Individual and health-related quality of life assessment in amyotrophic lateral sclerosis patients and their caregivers
Introduction
Amyotrophic lateral sclerosis (ALS) is a relentless neurodegenerative disorder affecting the voluntary motor system, of unknown aetiology. Its prevalence is about three to five in 100,000 individuals and generally the onset is in the fifth or sixth decade of life. The progressive decline of muscular function results in paralysis, speech and swallowing disabilities, and, ultimately, respiratory failure leading to death within 2 to 5 years [1].
As the disease progresses, ALS patients require assistance with mobility, eating, dressing, and nursing care; assistance is often provided by a family member who assumes the role of primary caregiver [2].
Despite the evidence that caregivers of ALS patients bear a greater physical and emotional burden than caregivers of other chronic diseases, because of the more rapid progression of ALS [2], there are relatively few studies on their health status, quality of life (QoL), and psychological well being.
Jenkinson et al. [3] reported that ALS has substantial adverse effects upon QoL of patients and caregivers [3]. Krivickas et al. [2] suggested that ALS has a negative impact for caregivers only [4], while Trail et al. [5] found no significant difference between ALS patients and caregivers on QoL scores [5]. Krivickas et al. [2] reported that primary caregivers feel either physically or psychologically unwell [2] and Gelinas et al. [6] found that caregivers were heavily burdened and their outside activities were severely limited [6].
Moreover, there have been only few attempts to identify specific clinical or demographic factors associated to QoL within ALS patients and caregivers.
We, then, performed a cross-sectional study aimed to evaluate the QoL and putative associate variables in both ALS patients and caregivers. Further, we sought to investigate concordance within patient–caregiver pairs for ratings of respective QoL.
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Patients and caregivers
Thirty-seven patients, followed at the ALS Clinic of the University of Palermo, participated in the study. At the time of examination, all cases were diagnosed as probable or definite ALS, according to the El-Escorial WFN revised criteria [7]. Demographic and clinical characteristics of the patients are summarized in Table 1. One patient could not be included in the study because of concurrent frontotemporal dementia. No patient had other serious illness other than ALS. At the time of the
SEIQoL-DW
The SEIQoL-DW index score (SIS) was low in both ALS patients and their caregivers, even if caregivers tended to score relatively higher: the median SIS was 45.5 (interquartile range: 27.3–66.6) for the patients, and 55.6 (interquartile range: 44.1–74.1) for the caregivers, and the difference was not statistically significant (p = 0.061) (Table 2).
Median SIS for the healthy spousal couples was 83.9 (interquartile range: 74.8–89.6), and it was higher than the median SIS for the ALS
Discussion
In the past few years, there has been a growing interest in exploring the caregiving experience, and the World Health Organization has defined addressing the needs of family and family caregivers as one of the primary objectives of palliative care [18]. In particular, family caregivers are key figures in ALS care, since they provide the bulk of support to patients and play a central role in clinical decision-making. Notwithstanding, there are only few studies that addressed QoL issues related
Acknowledgements
The authors gratefully acknowledge the ALS patients and their caregivers who made this study possible.
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