So often we associate caregiving with Alzheimer’s and other dementias, but there are a variety of other diseases, disorders and disabilities that necessitate a family member to assume the role of caregiver. When that happens, it is helpful to learn about the illnesses that afflict our loved ones. Because caregivers are often drawn to other caregivers through social circles or support groups, having a basic grasp of common maladies that require caregivers can prepare us to reach out to others with a sense of knowledge and understanding.

Two weeks ago, I wrote about Parkinson’s disease; yesterday when I was out to lunch with my granddaughter Jada, the daughter of a beloved former patient approached me and after greetings, disclosed, “Ruth, I’ve been diagnosed with Parkinson’s disease.”

I was thankful that I was able to converse meaningfully, and hopefully, encourage her. The more we know, the more effective our care and outreach will be. 

Today we will focus on amyotrophic lateral sclerosis, or ALS.

Where did ALS get its name? By breaking down amyotrophic into the three Greek words from which it comes – a means no; myo means muscle; and trophic means nourishment – we can better understand the process of this disease. When there is no muscle nourishment, the muscle atrophies (wastes away). Lateralrefers to the area of the spinal cord where the nerve cells that signal and control muscles are located. Sclerosis means scarring or hardening and indicates the results of the degeneration that takes place. 

ALS is often referred to as Lou Gehrig’s disease in reference to the famous New York Yankees baseball player who was forced to retire when he was stricken in 1939. As national attention was focused on ALS as Gehrig struggled with and succumbed to the disease in 1941, baseball fans and the public thought it was an appropriate designation. 

What is ALS? ALS is a progressive disease of the nervous system that affects nerve cells in the brain and spinal cord, resulting in the loss of muscle control. It belongs to a wider group of disorders known as motor neuron diseases. 

As the nerve cells of the brain and spinal cord gradually deteriorate the muscles are deprived are nourishment and become smaller and weaker. Eventually, the ability to stand, walk or use hands and arms is compromised. Swallowing and breathing become difficult. Cognitive functions usually – but not always – remain intact. It is rare for people with ALS to experience pain.

Atrophy spreads throughout the body as the disease progresses. An ALS diagnosis is made when a patient exhibits both upper motor neuron damage (abnormal reflexes, spasticity, muscle stiffness) and lower motor neuron damage (muscle atrophy, cramps, twitches) that have no other known causes.

ALS is classified by the parts of the body in which early symptoms are first experienced. Limb onset refers to symptoms such as loss of manual dexterity in a hand or arm or awkwardness, tripping or stumbling when walking or running. Bulbar onset refers to slurred or nasal speech or difficulty chewing or swallowing. 

Eventually, the muscles no longer work and the body is paralyzed. Senses remain intact as the nerves that allow these processes are not affected by ALS.

What are symptoms and signs? These vary greatly and depend upon which neurons (the specialized cells that transmit nerve impulses) are affected. More than half of people who are diagnosed have reported muscle weakness, especially in the arms and legs. As a result, common early signs include difficulty walking, tripping and falling.  Other signs are a change in the ability to perform normal activities of daily living; weakness in legs, feet, ankles or hands; and unusual clumsiness. Signs usually appear first in the hands, feet or limbs before spreading to other parts of the body. As other parts of the body are affected and weaken, swallowing, speaking and breathing are affected. 

Some people experience muscle cramps and twitching in their arms, shoulders and tongue. Others demonstrate inappropriate crying, laughing or yawning. 

Research indicates that about half of all people with ALS will not experience changes in cognitive ability or behavior. Those who do may only have mild symptoms, but some patients develop dementia.

How do age and gender play a role? The incidence of ALS is two per 100,000 people. It is considered fairly rare as only about 5,000 new cases are diagnosed each year in our country. ALS is 20 percent more common in males than females, but as with other diseases, because women live longer, the ratio eventually evens out. Most diagnoses are made between the ages of 40 and 70. 

It is estimated that about 20,000 Americans are currently living with ALS. (That compares with about 5.8 million who have Alzheimer’s; one million with Parkinson’s and 6.4 million stroke survivors.) 

What are risk factors? Many people consider ALS an inherited disease. Actually, Familial ALS accounts for only 5 to 10 percent of all cases diagnosed in the U.S. There is, however, a 50 percent chance that children will inherit the gene mutation and may develop the disease. 

As mentioned above, getting older is a risk factor.

Smoking is considered the main environmental risk factor, which is greatest for women, especially after menopause. Although much research has been done, there is some evidence but none conclusive that exposure to lead or some other agent or chemical in the workplace or at home may be associated.

Military veterans have twice as many diagnoses as those who did not serve; why this is so is a question that has not yet been answered.

What are treatments? While there is currently no way to prevent or cure ALS,  scientists are making progress in learning about this disease. 

Medicines are utilized to control symptoms and may slow the development of the disease. Physical therapy addresses breathing problems and muscle weakness. Medical equipment such as a power wheelchair can assist in mobility and a breathing machine such as a CPAP may be utilized for breathing problems. In

advanced stages, a tracheostomy (surgically created hole leading to the windpipe) may be considered so a respirator can assist with breathing. A feeding tube is an option when the ability to swallow is lost. This helps to ensure hydration and nutrition and prevent aspiration pneumonia which may occur if good, liquids or saliva enter the lungs.

As most people with ALS do not lose normal cognitive function, it is important to remember to treat them as normally as possible. Seeing a loved one lose the ability to function normally can be overwhelming for a caregiving and counseling is often recommended. Developing a new way to communicate may be a challenge but is worth the effort.

As I completed the above, I remembered a column I had written several years ago after a friend’s husband was diagnosed with ALS and she suddenly found herself caregiver for the previously healthy, active and young (50’s) love of her life. I am including here, especially for those who have a spouse that has been diagnosed with ALS.

Because ALS is more prevalent in people (especially men) between 40 and 70 years of age, spouses frequently become the in-home caregiver. These situations present additional challenges, as it is not only physically difficult, but also emotionally draining to watch as your loved one becomes dependent upon you. As in all caregiver situations, it is imperative that you take care of yourself, be willing to ask for help, connect with other caregivers, and educate yourself about the disease process.

As a spouse/caregiver, emphasis should be placed on maintaining the marital relationship and when possible, separate it from your role as caregiver. Try to see yourself first as a wife/husband and continue to celebrate that unique bond.

A bright side to ALS is that this disease does not usually affect cognitive function. Your loved one’s personality should remain intact. That is not to say that both of you will not experience times of despondency, but unlike the changes that take place in dementia, your loved one will continue to be the same person you have always known. According to the Net of Care website, the senses of those with ALS will remain intact, and pleasurable activities can continue to be experienced through taste, touch, smell, sight and hearing. Knowing this should encourage you to provide a favorite meal with candlelight and soft music; a movie night featuring a beloved film from days gone by; or a gentle massage which will not only help relieve cramps but will also enhance relaxation.

It is important that a patient with ALS has a health care provider who is experienced with this disease and can give you guidance concerning all available treatments, including non-drug therapies. Both physical and occupational therapists provide exercises as well as special equipment that can facilitate movement and ambulation. A nutritionist can help you plan nourishing meals as well as a schedule – one that includes smaller meals throughout the day – that better meets your loved one’s needs, especially if his appetite is poor. Speech therapists provide helpful strategies when communication is hampered by slow or slurred speech. 

A common troublesome symptom is episodic breathing difficulty. When this occurs, encourage deep breathing. Providing fresh air from a window or fan may help. Note what brings on these instances – such as strong smells or breathing cold air – and try to avoid them. Increases in duration, severity or frequency, should be reported to the doctor.

Muscle cramping and twitching are common and may interfere with the ability to relax. This can be addressed by assisting with recommended stretching exercises, encouraging liquids, and asking about applicable medications. 

You may find it helpful to research various relaxation techniques and employ these to foster calm and reduce stress. Never underestimate the power of pleasant activities to distract from the bothersome effects of ALS symptoms.  Being alone with nothing interesting to do provides the perfect atmosphere for dwelling on the negative. 

Learning all you can about ALS and using this knowledge will help improve the quality of life for you and your loved one as you battle ALS together.