J Rehab Med 2001; 33: 206–211

ACTIVITIES OF DAILY LIVING AND QUALITY OF LIFE IN PERSONS WITH

MUSCULAR DYSTROPHY
Birgitta NaÈtterlund1,2 and Gerd AhlstroÈm2,3
From the 1Department of Public Health and Caring Sciences, Section of Caring Sciences, Uppsala University, Uppsala,
2
Department of Caring Sciences, University of OÈrebro, and 3Department of Medicine and Caring, University of LinkoÈping,
Sweden

The study concerns 77 adults with muscular dystrophy experiences perceived as important (7, 8). Being af icted with a
(mean age 49 years) in two counties in Sweden. The purpose progressive disease has received little attention in research on
was to investigate activities of daily living, quality of life and quality of life. In order to meet this lack of research, the current
the relationship between these. Data collection was per- study focused on persons with MD and investigated what it
formed with “the Activity of Daily Living Staircase”, “the means in terms of QoL to be af icted with these progressive
Self-report Activity of Daily Living” and the Quality of Life diseases over time with repeated losses of activities of daily
ProŽ le. The results indicated that over half of the subjects living.
were dependent on others, chie y in activities requiring The instruments available for assessing quality of life are
mobility. Muscular dystrophy had mostly negative con- directed towards the person’s current life-situation. A particular
sequences, and nearly half stated that life would have instrument has been developed (9), and in contrast with earlier
offered more without it. Few signiŽ cant diagnosis-related instruments, it takes account of the person’s evaluation over a
(no gender-related) differences emerged regarding activities long period. The present study had the following purposes: to
of daily living and quality of life. Lower quality of life can describe activities of daily living in adults with MD and these
only partly be explained by greater disability (r = 0.30– persons’ experienced QoL, to investigate whether there are any
0.54). Therefore quality of life as a measurement of differences depending on gender or speciŽ c diagnosis, and to
rehabilitation outcomes might be based both on physical investigate whether ability to perform activities of daily living is
status, disability and psychosocial factors in terms of a predictor for QoL.
positive and negative consequences.
Key words: muscular dystrophy, quality of life, activities of
daily living, disability, chronic disease. METHODS
J Rehab Med 2001; 33: 206–211 The study is a questionnaire inquiry and constitutes part of a research
programme on MD (3). The research project has been approved by the
Correspondence address: Birgitta Nätterlund, Department research ethics committees of Örebro Medical Centre Hospital and
Linköping University Hospital.
of Caring Sciences, University of Örebro, SE-701 82
Örebro, Sweden. E-mail: [email protected]
Subjects
(Accepted January 15, 2001) The subjects were 77 adults with MD, the average age was 49 (range 24–
74) years and 61% of them were women. Thirty-eight were living in the
county of Örebro (Group A), the other 39 in the adjacent county of
Östergötland (Group B), Sweden (Table I). The subjects were divided
INTRODUCTION into three groups in accordance with type of MD (categorized in
accordance with clinical course): (1) myotonic dystrophy (MyD) with in
Few studies have focused on the consequences of muscular the Ž rst place distal muscular weakness and with a certain degree of
dystrophy (MD) in adults. MD is a term designating different general muscular weakness, (2) myopathia distalis tarda hereditaria
types of hereditary, primary and incurable muscular diseases (MDTH) which almost exclusively af icts distal muscle-groups, and (3)
Proximal MD, covering different diagnoses involving in the Ž rst place
characterized by progressive muscular weakness, often with a proximal muscle groups (Fascioscapulohumeral MD, Becker MD, Limb-
slow progress. The particular diseases involve different clinical girdle MD, Proximal MD without a deŽ nite diagnosis) (1, 10).
courses depending on whether they primarily affect proximal or Group A were recruited from the neurology clinic at Örebro Medical
Centre Hospital. All 38 persons were willing to answer the ques-
distal muscle groups (1, 2). The muscular weakness impedes tionnaires and to participate in a rehabilitation programme during the
mobility, often involving loss of the ability to walk and other period of the study (11).
restrictions concerning daily life and leisure activities. The Group B, who were in the same age-range and had similar diagnoses,
were selected from the Ž le of patients at the Department of Neurology,
decline in mobility causes the person to become dependent on Linköping University Hospital, in the county of Östergötland. All of the
the help and support of others and on technical aids (3–5). 40 selected were willing to participate, but one became seriously ill,
During the past two decades, there has been a change from a leaving 39 subjects for the study.
strict focus on physical health towards quality of life (QoL) as a
Instruments
measure of rehabilitation outcomes (6). In clinical investigation
Group A answered the ADL Staircase and the Quality of Life ProŽ le by
and patient care, QoL is a re ection of the way that patients way of structured interview questions at hospital and Group B at home.
perceive and react to their health status and to other subjective The Self-report ADL was administered as a mail questionnaire.

Ó 2001 Taylor & Francis. ISSN 1650–1977 J Rehab Med 33

 Activities of daily living and quality of life in muscular dystrophy 207

Table I. Distribution of the study group in two counties and by diagnosis, gender, age, duration of disease

Total group Group A Group B MyD MDTH Proximal MD

(n = 77) (n = 38) (n = 39) (n = 34) (n = 12) (n = 31)

Men/women n (%) 30/47 (39/61) 16/22 (42/58) 14/25 (36/64) 11/23 (32/68) 2/10 (17/83) 17/14 (55/45)
Mean age (range) 49 (24–74) 51 (25–71) 47 (24–74) 45 (24–62) 64 (50–71) 48 (25–74)
Disease duration, mean years (range) 23 (4–69) 23 (7–46) 23 (4–69) 22 (8–47) 25 (10–41) 24 (4–69)

MyD = myotonic dystrophy, MDTH = myopathia distalis tarda hereditaria, Proximal MD = proximal muscular dystrophy.
Group A = County of Örebro, Group B = County of Östergötland.

Disability The items are based on 120 interviews with adults with MD (3).
The ADL Staircase. This instrument assesses independence/depen- Furthermore, items included under Problems are based on the pattern of
dence in 10 activities of daily living. It is based on the Katz ADL Index, answers in a number of studies of MD where the Sickness Impact ProŽ le
and has six items concerning personal care (P-ADL): bathing, dressing, (16) has been applied over a period of 5 years (17). The instrument was
toileting, transfer, continence and feeding (12). The ADL Staircase tested in a study about persons with post-polio syndrome, and has been
comprises four complementary instrumental ADL (I-ADL): cleaning, found to have an acceptable discriminatory validity (9).
shopping, transportation and cooking (13, 14). ADL performance is
ranked according to a manual in cumulative ADL grades (14): Data processing
Independent, grade 0; Dependent in I-ADL, grades 1–4; Dependent in In addition to the use of descriptive statistics, gender and group-related
I- and P-ADL, grades 5–10. In the case of persons to whom none of these differences have been analysed by means of unpaired t-test (the Self-
grades applies, there is employed the category Other, signifying report ADL and the indices of the Quality of Life ProŽ le) and the Mann-
dependence in two or more activities but not classiŽ ed as above. A Whitney U test (the ADL Staircase). In order to compare the three
prerequisite for a reliable result is that this category does not exceed 5%. diagnosis groups, Analyses of Variance (ANOVA) (the Self-report ADL
The instrument has been found to have good reliability and validity in and the indices of Quality of Life ProŽ le) and the Kruskal-Wallis test
respect of several patient-groups (12–14). (the ADL Staircase) were used. Correlations have been studied by means
The Self-report ADL. This instrument has been developed inductively of Spearman’s correlation coefŽ cient for ranked data (rho) (nominal/
from interviews with persons with MD (3, 4). It comprises 29 items ordinal data) and the Pearson product–moment correlation coefŽ cient (r)
concerning difŽ culties regarding activities of daily living. In the revised (interval data).
version the subject marks one of the following answers: “No difŽ culty”
or “Can do it but don’t” (0.0), “ Sometimes difŽ culty ” (0.33), “Always
difŽ culty” (0.66) and “Fails” (1.0). The results are given in the form of a RESULTS
percentage of the maximum number of points with regard to each of the
following indices: Ambulation, Arm strength, Finger strength, Finger Comparisons between the two county groups showed few
subtle function and Total index. The higher the percentage, the greater
the difŽ culty. The instrument has been tested and has been found to be differences. For this reason the groups have been merged
valid (3, 4 9). (n = 77) for the presentation of the results. The signiŽ cant
differences found between the groups and also for gender are
Quality of life presented in the text.
The Quality of Life ProŽ le. The instrument has been developed The ADL Staircase. The results indicated that 52% (40/77) of
inductively (3), is designed for persons with progressive long-term the subjects were dependent in I-ADL (grades 1–4), and 18%
illness, and can be regarded as a health-related quality of life instrument.
The self-assessment questionnaire has 44 items, grouped as follows: (14/77) in both I-ADL and P-ADL (grades 5–10). Concerning
Life-picture (4 items), Life-areas (19 items), Problems (15 items) and the three diagnosis groups, the MyD group were signiŽ cantly
Acceptance (6 items). Life-picture involves a global assessment of the more dependent than the MDTH group (z = 2.34, p < 0.05). The
consequences of the disease. The respondent ticks one of the four items,
which best Ž ts his or her situation. Life-areas offers four alternative degree of dependency on others differed signiŽ cantly (z = 2.71,
answers: “in a positive direction”, “in a negative direction”, “don’t know p < 0.01) between Group B (85% or 33/39) and Group A (58%
in what direction” and “has not been important”. The 19 items concern or 22/38). One person was not classiŽ able according the ADL
the following: choice of occupation, educational opportunities, oppor-
tunities of gainful employment, marrying and settling down, relationship Staircase.
with spouse or equivalent, sex life, socializing with friends and The Self-report ADL. This showed that the MDTH group had
neighbours, meeting new people, emotional support from other people, the greatest disability concerning activities requiring distal
choice of dwelling, being or becoming a parent, bearing responsibility
for family and relations, standard of living, being free and independent of muscular strength. The Proximal MD group had the greatest
others, extent of worries and problems, being able to make decisions and disability for Ambulation, while the results for the MyD group
be in control of my everyday life, personal goals and ambitions, leisure re ect both proximal and distal muscular weakness (Table II).
activities, everyday freedom of movement. Problems has items
concerning mobility, fatigue, pain, sleep and leisure. Acceptance has The MyD and MDTH groups had signiŽ cantly more difŽ culties
six items concerning how the person and those closest to the person with regard to Finger strength than the Proximal MD group
accept the restrictions caused by the disease. In the case of both (MyD/Proximal MD t = 3.13, df = 63, p < 0.01; MDTH/Proxi-
Problems and Acceptance the person can tick two or more items if this
corresponds to his or her situation. Life-areas, Problems and Acceptance mal MD t = 2.91, df = 41, p < 0.01). With regard to Finger
have been assigned both a positive and a negative index. The indices subtle function the greatest disability was to be found in the
represent the sum of the responses “in a positive direction” or “in a MDTH group (MDTH/Proximal MD t = 8.03, df = 41,
negative direction”.
The instrument has been developed under the inspiration of p < 0.001; MDTH/MyD t = 2.98, df = 44, p < 0.01), whilst
Nordenfelt’s theory (15) of people’s vital goals and quality of life. there was greater disability in the MyD group than in the

Scand J Rehab Med 33

208 B. Nätterlund and G. Ahlström

Table II. Disability assessed by the Self-report ADL with regard to type of muscular dystrophy

Total group (n = 77) MyD (n = 34) MDTH (n = 12) Proximal MD (n = 31)

Score % Score % Score % Score %
The Self-report ADL Mean (S.D.) Mean (S.D.) Mean (S.D.) Mean (S.D.)

Ambulation 41.9 (30.8) 41.2 (33.6) 29.5 (20.3) 47.5 (30.3)

Arm strength 33.4 (27.2) 30.5 (32.1) 42.9 (20.9) 32.8 (23.0)
Finger strength 40.1 (26.2) 47.8 (28.6) 49.1 (24.3) 28.4 (19.6)
Finger subtle function 18.1 (20.1) 19.7 (22.2) 39.7 (10.6) 7.9 (12.0)
Total index 33.8 (22.8) 34.5 (27.2) 38.6 (15.9) 31.1 (19.8)

MyD = myotonic dystrophy, MDTH = myopathia distalis tarda hereditaria, Proximal MD = proximal muscular dystrophy.

Proximal MD group (MyD/Proximal MD t = 2.63, df = 63, Concerning Acceptance, nearly half gave an afŽ rmative
p < 0.05). In the case of the MyD group this disability can be response to the statements “I’ve entirely accepted the restric-
attributed to the myotonia that af icts the hand-muscles. tions caused by the disease” and “I have to some extent accepted
The Quality of Life ProŽ le. Concerning Life-picture, the the restrictions caused by the disease” (Table III).
statement which drew the largest proportion of afŽ rmative Again, from Table IV it can be seen that the most usual
responses (44%, 31/71) was “Life would have had more to offer consequencesof the disease were negative. There is a signiŽ cant
if the disease hadn’t got in the way, but I don’t go around difference with regard to Problems positive, where a greater
thinking about it”. Regarding the item “Life hasn’t become number of positive consequences were indicated by the MDTH
worse because of the disease, I’ve a good life” the proportion of group than by the other two diagnosis groups (MDTH/MyD
afŽ rmative responses was 35% (25/71). Eleven per cent (8/71) t = 2.8, df = 39, p < 0.01; MDTH/Proximal MD t = 2.7, df = 39,
gave an afŽ rmative response to the statement “Of course I’m p < 0.05). Regarding Problems negative Group B scored
disappointed, since the disease hampers me”, while 10% (7/71) signiŽ cantly higher than Group A (t = 3.37, df = 69, p < 0.01).
gave an afŽ rmative response to the statement “I’ve developed as The results indicate no other signiŽ cant diagnosis- or county-
a person: I probably wouldn’t have had the personal strength I do related differences, and no gender-related ones at all.
have if I’d been in perfect health”. Concerning Life-picture there Correlations between ADL and quality of life. Both the total
were no signiŽ cant differences related to diagnosis, gender or group (n = 77) and the three diagnosis groups separately gave 3
county groups. times as many negative as positive responses. This irrespective
The results regarding Life-areas indicate that the subjects of degree of dependence according to the ADL staircase. There
regarded the disease as having had few positive consequences. was a weak to moderate correlation between on the one hand
The commonest concerned “Emotional support from other Life-areas negative (rho 0.30, p < 0.05) and Problems negative
people” (18% or 13/71) and “Choice of dwelling” (16% or 12/ (rho 0.51, p < 0.001), on the other hand the ADL staircase.
71). The most common negative consequences concerned There was also a correlation between these two indices of the
“Everyday freedom of movement” (67% or 47/70), “Leisure Quality of Life ProŽ le and all four indices of the Self-report
activities” (60% or 42/70), “Opportunities for employment on ADL. The results indicated a moderate correlation between Life-
the labour market” (35% or 25/71), “Extent of worries and areas negative and both Ambulation (r = 0.40, p < 0.01) and
problems in my life” (34% or 24/70) and “Being free and Arm strength (r = 0.42, p < 0.001), and a somewhat weaker one
independent of others” (31% or 22/70). About a quarter of the between Life-areas negative and both Finger strength (r = 0.35,
subjects indicate a negative effect on “Financial standard of p < 0.01) and Finger subtle function (r = 0.33, p < 0.01).
living” (29% or 21/71), “Personal goals and ambitions” (26% or Further, there was a moderate correlation between Problems
18/70) and “Being able to make decisions and be in control of negative and Ambulation (r = 0.52, p < 0.001), Arm strength
my everyday life” (24% or 17/71). The subjects’ uncertainty is (r = 0.54, p < 0.001), Finger strength (r = 0.50, p < 0.001) and
greatest when it comes to “Emotional support from other Finger subtle function (r = 0.53, p < 0.001).
people” and “Personal goals and ambitions” (both 20% or 14/
71).
DISCUSSION
The results regarding Problems conŽ rm the effect of the
disease on mobility. No fewer than 70% (50/71) gave an The results indicated that muscular dystrophy has principally
afŽ rmative response to the statement “I don’t move around as negative consequences for the subjects. Earlier studies have
much as other people, giving priority instead to what I most want shown, similarly, that persons with MD are confronted with
to do or have to do”. Slightly more than half (54% or 38/71) gave many disease-related problems in everyday life, most of these
an afŽ rmative response to the statement “I can’t keep up the problems being caused by muscular weakness (3, 18, 19–22).
pace I used to, but it doesn’t affect my everyday life to any real Increased dependence on others and difŽ culty in performing
extent”. ADL were found in the present study to have no more than a

Scand J Rehab Med 33

 Activities of daily living and quality of life in muscular dystrophy 209

Table III. Descriptive data on the Acceptance of the Quality of Life ProŽ le in the study group

Number of agreement answers

Total group MyD MDTH Proximal MD
(n = 70) (n = 30) (n = 11) (n = 29)
Acceptance n (%) n (%) n (%) n (%)

1. My disease has not involved any restrictions 7 (10) 5 (17) 1 (9) 1 (3)
2. I have not yet accepted the restrictions caused by the 10 (14) 2 (7) 4 (36) 4 (14)
disease
3. I have to some extent accepted the restrictions 29 (41) 9 (30) 6 (55) 14 (48)
caused by the disease
4. I have entirely accepted the restrictions caused by 31 (44) 16 (53) 1 (9) 14 (48)
the disease
5. I accept being restricted by the disease, but my 6 (8) 6 (20) 0 0
closest relatives have not
6. My friends and workmates have not accepted the 6 (8) 3 (10) 1 (9) 2 (7)
fact that I have certain restrictions due to my disease

MyD = myotonic dystrophy, MDTH = myopathia distalis tarda hereditaria, Proximal MD = proximal muscular dystrophy.

weak to moderate correlation with lower quality of life. This samples and methods. Further research is needed to focus on
implies that a decline in quality of life can only partly be positive consequences in order to understand how they
explained by a decline in ADL-ability, a Ž nding that is in line contribute to the overall quality of life associated with disability.
with a recent study of persons with post-polio syndrome (9) and A third of the subjects indicated that their lives had not
with previous research on MD (3, 4). become worse because of the disease. One in 10 felt that they
We have been unable to Ž nd in the literature any quality of life had developed as persons, the trials and tribulations caused by
instrument speciŽ cally designed to assess what it is like to live the disease having given them a personal strength, which they
with a progressive disease. The Quality of Life ProŽ le measures probably would not have had if they had been in good health.
positive consequencesof the disease, and this feature is based on One in four of the persons with post-polio syndrome felt this
what persons with MD have said in the interviews (3). The way (9). These positive experiences may be attributable to the
present study showed comparatively few positive consequences fact that the persons have had the disease for such a long time.
of MD. The commonest concerned emotional support from other There occurs a gradual adaptation to the change in the conditions
people, as was also found in the previous study (9). The MDTH of one’s life (26).
group indicated the largest number of positive consequences, an Several studies have pointed to the subjective experience of
explanation is that these persons have the least disability with having a high quality of life, irrespective of physical state of
regard to mobility and few were dependent on others in ADL. health (7, 27, 28). To some extent subjects may seek to present
Some previous studies have focused on positive consequencesof their life in a positive light in interviews and questionnaires(29).
the disease (23–25). In the studies concerning the mobility- This means that in the interpretation of self-report data it must be
disabled approximately half of groups mentioned such advan- borne in mind that such data can be imbued with a certain over-
tages as challenge, goal or purpose, and the subjects had become estimation of quality of life (27, 29).
more sensitive, tolerant and patient as well as living a less hectic It emerges that persons with MD are more dependent in ADL
life and having more contact with other people (23, 24). The than the average elderly person in Sweden (14). Rather more
explanation of the differences between the results of these than half of our subjects needed help in one or more I-ADL,
studies and those of the present study may be the divergent whilst the proportion in the other study was 22% (14). Again,

Table IV. Indices of the Quality of Life ProŽ le in the study group

Total group MyD MDTH Proximal MD

(n = 71) (n = 30) (n = 11) (n = 30)
Indices (n answers) Mean (S.D.) Mean (S.D.) Mean (S.D.) Mean (S.D.)

Life-areas positive (0–19) 1.2 (2.1) 1.5 (2.4) 0.9 (1.3) 0.9 (2.0)
Life-areas negative (0–19) 4.6 (3.6) 4.4 (3.5) 4.5 (3.2) 4.8 (3.8)
Problems positive (0–2) 0.8 (0.8) 0.7 (0.8) 1.4 (0.8) 0.6 (0.8)
Problems negative (0–13) 3.8 (2.0) 4.2 (2.1) 4.1 (1.5) 3.2 (1.9)
Acceptance positive (0–3) 0.9 (0.5) 1.0 (0.6) 0.7 (0.6) 1.0 (0.4)
Acceptance negative (0–3) 0.3 (0.6) 0.4 (0.7) 0.4 (0.5) 0.2 (0.5)

MyD = myotonic dystrophy, MDTH = myopathia distalis tarda hereditaria, Proximal MD = proximal muscular dystrophy.

Scand J Rehab Med 33

210 B. Nätterlund and G. Ahlström

this comparison indicates that MD gives rise to a large number unemployment is cognitive impairment (36–38). Another thing
of difŽ culties in daily life, principally concerning mobility, is that the majority found it difŽ cult to ask fellow-workers for
personal care and transportation (3–5, 17, 30). Group B was help when there was some part of the job which they were
signiŽ cantly more dependent in ADL than Group A, and had a unable to manage (11).
signiŽ cantly greater number of problems experienced as The three instruments used in this study have been tested and
negative. A possible explanation of these differences is that validated in earlier studies. The Quality of Life ProŽ le can be
Group A were identiŽ ed by way of a population study from 5 regarded as a health-related instrument. Except in the case of the
years before (2), whereby this group included rather more index constituting a summary of the numbers of answers, the
persons with a milder MD progress than in the case of Group B answers of the Quality of Life ProŽ le are on a low scale-level.
(who were only consecutive patients). Another possible ex- This limits the possibilities of psychometric testing. Earlier
planation is that Group A had participated in a programme of research has demonstrated an acceptable discriminatory validity
recurrent rehabilitation during the past 18 months (11). Only a (9). Empirical validity is important when it comes to determin-
few persons in Group B had undergone rehabilitation within the ing whether the instrument achieves an acceptable validity (13).
conventional framework during this period. An explanatory Since the results of the present study do not disagree with earlier
factor may be that Group A had technical aids better adapted to research results, we judge the empirical validity to be
their particular needs. satisfactory. The instrument has a comparatively restricted
In the present study, almost half responded afŽ rmatively to range of application in that it presupposes that the persons got
the statement that life would have had more to offer if the the disease at an early age and had lived with it for a long time.
disease had not got in the way, while in the case of the post-polio In future research, there is a need of other studies of appropriate
group the proportion was somewhat lower (9). The persons patients rather than ones based on a normal population, in order
experience difŽ culties attributable to their restricted freedom of to obtain data for comparison. Up to now there has been just one
movement, both in everyday life and with regard to leisure study of this type (9).
activities they formerly engaged in. The results also indicated In conclusion, this study indicated that ADL would not be a
that rather fewer than half of the subjects had arrived at complete good predictor for quality of life. Instead, measurement of
acceptance, and that about the same number had arrived at rehabilitation outcomes in terms of quality of life should take a
partial acceptance. The majority of the subjects have had the multidimensional view of physical status and disability,
disease for more than 20 years, and the results indicated that the psychological status and well-being, social interactions and
temporal aspect could not alone explain acceptance/non- economic status (7). Another implication of this and also of
acceptance. previous results was that rehabilitation for persons with MD
Research has indicated that the progressive course of the should be recurrent, provided by a multidisciplinary team and be
disease involves signiŽ cant deterioration of functional capacity focused on both negative and positive consequences for the
over a 5-year period (30). Similar results have emerged from subjects. Knowledge of patients’ own perspective on their
Californian studies based on a 10-year period (19–22). quality of life is of fundamental importance concerning
Progressive functional impairment involves the loss of one rehabilitation for persons with MD.
important ability after another and demands renewed adaptation
on the part of the person af icted. Knowledge of the greater
vulnerability of people who have experienced repeated losses
ACKNOWLEDGEMENTS
has an important role to play in the professional encounter with
the patient in respect of care and rehabilitation (31–33). Staff The study was supported by grants from the Swedish Foundation for
Health Care Sciences and Allergy Research, Stockholm, and the
need to encounter patients in a spirit of openness and with Department of Caring Sciences, University of Örebro.
sensitivity, bearing in mind what it is like for them to live
with newly occurring functional impairment (11, 31–33). They
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