ALS: Amyotrophic Lateral Sclerosis

What is ALS?

Often called “Lou Gehrig’s Disease,” ALS is a degenerative disease of the nerve cells in the spine and brain.  As the neurons that connect the brain to muscles begin to die, the brain can no longer control muscle movement.  In later stages the patient may be totally paralyzed; for most, their minds are not affected.   

• About 5,600 cases are diagnosed annually; 60% are men, 93% are Caucasian
• Most people are between 40 and 70 years old

Early Symptoms

ALS may start with simple muscle stiffness and can differ a lot from one person to the next.  But 60% begin with muscle weakness.  A person may trip over carpet edges, have trouble lifting, or have slurred speech. They may drop things, have abnormally tired arms and legs, or even uncontrollable crying or laughing.

Later Symptoms

The rate of progression can vary, with an average survival time of 3 to 5 years, but many live 5, 10 or more years.  In a small number of people, ALS stops.  Later symptoms are:

• Muscle weakness in hands, arms, legs or muscles for speech
• Twitching and muscle cramping, especially in hands and feet
• “Thick” speech and difficulty speaking loudly
• Difficulty breathing and swallowing

End Stage ALS

Changes that may mean the death is nearing include a sense of breathlessness or the onset of a lower level of consciousness.   As respirations fail, the resident becomes less and less aware, then unconscious.   Nearly 60% of people with ALS have a sudden rapid decline and die within 24 hours.  Death also seems to happen most often at night when breathing naturally becomes slower and more shallow. 

Pain in ALS

Pain is common in later stages, probably from stiff joints, muscle cramps, or pressure on the skin and joints from immobility.  A combination of anti-inflammatory, anti-spastic and non-narcotic pain relievers may work until later stages when morphine often achieves the best pain relief.

Treating the Whole Person

Since the awareness and thinking usually remain intact, every effort should be made to continue communicating with the resident, even when he or she is too weak to speak.  This may mean using a communication board.

Knowing that death is near can lead an individual to seek resolution of “unfinished business.”  Hospice chaplains can provide spiritual support for the resident and reassure him that his family will have ongoing bereavement support.  Volunteers may supplement visits from family members.   Together we can support all the resident’s needs through the rest of his life.  Families will remember the end-of-life care you give for the rest of their lives.

This article was originally published in Pathways & Partners Newsletter - Issue 25.  To download this issue in PDF format, or past issues, visit our newsletter archives online at www.pathwayshealth.org/publications.