What is Lou Gehrig’s Disease (ALS)?
Lou Gehrig’s disease, formally known as amyotrophic lateral sclerosis or ALS, is a rare family of neurological diseases that involve the nerve cells responsible for controlling voluntary muscle movement, such as chewing, walking, breathing and talking. Lou Gehrig’s disease is progressive, and the condition becomes more severe over time. Lou Gehrig’s disease receives its name from Hall of Fame baseball player, Lou Gehrig, who was diagnosed with the condition in 1939 at age 36.
Lou Gehrig’s Disease (ALS) Symptoms
Lou Gehrig’s disease symptoms can initially be so subtle that they are overlooked. Nevertheless, the symptoms develop into more obvious weakness or atrophy that may cause a physician to suspect Lou Gehrig’s disease. Early Lou Gehrig’s disease symptoms may include:
- Muscle twitches in the arm, leg, shoulder, or tongue.
- Muscle cramps.
- Tight and stiff muscles (spasticity).
- Muscle weakness affecting an arm, a leg, neck or diaphragm.
- Slurred and nasal speech.
- Difficulty chewing or swallowing.
The first sign of Lou Gehrig’s disease may appear in the hand or arm as an individual experiences difficulty with simple tasks such as buttoning a shirt, writing, or turning a key in a lock. Regardless of where the symptoms first appear, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals may develop problems with moving, swallowing (dysphagia), speaking or forming words (dysarthria), and breathing (dyspnea). Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually individuals will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms.
What Causes Lou Gehrig’s Disease (ALS)?
The cause of Lou Gehrig’s disease is unknown, and scientists do not yet know why Lou Gehrig’s disease strikes some people and not others. However, evidence from scientific studies suggests that both genetics and environment play a role in the development of Lou Gehrig’s disease. An important step toward determining Lou Gehrig’s disease risk factors was made in 1993 when scientists supported by the National Institute of Neurological Disorders and Stroke (NINDS) discovered that mutations in the SOD1 gene were associated with some cases of familial Lou Gehrig’s disease.
Lou Gehrig’s disease researchers are also studying the impact of environmental factors. Researchers are investigating a number of possible causes such as exposure to toxic or infectious agents, viruses, physical trauma, diet, and behavioural and occupational factors.
Who Gets Lou Gehrig’s Disease (ALS)?
The Centers for Disease Control and Prevention estimate that between 14,000 – 15,000 Americans have Lou Gehrig’s disease. There are several potential risk factors for Lou Gehrig’s disease including:
- Although the disease can strike at any age, symptoms most commonly develop between the ages of 55 and 75.
- Men are slightly more likely than women to develop Lou Gehrig’s disease. However, as age increases, the difference between men and women disappears.
- Caucasians and non-Hispanics are most likely to develop the disease.
How is Lou Gehrig’s Disease (ALS) Treated?
Presently, there is no cure for Lou Gehrig’s Disease. However, there are treatments available that can help control symptoms, prevent unnecessary complications, and make living with the disease easier. They include:
- Physical therapy
- Speech therapy
- Nutritional support
- Breathing support
Most with Lou Gehrig’s disease have a lifespan of three to five years from when the symptoms first appear. However, about 10% of people with Lou Gehrig’s disease survive for 10 or more years.
Lou Gehrig’s Disease Research
The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge about Lou Gehrig’s disease. The goals of NINDS research on Lou Gehrig’s disease are to understand the mechanisms involved in the development and progression of the disease, investigate the influence of genetics and other potential risk factors, identify biomarkers, and develop new and more effective treatments.
Lou Gehrig’s Disease Areas of Research Include
- Cellular defects
- Stem cells
- Familial versus sporadic ALS
- Exploration of new treatment options
Mobility for those with Lou Gehrig’s Disease
A universal factor for those living with Lou Gehrig’s disease is the progressive loss of the ability to walk, necessitating the use of a power wheelchair. Quantum power chairs, puts an emphasis on mobility technologies specific toward those living with Lou Gehrig’s disease. Quantum Power Chairs incorporate power-adjustable seating for user repositioning and comfort; speciality drive controls, including using a single finger or one’s head to operate the power chair; 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 Lou Gehrig’s disease. 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 Lou Gehrig’s disease can operate much of their environment with the power chair drive control.
Mobility for those with ALS
A universal factor for those living with ALS is the progressive loss of the ability to walk, necessitating the use of a power wheelchair. Quantum Rehab, the global leader in individualized power wheelchair solutions, puts an emphasis on mobility technologies specific to those living with ALS. Quantum Power Chairs incorporate power-adjustable seating for user repositioning and comfort; speciality drive controls, including using a single finger or one’s head to operate the power chair; 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 ALS. 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 ALS can operate much of their environment with the power chair drive control.
In all, Quantum power chairs are designed to give those living with ALS optimal medical comfort and maximum independence.
Mobility and Assistive Technology Needs
Often, people with ALS require the assistance of power mobility very quickly due to the rapidly progressive nature of the disease. In the beginning stages of ALS, a client may require a power chair with high-back support to assist with positioning and performance of Mobility Related Activities of Daily Living (MRADLs).
If the individual has adequate hand movement for controlling the power chair, then a joystick should be used since it provides the most direct control for driving a power base. It is often advised to provide a client with a hand controller that has switch jacks. As the user’s ability changes, switches can be added to operate on/off and mode operations.
As the disease progresses, the individual may find he or she has a limited amount of time during the day when a joystick can be operated. It is at this time that more advanced electronics, such as a mini-proportional joystick, head array or chin control may need to be considered to operate the power chair. The individual will most likely require the use of postural support components and power positioning functions to assist in pressure relief, postural realignment and function.
These components may also help with transfers, sitting tolerance and comfort. The power positioning options for consideration include power tilt and recline systems, power-adjustable seat height, power articulating leg rests or a power articulating foot platform. Providing a power base that can accept oxygen and eventually a ventilator is also very important. Optional LED fender lights on the Q6 Edge 2.0 help clients see and be seen.
Based on the mobility needs of an individual with ALS, they may benefit from a power base with the ability to accept full seating and positioning options, various drive controls, power positioning, and can accommodate a vent tray and oxygen holder.