The Stages of ALS: Understanding the Progression of the Disease

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of the human motor system that can lead to muscular paralysis. As ALS develops, the motor neurons in the primary motor cortex, brainstem, and spinal cord begin to degenerate. The exact underlying cause of the disorder is still unknown; however, researchers consider it to result from various genetic mutations and environmental factors. 

Amyotrophic lateral sclerosis is more predominant among males than females, and its average incidence rate is 50,000 people per year. Researchers are developing cell-based therapies to help manage disease symptoms, slow disease progression, and maintain patients’ quality of life. 

Let’s take a look at the 7 stages of ALS, symptoms, treatment options, cell-based stem cell therapy, and the importance of early interventions in managing disease symptoms. 

What are the stages of ALS?

The devastating effects of Lou Gehrig’s disease are classified into 7 stages of ALS, which are:

1. The early stage of ALS.
2. Diagnosis stage of ALS.
3. The middle stage of ALS: involves the second region.
4. The middle stage of ALS: involves the third region.
5. Gastronomy stage of ALS.
6. Final stages of ALS.
7. Death stage of ALS.

The early stage of ALS

ALS is a rare disorder with subtle early symptoms that can be easily overlooked, leading to severe symptoms. The average onset age for ALS is 50 or 60 years; however, it may develop in young people as well. It’s important to note that the disease symptoms can vary from person to person, and the rate at which the disease progresses can also vary. 

Symptoms of the early stage

Initially, ALS early stages can be fairly hard to spot because of subtle symptoms that may look harmless. Some early signs of the disorder can look like this:

1. Weakness of muscles around the arms, legs, or other body parts.
2. Muscle cramping or twitching due to nerve damage.
3. Slurred speech or difficulty speaking due to weak tongue or throat muscles.
4. Difficulty swallowing because of weak muscles around the esophagus.
5. Tiredness as a result of excessive efforts in performing daily tasks.

Treatment options for early stages of ALS

Currently, no treatment options offer a cure for ALS progression stages; however, in order to manage symptoms, patients undergo the following:

• Medications
• Physical therapy
• Speech therapy
• Nutritional therapy
• Assistive devices
• Stem cell therapy

Stem cell therapy is known as a treatment option to help relieve disease symptoms without any side effects, unlike most medications. The treatment outcomes can be enhanced by combining stem therapy with other interventions, such as physical therapy and assistive technology.

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The middle stage of ALS

The middle stage of ALS involves both the second and third regions, leading to paralysis of most of the voluntary muscles, including the lungs. Most patients and families fail to notice the early symptoms of ALS; however, as Lou Gehrig’s disease stages advance to the middle stage, the symptoms become severe and much more noticeable. 

Symptoms of the middle stage

Middle-stage ALS symptoms can include:

• Difficulty breathing 
• Risk of respiratory failure
• Speech disorientation
• Difficulty swallowing and eating, leading to a greater risk of choking
• Contracture 
• Restricted mobility
• Fatigue
• Chronic headaches

Treatment options for middle-stage ALS

ALS progression stages such as the middle stage can be hard to treat; however, some most common options to manage disease symptoms are: 

1. Prescribed medications to help with sleep issues, depression, and pseudobulbar affect.
2. Nutrition therapy to help with nutritional issues.
3. Speech therapy to address speech and swallowing issues.
4. Respiratory support, such as ventilation for improved breathing.
5. Mobility aids like wheelchairs to support independence.
6. Physical therapy to promote the range of motion.
7. Palliative care to improve patient’s life quality.

However, these treatment options only help manage symptoms and improve them for a better quality of life.

Late stage of ALS

This is the sixth stage among the 7 stages of ALS, commonly referred to as the end stage. Most of the patient’s functions are impaired at this stage, and any hope of managing disease symptoms is little to none.

Symptoms of the late stage 

Symptoms of the late stage can vary from person to person, and not all patients experience the same symptoms. However, as ALS reaches its end stage, the body’s functions start to deteriorate exponentially, impacting daily life greatly. 

During the late stages of ALS, a patient may experience:

• Muscle paralysis and atrophy
• Respiratory failure
• Limited to no communication
• Swallowing difficulties
• Emotional and cognitive degeneration
• Severe pain and discomfort

How long does the end-stage of ALS last?

The end stage of ALS can last from weeks to several months, depending upon the rate of disease progression and the patient’s health condition.

Treatment option for the late stage

At the late stage of the disease, most treatments entirely focus on managing symptoms and improving quality of life. During the last ALS progression stages, only a few options are left to ease a patient’s life. For instance:

1. Non-invasive ventilation to improve breathing.
2. Percutaneous endoscopic gastrostomy for medication and nutrition.
3. Speech therapy to aid communication.
4. Physical therapy to promote strength and mobility.
5. Medications to mitigate cramps and spasticity.

Palliative care and end-of-life considerations

Early palliative care has been found in studies to help improve and/or increase the patient’s quality of life. It includes managing symptoms, along with emotional, psychological, and spiritual needs. Palliative care for ALS patients doesn’t necessarily have to be applied at the end stage; patients can receive it at all stages of ALS progression. 

Some patients might consider the end of life due to the emotional and physical pain and discomfort they experience during late ALS progression stages. To ensure that patient receives comfortable, dignified, and compassionate care during their last days, it is critical to work with professionals specializing in end-of-life care.

Stem cell research for ALS treatment 

Stem cell research for ALS has shown promising results in improving symptoms by replacing damaged nerve cells and promoting the survival of the existing ones.

A study on Lou Gehrig’s disease stages published in 2021 reported slow depreciation of motor neurons among subjects injected with multiple doses of mesenchymal stem cells along with intense physical rehabilitation. The therapy significantly slows down disease progression among ALS patients. Furthermore, the procedure was safe, well-tolerated, and effective in managing disease symptoms. 

What results can be expected?

Stem cell treatment outcomes can:

1. Provide a nurturing and neuroprotective microenvironment to motor neurons by secreting neurotrophic factors, which stimulate the growth of new neurons and prevent damage to healthy ones.
2. Improve muscle functions by healing damaged muscle tissues and stimulating muscle growth, which increases the quality of life.
3. Reduce nervous system inflammations by inducing anti-inflammatory effects, which in turn slow down ALS progression.

According to the clinical experience, during the first 3 months of cell-based therapy, the patient observes the following improvements in quality of life:

• Increased stamina and improved energy levels;
• Improved coordination;
• Increased muscle strength.

Importance of early diagnosis and management of ALS symptoms

Early diagnosis and management of ALS symptoms are critical for many reasons, for instance:

1. It helps patients access treatments that can improve life quality and prevent symptoms from worsening.
2. Early management of symptoms can help preserve muscle functions by reducing further muscle atrophy.
3. Patients can prevent respiratory failure by getting treatment at an early stage, and thus increase their life expectancy.
4. It helps patients and their families maintain better emotional and psychological care throughout the treatment.
5. Early diagnosis also helps improve the accuracy of prognosis and adopt an individualized treatment plan that caters to the patient’s specific needs.

Conclusion

Amyotrophic lateral sclerosis is a rare but hard-to-treat neurodegenerative disease that may lead to disability fast. Mesenchymal stem cells have shown promising results in delaying ALS onset and progression by slowing the loss of motor neurons and reducing neuroinflammation. When combined with physical therapy, stem treatment significantly improves the patient`s motor and sensory functions. If you want to know more about what the results of cell-based therapy may be achieved for your case of the disease, please feel free to contact us.

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Get a free online consultation to learn about the expected results of the treatment based on stem cells, as well as its cost and duration.

Dr. Aleksandra Fetyukhina, MD

Medical Advisor, Swiss Medica doctor

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Natalia Semeshina Author

Dr. Aleksandra Fetyukhina, MD Reviewer

Medical Advisor, Swiss Medica doctor