Caregiving

For many patients and their families recently diagnosed with ALS, making decisions regarding treatment, symptom management, and overall quality of life can be very confusing.  In all cases, Augie’s Quest strongly recommends that you discuss the following resources with your care team and your team of qualified doctors. We are not physicians, but we are dedicated to raising awareness and providing helpful information to our ALS champions.

First things first

With amyotrophic lateral sclerosis or ALS, it’s very important to be as communicative and proactive as possible to help manage your disease from the onset of symptoms.  There are many important steps in communication and disease management that you can take to improve your quality of life, your symptoms, and provide you with a longer, more meaningful outcome. 

The most important first steps in disease management as it complements your treatment options are:

• Gather your support system
• Establish a clinical care team of physicians and specialists
• Create a communication game plan
• Learn about successful intervention strategies
• Collect information on managing symptoms
• Study treatment options

Teamwork can enhance your outcomes 

It’s important to know and understand that current treatments can’t reverse or entirely halt ALS – yet. However, some innovative treatments can help ALS patients maintain and manage their disease effectively.  Current ALS treatments have been found to do the following: 

• Slow the progression of symptoms
• Manage the speed of deterioration in function
• Prevent unnecessary complications
• Increase individual comfort
• Prolong independence

You will want to make sure that you assemble an integrated, multidisciplinary team of physicians and specialists trained in all the areas of your healthcare as it relates to your ALS symptoms.  Specialists may include:

• ALS neuro specialist
• General neurologist
• Primary care physician
• Gastroenterologist
• Respiratory therapist
• Speech pathologist
• Social worker
• Occupational therapists
• Dietician
• Palliative care specialist
• Spiritual advisor
• Holistic practitioners
  

You will want to develop a communication plan with your team of physicians so as to establish consistency and transparency in your care.  This could not only prolong your survival, but it will improve the quality of your life. Your care team should work together to select the best course of treatment for you while explaining their therapeutic benefits and/or risks.

Join the Precision Medicine Program at ALS Therapy Development Institute

The Precision Medicine Program (PMP) at the ALS Therapy Development Institute (ALS TDI) is the most comprehensive and longest-running translational research study in ALS. The PMP literally designates patients as partners in ALS research and provides them with personalized information to assist them in their ALS journey.  

ALS patients from around the globe are enrolled in the program and share a wide variety of disease data and symptoms to better understand ALS and possible receptiveness to treatment options.  The data collected is studied at the Augie’s Quest Translational Research Center, collecting a wide variety of unbiased data sets allows the PMP to understand and comprehend all pathways of the disease and how it can progress and differ from one patient to the next.  

ALS patients who participate in this program contribute to discovering new ALS therapies and treatments, help with clinical trial effectiveness and efficiency, and PMP participants are given access to assistive data and a large variety of tools to manage their disease progression. 

The PMP program is changing the face and future of ALS treatment and research.

Targeted Medications Specifically for ALS

Currently, there are two major drug therapies and another probable and promising third drug thought to possibly be effective in the treatment of ALS. There is also an emerging natural supplement commonly found in over-the-counter strengths but now found to be effective when prescribed in a more powerful medical dose that can treat ALS symptoms.

As always, patients should carefully study each drug with their care team to determine if the benefits outweigh the risks. Augie’s Quest does not endorse any of the following possible treatments you may consider discussing with your doctor:

• Riluzole (Rilutek):  This is a glutamate blocker that is orally administered in tablet or liquid form.  It has been used to increase life expectancy and can also delay the need and use of a ventilator or tracheostomy.  Riluzole works to protect the nerves in the brain and spinal cord from the overproduction of glutamate in the body, which is the cause of nerve damage in ALS patients. Riluzole has been shown to increase survival three to six months longer in patients who consistently use the therapy. It can cause some side effects including dizziness, gastrointestinal conditions, and liver function changes. 
• Edaravone (Radicava):  This is an invasive IV drug (intravenous infusion) that is thought to possibly slow disease progression and reduce deterioration. It works by reducing the effects of oxidative stress, which is an imbalance in the production of free radicals that attack antioxidant cellular defenses. This is thought to be one of the causes of nerve cell death and nerve damage in ALS patients. Keeping motor neurons healthy may help preserve muscle function. Side effects are significant, can include bruising, headache, and shortness of breath, and may outweigh the benefits of this drug.
• Nuedexta: This is an oral medication combination of an established cough suppressant, dextromethorphan, and a commonly used cardiac drug, quinidine. This drug has been approved to treat patients who develop pseudobulbar affect (PBA), which is common in ALS patients.  ALS patients who have taken Nuedexta have found improvements in speech, swallowing, emotional control, and better management of oral secretions. A Phase II trial funded by the ALS Association found that Nuedexta favorably impacts bulbar function in people living with ALS.
• TUDCA (Tudcabil): Tudcabil (tauroursodeoxycholic acid) is an oral medication originally approved by the Italian Medicines Agency for the treatment of liver diseases. However, research suggests it may have a broader and more beneficial treatment application for ALS.  Tauroursodeoxycholic acid is a bile acid taurine combination derived from ursodeoxycholic acid. Bile acid is a beneficial human metabolite made in the liver and stored in the gallbladder.  It aids in digestion and can assist as an “anti-inflammatory agent, a neuroprotective agent, an apoptosis inhibitor, a cardioprotective agent, and a bone density conservation agent” (https://thesocialmedwork.com/tudcabil-tauroursodeoxycholic-acid). This is a possible treatment option to be discussed with your provider and prescribed as a medical-grade medicine from a reputable medical site.

Other medications to manage symptoms:  Due to the nature of ALS and the disease progression, you may find that you will require some prescribed and over-the-counter medication for the following symptoms and co-diagnosis:

• Muscle cramps and spasms
• Constipation
• Fatigue
• Excessive saliva and phlegm
• Pain
• Depression
• Sleep problems
• Uncontrolled outbursts of laughing or crying
  

Common ALS Assistive Therapies

Due to a variety of symptoms and the idiopathic tendencies of the disease progression, ALS patients tend to require the following therapies to assist in managing their symptoms

• Respiratory Therapy (RT): As muscles weaken ALS patients experience difficulty in breathing. Patients will need to have a relationship with a respiratory therapist who can provide devices to assist breathing.
• Physical Therapy (PT): Many ALS patients find that a physical therapist can address pain, walking, mobility, bracing, and equipment needs that help patients maintain independence. Therapists can assist in practicing low-impact exercises to maintain functionality.
• Occupational Therapy (OT): Many certified occupational therapists can assist ALS patients in remaining independent for as long as possible and prescribe adaptive strategies and equipment to modify a home for accessibility.
• Speech Therapy (ST): Speech therapists teach adaptive speech techniques to help ALS patients’ speech continue to be understandable. They can introduce other methods and devices for communication.
• Nutrition & Dietary Support: The ALS care team helps ALS patients and their families to make sure all nutritional needs are met and meals are easier to swallow and digest. 
• Mental Health & Social Support: It’s very important that your care team includes a social worker to assist ALS patients with insurance, financial arrangements, and obtaining service equipment and devices. Having a psychologist and mental health counselors and therapists is crucial to the mental wellbeing of patients and their caregivers. 

Participation in ALS Drug Trials and Research

Some ALS patients may be a candidate to participate in an ALS drug trial and/or research study.  Potential future treatments and clinical research trials can provide promising medications and treatments for ALS. When Augie Nieto founded Augie’s Quest to Cure ALS, it was his mission that all funds raised were invested directly towards the cure.  Our funding and support for the Augie’s Quest Translational Research Center at the ALS Therapy Development Institute (ALS TDI) are helping continue our quest in identifying new drugs like AT-1501 to fight ALS.

We’re working day in and out to fund the next promising ALS treatments like AT-1501 via our research partners at ALS TDI, and we won’t stop until we end ALS. With Augie’s Quest, ALS doesn’t stand a fighting chance. Support our quest to find a cure by making a donation.

What is ALS, and What Does it Do to the Body?

What is ALS?

ALS. Three powerful letters that, upon hearing, can change a person’s life instantly. You may have heard it called Lou Gehrig’s disease, after the famous Yankee player who was diagnosed with it. Maybe you first learned about ALS because of Stephen Hawking, or perhaps a close friend or beloved family member was recently diagnosed with ALS.  

Amyotrophic lateral sclerosis, or ALS, is a fatal motor neuron disease that targets nerve cells in the spinal cord and brain. 

ALS often begins with muscle twitching and/or weakness in a limb, symptoms that are easy for the average person to shrug off. However, as the disease progresses, ALS affects control of the muscles needed to move, speak, eat and breathe. 

The cause of ALS is unknown. Scientists do not yet know why ALS strikes some and not others, which is why the mission of Augie’s Quest to raise critical funds and awareness to advance cutting-edge research, fast-track effective treatments, and ultimately, find a cure for ALS is so urgent.

What Does ALS Do to the Body?

ALS is not experienced in the same way, and the disease progression varies greatly among all those diagnosed. Most people live 3-5 years after their first signs of disease.

• Many with ALS first feel muscle cramps, spasms or twitching in one of their arms or legs. 
• About 25 percent of people with ALS first have trouble talking clearly and slur their words. This form of the disease is called bulbar-onset ALS. 
• As the disease progresses, many muscles weaken and start to stiffen and breathing may become affected. 
• Depending on the progression of the disease, a feeding tube may be recommended to help meet nutritional needs.
• In the late stages of ALS, a person’s muscles become paralyzed, and they may lose the ability to move and speak. Many people with ALS require a wheelchair to move around. Some may communicate through assistive technology devices. 
• If someone with ALS has trouble breathing, they may choose invasive ventilation to help keep them alive. 
• More often than not, those in the late stages of ALS are cared for at home or in hospice. 

ALS is devastating to the function of nerves and muscles. However, ALS does not affect the five senses. In most cases, cognitive function, including learning, thinking, reasoning, remembering, problem solving and decision making, are not affected by ALS. 

Currently, there is no cure for ALS. But we know that ALS is a disease we can tackle, a problem we can solve. Because ALS is curable; it’s  just underfunded. 

Augie’s Quest to Cure ALS is dedicated to changing the experience of people living with ALS. Today, there is real hope, like never before. With Augie’s Quest, ALS doesn’t stand a fighting chance. Support our Quest to find a cure by making a donation.

Onset of ALS: What are the Early Signs of ALS?

Phil Green became a champion for the ALS community after his ALS diagnosis.  Augie’s Quest is proud to have him on the AQ Leadership Council.

Amyotrophic lateral sclerosis, or ALS, is a fatal motor neuron disease that targets nerve cells in the spinal cord and brain. Approximately 30,000 Americans are living with ALS, with a new case diagnosed every 90 minutes. Currently, there is no cure for ALS — making it one of the most devastating disorders to affect the function of muscles and nerves.  

What are the Symptoms of ALS? 

The initial symptoms of ALS can vary from person to person. For many, the onset of the disease is so subtle that the symptoms are often overlooked. However, as symptoms begin to develop into more obvious muscle weaknesses and/or atrophy, physicians are more likely to suspect ALS in their patients. 

It is extremely difficult to diagnose ALS. In fact, it’s often diagnosed months or even years after symptoms begin, by ruling out other diseases. It’s crucial to seek a neurologist with experience in ALS and/or neuromuscular diseases if you or someone if you or a loved one are showing symptoms. 

Some of the earliest and most common signs of ALS are: 

• Difficulty walking or doing normal, day-to-day activities
• Muscle twitching in the arms, shoulders, legs or tongue (also known as fasciculations)
• Muscle cramps, especially in the hands and feet
• Slow or slurred speech, known as bulbar-onset ALS
• Trouble chewing, swallowing and/or breathing,
• Muscle weakness in an arm, a leg, neck or diaphragm 
• Inappropriate crying, laughing or yawning

Similar to the symptoms, the rate at which ALS progresses varies by each individual patient. Although not all individuals may experience the same symptoms or patterns of progression, almost all patients are likely to develop progressive muscle weakness and eventually paralysis. 

What Causes ALS? 

Although the exact cause of this disease is unknown, recent research suggests that there are many complex factors that contribute to motor neuron failure in the body.  Between 5-10 percent of ALS cases are genetic, and the remaining are sporadic, meaning the cause is unknown.  Researchers continue to study the potential causes of ALS, and most theories center on a complex interaction between genetic and environmental factors.

Our Quest 

ALS is not incurable, it is underfunded. Augie’s Quest to Cure ALS raises the funds and awareness needed to advance cutting-edge research, fast-track effective treatments, and ultimately, find a cure for ALS. We are proud to support people with ALS around the world by sharing our data and research findings through the Augie’s Quest Translational Research Center in an effort to change the experience of all people living with the disease.  The work we fund at ALS TDI is the most comprehensive and longest running translational research study for ALS.

There are many ways to get involved in the fight against ALS. With your help, our organization can make a significant impact in the lives of those who are affected by this disease. Make your donation online today.