ALS Deep Dive: Stages, FAQs and More

Amyotrophic lateral sclerosis (ALS) catches many patients by surprise. One day, you’re doing a regular activity like walking your dog or driving to work, and the next, you start to realize that your movements are stiffer and more uncontrolled.

ALS is a complex disease to diagnose, treat and manage, but our multidisciplinary team at the UChicago Medicine AdventHealth Neuroscience Institute's Neuromuscular Medicine Program offers comfort, expertise and support.

The UChicago Medicine AdventHealth Neuromuscular Medicine Program is an ALS Association-affiliated clinic that offers a multidisciplinary team of specially trained healthcare professionals who address the many needs of ALS patients during a single visit.

Keep reading to learn about ALS, including its causes, symptoms, risk factors, answers to frequently asked questions and a brief overview of what to expect in each of the seven stages.

What Is ALS?

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurological disease that affects nerve cells in the brain and spinal cord, leading to weakened muscles, atrophy and eventually paralysis.

People with ALS eventually lose the ability to walk, dress, write, speak, swallow and breathe. Unfortunately, the disease is always fatal. The average survival time is three years. About 20% of people with ALS live five years, 10% survive ten years and 5% live 20 years or longer in rare cases.

There is currently no cure for ALS, but at UChicago Medicine AdventHealth, we’re always researching and learning more about the condition so that one day we can offer the hope patients and their loved ones are looking for. We’re currently offering a clinical trial for patients diagnosed with ALS.

What Are the Symptoms of ALS?

The hallmark of ALS is progressive weakness. ALS often begins with weakness in one limb before spreading to other parts of the body. Here are symptoms to be aware of:

• Difficulty with simple tasks like buttoning a shirt or holding a cup
• Difficulty with swallowing, talking, chewing or speech
• Fasciculations, which are spontaneous muscle twitches that occur anywhere in the body
• Weakness in the face, arms, legs or difficulty breathing

At the onset, ALS symptoms may be easily confused with those of other conditions, so a second opinion on an ALS diagnosis is common.

ALS Causes and Risk Factors

Although the exact cause of ALS is still unknown, research indicates several factors may put individuals at a higher risk. According to the ALS Association, these factors include:

• Brain and spinal cord injuries
• Exposure to environmental toxins
• Genetics
• Smoking
• Specific occupations, like serving in the military

The 7 Stages of ALS

While the progression of ALS can look different for every person, there are generally seven stages.

Stage 1: Early Symptoms

Early symptoms of ALS include muscle weakness in one hand, twitching, cramping or stiffness in the arms, shoulders and legs. Because these symptoms are subtle and can occur at any age, early diagnosis is often missed, as most people ignore them or attribute them to less serious concerns. But early diagnosis of ALS can help patients and their families learn more about the disease and explore different treatment options to help them maintain or slow down the decline of their quality of life.

Stage 2: Progressive Weakness

Once ALS reaches stage 2, symptoms worsen and become more noticeable. Patients’ motor neurons begin to decline, which leads to muscle weakness and atrophy. It’s during stage 2 that symptoms begin spreading to other areas of the body, making everyday activities like walking, breathing and swallowing a challenge.

Stage 3: Loss of Motor Control

Rapid muscle weakness and loss of motor control are the hallmarks of stage 3. Patients begin struggling to hold objects, walking with a cane or walker may no longer work, and breathing becomes difficult. Stage 3 is when assistance with everyday tasks is needed.

Stage 4: Severe Weakness and Limited Mobility

This is the stage when everyday care becomes a necessity. Paralysis often spreads to patients’ arms and legs, and they may require a wheelchair. The muscles in their throat and mouth begin to deteriorate, often requiring additional support from nutritionists, speech and respiratory therapists.

Stage 5: Final Decline and Respiratory Concerns

Paralysis in stage 5 spreads to almost every area of the body, severely affecting patients’ ability to speak, swallow and breathe, resulting in the need for constant use of a ventilator and feeding tube. Patients require 24/7 care for everyday activities.

Stage 6: Loss of Voluntary Movement

Stage 6 is when all voluntary movement is lost. However, patients may be able to move their eyes, which allows them to use eye-tracking devices to communicate. Although they can’t move their body, they’re mentally and cognitively aware of their surroundings and may want to interact with their loved ones and caregivers. It’s at this stage that hospice care or specialized neurological care is introduced.

Stage 7: End-Stage ALS

End-stage ALS culminates in complete respiratory failure, requiring patients to use ventilators to breathe or transition to palliative care services. During the final stage of ALS, patients’ mental health begins to decline due to complete paralysis.

How Is ALS Treated?

Unfortunately, no treatments currently exist to cure ALS; however, many treatments exist to manage symptoms and provide comfort. Two FDA-approved medicines can significantly delay symptom onset.

At our ALS clinic, we provide symptom management through whole-person care. Our approach is to make it easier to navigate this challenging diagnosis by providing trained experts from varying fields who can educate, teach and become a formal part of their support team during the allotted 2.5-hour appointment. Together, we determine goals of care and work collaboratively to ensure each person’s needs and goals for their care are heard.

Our clinic includes physical, occupational, speech and respiratory therapy services, as well as mobility specialists, a registered dietitian, a representative from the ALS Association (ALSA) and clinical social workers to address any psychosocial barriers to feeling whole. Unique to UChicago Medicine AdventHealth, we provide genetic testing and counseling by our free in-clinic genetic counselor.

ALS doesn't just affect a patient's physical health. That’s why we’re committed to caring for your whole health — body, mind and spirit.

An ALS diagnosis is devastating and life-altering for the person living with ALS as well as their care partner and family. Our goal is to provide supportive counseling and psychoeducation and assist with care navigation throughout the person’s continuum of care.

World-Class Neuromuscular Care Is Close to Home

ALS is a complex disease to diagnose, treat and manage, but our UChicago Medicine AdventHealth neurology team offers expert diagnosis and will support you with a referral for your care.

If you or a loved one experiences symptoms associated with ALS, request an appointment today at 630-856-8640.