TY  - JOUR
A1  - Peseschkian, Tara
A1  - Cordts, Isabell
A1  - Günther, René
A1  - Stolte, Benjamin
A1  - Zeller, Daniel
A1  - Schröter, Carsten
A1  - Weyen, Ute
A1  - Regensburger, Martin
A1  - Wolf, Joachim
A1  - Schneider, Ilka
A1  - Hermann, Andreas
A1  - Metelmann, Moritz
A1  - Kohl, Zacharias
A1  - Linker, Ralf A.
A1  - Koch, Jan Christoph
A1  - Büchner, Boriana
A1  - Weiland, Ulrike
A1  - Schönfelder, Erik
A1  - Heinrich, Felix
A1  - Osmanovic, Alma
A1  - Klopstock, Thomas
A1  - Dorst, Johannes
A1  - Ludolph, Albert C.
A1  - Boentert, Matthias
A1  - Hagenacker, Tim
A1  - Deschauer, Marcus
A1  - Lingor, Paul
A1  - Petri, Susanne
A1  - Schreiber-Katz, Olivia
T1  - A nation-wide, multi-center study on the quality of life of ALS patients in Germany
JF  - Brain Sciences
N2  - 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.
KW  - Amyotrophic Lateral Sclerosis (ALS)
KW  - Amyotrophic Lateral Sclerosis Assessment Questionnaire 5 (ALSAQ-5)
KW  - ALS treatment
KW  - “bulbar-onset” ALS (bALS)
KW  - “limb-onset” ALS (lALS)
KW  - EuroQol Five Dimension Five Level Scale (EQ-5D-5L)
KW  - health-related quality of life (HRQoL)
KW  - quality of life (QoL)
KW  - symptom-specific treatment
KW  - assistive devices
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-234147
SN  - 2076-3425
VL  - 11
IS  - 3
ER  - 
TY  - JOUR
A1  - Lulé, Dorothée
A1  - Kübler, Andrea
A1  - Ludolph, Albert C.
T1  - Ethical principles in patient-centered medical care to support quality of life in amyotrophic lateral sclerosis
JF  - Frontiers in Neurology
N2  - It is one of the primary goals of medical care to secure good quality of life (QoL) while prolonging survival. This is a major challenge in severe medical conditions with a prognosis such as amyotrophic lateral sclerosis (ALS). Further, the definition of QoL and the question whether survival in this severe condition is compatible with a good QoL is a matter of subjective and culture-specific debate. Some people without neurodegenerative conditions believe that physical decline is incompatible with satisfactory QoL. Current data provide extensive evidence that psychosocial adaptation in ALS is possible, indicated by a satisfactory QoL. Thus, there is no fatalistic link of loss of QoL when physical health declines. There are intrinsic and extrinsic factors that have been shown to successfully facilitate and secure QoL in ALS which will be reviewed in the following article following the four ethical principles (1) Beneficence, (2) Non-maleficence, (3) Autonomy and (4) Justice, which are regarded as key elements of patient centered medical care according to Beauchamp and Childress. This is a JPND-funded work to summarize findings of the project NEEDSinALS (www.NEEDSinALS.com) which highlights subjective perspectives and preferences in medical decision making in ALS.
KW  - ethics
KW  - quality of life (QoL)
KW  - care
KW  - amyotrophic lateral sclerosis (ALS)
KW  - well-being
KW  - depression
KW  - coping
KW  - psychosocial adaptation
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-196239
SN  - 1664-2295
VL  - 10
ER  -