The 3 phases of Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is a disease that affects the brain and spinal cord, causing a gradual loss of muscle function and control. The abilities to walk, speak, and swallow are progressively impaired to the point of complete loss.

ALS is a disease whose exact causes are still unknown, but it has multiple contributing factors: genetic and environmental. Although there is currently no known cure, there are therapies that can help people diagnosed with ALS live longer and improve their quality of life.

There are two main types of ALS whose symptoms manifest through specific phases. In this article we will talk in detail about the different stages of ALS as a pathology that unfolds its symptoms as time goes by.

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What is ALS?

Amyotrophic lateral sclerosis is a degenerative disease that attacks the nervous system and produces progressive loss of motor functions. Worldwide, the prevalence of ALS is 5 out of 100,000 people, which is why it is considered a rare disease.

It is a progressive disease, but the progression of symptoms can differ between people. Nerve cells called motor neurons or motoneurons control the voluntary muscle actions of the different parts of the body, such as the arms, legs and face, and ALS affects these nerve cells, which progressively lose their function and eventually they die. Then, messages that are sent through motor neurons stop being sent and the brain's ability to control voluntary movement is lost. Over time, not being used, the muscles begin to degenerate and become weaker.

Although progressive muscle weakness is a symptom that appears in all patients diagnosed with ALS, this may not be the first sign or indication of the disease.

The progression of the disease varies from person to person, and the beginnings of the disease can be difficult to notice, since no manifestations are perceived. As time passes, the signs and symptoms of the disease become more apparent. Some of the first visible symptoms include clumsiness, unusual tiredness in the limbs, muscle cramps and spasms, and slurred speech. Symptoms spread throughout the body as the disease progresses.

Brain function deteriorates over time, and in some people with ALS this can lead to a form of dementia, known as frontotemporal dementia. In addition, people's decisions and memories may also be compromised as the disease progresses. In terms of psychological functions, the emotional lability that results from the disease can also cause people to have more variable moods and emotional responses.

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ALS phases

There is no cure for ALS. The average lifespan of a patient with ALS is 3 years after diagnosis. 20% will live 5 years and 10% 10 years. Only 5% of people with ALS live 20 years or more. Most diagnosed patients die within 2 to 10 years after diagnosis due to respiratory failure resulting from progressive loss of function.

There is no official definition of the stages of ALS, as the disease progresses differently for each patient. Some people experience different symptoms at different times, and the progress of the condition and its consequences is variable, some people will lose function more quickly what others. Having said that, Broadly speaking, ALS can be divided into three stages: early, intermediate, and late..

early stage

Although there are cases of early onset, the average age of disease onset is 50 years. The disease initially progresses slowly and is often painless, which can cause patients to delay visiting the doctor for a long time.

If early symptoms affect patients' speech or ability to swallow, it is called "bulbar-onset ALS."

If the hands and feet are first affected and abnormal muscle movements occur, it is known as "limb-onset ALS."

Sometimes the disease begins with a loss of body weight or muscle mass, or abnormal breathing. About 3% of patients experience breathing problems as their first symptom.

At first, people typically experience muscle weakness, stiffness, and cramps. As there is no linear progression of the disease, some people may have more symptoms than others. People may be unable to perform everyday tasks, as symptoms affect muscles: ALS patients may fall, drop things, or be unable to button their T-shirt.

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intermediate stage

ALS is a variable disease in its development. Sometimes the progression is slow, occurs over years and has periods of stability with different levels of disability. Other times it is fast and there are no stable periods.

Different symptoms can be experienced during this stage of ALS: Difficulty swallowing and breathing. Muscles can become paralyzed, muscle loss is experienced and even these can become shorter. Joints lose movement, speech slows, and patients may laugh or cry in ways they cannot control.

Speech, swallowing and breathing are eventually affected by the disease: ALS patients often show signs of bulbar involvement when weakness and paralysis extend to the muscles of the trunks of their bodies.

The patient may lose the ability to speak completely, and have difficulty swallowing, and a reduced ability to cough. These problems put patients at risk for various respiratory problems (choking, respiratory infection, pneumonia) and malnutrition.

As the disease progresses, the muscles used for breathing can deteriorate and lose function, requiring the patient to be on a ventilator for life.

The muscle changes that a person may experience during the early stages of ALS may worsen during the middle stages of the disease. This means that the symptoms can also affect other parts of the body. Patients increasingly need help to perform daily activities as the disease enters this phase.

The patient will be treated by multiple specialized teams. Nowadays, pulmonology has made great advances, which can help the patient's quality of life in this stage of ALS.

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later stages

Over time, the patient becomes fully aware of his illness and its progression. Often this awareness causes psychological problems related to fear of dying and physical incapacity. The patient may be dealing with anxiety or depression. Quality of life is one of the main goals of ALS treatment, as the patient and her family have to deal with all aspects of the disease together.

When the patient has ALS, she becomes completely dependent on others. Patients and their families must face all phases of the disease with the help of specialized professionals.

When a person reaches the later stages of the disease, all of his muscles begin to paralyze, including those in his throat and mouth. This makes it impossible to speak, eat or drink. A tube may be needed to provide food and drink to the person.

The patient may also develop the following complications: respiratory failure, malnutrition, and pneumonia.

Some people who have ALS also develop frontotemporal dementia (DFT), while others who are initially diagnosed with DFT develop symptoms of ALS, FTD is also a progressive disorder that affects speech, behavior, and personality.

conclusion

ALS is a disease that affects the neurons that control movement and produces progressive loss of motor functions.

There is no cure for ALS, it is considered a progressive disease. Although its stages are not officially defined, since it is a fairly variable disease in the appearance of symptoms and its development.

Broadly speaking, amyotrophic lateral sclerosis can be divided into three stages. In the early stages it can cause muscle spasms or weakness. In the later stages there may be paralysis, breathing problems or loss of muscle mass. The most common cause of death is respiratory failure resulting from loss of function.

People with ALS can improve their lives using different treatment options that also include mental health support.