‘Mental health of children and adolescents with Duchenne muscular dystrophy’

SIR–There is limited literature available on the mental health of children and adolescents with DMD. Most of the information is based on anecdotal information obtained predominantly from parents. In completed studies that address the mental health of children with DMD, few standardized measures of mental health in children and adolescents with DMD have been used. A pilot study in which 10 male children and adolescents between the ages of 8 and 15 years and their parent(s) from the Thames Valley Children’s Centre, London, Ontario, Canada were randomly chosen to participate using a block randomization design. The children and adolescents were administered the following measures: The Schedule for Affective Disorders and Schizophrenia for School-Age Children (KSADS);1 Revised Children’s Manifest Anxiety Scale (RCMAS);2 Children’s Depression Inventory (CDI);3 Piers-Harris Children’s Self-Concept Scale;4 and Wechsler Intelligence Scale for Children: Third Edition (WISC-III).5 The following measures were administered to the parents: KSADS-Parent Version;1 Child Behavior Checklist (CBCL);6 and Conners’ Parent Rating Scale-Revised.7 To our knowledge, this is the first study of children and adolescents with DMD systematically using common standardized measures to evaluate their mental health. The KSADS appears to be a relatively good instrument to utilize with this population. It, however, is best administered to the adolescent with at least low average intelligence and the parents of children and adolescents with DMD. Some of the questions on the KSADS which address attention-deficit–hyperactivity disorder (ADHD) symptoms are not as useful as children with DMD cannot display the same type of hyperactivity symptoms, such as fidgetiness, due to their motor problems. The Conners’ Rating Scale and the CBCL, similar to KSADS, have measures regarding physical movement (e.g. restless, fidgety, running around at dinner time), which may not be rated high if the child has limited mobility. In addition, the KSADS questions on enuresis do not take into account the difficulty the child with DMD has going to the bathroom at night independently. Some very interesting findings were noted. Half of the children have ADHD, which has not been reported in the literature. Children with DMD have been identified as having acting-out behavior8–11 in the literature. This finding is particularly important because if ADHD is not identified in these children and adolescents, the child’s functioning may be impaired due to ADHD symptoms as well as due to their DMD. Children with ADHD often have substantial impairments in peer, family, and academic functioning.12,13 Therefore, these children may have improved quality of life if their ADHD symptoms are treated. In the area of internalizing disorders, none of these patients met criteria for depression, either on the KSADS or the self-administered CDI. These results are in contrast to some studies, which have cited depression9,14 and dysthymic disorder.14 However, it is consistent with a study of individuals with DMD and their siblings who were administered the CDI in which the individuals with DMD did not generally rate themselves as depressed.15 Six of these children were noted to have anxiety disorders on the KSADS but only three of the six rated themselves as anxious on the RCMAS; however, the lie scale (which is a scale in which the person gives a picture of one’s ideal behaviour) was high on the RCMAS. These children may want to minimize the anxiety symptoms because they are focused on their DMD; or they think the anxiety symptoms are part of their DMD; or they do not want to be viewed as different from others in additional ways; or they may not be able to articulate their feelings; or they think the anxiety symptoms are part of their medical illness. Previous studies have noted that as much as one-third of individuals with DMD have neurotic disturbance10 and internalizing symptoms.11 The children and adolescents with DMD studied did not endorse poor self-concept, with the exception of two children who scored low on popularity, on the Piers-Harris Self-Concept Scale. Some of the children may not have understood the questions, based on their intellect. The children may not examine themselves as they focus on their physical disability. This result is similar to that of a study by Tavormina16 in which 134 children with diabetes, asthma, cystic fibrosis, and hearing impairment were tested with a variety of self-esteem measures including the Piers-Harris and the results indicated that overall, the physically disabled children, not the hearing impaired, did not have poor self-esteem.16 In a study of adolescents with DMD, the males talked of their social isolation.17 It has been well documented that there is a relationship between IQ and muscular dystrophy such that the distribution follows a normal curve but the mean IQ is lower by about 10 to 25 points. There is a lack of agreement in the literature regarding whether IQ is stable over time, whether Verbal IQ decreases with age, or whether there are ages/stages at which IQ is more likely to decline.18–22 This study showed that in this sample of 10 children and adolescents with DMD, the average IQ was in the borderline range. There were also three of the 10 children with mental retardation,* two of the three were adolescents. (Mental retardation is defined in our study by using a Full-scale IQ less than 70 on the Wechsler Intelligence Scale for Children: Third Edition.) Therefore, one wonders if these adolescents were tested at age 8 years whether their IQ would have been higher. Given that this is a pilot study of only 10 children and adolescents, it is hard to determine if any of the results are significant. The measures that were used had not been tested on this population before so their use needs to be replicated in further studies. Also, it is a cross-sectional study so it will be important to determine if these mental health problems will persist into late adolescence and early adulthood. From this small pilot study, it appears that children and adolescents with DMD have comorbid mental health problems, particularly ADHD, anxiety disorders, and cognitive difficulties. A large multicentre study on the mental health of children and adolescents with DMD would be important to undertake. The use of standardized measures of psychiatric conditions on this population of children and adolescents is important and their usefulness needs to be determined in further studies. Studies on the treatment of these mental health problems in children with DMD will be important to determine if these individuals require different strategies to improve their mental health functioning. Following these individuals over time would be important to determine if the symptoms worsen and if these symptoms are part of the natural history of the DMD.

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