Becker Disease Management Care Team
There are no approved therapies for the treatment of Becker muscular dystrophy. A multi-disciplinary care team was recommended by the American Academy of Neurology (AAN) in 2014. The care team is recommended to include a neurologist and access to the following Healthcare Professionals as necessary.26
Muscular Dystrophy Multidisciplinary Care Team26
A multidisciplinary approach to providing care and a range of expertise required are key features of disease management.
Individual Monitoring
Becker muscular dystrophy is a chronic disease that requires regular monitoring throughout an individual’s lifetime to assess disease progression and to intervene where appropriate.
Patient Monitoring Objectives:3
A multidisciplinary evaluation can be adapted based on the patient’s needs at each visit.3
Disease Monitoring Recommendations Based on Becker Management Publications Including the 2023 Guideline Developed by The French BMD Working Group*
Neuromuscular Monitoring3
- Neuromuscular assessment is recommended based on tests used during initial evaluation citing the need for reproductibility over time.
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It is recommended that children and individuals most severely affected by Becker muscular dystrophy are screened for the following consequences of the disease:
- Muscle and joint contractures (Achilles tendons, hip, knee, elbow flexum)
- Kyphoscoliosis
- Assessment of performance of daily life activities
- Pain screening
Cardiac Monitoring
- As soon as the diagnosis of Becker muscular dystrophy is established, a comprehensive cardiac examination is recommeded.13
- It is recommended that the initial assessment include at least an electrocardiogram (ECG) and an echocardiogram. An echocardiogram is an important tool for the diagnosis of cardiomyopathy.3
- Findings should guide the frequency of cardiac monitoring.3
- The minimum monitoring frequency recommendation for individuals living with Becker with a normal ECG is 2 years.
- It is recommended that patients with LVEF < 50% are monitored at least once a year.3
- For an early diagnosis of cardiac fibrosis, cardiac MRI is recommended at least every 5 years.3
Respiratory Monitoring 3
- It is recommended that respiratory monitoring include clinical examination and systematic questionnaires to uncover any respiratory symptoms such as dyspnea or impaired sleep, and pulmonary function testing.
- If results from pulmonary function testing are abnormal, it is recommended that individuals living with Becker are referred to a pulmonologist for annual examination.
- Monitoring of respiratory function is recommended on a biennial basis every 2 years if findings are normal.
Laboratory Testing 3
- There are no standard laboratory tests for monitoring individuals living with Becker.
- While measuring the plasma level of CK is helpful for the diagnosis of Becker, it does not provide information on disease progression.
- If necessary, testing may be conducted to detect metabolic complications including glucose intolerance or diabetes, dyslipidemia or undernutrition (eg., anemia, iron deficiency, or vitamin deficiency, particularly D3 deficiency).
*The French Guideline was developed for clinical practice in France, and may not be applicable to clinical practices in other parts of the world
It is recommended that individuals living with Becker are also assessed for pain (rest and stress myalgia), swallowing, and cognitive disorders. The recommendation for neuropsychological assessment for children is every 2–3 years.3
Becker Management Considerations
Prevention and Management of Musculoskeletal Symptoms 3
- Physical therapy is the mainstay of managing individuals living with Becker. The goal is to optimize, maintain, or improve a patient’s muscle function, avoid complications secondary to a sedentary lifestyle, and help individuals maintain their independence.
- While a 30-45 minute physical therapy session conducted twice a week seems to provide the most benefit, therapy should be adapted based on a patient’s capacity, fatigability, and availability. Gentle and progressive exercises tailored to the effects of Becker on specific muscles are recommended.
- Exercise considerations can include:
- Maintenance of segmental and spinal muscular and joint flexibility
- Maintenance of segmental and spinal force
- Static and dynamic balance work in different positions
- Aerobic work
- Maintenance of functional capacities
- Promotion of adapted physical activity, and exercise at home
- Education on techniques for energy conservation, joint protection, prevention of overuse fatigue and falls
- Individuals living with Becker may require posture orthoses, while others with deformities such as anti-flexum knee or anti-equine orthosis, may be prescribed nocturnal posture orthoses. Orthopedic surgeries may be conducted to improve patients’ walking and posture stability.
Watch: Exercise and Physical Therapy in Becker MD
As muscle weakness progresses, in order to maintain their mobility and autonomy, individuals living with Becker may require assistive devices including scooters or wheelchairs. Changes in layout to living, working, and educational environments may also be warranted.3
Becker Management Considerations
Cardiac Treatments
- There are no specific guidelines for the treatment of dilated cardiomyopathy in individuals living with Becker.20 Duchenne muscular dystrophy guidelines may provide helpful management recommendations.
- Most cardiologists initiate treatment with an angiotensin converting enzyme (ACE) inhibitor when the left ventricular ejection fraction (LVEF) drops below 55%.14
- The treatment of individuals living with Becker with overt heart failure or left ventricular systolic dysfunction requires the same treatment approach as those in the general population, which can include pharmacotherapy (eg., ACE inhibitors or angiotensin II antagonists, beta blockers, mineralocorticoid receptor antagonists), cardiac resynchronization therapy and an implantable cardiac defibrillator if LVEF is below 30%.3
Cardiac transplantation
- Heart transplantation is generally limited to ambulant individuals living with Becker with terminal heart failure, who do not have significant respiratory compromise.3 Transplantation has been shown to prolong life expectancy; the correct dose of immunosuppressive drugs needs to be used to avoid worsening myopathy and maintain follow up with proper respiratory and muscle functional rehabilitation.14
As soon as the diagnosis of Becker muscular dystrophy is established, a comprehensive cardiac examination is recommended.3 Cardiac involvement in Becker is progressive and therapy is available. Cardiac examinations need to be regularly repeated.
Additional Preventative Considerations
Respiratory
- Pneumococcal and influenza annual immunization, as well as annual spirometry is recommended for all individuals living with Becker to avoid respiratory complications. Supportive respiratory aids may be needed for individuals with compromised forced vital capacity.3
Joint Contractures
- Joint contractures may be prevented by physical therapy to promote mobility and reduce cramps and muscle fatigue.14
Endocrine Considerations
- The importance of testosterone in the maintenance of muscle mass is critical, and testosterone therapy is recommended when hypogonadism is present.24
The range of expertise offered through a multidisciplinary approach is critical to caring for individuals living with Becker.
Medical treatments – Corticosteroids
- Corticosteroids
- Although treatment guidelines for Duchenne patients strongly recommend the use of the glucocorticosteroids prednisone or deflazacort, their use in Becker muscular dystrophy is still uncommon.21,22
- The uncertainty of benefit must be considered with the potential side effects that require monitoring and treatment.23
Investigational Therapies
- Investigation of new treatment modalities are ongoing, targeting different upstream and downstream disease drivers, such as contraction-induced muscle injuries, inflammation, and healing/regeneration.
Long-term corticosteroid use is associated with numerous side effects, warranting careful weighing of risk/benefit and diligent monitoring23
