Symptom Care and Support

ALS leads to speech problems when it attacks bulbar neurons. These are the nerve cells responsible for bringing messages from the lower parts of the brain (bulbar region) to the muscles that move the lips, tongue, soft palate (back of roof of mouth), jaw, and vocal folds (voice box). As nerves are lost to the disease, the muscles they control become weak and tight. This causes dysarthria – the term used to describe slow, effortful, slurred speech, and breathy or hoarse voice. Weakening lung muscles affect speech as well. Speaking may make you tired, especially later in the day. You may find yourself speaking in shorter sentences and that projecting your voice is difficult.

There is an important distinction between the dysarthria associated with ALS and the dysarthrias resulting from other conditions like a stroke. In ALS the muscles that are receiving signals from the brain must compensate for the muscles that are already weak. This means the functional muscles are doing extra work all the time to compensate and therefore, require more frequent and extended rest. In fact, rest can often help temporarily improve speech. Therapeutic oral exercises designed to strengthen muscles for people with other forms of dysarthria have not been demonstrated to improve speech for people with ALS. The movements required for speaking provide ample exercise. Stretch and massage may be recommended to reduce tightness or maintain range of movement.

Partnering with your Speech Therapist

Beginning work with a speech therapist (i.e. speech language pathologist or SLP) familiar with ALS at the earliest sign of speech or voice changes, and even prior to changes occurring, can be invaluable. As your abilities change, your speech therapist can help you to maintain or improve your communication. A speech therapist will make sure you have the tools and training to achieve each of these seven vital communication competencies throughout the progression of ALS. I can alert people in other rooms or outside the home when I have a need or emergency.

1. I can use strategies that improve communication speed, success, and reduce fatigue (whether using speech or AAC).
2. I can use a low-tech or rapid access communication system.
3. I can produce voiced messages via speech, SGD, or TTS app.
4. I can communicate with people not in my immediate environment (email, text, phone, social media, etc.)
5. My communication partners and I can independently set up, customize, and use all of the elements of my communication system.
6. I can describe a proactive strategy designed to prepare for typical changes in speech and/or computer access that I may experience.

It is essential though that you understand the abilities you can expect to maintain, and alert your therapist when updates in your system are needed.

Adapted from the ALS Functional Communication Scale, Roman 2014

While good nutrition is important for everyone, it can be very challenging for people living with ALS to maintain a healthy diet due to chewing and swallowing difficulties often experienced at some point in the disease progression. Improper nutrition can cause people to feel tired, lower their resistance to infection, speed the loss of muscle mass, cause constipation and lead to a host of other health problems.

A nutritionist or speech therapist can help people living with ALS and their caregivers put together a healthy diet that works to meet each person’s specific needs. In general, though, when supplied with the right education and information, planning and preparing healthy meals the whole family will enjoy can be easy and stress free.

Below are some important facts to know and tips on maintaining good nutrition despite having ALS.

1. Protein provides the building blocks for muscle and many other tissues in your body.

Excluding water, about three-quarters of our body’s solid mass is made up of protein. If you don’t consume enough protein, your body may break down muscle tissue to supply the quantity it needs. While protein is very important for people living with ALS, it can be difficult to consume the necessary amount. Meat, including beef, poultry and fish, eggs, beans, nuts, milk and cheese are all-natural sources of protein.

2. Fiber can help you maintain a healthy weight and improve your digestive health.

Fruits, vegetables, whole-grain breads, and cereals are all good sources of fiber. Unfortunately, fibrous foods can also be hard for some people living with ALS to swallow. To make sure you’re getting enough fiber in your diet, consider eating fruits or vegetables with a soft consistency, such as bananas, canned peaches or cooked beans or applesauce and other fruits that have been pureed.

3. Most people need a half-gallon of water each day, or eight 8-ounce cups of liquid.

Dehydration can be another problem for people with swallowing difficulties, and getting enough water is every bit as important as getting enough food. If you’re having trouble swallowing liquids, you can get the water you need by drinking thick liquids or adding thickening powder to your favorite beverages. Eating food with a high-water content, such as canned fruits or pudding can also be a big help for people living with ALS. People with mobility challenges may limit their liquid intake to decrease trips to the bathroom. Strategies and equipment for easier transfers and independence are available.

4. Getting enough calories daily is critical for people living with ALS.

Getting enough calories to maintain a healthy weight can be challenging for people living with ALS. Though many people think it’s healthy to diet or limit fat intake, for people living with ALS it’s much more important to make sure you’re getting enough calories to fuel your body and prevent it from breaking down muscle tissue. If you quickly feel full or grow tired when eating, try having six to eight small meals each day instead of three large ones. You may also consider a feeding tube to supplement your calorie intake throughout the day.

5. Vitamins and minerals help boost the immune system, support normal growth and development, and help cells and organs do their jobs.

While there’s not yet clear evidence that vitamin supplements can help the body fight ALS, it’s been suggested that vitamin E may be beneficial to people living with ALS, but the evidence is inconclusive. However, it’s still a good idea to consider taking a daily multivitamin and perhaps an additional vitamin E supplement. Some vitamins come in either solid or liquid form, so you can choose whichever is easiest for you to consume.

Additional information regarding maintaining good nutrition while living with ALS can be  obtained from your physician, dietitian or ALS health care team.

Receiving an ALS diagnosis can be overwhelming, accompanied by a long list of tasks to handle. At some stage, the majority of families living iwth ALS find themselves in need of home care services. However, the costs linked to such care can accumulate rapidly. While public insurance programs like Medicare and Medicaid, as well as private insurance, may offer partial coverage for medically based home healthcare, the primary financial responsibility typically falls on the family for the day-to-day assistance. 

Home Care (Non-medical)

What do they do?

Home care services are supportive services that can be arranged for any amount of time needed, up to 24 hours a day, 7 days a week. They can be used on a regular schedule or on a temporary basis for respite.

They can include:

• Bathing and dressing assistance
• Toileting assistance
• Transfer and ambulation assistance
• Medication reminders
• Nutrition management
• Light housekeeping
• Laundry and lines assistance
• Transportation to appointments and errands
• Companionship
• Peace of mind and safety

Who pays?

Home Care is usually paid for out–of-pocket. Some long-term care insurance policies will cover home care, but it is not a benefit of Medicare or most private health insurance plans. Medicaid does have a benefit for home care that you can apply for if you qualify.  Veteran’s programs also cover home care.

Home Health Care (Medical)

What do they do?

Home health services are skilled services provided on an intermittent basis, with scheduled home visits to homebound clients only, under a plan of treatment ordered by a physician. You must have a skilled need (i.e. need a nurse) to qualify. Services can include:

• Nursing Care
• Occupational Therapy
• Physical Therapy
• Speech Therapy
• Respiratory Therapy
• Medical Social Worker
• Following a specific plan of care, and under the supervision of a skilled discipline such as a nurse or a therapist, a certified home health aide may provide personal care for a limited time.

Who pays?

Home health care services are covered by Medicare, Medicaid and private insurance.

Understanding Palliative Care

Palliative Care is a specialized medicine focusing on symptom management with people who have a terminal or chronic illness. Palliative care is available to a person at any stage of an illness, like ALS, when symptoms require more regular intervention than a typical doctor’s appointment.  Symptoms managed may be pain, anxiety, shortness of breath, fatigue, constipation, nausea and any symptom causing distress. This specific type of care is available at someone’s home where the team comes to the patient.  The philosophy allows for symptoms to be managed at home, with the care and recommendations being available without having to leave the home. This can provide security of knowing a medical professional is available for evaluation and management of that person’s medical condition. The team explores your goals for care, helping you and your medical team understand your wishes to improve your quality of life.

Palliative care is different than hospice care. While on palliative care a person my still access all medical treatments they wish while when on hospice care, the focus is on comfort and certain medical treatments are not pursued. 

Palliative Care Goals:

• Optimize Symptom Control
• Enhance quality of life for the patient and family
• Optimize functional status when appropriate
• Help with challenging care decisions
• Educate patients and families to promote understanding of disease process and expected further course of the illness
• Promote a system of care the fosters timely access to Palliative Care Services

Questions to consider when contemplating Palliative Care:

• What kind of training does the Palliative Care staff have?
• Is there a cost involved for me?
• How often will I get visits and/or contact?
• How do you communicate with my other providers?

Palliative Care should be considered when you or a family member have:

• Recommendation from physician
• Have a serious or life limiting illness
• Progressively declining health
• Frequent hospitalizations
• Decreased ability to perform regular activities of daily living