ALS prognosis

ALS Prognosis

Amyotrophic Lateral Sclerosis (ALS) is a rare, neurodegenerative disease that causes the death of nerve cells in the body. According to available statistics related to ALS incidence and prevalence, it’s estimated that there are currently around 450,000 people currently living with the disease worldwide.

The progression of ALS can be rapid and typically an individual diagnosed with the disease will live for between 3 and 5 years following their diagnosis. However, there are occasionally cases of people, such as the late physicist Stephen Hawking, who have lived many years with the disease.

Here we take a closer look at the prognosis for ALS and what those diagnosed with this condition can expect.

Is ALS Fatal?

The progressive nature of the disease means that the overall prognosis is poor and only half of those diagnosed with the condition live beyond three years following their diagnosis. Symptoms associated with ALS tend to develop rapidly.

While initial symptoms can be managed by the individual, once the disease starts to significantly affect respiration and the ability to eat, full-time medical care is required.

A typical ALS progression timeline takes place over three stages:

  • Early-stage ALS: This is characterized by muscle weakness which may, for example, make the individual drop things or have difficulty with routine tasks such as doing up shirt buttons. It usually starts in a specific area of the body such as the hands.
  • Middle-stage ALS: The muscle weakness starts to spread throughout the body as more nerves become damaged and begin to die. Simple acts like swallowing and speaking can become difficult as the disease progresses.
  • Late-stage ALS: Almost all ability to move muscles voluntarily are impaired. Muscles for breathing and swallowing, for example, will become damaged and the process of eating may have to be aided with a feeding tube.

It’s at this final stage that the disease becomes fatal. The most common cause of death is respiratory failure where the nerves to the lungs are damaged to such an extent the individual can no longer breathe.

How Long Can you Live with ALS?

The statistics for ALS survival rate are stark. Only 50% live beyond 3 years, 25% beyond five and only 10% survive longer than 10 years. There is currently no cure for the disease and the speed with which symptoms develop can vary considerably from one patient to another.

There is some evidence to suggest that people who develop ALS early in life, before the age of 40, are likely to live longer than those diagnosed later on in their life.

Professor Stephen Hawking
Professor Stephen Hawking

This theory is supported by the example of Stephen Hawking, who was actually diagnosed with ALS in his twenties. The eminent physicist died in 2018, 55 years after his diagnosis.

Those who have a certain genetic component to the disease may also find that the prognosis is bleaker, with many not living 12 months beyond their diagnosis. Certain types of degeneration and muscle weakness can also dictate the course of the disease.

For example, those with a neck weakness are likely to see a shorter path to full paralysis. Studies also suggest that around 14% of people diagnosed with ALS will develop a form of dementia that is again associated with a shorter lifespan.

Is ALS Treatable?

There is currently no cure for ALS, although there are some treatment options that can be used to slow down its progression and alleviate symptoms.

There are currently two drugs that are used in the USA that can help extend ALS life expectancy. These are:

  • Radicava, an antioxidant which is thought to help slow down the degeneration of nerve cells It’s not entirely sure how this works. It may prevent free radicals from having a toxic effect on the nerves which hastens damage. The drug is administered via an IV drip and can have side effects such as headaches and balance problems.
  • Riluzole or Rilutek cuts down the amount of glutamate, a substance which is responsible for carrying chemical messages but which in larger amounts will cause nerve damage. This can produce more severe side effects such as dizzy spells stomach upset.

Pain relief and muscle relaxants are also used, particularly as the disease progresses.

ALS patient tracheostomy
A tracheostomy can help ALS patients with their breathing

Most of the treatment options for ALS revolve around the management of the associated symptoms of the disease. When someone is unable to eat properly, for example, a feeding tube is normally employed to supply nutrition. They will also need a respirator once breathing starts to become impaired.

In the earlier stages of the disease, physical therapy and exercise, as well as the use of hot tubs and hydrotherapy can all help ease symptoms. Diet can be a significant factor in maintaining health in the early and middle stages of ALS.

Continuous Research

The prognosis for ALS is poor. There are a number of factors can either slow or speed up the progression but there is currently no cure for the disease. However, with continuous scientific research into the disease, there is hope that one day a cure can be found.