AMYOTROPHIC LATERAL SCLEROSIS

(ALS) a.k.a. Lou Gehrig's disease

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5 %

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Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a rare neurological disease that affects the motor neuron nerve cells in the brain and spinal cord that are responsible for voluntary muscle control.

This rapidly progressing neurodegenerative disease causes oxidative damage to these neurons, which then leads to muscular atrophy and neuronal cell death.

ALS Lou Gehrig's Disease
ALS Lou Gehrig's Disease

Although not officially categorized, ALS is typically grouped into three stages; early, middle and late. Each stage brings its own set of symptoms, most of which worsen over time and eventually require constant care and medical assistance.

  • Early
    Early symptoms may include cramping, stiffness, muscle twitching or weakness in the neck, arms or legs, weight-loss, slurred speech (dysarthria) and/or difficulty chewing/swallowing (dysphagia). Anxiety and depression are also quite common.
  • Middle
    As the ALS progresses, patients will begin to show signs of muscle atrophy and start to become weaker, often to the point of becoming wheelchair-dependent.
  • Late
    Eventually symptoms can become so severe patients may not be able to stand, walk, or get out of bed on their own accord. Other less common symptoms that may develop over time can include a mild form of dementia, and problems decision-making.

ALS doesn’t affect your ability to taste, touch, or smell, or hear.  However once noticeable symptoms appear most patients live between 2-5 years, although approx. 10 percent of people with the condition survive for a decade or more.

Limb Onset ALS

Roughly two thirds of patients with typical ALS have a spinal form of the disease, known as limb onset. These patients present with symptoms related to specific muscle weakness and atrophy, which often starts in either in the upper and lower limbs.

In these cases spasticity may develop rapidly in the weakened atrophic limbs, in turn affecting ones gait and dexterity.

Bulbar Onset ALS

Patients who develop bulbar onset ALS, a form of ALS that mainly affects the bulbar region of the brainstem, usually present with dysphagia issues swallowing solids or liquids, and/or dysarthria problems with speaking. Wakened limb symptoms are also known to develop almost with bulbar symptoms, and in the majority of cases occur within 1-2 years.

 

Causes

The diagnosis of ALS is based on examination, clinical history, and electromyography, whilst also excluding screening for 'ALS-mimics’ such as Kennedy's disease, multifocal motor neuropathy, and cervical spondylotic myelopathy) to name just a few.

Most ALS treatments are currently supportive and multidisciplinary. However there are several medications shown to increase the life span of ALS patients. Non-invasive ventilation can also help prolong survival and improve a patient’s quality of life. So whilst there is no cure to reverse damage to motor neurons or cure ALS, modern treatments can make living with the disease easier.

Multidisciplinary Health Care

The best health care is best provided by a team of multidisciplinary professionals who work together to devise the best course of action for a patient’s specific treatment regime. This team may include medical practitioners such as respiratory therapists; nutritionist’s physicians; physical, occupational, speech therapists; social workers; clinical psychologists; pharmacists; and homecare/hospice nurses.

Medication For ALS

When it comes to medications for ALS, doctors can use one of two approved by the U.S. Food and Drug Administration (FDA). These are typically employed as a support plan for other, more natural ALS treatments:

  • Riluzole (Rilutek); oral medication designed to reduce damage to motor neurons by decreasing levels of glutamate. Clinical trials have shown those taking Riluzole may increase life expectancy by several months.
  • Edaravone (Radicava) is a drug administered intravenously and has been shown to slow the decline in the daily functionings of those with ALS.

New Treatment
Options

There are several new approaches being developed to help cure/treat ALS. These involve gene therapy approaches, testing drug-like compounds, and antibodies, as well as cell-based therapies in a range of these disease models. Furthermore, there are number of experimentory treatments being trialed in people with ALS.

Specialized Medical Assistance Equipment

In each case an individualized treatment plan and special equipment is often provide to assist patients in keeping as mobile, comfortable, and as self-dependent as possible.

Have questions?

In the U.S. it is reported that fewer than 50,000 people have ALS, and an estimated 200,000 people  worldwide are thought to suffer from the disease.

Yes, ALS is the same rare condition also known as Lou Gehrig’s disease.

Following diagnosis approx. 50% of patients with ALS live around 3 years, 25% live 5 years or more, and 10% live more than 10 years.

There is currently no cure for ALS, however there are several treatments, natural and manmade, that can improve quality of life and even increase life expectancy.

Since diet attributes greatly to quality of life, it is important to have professional nutritionists teach, and/or prepare small meals throughout the day that are both easy to eat and provide enough fiber, calories, and fluid.

Suction devices may also be employed in situations where excess fluids or saliva must be removed to prevent choking.

Eventually, when patients can no longer eat without assistance, doctors may advise inserting a feeding tube, which not only ensure the patients can consume the correct amount of nourishment, it also reduces the risk of choking and pneumonia that can result from inhaling liquids into your lungs.

Most treatment plan for ALS also includes some form of rehabilitation and physical therapy with the aim of giving patients the most range of motion possible. This treatment is different for every patient, and will be devised on a case-by-case basis dependent on the patients needs.

A speech therapist may also be called upon to assist with any speech impediments that may develop.

In more severe cases, computer-based speech synthesizers use eye-tracking technology and other tech devices can help patients develop ways to respond, much like the famous scientist Stephen Hawkins.

And whilst it may seem like sci-fi tech from the future, brain-computer interfaces (BCI) are being developed that will allow ALS suffers to communicate and control equipment using only their brain activity.

Unfortunately, the most damaging outcome to the health of ALS suffers becomes the inability to breath properly. As the repertory muscles start to weaken, the lungs fail to intake enough oxygen, and as a result, this is often the cause of death in those with severe ALS.

For this reason breathing support is often employed in the later years as breathing becomes more labored, and the shortness of breath drastically affects ones physical abilities.

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