Coping with amyotrophic lateral sclerosis: an integrative view.

Abstract

To identify predictors of psychosocial adjustment to motor neurone disease.
A total of 27 individuals with a confirmed diagnosis of amyotrophic lateral sclerosis (ALS) participated in the study. The ALS functional rating scale mean score indicated a high physical impairment of the sample. Months since diagnosis varied between 4 and 129 (median 36). Adjustment outcomes were severity of depressive symptoms and individual quality of life (QoL). Predictors included social support, cognitive appraisal, coping strategies and illness parameters.
Multiple regression analysis revealed that approximately 60% of the variance of depression and QoL were accounted for by social support, coping strategies and cognitive appraisal. The degree of physical impairment did not explain any variance of the adjustment outcomes. The best predictors for the severity of depressive symptoms were perceived social support and appraisal of coping potential (internal locus of control) and for individual QoL perceived social support.
The focus on medical issues in treatment of ALS is not sufficient. A palliative approach to ALS must equally imply advice with regards to adequate coping strategies, provide the adequate amount of disease- and support-related information at any one time, and encourage patients to seek social support. Sufficient medication and psychotherapy has to be provided for those patients who show depressive symptoms or disorder.

Full text

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TITLE PAGE

Coping with amyotrophic lateral sclerosis: an integrative view

Tamara Matuz Dr.1, Niels Birbaumer Prof. Dr. 1,2, Martin Hautzinger Prof. Dr. 3, Andrea
Kübler Prof. Dr. 1,4
1 Institute of Medical Psychology and Behavioral Neurobiology, Eberhard-Karls-University
Tübingen, Germany
2 Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Ospedale San Camillo, Venezia,
Lido, Italy
3 Department of Psychology, Eberhard-Karls-University Tübingen, Germany
4 Department of Psychology I, Biological Psychology, Clinical Psychology, and
Psychotherapy, University of Würzburg, Germany
Correspondence to: Tamara Matuz
Address: Eberhard-Karls-University Tübingen, Institute of Medical Psychology and
Behavioral Neurobiology, Gartenstr. 29, 72074 Tübingen, Germany. Telephone number: +49
(0)7071 2975997; Fax number: +49 (0)7071 295956.
Email address: tamara.matuz@medizin.uni-tuebingen.de
Email address of the co-authors: niels.birbaumer@uni-tuebingen.de; martin.hautzinger@uni-
tuebingen.de; andrea.kuebler@uni-wuerzburg.de
Word count for the paper 3193 and for the abstract 217.
Number of tables: 5; Number of figures: 2; Number of references: 27
Search Terms: 1. psychosocial adjustment; 2. ALS; 3. coping; 4. depression; 5. quality of life
peer-00557441, version 1 - 19 Jan 2011
Author manuscript, published in "Journal of Neurology, Neurosurgery & Psychiatry 81, 8 (2010) 893"
DOI : 10.1136/jnnp.2009.201285

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ABSTRACT
Objectives - To identify predictors of psychosocial adjustment to motoneurone disease.
Methods - A total of 27 individuals, with a confirmed diagnosis of amyotrophic lateral
sclerosis (ALS), participated in the study. The ALS functional rating scale mean score
indicated a high physical impairment of the sample. Months since diagnosis varied between 4
und 129 (Mdn 36). Adjustment outcomes were severity of depressive symptoms and
individual quality of life (QoL). Predictors included: social support, cognitive appraisal,
coping strategies, and illness parameters.
Results - Multiple regression analysis revealed that approximately 60% of the variance of
depression and QoL were accounted for by social support, coping strategies and cognitive
appraisal. The degree of physical impairment did not explain any variance of the adjustment
outcomes. The best predictors for the severity of depressive symptoms were perceived social
support and appraisal of coping potential (internal locus of control) and for individual QoL
perceived social support.
Conclusions - The focus on medical issues in treatment of ALS is not sufficient. A palliative
approach to ALS must equally imply advice with regards to adequate coping strategies,
provide the adequate amount of disease and support related information at any one time, and
encourage patients to seek social support. Sufficient medication and psychotherapy has to be
provided for those patients who show depressive symptoms or disorder.

List of acronyms: ALS = amyotrophic lateral sclerosis; MS = multiple sclerosis; QoL =
quality of life; BDI = Beck’s Depression Inventory; SEIQoL- DW = The Schedule for the
Evaluation of Individual Quality of Life Direct Weighting; BSSS = Berlin Social Support
Scales; MNDCS = Motor Neuron Disease Coping Scale;

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INTRODUCTION

People with fatal diseases such as HIV, cancer, multiple sclerosis (MS) or amyotrophic lateral
sclerosis (ALS) are permanently challenged by disease progression. The impact of symptoms,
in ALS and MS particularly the increasing physical impairment due to motor paralyses, and
the lack of a curative treatment require adaptive mechanisms. In the context of chronic illness
and physical disability the person-environment equilibrium is disrupted and for its restoration
patients have to adjust their internal needs to the new external demands. This process has been
referred to as the psychosocial adjustment to chronic illness and disabilities [1].
There is a general agreement that despite the continuously increasing physical impairment
ALS patients adjust effectively to their illness and are often perceived as particularly positive
people. These observations are supported by a number of studies investigating different
adjustment variables, e.g. quality of life, depression or anxiety, social support (for an
elaborated review see McLeod & Clarke, 2007 [2]) as well as by few studies exploring the
neural correlates of emotional processing in ALS [3, 4].
Behavioral and emotional changes in motor neuron disease (MND), when present, have been
shown to be partially in line with the spectrum of symptoms that characterize the behavioral
variant of frontotemporal dementia (bvFTD) (e.g. apathy, self-centredness, blunting of
emotions)[5]. Findings of imaging studies on emotional processing in ALS could, however,
not confirm a causal relationship between the altered emotional responses in ALS (e.g. more
positive valence and decreased excitability to extreme emotional stimuli) and frontal lobe
dysfunction [3].
Although there are several studies investigating different psychosocial aspects of MND/ALS,
to our knowledge, no effort has been made to integrate the different determinants of
psychosocial adjustment into a model of coping with ALS. To have a model of psychosocial
adjustment to ALS would be of value to guide intervention and therapy when patients fail to
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cope with the disease leading to depression, poor quality of life, and the wish for a hastened
death [6]. The current study aimed at providing such a model. As we assumed that living with
ALS constitutes a stressful event in life, we based our approach on a stress-coping model (e.g.
Lazarus and Folkman [7]) adapted to chronic diseases [8, 9]. According to this model,
psychosocial adjustment to illness is determined by a complex interaction between four
variables: illness parameters, cognitive appraisal, coping resources and coping strategies.
Possible outcome measures of psychosocial adjustment are the affective state and quality of
life (QoL) [10].


The objective of the present study was firstly, to examine the utility of Lazarus and Folkman’s
stress-coping model, i.e. how much of the variance in psychosocial adjustment could be
explained by the model in a sample of people with ALS. Secondly, we aimed at determining
to what extend the model could predict successful psychological adjustment to ALS.

METHODS
Participants and procedure
The study was approved by the Ethical Committee of the Medical Faculty, University of
Tübingen. The inclusion criterion for patients was a neurologist’s diagnosis of ALS. Patients
with diagnosed frontotemporal dementia, alcoholism and poor knowledge of the German
language were excluded. Twenty seven ALS patients (mean age ± SD: 55.3 ± 11.1; 35-73; 12
women) were included. Fifteen patients were recruited from the ALS outpatient clinic of the
Department of Neurology, University of Ulm, 6 from the Institute of Medical Psychology and
Behavioural Neurobiology, University of Tübingen and 6 through an appeal for participation
in the study in a biannual magazine published by the German Society for Muscular Diseases.
All patients were visited at their homes by a clinical psychologist (TM), signed informed
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consent and were interviewed. Patients were interviewed alone, except those (N = 2) who
used eye movement for communication and needed occasional communicative support by
caregivers or family members in the case the interviewer could not decode/understand the
message. According to the interviewer, the presence of the third person did not influence the
respondent’s answers. However, an eventual bias can not be totally excluded based on this
assessment.

Measures

Background data. Patients’ demographic data, including age, sex, marital status, level of
education, and living arrangement and communication abilities were obtained with a semi-
structured interview.

Illness parameters. Three illness related variables were assessed: duration of illness (month
since diagnosis), dependence on life sustaining treatment (ventilation and nutrition) and
physical disability (ALS Function Rating Scale [11] with scores range from 0 = complete
paralysis to 40 = normal physical functioning).

Appraisal components. Patients’ primary and secondary appraisals were assessed with four
face-valid items designed to measure motivational relevance, motivational congruence,
problem focused and emotion focused coping potential [12]. Patients rated each item on a 9-
point Likert scale (1 referred to not at all and 9 to extremely). The larger the difference
between the two items of primary appraisal “motivational relevance” and “motivational
congruence” the more patients feel threatened by the disease.

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