Amyotrophic lateral sclerosis (ALS) is also known as Lou Gehrig’s disease. More than 50% of individuals diagnosed with ALS live longer than three years after they are diagnosed, although the average life expectancy is 2 to 5 years.
This guide will help you understand more about ALS if you have a family member or friend who is affected by the disease. You will learn more about the care needs associated with the disease as well as the treatment and long-term care for ALS options to meet these needs. Financial and legal concerns for ALS patients will also be discussed.
What Is Amyotrophic Lateral Sclerosis?
ALS is a progressive disease of the nervous system in which nerve cells to muscles begin to die, causing muscles to become weak and atrophy. The cause of the disease is not known, although there appears to be a genetic factor for some individuals. Currently, there is no cure.
ALS does not usually affect a person’s ability to think or communicate with others until they are no longer able to talk. The disease usually leads to death because of the failure of the muscles used to breathe.
Signs And Symptoms Of Amyotrophic Lateral Sclerosis
ALS may develop so slowly that symptoms are often not recognized initially until they become more pronounced and problematic.
Some of the early symptoms include:
- Muscle twitches in an arm, leg, shoulder, or the tongue
- Muscle cramps
- Tight and stiff muscles
- Muscle weakness affecting an arm, leg, neck or diaphragm
- Slurred and nasal speech
- Difficulty chewing or swallowing
For many individuals, the first sign of ALS may appear in a hand or arm as they experience difficulty with simple tasks such as buttoning a shirt, writing, or turning a key in a lock.
Sometimes ALS symptoms are more obvious and begin with difficulties using an arm or leg, which is called limb onset. Other individuals begin having difficulties speaking or swallowing, which is known as the bulbar onset.
As the disease progresses, muscle weakness spreads to other muscles of the body leading to problems with speaking, swallowing, moving, and eventually breathing. Choking becomes a great risk for these individuals, particularly as the disease progresses.
Total disability eventually occurs, and an individual needs complete 24-hour care, including assistance with breathing, if this is something that they and their family choose.
Types Of Amyotrophic Lateral Sclerosis
Sporadic ALS refers to cases of the disease in which the cause is unknown. In the U.S., sporadic ALS is by far the most common form of the disease, with 90% to 95% of individuals diagnosed having this form. Individuals with familial ALS have a genetic or hereditary link to the disease in their family.
Although these two forms of the disease were thought to have different causes, researchers now know that genetic mutations are involved in both forms of the disease, making these forms more similar than once thought.
It has also been identified that ALS is twice as common in military personnel as it is in the general population.
Care Needs With Amyotrophic Lateral Sclerosis
As the disease progresses, people with ALS experience complications requiring medical care, including:
Breathing Problems With Amyotrophic Lateral Sclerosis
Some people with advanced ALS choose to have a tracheostomy, a surgically created hole at the front of the neck leading to the windpipe (trachea) to which a ventilator is connected that helps them breathe. As noted earlier, respiratory failure is usually the cause of death for ALS patients.
Speaking Problems With Amyotrophic Lateral Sclerosis
Because muscles of the throat, tongue, and mouth are used in speech, these muscles also become increasingly impaired as the disease progresses, resulting in difficulty speaking. Initially, these difficulties may manifest as a gradual slurring of speech until a person becomes unable to make any vocal sounds at all.
It is estimated that up to 75% of all individuals with ALS will develop significant problems communicating, particularly in the advanced stages of the disease. When this occurs, there are a variety of options that a person may choose from to ensure they are able to communicate with those around them for as long as possible.
Eating Problems With Amyotrophic Lateral Sclerosis
Because of difficulties using the muscles of the throat and mouth for chewing and swallowing, an ALS patient becomes malnourished and may become dehydrated if they are not carefully monitored. The body also uses calories more quickly in ALS, which puts individuals with the disease at an even higher risk of nutritional deficiencies.
As noted earlier, choking also becomes more of a problem as a person has more difficulties chewing and swallowing. Choking also increases one’s risk of aspiration pneumonia as a result of inhaling food or fluid into the lungs. A soft or puréed diet and thickened fluids may be needed until the person can no longer eat safely at which time they may want to consider a feeding tube or parenteral nutrition to help meet their nutritional needs.
Cognitive Problems With Amyotrophic Lateral Sclerosis
Although many individuals with ALS do not develop any cognitive or mental impairment, some people with the disease experience problems with memory and decision-making. In addition, some individuals are eventually diagnosed with a form of dementia called frontotemporal dementia as well.
Treatment Of Amyotrophic Lateral Sclerosis
Because there is no cure for ALS, treatment is aimed at managing symptoms, preventing complications, and making an individual as comfortable as possible to maintain some quality of life.
Members Of The Healthcare Team
Supportive care for those living with ALS is best provided by multidisciplinary teams of health care professionals, including:
- Physical, occupational, and speech-language pathologists
- Social workers
- Respiratory therapists
- Clinical psychologists
- In-home care nurses
- Hospice nurses
These teams are able to design an individualized treatment plan and provide special equipment aimed at keeping an individual as mobile, comfortable, and independent as possible.
Medications For Amyotrophic Lateral Sclerosis
It is thought that riluzole works to decrease damage to motor neurons (motor nerves) by decreasing glutamate levels in the brain and spinal cord. Edaravone has been shown to slow the decline of daily functioning in persons living with ALS, although it is not clear how the drug works.
A doctor may also prescribe other medications to treat other symptoms such as pain, depression, muscle cramping and spasticity, and sleep problems.
Physical Therapy For Amyotrophic Lateral Sclerosis
Despite the decline of muscle size and strength as ALS progresses, a physical therapist may be able to help an individual remain active and maintain muscle strength through gentle exercises such as swimming, walking, and other low-impact activities in the early stages of the disease.
A physical therapist may also be able to recommend various stretching and range of motion (ROM) exercises for relieving muscle cramps and spasticity when these symptoms occur.
A physical therapist will also be able to recommend and teach an individual how to use special equipment, which may be useful in helping them complete daily living tasks once they become weaker. This may include equipment such as wheeled walkers, bath chairs, and other adaptive equipment.
Speech Therapy For Amyotrophic Lateral Sclerosis
As an individual becomes weaker, they will have more difficulty using the muscles of their throat, mouth, and tongue to speak. A speech-language pathologist (SLP) can teach different techniques to help an individual who is having difficulty speaking modify how they speak, so they are able to communicate verbally for as long as possible.
An SLP may also teach an individual how to communicate using computer-based programs and equipment when they are no longer able to speak. Devices known as speech-generating devices (SGDs) can be a vital communication tool to prevent an ALS patients from becoming isolated from those around them and unable to communicate with their care providers.
SGDs are considered to be durable medical equipment (DME) by Medicare and are covered as such under Medicare Part B (outpatient services).
It is important to note that Medicare covers SGDs for individuals for use in their homes which includes various long-term care facilities if this is where a person resides as the disease progresses. However, Medicare does not cover SGDs for individuals in skilled nursing facilities or inpatient facilities.
Assistance With Nutritional Needs For Amyotrophic Lateral Sclerosis
Nutritional support is extremely important for individuals with ALS since their caloric requirements are higher as a result of the disease process. An SLP is able to assess how well an individual uses the muscles of their throat, mouth, and tongue to determine their risk for choking when eating and drinking.
If an SLP determines that an individual is at a high risk of choking, they may make recommendations for a modified diet, or they may recommend other sources of nutritional supplementation rather than eating.
If it is determined that an individual is at high risk for choking but can still eat, it may be necessary to provide them with a modified diet of soft or puréed foods and thickened fluids. Providing smaller, more frequent, calorie-dense meals is extremely important in ALS.
Because swallowing becomes increasingly difficult for a person with ALS, eating may require a significant amount of time and effort, resulting in fatigue. In addition, an individual may also experience a great deal of anxiety about eating if they have already had the experience of choking while eating.
Due to the progressive loss of muscle control over swallowing, an individual and their family will need to decide as soon as possible how much medical intervention they want in order to meet the person’s nutritional needs. When an individual is no longer able to eat, a feeding tube or parenteral nutrition may be options for providing ongoing nutrition.
Assistance With Breathing For Amyotrophic Lateral Sclerosis
As ALS progresses and breathing becomes more difficult, an individual may begin to feel short of breath when more physically active or when lying down or trying to sleep. When this occurs, a doctor may recommend non-invasive ventilation (NIV) to help provide more oxygen, so the individual is able to breathe more easily.
NIV equipment is similar to equipment that is used for sleep apnea and may be used easily and conveniently at home or elsewhere.
When NIV is no longer able to meet an individual’s oxygen and ventilation needs, a doctor may suggest the person consider the option of being put on a ventilator which would perform the task of breathing for them.
To be placed on a ventilator, a breathing tube must be inserted into the windpipe of the throat (the trachea) through the mouth, which is attached to a ventilator. Alternatively, the breathing tube may be inserted into the windpipe (trachea) through a hole that is surgically created in the neck called a tracheostomy.
Although a ventilator may make breathing easier for an individual, they will not be able to speak or move about freely, and the disease will continue to progress. The implications of being placed on a ventilator can significantly affect a person’s quality of life. As a result, this is a decision that an individual and their family should take plenty of time to carefully consider in consultation with one’s doctor.
Long-Term Care Options For Amyotrophic Lateral Sclerosis
According to the Amyotrophic Lateral Sclerosis Association (ALS Association), long-term care “refers to a wide range of settings and services including health care, home care, adult day care, respite care and various living facilities.”
Home Care For Amyotrophic Lateral Sclerosis
Home care refers to a variety of services that enable individuals with ALS to remain in their homes for as long as possible. These include services such as:
- Assistance with personal care, including activities of daily living (ADLs)
- Housekeeping and laundry services
- Meal preparation or delivery
- Assistance with other health care needs
Home care is a good option for individuals who want to remain in their own homes as long as possible. Ideally, an individual with ALS living at home would also have a spouse or family caregiver living with them who may assist them when in-home care staff are not present, particularly as the disease progresses.
Adult Day Care For Amyotrophic Lateral Sclerosis
Adult day care centers are a good option for individuals who have a spouse/partner or family caregiver who works during the day, needs to run errands, or simply needs a break from caregiving. An individual is usually brought to the facility sometime during the day and then returns home with their caregiver in the evening.
Adult day care centers consist of two basic types of adult day care: adult social day care and adult day health care. Adult social day care programs provide clients with an opportunity for socialization and activities geared to their abilities. Meals and some health services are provided. Adult day health care is designed for individuals with more advanced health problems who want assistance with therapy and social services support.
Both of these types of adult day care offer opportunities for socialization while ensuring the individual is kept safe and their health care needs are met.
Respite Care For Amyotrophic Lateral Sclerosis
Respite care provides caregivers with the opportunity to take a break from caregiving to meet their own needs, run errands, or simply have some personal time to themselves to rest and rejuvenate.
Respite care may be provided in the individual’s own home or outside the home in a respite care facility. Depending on the needs of the caregiver, respite care may be provided for a few hours or for a number of days, depending on the need.
Assisted Living Facilities For Amyotrophic Lateral Sclerosis
In assisted-living facilities (ALFs), individuals live in their own apartments as independently as possible. However, care staff are present in the facility 24 hours a day to assist the individual with ADLs and to respond to emergencies as necessary.
ALFs provide services such as meals, laundry, housekeeping, and transportation services. A limited range of health care services is also provided, such as assistance with medication management.
The care staff in an ALF will assess an individual prior to admission to ensure that they are able to provide the care that the person requires. If the care staff determines that an individual’s care needs exceed the care they can provide, they will often make recommendations for a higher-level care facility.
Continuing Care Retirement Communities For Amyotrophic Lateral Sclerosis
Continuing care retirement communities (CCRCs) provide different residential living options and levels of care all on one property. CCRCs typically include options for independent living, assisted living, as well as skilled nursing care and memory care.
The benefit of a CCRC to an individual with ALS is that the person can transition to higher levels of care as the disease progresses without moving to a different care provider. This provides the opportunity for continuity of care since staff get to know the person quite well. Since patients with ALS can become quite anxious as their symptoms progress, this may provide some peace of mind knowing that the care staff understands and can anticipate their needs.
One drawback with these communities is that they can be quite expensive. Costs often include a substantial entry fee as well as a high monthly cost to live in the community. For individuals with the financial means, however, CCRCs may be ideal.
Nursing Homes For Amyotrophic Lateral Sclerosis
When an individual‘s disease has progressed to the point that they cannot or do not want to live at home anymore because they require frequent nursing care, a nursing home may be the best care option. Care staff in a nursing facility consists of licensed nurses, personal care aides, doctors, and usually physical therapists.
When an individual is admitted to a nursing facility, the nursing staff meets with the individual and their family members to identify their care needs and preferences. Based on this information, an individualized care plan is established to help ensure the person’s care needs are met.
As the needs of the person with ALS change, the nursing staff will meet with the individual and their family to re-evaluate the care plan and make any needed changes.
Skilled Nursing Facilities For Amyotrophic Lateral Sclerosis
While similar to nursing homes, skilled nursing facilities (SNFs) offer more specialized medical and nursing care for individuals with complex medical conditions such as ALS. A variety of medical professionals work in SNFs, including physical therapists, occupational therapists, and SLPs, in addition to nurses, social workers, and doctors.
An SNF may be the best option for individuals whose ALS is more advanced, resulting in the need for more highly skilled care. This may include individuals who are having substantial difficulties swallowing or breathing. An SNF is also able to care for patients with ALS who choose to be put on a respirator when they can no longer breathe on their own.
Hospice Care For Amyotrophic Lateral Sclerosis
Hospice care focuses on providing physical, spiritual, and emotional comfort to individuals and their families during end-of-life care.
Hospice care may be provided in an individual’s own home or in a hospice care facility. It is also possible for a hospice care team to make arrangements with other residential long-term care facilities to come into the facility to provide end-of-life care to an individual with ALS.
Some goals of hospice care include quality of life for the dying person as well as effective management of pain and other symptoms while ensuring the individual is kept as comfortable as possible.
A hospice care team usually consists of a variety of professionals and other individuals, including:
- Care aides
- Social workers
Selecting A Long-Term Care Facility For Amyotrophic Lateral Sclerosis
The type of long-term care facility an individual with ALS requires will depend on how advanced their disease has become. However, regardless of the level of care that an individual requires, the ALS Association recommends asking facility staff the following questions when researching an appropriate long-term care facility for a family member or friend with ALS:
- What experience does the facility staff have in caring for individuals with ALS?
- Would it be possible to arrange for transportation to another facility for ALS-specific treatment when needed?
- Does the facility have a case manager who is familiar with ALS who would be assigned to the individual with ALS? – This would be important for the continuity of care and for the specialized knowledge of the disease that the case manager possesses.
- What were the results of their most recent inspections? How are they rated compared to other facilities of the same type? Do they possess any particular accreditation?
- What services are provided and what services are not provided in the facility, and what are the costs of these services?
- What is the monthly cost of living in the facility?
Other important questions to ask include:
- Does the facility allow other service providers, such as physical therapists and SLPs, to come into the facility to provide therapy for residents who request this care if the facility does not provide these services?
- Does the facility allow hospice care teams to come into the facility to provide hospice care to residents making end-of-life decisions?
- What forms of insurance and payment are accepted by the facility?
Effects Of Amyotrophic Lateral Sclerosis On Caregivers
Due to the progressive and devastating effects of ALS on an individual, it is easy for caregivers to become consumed with the seemingly rapid and debilitating changes in the individual with ALS and the accompanying care needs that occur with these changes.
As the person with ALS begins to lose their ability to speak and communicate their needs and preferences, caregivers often become reluctant to leave the individual’s side. This is understandable since the caregiver becomes, in essence, the “voice” and advocates for the person who can no longer speak for themselves.
However, this advocacy role is often mentally challenging and emotionally draining, as well as extremely time-consuming for caregivers. It can be hard for caregivers to pull themselves away for even a few hours in order to meet their own basic needs.
Nevertheless, it is critical for caregivers to ensure they are getting enough rest, eating regular healthy meals, caring for their own health conditions, engaging in some physical activity, and connecting with other individuals for support when needed.
The physical and mental stressors faced by caregivers are compounded by several complex feelings as well, such as sadness, loss, worry, guilt, grief, and anger, to name a few. Because of the complex interplay of these emotions and the thoughts that go with these feelings, caregivers often find it helpful to share their thoughts and feelings with a support group for caregivers of people with ALS.
Support groups for caregivers of individuals with ALS are particularly helpful because of the common shared experiences of the caregivers and professionals who make up these groups. Caregivers often find comfort, encouragement, and strength from others who are going through or who have gone through similar experiences and challenges with a family member or loved one.
Support groups for ALS caregivers are available both in-person and online. The ALS Association in one’s local area can provide recommendations for local support groups. Local support groups may also be found online through the link provided here.
Financial Considerations With Amyotrophic Lateral Sclerosis
ALS often presents financial challenges to an individual and their family members. There are a number of financial resources that may help cover the costs associated with ALS that are outlined below.
It is also recommended that an individual with ALS review any life insurance policies they may have. When reviewing life insurance policies, terms such as “viatical settlements” and/or “accelerated benefits” are important for the person with ALS to take note of since these types of provisions may provide a significant amount of cash to the individual while they are waiting to receive disability benefits.
Some common sources of income for individuals with ALS include:
- Social Security Disability Insurance (SSDI)
- Supplemental Security Income (SSI)
- Medicare Benefits
- Medicare Waiver
- Viatical settlements
- Accelerated benefits
Social Security Disability Insurance
If an individual or their spouse has paid sufficient federal insurance contributions act (FICA) taxes and Medicare taxes while employed and they qualify as having a “disability” according to the Social Security Administration (SSA), they may be eligible for a monthly cash benefit through Social Security Disability Insurance (SSDI). A disabled individual’s spouse and children may also be eligible for a cash benefit under this insurance plan.
Once an applicant is approved for SSDI, there is a five-month waiting period before payments begin.
It is also important to know that the SSA has a program known as “TERI,” which allows applications for SSDI to be expedited for individuals with advanced or terminal health conditions. When applying for SSDI benefits, an individual and their family should inform SSA that the person applying for benefits has ALS, so their application may be processed more quickly than usual.
Supplemental Security Income
Supplemental Security Income (SSI) is a federal income supplement program that provides cash benefits to low-income individuals who are 65 years or older, as well as individuals who are disabled or blind, regardless of an individual’s work history.
Individuals who apply for SSDI are automatically assessed for SSI eligibility as well. For individuals who qualify for SSI, payments may begin before monthly payments of SSDI (if an individual is eligible for SSDI). The person applying for SSI must meet certain eligibility requirements outlined here.
Individuals who are eligible for SSI may also automatically be eligible for Medicaid.
The SSA also has policies which allow SSI claims to be expedited for individuals with ALS so it is important to inform the SSA that the person applying for SSI has ALS at the time the application is submitted.
Medicare is a national health insurance program that is available to individuals who are 65 years and older, as well as disabled individuals who are eligible for SSDI payments. To receive Medicare benefits, an individual under the age of 65 must be eligible for SSDI benefits. As soon as an individual with ALS begins receiving SSDI payments, they are eligible for Medicare.
As noted above, individuals who become disabled must first be recognized as having a “disability” as outlined by the SSA, and then they must wait five months before receiving SSDI cash benefits. In order to receive Medicare, most disabled individuals must receive SSDI cash benefits for 24 months before they are eligible to receive Medicare coverage.
However, individuals with ALS are eligible for Medicare coverage as soon as they begin receiving SSDI cash benefits which eliminates this 24-month waiting period.
Due to the high medical costs associated with managing ALS, the importance of Medicaid planning early when a diagnosis of ALS is received cannot be stressed enough. It is also recommended that an elder law attorney or Medicaid expert be consulted about the best way to organize finances legally in order to qualify for Medicaid when needed.
For more information about Medicaid planning and asset protection, please refer to the guidelines provided here.
A viatical settlement allows an individual who has been diagnosed with a terminal condition to sell their life insurance policy to a viatical settlement company in exchange for a cash lump sum. The viatical settlement company then owns the policy, must pay the ongoing premiums, and collects the death benefit when the person named in the policy dies.
The amount of the cash lump sum is a percentage of the face value of the life insurance policy. This money may be used to pay for anything the person with ALS wishes to purchase. This may include paying for much-needed medical care and supplies.
Accelerated benefits are another type of provision in some life insurance policies that may be included with the policy when it is initially purchased, or in some cases, it may be added to the policy later for an additional cost.
With this option, an individual with a terminal condition is able to access a portion of the death benefit on the policy to use before they die, which may be between 25% to 95% of the payable death benefit. The remaining portion of the death benefit is paid out to beneficiaries named on the policy when the policyholder dies. These funds, also known as accelerated death benefits, may be used to pay for a variety of things, including health care needs.
Legal Considerations With Amyotrophic Lateral Sclerosis
When a person is diagnosed with ALS, a number of important legal decisions should be made as soon as possible while the individual with the disease is still able to participate and make important choices that will affect them going forward.
Designating a power of attorney and clarifying one’s wishes in advance medical directives such as a living will are some of the most important decisions to be made since individuals with ALS will eventually lose the ability to speak for themselves. Any legal documents that have previously been prepared should be reviewed and revised as necessary.
Some legal documents to consider preparing include:
Durable Financial Power of Attorney
A durable financial power of attorney refers to appointing someone to make financial decisions on one’s behalf. The term “durable” means that the decision-making power that is granted is always in effect until the individual who has had the document prepared dies.
The power to make financial decisions is usually effective immediately. However, if an individual does not wish to grant this power until they become incapacitated, they would need to indicate in the document that this is a “springing power” only.
Durable Power Of Attorney For Health Care
A durable power of attorney for health care involves appointing a person (called an agent) to make health care decisions on one’s behalf when an individual is unable to do so for themselves. An individual’s health care preferences may be outlined within the durable power of attorney for health care documents or they may be identified in a living will.
In a living will, an individual outlines specific medical care they do and do not wish to receive in specific circumstances.
A living will is different from durable power of attorney for health care because it does not allow anyone to make decisions on behalf of an individual. Instead, a living will provides guidance to health care personnel about what medical treatment to administer to an individual if they become incapacitated and unable to speak for themselves.
A living will and durable power of attorney for health care may be prepared together to ensure clarification of an individual‘s preferences regarding medical treatment.
The preparation of a will is also important. In a will, an individual appoints an executor who will carry out the directions in the will and pay any debts and taxes owed when the individual dies. The person also needs to identify beneficiaries and how they wish to have their estate, assets, and belongings distributed to these beneficiaries after all debts and taxes have been paid.
A will can help provide certainty that a deceased individual‘s wishes will be carried out. In addition, a will can help reduce the uncertainty for a surviving spouse or partner and family members who may be left to settle a deceased family member’s financial affairs.
Organizational Support For Amyotrophic Lateral Sclerosis
There are a number of organizations which provide information and support for individuals with ALS and their caregivers, including the:
- ALS Association
- ALS Therapy Development Institute
- Les Turner ALS Foundation
- Muscular Dystrophy Association
- National ALS Registry
FAQs About Long-Term Care For ALS
1. A friend of ours has ALS, and we’ve noticed that she seems to cry for no reason at times. Is she maybe depressed, or can you explain what might be happening?
It is also possible that your friend is depressed since depression is common in individuals with terminal conditions such as ALS. In fact, researchers have determined that up to 77% of individuals with a terminal illness develop depression at some point after diagnosis.
Depending on the relationship you have with your friend, it may be helpful to talk with her to try and determine how she is feeling and if she is willing to speak with a mental health professional about her feelings. It may also be helpful to share this information with her doctor, who will be able to assess her for depression and possibly prescribe medication for depression if appropriate.
2. Why is ALS also called Lou Gehrig’s disease?
Lou Gehrig is the name of a famous professional baseball player who played for the New York Yankees. He was diagnosed with the disease in 1939 and was unable to continue playing baseball.
3. Is it possible to recover from ALS?
Although ALS is usually considered a terminal illness, the ALS Association has identified that approximately 20% of individuals diagnosed with the disease live 5 years or longer, and 10% of individuals will live 10 years or longer. This increased life expectancy is attributed to improved management of the disease, including various medications that are being used, both FDA-approved and experimental drugs.
In addition, there are documented cases of what is known as “ALS reversals” in which individuals severely incapacitated by ALS have recovered most of their functioning again, which has been verified by medical doctors. To learn more about how ALS reversals may be possible, please see this link to the ALS Association provided here.
4. Are there ways to slow the progression of the disease?
As noted earlier, Edaravone is a medication approved by the FDA, which appears to slow loss of function and slow disease progression. Researchers and doctors have also determined that a high carbohydrate, high-calorie diet may help slow the progression of ALS. New research has also identified other potential ways to stop the progression of ALS, which are showing a great deal of promise.