Muscular Dystrophy
What is Becker muscular dystrophy in children?
Muscular dystrophy (MD) is an inherited (genetic) disorder of the muscles. It is called
a neuromuscular disease. There are several types. Becker muscular dystrophy (BMD)
is a rare type. It has milder symptoms than Duchenne muscular dystrophy (DMD), which
is more common. BMD causes weakness of skeletal muscles, breathing muscles, and the
heart muscle. Over time, the muscle fibers break down.
What causes Becker muscular dystrophy in a child?
BMD is a genetic disease. This means it passes from parents to children. It is caused
by a faulty gene on the X chromosome. It mostly affects boys. Girls are only carriers
and usually don’t have symptoms.
People normally have 23 pairs of chromosomes in each cell of their body. The 23rd
pair controls a person’s gender. This pair is 2 X chromosomes for females. The pair
is 1 X and 1 Y chromosome for males.
The faulty BMD gene is a recessive gene. This means the gene needs to be on each X
chromosome for symptoms of the disease to occur. A girl would need 2 copies--1 copy
on each X chromosome. If she has only 1 copy on 1 X chromosome, she is a carrier of
the disease but will not have symptoms. Boys only have one X chromosome. If their
X chromosome has a faulty gene, they will have symptoms.
Which children are at risk for Becker muscular dystrophy?
A child is more at risk for BMD if he or she has a family member with the disease.
Girls are rarely affected by this type of muscular dystrophy.
What are the symptoms of Becker muscular dystrophy in a child?
Symptoms usually start during teen or early adult years. They can include:
- Muscle cramping and feeling weak when exercising
- Trouble walking, running, jumping, climbing stairs
- Falling often
- Muscle pain
- Enlarged calves
- Walking on tips of toes
- Weakness in arms and shoulders
- Tiredness
The symptoms of Becker muscular dystrophy can be like other health conditions. Make
sure your child sees his or her healthcare provider for a diagnosis.
How is Becker muscular dystrophy diagnosed in a child?
The healthcare provider will ask about your child’s symptoms and health history. He
or she may also ask about your family’s health history to find out if other family
members have had muscular dystrophy. He or she will give your child a physical exam.
Your child may also have tests, such as:
- Blood tests. These may include genetic blood tests.
- Electromyogram (EMG). This is a test to check if muscle weakness is a result of destroyed muscle tissue
or nerve damage.
- Muscle biopsy. A small sample of muscle tissue is taken and looked at under a microscope.
- Electrocardiogram (ECG). This is a test that records the electrical activity of the heart. It shows abnormal
rhythms (arrhythmias), and detects heart muscle damage.
How is Becker muscular dystrophy treated in a child?
Treatment will depend on your child’s symptoms, age, and general health. It will also
depend on how severe the condition is.
There is no known cure for BMD, or treatment to stop the muscles from weakening. The
goal of treatment is to keep muscles as strong as possible to allow a child to function
independently.
Treatment choices include:
- Physical therapy to strengthen muscle and help prevent muscle contractures
- Braces and splints
- Steroid medicine to help build muscle
- Surgery to treat contractures
- Medicine for heart disease
- Medicines from clinical research trials
- Wheelchair use
- Nutritional counseling
- Psychological counseling
Talk with your child’s healthcare providers about the risks, benefits, and possible
side effects of all treatments.
What are the possible complications of Becker muscular dystrophy in a child?
Children with BMD may have additional health problems, such as:
- Heart muscle weakening and not pumping blood well (dilated cardiomyopathy)
- Breathing and lung problems
- Scoliosis, a sideways curving of the back bones (vertebrae)
- Diet, nutrition, and digestive problems
- Trouble focusing, learning, or controlling emotions
How can I help prevent Becker muscular dystrophy in my child?
Your healthcare provider may advise genetic counseling. You can discuss with a counselor
the risk for a Becker muscular dystrophy in a future pregnancy.
How can I help my child live with Becker muscular dystrophy?
BMD is a condition that gets worse over time (progressive). It needs life-long management.
Walking and sitting often become more difficult as a child grows. In time your child
will need a wheelchair in his or her late teen years or early 20s. This is because
the leg muscles become too weak to work. In some cases a child may need a wheelchair
earlier.
Your child will also need a heart imaging test every 1 to 2 years. This may be a heart
echocardiogram or an MRI. These tests look at the function and health of the heart.
A child with BMD will need special care during surgery to prevent problems from anesthesia.
People with BMD usually live until their 40s. Problems from cardiomyopathy are a common
cause of death.
A healthcare team will work with your family to improve your child's function and
to give support as you learn to care for your child's needs.
You can contact the Muscular Dystrophy Association (www.mda.org) for support, resources,
and help.
When should I call my child’s healthcare provider?
Call the healthcare provider if your child has:
- Symptoms that don’t get better, or get worse
- New symptoms
Key points about Becker muscular dystrophy in children
- Becker muscular dystrophy (BMD) is a rare inherited disorder of the muscles.
- BMD causes weakness of skeletal muscles, breathing muscles, and the heart muscle.
Over time, the muscles may become too tight and pull together painfully.
- A child is more at risk for BMD if he or she has a family member with the disease.
Girls are rarely affected.
- There is no known cure for BMD, or treatment to stop the muscles from weakening. The
goal of treatment is to keep muscles as strong as possible to allow a child to function
independently.
- Your healthcare provider may advise genetic counseling. You can discuss with a counselor
the risk of a Becker muscular dystrophy in a future pregnancy.
Next steps
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the visit, write down the name of a new diagnosis, and any new medicines, treatments,
or tests. Also write down any new instructions your provider gives you for your child.
- Know why a new medicine or treatment is prescribed and how it will help your child.
Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
- Know how you can contact your child’s provider after office hours. This is important
if your child becomes ill and you have questions or need advice.
Online Medical Reviewers:
- Cunningham, Louise, RN
- Turley, Raymond Kent, BSN, MSN, RN