Stages of ALS

  • MDA is helping people with ALS live longer, fuller lives.
  • Since its inception, MDA has dedicated almost $325 million to ALS research and health care services.
  • MDA's nationwide network of nearly 200 specialized neuromuscular disease clinics – including 44 designated ALS centers – is the largest in the country.
  • 13,500 ALS patients are registered with MDA and have access to our specialized clinics, staffed by top health professionals skilled in the diagnosis and medical management of ALS.
  • MDA's Neuromuscular Disease Registry is helping optimize clinical outcomes and evaluate best practices in ALS care. It complements the CDC's ATSDR National ALS Registry, which focuses on the causes of ALS.

Early Stages

Muscles

  • Muscles may be weak and soft or stiff, tight and spastic. Muscle cramping and twitching (fasciculations) occurs, as does loss of muscle bulk (atrophy).
  • Symptoms may be limited to a single body region or mild symptoms may affect more than one region.

Physical effects

  • The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking or other minor symptoms.
  • Sometimes this stage occurs before a diagnosis is made.

Middle Stages

Muscles

  • Symptoms become more widespread.
  • Some muscles are paralyzed, while others are weakened or unaffected. Fasciculations may continue.

Physical effects

  • Unused muscles may cause contractures, in which the joints become rigid, painful and sometimes deformed.
  • If a fall occurs, the person may not be able to stand back up alone.
  • Driving is relinquished.
  • Weakness in swallowing muscles may cause choking, and greater difficulty eating and managing saliva.
  • Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down.
  • Some people experience bouts of uncontrolled and inappropriate laughing or crying (pseudobulbar affect). Despite how it seems, the person usually doesn’t feel particularly sad or happy.

Late Stages

Muscles

  • Most voluntary muscles are paralyzed.
  • The muscles that help move air in and out of the lungs are severely compromised.

Physical effects

  • Mobility is extremely limited, and help is needed in caring for most personal needs.
  • Poor respiration may cause fatigue, fuzzy thinking, headaches and susceptibility to pneumonia. (Respiratory insufficiency is a leading cause of death in ALS.)
  • Speech, or eating and drinking by mouth, may not be possible.

At the End

  • The vast majority of deaths in ALS are the result of respiratory failure, a process that progresses slowly over months. Medications can relieve discomfort, anxiety and fear caused by respiratory insufficiency.
  • A common phrase heard from family members is, “It was very peaceful at the end.” Their loved ones with ALS simply “went to sleep and just slipped away.”
  • Far less common causes of death in ALS include malnutrition as a result of swallowing problems, pulmonary embolism (a blockage in one of the arteries of the lungs), abnormalities in the heart’s electrical pacing system called cardiac arrhythmias, and pneumonia as the result of aspiration (when food or fluid gets into the lungs).
  • Hospice care (in a facility or in the home) focuses on providing comfort and quality of life by supporting the physical, emotional and spiritual needs of the individual with ALS and his or her family members. Families should contact hospice early on to see what sorts of helpful in-home services are available even before "the final days" have been reached.
  • At MDA clinics, physicians work closely with palliative care teams to coordinate treatment with in-home hospice care providers, assisted living facilities or inpatient hospice settings. Such cooperation helps ensure the person with ALS has the most peaceful and painless experience possible.