Managing the Symptoms of Amyotrophic Lateral Sclerosis

How to Stay as Comfortable and Independent as Possible Living with ALS

Physical Therapy
Physical Therapy. Stefano Oppo / Photodisc / Getty Images

Amyotrophic lateral sclerosis (ALS) is a terribly debilitating disease. As people with ALS get weaker, many serious, life-threatening changes occur that need to be addressed by a team of medical professionals. In addition, there are changes that are not life-threatening, but nevertheless, have an impact on the day-to-day lives of those with ALS. Addressing these components of ALS can help improve the quality of life for those who suffer from this devastating neurological illness.

Pain and Cramping

By causing neurons in the anterior horn of the spinal cord to degenerate, ALS disconnects the signals sent from the brain to the muscles. In addition to weakness, muscles may feel a cramping sensation. Furthermore, people with ALS may be too weak to make the innumerable small shifts in a position most people ordinarily make to stay comfortable, which can result in significant discomfort and pain.

It's not clear how to best relieve the discomfort that can be associated with ALS. Studies have shown that exercise has some benefit. Simple stretches and physical therapy can help relieve spasticity associated with the disease. Some medications that have been shown to help include baclofen, dantrolene, and tizanidine for muscle spasms. Pain associated with ALS can be treated with the same medications normally used to treat discomfort, such as ibuprofen or acetaminophen, as guided by a physician.

As the disease progresses, stronger opiate-based medications may be needed, but must be used with caution due to their risk for respiratory depression, especially in someone already weakened by their illness.

Cognition

Amyotrophic lateral sclerosis sometimes comes hand-in-hand with dementia. An estimated 15 to 41-percent of ALS patients have cognitive changes, depending on how these changes are tested for.

The most common type of dementia associated with ALS is frontotemporal dementia, with personality changes and less impulse control. There is little consensus on how these dementias may be best managed, but there is certainly no easy cure. Sometimes, apparent dementia may actually be the result of inadequate sleep or depression, so it is important to make sure these factors are being treated. Otherwise, the management of these dementias relies on working with a team that may include a social worker, neurologist, and psychiatrist to manage the patient's behavior. As always, planning ahead is key.

Mood

With all the negative changes that come with a diagnosis of ALS, some depression and anxiety has to be expected. Surprisingly, these feelings tend to be less common among those with ALS than other diseases, such as epilepsy. If these feelings are present, they are best managed with a combination of counseling, therapy, and medications, such as antidepressants.

ALS can cause other changes that mimic depression and anxiety.

For example, what may feel like depression may actually be fatigue due to muscular effort and poor sleep. Furthermore, due to the neurological changes of ALS, patients may suffer from a pseudobulbar affect, also known as involuntary emotional expression disorder, or IEED. This means that their emotions are unstable, and they may find themselves laughing or crying in inappropriate situations. A combination of dextromethorphan and quinidine has been shown to be effective in helping this symptom.

Sleep

Insomnia in ALS can worsen fatigue, mood, and concentration. In ALS, a diminished ability to fall or stay asleep probably results from early respiratory weakness, anxiety, depression or pain. The best approach is to try to treat the underlying problem. For example, adequate respiratory therapy has been shown to often be beneficial to ALS patients who suffer from sleep problems. If possible, sedative or hypnotic medications should be avoided except as a last resort, as these often decrease respiratory drive.

Communication

One of the most distressing aspects of ALS is a loss of the ability to communicate normally. As the muscles of the face and larynx weaken, new ways to communicate will need to be explored. Fortunately, there are a large number of different communication devices available, including alphabet boards, computerized systems, Morse code, utilization of the anal sphincter, and infrared eye movements, depending on the level of the person's need. Working with a speech therapist can ensure that the ability to communicate remains intact for as long as possible.

Utilizing Palliative Care for ALS

Experts in palliative care specialize in keeping patients comfortable. This is not the same thing as hospice, which is a form of palliative care reserved for patients in their last six months of life. As the symptoms of ALS progress, many people benefit from working with specialists in palliative care and hospice to ensure that their comfort and dignity is being maintained as well as possible.

The Importance of Planning Ahead

Part of being comfortable today is knowing that a plan is in place for tomorrow. ALS is a terminal illness, usually leading to death within five years. While some people with ALS live longer, it is critical to plan ahead. Furthermore, an inevitable loss of the normal means of communication makes very clear plans for future health care all the more important. Exactly what kinds of treatments would be desirable under certain conditions, such as complete paralysis and possible dementia, need to be considered and discussed. These are very personal decisions, and it's important that your wishes be respected. Arranging for a living will or power of attorney can help ensure that your wishes are respected.

ALS Resources and Support

If you've recently been diagnosed with ALS or another motor neuron disease, you are not alone. Others have gone through what you are experiencing, and there are many books and other resources available to you. Working with a multidisciplinary neurology clinic is probably best for enhancing the quality of life and optimizing health care delivery. You will also need help from friends, family, and possibly caregivers as your condition progresses. Don't hesitate to reach out to those who want to support you.

Sources:

R. G. Miller, MD, FAAN, C. E. Jackson, MD, FAAN, E. J. Kasarskis, MD, PhD, FAAN, J. D. England, MD, FAAN, D. Forshew, RN, W. Johnston, MD, S. Kalra, MD, J. S. Katz, MD, H. Mitsumoto, MD, FAAN, J. Rosenfeld, MD, PhD, FAAN, C. Shoesmith, MD, BSc, M. J. Strong, MD and S. C. Woolley, PhD. Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology, Neurology, October 13, 2009 vol. 73 no. 15 1218-1226

R. G. Miller, MD, FAAN, C. E. Jackson, MD, FAAN, E. J. Kasarskis, MD, PhD, FAAN, J. D. England, MD, FAAN, D. Forshew, RN, W. Johnston, MD, S. Kalra, MD, J. S. Katz, MD, H. Mitsumoto, MD, FAAN, J. Rosenfeld, MD, PhD, FAAN, C. Shoesmith, MD, BSc, M. J. Strong, MD and S. C. Woolley, PhD. Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology, Neurology October 13, 2009 vol. 73 no. 15 1227-1233

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