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Becker Muscular Dystrophy (BMD)

Medical Management

Thanks to general medical advances, particularly in cardiology, people with Becker muscular dystrophy (BMD) are living longer in the 21st century than in previous decades. As of 2019, most therapies are supportive in nature, although truly disease-modifying therapies are the subject of intense research.

MDA Care Center physicians can provide referrals to specialists and therapists for these forms of care. The use of available therapies can help maintain comfort and function and prolong life expectancy.

To view a presentation on medical management of BMD by pediatric neurologist Brian Tseng, see the August 2012 Taking the Reins of Your Medical Care and Participating in Clinical Trials.

Anesthesia

People with BMD may have unexpected adverse reactions to certain types of anesthesia. It's important that the surgical team know about that a patient has BMD so that complications can be avoided or quickly treated.

Braces, scooters, and wheelchairs

Braces, also called orthoses, can support just the ankle and foot or extend over the knee. Ankle-foot orthoses are sometimes prescribed for night wear to keep feet from pointing downward and keep the Achilles tendon stretched. (Orthoses also are known as orthotics.)

To view an August 2012 video presentation on this topic, see Functional Treatment Considerations: Orthotics.

Some people with BMD ultimately require wheelchairs or scooters. Although some look at these devices as symbols of disability, most users find using devices allow one to actually be more mobile, energetic and independent than when trying to walk on very weak legs. Scooters and wheelchairs are especially valuable when covering long distances.

To view a presentation by an occupational therapist, see the August 2012 video Functional Treatment Considerations: Occupational and Physical Therapy.

Cardiac care

Cardiomyopathy, which means deterioration of the heart muscle, is common in BMD. The American Academy of Pediatrics recommends that those with BMD have cardiac evaluations at least every other year beginning at age 10 or even earlier. Cardiac transplantation may be offered as an option to patients with BMD with severe cardiomyopathy and limited or skeletal (voluntary) muscle disease.

Carriers of BMD also are at higher-than-average risk of developing cardiomyopathy. The academy suggests that carriers should undergo a complete cardiac evaluation in late adolescence or early adulthood, or sooner if symptoms occur, and should be evaluated frequently.

Also see:

Contractures

As muscle deteriorates, a person with muscular dystrophy often develops fixations of the joints, known as contractures. If not treated, these can become severe, causing discomfort and restricting mobility and flexibility. The impact of BMD can be significantly minimized by keeping the body as flexible, upright, and mobile as possible.

There are several ways to minimize and postpone contractures. Range-of-motion exercises, performed on a regular schedule, help delay contractures by keeping tendons from shortening prematurely. It’s important that a physical therapist demonstrate the correct way to do range-of-motion exercises.

Braces on the lower legs help keep the limbs stretched and flexible, delaying the onset of contractures.

When contractures have advanced, surgery may be performed to relieve them. A tendon release procedure, also called heel cord surgery, can treat ankle and other contractures while a person is still walking.

Diet

No special dietary restrictions or additions are known to help in BMD. Most doctors recommend a diet similar to that for any growing boy, but with a few modifications.

A combination of immobility and weak abdominal muscles can lead to severe constipation, so the diet should be high in fluid and fiber, with fresh fruits and vegetables dominant.

For boys and men who use power wheelchairs, who aren’t very active, or who take prednisone (steroidal drug), excessive weight gain can occur. Caloric intake should be restricted to keep weight down, as obesity puts greater stress on already weakened skeletal muscles and the heart. Supplementing vitamin D and calcium is also recommended. Doctors have found that a low-calorie diet doesn’t have any harmful effect on the muscles. BMD patients that present dysphagia (difficulty with swallowing) should be referred to a speech and language therapist for swallowing assessment. Patients with constipation, gastroesophageal reflux, or gastrointestinal motility problems should be referred to a gastroenterologist. Some patients may need placement of a feeding tube.

Also see:

Exercise

Exercise can help build skeletal muscle, keep the cardiovascular system healthy and contribute to feeling better. But in muscular dystrophy, too much exercise could damage cardiac and skeletal muscle. Consult with your doctor about how much exercise is best. A person with BMD can exercise moderately but shouldn’t go to the point of exhaustion.

Some experts recommend swimming and water exercises (aquatic therapy) as a good way to keep muscles as toned as possible without causing undue stress on them. The buoyancy of the water helps protect against certain kinds of muscle strain and injury.

Before undertaking any exercise program, make sure to have a cardiac evaluation.

Learning disabilities and mental health

Dystrophin deficiency can cause some cognitive problems in some people. Children and adults with BMD who are suspected of having a learning disability can be evaluated by a neuropsychologist through a school system’s special education department or at a medical center with a referral from an MDA Care Center.

If a learning disability is diagnosed, educational and psychological interventions can begin right away. The specialist may prescribe exercises and techniques that can help improve these deficits, and schools can provide special help with learning.

BMD patients should be assessed for their psychosocial status, as should their family, at every Care Center visit. Referral to a psychiatrist or psychologist may be needed.

Medications

Medications that lessen the workload on the heart are sometimes prescribed for BMD. There’s some evidence that treatment with angiotensin converting enzyme (ACE) inhibitors and beta blockers can slow the course of cardiac muscle deterioration in BMD if the medications are started as soon as abnormalities on an echocardiogram (imaging of the heart) appear, but before symptoms occur. It has been shown that early treatment with perindopril delayed the onset and progression of prominent left ventricle dysfunction in children with DMD.1

Medications belonging to a group known as corticosteroids – or glucocorticoids –  have been found effective in slowing the course of DMD. Data for or against the use of corticosteroids in BMD are lacking. However, some physicians prescribe corticosteroids for severe BMD in much the same way as they would for DMD, if the patient or family wants to try this type of medication.

Prednisone is by far the most commonly prescribed corticosteroid for DMD in the United States. When taken at relatively high doses for long periods of time, it can have significant side effects, such as weight gain, short stature, delayed puberty, decreased bone density, vertebral bone fractures, behavioral abnormalities, and cataracts. It has been shown that prednisone therapy can be helpful for patients diagnosed with BMD as well.2 However, little is known about the effect of the benefits of glucocorticosteroid (a class of corticosteroid) for BMD patients. Another commonly used corticosteroid for DMD is deflazacort (Emflaza), which was approved by the US Food and Drug Administration (FDA) in February 2017 to treat the disease. Emflaza has been proven to improve motor function, strength, and pulmonary function and to reduce the risk of lumbar spine deviations and loss of walking ability.

Glucocorticoid treatment should be stopped progressively to avoid adverse reactions.

Physical and occupational therapy

physical therapy program is usually part of the treatment for BMD. Your MDA Care Center physician will refer you to a physical therapist for a thorough evaluation and recommendations.

The primary goals of physical therapy are to allow greater motion in the joints and to prevent contractures and scoliosis (spinal curvature). Occupational therapy focuses on specific activities and functions, such as work tasks, recreation, driving, dressing, or using a computer.

For an August 2012 video on this topic, see Functional Considerations: Occupational and Physical Therapy.

Respiratory care

In some people with BMD, particularly as they age, breathing muscles can weaken, resulting in less-than-optimal breathing, particularly during sleep. This can be treated by a noninvasive strategy known as bilevel positive airway pressure (BiPAP).

Coughing muscles also can become weak, allowing mucus to build up in the respiratory tract, which can lead to obstruction and infection. A device known as a cough assist can help with this problem.

To see a presentation by a pulmonary medicine specialist, see the August 2012 video Lung Health in Neuromuscular Disease.

Immunizations

Children with BMD should receive all vaccinations recommended by the US Centers for Disease Control and Prevention (CDC). Some vaccines should be given before the start of glucocorticoid treatment. Ask your doctor for more information.

References

  1. Duboc, D. et al. Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy. J. Am. Coll. Cardiol. (2005). doi:10.1016/j.jacc.2004.09.078
  2. Johnsen, S. D. Prednisone therapy in Becker’s muscular dystrophy. J. Child Neurol. (2001). doi:10.1177/08830738010160111406

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