Caregiver Guide to ALS
What is ALS?
Amyotrophic Lateral Sclerosis (ALS), also called Lou Gehrig’s Disease or Motor Neuron Disease, is a progressive, neuromuscular disease that attacks nerve cells and pathways in the brain and spinal cord.
Motor neurons, among the largest of all nerve cells, reach from the brain to the spinal cord and from the spinal cord to muscles throughout the body. When these motor neurons die, the brain can no longer start and control muscle movement. At this time there is no cure for the disease; however, over the past decade, we have made amazing strides in our understanding of the brain, the nervous system and genetics. Discoveries in each of these areas bring hope to people with ALS (PALS) and their families that some day a cure will be found.
Amyotrophic comes from the Greek language. “A” means no or negative. “Myo” refers to muscle, and “trophic” means nourishment–“no muscle nourishment.” When a muscle receives no nourishment, it atrophies or wastes away. “Lateral” identifies the areas in a person’s spinal cord where portions of the nerve cells that nourish the muscles are located. As this area degenerates, it leads to scarring or hardening (“sclerosis”) in the region.
As more and more nerves and muscles are affected, the person with ALS loses the ability to move, eventually suffering complete paralysis. The muscles used for breathing, speaking and swallowing also become affected. ALS does not affect the person’s mind, senses (feeling, tasting smelling etc.), bladder and bowel function or sexual drive and function.
Who gets ALS?
There are 30,000 people living with ALS in the United States and approximately 6,000 Americans are newly diagnosed with ALS each year. Symptoms usually appear in individuals between the ages of 45-65, though the disease has been reported in both younger and older persons. Survival after the confirming diagnosis is, on average, two to five years.
The progression of ALS varies with each individual. Ten percent of those diagnosed with ALS live for ten years or more. Family caregivers, physicians, nurses, physical therapists, speech therapists/pathologists, occupational therapists and social workers all working together with the person with ALS can help ensure a high quality of life and as much independence as possible.
ALS occurs more frequently in men than in women. For 90-95% of all ALS cases, there is no known cause. These cases, which appear to occur at random, are referred to as sporadic ALS. Between 5-10% of ALS cases are inherited. This genetic form of ALS is known as familial ALS. Sporadic and familial ALS do not differ in symptoms or progression. ALS is not contagious.
Some patients become aware of the onset of the disease when their hands become clumsy, causing difficulty in the performance of tasks like unlocking doors or writing. Others experience weakness in the legs and may trip or stumble. Other people notice they have problems speaking or difficulty swallowing.
ALS may be present for some time before any symptoms are noticed. This lack of noticeable symptoms occurs because the remaining functioning nerve cells compensate for the lost or damaged nerve cells. One early symptom is generalized fatigue. As muscle cells deteriorate, patients may experience stiffness, occasional jerking of the arms or legs, or twitching (fasciculations). Often symptoms begin in the hands and feet, then travel inward toward the center of the body. One side is usually more affected than the other. Eventually paralysis may be complete, except for the muscles of the eyes.
There is no specific clinical test that can identify ALS. Diagnosis is made by a neurologist through a physical examination, a thorough patient medical history, and neurological testing. Diagnostic testing often includes the electromyogram (EMG) to test muscle activity, CT Scan or MRI (Magnetic Resonance Imaging), and extensive blood work. Sometimes muscle and/or nerve biopsies are performed.
The diagnostic process involves ruling out other potential causes of the symptoms the person is exhibiting. Because there is no conclusive test, people may find themselves with a diagnosis of probable or possible ALS until further identifying symptoms appear.
A diagnosis of ALS can be devastating to both the individual and the family. Anger, denial, fear, and a sense of loss are common reactions. For those who have been through a lengthy diagnosis process and the anguish of not having a diagnosis, finally knowing that they have ALS may actually bring a feeling of relief.
Treatment and Management of ALS
The Physician’s Role
The American Academy of Neurology has established guidelines for physicians treating individuals with ALS. These are based upon the following four principles:
Patient self-determination and autonomy are priorities; however, the way in which care and information are delivered to the person with ALS and/or his or her family should take into account the social, psychological and cultural background of the family. For example, in some instances the patient may prefer not to know the diagnosis and/or prognosis and the family may appoint someone else as medical decision-maker.
Physicians should provide the family with information well in advance of the time that decisions will have to be made regarding treatment options. Everyone involved should understand that decisions may change over time.
Caring for a person with ALS requires a coordinated effort among many people including family caregivers, therapists, nurses, and physicians. The physician should be responsible for ensuring the coordination of care from the time of diagnosis to the last stages of the disease.
A Living Will (Advance Directives, Power of Attorney for Healthcare) defining the treatment preferences of the person with ALS can be an excellent tool for communication among the person with ALS, the family, and the medical care team. The Living Will document should be reviewed every six months to reflect possible changes in preferences. This can often be a difficult topic and the physician should help by offering the family a chance to talk about it and to review it. In preparation, the physician should provide the family with information about the terminal phases of the disease.
You can create, save and print out a legal Living Will/Health Care Directive simply and inexpensively online at LawDepot. You can also log back in to make changes as necessary. This service is available to U.S., Canadian, UK and Australian citizens.
See a Sample Health Care Directive
Helping people with ALS and their families requires empathy and sensitivity on the part of everyone involved.
In 1995, the FDA approved riluzole (Rilutek) the first and only drug for the treatment of ALS. Clinical trails of riluzole showed that the drug had a modest effect (2-3 months) on slowing the progression of ALS. Researchers continue to conduct clinical trails of potential new treatments for ALS. People with ALS (PALS) may want to discuss with their physician the option of participating in a clinical trial.
Approximately 50% of those affected by ALS may exhibit uncontrollable laughter or crying. This is referred to as psuedobulbar affect. Depression can also be a symptom. Some PALS experience Sialorrhea, drooling. All of these symptoms (depression, uncontrollable laughing/crying, and drooling) can be treated with medications and should be brought to the attention of the physician.
Living with ALS
While it is true that there is no cure for ALS, much can be done to help the person live with the disease. Treatment aimed at relieving symptoms can be very effective.
Generally, people with ALS should continue their usual daily activities, stopping before they become fatigued. They should be encouraged to set their own limits of exertion, and to plan how they will use their energy and strength. The physician will probably suggest exercises, including breathing exercises, to strengthen unaffected or less-affected muscles. These exercises are not vigorous or tiring, but are intended to help maintain mobility and prevent joint stiffness and muscle contracture.
There are a wide variety of assistive devices for enhancing the quality of life for the person with ALS:
By working with physical and occupational therapists, PALS and their caregivers can identify appropriate devices to help maximize independence and reduce caregiver strain as changes in mobility occur. Braces, canes and walkers can help PALS maintain independent mobility for as long as possible.
As the disease progresses, wheelchairs, lifts and other special equipment can enable PALS and their caregivers to work as a team to keep the person with ALS connected to the world around them and to ensure appropriate care.
A speech therapist can help PALS maintain their speaking ability for as long as possible; however, the loss of speech does not mean the loss of the ability to communicate. Assistive communication devices, from simple boards to sophisticated electronic devices and computer applications, permit communication even into the latest stages of ALS.
For more communication help, see Caregivers Can Help With Aphasia
Rapid advances in technology have resulted in products that dramatically increase the independence of people with very limited mobility, allowing them to “speak,” operate lights and other controls, and remain contributing members of their families and communities. As of January 2001, Medicare policy was changed to include coverage of many of these communication devices.
As muscles weaken, breathing becomes more difficult and less effective in the person with ALS. Individuals may find that they have problems sleeping, wake up feeling tired or wake up with a headache. Several options exist for individuals whose breathing becomes inadequate.
Several non-invasive options for helping breathing exist. One of the most frequently used is called a BiPAP (biphasic positive airway pressure). The person with ALS wears a mask over his or her nose at night connected to the BiPAP, which helps increase the flow of air into and out of the lungs.
When the person requires more help breathing than can be offered with any of the noninvasive methods, a permanent ventilator can be used. The ventilator then does the person’s breathing for him or her. This option can prolong the life of a person with ALS but it increases the need for care and the cost of care. The willingness and availability of caregivers can impact this decision.
Deciding whether or not to use the various types of assistive breathing devices will be one of the hardest decisions PALS and their families need to make. Preferences regarding breathing support should be discussed with the physician and included in the Advanced Directives.
It is important for people with ALS to receive proper nutrition and to maintain a normal weight. As problems with swallowing develop, food can be processed to make it easier to swallow. A nutritionist or registered dietitian and speech therapist or speech pathologist can help develop strategies to ensure that the person is receiving enough nutrients, calories and fluids.
If the person is not getting enough food, or if choking and food inhalation become a problem, he or she may want to consider a feeding tube. The tube, referred to as a PEG, is surgically inserted into the person’s stomach. Food goes through the tube and enters the stomach directly. This can be another very difficult decision for PALS and their families. Preferences regarding the placement of a PEG should be discussed before the need arises and included in the Advanced Directives. As with all treatment decisions, they should be reviewed periodically.
For more help, see End of Life Feeding and Nutrition
Gathering the appropriate resources to meet the challenges of ALS can be financially difficult for many families. Both the Amyotrophic Lateral Sclerosis Association (ALSA) and the Muscular Dystrophy Association (MDA) have programs through which people with ALS can borrow assistive devices and equipment that they might otherwise not be able to afford.
It is important that the person with ALS and his or her primary caregiver get the information and support they need. Financial, emotional and caregiving challenges can take their toll on PALS and their caregivers. Preparing for upcoming changes, establishing good communication with the physician and other medical team members, and pulling together a supportive community of family, friends, and social service professionals will help ensure the highest possible quality of life.
While the care needs of PALS can be intense, caregivers must also be aware of their own needs for respite, rest and emotional support. A diagnosis of ALS does not mean an end to enjoyment. PALS and their caregivers will find that they continue to laugh, to find joy in their lives and to gain strength from the love of their families and friends.
Amyotrophic Lateral Sclerosis – A Guide for Patients and Families by Hiroshi Mitsumoto, MD (Third Edition)
This extensively revised and rewritten new edition of the bestselling Amyotrophic Lateral Sclerosis: A Guide For Patients and Families addresses all of those needs, and brings up-to-date important information to those living with the reality of ALS.
The book is completely revised throughout and contains NEW information on:
- Recently developed approaches to treating ALS symptoms
- Use of non-invasive ventilators
- Multidisciplinary team care
- New guidelines being developed by the American Academy of Neurology for patients with ALS
- The use of riluzole (Rilutek) to treat ALS
Amyotrophic Lateral Sclerosis covers every aspect of the management of ALS, from clinical features of the disease, to diagnosis, to an overview of symptom management. Major sections deal with medical and rehabilitative management, living with ALS, managing advanced disease, end-of-life issues, and resources that can provide support and assistance in this time of need.
Don’t Buy Too Many Green Bananas – Living With ALS by Delores M. Warner
This memoir was from a journal the author kept regarding the progression of ALS (Lou Gehrig’s Disease) as her husband fought this devastating disease.
She and her husband were problem solvers, and as she cared for him at home, the two of them worked out ways to compensate for his declining strength. If you know someone who has been diagnosed with ALS, they and their families will find this book provides information on resources and approaches to dealing with the physical aspects, in the story as well as the back of the book for quick reference. As well, the author writes an unflinching account providing insights into coping with such diagnosis with grace.
I Remember Running – The Year I Got Everything I Ever Wanted – and ALS by Darcy Wakefield
A little over a year ago, Darcy Wakefield was a single, 33-year-old, athletic, workaholic English professor, a vegetarian who had never had a serious health problem or injury.
Then she was diagnosed with ALS, and her world turned upside down.
I Remember Running is Darcy’s story of change and loss and challenges during her first year with ALS, as she struggles to make sense of her diagnosis and redefine herself in the face of this terminal illness.
With unflagging courage, wit, and eloquence, Darcy shares what she calls her “fast-forward” life, a life in which she applies for disability, leaves her job, and plans her own funeral as well as meets and moves in with her true love, buys a house, and gives birth to her first child in less time than it takes most of us to accomplish even one of these things.
Beautifully written and wholly inspiring, I Remember Running proves that it is possible to live a rich, meaningful life after being diagnosed with a terminal illness. This book will move readers to see the world in a different light.
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