Motor neurone disease (MND) is a devastating condition. This preliminary study aims to identify relevant personal factors affecting the experience of living with MND from the perspective of persons with MND (pwMND) in an Australian cohort. A prospective cross-sectional survey of pwMND (
Motor neurone disease (MND) (amyotrophic lateral sclerosis) is a relatively rare neurodegenerative disorder of the motor system in adults characterized by the loss of motor neurons in the cortex, brain stem, and spinal cord, manifested by upper and lower motor neuron signs and symptoms affecting bulbar, limb, and respiratory muscles. Death usually occurs three-to-five years after onset from respiratory failure, but some may survive for a decade or more [
With no cure currently available, the challenge in MND is to prolong independence, prevent complications, and optimise quality of life (QoL). This is best met by a multidisciplinary team with a focus on symptomatic, rehabilitative, and palliative care [
The International Classification of Functioning, Disability and Health (ICF) [
This preliminary study aims to identify the personal factors that are relevant in persons with MND from their perspective, in an Australian cohort.
A community-based MND group was recruited through a tertiary MND multidisciplinary clinic that services Victoria, Australia, including metropolitan and rural regions. Selection criteria included diagnosis of MND according to the El Escorial criteria [
All participants (
Interviews commenced with an open-ended self-report questionnaire. Participants were asked, “What are the main problems you face in your everyday life? If possible, can you list and prioritize up to 10 issues that you feel are the most pressing problems you face in everyday life?” Participants were asked to include intrinsic factors that impacted on their experience of these problems, such as their ability to cope. Some who had difficulties with verbal communication chose to write their responses and/or use communication devices.
From the participant responses (from the open-ended questionnaire), all problems relating to personal factors (currently not coded within the ICF) were grouped under “personal factors” and categorized thematically under major headings, which included demographic factors (gender, race, age, and educational status), emotional states (depression, stress, anxiety, and fear), coping strategies and styles (problem-based coping and denial), personality, beliefs (includes self-efficacy, religious beliefs and values, personal and cultural), attitudes (of the patient), and “other” (perceived social support). Reports unrelated to personal factors have not been included in this paper as they are not the primary focus.
Self-administered (patient) questionnaires followed the open-ended questionnaire: sociodemographic and medical status questionnaire, amyotrophic lateral sclerosis functional rating scale (ALSFRS) [ depression, anxiety, and stress scale (DASS) [ brief COPE [
Assistance was provided where necessary by the interviewer and all patient responses were clarified where possible with their caregivers.
Results were described by mean and standard deviation (SD) for continuous nonskewed data and as frequency (%) for categorical data. Each identified personal factor was listed once regardless of the frequency of identification either by a single or multiple participant(s) for simplicity.
Mean age of participants was 61 years (SD 9.8), and male: female ratio was 3 : 2. Mean time since diagnosis was 3.6 years. Half (
Characteristics of motor neurone disease/amyotrophic lateral sclerosis (MND/ALS) participants—demographics and disease features.
Variable | Average/Frequency |
---|---|
Age (mean ± SD (range) | |
Sex [ | |
Male | 29 (65.9) |
Female | 15 (34.1) |
Marital status ( | |
Married/partner | 34 (77.3) |
Divorced/separated/single | 10 (22.7) |
Race ( | |
Caucasian | 44 (100%) |
Living [ | |
Alone | 8 (18.2) |
Family | 36 (82.8) |
Geographical area | |
Metropolitan [ | 27(61.4) |
Rural [ | 17 (38.6) |
Diagnosis of ALS (El Escorial criteria) [ | |
Clinically definite ALS | 14 (31.8) |
Clinical probable ALS | 17 (38.6) |
Probable ALS (Lab Supported) | 5 (11.3) |
Possible ALS | 4 (9.1) |
Suspected ALS | 4 (9.1) |
Amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS—R [ | |
0–12 | 3 (6.8) |
13–24 | 15 (34.1) |
25–36 | 12 (27.3) |
37–48 | 14 (31.8) |
Comorbidities ( | |
Yes | 28 (63.6) |
None | 16 (36.4) |
≥2 comorbidities | 14 (50.0) |
Clinical symptoms ( | |
Fatigue | 34 (77.3) |
Pain | 22 (50.0) |
Spasticity/cramps/spasms | 32 (72.7) |
Emotional lability | 21 (47.7) |
Shortness of breath | 8 (18) |
Personal factors reported by participants with motor neurone disease (MND) which affected their experience of living with MND.
Personal factors category | Personal factors examples |
---|---|
Demographic factors | Gender |
Race | |
Age | |
Educational status | |
Socioeconomic status | |
Emotional states | Frustration |
Depression | |
Stress | |
Anxiety | |
Fear | |
Worry | |
Degrading | |
Grumpy | |
Loss of confidence | |
Anger | |
Self-esteem | |
Embarrassment | |
Hope (both hopeful and hopeless) | |
Guilt | |
Grief | |
Loss | |
Gratitude | |
Coping strategies and styles | Problem solving |
Search for information | |
Planning | |
PositivityAcceptance | |
Humour | |
Religion | |
Using support | |
Denial | |
Avoidance | |
Personality | Stubborn |
Easy-going | |
Beliefs | Religious beliefs |
Self-esteem | |
Attitudes (of the patient) | Grateful attitude (towards family and health professionals) |
Fighting attitude | |
Attitude towards assisted suicide | |
Being organised | |
“Other” | Perceived support |
Results of depression, anxiety, and stress scale (DASS) and brief COPE.
DASS | [ | |
---|---|---|
Normal | 25 (56.8%) | |
Mild | 9 (20.5) | |
Moderate | 4 (9.1%) | |
Severe | 3 (6.8%) | |
Extreme | 3 (6.8%) | |
Normal | 26 (59.1%) | |
Mild | 6 (13.6%) | |
Moderate | 4 (9.1%) | |
Severe | 4 (9.1%) | |
Extreme | 4 (9.1%) | |
Normal | 33 (75.0%) | |
Mild | 6 (13.6%) | |
Moderate | 2 (4.5%) | |
Severe | 3 (6.8%) | |
Extreme | 0 | |
Brief COPE variables | Mean (SD) | Range |
Active coping (2–8) | 6.3 (1.6) | 2–8 |
Planning (2–8) | 6.2 (1.7) | 2–8 |
Positive reframing (2–8) | 6.0 (1.8) | 2–8 |
Acceptance (2–8) | 7.4 (1.1) | 2–8 |
Humour (2–8) | 5.0 (2.4) | 2–8 |
Religion (2–8) | 4.0 (2.3) | 2–8 |
Using emotional support (2–8) | 6.3 (1.5) | 3–8 |
Using instrumental support (2–8) | 5.7 (1.7) | 2–8 |
Self-distraction (2–8) | 5.7 (2.0) | 2–8 |
Denial (2–8) | 2.9 (1.4) | 2–8 |
Venting (2–8) | 3.6 (1.6) | 2–8 |
Substance use (2–8) | 2.7 (1.5) | 2–8 |
Behavioural disengagement (2–8) | 2.8 (1.4) | 2–7 |
Self-blame (2–8) | 2.7 (1.0) | 2–6 |
This is the first study that has identified personal factors that shape a person with MND’s experience of their condition. These factors include demographic factors (gender, race, age, educational status, and socioeconomic status), emotional states (depression, stress, anxiety, and fear), coping strategies and styles (problem-based coping and denial), personality, beliefs (includes self-efficacy, religious beliefs, and personal values), attitudes (of the patient) and “other” (perceived social support). Rates of depression and anxiety were high and a broad range of coping strategies were used although problem-focused coping strategies were preferred. The mean participant age, gender, time since diagnosis, distribution of type, and severity of disease (based on ALSFRS-R) were similar to those reported by others [
These findings (of depression, anxiety, and a range of coping strategies) and the identified personal factors are consistent with other reports in MND literature. Rates of depression and anxiety are reported to be 0–44% and 0–30%, respectively, in persons with MND [
Personality appears to be another personal factor that plays a significant role in the experience of MND. It has been found that MND patients (
Under the UK Department of Health’s National Service Framework for Long-term Neurological Conditions [
MND takes its toll on the patient and family especially as the disease progresses and loss of independence occurs. Understanding the personal factors involved helps with the palliative rehabilitation process. As part of this process, advice with regards to adequate coping strategies and provision of an adequate amount of disease- and support-related information at any one time and encouraging patients to seek social support [
The limitations in this study include cross-sectional survey (no longitudinal information), highly selective cohort (all already receiving tertiary multidisciplinary care, and willing research participants). Interviews were challenging given the fragile emotional and physical status of the participants. Self-reported information was used and validated as best with caregiver and medical records. The cohort covers a wide geographical population in Victoria and is representative of the wider sample of pwMND.
MND is a devastating illness for patients and families. Palliative care and rehabilitation has much to offer this population. For improved consensus of care and communication amongst treating clinicians, the framework of ICF should be explored in this population and further expanded to take into consideration individual personal factors which impact significantly upon the experience of illness and the rehabilitation process. This preliminary study identifies intrinsic factors reported by patients with MND and may be the first step in the development of personal factors within the ICF classification.
The authors would like to thank their participants with MND without whom this study would not have been possible. They would also like to thank Associate Professor Paul Talman for his assistance in participant recruitment.