Archives of Physical Medicine and Rehabilitation journal homepage: www.archives-pmr.org Archives of Physical Medicine and Rehabilitation 2015;96:1242-7
ORIGINAL RESEARCH
Prevalence of Fatigue, Pain, and Affective Disorders in Adults With Duchenne Muscular Dystrophy and Their Associations With Quality of Life Robert F. Pangalila, MD,a,b Geertrudis A. van den Bos, PhD,c Bart Bartels, PT,a,b Michael Bergen, MD, PhD,b Henk J. Stam, MD, PhD,a Marij E. Roebroeck, PhDa,b From the aDepartment of Rehabilitation Medicine and Physical Therapy, Erasmus University Medical Center, Rotterdam; bRijndam Rehabilitation, Rotterdam; and cDepartment of Social Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
Abstract Objectives: To assess the prevalence of fatigue, pain, anxiety, and depression in adults with Duchenne muscular dystrophy (DMD), and to analyze their relationship with health-related quality of life. Design: Cross-sectional study. Setting: Home of participants. Participants: Adults (NZ80) with DMD. Interventions: Not applicable. Main Outcome Measures: Fatigue was assessed with the Fatigue Severity Scale; pain with 1 item of the Medical Outcomes Study 36-Item ShortForm Health Survey and by interview; and anxiety and depression by using the Hospital Anxiety and Depression Scale. Health-related quality of life was assessed using the World Health Organization Quality of Life ScaleeBrief Version. Associations between these conditions and quality of life were assessed by means of univariate and multivariate logistic regression analyses. Results: Symptoms of fatigue (40.5%), pain (73.4%), anxiety (24%), and depression (19%) were frequently found. Individuals often had multiple conditions. Fatigue was related to overall quality of life and to the quality-of-life domains of physical health and environment; anxiety was related to the psychological domain. Conclusions: Fatigue, pain, anxiety, and depression, potentially treatable symptoms, occur frequently in adults with DMD and significantly influence health-related quality of life. Archives of Physical Medicine and Rehabilitation 2015;96:1242-7 ª 2015 by the American Congress of Rehabilitation Medicine
Duchenne muscular dystrophy (DMD) is an X-linked, recessive neuromuscular disease in which there is an absence of the protein dystrophin, which among other things functions as a stabilizer of muscle cells, leading to progressive loss of muscle strength. The disease leads to severe physical disabilities and heavy dependency on care. In the past, most patients died in adolescence or young adulthood as a result of respiratory failure.1
Supported by the Children’s Fund Adriaanstichting (KFA grant no. 2005/0143), Johanna Children’s Fund (JKF grant no. 2005/0143), and Nuts Ohra Foundation (grant no. SNO-T0701-85). Disclosures: none.
As a result of improved care, especially the introduction of home mechanical ventilation, survival has increased significantly in the past few decades.2,3 The probability of reaching the age of 30 years has been found to have risen up to 85%.4 With an incidence of around 1 in 3500 live male births,1 there now is a considerable and relatively new group of adults with DMD. Without exception, individuals in this group are severely disabled. From studies of other neuromuscular diseases, it is known that patients experience a multitude of physical and psychological disorders. In a systematic review5 of quality of life in adult neuromuscular disease, there was a high level of evidence that quality of life in adults with various muscle diseases was significantly affected by disease severity, pain, fatigue, and mood.
0003-9993/15/$36 - see front matter ª 2015 by the American Congress of Rehabilitation Medicine http://dx.doi.org/10.1016/j.apmr.2015.02.012
Pain, fatigue, affective disorders in adult Duchenne muscular dystrophy In neuromuscular disorders, fatigueddescribed as an overwhelming sense of tiredness, lack of energy, and feeling of exhaustiondis a common symptom.6-9 It is related in daily life to impairment caused by facioscapulohumeral muscular dystrophy, myotonic dystrophy, and hereditary motor and sensory neuropathy type 1.10 Pain, too, is known to be a significant problem in many chronic neuromuscular diseases.11-13 Several studies12,14 have found that pain has a negative effect on health-related quality of life in different neuromuscular diseases. As for affective disorders, their prevalence differs among neuromuscular diagnoses15; depression and anxiety are moderately correlated to quality of life in adult patients with neuromuscular disease.16 Depression appears to go underdiagnosed in patients with chronic somatic diseases.17 In amyotrophic lateral sclerosis, depression plays a major role in determining quality of life.18 Identifying fatigue, pain, and affective disorders, when present, may allow effective treatment strategies to be used by health care providers. It is uncertain whether these conditions are more prevalent in adults with DMD than in the general population, how frequently they occur concurrently, and whether their presence is associated with lower health-related quality of life. In the present study, we posed the following 2 research questions: (1) What is the prevalence of fatigue, pain, and affective disorders in the adult DMD population? and (2) Are fatigue, pain, and affective disorders associated with lower health-related quality of life in this population?
Methods Participant selection and procedure This study was part of a larger cross-sectional study into the functioning and quality of life of adults with DMD and their informal caregivers.19,20 Patients were recruited by all 4 Centres for Home Ventilation in the Netherlands, and by Dutch rehabilitation centers and the Dutch patient organization for neuromuscular diseases, Spierziekten Nederland. Inclusion criteria were a diagnosis of DMD and an age 20 years. Subjects were interviewed during 2 consecutive visits to their homes. The study was approved by the Medical Ethical Committee of the Erasmus University Medical Center, Rotterdam, the Netherlands. All subjects gave written informed consent for participation.
Measurements We recorded age, data on physical situation (ventilation, gastrostomy, ambulation, hand function), and on participation (living situation, relational status, education level, work) to describe our population. Fatigue was assessed using the Dutch version of the Fatigue Severity Scale. This is a self-rated questionnaire consisting of 9 statements on the effect of fatigue; for instance, “My motivation is influenced by fatigue,” or “Fatigue interferes with my physical functioning.” Mean scores on the 9 questions range from 1 (no signs
List of abbreviations: DMD Duchenne muscular dystrophy HADS Hospital Anxiety and Depression Scale WHOQOL-BREF World Health Organization Quality of Life ScaleeBrief Version
www.archives-pmr.org
1243
of fatigue) to 7 (most disabling fatigue). The Fatigue Severity Scale has good validity and reliability.21,22 A score 4 is considered indicative of significant fatigue.23 We defined the presence of fatigue as a score 4; scores between 4 and 5 were defined as intermediate, and scores 5 as severe fatigue.24 Pain was assessed using the single-item ratings of pain of the Medical Outcomes Study 36-Item Short-Form Health Survey,25 which asks about the presence of bodily pain in the last 4 weeks, with scores from 1 (no pain) to 6 (very serious pain). Scores 2 are considered to be indicative of the presence of pain26; scores of 2 to 4 are labeled intermediate, and scores of 5 and 6 severe. We also assessed the pain locations and the number of locations per individual, and asked how long pain had been present. We defined pain with a duration 3 months as chronic pain. Affective disorders were assessed using the Hospital Anxiety and Depression Scale (HADS). The HADS, with separate subscales for anxiety (HADS-A) and depression (HADS-D), performs well in assessing symptom severity. Subjects are asked to score statements such as “I can sit at ease and feel relaxed” (anxiety) or “I feel cheerful” (depression), on a 4-point scale. On both subscales, scores can range from 0 to 21; scores of 8 to 11 indicate intermediate disorders, and 12 indicate clinical disorders.27 As with pain and fatigue, we considered intermediate and clinical scores on the HADS subscales to be indicative of the presence of disorders. The HADS has been shown to be reliable and valid for assessing anxiety and depression.28 In this study, both anxiety and depression scores 8 were considered to be indicative of the presence of disorders. Quality of life was assessed using the World Health Organization Quality of Life ScaleeBrief Version (WHOQOL-BREF). This is a questionnaire developed for cross-cultural comparison of quality of life relevant to global well-being.29 It consists of 26 items, with 1 item measuring overall quality of life, 1 measuring satisfaction with health, and 24 items that can be combined into subscales for the 4 domains of physical functioning, psychological functioning, social relationships, and environment. All items are assessed with a 5-point Likert scale. Item scores are converted to domain scores ranging from 4 to 20, with higher scores representing better quality of life. The WHOQOL-BREF has good validity, internal consistency, and test-retest reliability.30 In this study, the results on item 2 (satisfaction with health) were not used.
Statistics Data were analyzed using SPSS version 20.a Descriptive statistics were used to assess the results for the various measurements. Correlations between fatigue, pain, anxiety, and depression were assessed using Spearman rho. Because the results on the WHOQOL-BREF had no normal distribution, we dichotomized the scores to compare persons in the lowest range with the others.26 Overall quality of life was scored as good (item scores 4e5) versus poor (scores 1e3). For the WHOQOL-BREF domains, we used a cutoff of 1 SD below the reference mean. Subsequently, univariate and multivariate logistic regression analyses were performed with fatigue, pain, anxiety, and depression as independent variables. Since we found no significant associations of age, level of education, and ventilation type with the various domains of quality of life (results not shown), we did not adjust for these factors in the main analyses. In addition, we analyzed the difference in prevalence of fatigue, pain, anxiety, and depression between subgroups of men with good (WHOQOL-BREF item 1, score 4e5) and poor
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(score 1e3) overall quality of life, using the Pearson chi-square test. In view of the number of analyses involved and the fact that in all analyses the same 4 independent variables were entered, we decided to set alpha at .01.
Results We located 151 patients; 80 agreed to participate in the study. One patient was excluded because too much data were missing. The mean age and the distribution of ventilation type (none, noninvasive, or invasive) of the patients who refused to participate in the study were comparable to those of the patients who agreed to participate. The mean age of the participants was 28.2 years (range, 20e44y). All participants but one were dependent on mechanical ventilation, with 46% receiving noninvasive ventilation and 53% receiving invasive ventilation. All were wheelchair dependent and severely impaired in upper limb function. Participation (employment, intimate relationships) was generally low (table 1). The prevalence of fatigue, pain, anxiety, and depression is shown in table 2. Fatigue was present in 40.5% of participants (95% confidence interval, 29.7%e51.3%), pain in 73.4% (63.7%e83.2%), anxiety in 24.0% (14.6%e33.4%), and Table 1
Demographics (NZ79)
Characteristics
Values
Age (y) Ventilation None Noninvasive Invasive Gastrostomy Mobility Vignos 9 Vignos 10 Hand function Brooke 3 Brooke 4 Brooke 5 Brooke 6 Living situation Parental home Individual Residential Relational status Steady partner On-off Single Educational level Practical Lower Intermediate High Employment (nZ70) None 24h >24h
28.26.3 (20e44) 1.3 45.6 53.2 27.88 98.7 1.3 2.5 6.3 88.6 2.5 58.2 7.6 34.1 9.0 3.8 87.2 46.8 24.1 20.3 8.9 84.3 11.4 4.3
NOTE. Values are mean SD (range) or percentages. Vignos 9, wheelchair dependent; Vignos 10, bedridden; Brooke 3, brings glass of water to mouth; Brooke 4, brings hands to mouth; Brooke 5, table-top activities; Brooke 6, no functional use of hands.
depression in 19.0% (14.6%e23.4%). For pain, anxiety, and depression, only small proportions of affected patients indicated severe disorders. The Venn diagram in figure 1 shows the co-occurrence of the studied conditions, with anxiety and depression grouped: 23% had 3 of the conditions, 19% two, 41% one (mostly pain), while 18% of the participants had none of the conditions. Correlations between the conditions were all below 0.5 (Spearman r). Pain locations and number of locations per individual are shown in table 3. More than half of the individuals had pain in more than 1 location; 65% of the subjects had had pain for longer than 3 months; and 25% used pain medication, mostly nonsteroidal anti-inflammatory drugs. Table 4 shows the quality of life overall and in the separate domains. The mean scores were relatively high; only in the domain of social relationships was the mean score statistically significantly lower than in the Dutch reference population.31 In table 5 the results of the univariate logistic regressions are shown. Fatigue had a significant relationship with overall quality of life and with the domains of physical health, psychological functioning, and environment. Pain was associated only with the domain of physical health. Anxiety was associated with overall quality of life and with the domains of physical health and psychological functioning, and depression with overall quality of life and physical health. Figure 2 shows the prevalence of fatigue, pain, anxiety, and depression in those with a good and those with a poor quality of life. In those with a poor overall quality of life, the prevalence of all conditions was higher than in those with a good overall quality of life, except for pain (Pearson c2Z.83, PZ.36). In the multivariate analyses (table 6), fatigue emerged as significant for many domains: overall quality of life, physical health, and environment. Anxiety was related to the domain of psychological functioning; depression showed a trend toward an association with the domain of social relationships.
Discussion Our study population of adult men with DMD is a severely disabled group. To date, data on prevalence of fatigue, pain, and affective disorders have been limited in adults with DMD. Of the 4 Table 2 (NZ79)
Prevalence of fatigue, pain, anxiety, and depression Prevalence of Severity Level
Symptom Symptoms Scores
Overall Prevalence (95% CI)
Intermediate Severe
Fatigue Pain Anxiety Depression
40.5 73.4 24.0 19.0
21.5 67.1 17.7 17.7
3.651.4* 2.591.21y 5.033.68z 5.432.46x
(29.7e51.3) (63.7e83.2) (14.6e33.4) (14.6e23.4)
19.0 6.3 6.3 1.3
NOTE. Values are mean SD or percentages. Abbreviation: CI, confidence interval. * Fatigue measured with the Fatigue Severity Scale. Scoring: none, 1 to
ORIGINAL RESEARCH
Prevalence of Fatigue, Pain, and Affective Disorders in Adults With Duchenne Muscular Dystrophy and Their Associations With Quality of Life Robert F. Pangalila, MD,a,b Geertrudis A. van den Bos, PhD,c Bart Bartels, PT,a,b Michael Bergen, MD, PhD,b Henk J. Stam, MD, PhD,a Marij E. Roebroeck, PhDa,b From the aDepartment of Rehabilitation Medicine and Physical Therapy, Erasmus University Medical Center, Rotterdam; bRijndam Rehabilitation, Rotterdam; and cDepartment of Social Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
Abstract Objectives: To assess the prevalence of fatigue, pain, anxiety, and depression in adults with Duchenne muscular dystrophy (DMD), and to analyze their relationship with health-related quality of life. Design: Cross-sectional study. Setting: Home of participants. Participants: Adults (NZ80) with DMD. Interventions: Not applicable. Main Outcome Measures: Fatigue was assessed with the Fatigue Severity Scale; pain with 1 item of the Medical Outcomes Study 36-Item ShortForm Health Survey and by interview; and anxiety and depression by using the Hospital Anxiety and Depression Scale. Health-related quality of life was assessed using the World Health Organization Quality of Life ScaleeBrief Version. Associations between these conditions and quality of life were assessed by means of univariate and multivariate logistic regression analyses. Results: Symptoms of fatigue (40.5%), pain (73.4%), anxiety (24%), and depression (19%) were frequently found. Individuals often had multiple conditions. Fatigue was related to overall quality of life and to the quality-of-life domains of physical health and environment; anxiety was related to the psychological domain. Conclusions: Fatigue, pain, anxiety, and depression, potentially treatable symptoms, occur frequently in adults with DMD and significantly influence health-related quality of life. Archives of Physical Medicine and Rehabilitation 2015;96:1242-7 ª 2015 by the American Congress of Rehabilitation Medicine
Duchenne muscular dystrophy (DMD) is an X-linked, recessive neuromuscular disease in which there is an absence of the protein dystrophin, which among other things functions as a stabilizer of muscle cells, leading to progressive loss of muscle strength. The disease leads to severe physical disabilities and heavy dependency on care. In the past, most patients died in adolescence or young adulthood as a result of respiratory failure.1
Supported by the Children’s Fund Adriaanstichting (KFA grant no. 2005/0143), Johanna Children’s Fund (JKF grant no. 2005/0143), and Nuts Ohra Foundation (grant no. SNO-T0701-85). Disclosures: none.
As a result of improved care, especially the introduction of home mechanical ventilation, survival has increased significantly in the past few decades.2,3 The probability of reaching the age of 30 years has been found to have risen up to 85%.4 With an incidence of around 1 in 3500 live male births,1 there now is a considerable and relatively new group of adults with DMD. Without exception, individuals in this group are severely disabled. From studies of other neuromuscular diseases, it is known that patients experience a multitude of physical and psychological disorders. In a systematic review5 of quality of life in adult neuromuscular disease, there was a high level of evidence that quality of life in adults with various muscle diseases was significantly affected by disease severity, pain, fatigue, and mood.
0003-9993/15/$36 - see front matter ª 2015 by the American Congress of Rehabilitation Medicine http://dx.doi.org/10.1016/j.apmr.2015.02.012
Pain, fatigue, affective disorders in adult Duchenne muscular dystrophy In neuromuscular disorders, fatigueddescribed as an overwhelming sense of tiredness, lack of energy, and feeling of exhaustiondis a common symptom.6-9 It is related in daily life to impairment caused by facioscapulohumeral muscular dystrophy, myotonic dystrophy, and hereditary motor and sensory neuropathy type 1.10 Pain, too, is known to be a significant problem in many chronic neuromuscular diseases.11-13 Several studies12,14 have found that pain has a negative effect on health-related quality of life in different neuromuscular diseases. As for affective disorders, their prevalence differs among neuromuscular diagnoses15; depression and anxiety are moderately correlated to quality of life in adult patients with neuromuscular disease.16 Depression appears to go underdiagnosed in patients with chronic somatic diseases.17 In amyotrophic lateral sclerosis, depression plays a major role in determining quality of life.18 Identifying fatigue, pain, and affective disorders, when present, may allow effective treatment strategies to be used by health care providers. It is uncertain whether these conditions are more prevalent in adults with DMD than in the general population, how frequently they occur concurrently, and whether their presence is associated with lower health-related quality of life. In the present study, we posed the following 2 research questions: (1) What is the prevalence of fatigue, pain, and affective disorders in the adult DMD population? and (2) Are fatigue, pain, and affective disorders associated with lower health-related quality of life in this population?
Methods Participant selection and procedure This study was part of a larger cross-sectional study into the functioning and quality of life of adults with DMD and their informal caregivers.19,20 Patients were recruited by all 4 Centres for Home Ventilation in the Netherlands, and by Dutch rehabilitation centers and the Dutch patient organization for neuromuscular diseases, Spierziekten Nederland. Inclusion criteria were a diagnosis of DMD and an age 20 years. Subjects were interviewed during 2 consecutive visits to their homes. The study was approved by the Medical Ethical Committee of the Erasmus University Medical Center, Rotterdam, the Netherlands. All subjects gave written informed consent for participation.
Measurements We recorded age, data on physical situation (ventilation, gastrostomy, ambulation, hand function), and on participation (living situation, relational status, education level, work) to describe our population. Fatigue was assessed using the Dutch version of the Fatigue Severity Scale. This is a self-rated questionnaire consisting of 9 statements on the effect of fatigue; for instance, “My motivation is influenced by fatigue,” or “Fatigue interferes with my physical functioning.” Mean scores on the 9 questions range from 1 (no signs
List of abbreviations: DMD Duchenne muscular dystrophy HADS Hospital Anxiety and Depression Scale WHOQOL-BREF World Health Organization Quality of Life ScaleeBrief Version
www.archives-pmr.org
1243
of fatigue) to 7 (most disabling fatigue). The Fatigue Severity Scale has good validity and reliability.21,22 A score 4 is considered indicative of significant fatigue.23 We defined the presence of fatigue as a score 4; scores between 4 and 5 were defined as intermediate, and scores 5 as severe fatigue.24 Pain was assessed using the single-item ratings of pain of the Medical Outcomes Study 36-Item Short-Form Health Survey,25 which asks about the presence of bodily pain in the last 4 weeks, with scores from 1 (no pain) to 6 (very serious pain). Scores 2 are considered to be indicative of the presence of pain26; scores of 2 to 4 are labeled intermediate, and scores of 5 and 6 severe. We also assessed the pain locations and the number of locations per individual, and asked how long pain had been present. We defined pain with a duration 3 months as chronic pain. Affective disorders were assessed using the Hospital Anxiety and Depression Scale (HADS). The HADS, with separate subscales for anxiety (HADS-A) and depression (HADS-D), performs well in assessing symptom severity. Subjects are asked to score statements such as “I can sit at ease and feel relaxed” (anxiety) or “I feel cheerful” (depression), on a 4-point scale. On both subscales, scores can range from 0 to 21; scores of 8 to 11 indicate intermediate disorders, and 12 indicate clinical disorders.27 As with pain and fatigue, we considered intermediate and clinical scores on the HADS subscales to be indicative of the presence of disorders. The HADS has been shown to be reliable and valid for assessing anxiety and depression.28 In this study, both anxiety and depression scores 8 were considered to be indicative of the presence of disorders. Quality of life was assessed using the World Health Organization Quality of Life ScaleeBrief Version (WHOQOL-BREF). This is a questionnaire developed for cross-cultural comparison of quality of life relevant to global well-being.29 It consists of 26 items, with 1 item measuring overall quality of life, 1 measuring satisfaction with health, and 24 items that can be combined into subscales for the 4 domains of physical functioning, psychological functioning, social relationships, and environment. All items are assessed with a 5-point Likert scale. Item scores are converted to domain scores ranging from 4 to 20, with higher scores representing better quality of life. The WHOQOL-BREF has good validity, internal consistency, and test-retest reliability.30 In this study, the results on item 2 (satisfaction with health) were not used.
Statistics Data were analyzed using SPSS version 20.a Descriptive statistics were used to assess the results for the various measurements. Correlations between fatigue, pain, anxiety, and depression were assessed using Spearman rho. Because the results on the WHOQOL-BREF had no normal distribution, we dichotomized the scores to compare persons in the lowest range with the others.26 Overall quality of life was scored as good (item scores 4e5) versus poor (scores 1e3). For the WHOQOL-BREF domains, we used a cutoff of 1 SD below the reference mean. Subsequently, univariate and multivariate logistic regression analyses were performed with fatigue, pain, anxiety, and depression as independent variables. Since we found no significant associations of age, level of education, and ventilation type with the various domains of quality of life (results not shown), we did not adjust for these factors in the main analyses. In addition, we analyzed the difference in prevalence of fatigue, pain, anxiety, and depression between subgroups of men with good (WHOQOL-BREF item 1, score 4e5) and poor
1244
R.F. Pangalila et al
(score 1e3) overall quality of life, using the Pearson chi-square test. In view of the number of analyses involved and the fact that in all analyses the same 4 independent variables were entered, we decided to set alpha at .01.
Results We located 151 patients; 80 agreed to participate in the study. One patient was excluded because too much data were missing. The mean age and the distribution of ventilation type (none, noninvasive, or invasive) of the patients who refused to participate in the study were comparable to those of the patients who agreed to participate. The mean age of the participants was 28.2 years (range, 20e44y). All participants but one were dependent on mechanical ventilation, with 46% receiving noninvasive ventilation and 53% receiving invasive ventilation. All were wheelchair dependent and severely impaired in upper limb function. Participation (employment, intimate relationships) was generally low (table 1). The prevalence of fatigue, pain, anxiety, and depression is shown in table 2. Fatigue was present in 40.5% of participants (95% confidence interval, 29.7%e51.3%), pain in 73.4% (63.7%e83.2%), anxiety in 24.0% (14.6%e33.4%), and Table 1
Demographics (NZ79)
Characteristics
Values
Age (y) Ventilation None Noninvasive Invasive Gastrostomy Mobility Vignos 9 Vignos 10 Hand function Brooke 3 Brooke 4 Brooke 5 Brooke 6 Living situation Parental home Individual Residential Relational status Steady partner On-off Single Educational level Practical Lower Intermediate High Employment (nZ70) None 24h >24h
28.26.3 (20e44) 1.3 45.6 53.2 27.88 98.7 1.3 2.5 6.3 88.6 2.5 58.2 7.6 34.1 9.0 3.8 87.2 46.8 24.1 20.3 8.9 84.3 11.4 4.3
NOTE. Values are mean SD (range) or percentages. Vignos 9, wheelchair dependent; Vignos 10, bedridden; Brooke 3, brings glass of water to mouth; Brooke 4, brings hands to mouth; Brooke 5, table-top activities; Brooke 6, no functional use of hands.
depression in 19.0% (14.6%e23.4%). For pain, anxiety, and depression, only small proportions of affected patients indicated severe disorders. The Venn diagram in figure 1 shows the co-occurrence of the studied conditions, with anxiety and depression grouped: 23% had 3 of the conditions, 19% two, 41% one (mostly pain), while 18% of the participants had none of the conditions. Correlations between the conditions were all below 0.5 (Spearman r). Pain locations and number of locations per individual are shown in table 3. More than half of the individuals had pain in more than 1 location; 65% of the subjects had had pain for longer than 3 months; and 25% used pain medication, mostly nonsteroidal anti-inflammatory drugs. Table 4 shows the quality of life overall and in the separate domains. The mean scores were relatively high; only in the domain of social relationships was the mean score statistically significantly lower than in the Dutch reference population.31 In table 5 the results of the univariate logistic regressions are shown. Fatigue had a significant relationship with overall quality of life and with the domains of physical health, psychological functioning, and environment. Pain was associated only with the domain of physical health. Anxiety was associated with overall quality of life and with the domains of physical health and psychological functioning, and depression with overall quality of life and physical health. Figure 2 shows the prevalence of fatigue, pain, anxiety, and depression in those with a good and those with a poor quality of life. In those with a poor overall quality of life, the prevalence of all conditions was higher than in those with a good overall quality of life, except for pain (Pearson c2Z.83, PZ.36). In the multivariate analyses (table 6), fatigue emerged as significant for many domains: overall quality of life, physical health, and environment. Anxiety was related to the domain of psychological functioning; depression showed a trend toward an association with the domain of social relationships.
Discussion Our study population of adult men with DMD is a severely disabled group. To date, data on prevalence of fatigue, pain, and affective disorders have been limited in adults with DMD. Of the 4 Table 2 (NZ79)
Prevalence of fatigue, pain, anxiety, and depression Prevalence of Severity Level
Symptom Symptoms Scores
Overall Prevalence (95% CI)
Intermediate Severe
Fatigue Pain Anxiety Depression
40.5 73.4 24.0 19.0
21.5 67.1 17.7 17.7
3.651.4* 2.591.21y 5.033.68z 5.432.46x
(29.7e51.3) (63.7e83.2) (14.6e33.4) (14.6e23.4)
19.0 6.3 6.3 1.3
NOTE. Values are mean SD or percentages. Abbreviation: CI, confidence interval. * Fatigue measured with the Fatigue Severity Scale. Scoring: none, 1 to
