Amyotrophic Lateral Sclerosis - Diagnosis and Treatment | Care Tips for living with ALS

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease is a motor neuron disease (MND) that weakens muscles and impacts physical function.

In this article, you will learn how to diagnose amyotrophic lateral sclerosis?

What is the best treatment for ALS? Medications or Therapies?

And how do you cope with ALS diagnosis?

ALS is a nervous system disease that weakens muscles and impacts physical function.

Amyotrophic Lateral Sclerosis - Diagnosis and Treatment - Care Tips for living with ALS

What is Amyotrophic Lateral Sclerosis? 

Fast facts on ALS

Amyotrophic lateral sclerosis (ALS) is the most serious and widespread motor neuron disease (MND). It is also known as Lou Gehrig's disease, after the well-known American baseball player who developed this disease.

ALS is a group of degenerative diseases characterized by a motor neuron injury in the brain, in the spinal cord and in the pathways responsible for crossing the nerve stimulation between them.

ALS affects the motor nerve cells in the majority of the voluntary muscles in the body, in which the brain monitors and controls the muscle action.

These nerve cells are damaged and inactivation and non-innervation of muscles lead to weakness and even complete paralysis.

With continued amyotrophic lateral sclerosis and the passage of time, the muscles responsible for operating the limbs, for swallowing, for speech and for breathing, are gradually damaged in an indefinite and inconsistent order.

However, ALS does not affect the five senses, mental performance or internal organs (heart, bladder, digestive system, etc.).

The prevalence of amyotrophic lateral sclerosis ranges between 1 - 6 cases out of every 100,000 people each year, while ALS in 5% - 10% of cases is genetically predominant.

The common age group for amyotrophic lateral sclerosis is between 40 and 70 years old (55 years old, on average), while there are cases of ALS at an older age than these.

It is very rare for the emergence of ALS under the age of 40, although it is not entirely impossible.

The signs of amyotrophic lateral sclerosis are often easy at first and not specific, and for this reason, there is often a delay in the diagnosis of ALS.

The basic and primary complaints are those resulting from muscle weakness. The patient complains of difficulty writing, lifting, climbing and walking.

Muscle cramps, change in sound and speech, difficulty swallowing, and even drooping of the head may appear in the initial stages of ALS, due to weakness in the neck muscles.

The emergence and development of amyotrophic lateral sclerosis vary from patient to patient.

In general, a typical development is an appearance of advanced weakness in the muscles of the limbs and in the muscles responsible for swallowing, chewing and speaking, even weakness in the breathing muscles. This condition requires the patient to be connected to the ventilator.

The average period of time for a patient to survive ranges from 3 to 5 years.

20% of patients live more than 5 years and about 10% of them live more than 10 years.

Read here: Amyotrophic Lateral Sclerosis (ALS) -Types, Symptoms and Causes 

Diagnosis of Amyotrophic Lateral Sclerosis (ALS)

It is difficult to diagnose ALS from a single test, so the accurate diagnosis of the condition made by doctors and specialists depends on the symptoms that exist in the patient, in addition to the results of physical tests, in order to exclude other conditions from diseases with similar symptoms.

Here, we have mentioned some tests that may help diagnose ALS:

Electromyography (EMG): The doctor inserts a needle electrode into the various muscles through the skin. Muscle abnormalities seen in EMG can help doctors diagnose or rule out ALS.

EMG is responsible for detecting electrical energy in muscles when they're at rest and when they contract. EMG also helps guide the exercise therapy.

Nerve conduction study (NCS): NCS test measures the ability of nerves to send impulses to the body's muscles and determines whether there is nerve damage or certain muscle or neurological disorders in different areas of the body.

Magnetic Resonance Imaging (MRI): MRI scans use radio waves and a powerful magnetic field to produce detailed images of the brain and spinal cord.

An MRI scan detects herniated disks in the neck, spinal cord tumors or other problems that can cause ALS symptoms.

Blood and urine tests: Analyzing the blood and urine samples in the laboratory can help the doctor eliminate ALS signs and other possible causes of ALS symptoms.

Lumbar puncture (spinal tap): A lumbar puncture test uses a small needle inserted between two vertebrae in the lower back, most commonly to remove a sample of the spinal fluid and collect cerebrospinal fluid for diagnostic lab testing.

Muscle biopsy: A muscle biopsy is a relatively simple procedure in which a piece of muscle tissue is removed for laboratory testing.

This test can help the doctor see if you have a muscular disease rather than ALS and may discover problems with the nervous system, vascular system, musculoskeletal system, or connective tissue.

These tests can help, in excluding conditions with similar symptoms such as peripheral nerve damage, peripheral neuropathy, myopathy or muscle diseases.

Diseases and medical problems that can cause symptoms similar to ALS include: multiple sclerosis (MS), human immunodeficiency viruses (HIV), poliovirus, Lyme disease and West Nile viruses.

If symptoms appear in both the upper and lower motor neurons, ALS may be present in people who suffer from these symptoms.

Symptoms of the upper motor neurons include resistance to movement in the muscles, stiffness, brisk reflexes and rapid reactions. Symptoms of the lower motor neurons include muscle atrophy, weakness, twitching and tingling feeling.

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What is the Best Treatment for ALS?

What is the best treatment for amyotrophic lateral sclerosis (ALS)?

Treatment of Amyotrophic Lateral Sclerosis 

There is no cure for amyotrophic lateral sclerosis and treatments cannot reverse the damage of ALS, but current treatments aim to slow the progression of symptoms and prevent serious or unnecessary complications and make you more comfortable and independent.

ALS causes a set of physical, mental and social changes that occur in the patient during the stages of disease development.

Therefore, it is often necessary to have an integrated team of health care professionals and doctors trained in many areas to help patients manage their symptoms, provide them with the necessary care, support their psychological and emotional states, improve their quality of life and new lifestyle and prolong their survival.

An integrated team of doctors and other healthcare professionals can help you select the right treatment options for you. You can choose any type of treatment option that suits you best.

Medications for ALS

Several drugs have been tried to treat amyotrophic lateral sclerosis, but they could not show a significant effect.

However, there are some drugs approved by the United States Food and Drug Administration (FDA) for treating ALS.

Rilutek (Riluzole): Rilutek is an oral tablet available to treat ALS approved by the U.S FDA in 1995.

Rilutek inhibits the release of glutamate, slows the disease progression and extends life expectancy from three to six months. This process may involve lowering the levels of glutamate in the body.

Glutamate is an excitotoxin that has been linked to nerve cell damage.

Riluzole has some side effects such as gastrointestinal conditions, changes in liver function and dizziness. If you are on medication, your doctor will monitor your liver functions and blood counts.

Tiglutik (Riluzole): Tiglutik is an oral suspension approved by the FDA in September 2018 for the treatment of ALS.

Tiglutik is a liquid oral formulation of riluzole that has been on the market for ALS treatment for more than 20 years.

Exservan (riluzole): Exservan is an oral film approved by the FDA, in November 2019.

Exservan is administered without the need for water, which can be beneficial for ALS patients who have difficulty swallowing.

Radicava (Edaravone): Radicava was approved by the FDA in May 2017 to treat ALS.

Radicava has been shown to reduce the decline in physical activity by one third.

Some side effects of Radiceva are headache, bruising, and shortness of breath. It is given daily for two weeks a month.

Nuedexta (dextromethorphan/quinidine): Nuedexta is the first FDA-approved drug for the treatment of pseudobulbar affect (inappropriate laughing or crying), which some people living with ALS develop. It was approved by the FDA in 2011.

Nuedexta is a combination of dextromethorphan and quinidine and improves swallowing in bulbar onset ALS patients with pseudobulbar affect.

The doctor can also prescribe appropriate medication to provide relief from the various symptoms associated with ALS, including:

• Excessive saliva and phlegm
• Muscle cramps and spasms
• Uncontrolled outbursts of laughing or crying
• Sleep problems
• Speech disorders and problems
• Depression
• Fatigue
• Pain
• Constipation

There are several scientific projects that are looking for different ways to use new and existing drugs to treat various aspects of ALS disease.

Therapies for ALS

Physical therapy: Physical therapy helps people with ALS deal with pain and mobility problems and helps their muscles function at their best.

The physical therapist can address the pain, mobility, walking, stiffness, equipment needs that help patients make their lives easier to get around and stay independent.

The physical therapist provides assistance and information by:

Mobility aids, such as wheelchairs and walkers

Low-impact exercises, to maintain cardiovascular fitness, muscle strength, and overall health.

The devices needed to make the patient's life easier such as ramps.

Breathing therapy: Breathing therapy may be needed in a time when the respiratory muscles become weaker than before.

Respiratory problems are treated by respiratory devices. Doctors might provide the patient with devices to assist them in breathing at night.

In cases where the patient is unable to breathe with his own strength independently, this means that he suffers from ALS in a very severe case and the majority of his muscles are in severe paralysis, but the patient is conscious and aware of his condition.

Breathing devices, in this case, help the patient to breathe better at night, and some patients may need mechanical ventilation.

Doctors connect one end of a tube to a respirator, while the other end is inserted in a surgically created hole at the front of the neck, leading to the windpipe.

Occupational therapy: Occupational therapy can help the patient maintain their independence for a longer period despite hand and arm weakness.

Adaptive equipment and techniques can help the patient perform daily activities such as bathing, dressing, grooming, and eating.

Occupational therapy can also train the patient to compensate for the areas of weakness in both the hand and arm and help them modify their home to allow access if they have trouble walking safely.

Speech therapy: Speech therapy is useful when ALS symptoms begin to make speaking more difficult. A speech therapist can teach patients how to do adaptive techniques for easier communication with others and make their speech more understandable.

Other communication methods include an alphabet board or pen and paper,  computer-based equipment with the synthesized speech or tablet computers with text-to-speech applications. These devices can help patients communicate with others.

Psychological and social support: Psychologists, social workers, and others provide emotional support for patients and their families.

They can help them with insurance, financial issues and getting equipment and paying for devices they need.

What they can do to counter this type of disease will depend primarily on their money to some extent, but local support groups can help patients cope with the emotional and financial challenges caused by ALS, by providing practical help or advice.

Nutritional support: Nutritional support is an important remedy because the difficulties of swallowing that a patient may encounter can make it difficult for him to get enough nutrients.

With the development of ALS and the appearance of swallowing problems prominently, the possibility of ingesting food into the stomach can be examined directly.

Nutritionists will work with patients and their family members to ensure the patients are eating nutritious meals that are easier to swallow and meet their needs. Feeding tubes and suction devices might help.

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How do you cope with ALS diagnosis?

It can be a common challenge for people with ALS and their loved ones to maintain a sense of normal life and manage daily activities. Here, we have compiled some of the best tips and tools that can help both caregivers and patients adapt to living with ALS and make daily life functioning smooth and easy.

Stephen Hawking - Stephen Hawking has lived past 70 with ALS

Care Tips for living with ALS

These amazing tips we will mention in the next paragraph may help people with ALS and their loved ones to adapt to the changing stages of the disease and adjust to the changing situation.

Keep in touch:

Living life is important to everyone, regardless of illness, but staying in touch and staying positive is one of the most important tips we can give you.

Social communication with others is an important factor in relieving the symptoms of the disease. It is therefore advisable to keep in contact with friends and loved ones and keep up with the greatest number of past activities.

Don’t lose contact with your loved ones and don’t isolate yourself.

There may be online or local support groups that can provide insights through shared experiences and answer questions from patients with ALS.

Find a trustworthy caregiver:

Caregivers help you focus on healthy living and your abilities.

Find a friend, relative, or trustworthy caregiver to take you out for the day or to come and stay for a weekend.

You can use back braces if you want to go out. The back brace can help you improve posture and muscular support. But if you don’t feel comfortable going out, stay in and invite your friends over.

Think beyond your physical changes:

Many people with ALS live rewarding lives despite physical changes and limitations. Don't think of ALS as your entire identity but try to consider it as only part of your life.

Be hopeful:

Believe in yourself and don't lose your hope. Some people with ALS live more than three to five years and some live 10 years or more. Be optimistic and maintain your positive outlook. This will help you improve the quality of your life.

Be practical:

Make the necessary changes to your home or your living environment in order to make your daily functioning safe and accessible such as purchasing a lift chair, raising toilet seats, or adding a safety frame and stability devices like grab bars or bedrails.

If you have a car, register to get a disability placard for your car.

If you want to go out, get a bag ready with hand wipes, tissues, toileting aid, easy-to-hold cutlery, extra medication, and adaptive silverware.

Plan ahead:

It can be difficult when a family member finds that he can no longer do what he could have done before, but planning for the future and foreseeing possible restrictions will help you make decisions about your life and your care and help calm you and your loved ones.

If you decide now about your future medical care, you can easily decide where you want to spend your last days and where you want some life-extending procedures.

Research financial help

Treatment may become very expensive as the disease progresses, so it is advisable to research whether you are entitled to help through ALS Recovery Fund, Medicaid, Medicare, Social Security Disability, ALS Guardian Angels, Les Turner ALS Foundation, ALS Therapy Development Institute, Asha Ek Hope Foundation and MND Scotland.

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