ORIGINAL ARTICLE

Activities of Daily Living (ADL) Structure of Patientswith Duchenne Muscular Dystrophy, Including Adults
Toshiyuki Fujiwara, Aya Tanabe, Ken Uchikawa, Tetsusya Tsuji, Akira Tanuma,
Kimitaka Hase and Meigen Liu

Department of Rehabilitation Medicine, School of Medicine, Keio University, Tokyo, Japan(Received for publication on January 16, 2009)(Revised for publication on April 11, 2009)(Accepted for publication on May 21, 2009)

Abstract
Objective : To study the activities of daily living (ADL) structure of patients with Duchenne
muscular dystrophy (DMD).
Design : Cross-sectional survey.
Subjects : Seventy-two patients with DMD (mean [SD] age, 17.2 [8.1] years), recruited in
160-bed regional center for neuromuscular disease.
Methods : We assessed 72 participants with the Functional Independence Measure (FIMSM),
and determined the difficulty order of the FIMSM items with the percentage of patients who
were independent (6 or 7) for each FIM item (% independence).
Results : The ADL of patients with DMD had an order in terms of difficulty. For the motor subscale, the most difficult item was stairs, and the easiest item was locomotion. On the cognitive
subscale, problem solving was the most difficult item. When we compared item difficulty between patients <15 years of age and those ≥ 15 years, the % independence was lower in theolder age group for all motor items. The decrease in the % independence of the eating itemwith age was significant; however bladder management was well maintained.
Conclusions : ADL of patients with DMD has a specific difficulty order. The order of difficulty isbased on the relative level of independence/dependence. The difficulty order was maintained,
although the structure of ADL changed with age. (Keio J Med 58 (4) : 223－226, December 2009)

Keywords: activities of daily living, muscular dystrophy, functional independence measure
Introduction
Duchenne muscular dystrophy (DMD) is an X-linked
recessive dystrophic myopathy caused by complete absence of the protein dystrophin in the skeletal muscle,myocardium, and brain. Patients develop progressive
weakness, contractures, spinal deformity, restrictive lungdisease, cardiomyopathy, and variable degrees of cognitive impairment - all contributing to progressive impairment in the ability to complete activities of daily living(ADL). With advances in respiratory management, life
expectancy of patients with DMD has increased, and themean age at death has increased from 14.4 years in the1960s to 25.3 years in the 1990s.1 Despite this improve-

ment in survival, disease progression is inevitable, leading to a state of severe physical dependence. Therefore,
one of the important goals of patient management is themaintenance of ADL as long as possible. However, only
a few reports investigating ADL in patients with DMD
have used valid and reliable instruments,2, 3 and no study
includes patients aged 15 and older, despite the fact thatmany patients now survive into adulthood. The aim of thisstudy was to use the Functional Independence Measure(FIMSM),4, 5 a validated functional instrument, to assess
disabilities in patients with DMD over a wide age range
and to analyze the percent of independence associatedwith each FIMSM item.3

Reprint requests to: Toshiyuki Fujiwara, MD, PhD, Department of Rehabilitation Medicine, School of Medicine, Keio University, 35 Shinanomachi, Shinjyuku, Tokyo 160-8865, Japan, Tel: +81-3-5363-3833, Fax: +81-3-3353-6209, E-mail: tofuji@xc5.so-net.ne.jp223

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224

Methods
Participants were recruited from male inpatients andoutpatients of a 160-bed regional center of neuromuscular diseases in the Saitama Prefecture in the northern partof the Tokyo Metropolitan area with a population of approximately 7 million. The number of patients withDMD in this area is estimated to be 240 based on the incidence of DMD (6.3/100,000 live births) and the number of males (3.5 million) in the prefecture. The diagnosis of DMD was established with clinical criteria, family
history, dystrophin analysis of muscle biopsy specimens,
and gene analysis by child neurology specialists. We selected patients aged 7 and older. We excluded patientswith acute illness or other conditions that would prevent
us from obtaining informed consent. Female carrier cases were excluded.
The institutional ethical committee approved the study,
and informed consent was obtained from patients or family members before data collection in accordance withthe Declaration of Helsinki.
Three physiatrists trained and experienced in the instrument assessed the FIMSM both by interviewing theparents or nurses and by directly observing the patients’performance. We also calculated the percentage of patients who were independent (scored at 6 or 7) for each
FIM item (% independence), which was used as an indicator of item difficulty in a previous study.3 Furthermore,
we compared item difficulty, indicated as % independence, between patients aged ≥15 years and those <15years.
Results
Seventy-two male patients with DMD were recruitedfor this study. There were 35 inpatients and 37 outpatients (mean [SD] age, 17.2 [8.1] years, range 7 to 40years, median age, 15.5 years). Twenty-nine patientsused electrical powered wheelchairs, 19 patients used
manual wheelchairs, 12 patients used assisted typewheelchairs, and 12 patients were ambulatory. Fifteenpatients used a ventilator part-time and 7 patients used aventilator 24 hours a day; ventilators were not needed forthe remaining 50 patients. The FIM SM motor scores
ranged from 14 to 91, and the mean (SD) and medianscores were 37.3 (18.1) and 33, respectively. The FIMSM
cognitive scores ranged from 11 to 35, and the mean(SD) and median scores were 32.0 (5.0) and 35. The
FIMSM motor score significantly correlated with age(Spearman’s rank correlation method [Rs= -0.66,p<0.001]) (Fig. 1). The mean FIMSM motor score for patients aged <15 years (n=34) and those ≥15 years (n=38)
were 47.4 and 28.3, respectively (p<0.001, Mann-Whitney U test).
The FIMSM motor score was analyzed by the type oflocomotion: ambulatory (n=12), manual wheelchair

Fig. 1 The relationship between FIM motor score and age.
The correlation between the FIM motor score and age wasstatistically significant with Spearman’s rank correlation method(Rs= -0.66, p<0.001).

(n=19), electrical powered wheelchair (n=29), and assisted type wheelchair (n=12). The mean FIMSM motorscore for each type of locomotion was 66.6, 38.7, 31.2,
and 20.5, respectively (p<0.001, Kraskal-Wallis test). Inpatients ≥15 years, the mean FIMSM motor score of ventilator users (n=22) was significantly lower than that ofnon-ventilator users (n=16) (25.2 vs. 32.5; p<0.001,Mann-Whitney U test).
Table 1 shows the % independence of FIM items. The
most difficult item was stairs, and only 5 patients scoredat independent levels of 6 and 7. Easier items included
locomotion (the easiest item), bowel management, andeating. Regarding the locomotion item, 60 of 72 patients(83.3%) used wheelchairs and 41 of those 60 (68.3%)scored 6. As a rule, ADL items related to lower extremity
functions (e.g., transfer items and dressing the lower partof the body) were relatively difficult, whereas items related to upper extremities (e.g., eating, grooming, bathing, and dressing the upper part of the body) were easier.
When comparing the item difficulty between patients<15 years and those ≥15 years, the % of independence
was lower in the older age group for all motor items. The% independence of eating items was 7.8 in ≥15 groupbut 76.4 in <15 group. The decrease in % independence
was biggest in the eating item and smallest in the bladdermanagement item. Chi-square test showed no significantdifference of % independence between the ≥15 years and<15 years groups in bladder management, bowel management, comprehension, and expression items.
Figure 2 illustrates the percentage of patients requiring
assistance (FIM SM score ≦4), supervision (FIM SM
score=5), and those who were independent (FIMSM score≧6) for each FIMSM item.
With regard to cognitive items, the % independence ofproblem solving was the lowest (i.e., it was the most difficult item). The % independence for problem solving
item was low in patients <15 years, but not in those ≥15years. The % independence of memory, social interac-Keio J Med 2009; 58 (4): 223－226

225

Table 1 Item difficulties of the FIM
Items
Motor
Locomotion
Bowel
EatingBladder
Grooming
Bathing
Dressing U
ToiletToilet transfer
Dressing L
Bath transfer
Bed transfer
StairsCognition
Expression
MemorySocialComprehension
Problem% of independence (total)% of independence(<15)% of independence( ≧ 15)

Chi-square

66.6
63.8
40.2
23.6
15.2
12.5
15.2
9.7
12.5
11.1
11.1
9.7
6.9

88.2
76.4
76.4
26.4
26.4
23.5
32.3
20.5
26.4
23.5
23.5
20.5
14.7

65.7
55.2
7.8
21.0
5.2
2.6
00
0
0000

p=0.025p=0.059
p<0.001p=0.589p=0.003
p=0.007p<0.001p=0.003
p=0.001p=0.002
p=0.002
p=0.003
p=0.004

87.5
88.8
90.2
73.6
72.2

91.1
79.4
82.3
73.5
52.9

84.2
97.3
97.3
73.6
89.4

p=0.372
p=0.016
p=0.03p=0.98p=0.001

Bowel; bowel managementl, Bladder; bladder management, Dressing U; dressing upper body, Toilet; toileting,Dressing L; dressing lower body, Social; social interaction, Problem; problem solving.
The difference of % of independence between <15 and ≧15 was statistically analyzed with chi-square test.

Discussion

Fig. 2 Percentages of patients requiring assistance (FunctionalIndependence Measure (FIMSM) score ≦4), supervision (FIMSM
score = 5), and who were independent (FIMSM score ≧6) for
each FIMSM item.

tion, and problem solving items were significantly higher
in those ≥15 years than in those <15 years (chi square
test, p=0.016, 0.03, and 0.001, respectively).

Few studies use validated instruments to examine theADL of persons with DMD. Nair, et al.2 used the Barthel
Index7 to assess ADL in 31 children with DMD, and ourgroup3 measured the FIMSM in 27 children. The FIMSM
is a standardized functional instrument widely used toassess various disabling conditions. This study assessed
FIMSM of patients with DMD including those < 15 years
and ≥15 years, the latter of which have not been included
in previous reports. The FIM motor score was negativelycorrelated with age. The difference in the FIM motorscore between patients <15 years and those ≥15 years
was statistically significant. The relative stability of theFIM motor score in patients ≥15 years indicates that thelevel of assistance required is relatively stable from thelater stages of the second decade on. It appears that someFIM domains, such as locomotion, are relatively insensitive measures of disease severity because of the use ofpower wheelchairs to maintain modified independence.
In terms of item difficulties, Nair, et al.2 analyzed theprevalence of partial or total dependence among Barthel
Index items, and our group3 assessed them by calculating
the percentage of patients requiring assistance. In ourprevious study,3 eating was the easiest item, followed bybowel management and locomotion. In the current study,locomotion was the easiest item, followed by bowel
management and eating. Eating item ranked third from

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226

the easiest. This discrepancy could be explained by thedifference in age distribution, which ranged from 7 to 40
years in the present sample and from 7 to 14 years in ourprevious sample. The progressive decline in muscle
strength that occurs with aging is likely to have renderedmore patients to become dependent in eating, as evidenced by the smaller % independence for eating in thepresent study compared with our previous study (38.8%
vs. 85.1%). This finding was confirmed by performing% independence analysis separately for the two age
groups.
For the bladder management item, the % independence
was 23.6%, whereas the median item score was 5. Thisfinding could be due to the fact that many patients maintained the voiding sensation and intact bladder function,
while at the same time they needed assistance to set or
hold their urinal.
Locomotion was listed as one of the easier items inthis study. This is because many patients maintained
their capability for locomotion –even with advancing
age– with the help of an individually tailored electricalwheelchair equipped with a seating system and a non-invasive ventilator.
Among the three transfer items, toilet transfer was theeasiest and bed transfer was the most difficult. This
could be related to proximal weakness of the lower extremities, because it is necessary for patients to lift theirfeet while performing bed transfers but not while performing toilet transfers.
DMD is a progressive disease in which the independence of ADL decreases with time. Our study showed
the decrease of % independence for each FIM item was
not always correlated with its % independence value. For
example, in patients <15 years, the % independence ofgrooming and toilet transfer items were the same. However, in subjects ≥15 years, the % independence ofgrooming was higher than that of toilet transfer. It is also
possible that the cross-sectional nature of this study andthe small sample size were not enough to establish a taskdifficulty hierarchy in younger and older age groups. Future studies with larger sample sizes and longitudinal
data collection are warranted.
Our study also indicated that there was a difficulty order in the cognitive items of the FIMSM. In this population, an intellectual impairment has been documented,7
and an early non-progressive impairment of verbal intelligence and mental retardation are reported.8 Our study
showed that problem solving was the most difficult item,
for which more than 20% of the patients required someassistance. The % independence of problem solving waslower in patients <15 years than those ≥15 years. It islikely that it is more difficult for younger children to accept their impairment and disability. It is therefore necessary to consider more mental care for these younger patients. Problems related to verbal impairment and attentional complexities are known to occur in DMD.8 Whenmental retardation is present, motor development landmarks could be slowed, and the children are likely tocease ambulation earlier.7 This, in turn, could influenceeducational placement. We therefore recommend paying
attention not only to motor function but also to cognitive
function when helping patients with DMD manage theirADL.
The major goals of rehabilitation for patients withDMD are maintenance of ADL and the development of aprogram for prevention of progression of disability as
well as supportive counseling for patients and families.7
It is therefore necessary to know the level of difficulty
associated with each ADL and how ADL declines withage. The results of this study might be useful in predicting the progression of difficulties in completing ADL for
patients with DMD and to help form a plan to maintainADL as long as possible.
Conclusions
This report demonstrated that ADL of patients with
DMD had a specific order of difficulty. The order of difficulty in this study is slightly different from previousstudies, whose subjects were younger.
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