Frequently Asked Questions About ALS
Greg Kenoyer battled ALS for 7 years. He was one of the most knowledgeable advocates in our community and championed this effort. In his honor, we want to share with you what he once shared with us:
“The darkness of our diagnosis is lessened by the light of knowledge. For the last several years my mission has been to share the knowledge I’ve gained to educate, support, and give a bit of hope back to my peers. As part of this mission I was honored and eager to assist in compiling and editing these FAQs. Please use them; please share widely. And please let us know what we missed. We, I AM ALS, need your help to make these FAQs the best resource available!” – Greg Kenoyer (Someone we’ve lost to ALS and incredible advocate)
Education about ALS
- I was just diagnosed. What do I do?
We’re so sorry you’ve heard those words, but we’re so glad you found us. We personally know how devastating this news can be. We want you to know you’re not alone. We are here for you. A good start is reading this.
- What are the symptoms of ALS?
There are many types of ALS symptoms and they can vary person to person.
Limb-onset ALS occurs in your spinal cord and affects your arm, legs, hands, or feet. One of the first symptoms could be unexplained tripping or foot drop. (Foot drop is when you have trouble lifting the front part of your foot.) This is the most common type of ALS.
Bulbar-onset ALS occurs in the bulbar area — your face, mouth, throat, or tongue. A telltale sign of bulbar-onset ALS is changes in speech or singing. This type of ALS is less common than limb-onset ALS.
Some people have a very slow progression spanning over a decade or more, while others will experience rapid progression over several months.
Unfortunately, there is no way to predict how an individual’s ALS will progress and whether progression will be consistent. This is why it’s so important that you continue assessments by your neurologist and health care team.
Remember: ALS symptoms do not begin all at once or suddenly. Symptoms may start subtle and progress to difficulty climbing stairs, walking, lifting items, writing/typing, feeding yourself, swallowing liquids, experiencing shortness of breath, speech changes, trouble with decision-making, or uncontrolled crying or laughing. We know it can be overwhelming to navigate the many challenges that come up as you progress in your ALS diagnosis and we are here to help. Reach out today and get support.
- Are there different kinds of ALS?
Yes. There are generally two types of ALS — sporadic or familial — with many subtypes. Sadly, we don’t know yet what causes sporadic ALS. It may be environmental or genetic factors. And the scary thing is approximately 90–95% of ALS cases are sporadic.
Familial ALS (fALS) refers to ALS cases in which there is a genetic history of ALS in the family (meaning more than one person in the family has been diagnosed). This type of ALS accounts for 5–10% of cases. Over the past ten years, the discovery of genes related to ALS has really advanced. Researchers have found 30 genes implicated as risk factors for ALS. This is great news because it shows several potential targets for therapies that will focus on ALS. Learn more about ALS and genetics here.
There are several types of motor neuron disease (MND) and ALS is the most common. Others include primary lateral sclerosis (PLS) and hereditary spastic paraplegia (HSP). Unlike the other MNDs, ALS is characterized by progressive degeneration of both upper and lower motor neurons. In other words, ALS affects all of your body rather than one part.
Today, researchers think there may be a variety of factors in each individual with ALS that lead to this progressive motor neuron degeneration. The onset, progression, timing and sequence of the degeneration may be different for each person — and typically is different. In other words, everyone’s ALS journey will be unique. Please take the time to educate yourself and take steps, with your doctors, to develop the best quality of life possible for you and your family. If you’re feeling lost on where to start, we are here to help. Reach out at any time.
- Is there a cure for ALS?
That is what is so cruel about this disease: at this time, there are no cures for ALS. There are some treatments that are believed to slow progression, but no treatment is currently able to stop or reverse progression. However, one of I AM ALS’ unwavering missions is to make these vital drugs and treatments available to people living with the disease today. Read more about the treatments and trials available to people living with ALS.
- Is there a gene responsible for ALS?
Good question! Familial ALS accounts for about 10% of ALS cases, while the remaining 90% have no known family history of ALS.
For the past 10 years, gene discovery related to ALS has been a big deal in the scientific world. Researchers have found more than 30 genes as risk factors for ALS. Still to understand: how these genes interact with the environment. This shows us just how complicated ALS is, but it also gives us several potential targets for ALS therapies. Yay science! You can learn more about ALS and genetics here.
- Is there any pain with ALS?
Emotionally, spiritually, mentally — yes. If you’re asking about physical pain — it all depends on the individual and type of ALS. Studies suggest 15–85% of people living with ALS experience some degree of pain depending on the duration of the disease. Muscle cramps and joint pain appear to be a main source of pain. There are both pharmacological and non-pharmacological treatments available, and these are tailored to the individual. Let your health care team know if you’re experiencing pain so they can pursue pain management options for you.
- What are the top misconceptions about ALS?
Since ALS is a mystery illness, many folks are completely unfamiliar with the disease. They may have a lot of misconceptions.
Here are a few misconceptions we’d like to clear up:
- ALS is not Lyme disease.
- ALS can affect ANYONE, in any age group (including, in rare cases, teenagers), and of any gender, race, or ethnicity.
- ALS is not contagious (no instance of contagion has ever been discovered).
- How has ALS treatment evolved over the years?
Over the past 20 years, knowledge and care of people living with ALS have improved significantly!
Until recently, there was only ONE medication for ALS — the ventilator-delaying riluzole.
Now there is edaravone (Radicava), which may slow the progression of ALS in some people, and there are several promising treatments in clinical trials. Improving clinical trials for ALS is a major focus for I AM ALS.
Technology also has improved dramatically. Power wheelchairs provide healthy seating, mobility, safety from falls, and a level of independence that wasn’t possible even 15 years ago, and communications hardware and software enable people with ALS to interact and contribute to conversations and work.
The FAQs you’re currently reading were created by a 95% paralyzed person living with ALS (Greg Kenoyer) using eye gaze only. How incredible is that?
- What are the most commonly misdiagnosed ALS-like diseases?
There are numerous motor neuron diseases besides ALS.
These diseases include cervical spondylosis with myelopathy and radiculopathy, multifocal motor neuropathy, myasthenia gravis, benign fasciculations, post-polio syndrome, primary lateral sclerosis and progressive muscular dystrophy (Source: Epocrates 2019).
- What are the secondary health issues/concerns that arise from ALS?
This answer is complicated (you’ll hear that a lot with ALS), and it’s an excellent reason to have a multidisciplinary clinic team helping you.
Depending on the degree of impairment, people living with ALS are prone to issues ranging from constipation to yeast infections to dental care. Falling and subsequent injuries are common. With our breathing muscles compromised, we’re at risk of pneumonia, which can be fatal. Make sure to talk to your health care team about which secondary health issues you should be aware of.
- There are so many new terms and acronyms. Is there a list of explanations?
We’re glad you asked. We have a helpful list of acronyms here.
- Should I avoid sick people?
The short answer is yes. people living with ALSs’ bodies are weakened and more susceptible to disease. It also takes longer to recover from illness, because the body is constantly stressed from fighting ALS and has scant reserves.
If you live or work in an environment that brings you into contact regularly with those who are sick, you should discuss this with your doctors.
ALS Treatment and Care
- Are there ALS specific therapists?
Yes. The ALS Association designated Centers of Excellence, Muscular Dystrophy Association-sponsored clinics, and The Les Turner ALS Foundation provide specific information regarding specialists throughout the country. You can find an ALS clinic near you here or contact your nearest medical center’s department of neurology. You can also contact our ALS Support Team for assistance or visit our website for more information.
- Are there any treatments for ALS?
While there are currently no cures for ALS, there are a few treatments and medications that can be used to help combat ALS and its symptoms.
Every person living with ALS progresses differently. You’ll need to discuss your symptoms with your doctors to figure out which, if any, treatments and medications might be right for you. You can see which treatment and clinical trials are currently available here.
- What is the ALSFRS-R?
The ALS Functional Rating Scale (revised) (ALSFRS-R) is used in the ALS clinic and as a part of research trials to monitor your function and how ALS is affecting your body.
It’s a questionnaire-based scale that measures and tracks the changes in physical function over time. It’s widely used in clinical trials and has been used and revised since the 1990s. It measures 12 aspects of function, ranging from ability to swallow and use utensils, to climbing stairs and breathing. Each function is scored from 4 (normal) to 0 (no ability). There is a maximum score of 48.
A series of these tests can be used to judge the rate of progression.
Currently, the FDA will accept ALSFRS-R as a primary outcome measure for pivotal ALS trials for possible approval. They do ask that missing data from any cause is addressed in any analysis of this measure. Because the ALSFRS-R is not sensitive to some important changes and requires rigorous training to acquire good data, there is great interest to develop better outcome measures.
A possible alternative to ALSFRS-R, the ROADS (Rasch-Built Overall ALS Disability Scale), is in development.
- What is ROADS?
ROADS (Rasch-Built Overall ALS Disability Scale) is a relatively new mathematically derived self-reported disability scale that may become a useful approach in ALS clinical trials. It appears to be more sensitive than ALSFRS-R in measuring changes in function.
ROADS includes 28 daily activity questions such as “Can you blow out a candle?” or “Can you roll over in bed?” The person living with ALS answers either 0 (unable to perform), 1 (abnormal: able to perform but with difficulty), and 2 (normal: able to perform without difficulty), allowing for improved answering that may be beneficial for ALS clinical trials. It is important to start to incorporate ROADS in trials of new therapeutic agents in ALS. This is how we will learn if it is better than current outcome measures and its validity.
- What happens in an ALS clinic?
Many ALS clinics use a multidisciplinary team approach to manage your care. Many specialists are present and will meet with you and your caregiver(s) individually. The team members typically coordinate your care under the direction of a neurologist experienced in ALS.
Your team members may include a respiratory therapist or pulmonologist, nurse or nurse practitioner, physical therapist, occupational therapist, speech language pathologist, dietitian, social worker and a liaison from The ALS Association or Muscular Dystrophy Association.
During your clinic visits, you can expect to receive strength (grip) tests, pulmonary function tests (the status of your breathing), various blood tests and other specialized tests depending upon your unique needs.
Remember, YOU are the most important member of your team and don’t be afraid to ask questions. Click here to find your ALS clinic and click here to review questions to ask your health care team at the clinic.
- Can other illnesses/medications affect ALS?
Good question. Your neurologist will complete a thorough review of your medical history, current and past, as well as all medications. Chronic illnesses such as chronic obstructive pulmonary disease (COPD) may have an impact on your breathing. In addition, acute illnesses that occur, such as viral syndromes, may cause fatigue. Whatever illnesses you have or medication you take should be discussed with your health care team.
- Should I exercise? Or save my energy?
Exercising can be great, but there’s a lot of debate about working out when you have ALS. The answer depends on whether you worked out before your diagnosis or not. It’s truly best to discuss exercising with your doctor.
- Should I stop drinking alcohol?
Alcohol can sometimes cause liver damage, as can Riluzole. Alcohol can also temporarily impair speech and motor function. People with ALS may notice that their speaking and coordination get temporarily worse when consuming alcohol. Excessive alcohol (more than 2 drinks in a day) use should be avoided in people taking Riluzole. Please discuss whether it is safe to consume alcohol with your doctor, especially if you are on Riluzole to try and minimize the risk to the liver.
- Should I change my diet?
Studies have shown that a high-calorie, high-carb diet is beneficial for ALS. This, of course, doesn’t mean you should adopt an ice cream and cake diet. (Though ice cream and cake are super tasty.) Eat healthy, but don’t skimp the calories. If your neurologist has not connected you with a nutritionist, ask that they do so. To read an FAQ created by a dietician who works with people living with ALS, click here.
- Should I get oxygen?
Great question. This is a question you should discuss with your health care team.
- When should I consider a non-invasive positive pressure ventilation (NIPPV)?
People with ALS frequently don’t recognize the weakness in their diaphragm, the primary muscle for breathing. The symptoms may be subtle, such as difficulty taking a deep breath when lying flat, feeling groggy, having headaches upon awakening and less breath to finish sentences.
Commonly, a pulmonologist, respiratory therapist or neurologist will discuss the use of NIPPV when forced vital capacity (FVC), a breathing test conducted in clinic, is <50% of predicted or when negative inspiratory force (NIF), or force generated while sucking in, is abnormal. In the beginning of your ALS journey, this non-invasive ventilation may be offered in different forms — bilevel positive airway pressure (BiPAP) or trilogy (AVAPS) — and is normally used at night and may be used up to 24 hours/day as ALS progresses.
When and whether to use NIPPV is something you should discuss with your doctor. The use of non-invasive ventilation has shown to have a positive impact on the quality of life and survival of people with ALS. Learn more here.
- What is a tracheostomy?
“A tracheotomy or a tracheostomy is an opening surgically created through the neck into the trachea (windpipe) to allow direct access to the breathing tube and is commonly done in an operating room under general anesthesia. A tube is usually placed through this opening to provide an airway and to remove secretions from the lungs. Breathing is done through the tracheostomy tube rather than through the nose and mouth. The term ‘tracheotomy’ refers to the incision into the trachea (windpipe) that forms a temporary or permanent opening, which is called a ‘tracheostomy;’ however, the terms are sometimes used interchangeably. “
Be aware that invasive ventilation treats respiratory failure but does not stop the disease progression. Twenty-four-hour care will be required. There are resources and webinars available through NEALS and Your ALS Guide.
- How do I determine whether I should get a tracheostomy or not?
Getting a trach is one of the most difficult questions people living with ALS face. On the one hand, it can prolong life. On the other hand, the cost and impact of care are significant. Whether it is right for you is a question only you and your doctors can decide. Learn more through this NEALS webinar, this I AM ALS series with people living with ALS and trachs (Part 1 and Part 2), or this guide, and consider reflecting on your personal values before making this decision.
- Can you speak if you have a tracheostomy?
This is a question to ask your doctors. It depends on how much air is allowed to “escape” through your vocal chords during mechanical ventilation. Some people living with ALS can speak with their trach, but we aren’t able to say how many actually can.
- What are the different types of feeding tubes?
The operations used to place G-tubes are:
- PEG (percutaneous endoscopic gastronomy), which may be done under general anesthesia. This is the most common type of G-tube.
- RIG (radiologically inserted gastrostomy), which is done under twilight sleep and/or local anesthesia. This G-tube is used when breathing is an issue.
- PIG (per-oral image guided gastrostomy), which is least common.
The various types of tubes include:
- Danglers, which are tubes with 12-18” of external tubing and usually an internal bolster to prevent the tube from pulling out (which is a scary but not grave problem). These can last for years before needing replacement. Some danglers can use internal balloons instead of the solid bolster. These will eventually fail and need replacement.
- Low profile or button, which does not have the long external tube but does require an extension tube for use. This type can have an internal bolster but more commonly uses an internal balloon. Just as the above, these balloons will eventually fail.
- When should I get a feeding tube (G-Tube)?
This is a personal decision that only you can decide, with support from your loved ones and your health care team.
Consider a feeding tube when you have difficulty swallowing or chewing food, maintaining weight, staying hydrated, or if your breathing tests show decreased function.
The operations used to place a feeding tube are normally done on an outpatient basis. While tubes can be placed under twilight or mild sedation for people with breathing impairment, there is a point at which any operation is too risky. If possible, get surgery done early in your ALS journey. Many people living with ALSs receive their tubes while they can still eat and continue to enjoy regular food. They then begin to use their tubes gradually, sometimes for extra hydration or to avoid swallowing pills.
Some people living with ALS choose to forego a feeding tube — this is entirely your choice.
- When should hospice be called?
When you choose to end mechanical or invasive life-prolonging measures, hospice care should be considered. The hospice team develops an individualized plan of care to meet your needs for pain management and symptom relief, as well as emotional and spiritual needs. In addition, members of the hospice team can assist caregivers within the home. Often the team neurologist will suggest contacting hospice to discuss the many services provided.
Familiarize yourself with Medicare hospice benefits so you can make an informed decision when the time comes. Many people living with ALS have lived a long time while receiving Medicare hospice benefits. Some with ALS opt for hospice teams while they continue to use normal benefits and insurance and receive approved treatments. The word hospice has both a benefit meaning and a care meaning, and it’s important to understand both.
Once under hospice care, your treatment choices may be limited, especially if those treatments are considered life-extending. For example, you may not be allowed to begin using a ventilator, to have Radicava infusions or enroll in a clinical trial.
Before entering hospice care, talk with your health care team, family and support network to ensure this is the next best step for you.
- Does marijuana help with ALS symptoms?
Cannabis/Marijuana is associated with anecdotal improvements in ALS symptoms including pain, cramps, anxiety, insomnia, excessive secretions and poor appetite. Some of these benefits have been confirmed in small studies in patients with other diseases such as cancer, MS and spinal cord injury.
There is also preclinical evidence that cannabis could impact the progression of ALS but there is no clear consensus on a specific strain, route of administration or dose for use in ALS. There are a couple of ALS trials underway to test some of the benefits of cannabis.
Cannabis laws vary widely between states. Talk to your health care team before trying marijuana.
Getting an ALS diagnosis is difficult for many reasons. One of the challenging aspects is not knowing what treatment is right for you. The good news is there are an increasing number of clinical trials that are ongoing that have the potential to slow down disease progression and/or manage your ALS symptoms. This document gives you answers to some frequently asked questions on clinical trials and how you may access further information on getting involved in clinical trials for ALS. We hope you find this information helpful in learning about ALS research.
- What is a clinical trial? How is it different from an observational study?
Clinical trials are studies in which researchers investigate whether a therapy works in treating or curing a health condition. People are assigned to control and intervention groups — the investigational therapy is given to people in the intervention group, and a placebo (see below for definition) is given to people in the control group. Before any clinical trial begins for a new therapy, the researchers must submit an Investigational New Drug Application to the FDA.
In observational studies, researchers study people without trying to affect them by giving them drugs, treatments or therapies. The goal is to gather information and compare changes over time. This may lead to new clinical trial possibilities, diagnostic tools and trends about people living with ALS. This article provides a detailed description of clinical trials. This page from Neals.org provides terms and definitions you may come across while looking for a clinical trial.
- Why should I consider participating in an ALS clinical trial?
By participating in a clinical trial, you contribute valuable medical information to help advance understanding and treatment of ALS for you and others. Clinical trials offer the possibility to receive investigational therapies and products not currently available. Trials help determine if a treatment is safe, effective and whether it can slow the progression of ALS symptoms. Some trials look at ways to improve quality of life issues and find diagnostic tools. While in a trial you have access to an expert team that may give you a deeper understanding of ALS and the treatments available.
Some studies have shown the increased assessments and visits during trials lead to better patient outcomes. There is increased hope patients experience participating in a trial.
- What are the potential risks of participating in a trial?
Some risks of clinical trials include experiencing possible side effects of the investigational therapy, receiving a therapy that may not be effective, potentially having to discontinue other medications and supplements in order to participate in the trial, and more. It is also important to consider direct and indirect costs associated with trial participation such as travel to/from a trial site, undergoing additional tests, going to more medical appointments, possible time away from work for you and/or your caregiver, and more.
Please talk with a trial research coordinator to get a detailed understanding of the risks and costs associated with a specific clinical trial. Trial sponsors will cover costs of the trial drug, assessments, and sometimes travel expenses but there may be some costs billed to your insurance. This information will be in the informed consent form. It is important to read it and ask any questions before agreeing to participate.
- Do all clinical trials involve taking a new medication?
Although many involve investigational medications, some studies test other elements such as your voice, technology, diet, supplements and therapeutic devices.
- What is a placebo?
A placebo is made to look like the investigational therapy but is made with inactive ingredients that should not have an effect on a trial participant’s health. Some people experience a placebo effect. A phenomenon where people experience a benefit due to expectations that the treatment is working. You and typically the medical team administering the trial, will not know whether you’re receiving the placebo or the investigational therapy. This article provides a detailed description of how placebos are used in clinical trials.
- Why do we use placebos in clinical trials? Do all trials use placebo?
By having some clinical trial participants receive a placebo instead of the investigational therapy, researchers can measure the effect of the investigational therapy in comparison to its absence on someone living with ALS. This allows doctors and researchers to scientifically verify if the therapy is effective at treating the condition.
Open-label trials do not require placebo as all participants receive active treatment. Thank
This article provides a good summary of placebo use in clinical trials.
- What is a double blind placebo controlled trial?
This refers to a trial design where participants are randomly assigned into two groups, one that will receive the investigational therapy and one that will receive the placebo, and neither the researchers nor research participants know who is getting the investigational therapy and who is receiving placebo.
- When will I know if I received the investigational therapy or placebo?
Researchers can disclose whether you received the investigational therapy or placebo after a trial has been completed by everyone participating. Typically you will not know until the data is analyzed or already underway. Some companies do not share this information until after they talk about the results to the FDA in case there are follow-up questions that need to be addressed. If you choose to participate in an open-label extension, you may not know if you had active treatment or placebo during the active portion of the trial. Participating in an OLE though will contribute valuable data about the investigational therapy.
- What does randomization mean? What do the ratios mean?
Randomization is the process of assigning participants by chance to groups that receive different treatments. A 1:1 ratio means for every participant who receives the investigational therapy, one will receive the control treatment. A 2:1 ratio means for every two participants who receive the investigational therapy, one participant will receive the control treatment. The control treatment is typically the standard of care and a placebo.
- Who can I ask about current trials?
Start by asking your doctor and healthcare team if they participate in trials, are aware of current trials, and what trials may be best for you. Here are some questions that can be helpful in guiding an initial conversation about ALS trials with your care team. Also, you can learn more about what trials are available through ALS Signal: Clinical Research Dashboard, which was designed for and by patients and caregivers impacted by ALS. It includes a detailed list of treatments and supplements that are currently being tested for ALS. It also includes the Patient-Centric Trial Design (PaCTD) Rating Criteria, which uses a 5-star rating system to indicate the level of humane and efficient trial designs. NEALS Consortium also provides clinical trials information here.
It is important for you or your caregiver to be proactive and be your best advocate in finding the appropriate trial for you.
- I’m feeling overwhelmed. Where can I find support with learning about and finding clinical trials for ALS?
The ALS Support Team is here for you. They can support you with learning more about clinical trials, what trials are available and help connect you with a site. You can request their support online or call them at 866.942.6257 Monday to Friday 9 am to 5 pm CST.
NEALS has a clinical trials liaison and their contact information can be found here.
The Healey Center for ALS offers coordination services to help you understand options that are available. Here is their link.
- How many phases are in a trial and what do researchers hope to learn in each phase?
Clinical trials advance through four phases to test a treatment, find the appropriate dosage, look for side effects and evaluate short-term and long-term effects. If, after the first three phases, researchers find a drug or other intervention to be safe and effective, the FDA may approve it for clinical use and require that researchers continue to monitor its long-term effects.
A phase I trial tests an investigational therapy or product on a small group of people living with ALS, sometimes the trial includes healthy participants. There may be several small groups in the phase 1 trial, one group starts once the previous group finishes. This is also referred to as a learning phase as the goal is to judge the investigational therapy’s safety and side effects and to find the correct drug dosage. Researchers also look at pharmacokinetics (distribution, metabolism, excretion) and pharmacodynamics (effects and mechanism of action).
A phase 2 trial may enroll more people than a phase 1 and may also enroll participants in small groups like in a phase 1. While the emphasis in phase I is on safety, the emphasis in phase 2 is on the preliminary effectiveness of the investigational therapy and how it works biologically. Phase 2 trials continue to study safety, including short-term side effects, and may also investigate some measures of clinical efficacy as a secondary objective. Phase 2 can be a learning phase and begin a confirmatory phase as to whether the experimental treatment works.
A phase 3 trial gathers more information about safety and dosing but the emphasis is on clinical effectiveness. There are usually a larger group of participants. If the FDA thinks the trial shows statistically significant positive results, for example, at slowing ALS disease progression, then it will approve the experimental drug. Phase 3 is also referred to as a confirmatory phase. Does the experimental therapy work or not.
A phase 4 trial takes place after the FDA approves their use. An investigational therapy’s effectiveness and safety are monitored in large, diverse populations. Sometimes, the side effects of a drug may not become clear until more people have taken it over a longer period of time.
- How do I enroll in a clinical trial?
The research coordinator or research nurse at the trial site will walk you through the prescreening and screening process to determine your eligibility to participate in the trial based on the inclusion and exclusion criteria. If the contact information for the site is not listed, there should be contact information for the pharmaceutical company conducting the trial for you to reach out to. They will be able to provide you with contact information for sites enrolling in their trial and possibly sites that haven’t been activated yet and therefore aren’t on the list yet.
- What medical information will I need? How will the trials team get it?
The personal and medical information needed can vary depending on the study. Most often your medical records will need to be sent from your doctor’s office, and the research coordinator for the trial will email or call you for additional information. You might be able to answer their questions yourself, although in some cases you may need additional support from your care team to ensure you’re providing appropriate and accurate information. We have compiled a list of information commonly requested by trial research coordinators here and have also created an email/fax template for you to send this information to them here.
- What are inclusion and exclusion criteria? If I meet these, am I guaranteed a spot?
Inclusion criteria are characteristics that potential trial participants must have to be considered eligible for a clinical trial. In ALS trials this could be age, time since your symptoms began, diagnosis date or breathing score known as vital capacity. Meeting these criteria does not guarantee a spot in the trial.
Exclusion criteria are characteristics that disqualify potential trial participants from being eligible for a clinical trial. In ALS, this may include medications, supplements or treatments that interfere with investigational medication being tested. Most trials allow a washout period before the trial begins.
- What will be required of me when participating in a trial? Are there questions I should ask about a specific trial?
Each trial is designed differently, so it is important to ask the research coordinator what will be required of you during the trial. For example, the number of visits, tests administered, route of administration and length of study are among some questions to ask when applying to the study. Here is a list of questions that may be helpful in guiding an initial conversation with your trial team.
- What happens if I’m not approved for a trial?
Don’t give up! If you weren’t turned down due to inclusion and exclusion criteria, you may want to reach out to a different location conducting the trial. You can find another location by asking the research coordinator or looking for one on ALS Signal. You can also ask your care team if there is a different study that you may qualify for.
If you don’t qualify for the trial, you can ask if an Expanded Access Program (see below for definition) is available.
- What are the most common reasons patients don’t qualify for a trial?
For some trials, your first symptom of weakness from ALS has been present for longer than the criteria for the trial allows. For other trials, your diagnosis date may be outside the criteria window for the trial. Some trials use symptom onset as criteria and others use diagnosis date.
Vital capacity of lungs is below the cutoff listed in the criteria. Even if your most recent vital capacity test result was done at your clinic recently, the test has to be done on the day of screening and has to meet or exceed the criteria listed in the study description.
Pre-existing medical conditions that may make it unsafe to participate in the trial or may be known to interfere with the trial treatment.
Taking an excluded medication or supplement may require a wash out period which is often 30 days. This information can be shared with you by the research team.
- What is a biomarker?
Per the FDA, “Biomarkers are characteristics of the body that you can measure.” They are an indicator for having the disease or the disease progressing, and can be compared to someone who does not have the disease or is not progressing. For example, blood pressure is a biomarker for heart health. They can be helpful in diagnosing disease and evaluating the progression of it. In clinical trials for ALS, biomarkers are valuable in measuring the effects of an investigational therapy on people with ALS.
- What is the difference between EAP and OLE?
Expanded Access Programs (EAP) allow people who have a life-threatening illness and who are ineligible for a clinical trial to potentially access an experimental therapy outside of the clinical trial process. This is often only possible if the trial has an EAP program. EAP requires FDA approval. It is important to note that even if you are eligible for EAP, the drug company is the final decision-maker.
Open Label Extension (OLE) of an experimental treatment follows the completion of a clinical trial phase. Trial participants can receive the experimental therapy after the trial completion without receiving the placebo. OLE is extremely important in trials as Researchers will continue to evaluate long-term effectiveness and safety. Similar to EAP, this is only possible if the trial has this program.
- Can I continue my current medications while I’m in a trial?
This varies from trial to trial, and you should talk to the trial coordinator about this. Most studies allow for continuing the standard of care, which is the widely accepted and used treatment by medical professionals for a specific disease. If there is a medication or supplement you’re not allowed to use during the trial, some trials allow for a washout period where the medication is no longer in your system. Definitely a question to ask the researchers of a specific trial. You cannot be on an investigational therapy while in a clinical trial for another therapy.
- How much does ALS cost?
Unfortunately, like many chronic diseases, ALS can be expensive. Some common expenses include caregiving, transportation, medications, paying for conveniences, handicap remodeling and more. The good news is there are many resources available to help you with some of the costs. The ALS Support Team can assist you in understanding which resources are available to you. And it’s free. Contact us here or explore resources here.
- How can I receive financial assistance?
There are a number of organizations, beyond state and federal governments, that can help alleviate the financial burdens caused by ALS. We offer a helpful list of organizations here or contact our ALS Support Team team to learn more.
- What does insurance cover?
Sigh … insurance can be so complicated. Medicare is often the common denominator for people with ALS, so it pays to find out as much as possible about your Medicare coverage. You can call 1-844-244-1306 to talk to a case manager at the ALS Medicare Resource Line.
If there is a long-term disability policy included in your insurance plan, we recommend pulling that out and reading it.
If you’re still working and have employer coverage, it’s important that you understand how your plan covers your care and treatment so you can make smart decisions regarding SSDI (Social Security disability benefits) and Medicare. Please talk to your HR rep at work for details.
You can also contact I AM ALS and an ALS Support Specialist may be able to help you with your insurance questions.
- Can I apply for Medicare?
If you’re eligible for Medicare, an ALS diagnosis will allow you to apply for benefits regardless of age. Please note: The five-month waiting period still applies. Medicare.gov offers an Eligibility & Premium Calculator that may show you your eligibility and premium costs.
- Am I eligible for disability? Can I apply for Medicare and Social Security benefits, regardless of my age?
Once you’ve confirmed a diagnosis of ALS and have made the decision to stop working, you should promptly file an application for Social Security Disability Insurance. The easiest way to apply is here. Social Security may code ALS as Lou Gehrig’s Disease; therefore, when applying, you need to be sure to reference Lou Gehrig’s Disease.
- I am a veteran diagnosed with ALS. How do I apply for benefits?
ALS is one of the few diseases that receives a presumptive 100% service-connected disability rating. If diagnosis was made by a civilian physician, you’ll need to make sure you’re enrolled in the Veterans Health Administration and apply for benefits with the Veterans Benefits Administration. Then schedule an appointment with your VA health care team, submit copies of medical records reflecting diagnosis and ask about connecting with a VA ALS clinic or a VA neurologist who specializes in ALS. This will start the medical process. At the same time, you can start the claims process for benefits. File a claim at VA.gov OR contact your local VA office so they may help with your claims process.
The VA is a complex organization that can be challenging to navigate, and its processes can feel convoluted and overwhelming. We recommend having a Veterans Service Officer (VSO) of the Paralyzed Veterans of America (PVA) or Disabled American Veterans (DAV) represent you. PVA and DAV are extremely adept at the claims processes for vets with ALS and have exclusive knowledge of and access to the internal VA systems.
- My emotions are all over the place. Is this normal?
Fear, anger, shock, confusion, denial — everything you’re feeling is normal. An ALS diagnosis is devastating and brings incredible loss. You and your loved ones may start to experience different levels of grief or anticipatory grief, which is grief related to expecting loss that has not yet occurred.
Please do not suppress your emotions or keep them to yourself. Not talking about your thoughts and emotions can lead to feeling isolated and also can take a toll on your physical health. Reach out for support, maybe even before you feel like you need it. Not sure how to get support? Contact our ALS Support Team to learn more about your options for professional counseling, peer support and support groups.
If you experience uncontrollable crying or laughing, you may have pseudobulbar affect. Talk to your health care team about your symptoms as soon as possible to discuss treatment options.
- How do I know if or when I need help?
Hearing the words “You have ALS” can be devastating. It’s not uncommon to feel sad, hopeless or depressed, and it may take some time for you to absorb the information. This is completely normal.
While an ALS diagnosis is something no one is ever prepared for, we want to remind you that your diagnosis does not and should not define who you are.
If you’re experiencing feelings of depression, including low mood, loss of interest and enjoyment, sleep and appetite changes, poor concentration, and reduced energy, please contact your health care team. There are mental health providers with experience working with people with ALS who are ready to help. You also can contact our ALS Support Team for support with your emotional and mental health.
Studies have shown that people with ALS who have clinical depression fare so much better when their depression is treated. Please do not suffer alone — there is help. You’ve got this, friend.
- How do I tell my children about my ALS diagnosis? What do I tell my family? What do I tell my friends? What do I tell my coworkers?
There is no right or wrong way to share the news of your diagnosis. People respond in their own ways as well — any reaction is normal. Often people do not know how to respond or are uncomfortable. Family and close friends may want to know about your diagnosis, and when you tell them is your decision. Being honest and open is key. Read more on sharing your diagnosis here.
As with any family “secret,” children may notice something has changed and, if not informed about your diagnosis, may feel guilty and fearful. Tell the truth in age-appropriate language. Wonders & Worries can be a wonderful resource for figuring out how to have this discussion.
- What support groups should I join?
Meeting people who are going through the same experience as you is incredibly important for your emotional well being. Joining a support group can remind you that you are not alone in your ALS journey. Whether you’re a person living with ALS, caregiver or child of a person living with ALS, there are communities of individuals who want to support you and provide insight, resources, and guidance. Sign up for a virtual support group, contact our ALS Support Team to learn more about support groups in your area, or head here to learn more.
- What services and support are there for caregivers?
Being a caregiver for a person with ALS can be physically and emotionally trying. If you are a caregiver, we want you to know you’re not alone. We have a variety of resources to help you with your physical, emotional, and financial well being.
You can choose to speak with a licensed counselor or an I AM ALS peer mentor for one-on-one support, or you can choose to participate in a support group. Your clinic may have a licensed social worker or counselor who you can discuss this with. There also are numerous support groups available for people living with ALS and caregivers, whether in-person or online.
In addition, the VA provides social workers and direct support to both veterans and spouses. You can learn more here.
- What foundations might help me?
There are numerous ALS foundations, like I AM ALS, that are standing by ready to help you and your loved ones. You can view a list here. Our ALS Support Team can help by supporting you emotionally, connecting you with essential resources, sharing relevant medical and research information and helping you build a community of ALS support.
- What Facebook groups are helpful for people living with ALS and caregivers?
There are numerous Facebook groups for ALS, but not all of them will be helpful to you. Be cautious of any medical information shared, as well as the impact of each group on your mental and emotional health. Here is a list of known ALS Facebook groups for people living with ALS and caregivers.
- Do I need a foot brace (AFOs)?
An ankle foot orthotic (AFO) helps compensate for foot drop and can make walking significantly easier and less prone to tripping.
If you have foot drop, doctors can help you learn how to combat it so you don’t fall.
- What is a loaner closet?
ALS is a demanding disease when it comes to equipment.
People generously donate the equipment they no longer need to loaner closets (often managed by The ALS Association or Muscular Dystrophy Association chapters) so that someone else can use it without having to purchase a new one. It’s a wonderful resource that can help you try things or save you major expenses.
- What kind of bed and/or mattress should I get?
Getting a comfortable bed and a good night of sleep is super important. A quality hospital bed (frame or frame/mattress combo) that raises and lowers your head, feet and the entire bed is recommended for both patient care and ease of use for the caregivers.
The size and weight of hospital beds can be a problem in some homes. To address this, some manufacturers like Hillrom are producing hospital-quality beds that work in homes. A hospital bed also allows space for a lift to slide under — an important consideration if you’re looking at consumer adjustable beds.
Air mattresses or memory foam mattresses are generally most comfortable, with a lateral rotation air mattress being best.
Most beds are similar to a twin bed in size and can be placed alongside a spouse’s bed.
Stay clear of static, non-adjustable beds. people living with ALSs lose the ability to adjust their position in bed and to avoid bed sores they must be frequently moved, either by the caregiver or by lateral rotation.
Not sure what sort of bed to purchase? Ask your health care team what they recommend and discuss with other people living with ALS and caregivers to learn what’s worked for others.
- How do I go about making house modifications?
Great question. There are a number of resources available to you. The local ALS Association and Muscular Dystrophy Association chapters may know contractors in the area who can help you modify your house. Similarly, the organizations listed on our Discover ALS Resources are great connectors to contractors and the modifications needed to make a house liveable for someone with ALS. Your ALS Guide and ALS From Both Sides are helpful sources on home modifications.
Veterans are eligible for several remodeling grants. Learn more here.
- Can I fly?
Per the CDC’s guidelines, please avoid all non-essential travel during the COVID-19 pandemic as travel increases your risk of getting and spreading COVID-19. Learn more about the guidelines here.
For those with high breathing scores and/or no movement impairment, fly on, but always take precautions. However, if you’re further along in your ALS journey and have to travel with a wheelchair or are vented, it’s important to work with your doctor to make sure you are okay to fly, and the airport and airline to make travel as simple as possible.
If you get the green light to travel from your doctor, call ahead to the airport and airline to clear up any questions and ask about the specific airport, TSA or airline procedures and requirements. The more you know and plan ahead, the better your trip will be. Bon voyage!
- Can I ride a train?
Per the CDC’s guidelines, please avoid all non-essential travel during the COVID-19 pandemic as travel increases your risk of getting and spreading COVID-19. Learn more about the guidelines here.
Yes, the train is a wonderful way to travel, and both U.S. and Canadian railways have handicapped accommodations; however, please note there are neither hoists (hoyer lifts) nor room to use one even in the handicapped rooms.
If your train travels involve high altitudes and you have trouble breathing, make sure to discuss with your doctor before traveling. More information on making Amtrak reservations is available here and accessible services here.
- What are the questions I should ask hotels or stores about ALS-related handicap accomodations?
When it comes to hotels, accessible is a word without real meaning. Some hotels consider a wide door and a grab bar to be handicapped accessible.
Know what you need (a roll-in shower, x feet around the bed, raised commode, etc.) and contact the hotel directly to make sure they assign you to a room that accommodates your needs. Most hotels are great about being helpful. Need help knowing what to ask? Contact our ALS Support Team and we’ll get you the information you need.
For things to consider when staying with a friend, read this helpful blog post.
These FAQs were created in collaboration with SLPs from I AM ALS, Bridging Voice, and Team Gleason.
- What types of changes may I experience with swallowing and eating?
ALS can impact your swallowing muscles just like it impacts other muscles in your body. Approximately 85% of people living with ALS experience a swallowing disorder at some point in the disease progression. You may notice that food or drink spills out of your mouth, or you may have a difficult time managing food in your mouth. You may cough or choke more often during mealtimes or experience regurgitation. The ALS Association has put together this guide which provides information about how to adjust to swallowing changes and how to maintain adequate nutrition when living with ALS.
- I’m drooling a lot. Is there anything I can do to manage my saliva?
We know that drooling can be a frustrating problem and we recommend you talk with your doctor about ways to help manage your saliva.The ALS Association provides some insight about this topic here.
These FAQs were created in collaboration with SLPs from I AM ALS, Bridging Voice, and Team Gleason.
- Are there any strategies I can try to help my loved ones better understand my natural speaking voice?
Communication changes that come with ALS impact the person living with ALS, as well as their communication partners. Knowing these strategies to make the most of one’s natural speech, voice amplification and partner training can make a big difference when learning new ways to communicate.
- What is voice banking and how do I get started?
Voice Banking refers to the process of creating a synthesized voice that sounds like your natural speaking voice. You may also be interested in exploring the BCH Message Banking process. Message Banking is when you use a voice recorder to digitally record personally meaningful words, messages, or sounds using your natural speaking voice. You can later use your synthesized voice created via Voice Banking or your Message Bank recordings on a speech-generating device. Double Dipping combines the message and voice banking processes. If you’re unsure where to start with preserving your voice or if you’re seeking assistance in accessing equipment to complete these processes, you may want to connect with Team Gleason. Team Gleason is in collaboration with The Jay S. Fishman ALS Augmentative Communication Program at Boston Children’s Hospital to provide both support and funding for Voice Banking and Message Banking.
- I already have a communication device. Do I need a low-tech way to communicate?
Yes! Having a way to communicate without having to rely on technology is imperative. High-tech devices can break or be unusable in certain situations, such as when you’re in the shower or using the toilet. Strategies like letter and topic cueing and using low-tech tools can reduce frustration.
- My speech is difficult to understand, but my hands work well. Is there anything I can use to make it easier for others to understand me?
Yes. Some people choose to simply carry a notebook or Boogie Board to write messages to communication partners (without any voice output). There are also low-cost text-to-speech Apps that you could download to your smartphone or tablet to aid in communication. If you notice that your communication partners aren’t understanding your natural speech, you could type out keywords or phrases on your smartphone or tablet to make conversing a bit easier.
Alternative Augmentative Communication (AAC) Evaluation
These FAQs were created in collaboration with SLPs from I AM ALS, Bridging Voice, and Team Gleason.
- What do all these acronyms mean? AAC, SGDs, and SLP.
AAC stands for Augmentative and Alternative Communication. AAC includes low-tech/no-tech tools like pointing, gesturing, or writing down messages on a piece of paper or on a Boogie Board when communication partners have a difficult time understanding you. AAC also includes Speech-Generating Devices (SGDs). SGDs are high-tech computers that can be recommended by a SLP and often covered by your medical insurance. SLP stands for Speech-Language Pathologist. You may have a SLP as part of your ALS Clinic Team, or you may choose to work with an outpatient or home-health SLP to help manage speech, cognitive, and swallowing changes.
- What is the process like for obtaining a high-tech communication device through my insurance company, Medicaid, or Medicare?
Most insurance companies cannot pay for a high-tech speech device until your doctor and Speech Language Pathologist (SLP) can document that a high-tech communication device is warranted. Like other types of Durable Medical Equipment, Medicare typically covers a device at 80% and supplemental insurance may cover the remaining cost. Private insurance and Medicaid may also cover a device. You can contact your insurance company to see how they cover high-tech speech-generating devices (HCPCS code E2510). If you don’t have insurance coverage for a device, check with your local ALS loan closet or connect with Team Gleason to see if they can help! Team Gleason may be able to provide loaner equipment or provide copay assistance when insurance doesn’t cover the device cost at 100%.
- When should I consider getting a communication device?
Insurance companies typically won’t cover a device until your SLP and doctor can document there is a need for an alternative way to communicate. However, acquiring equipment through insurance, Medicare, or Medicaid can take time. Because this process may require multiple visits with a Speech-Language Pathologist (SLP), trials with different types of equipment, written documentation from your SLP and physician, and possibly a prior authorization, it’s recommended that you talk with your ALS care team early on to develop a plan for what you’ll do if/when you notice speech changes and want to get a device.
- Can I upgrade to a new communication device if newer versions come out with features I want?
Usually, no, but you may want to check with device manufacturers to make sure. Medicare and most insurance companies will only cover a new speech-generating device every five years. It’s crucial that you select a device that meets your needs now and will also meet your communication needs in the future, as well.
- Are there ways that I can continue using my phone or computer if I'm having trouble using my hands but my speech is clear?
Yes. You may already have a smartphone, tablet, or computer you’d like to continue to use. The Les Turner ALS Foundation hosted this webinar that covers some ways you can use, adapt, and modify your existing technology to meet your communication needs.
- Are there any text-to-speech Apps I can download on my phone or tablet to support my communication?
Yes! Your SLP may be able to provide recommendations on specific Apps to try. Many Apps may be available to try for free before you purchase them. Some popular and low-cost text-to-speech Apps include Speech Assistant AAC and Text to Speech. Be aware that some Apps may not work with message and voice banking files. Team Gleason may be able to help with an App like Predictable, which is compatible with most message and voice banking files. Please know this list of Apps is non-exhaustive and there may be other Apps that work well for you. It’s recommended that you explore Apps with a SLP knowledgeable with AAC prior to purchasing an App.
- How can I use a communication device if I lose the ability to use my hands?
You may have seen or heard people talk about eye-tracking technology. In addition to eye-tracking technology, there are many other ways to access a computer or communication device. An Occupational Therapist (OT) with expertise in Assistive Technology (AT) may be able to support you with alternative access methods.
- Is there one best communication device, or should I try multiple?
It’s recommended that you try several devices. All devices function a bit differently and not all access methods work well for everyone! SLPs and OTs with AAC experience can help guide conversations around devices, software, access methods, and mounting equipment.
- Do organizations exist that can help me learn to use a communication App or communication device?
We’re lucky to know a few amazing organizations that can help. Bridging Voice or Team Gleason may be able to help with this. You can also check with your ALS Association Chapter to see if they have a SLP who could help you.
- I’m pretty tech-savvy and I don’t think a SGD purchased by my insurance has the computer specs I want. What are my options?
You can always build your own device if you have the resources, time, and knowledge to do so. However, there are a few things to think through before foregoing a dedicated device that may be paid for by insurance. When you build your own device, it can get expensive. Perhaps you want to buy a peripheral eyetracker to plug into your existing computer. Accessing a computer isn’t considered a “medical necessity,” so insurance companies likely won’t pay for the standalone eyetracker. Additionally, eyetracking and other forms of alternative access require that the device be precisely positioned. If your insurance company didn’t pay for the device, they likely won’t pay for a mounting accessory for the device to position your computer in the location required for access. You may also not have access to tech support and/or in-person support from a device manufacturer representative, and product warranties are likely much different if you choose to build your own device. If you’re interested in connecting with others who may have similar assistive technology needs, you may find it helpful to talk with a SLP or OT with assistive technology expertise, or connect with members in one of these Facebook groups: Tobii Dynavox Community, Tobii Dynavox AAC Users, Smartbox Community, AAC Friends, and ALS Assistive Technology Group.
These FAQs were created in collaboration with SLPs from I AM ALS, Bridging Voice, and Team Gleason.
- Does ALS affect cognition or memory?
Maybe. The ALS Association shares this information about cognitive changes that may occur alongside physical changes people experience with ALS. Recent studies suggest that up to 50% of people living with ALS may experience mild-moderate cognitive impairments, and up to 20% of people living with ALS may experience more severe changes that could lead to a dementia diagnosis. When cognitive impairment or dementia is present, people with ALS typically experience signs of Frontotemporal Dementia (FTD).
You might find these helpful:
Caregivers – Start Here
This page from I AM ALS shares helpful information on what to do when you're first diagnosed with ALS.
Starting the Hard Conversations Around ALS
This video from I AM ALS founders Sandra and Brian explores the importance of addressing tough questions about ALS.