Amyotrophic Lateral Sclerosis (ALS)

Stages of ALS

Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual's disease will progress. For example, symptoms may appear gradually over time, or they may occur rapidly and then plateau. The stages and strategies outlined below offer a general idea of the physical progression of ALS, the types of assistance needed as symptoms worsen, and the role caregivers can play.

Early stages

Physical effects

• Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, 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.
• 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.

Assistance needed

• The person may need help with some physical tasks, and might benefit from the use of a cane, leg brace, or other simple device.

Caregivers should:

• Provide assistance when asked.
• Provide emotional support, including the assurance, “We’ll get through this together.”
• Look for helpful ways to adapt the environment for safety and independence.
• Review and update legal, financial, medical, and other affairs, and investigate government and insurance benefits.
• Begin to share the news of the ALS diagnosis with children, relatives, and friends.

Middle stages

Physical effects

• Symptoms become more widespread.
• Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue.
• 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 discontinued.
• Weakness in swallowing muscles may cause choking and 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).

Assistance needed

• Range-of-motion exercises, especially for the shoulders, help keep joints limber.
• Leg braces and hand and wrist splints help extremities remain stretched and in a good position.
• Feeding tubes end the fear of choking and keep weight stable.
• Noninvasive ventilation (i.e., a BiPAP by Philips Respironics) helps compensate for weak breathing, especially at night.
• Adaptive equipment becomes more valuable.
  • Wheelchairs conserve strength, prevent falls, and promote independence.
  • Communication devices enable people to stay part of the conversation.
  • Adaptive eating, writing, and computer tools preserve independence.
  • Shower chairs make hygiene easier and safer.
  • Lift chairs and rising toilet seats help with getting up from a seated position.
  • Transfer boards and mechanical lifts enable safer, less strenuous transferring from one location to another.
• Antidepressants and anti-anxiety medication help bolster coping skills.
• Several drugs help with uncontrolled laughing or crying (pseudobulbar affect).

Caregivers should:

• Ask for help; do not try to do it all alone.
• Give physical assistance with eating, drinking, bathing, toileting, dressing, communication, and mobility.
• Ensure range-of-motion exercises are performed each day.
• Evaluate standing, walking, and swallowing abilities, and make changes to ensure safety.
• Help obtain needed assistive equipment.
• As much as possible, help the person with ALS stay involved in the affairs of daily living, including decision-making and planning.
• Watch for signs of depression and discuss them with the doctor.
• Be aware of caregiver depression and/or anxiety. Discuss it with a doctor and work out coping strategies. Caregivers must keep on top of their own physical health.
• Learn how to operate assistive equipment and perform care tasks from nurses, therapists, and others on the healthcare team.

Late stages

Physical effects

• Most voluntary muscles are paralyzed.
• The ability to move air in and out of the lungs is severely compromised.
• Mobility is extremely limited; needs must be attended to by a caregiver.
• Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia.
• Speech may be no longer possible.
• Eating and drinking by mouth aren’t possible.

Assistance needed

• Power wheelchair, hospital bed, mechanical lift, and switches that enable any moving body part to operate computers, environmental control units, and communication devices.
• Assisted ventilation, either noninvasive or invasive (tracheostomy).
• Feeding tube.
• Urinary catheters aren’t required but can make toileting easier.

Caregivers should:

• Find and train caregiving help.
• Oversee 24-hour care operations.
• Find ways to help the person with ALS stay socially and mentally active.
• Get into a routine that supports themselves as well as the person with ALS.
• Know that although this stage is all-consuming, surprisingly many caregivers report great stability and satisfaction in their daily lives at this later stage of the disease.