What is Muscular Dystrophy?
Muscular dystrophy (MD) encompasses a group of more than 30 genetic diseases characterized by progressive weakness and degeneration of the skeletal muscles that control movement. The particular form of muscular dystrophy dictates which muscles are primarily affected, the degree of weakness, how fast they worsen, and when symptoms begin. Some forms of muscular dystrophy are seen in infancy or childhood, while others may not appear until middle age or later. The disorders differ in terms of the distribution and extent of muscle weakness (some forms of muscular dystrophy also affect cardiac muscle), age of onset, rate of progression, and pattern of inheritance.
Primary Types of Muscular Dystrophy
- Duchenne muscular dystrophy (DMD), the most common type of the disease, is caused by a problem with the gene that makes a protein called dystrophin. Muscles break down and a person gradually becomes weaker. DMD affects boys. Symptoms usually start between ages 2 and 6. By age 10 or 12, kids with DMD often need to use a wheelchair. Eventually, even the muscles that control breathing get weaker, and a person might need a ventilator to breathe.
- Becker muscular dystrophy (BMD), like DMD, affects boys. The disease is remarkably similar to DMD, but its symptoms may start later and can be less severe. With BMD, symptoms like muscle breakdown and weakness sometimes don't begin until age 10 or even until adulthood. People with BMD can also have breathing, heart, bone, muscle, and joint problems. Many people with BMD can live long, active lives without using a wheelchair
- Emery-Dreifuss muscular dystrophy (EDMD) usually starts causing symptoms in late childhood to early teens and sometimes as late as age 25. EDMD is another form of muscular dystrophy that affects mostly boys. It involves muscles in the shoulders, upper arms, and shins, and it often causes joint problems. The heart muscle may also be affected.
- Limb-girdle muscular dystrophy (LGMD) affects boys and girls equally, weakening muscles in the shoulders and upper arms and around the hips and thighs. LGMD can begin as early as childhood or as late as mid-adulthood, and it often progresses slowly. Over time, a wheelchair might be necessary to get around.
- Facioscapulohumeral muscular dystrophy (FSHD) can affect both guys and girls, and it usually begins during the teens or early adulthood. People with this type of muscular dystrophy might have trouble raising their arms, whistling, or tightly closing their eyes. This form of muscular dystrophy may be mild in some people and more severe in others.
- Myotonic muscular dystrophy (MMD) is a form of the disease in which the muscles have difficulty relaxing. In teens, it can cause a number of problems, including muscle weakness and wasting (where the muscles shrink over time), cataracts, and heart problems.
- Congenital muscular dystrophy (CMD) is the term for all types of muscular dystrophy that show signs in babies and young children, although muscular dystrophy isn't always diagnosed right away.
- Distal muscular dystrophy (DYSF) has its onset between 20 to 60 years of age. Symptoms include weakness and wasting of muscles of the hands, forearms, and lower legs. Progression is slow and not life-threatening.
- Oculopharyngeal muscular dystrophy (PABPN1) has an age at onset of 40 to 70 years. Symptoms affect muscles of eyelids, face, and throat followed by pelvic and shoulder muscle weakness.
How is Muscular Dystrophy Diagnosed?
Muscular dystrophy is diagnosed based on the results of muscle biopsy, increased creatine phosphokinase, electromyography, and genetic testing. Other tests that can be done are chest X-ray, echocardiogram, CT scan, and magnetic resonance image scan, which via a magnetic field can produce images whose detail helps diagnose muscular dystrophy.
How is Muscular Dystrophy Treated?
There is currently no cure for muscular dystrophy, but much research is ongoing. Physical therapies and medications are used to address muscle and respiratory issues.
Prognosis for Muscular Dystrophy
The prognosis for muscular dystrophy depends on the form one has. In some cases, a person with muscular dystrophy will get progressively weaker to the extent that it shortens lifespan due to heart and breathing complications. However, some of the forms do not affect life expectancy at all, and ongoing research is attempting to find cures and treatments to slow muscle weakness.
Mobility for Those with Muscular Dystrophy
Many with muscular dystrophy experience the progressive loss of the ability to walk and position themselves, necessitating the use of a specialized power wheelchair. Quantum Rehab®, the global leader in individualized power chairs, puts an emphasis on mobility technologies specific toward the needs of those with muscular dystrophy.
Quantum Power Chairs incorporate power-adjustable seating for user repositioning and comfort; specialty drive controls, including those requiring minimal hand strength; and a highly adaptable design to meet an individual’s current and future needs.
Quantum Power Chairs feature the latest advanced technologies to increase the independence of those living with muscular dystrophy. iLevel® seat elevation technology allows a user to operate the power chair at seated or standing height. Bluetooth® is also integrated into Quantum’s Q-Logic 3 electronics, so those with muscular dystrophy can operate much of their environment with the power chair drive control, itself.
For those with muscular dystrophy, Quantum Power Chairs are designed to provide optimal medical comfort and maximum independence. Please click here for more information on Quantum Power Chair solutions for those living with muscular dystrophy.
Mobility and Assistive Technology Needs
Depending on the severity and progression of the condition, some clients may not require assistive technology and may only have an affected gait. Others may only require the use of a walker or braces to accomplish independent mobility.
Once an individual shows a decreased ability in the utilization of a manual wheelchair, it is suggested that the individual be evaluated for power mobility in order to promote increased independence and continued social interaction with peers.
The client should be fitted with a power base that can meet various seating, positioning and electronics needs. A client may require a power positioning system, like power tilt and recline, to help with pressure relief or independent weight shifting. The electronics of the power base should be expandable so they can continue meeting the individual’s needs as the condition progresses.
A customized seating system is an important part of helping a client compensate for asymmetries, providing proper stability and support and managing pressure while sitting. There are also many custom accessories that can be adapted in order to meet the needs of your client. Optional LED fender lights on the Q6 Edge® 2.0 help clients see and be seen.
Often, clients change over time due to growth. To adapt to these changes, the seating system should be modular to grow with the client.
Another consideration for a client with MD is to be sure a power base can accept a ventilator if the requirement arises.
Clients with MD who have advanced mobility needs may benefit from a power base that has the ability to accept full seating and positioning options. The Edge 3 fulfils client seating and positioning needs and is compatible with an array of drive controls. Optional 4.5 mph iLevel® Power Adjustable Seat Height lifts clients up to 12”, promoting greater social interaction and independence.
The Q6 Series also accepts a selection of seating and positioning options, and possesses a proven track record of quality, reliability and customer satisfaction. All chairs in the series have the capability to accept expandable electronics.
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