What is ALS: Understanding the Different Stages of ALS

ALS is a brutal neurodegenerative disease (PC: York Rehab Clinic)

ALS is a brutal neurodegenerative disease impacting the complete mobility of the affected individuals with extreme muscle weakness and loss of voluntary movement. The onset of ALS is a gradual process, and manifests as different stages of ALS based on the severity of the disease and the course of treatment. Let’s understand the different stages of ALS in this article and how it impacts the people living with ALS in this article.

Introduction to ALS

What does ALS stand for?

ALS, a progressive degenerative disease stands for Amyotrophic Lateral Sclerosis, severely impacting the mobility and muscle control, deteriorating the ability for elementary activities such as eating, speaking, and breathing.

Decoding “Amyotrophic Lateral Sclerosis” further, Amyotrophic comes from the Greek language, with ‘myo’ referring to muscles, ‘trophic’ meaning nourishment and ‘A’ meaning without, thereby implying ‘no muscle nourishment’. The word ‘Lateral’ refers to the specific part of the spinal cord containing particular nerve cells responsible for signalling and muscle control. With degeneration of this region, the outcome is scarring or hardening of the specific region, known as ‘Sclerosis’.

Understanding the ALS disease

ALS, also known as Lou Gehrig’s disease, has a gradual onset, with the early signs and symptoms mimicking several other neurodegenerative diseases or issues with the muscle, or spine, causing early ALS diagnosis difficult. Generally, ALS diagnosis takes about 12 – 15 months to confirm considering the gap for recovery and process of eliminating other diseases. With the average life span from onset to progressing through different stages of ALS till death is about 3 – 5 years(1). This emphasizes the importance of early ALS diagnosis to manage ALS treatment for better life expectancy.

While determining the exact cause of ALS is a complicated process, it involves genetic mutations and unusual environmental factors leading to sporadic cases of ALS without a clear cause. While the mechanisms are not clear, the occurrence of abnormal protein levels and neuron damage are observed in the progression of ALS disease. The journey of every patient living with ALS is unique, and the pace of progression can vary for each based on the onset, genetic factors and initiation of treatment.

Summarizing different stages of ALS (PC: ALS News Today)

Different stages of ALS

ALS onset may present differently for each  affected individual, and the different stages of ALS progression will also change according to the age, genetics, sex and lifestyle in certain cases. ALS diagnosis is typically confirmed once muscle weakness progresses beyond the more subtle preliminary signs. Usually, the average life expectancy of ALS patients is 2 – 5 years after diagnosis.( 2 )However, with treatment and personalized care tailored to the progression of the different stages of ALS, quality of life can be improved significantly. The different stages can be categorized as early stages, advanced stages and end stages.

The early stages of ALS consist of muscle weakness and twitching. The disease progresses with respiratory and motor impairments in the advanced stages, and ultimately leading to complete loss of motor function and respiratory failure in the end stages. Awareness of the different stages of ALS helps caregivers better understand the condition of the person living with ALS (pALS) and support them with informed care choices.

Let’s take a closer look at the different stages of ALS and get an overview on managing ALS disease better.

Stage 1: Early Muscle Weakness and Twitching

The early stages of ALS have common symptoms indicating muscle weakness and twitching mimicking other diseases. It starts subtly impacting the functioning of arms and legs with difficulty in fine motor tasks such as buttoning shirts. Muscle twitching, cramping, and frequent tripping is also observed in the early stages of ALS.

The people living with ALS can also experience muscle stiffness increasing the discomfort and impacting mobility. Some of them can also have minor difficulty swallowing or slurred speech for patients whose bulbar muscles are affected in the early stages of ALS. While recognizing these early signs as ALS diagnosis is challenging, early intervention with medication and physical therapy can delay the disease progression.

Stage 2: Progressive Muscle Weakness

The second stage of ALS worsens the muscle weakness and the individual affected finds doing the daily tasks hard. The people living with ALS are struggling with movement, balance, coordination and simple tasks to upgrade their difficulty level. The worsening muscle atrophy and losing strength makes mundane muscle tasks such as walking, standing, controlling hand movement difficult, and individuals can experience frequent falls.

The quality of life can be improved by using assisting devices and making it possible to adapt to the changes in mobility. The caregivers need to make care choices considering muscle weakness and atrophy, communication and respiratory issues and decline in physical function.

Stage 3: Intermediate Functional Impairment

Progressing through different stages of ALS, the intermediate functional impairment involves significant challenges. With severe muscle weakness and atrophy, mobility is limited, making walking and standing challenging without support. Further, the swallowing and speaking problems have aggravated with increased risk of choking.

The people living with ALS may also have weakened muscles causing difficulty in breathing, which leads to exhaustion and shortness of breath doing mundane activities. The quality of life can be enhanced with assistance devices paired with medicine and physical therapy for easy management of ALS.

Stage 4: Advanced Functional Decline

This is one of the stages of ALS where the people living with ALS can have independence in mundane and routine activities with assistive devices only. This phase worsens the ability to communicate which can be resolved with using speaking aids. The affected person would require breathing support with aids to manage needs, ensure comfort and safety. The progression of ALS disease can vary from person-to-person based on genetic factors and the age at which the first signs and symptoms appear. When going through the advanced stages of ALS, the assisted devices for mobility, communication and breathing are essential.

Stage 5: Advanced Medical Care

One of the advanced stages of ALS, stage 5 requires definite attention of caregivers with advanced medical care for the person living with ALS to function. Beyond the ALS medication and physical therapy, this phase requires caregivers to render emotional support and psychological tapping to motivate the people living with ALS to fight through the brutal challenges of the disease and continue receiving medical care. At this stage, the patient might require feeding tubes to cope with complete chewing impairments.

Stage 6: Severe Impairment

The sixth stage is the intermediate phase from advanced to end stages of ALS with severe impairment. The people living with ALS can barely move, with extremely minimal voluntary control, impacting the competence for most functions of the body. Eventually, this leads to muscle paralysis and severe breathing issues. This stage could last from a few weeks to months, based on the specific progression of the disease.

The respiratory functioning is critically damaged, swallowing is also impossible, leading to complete dependence on feeding tubes. The people living with ALS might have cognitive function, but complete physical dependence on the caregivers with 24*7 intervention. The objective at this stage is to mentally support the affected patients and improve their quality of life.

Stage 7: End-Stage ALS

The end stage of ALS remarks complete loss of mobility, leading to muscle paralysis and respiratory failure. The people living with ALS can only control eye movements for communication. The patients are completely dependent on life-sustaining support and interventions such as ventilators and feeding tubes. All the stages of ALS lead to the end-stage with life expectancy for ALS patients limited to a few months till complete respiratory failure causes death.

Conclusion

ALS is a brutal neurodegenerative disease impacting the mobility of the affected people, taking away complete voluntary control with the declining progression of the disease. When ALS diagnosis is confirmed, it is crucial to understand the stages of the disease with the current condition for personalized care options. The early stages of ALS cause muscle weakness and twitching. The condition worsens in the advanced stages of ALS with respiratory and motor impairments, eventually leading to complete loss of voluntary functions, respiratory failure and muscle paralysis by the end-stage. The different stages of ALS require personalized intervention based on the gradual decline in health and response to treatment – physical therapy and ALS drugs. As ALS disease progresses further, assisted devices are required for communication, feeding, mobility and ultimately to support respiratory function.