QUICK GUIDE – Management of DMD
Maintaining muscle strength and function. What assessments should be done and why?
Your son should have regular check-ups with a neuromuscular specialist, who has the expertise to monitor how things are going and to detect anything unusual that may need additional evaluation.
You need this information:
- To make timely decisions about treatment
- To anticipate and prevent problems that may arise
It is recommended that your son see your family doctor every six months and the neuromuscular specialist, physiotherapist and/or occupational therapist about every four months, if possible.
Tests that monitor DMD may vary in different clinics, so it’s important to use the same clinic consistently unless your doctor advises otherwise. The most important thing is to have regular tests to monitor the progress of DMD so that your doctor can properly evaluate the effects of your child’s current therapy and advise when to consider other forms of treatment.
Monitoring a child’s ability to move – Assessment of motor function
Physicians, specialists, orthopedic physical therapists, occupational therapists, medical technicians, wheelchair and other device providers, and orthopedic surgeons may assess your child’s motor function to monitor muscle extensibility and joint contractures, as part of routine follow-up examinations and rehabilitation.
Looking after bones – Orthopedic management
People with DMD who are not treated with corticosteroids have a 90% chance of developing scoliosis – a lateral curvature of the spine that worsens over time. Researchers have found that daily administration of steroids reduces the risk of scoliosis or at least delays its onset.
Proactive management of the risk of scoliosis requires:
- Preventive measures
- Bone health management
- Management of long bone fractures
Looking for breathing muscles – Pulmonary management
Usually, children with DMD don’t have trouble breathing or coughing while they are still walking. However, as they get older, their breathing muscles are eventually affected. They are at risk of chest infections, often due to an ineffective cough. Later, they may develop problems with breathing while sleeping. Eventually, they may require help with breathing during the day.
As breathing problems worsen in stages, a planned and proactive approach to respiratory care is possible, based on timely observation, prevention, and treatment. The care team must include a doctor and therapist with skill in the delivery of non-invasive ventilation and related techniques for increasing the amount of air to enter the lungs (lung volume recruitment) as well as manual and mechanically assisted cough.
Looking after the heart – Cardiac management
Why does your child need to see a heart specialist? A cardiologist can detect and treat the deterioration of heart muscle function. Children with DMD often develop heart muscle problems (cardiomyopathy), which can cause heart rhythm problems, such as palpitations. Heart problems usually happen with the overall progression of DMD.
As heart problems often happen silently – without the development of significant symptoms – they can be overlooked. Early detection means prompt treatment. The key factors to consider are routine observation and proactive management. Ensure that a cardiologist is part of your child’s care team.
Looking after the gut – Gastrointestinal management
You will probably consult the following specialists at various stages of DMD: a dietician or nutritionist, a swallowing/speech and language therapist, and a gastroenterologist
Looking after psychological and social problems – Psychosocial care
People with DMD have a greater risk of psychosocial problems, such as behavioural and learning disabilities. For that reason, it’s important to supplement medical care with psychosocial care. Some difficulties may be due to lack of certain psychosocial skills, such as knowing how to deal with others and judging social situations and different points of view. The physical deficits of DMD may lead to social isolation, withdrawal and reduced access to social activities. Some parents run into difficulty in having psychosocial problems recognized and properly treated. For many, the stress caused by their child’s psychosocial problems outweighs the stress of the physical aspects of DMD.
Children with DMD often understand their disease more than parents believe. If you think your child worries about his illness, be open and available to answer questions. Respond candidly, give age-appropriate answers, and limit answers to information that your child has requested. Open communication can be very hard, but hospital staff and family support groups can help by telling you what works for them.
Parents of children with DMD should focus on preventing psychosocial problems and seeking help, including treatment, as soon as possible for best results.
Not everyone with DMD has psychosocial difficulties, but families should watch for:
- Any weaknesses in language development, comprehension, and short-term memory
- Learning disabilities
- Difficulties in making or keeping friends (social immaturity, insufficient social skills, withdrawal/isolation)
- Frequent disputes
- Mood swings
Children with DMD have a higher risk of behavioural problems and neurological developmental disorders, including autism spectrum disorders, attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD).
Limitations in adaptation skills may appear. A lack of flexibility or mental adaptability (resistance to change) may aggravate anxiety. Depression, frequent arguments (oppositional behaviour), and character-related problems may occur.
The very high rate of depression among parents of children with DMD stresses the need to offer psychological support to all family members. It’s important to seek help.