Aspectos clínicos dos pacientes com Esclerose Lateral Amiotrófica acompanhados em centro de referência em doenças e cuidados paliativos neuromusculares

Abstract

Introduction: Amyotrophic Lateral Sclerosis (ALS) is the most common motor neuron disease, leading to progressive loss of muscle strength in the limbs and impairment of bulbar and respiratory muscles. No disease-modifying treatments are available, and it has a profound impact on all aspects of the patient's and their family's life. Despite the benefits of care provided by palliative care (PC) professionals, there are still many barriers to its implementation. Objective: Evaluate the impact of integrating the multidisciplinary team of the NDRC (Neuromuscular Disease Reference Center) and the palliative care team on the clinical aspects of patients diagnosed with ALS in a tertiary outpatient setting. Methods: This Master's degree Dissertation includes an article to be submitted for publication in the journal “Palliative Medicine” - ISSN: 0269-2163 This retrospective observational study analyzed data from participants diagnosed with ALS, followed at the Tertiary Referral Center for Neuromuscular Diseases in Brasília between January 1, 2020, and December 31, 2022. We used a longitudinal observational design, and data were collected from electronic health records (EHR) to assess the clinical and ociodemographic characteristics of the participants. The study included consecutive patients who met the AWAJI criteria for probable or definite ALS. Patients were excluded if they had treatment discontinuation, evidence of other causes of motor neuron degeneration, a history of obstructive pulmonary disease or cancer, or a history of head and neck cancer or surgery. Results: The study included 43 ALS patients with a mean age of 59 ± 13 years. The majority of patients were female (51.2%) and had spinal onset ALS (79.1%). Most patients received a diagnosis more than 1 year after the onset of symptoms. The average duration of follow-up was 26 ± 15 months. The mean Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) score at baseline was 34.2 ± 8.7, decreasing to 26.8 ± 8.9 during follow-up. Amyotrophic Lateral Sclerosis Assessment Questionnaire-40 (ALSAQ) assessments indicated that the patients' quality of life also worsened. Despite the recommendation for joint follow-up with the PC team, only 17 (39%) of the patients actually received such care. Patients receiving joint follow-up exhibited lower ALSFRS-R scores and higher ALSAQ-40 scores, indicating greater disease severity. Tracheostomy was performed in 2 patients who were not under PC follow-up, and greater opioid use and the creation of advance directives of will were observed in the PC group. Conclusion: The integration between the palliative care and neuromuscular teams in our hospital led to greater use of opioids and the creation of advance directives, suggesting better symptom management and ensuring the patient's wishes. This study should serve as a foundation for further research and the consolidation of neuromuscular palliative care services and as a basis for future studies.