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What is ALS (Amyotrophic Lateral Sclerosis)?

ALS, formally known as amyotrophic lateral sclerosis, is a rare neurological disease involving the nerve cells responsible for controlling voluntary muscle movement, such as chewing, walking, breathing and talking. ALS is progressive, where the condition becomes more severe over time. ALS is also known as Lou Gehrig’s Disease.

ALS Symptoms

ALS 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 ALS.

Early ALS 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 ALS may appear in hand or arm as one has trouble 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, individuals will eventually be unable to stand or walk, get in or out of bed on their own, or use their hands and arms.

What Causes ALS?

The cause of ALS is unknown, and scientists do not yet know why ALS strikes some people and not others. However, evidence from scientific studies suggests that both genetics and environment play a role in the development of ALS. An important step toward determining ALS 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 ALS. ALS researchers are also studying the impact of environmental factors. Researchers investigate many possible causes, such as exposure to toxic or infectious agents, viruses, physical trauma, diet, and behavioural and occupational factors.

Who Gets ALS?

The Centers for Disease Control and Prevention estimate that between 14,000 – 15,000 Americans have ALS.

There are several potential risk factors for ALS, 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 ALS. However, as age increases, the difference between men and women disappears.
  • Race and ethnicity. Caucasians and non-Hispanics are most likely to develop the disease.

How is ALS Treated?

Presently, there is no cure for ALS. However, treatments available can help control symptoms, prevent unnecessary complications, and make a living with the disease easier.

They include

  • Medication
  • Physical therapy
  • Speech therapy
  • Nutritional support
  • Breathing support

Prognosis for ALS

Most with ALS have a lifespan of 3 to 5 years from when the symptoms first appear. However, about 10% of people with ALS survive for 10 or more years.

ALS Research

The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge about ALS. The goals of NINDS research on ALS 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.

ALS Areas of Research

  • Cellular defects
  • Stem cells
  • Familial versus sporadic ALS
  • Biomarkers
  • Exploration of new treatment options

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, emphasizes mobility technologies specific to ALS patients. 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 a 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. Quantum power chairs are designed to give those living with ALS optimal medical comfort and maximum independence.

Mobility and Assistive Technology Needs

People with ALS often require power mobility assistance 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 with 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.

Solution

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, and power positioning, and can accommodate a vent tray and oxygen holder.

The Edge 3, and Q6 Edge® 2.0 fit this criterion and are options for a person in the beginning stages of ALS.