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.

Dysphagia & Managing Oral Secretions

As residents near the end of life, one of the signs most often encountered is the loss of the ability to swallow.  This article will look at how to manage this problem.

How It Begins

It often begins before the resident is actively dying with dysphagia, or trouble swallowing, usually caused by weakness and poor neurologic function.  The resident may cough, clear his throat, or sputter while eating or drinking.  Thickening fluids may help to start with, since thicker liquids are less likely to pass into the trachea which can lead to aspiration pneumonia.

Preventing Pneumonia

Later, even making fluids thicker is not enough.  The gag reflex and reflexive clearing of the throat decline.  It is probably time to stop feeding the resident at this point to prevent pneumonia.  Usually this comes at the same time as loss of appetite; the resident does not experience hunger any longer and though feedings stop, it does not cause distress.  It is important to take the time to explain this process to the family.

Noisy Breathing

As the resident becomes less aware and it is clear that death is nearing, those at the bedside may hear a rattling, gurgling, crackling noise with each breath.  This is caused by the build-up of secretions in the throat; the resident cannot swallow them the way most people normally do.  This noise is sometimes called a “death rattle,” (although this term should never be used around families and caregivers).

Medications

These noisy respirations can be very disturbing to family and caregivers.  It may be helpful to try to dry the secretions by using an anticholinergic medication such as atropine or scopolamine.

 
Atropine drops (normally used in the eye) may be ordered for administration under the tongue and scopolamine patches (often used to prevent motion sickness) can be applied behind the ear.  Both seem to work equally as well, and neither affects survival time.  These drugs can be used in the unconscious dying patient before noisy breathing begins to prevent it from happening.

Non-Drug Treatments

Repositioning the resident can help to clear the secretions.  Turning the resident far to one side and then the other (to a semi-prone position) may allow the secretions to drain through the mouth (be sure to have a towel ready for drainage).  Raising the foot of the bed very briefly while on the side may also help, but never leave the resident in this position for more than two or three minutes at the most.

Suctioning

Oropharyngeal suctioning is not recommended.  Suctioning can be very distressing to the resident and family, and it is often ineffective since the secretions are usually beyond the reach of the suction catheter.  Suctioning can also stimulate more secretions.