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 inheritance pattern.
Primary types of muscula dystrophy
Muscular dystrophy encompasses various genetic disorders characterized by progressive muscle weakness and degeneration. Here are some of the main types:
- Duchenne Muscular Dystrophy (DMD): The most common and severe form, primarily affecting boys. It appears in early childhood, progresses rapidly, and leads to muscle weakness and the need for a wheelchair.
- Becker Muscular Dystrophy (BMD): Similar to DMD but milder and slower progressing. It appears in late childhood or early adulthood, with varying severity and prolonged walking ability.
- Limb-Girdle Muscular Dystrophy (LGMD): A group of dystrophies primarily impacting shoulder and hip muscles. Onset occurs in late childhood, adolescence, or adulthood, causing muscle weakness and mobility difficulties.
- Facioscapulohumeral Muscular Dystrophy (FSHD): Characterized by facial, shoulder blade, and upper arm muscle weakness. It typically begins in adolescence or adulthood, progressing slowly.
- Myotonic Dystrophy (DM): The most common adult-onset form, featuring muscle stiffness (myotonia) and weakness. It includes DM type 1 (DM1) and DM type 2 (DM2), with varying symptoms and age of onset.
Remember, this is not an exhaustive list, and there are other types of muscular dystrophy.
Each type has distinct genetic causes, age of onset, progression, and affected muscle groups.
Management involves a multidisciplinary approach tailored to the specific type of muscular dystrophy.
How is muscular dystrophy diagnosed?
Muscular dystrophy is diagnosed based on muscle biopsy results, 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.
The 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 their lifespan due to heart and breathing complications.
However, some forms do not affect life expectancy at all, and ongoing research attempts to find cures and treatments to slow muscle weakness.
Mobility for those with muscular dystrophy
A speech therapist can help improve the ability to speak clearly or teach the use of communication devices, as well as address difficulties with muscles used in eating and swallowing.
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 powerchairs, emphasizes mobility technologies specific to the needs of those with muscular dystrophy.
Quantum powerchairs 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 powerchairs feature the latest advanced technologies to increase the independence of those with muscular dystrophy.
iLevel seat elevation technology allows users to operate the powerchair seated or standing.
Bluetooth is also integrated into Quantum’s Q-Logic 3 electronics, so those with muscular dystrophy can operate much of their environment with the powerchair drive control itself.
Quantum powerchairs are designed to provide optimal medical comfort and maximum independence for those 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 to utilise a manual wheelchair, it is suggested that the individual be evaluated for power mobility to promote increased independence and continued social interaction with peers.
The client should have a power base to meet various seating, positioning and electronics needs.
A client may require a power positioning system, like a 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 important in helping a client compensate for asymmetries, providing proper stability and support and managing pressure while sitting.
Many custom accessories can be adapted in order to meet the needs of your client.
Optional LED fender lights on the Q6 Edge 2 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 can accept full seating and positioning options.
The Edge 3 fulfills 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 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 can accept expandable electronics.
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