ALS - The Caregiver Guide

ALS (amyotrophic lateral sclerosis), also known as Lou Gehrig's disease, is a progressive disorder of the nervous system. It causes muscle problems, disability, and eventually, death. People with ALS face unique challenges, as do their care takers. In this post, we describe the causes and symptoms of ALS, along with some treatment options and helpful coping techniques.

What is ALS?

ALS is a neurological disease that results in the destruction of nerve cells in the brain and spinal cord. When these nerve cells die, people lose the ability to control the movement of their muscles.

ALS disease is a form of motor neuron disease that eventually results in paralysis. Usually, the mind continues to function normally despite the person being physically disabled. But, ultimately, ALS is fatal.

According to the ALS Association, the latest ALS statistics report that fifteen people are newly diagnosed with the condition each day. That’s more than 5,600 people annually. ALS causes two deaths per every 100,000 people.

Types of ALS

There are two types of ALS disease:

• Sporadic ALS. Sporadic means there is no clear cause. This form is the most common, affecting up to 95 percent of people with ALS.
• Familial ALS (FALS). This type has a genetic component, and is passed from parent to child. If one parent has FALS, each child has a 50 percent chance of getting it. Just five to ten percent of people with ALS have this form.

ALS Progression

ALS is a progressive disease, which means it gets worse over time. The stages of ALS are as follows:

• Early Stage
  

  Here, physical effects include weak, stiff, or tight muscles that cramp and twitch. The muscles also begin to waste away (atrophy). It can affect one or more body regions. Sometimes, people do not get a diagnosis in this stage.

• Middle Stage
  

  Symptoms spread throughout the body, and some muscles become paralyzed, while others may remain unaffected. Joints can become rigid and deformed. Breathing may become labored and swallowing is difficult.

• Late Stage
  

  Paralysis is widespread. Breathing, speech, and mobility are severely compromised. It is not possible to eat or drink by mouth. This stage can be particularly challenging for caregivers.

What Causes ALS?

Approximately five to ten percent of cases of Lou Gehrig's disease are genetic. For the other 90 to 95 percent of cases, the cause remains unknown.

Experts suspect the following may play a role in disease onset:

• Chemical imbalance
  

  Those with ALS usually have excess glutamate in their brains. Too much of this chemical messenger is toxic to nerve cells.

• Protein mishandling
  

  If nerve cells mishandle proteins, it may cause the proteins to build up in the cells over time, eventually causing cell death.

• Immune system dysfunction
  

  Sometimes, the body’s immune system can mistakenly attack its own cells, causing nerve cells to die.

In addition to the above Lou Gehrig's disease causes, certain factors increase a person’s risk of developing the condition. These include:

• Children of a parent with FALS have a 50 percent chance of developing ALS themselves.
• The risk of ALS gets higher with age. It is most common in those aged 40 to 60.
• Before age 65, men are slightly more at risk of ALS than women. This risk evens out between the sexes after age 70.
• Some genetic variations may increase the risk of both familial ALS and non-inherited ALS. More research is needed in this area before researchers fully understand the connection.
• People who smoke, especially post-menopausal women, have a higher ALS risk.
• Exposure to toxins. Exposure to lead or other toxic environmental substances may be linked to ALS.
• Military service. Former military personnel are more at risk of ALS than others. Researchers are unsure why this is, although it may have something to do with exposure to chemicals, severe injuries, infection, or intense physical exertion.

Despite these risk factors, ALS can affect anyone—regardless of their geographical location, race, ethnicity, or socioeconomic status.

ALS Symptoms

The early signs of ALS include:

• Weak, stiff, soft, or tight muscles
• Muscle cramping and twitching
• Difficulty walking or carrying out everyday tasks
• Fatigue
• Difficulty swallowing
• Poor balance and posture
• Slurred words
• Weak hand grip

Symptoms may initially affect a single body region or several. It usually starts in the hands, feet, or arms. Those with early stage ALS may require the use of a cane or leg brace.

As the disease progresses, other Lou Gehrig's disease symptoms include:

• Rigid, painful, and deformed joints
• Inability to get up after falling
• Difficulty eating
• Trouble breathing, especially when lying down
• Periods of uncontrolled laughing or crying
• Headaches
• Higher risk of pneumonia and infection
• Loss of speech

In the late stages, paralysis is widespread. Ultimately, the condition results in death.

ALS Diagnosis

There is no one ALS diagnostic test. ALS is often difficult to diagnose, especially in the early stages, because it resembles several other conditions.

To make a diagnosis, a doctor may perform tests such as an electromyogram (EMG). During this test, the doctor will place an electrode into various muscles to check for electrical activity. Abnormal results indicate ALS or another muscle or nerve condition.

Your doctor may also carry out an MRI scan of your brain and spinal cord to check for tumors or herniated disks, blood and urine tests to look for underlying issues, a spinal tap to analyze your spinal fluid, or a muscle biopsy to check for muscle diseases.

ALS Treatment

There is no ALS cure, although people can manage the condition with medical treatments and lifestyle changes. These treatments can slow down disease progression, avoid complications, and enhance well being.

For the best treatment plan, work with your team of doctors and healthcare professionals. Here are some of the Lou Gehrig's disease treatments they may recommend:

Medications

Most people with ALS will need to take medication to manage their symptoms. Riluzole (Rilutek) slows disease progression by reducing glutamate levels in the brain. This pill may cause dizziness, digestive issues, and changes in liver function.

Edaravone (Radicava) reduces the decline in everyday functioning. People take this once a month for ten to fourteen days via IV line. Side effects include shortness of breath, bruising, changes in gait, and swelling.

Take a pill organizer with you anywhere, to keep all your pills in order. ( See Product )

Make sure to stay on top of your medicine regimen with a pill organizer. These handy kits can be taken anywhere, so even those on many different medications can stay organized at all times.

Breathing Care

ALS eventually causes breathing problems. You may need to use mechanical ventilation devices to help you breathe, especially at night. Your doctor can give you more information about your options.

Physical Therapy and Exercise

Physical therapy can reduce pain and help you manage changes in your mobility. Learning how to adapt to changes in your physical capabilities will help you stay independent for as long as possible.

A stretch strap makes any yoga or stretching exercise easy, with long durable straps. ( See Product )

Practice low-impact activities to maintain a healthy heart along with good flexibility and muscle strength. Exercise is also a fantastic mood booster. Try swimming, walking, or chair-based exercises. Additionally, engage in regular stretching to alleviate pain and reduce muscle decline.

We recommend trying yoga or Pilates and using a stretch strap.

Assistive Devices

Button hooks are perfect for anyone who wants to look their best but needs a bit of help when it comes to dexterity. ( See Product )

Living with ALS means changing how you perform basic functions and daily activities. Working with an occupational therapist can help people learn how to modify their actions and their living space to make it more ALS-friendly. They can also advise you on computers and assistive technology.

Make sure to have a reacher grabber on hand, any time you need to grab something too high or too low. ( See Product )

Use assistive devices to help you remain independent for as long as possible. These include implements such as a button hook to help you get dressed or a reacher grabber tool for many everyday tasks.

Learn more about assistive devices here.

Bathroom and Bedroom Safety

A sturdy shower chair is the perfect way to stay comfortable during your daily hygiene routine. ( See Product )

Staying mobile and safe in the home is a key concern for persons with ALS and their care givers. In the bathroom, use shower chairs to take a load off your feet, letting you get clean in comfort.

Try a transfer bench to make getting into and out of the tub even easier. ( See Product )

Transfer benches are a great option for safer showering, letting you enter and exit the shower without risk of falls. Suction cups on the feet keep it in place during use.

Braces and Splints

Leg braces are an essential tool to keep weak muscles strong and aligned. ( See Product )

In the early stages of ALS, using leg braces and wrist splints can help stretch the muscles and keep them in correct alignment. This reduces pain, cramping, and discomfort.

Wrist splints can be worn to keep these joints strong during any of your favorite activities. ( See Product )

Massage for Pain Relief

Just a dab of this pain relief cream is enough to relieve aching muscles, night or day. ( See Product )

Alleviate muscle pain, spasms, and stiffness with regular massages. Ask a care giver to lightly stretch and massage the muscles in your legs, arms, and other affected body parts. For extra benefit, use a soothing pain relief cream.

Wheelchairs and Walkers

Wheelchairs and walkers help people gain independence, avoid falls, and reduce fatigue. In the early stages, people with ALS may get by with a folding walker, although eventually they may need to upgrade to a wheelchair or power chair.

Try a folding walker to stay mobile, even while traveling near or far. ( See Product )

Be sure to remove rugs from floors and use ramps where appropriate to prevent trips and falls and to facilitate smooth movement around the home.

Speech Therapy

ALS affects the speech muscles, so later stages of ALS inhibit people’s ability to speak. It can be helpful to work with a speech therapist to learn different techniques to prolong your speaking abilities. Speech therapists will also teach you other ways to communicate, such as writing or an alphabet board.

Some people with ALS may wish to consider devices to facilitate easier speech, such as computer-based equipment with a synthesized voice.

Dietary and Nutritional Support

Those with ALS will eventually struggle to eat by mouth as the muscles in their mouth and throat become affected. At first, this can be managed through dietary changes, such as by cooking soft, easy-to-swallow foods. Eventually, it will be necessary to use a feeding tube. Your healthcare team will be with you throughout this process.

Social and Emotional Support

A social worker may be available to help you with insurance and financial considerations, while psychologists or therapists can provide emotional support for you and your loved ones. Try to find support groups for people with ALS. There are also groups for care givers and family members.

ALS Prognosis

The average ALS life expectancy after diagnosis is two to five years. Many ALS patients live longer thanks to medical advances. In fact, half of people with ALS live for at least three years after they receive a diagnosis, and one in five survive for five years or longer. Up to one in ten ALS patients live for over ten years.

Due to ALS disease progression, people may experience the following complications:

• Breathing problems
• Dementia
• Eating problems
• Speech problems

Managing ALS after Diagnosis

Receiving a diagnosis of ALS can be frightening and overwhelming, both for people with the condition and their care givers. But medical advances mean that those with ALS can live up to ten years or more after diagnosis. Maintaining an optimistic attitude can improve quality of life, as can using supports and mobility devices and receiving physical therapy. Be sure to seek social and emotional support from loved ones and professionals as you come to terms with the diagnosis and its implications.