Symptomatic pharmacotherapy in ALS: data analysis from a platform-based medication management programme

Meyer, Thomas; Kettemann, Dagmar; Maier, André; Grehl, Torsten; Weyen, Ute; Großkreutz, Julian; Steinbach, Robert; Nordén, Jenny; George, Annette; Hermann, Andreas; Guenther, René; Petri, Susanne; Schreiber‐Katz, Olivia; Dorst, Johannes; Ludolph, Albert C.; Walter, Bertram; Münch, Christoph; Spittel, Susanne

doi:10.1136/jnnp-2020-322938

Cited by 20 publications

(19 citation statements)

References 7 publications

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“…In addition, we observed that patients who did not use antidepressants had a significantly better QoL, whereas it is known that up to 50% of ALS patients are symptomatically treated with antidepressants [ 81 ]. Therefore, our results support the above-mentioned association between depression and worse QoL in ALS.…”

Section: Discussionmentioning

confidence: 99%

A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany

et al. 2021

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Improving quality of life (QoL) is central to amyotrophic lateral sclerosis (ALS) treatment. This Germany-wide, multicenter cross-sectional study analyses the impact of different symptom-specific treatments and ALS variants on QoL. Health-related QoL (HRQoL) in 325 ALS patients was assessed using the Amyotrophic Lateral Sclerosis Assessment Questionnaire 5 (ALSAQ-5) and EuroQol Five Dimension Five Level Scale (EQ-5D-5L), together with disease severity (captured by the revised ALS Functional Rating Scale (ALSFRS-R)) and the current care and therapies used by our cohort. At inclusion, the mean ALSAQ-5 total score was 56.93 (max. 100, best = 0) with a better QoL associated with a less severe disease status (β = −1.96 per increase of one point in the ALSFRS-R score, p < 0.001). “Limb onset” ALS (lALS) was associated with a better QoL than “bulbar onset” ALS (bALS) (mean ALSAQ-5 total score 55.46 versus 60.99, p = 0.040). Moreover, with the ALSFRS-R as a covariate, using a mobility aid (β = −7.60, p = 0.001), being tracheostomized (β = −14.80, p = 0.004) and using non-invasive ventilation (β = –5.71, p = 0.030) were associated with an improved QoL, compared to those at the same disease stage who did not use these aids. In contrast, antidepressant intake (β = 5.95, p = 0.007), and increasing age (β = 0.18, p = 0.023) were predictors of worse QoL. Our results showed that the ALSAQ-5 was better-suited for ALS patients than the EQ-5D-5L. Further, the early and symptom-specific clinical management and supply of assistive devices can significantly improve the individual HRQoL of ALS patients. Appropriate QoL questionnaires are needed to monitor the impact of treatment to provide the best possible and individualized care.

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Section: Discussionmentioning

confidence: 99%

A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany

et al. 2021

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“…Subjects meeting the following inclusion criteria were included in the cohort study: (i) genetically confirmed diagnosis of 5q-SMA, (ii) current or planned nusinersen treatment, (iii) age 18 years or older and (iv) informed consent for participation in the research platform APST [15][16][17][18].…”

Section: Participantsmentioning

confidence: 99%

Treatment expectations and perception of therapy in adult patients with spinal muscular atrophy receiving nusinersen

Meyer

Maier

Uzelac

et al. 2021

Euro J of Neurology

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“…Despite continuous efforts to develop curative therapies, the only globally approved therapy, Riluzole, was discovered over two decades ago [ 5 , 6 ]. Patient care entails extensive symptomatic and supportive therapies throughout the disease course and requires constant patient counselling and allied health care [ 7 , 8 , 9 ]. In keeping with this, an increased number of tertiary centers provide individualized multidisciplinary care for patients with ALS [ 7 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

Triage of Amyotrophic Lateral Sclerosis Patients during the COVID-19 Pandemic: An Application of the D50 Model

Steinbach

Prell

Gaur

et al. 2020

JCM

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Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease, the management of which requires the continuous provision of multidisciplinary therapies. Owing to the novel coronavirus disease (COVID-19) pandemic, regular contact with ALS patients at our center was severely restricted and patient care was at risk by delay of supportive therapies. We established a triage system based on the D50 disease progression model and were thus able to identify a prospective cohort with high disease aggressiveness (D50 < 30). Thirty-seven patients with highly aggressive disease were actively offered follow-up, either via telephone or on-site, depending on their disease-specific needs and abilities. We describe here the procedures, obstacles, and results of these prescient efforts during the restrictions caused by COVID-19 in the period between March and June 2020. In conclusion, four patients with highly aggressive disease were initiated with non-invasive ventilation and two received a gastrostomy. We could show that a comparable amount of advanced care was induced in a retrospective cohort within a similar time period one year prior to the COVID-19 outbreak. Our workflow to identify high-risk patients via D50 model metrics can be easily implemented and integrated within existing centers. It helped to maintain a high quality of advanced care planning for our ALS patients.

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Symptomatic pharmacotherapy in ALS: data analysis from a platform-based medication management programme

Cited by 20 publications

References 7 publications

A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany

A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany

Treatment expectations and perception of therapy in adult patients with spinal muscular atrophy receiving nusinersen

Triage of Amyotrophic Lateral Sclerosis Patients during the COVID-19 Pandemic: An Application of the D50 Model

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