After receiving a diagnosis of a progressive condition like amyotrophic lateral sclerosis (ALS), it’s natural to want to know what to expect.
ALS, also known as Lou Gehrig’s disease, is a progressive neurodegenerative condition affecting motor neurons in the spinal cord. Motor neurons are the nerve cells responsible for muscle control and movement. The more of them you lose, the more severe symptoms of ALS become.
Although ALS has a generally predictable progression, the specific rate of progression and the order of abilities affected may not follow the same pattern for each person. Here’s what to know about the stages of ALS.
Below is a framework of ALS staging that can help you know what to expect.
Early stage ALS can be subtle — so subtle that you may not yet have a diagnosis. A
- muscle weakness
- labored breathing
- difficulty speaking
- trouble swallowing
- muscle twitches
Of all of these, labored breathing was the presenting symptom mostly likely to go the longest without a diagnosis.
Not everyone has such noticeable symptoms in early stage ALS. Other common symptoms include:
- muscle cramps
- tightness or “stiff” muscles
- slurred or pitch changes in speech
Where ALS begins also matters in the early stage. Most cases cause weakness of the limbs (limb-onset ALS) before they affect the “trunk” — the center of the body where internal organs are housed. Once the trunk is affected, a person will begin to experience weakness in breathing.
Bulbar-onset ALS, however, doesn’t begin in the limbs. It starts in the head and neck and can create challenges with chewing and swallowing, for example, much sooner than limb-onset ALS.
Middle stage ALS occurs when early stage symptoms worsen and more areas of your body are affected.
This is the zone between muscle weakness and full paralysis. You may notice some muscles barely work while others are not as severely affected.
Other symptoms can include:
- difficulty chewing or swallowing
- trouble speaking
- weight loss
- muscle cramps
- difficulty breathing
Both early and middle stage ALS can share symptoms. What sets them apart is the level of impairment and how much of the body is affected.
During this stage, some people may undergo their first necessary ALS procedure, like insertion of a feeding tube.
Late stage ALS, or advanced ALS, is characterized by muscle paralysis, major loss of function, and the need for life supporting procedures, like:
At this stage, mobility is extremely limited. You may not have the muscle control to chew or swallow. You might not be able to maintain your posture, walk, or hold up your head.
A 2018 review paper estimates that in
Others consider the end stage as when you can no longer breathe on your own and life expectancy is fewer than 6 months.
King’s clinical staging system
Formal ALS staging models do exist. They were developed to help the diagnostic process. One of the more straightforward frameworks is King’s clinical staging system. It breaks down ALS stages by how much of the body is affected.
King’s stages are:
- Stage 1: first region of symptom onset
- Stage 2A: time of diagnosis
- Stage 2B: second area of the body is affected
- Stage 3: progression to the third area of the body
- Stage 4A: need for gastrostomy
- Stage 4B: approximately 80% to 90% through ALS course; noninvasive ventilation is needed
Other clinical staging models include the Milano-Torino system and the World Federation of Neurology (WFN) ALS staging model.
There’s no clear-cut timeline for ALS progression. You may experience a slow and steady onset of symptoms or go months without any change.
Some people may even notice a temporary improvement in their function throughout the stages of ALS.
In the AI-based
By that time, 16.4% of people were at the point of requiring noninvasive ventilation.
The complete inability to perform necessary functions of life, like eating, breathing, or swallowing, marks the end stage of ALS.
Most voluntary muscles will become paralyzed. There are essentially no tasks a person can complete independently.
However, some people live with ALS for over 10 years after diagnosis.
An ALS diagnosis can be overwhelming and scary. There is no cure, and while treatment can help manage symptoms and possibly slow loss of function, ALS is always fatal.
One of the best ways to support a loved one during this time is by offering your help in the decision making process that comes with a terminal illness.
This involves discussing your loved one’s wishes regarding care, treatment options, financial decisions, and hospice. There may come a point when they cannot advocate for themselves. Knowing their wants can help ensure they’re respected as ALS progresses.
You can also provide support by:
- attending appointments with them
- mediating communication between healthcare professionals, insurance companies, and caretakers
- arranging palliative care or doula services
- monitoring your loved one for signs of pain, discomfort, or mental health challenges
- helping with household chores or errands
- spending companionable time with them talking, listening, reading, etc.
When a loved one has a terminal condition, it’s hard on you, too. Make sure to care for your needs as well.
Self-care is not selfish — it’s one way to help maintain your resiliency during this difficult time.
ALS is a progressive condition caused by the loss of motor neurons in the spinal cord.
Early symptoms of ALS tend to impact one area of the body and cause mild impairment. As ALS progresses, more regions are affected and voluntary muscle control may be entirely lost.
ALS is a terminal illness, but life expectancy varies. Some people live more than a decade after diagnosis. Treatment can help manage symptoms and slow disease progression.
If you or a loved one has received an ALS diagnosis, you can find educational and support resources by visiting: