Progressão clínica e funcional de indivíduos com Esclerose Lateral Amiotrófica durante a pandemia de COVID-19 no Brasil por meio de telemedicina: um estudo longitudinal do tipo série de casos

Abstract

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a neuromuscular disease marked by progressive motor losses and consequent functional decline. In addition to functional weakness, these patients have a greater susceptibility to respiratory infections due to the impairment of respiratory muscles. Because of these factors, these patients require regular evaluation and follow-up by a multidisciplinary team. With the Covid-19 pandemic in Brazil this care routine was interrupted leading to possible clinical and functional losses in these patients. OBJECTIVE: To follow the clinical and functional progression, symptom control and ventilatory adjustments using telemedicine as an assessment tool for patients with Amyotrophic Lateral Sclerosis during the Covid-19 pandemic in Brazil. METHODS: This is a longitudinal case series type study that followed 11 patients diagnosed with ALS from January 2019 to June 2021. In this period, 5 assessments were performed: A1, A2, A3, A4, and A5. The A1 and A2 evaluations were performed between January 2019 to March 2020, prior to the pandemic of COVID-19 in Brazil, while the A3, A4 and A5 evaluations were performed via telemedicine in the period between April 2020 to June 2021. Assessments A1 and A2 were face-to-face and consisted of pulmonary function test, respiratory muscle strength, functionality according to the ALS Functional Rating Scale-Revised (ALSFRS-R / BR) and ALS disease staging according to the King's College criteria. Assessments of A3, A4 and A5 were performed remotely by telemedicine with a clinical assessment form, evaluation of the use of non-invasive ventilation (NIV), in addition to the ALSFRS-R scale and disease staging using King ́s College. To quantify the rate of functional decline between assessments, we calculated the difference between the total ALSFRS-R score between assessments (A1-A2 and A3-A5) and divided by the time (in months) between assessments. We used the value of 0.77 as the cutoff point to characterize the speed of functional decline. RESULTS: Eleven patients were evaluated (8 males), 8 spinal ALS and 3 Bulbar ALS, age 51 (43-55) years, forced vital capacity (FVC): 69.6 (56.5 - 96.7)%pred and maximal nasal inspiratory pressure (SNIP): 64.5 (42.6 - 85.9)%pred. In clinical and functional progression, it was observed that there was a significant reduction (p<0.01) in the total score of the ALSFRS-R scale, where the A4 and A5 assessments differed from the pre-pandemic assessments (A1 and A2). A similar behavior was also observed in the motor domain of the scale (p<0.01). Furthermore, it was evidenced that the present sample has a characteristically slow functional decline, where at the beginning of the study 90.9% of the patients had a slow decline and at the end of the study 81.8% of the patients had this characteristic. There was no difference in the rate of functional decline between the evaluations. Regarding ventilatory support, there was an increase in the prescription of NIV from 54.4% to 83.3% in addition to remote adjustments in the NIV equipment. CONCLUSION: This study demonstrated that it is possible to follow the clinical and functional progression of patients with ALS through telemedicine. It was evidenced that there was a loss in functional capacity in patients with ALS during the pandemic when compared to the previous moment, and that the use of the King's College system and the subjective sleep assessment using telemedicine may be useful for clinical decision making.