Becker Muscular Dystrophy

GENERAL INFORMATION:

What is Becker muscular dystrophy? Becker muscular dystrophy (DIS-trah-fee), or BMD, is a genetic disease affecting different groups of muscles in the body. A genetic disease is one that you are born with and you may have inherited from your family. BMD causes muscle cells to die, and results in the muscle becoming weak, small, and deformed. BMD is similar to Duchenne muscular dystrophy (DMD), except that it develops at a much slower rate. Like DMD, BMD usually occurs in young boys and is rarely found in girls.

What causes Becker muscular dystrophy? Becker muscular dystrophy is caused by problems with the genes and chromosomes. Genes are little pieces of information that tell your body what to do or what to make. Chromosomes are like packages that hold all the genes.

What are the signs and symptoms of Becker muscular dystrophy? The most common problem in BMD is muscle weakness that slowly worsens. Symptoms usually start in late childhood or early adolescence and affects the lower limbs, in particular the thigh muscles. Other signs and symptoms may include:

• Early phase:
  
  
  • Clumsiness, frequent falling, a waddling type of walk, and difficulty climbing stairs.
    
    
  • Large calf muscles.
    
    
  • Muscle cramps with severe exercise.
    
    
  • Problems thinking, learning, focusing, and remembering.
    
    
  • Problems with the facial muscles, such as in closing the eyes or puckering the lips.
  
  
• Late phase:
  
  
  • Contractures (painful shortening of the muscles) or muscle wasting.
    
    
  • Heart problems.
    
    
  • Inability to walk or stand.
    
    
  • Lordosis (spine bends forward) or scoliosis (bending of the spine sideways).
    
    
  • Trouble swallowing or breathing and frequent lung infections.

How is Becker muscular dystrophy diagnosed? You may have one or more of the following tests:

• Biopsy: Caregivers remove a small piece of tissue from the muscle that is sent to the lab for tests.
  
  
• 12-lead ECG: This test, also called an EKG, helps caregivers look for damage or problems in different areas of the heart. Caregivers may need to prepare your skin by shaving off some hair, or cleaning it with a gritty lotion. Sticky pads are placed on your chest, arms, and legs. Each sticky pad has a wire that is hooked to a machine or TV-type screen. A short period of electrical activity in your heart muscle is recorded. Caregivers will look closely for certain problems or changes in how your heart is working. This test takes about 5 to 10 minutes. It is important that you lie as still as possible during the test. You may need this test more than once.
  
  
• Electromyography (EMG): Electromyography is a test that measures the electrical activity of your muscles. Your muscles are tested at rest and while you are using them. An EMG test may also check the nerves that control your muscles.
  
  
• Genetic test: This test provides genetic information to learn if a hereditary disease is causing the problem.
  
  
• Magnetic resonance imaging: This test is also called an MRI. Pictures of the muscles are taken during this test. Caregivers use these pictures to look for changes in your muscles.

How is Becker muscular dystrophy treated? There is no treatment for the muscle weakness and wasting of BMD. Medicines may be given to decrease BMD symptoms, such as muscle stiffness and pain. Other supportive therapies may be needed such as:

• Assistive devices: These are devices that protect and support the body to prevent further injury. These devices may include braces, crutches, or wheelchairs.
  
  
• Genetic counseling: You and your family will learn more about genetic or inherited diseases. This information may help you and your family in making make important decisions, such as planning a family.
  
  
• Medicines: Steroids and pain medicines may be given to decrease redness, pain, and swelling. Medicines may also be given to treat other conditions that are found with BMD, such as heart medicines.
  
  
• Rehabilitation therapies: Physical and occupational therapies may be needed to help you become better able to take care of yourself. Physical therapies are exercises to help make your bones and muscles stronger. Occupational therapy uses work, self-care, and play activities to help you in your daily life.
  
  
• Surgery: This may be needed to treat complications from BMD, such as contractures (shortened muscles).

Where can I find support and more information? Becker muscular dystrophy is a life-changing disease for you and your family. Accepting that you or a family member has BMD is hard. You and those close to you may feel angry, depressed, or frightened. These are normal feelings. Talk to your caregivers, family, or friends about your feelings.

• Muscular Dystropy Association
  3300 E. Sunrise Drive
  Tucson, AZ 85718
  Phone: 1-800-344-4863
  Web Address: http://www.mdausa.org
  

• National Society of Genetic Counselors
  401 N. Michigan Ave.
  Chicago, IL 60611
  Phone: 1-312-321-6834
  Web Address: www.nsgc.org
  

CARE AGREEMENT:

You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.

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