making muscle wasting history

People with DMD may have an increased risk of psychosocial difficulties, such as problems with behaviour and learning, and medical care is not complete without support for psychosocial wellbeing. Difficulties in social functioning may be due to specific challenges in particular skills, such as getting on with others, judging social situations, and perspectives, while the consequences of DMD (such as physical limitations) may result in social isolation, social withdrawal, and reduced access to social activities. For many parents, the stress caused by the psychosocial problems of the child and difficulties in getting them recognised and properly treated exceeds the stress associated with the physical aspects of the disease.
IMPORTANT FACTS TO REMEMBER:

• The psychosocial health of your son and your family is important.
• Your son may have a higher chance of having psychosocial difficulties.
• You and your family are at risk of some problems such as depression.
• The best way to manage psychosocial problems is to identify them early and start treatments.
• Correct use of language may be a problem, as may continuing difficulties at school. These behaviors are often seen in DMD and can be helped with proper assessment and input.
• Learning problems in DMD are not progressive and most boys catch up when they receive good help. If you think your child has worries about his condition, openness and a willingness to answer his questions can go a long way to preventing further problems. Boys with DMD often understand more about their condition than their parents think. It is important to answer questions openly, but be age appropriate in your answers and just answer what is being asked. This can be very difficult, but the staff at your clinic can offer help and guidance about what has worked for other families, as can patient support groups. Not everyone with DMD will have psychosocial difficulties, but families should keep an eye out for:
• Weaknesses in language development, comprehension, and short-term memory;
• Learning problems;
• Difficulty with social interactions and/or making friendships (i.e., social immaturity, poor social skills, withdrawal or isolation from peers);
• Anxiety/worry;
• Frequent arguing and temper tantrums;
• There is also increased risk of neurobehavioural and neurodevelopmental disorders, including autism-spectrum disorders, attention-deficit/hyperactivity disorder (ADHD), and obsessive-compulsive disorder (OCD);
• Problems may be encountered with emotional adjustment and depression. Anxiety may also be an issue and can be made worse by deficits in mental flexibility and adaptability (i.e. an overly-rigid thought process);
• This can also result in oppositional/argumentative behaviour and temper problems;
• In addition, increased rates of depression in parents of children who have DMD underscore the need for assessment and support of the entire family. The emphasis in psychosocial management should be strongly on prevention of problems and early intervention, as this will maximise the potential outcome. In general, the psychosocial problems should be treated with the same effective, evidence-based interventions that are used in the general population. This means it is important to look for help if you think there are problems in this area.

There is a well-documented pattern of speech and language deficits in some children with DMD, including problems with language development, short-term verbal memory, and phonological processing, as well as impaired IQ and specific learning disorders. These do not affect all children with DMD but should be looked out for and helped if they are present.

• Delay in attainment of early language milestones is common in boys who have DMD compared to children of the same age. The differences in gaining and improving language may be something that can be seen across childhood. It is important that this problem is looked for and treated. Evaluate and treat for delayed speech and language problems.
• Referral to a Speech Language Therapist (SLT) for speech and language evaluation and treatment is necessary if problems in this area are suspected.
• Exercises for the muscles involved in speech and help with articulation are appropriate and necessary for both young boys who have DMD with difficulties in this area and in older individuals who have deteriorating oral muscle strength and/or impaired speech intelligibility.

For older individuals, compensatory strategies, voice exercises, and speech amplifications are appropriate if it becomes difficult to understand the person with DMD due to problems with respiratory support for speech and vocal intensity. Voice Output Communication Aid (VOCA) assessment may be appropriate at all ages if speech output is limited.
Assessments
Although the needs of each child will vary, crucial times to consider assessments include at or near the time of diagnosis (a 6- to 12-month window for some evaluations may be beneficial in order to allow for adjustment following diagnosis), prior to entering school, and following a change in functioning. While not every clinic will have direct access to all of the assessments and interventions listed, these recommendations can serve as a guide to filling gaps in clinical staff and directing referrals, where appropriate.

• Areas of emotional adjustment and coping, development in learning relative to age, speech and language development, the possible presence of autism spectrum disorders, and social support should be assessed. (A social services professional can help access financial resources, develop social support networks, or provide mental health support to the family as needed).
• The psychosocial wellbeing in the individual with DMD, parents, and siblings should be a routine part of care for DMD.

Interventions
Care and support interventions

• A care coordinator can be the crucial person here: they can serve as a point of contact for families and become a trusted person. This person needs to have sufficient knowledge and background in neuromuscular disorders to be able to meet routine family information needs.
• Proactive intervention is essential to help avoid social problems and the social isolation that can occur in the context of DMD. Examples of useful interventions include increasing awareness and education about DMD in school and with peers, ensuring participation in appropriate sports and camps, provision of service dogs and contact with others via the internet and other activities. A special individualised education plan should be developed to address potential learning problems and to modify activities that might otherwise prove harmful to the child’s muscles (e.g. physical education), reduced energy/fatigue (e.g. walking long distances to/from lunch), safety (e.g. playground activities), and accessibility issues.
• Making sure the school is fully informed about DMD is important. Share with them all the information you have and identify the person at the school who is there to support children with additional needs. A proactive approach is important to make sure that the child with DMD accesses the full range of education he needs to develop good social interactions and prepare for further education and employment. So the school needs to be on side!
• Promoting independence and involvement in decision making (in particular, as relates to medical care) is necessary and of significant importance to promote autonomy and independence. This should be part of a planned transition program from paediatric to adult care.
• Helping to develop social and learning skills will make it easier to find a job and be part of normal daily life in adulthood. Boys with DMD benefit from having support to reach their personal goals.
• Access to palliative care services is appropriate to relieve or prevent suffering and to improve quality of life, as needed. In addition to pain management, palliative care teams may also be able to provide emotional and spiritual support, assist families in clarifying treatment goals and making difficult medical decisions, facilitate communication between families and medical teams, and address issues related to grief, loss, and bereavement.

Psychotherapy and drug interventions
Several well-known techniques exist to help in various areas. These include training for parents in trying to cope with bad behavior and conflicts, individual or family therapy and behavioural interventions. Applied behavior analysis may help with certain behaviours related to autism. Some children and adults may get benefit from the use of prescribed medicines to help with emotional or behavioural problems. These medicines can be used under specialized supervision and monitoring for depression, aggression, OCD or ADHD when these problems have been specifically diagnosed by specialist doctors.