- Muscles may be weak and soft or 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.
- The person may need help with some physical tasks, and might benefit from the use of a cane, leg brace or other simple device.
- 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.
- 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 ).
- 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).
- Ask for help; don’t try to do it all alone.
- Give physical assistance with eating, drinking, bathing, toileting, dressing, communicating 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 health care team.
- Most voluntary muscles are paralyzed.
- The ability to move air in and out of the lungs is severely compromised.
- Extremely limited mobility; unable to care for own needs.
- 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.
- 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.
- 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 the caregiver as well as the person with ALS.
- Although this stage is all-consuming for caregivers, surprisingly many report great stability and satisfaction in their daily lives at this later stage of the disease.