Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurodegenerative disease affecting nerve cells that control voluntary muscle movement. Understanding ALS's progression can help patients, caregivers, and families better prepare for the disease's impact. This article will discuss the different 7 stages of ALS, including the general timeframes associated with each stage, using two primary scales: the King’s College staging system and the Milano-Torino (MiToS) system.
Two formal scales help doctors and researchers track ALS progression:
While ALS progression is variable from person to person, these staging systems provide insight into the general duration of each stage.
According to the Muscular Dystrophy Association, ALS can be broken down into four general stages: early stage, middle stage, late stage, and end stage. Here’s a look at what happens in each stage and the estimated duration based on research.
In the early stage, ALS symptoms are usually mild and affect only one region of the body. For about two-thirds of people with ALS, the disease initially appears in the limbs — this is known as limb-onset ALS. Symptoms can include difficulty with fine motor skills, trouble gripping, and balance problems.
The other third of patients experience bulbar-onset ALS, where initial symptoms involve the muscles around the mouth and throat. This can lead to slurred speech, hoarseness, and swallowing difficulties. Additionally, early signs of ALS may include muscle stiffness, twitching, cramping, fatigue, and atrophy (muscle loss).
Most people with ALS retain considerable independence in the early stage, which typically lasts around a year. However, due to diagnostic delays, many patients have already progressed beyond this stage by the time of diagnosis. For those diagnosed in the early stage, establishing a treatment plan and preparing for future stages is key.
As ALS progresses into the middle stage, symptoms become more severe and widespread. Muscles begin to weaken significantly, with some areas potentially becoming completely paralyzed. In this stage, patients experience intensified atrophy and are at increased risk of contractures (shortening of muscles that limit joint movement).
For some patients, pseudobulbar affect may develop, leading to uncontrolled bouts of laughter or crying unrelated to their actual emotions. Weakness in the mouth and throat muscles can cause difficulty with speech, swallowing, and saliva management, raising the risk of choking.
Breathing difficulties often start in the middle stage, especially when lying down, due to weakened chest muscles. At this point, mobility becomes increasingly challenging, and patients may require assistance with daily activities. Many turn to aids like wheelchairs and walkers, and medical interventions like feeding tubes and respirators become necessary.
The middle stage generally lasts from several months to just over a year, depending on individual disease progression.
In the late stage of ALS, most voluntary muscles are paralyzed. By this point, patients lose most or all independent movement. They require support for nearly all day-to-day tasks and often depend on alternative communication methods, as speech may become impossible.
At this stage, breathing difficulties become severe due to weakened chest muscles, often leading to ventilator support. Low oxygen levels resulting from breathing difficulties may contribute to fatigue, mental cloudiness, and frequent headaches.
Typically, patients in the late stage are also unable to eat or drink by mouth, relying solely on a feeding tube for nutrition. The late stage usually lasts around four to seven months. The duration can vary depending on the individual and the specific life-support treatments they receive.
ALS is ultimately fatal, and the end stage of the disease is marked by an almost complete loss of voluntary muscle function. Respiratory failure is the most common cause of death, as breathing becomes impossible without support.
Other potential causes of death include malnutrition due to swallowing issues, lung complications from choking, and heart arrhythmias.
In the end stage, care is generally focused on maximizing patient comfort. Hospice care is a common form of end-of-life support for ALS patients, and it aims to address the physical, emotional, and spiritual needs of both patients and their families. Families are encouraged to plan for end-of-life care early, so their loved ones can receive the support they need during this difficult time.
The end stage typically lasts about three months, though, like the other stages, it can vary depending on the individual.
While the average survival time for ALS patients is around two to five years after symptom onset, a few factors can influence how long each stage lasts. People with limb-onset ALS or those who receive early treatment may progress more slowly through the disease stages. About 20% of patients live for five years or longer, with around 5% living two decades or more.
While no treatment can cure ALS or halt its progression, some therapies can slow functional decline and extend survival by several months. Current treatments focus on managing symptoms to enhance the quality of life and maintain functionality for as long as possible. As such, these treatments may help prolong certain stages of ALS, although the disease will ultimately progress.
ALS progression varies greatly among individuals, making it challenging to predict how long each stage will last. Generally, the early stage lasts about a year, the middle stage spans several months to a year, the late stage lasts four to seven months, and the end stage is about three months. Understanding these stages helps patients and families plan for ALS’s impact and make informed decisions about treatment and care options.
Notably, the Shiney Wellness stem cell therapy for ALS patients has gained attention for its structured and comprehensive approach. Their protocols emphasize rigorous patient selection and follow-up, ensuring that patients receive the highest quality of care while exploring the potential of stem cell therapy interventions.
