychological toll due to facing terminal illness, adapting to continuous losses and affecting family dynamics.