Beruflich Dokumente
Kultur Dokumente
Walton Centre for Neurology and Neurosurgery, Lower Lane, Fazakerley, Liverpool L9 7 LJ, UK
b
Department of Psychology, University of Liverpool, Liverpool, UK
Abstract
Motor neurone disease (MND) is a progressive, unremitting and fatal disease. Respiratory dysfunction is common and a significant
cause of morbidity. The relationship between subjective dyspnoea and objective measures of lung function have been unexplored in
MND. Increasing interest in the specific treatment of respiratory symptoms in MND has highlighted the need for simple, reliable and valid
measures to quantify the degree of dyspnoea in this condition. Several generic questionnaires have been developed to rate subjective
breathlessness but are inappropriate for use in MND patients as they often assess dyspnoea by exercise-limitation. As yet, there are no
published disease-specific measures to assess dyspnoea in MND. In order to accurately and reproducibly measure the subjective
experience of dyspnoea in this patient group, we have developed and validated a novel patient-specific dyspnoea questionnaire, the MND
dyspnoea rating scale (MDRS). It comprises three domains covering dyspnoea, emotion and mastery and is valid for use in MND patients
at all stages of disease progression. In our cohort of 40 unselected patients with MND we have shown that the patients subjective
experience of dyspnoea is closely related to emotion and psychological control over the disease. Dyspnoea is not related to objective
measures of lung function such as vital capacity, irrespective of limb or bulbar presentation. In conclusion, vital capacity, although useful
prognostically, is only one aspect of respiratory function in MND. The MDRS is a reliable and valid tool to rate subjective dyspnoea in
MND. 2000 Elsevier Science B.V. All rights reserved.
Keywords: Motor neurone disease; Amyotrophic lateral sclerosis; Dyspnoea; Questionnaire; Vital capacity
1. Introduction
Dyspnoea, the unpleasant sensation of breathlessness, is
a distressing symptom which may adversely affect quality
of life of people with motor neurone disease (MND). The
mechanism by which the individual experiences breathlessness is complex with integration of both physical and
psychological components. Despite the frequent involvement of the respiratory system in MND [1,2] the
relationship between dyspnoea, physiological measures
and functional state remain unexplored.
Dyspnoea is significant in contributing to poor quality of
life in other disease states and several questionnaires have
*Corresponding author.
E-mail address: young-c@wcnn.co.uk (C.A. Young).
0022-510X / 00 / $ see front matter 2000 Elsevier Science B.V. All rights reserved.
PII: S0022-510X( 00 )00415-9
2. Methods
87
88
3. Results
Number of
patients
Median
Range
Age (years)
40
60
3480
Gender
Male
Female
25
15
Type of onset
Limb
Bulbar
28
12
Bulbar symptoms
at time of study
Duration (months)
Percent predicted vital
capacity
ALSFRS
Table 2
Rotated factor loadings
Item number a
Factor 1
emotion
Factor 2
dyspnoea
Factor 3
mastery
14, satisfied
11, relaxed
13, depressed
9, upset, worried
6, frustration
8, confident
16, tense
12, fatigue
2, dyspnoea
3, dyspnoea
4, dyspnoea
1, dyspnoea
5, dyspnoea
15, fear
7, panic
10, control
0.89
0.78
0.77
0.72
0.67
0.60
0.58
0.45
0.24
0.00
0.26
0.15
0.25
0.27
0.31
0.33
0.00
0.19
0.32
0.24
0.35
0.22
0.34
0.41
0.81
0.67
0.67
0.66
0.60
0.43
0.37
0.27
0.22
0.41
0.39
0.22
0.21
0.52
0.44
20.21
0.18
0.30
0.23
0.23
0.34
0.80
0.77
0.67
a
The word next to the item number is a brief cue. The full question
corresponding to each item number is given in full in Appendix A. Bold
type indicates the strongest factor loadings for each domain.
24
40
40
34
57
799
496
40
24
537
89
Fig. 1. (a) Testretest reliability: difference in MDRS scores over 7 days plotted against their average. (b) Reliability: difference between MDRS dyspnoea
domain scores plotted against their average.
90
4. Discussion
This study shows that dyspnoea is an important and
common symptom in MND patients regardless of whether
they have bulbar involvement or not. We have developed a
novel disease-specific questionnaire which appears to be a
valid, reliable measure to quantify dyspnoea in MND. The
perception of breathlessness in MND is not related to
disease duration, level of disability or vital capacity. This
study also demonstrates that mastery of breathlessness and
anxiety are important psychological components to the
patients perception of dyspnoea.
Previous measures to quantify breathlessness have been
developed for use in lung and cardiac disease [35] but
these often rate breathlessness according to exercise
capacity which is clearly not applicable in many MND
patients. Guyatt et al. [3] developed the chronic respiratory
disease questionnaire to assess the impact of dyspnoea on
the quality of life. The dimensions of this questionnaire
were dyspnoea, fatigue, emotion and mastery. It appears
that fatigue is not so important to MND patients as patients
with chronic lung disease. This may be because MND
patients are more disabled physically and are likely to have
reduced levels of activity compared to patients with pure
lung disease.
The Borg [4] and visual analogue scales [8,9] are global
rating scales and only give limited information about the
factors contributing to breathlessness. In contrast, the
MDRS dyspnoea domain is patient-specific and allows the
patient to quantify how dyspnoea affects their daily life.
Acknowledgements
We are indebted to Mary O. Brien and Professor D.
Mitchell, Royal Preston Hospital and Dr. Schady, Manchester Royal Infirmary for helping with data collection.
Sister Dott Marshall and Dave Watling, Walton Centre for
Neurology and Neurosurgery who assisted with clinics and
vital capacity measurement.
Appendix A
A.1. The MND dyspnoea rating scale ( MDRS)
We would be grateful if you could take time to complete
this questionnaire. Please attempt to answer all of the
questions. We are interested in your answers even if you do
not have a problem with breathlessness.
If you have any problems with completing the questionnaire we can help you complete it at the clinic visit.
(A) I would like you to think of the activities that you
have done during the last 2 weeks that may have made you
feel short of breath. These should be activities which you
do frequently and which are important in your day to day
life. Please list as many activities as you can that you have
done during the last 2 weeks that have made you feel short
of breath.
(1) Activity 1: . . .
Extremely
short
of breath
Very short
of
breath
Moderate
shortness
of breath
2
Slight shortness
of breath
1
Not at all
short
of breath
0
(2) Activity 2: . . .
Extremely
short
of breath
1.
2.
3.
4.
5.
91
Very short
of
breath
Moderate
shortness
of breath
2
Slight shortness
of breath
1
Not at all
short
of breath
0
(3) Activity 3: . . .
Talking
Eating
Dressing
Preparing meals
Carrying such as carrying groceries
Playing with children / grandchildren
Walking
Moving around your own home (aided or unaided)
Having a bath or shower (aided or unaided)
Bending
Being angry or upset
Lying flat
While trying to sleep
Extremely
short
of breath
Very short
of
breath
Moderate
shortness
of breath
2
Slight shortness
of breath
1
Not at all
short
of breath
0
(4) Activity 4: . . .
Extremely
short
of breath
Very short
of
breath
Moderate
shortness
of breath
2
Slight shortness
of breath
1
Not at all
short
of breath
0
(5) Activity 5: . . .
Extremely
short
of breath
4
Very short
of
breath
3
Moderate
shortness
of breath
2
Slight shortness
of breath
1
Not at all
short
of breath
0
(6) In general, how much of the time during the last 2 weeks have you
felt frustrated or impatient?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(7) How often during the past 2 weeks did you have a feeling of fear or
panic when you had difficulty getting your breath?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(8) In the last 2 weeks, how much of the time did you feel very confident
and sure that you could deal with your illness?
All of the
time
The next section uses the five activities which you have
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
92
(9) In general, how much of the time did you feel upset, worried, or
depressed during the last 2 weeks?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(10) How often during the last 2 weeks did you feel you had complete
control of your breathing problems with shortness of breath and tiredness?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(11) How much of the time during the last 2 weeks did you feel relaxed
and free of tension?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(12) How often during the last 2 weeks have you felt low in energy?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(13) In general, how often during the last 2 weeks have you felt
discouraged or down in the dumps?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(14) How happy, satisfied, or pleased have you been with your personal
life during the last 2 weeks?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(15) How often during the last 2 weeks did you feel upset or scared when
you had difficulty getting your breath?
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
(16) In general, how often during the last 2 weeks have you felt restless,
tense or uptight:
All of the
time
4
Most of the
time
Some of the
time
Hardly any
of the
time
None of the
time
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