The National ALS Registry is the single largest ALS research project ever created and the only population-based registry in the U.S. The registry collects information and demographics on people with ALS, connects patients to clinical trials, and funds ALS research. The purpose of the registry is to learn more about who gets ALS and what causes ALS. This will hopefully give scientists a more complete understanding of the disease and enable them to find a cure.
The National ALS Registry also includes a Biorepository that stores samples for future research.
Recently, the Registry was used for the first time to recruit patients and caregivers into a landmark patient-focused drug development survey that captured important information on the burden of ALS across multiple dimensions, including: symptom experience, quality of life, health care resource utilization, and emotional well-being. Other important advances made possible by the ALS Registry include attaining a 96 percent enrollment rate for registrants to be notified about ALS research opportunities (more than 100,000 such notifications being sent for over 30 clinical trials) and research funding for 13 institutions to examine what factors may contribute to ALS. ATSDR recently released the third report on the results of the data gathered by the National ALS Registry. You can read the report here.
If you are interested in joining the National ALS Registry or learning more about the latest news from the Registry, please visit https://www.cdc.gov/als/.
It is vital that the National ALS Registry continues to be fully funded. Congress has started work on the Fiscal Year (FY) 2019 appropriations process and Representatives Eliot Engel (D-NY) and Peter King (R-NY) are circulating a bipartisan Dear Colleague letter in the House of Representatives (see it here), urging their colleagues to support $10 million in funding for the Registry. The deadline for this letter has passed, but there is still more you can do to help make sure your Representatives are aware of the importance of issues affecting people with ALS.
Visit the ALS Association’s Advocacy Action Center to learn about the latest updates and news about legislative and regulatory issues being addressed by The ALS Association and to sign up to receive alerts when your action is needed to support people living with ALS.
FTD (frontotemporal degeneration or frontotemporal dementia) refers to a group of disorders that causes progressive damage to the temporal and frontal lobes of the brain associated with personality, behavior and language. Loss of function in this area of the brain can lead to impulsive behavior and speech difficulties. Usually FTD does not affect the parts of the nervous system that control muscle movement, but about 10-15% of people with FTD also experience motor neuron degeneration called FTD with motor neuron disease (FTD/MND) or FTD with ALS. Over the past 15 years, doctors and scientists’ knowledge of the connection of these diseases has rapidly grown through genetic discovery, brain imaging studies and biomarker studies. Specifically, researchers were able to confirm the connection between FTD and ALS when the TAR DNA-binding protein 43 (TDP-43) was identified as the central protein in both ALS and the most common type of FTD. Additionally, up to 40% of FTD cases have been found to carry a C9orf72 gene mutation, which is most common in genetic causes of ALS.
In many cases, FTD symptoms are noted prior to the diagnosis of ALS. FTD symptoms include:
increase in inappropriate actions
loss of empathy and other interpersonal skills
lack of judgment and inhibition
repetitive compulsive behavior
decline in personal hygiene
changes in eating habits
lack of awareness of thinking or behavioral problems
difficulty in using and understanding written and spoken language
loss of knowledge of word meaning
Paula Richard describes what it is like to experience a loved one living with the symptoms of FTD/ALS:
In 2015 my husband was finally given a diagnosis of ALS/FTD . ALS alone is a terrible disease, but to have FTD with it is awful. Seeing my loved one go through the everyday struggles is heartbreaking. The cognitive issues outweigh the ALS part sometimes. He has bad comprehension and doesn’t know right from wrong. His behavior is like taking care of a little kid. I’m always getting onto him to not do something or to do something. I know none of this is his fault, but dealing with it is very stressful and overwhelming. I get angry with him and then I feel guilty. In addition to having FTD , he has also has PBA ( excessive laughing). When he laughs it’s like a kid laughing at you. He can embarrass you. My loved one is slowly having his life taken away from him. Not being able to do the things he loved to do and just everyday things. Having a support system is very important, having patience and understanding is a must, but very challenging. Life is so very different now, but having God in our lives helps. We just try and take one day at a time.
As Paula mentions, FTD with ALS creates additional challenges for caregivers and it is important to have a strong support system and for caregivers to be able to acknowledge the changes in the behavior and thinking of their loved one.
Some helpful recommendations for caregivers include:
Simplify communication with the affected person. Break up sentences into short phrases. Ask yes/no questions. Slow down when speaking.
Provide supervision and accompany the person to all appointments to make sure information is accurately relayed and retained.
Set realistic expectations for the person with ALS and set realistic expectations for yourself. If your requests introduce frustration, irritability or withdrawal for either you or the person with ALS, your expectations need to be modified and most likely simplifed to meet the needs of your loved one’s current thinking abilities. Also, if you are feeling overwhelmed do not wait until you are feeling underwater. Think ahead to what options you have for help or time away, if needed.
Educate providers and caregivers about where to set expectations for your loved one. In an ideal world, all healthcare providers would be on the same page about what to do and how to accomplish it. But many healthcare provides outside of dedicated ALS clinics may not have much experience with ALS/FTD and how it affects people.
Continue to enjoy activities that bring joy and can be conducted safely, while refraining from activities the result in stress or risk of safety or liability.
While FTD with ALS is rare, current research suggests that up to 50% of people with ALS might experience some degree in change in thinking and behavior. It is important for all who are impacted by ALS to know that cognitive impairment is possible and how to help those affected adjust to the changes. For more information on FTD visit The Association for Frontotemporal Degeneration.
In these times we live in — the information age — we have access to more facts and data than ever before, but not everything we read or watch is correct. The Ice Bucket Challenge brought unprecedented awareness to the general public about ALS, but with more exposure also came more misinformation. Below we break down 5 of the most common misunderstood “facts” about ALS.
ALS is a hereditary disease only.
There has been a lot of media coverage recently about the amazing breakthroughs that have happened identifying mutations in genes that cause familial ALS. Familial ALS accounts for about 5-10% of people diagnosed with ALS. In those families, there is a 50% chance that each offspring will inherit the gene mutation and may develop the disease. The majority of people diagnosed with ALS have no family history of the disease.
Only older people are affected by ALS.
Most people who develop ALS are between the ages of 40 and 70, with an average of 55 years old. But there are many cases of people in their twenties and thirties diagnosed with ALS. This “Young-onset” ALS typically refers to patients younger than 45 years old and accounts for about 10% of all cases. The disease has even struck people in their teens.
Brain function in not affected in people with ALS
While it is common for brain function to NOT be affected in people with ALS, approximately 20% of individuals with ALS also develop frontotemporal degeneration (FTD) which is characterized by changes in personality, behavior and speech. Another rare form of ALS, known as ALS-parkinsonism-dementia complex (ALS-PDC) is characterized by signs and symptoms of ALS in addition to movement abnormalities (slow movements, stiffness and tremors) and loss of intellectual function.
If you’re paralyzed with ALS you can’t feel your body.
The latter stage of ALS is commonly described as someone being completely paralyzed. The definition of paralysis is a loss or impairment of voluntary movement, but many people also associate paralysis with a loss of feeling as well as function. This belief may be because many people with many people with brain or spinal cord injuries that are paralyzed also have lost feeling in the part of their body that is affected. People with ALS can still feel their body, even though they can’t control its movement.
ALS affects everyone the same way.
From the initial symptoms to how the disease progresses, ALS can affect people very differently. The first symptoms of ALS can vary widely depending on the type of onset, bulbar or limb. People with limb onset usually notice a weakness in their hands or feet and people with bulbar onset might notice a change in their voice first. The rate at which ALS progresses is also quite different person to person. While, most people with ALS live with the disease for 2 to 5 years, some people may live 10 or more years with the disease.
Powerful blog post from our friend Pat Quinn, courtesy of WebMD.
By Pat Quinn
When you’re diagnosed with a disease that has a life expectancy of 2-5 years, you will do anything to change that. Almost 5 years ago, I was stunned as I heard my doctor say, “It’s conclusive, we can confidently diagnose you with ALS.” It was the most surreal moment of my life. Sure, I had had some crazy twitching in my arms. Yes, my hands had become weak. But, 2-5 years to live? No, that was unacceptable to me. I was only 30 years old! So, after the initial shock wore off, I decided that I was going to fight.
The disease that was supposed to take my strength started turning me into a pit bull. I became obsessed with bringing attention to ALS – local events, community outreach, speaking, networking – I did everything I could think of to make people aware. I can’t lie – I had selfish motives. I wanted to live. I was entering what should have been the prime of my life – I should have been focusing on the joys of finding love and starting a family, not fighting a terminal disease! My fight to add years to my life grew into a passion to fight not just for myself, but for everyone with ALS. That passion got stronger every time I met a new ALS patient. One of those patients was Pete Frates in Boston, whose website I happened to come across in my online searches. Pete was young, a stellar athlete, and diagnosed with ALS a year before me. I emailed, he answered, and soon we formed a special bond that would eventually play a major role in the biggest movement in the history of philanthropy: the ALS Ice Bucket Challenge.
You may not know it, but the Ice Bucket Challenge wasn’t originally created specifically for ALS. It was going around for a number of different causes until one golfer in Florida took the challenge for his cousin’s husband who had ALS. It caught on in that patient’s town, and the power of social media brought it to my attention. (Thank you Facebook!) I will never forget the day I saw a friend from grade school take the challenge and said he was doing it in my honor. I was blown away, but more importantly, it clicked: If this can connect so easily from one individual to another, we might have something here. Excited about the possibilities, I got my closest friends to dump buckets of ice water over their heads and it spread throughout my network – the one I had so worked so hard to build after diagnosis. Pete took the challenge, too, and shared it to his huge network. No one could have imagined what would unfold next. Soon we were right in the middle of a social media phenomenon! Hundreds of likes on every Facebook post. New videos were being uploaded every couple seconds – literally, seconds! It’s one thing to see your family and friends take the challenge. But somehow, every day it got bigger and bigger! The support for ALS I was hoping to create was taking place right before my eyes. As it happened, my head was spinning. It was surreal. Within a week, while you were probably filming your Ice Bucket Challenge in the backyard, so were the biggest athletes, famous celebrities, and even world leaders.
A worldwide movement like the Ice Bucket Challenge doesn’t just happen. It’s not like any of us were sitting in a dark room drawing up blueprints to orchestrate a massive social media movement. I believe that certain things in your life happen for a reason. Not everything, of course (I’m not crazy), but I truly believe parts of your time here on earth happen for a reason. Life presents us with opportunities, and then it’s up to us to make something happen.
Do I believe I was diagnosed with ALS for a reason? YES. Without getting too spiritual, I know God chose me to take on ALS. I have to believe this. It keeps me going. I was young. I was strong. The people I’ve met throughout my life would do anything for me. That kind of network would have the ability to create necessary awareness.
Do I think the Ice Bucket Challenge started in a town 10 to 15 minutes away from where I lived for a reason? YES.
Do I think I reached out to Pete Frates after diagnosis, became friends, eventually leading us to catapult the Ice Bucket Challenge for a reason? YES. There is no doubt in my mind that we met each other to change the course of ALS. It was our job to recognize its potential and make something happen…that’s exactly what we did!
These days, there’s no awkward pause after I tell someone I have ALS. I no longer have to follow up with “you know, Lou Gehrig’s disease,” because people are aware – and that was the reason for it all. The Ice Bucket Challenge allowed me to accomplish what I set out to do: create unparalleled awareness leading to research that will one day make ALS treatable and, God-willing, cured.
I would do anything to go back to being my normal, pre-ALS self. Anything. But I can’t dwell on that. I am facing an undefeated opponent, and I’m going to do everything I can to beat it. I know that ALS may one day take my physical life, but my fight will last forever.
Pat Quinn was diagnosed with ALS (Amyotrophic Lateral Sclerosis) in 2013, a month after his 30th birthday. Since then, Pat has become a strong advocate, explaining to others that having no effective treatments or a cure is not acceptable Pat was at the forefront of the ALS Ice Bucket Challenge, which raised over $ 220,000,000 globally. He has spoken before Congress, at companies like Google and Facebook, and at universities across the country. Pat has received many accolades for his advocacy including a nomination for TIME Magazine Person of the Year. To learn more, please visit www.q4tw.com.
Growing up my chores included: cleaning my room, doing the dishes, putting the laundry away, and suctioning my dad’s throat cannula. The last chore isn’t typical of most kids, but my growing up wasn’t typical. My dad was diagnosed with Lou Gehrig’s disease in October 1993. I was born in May of 1995, and my dad is still kickin’ it, so my relationship with ALS has been longer than most. My childhood and teen years didn’t only include household chores that were different, but also different life lessons.
So, here is what I learned growing up in an ALS household…
I learned about how fragile life is.
Some of the first memories I have as a kid are of me laying on the grass, staring up at the sky, and trying to understand the concepts of life, death, and why we are on Earth. While this isn’t the typical worries of a kindergartner, these concepts are what I had to process through at a young age. I had to understand that life is a gift that needs to be cherished and shared with others. The idea that our time on Earth is fleeting and could change or end at any moment has been a focal point in how I choose to live my life. My sisters and I are all people who try to live each day to its fullest and achieve all that we can, which I know is a direct result of our understanding that life doesn’t always go as planned.
I learned about tenacity.
Tenacity, perseverance, and confidence are the attributes I have seen in my dad as he has battled ALS. Seeing these traits in him while growing up has shaped how I view the struggle of life, and to what extent I let life get me down. Life can throw curve balls at us at times, but doesn’t mean that we can’t stand back up after we’ve been hit. My life isn’t an easy walk, but I can stand tall knowing that nothing can happen where I can’t choose to persevere. My dad used to joke when I would have to give my life quote for school activities that it would be, “build a bridge and get over it,” which I did use for my high school graduation speech. My dad’s fight has shown me that each mountain has a valley, but that the trek back up the mountain is worth it. I know that life can be hard, but that in the grand scheme of things, our lives, even with ALS, are filled with blessings.
I learned what loving another person means.
Being in an ALS household doesn’t end with my dad having ALS. Having a parent who has ALS, also means that I have a full-time caregiver as a parent. My mom has stood next to my dad through all the stages as his mobility decreased, and has taken care of him 24/7 for over twenty-two years. My parents are a testimony that love and marriage aren’t only about the milestones you want to experience with another person, but also about the sacrifices you are willing to make for another person. My parents have sacrificed everything for each other and our family, which shows since they are still married in a society that has a divorce epidemic. My parents aren’t perfect, but they have, through ALS, set a standard for what love is.
And most of all…
I learned to laugh.
My family is always laughing. I would say that my parents have more reason than most people to be angry at the world, and to live a life of negativity. Instead, my parents chose positivity. With each hard time that my family has gone through, we have been there to pick each other up, and enjoy our time together by making fun of each other and the world that we live in. While I would say the downside to this is my crude sense of humor, I know that the upside of being able to laugh anything off and truly enjoy the company of others. Nothing can stop the laughter of my family, not even ALS.
Kelsey Lester, a recent graduate of Missouri State University, served as a communications and marketing intern for our Chapter last summer, and was our Walk to Defeat ALS Facebook Live correspondent this past June. Since getting her bachelor’s degree in May, Kelsey has moved to St. Louis, traveled to Africa, and is preparing to apply to law school.
Nearly three years ago, on August 4th, a peculiar news item showed up on our social media feed at The ALS Association St. Louis Regional Chapter. Someone had posted an article from Shape Magazine that described something called an “Ice Bucket Challenge,” where people across the country were dumping buckets of water on their heads and challenging three other people to do the same thing. It seemed like a fun, interesting way to raise awareness for ALS, so we shared it to our page and asked our Facebook fans, “Have any of you heard of this?” The rest of August became a blur of ice and water – it was incredible.
How it started
The ALS Ice Bucket Challenge – the largest viral social media craze the world has ever seen – was an organic movement started and carried by the community. It was not a planned fundraising or awareness campaign that The ALS Association created – it was instead initiated by people who were impacted by this fatal disease.
Chris Kennedy, a golfer in Sarasota, Florida, was nominated and took the “ice water” challenge, which was, at the time, not connected with any specific charity. Kennedy then nominated a family member whose husband suffered from ALS, Anthony Senerchia, which was the first time the challenge became associated with ALS and The ALS Association.
The “ALS Ice Bucket Challenge” spread quickly on social media, linking to another person battling ALS, Pat Quinn, whose network of friends overlapped with Pete Frates, a young Boston College Baseball player and team captain, who had been diagnosed with ALS in his 20’s. Frates involvement led to participation by Boston’s athletic community, where it really went viral as professional athletes helped it to cross over into the celebrity realm.
From there – well you’re familiar with the rest – it spread across the nation and eventually went global. By the peak of the challenge, which was somewhere near the third week of August, nearly 1.2 million videos – including those by Bill Gates, Oprah, George Bush, Jimmy Fallon, Shaquille O’Neal and others – had been posted to social media. The whole world was talking about ALS.
How it Helped
In addition to the incredible awareness generated by the challenge, the dollars raised made a huge difference to people with ALS – through programs that eased the physical, emotional and financial burdens brought on by the disease. It also re-energized ALS-specific research and accelerated the search for treatments and a cure.
Monies raised tripled annual research spending, leading to a greater understanding of the disease pathway and potential strategies for new therapies. Funding provided by Ice Bucket Challenge donations supported new treatment approaches in clinical trials, along with the discovery of new genes like NEK-1.
Currently, as a result of Ice Bucket Challenge funds, two antisense drugs targeting the two most common inherited causes of ALS are in or entering into clinical trials. We’ve formed two new global partnerships – ALS ONE and NeuroLINCS, and we’re actively funding 180+ global research projects in 11 countries.
At The ALS Association, we’ve increased efforts to educate our legislators about the vital needs of people with ALS and potential changes in laws that affect individuals battling the disease. We allocated Ice Bucket Challenge funds to develop an FDA guidance document, which helped to navigate the regulatory pathway for approval of effective therapies. This document has been credited in helping expedite the process of FDA approval of Radicava by reducing obstacles that would limit access to effective treatments.
Locally, donations from the Ice Bucket Challenge had an immediate impact on people with ALS in eastern Missouri and central and southern Illinois. It enabled us to increase the amount of medical equipment, nutritional supplements, home modification and adaptive devices for people with ALS, as well as doubled the amount of respite care provided to much-deserving caregivers.
We’ve doubled financial support to our area’s only ALS Certified Treatment Center, located at Saint Louis University, and strengthened partnerships with our affiliated clinics. And, in order to better serve our nation’s heroes – who are twice as likely to be diagnosed with ALS as the general public – we are proud to be participating in a National Veterans Administration initiative, collaborating with clinicians in the St. Louis VA hospital system to establish an ALS specific clinic in our service area.
Why it Mattered
While some people labeled the challenge a flash in the pan, a gimmick, or “slacktivism,” to the ALS community, it was no joke. Aside from the incredible awareness and the flood of donations, the unprecedented outpouring of support provided hope for many who were living with an ALS diagnosis. We heard from so many of the people we serve who told us that watching the videos made them feel like “the world was on their side, rooting them on.”
One gentleman in particular, Don Stewart, who had fought ALS for seven years, was so touched by the thousands of people posting their videos to social media that he decided to make his own video to thank everyone who had participated in the challenge. Don, a longtime banjo player before ALS robbed him of his ability to play, needed oxygen to breathe, but decided to go without it for the time it took to express his gratitude.
Don died in 2016 after fighting ALS for nine years. “He may have lost this one battle,” Don’s wife Saundra said, “but we’re still waging the war.”
What you can do
This August, we’d like people to continue talking about ALS. We know lightning doesn’t strike twice, but we’ve seen what an outpouring (pardon the pun) of attention can bring. We’re asking for your help getting people talking again — about support for families, about the need for research, about what a difference those buckets of ice water made.
During the month of August, we’re asking you to re-post your Ice Bucket Challenge videos and photos to your social media accounts using the hashtag #TBIBC.
With your post, please consider sharing a fact or two about the incredible impact that the ALS Ice Bucket Challenge has had for people with ALS and their families.
Thank you to all of you who participated in the ALS Ice Bucket Challenge of 2014 (and in subsequent years). You have truly changed the course of this disease forever. We look forward to seeing your photos and videos throughout August and appreciate your keeping the conversation about ALS on the forefront. We’re grateful for your support of people and families fighting ALS!
An important part of our mission at the ALS Association is to advocate for changes in laws and regulations that affect thousands of people living with ALS and their families. ALS advocacy has resulted in policies that not only advance the search for treatments and a cure, but has also helped to ensure that people living with ALS have access to the healthcare they need and deserve to maintain a higher quality of life. ALS advocates from around the country have been instrumental in passing legislation that expanded veteran’s benefits, increased national data collection through the ALS Registry, and improved insurance and disability coverage for people with ALS. More than $950 million in federal funding has been generated for ALS-specific research since 1998.
Advocacy is especially important now because of the many legislative changes being proposed to healthcare, social assistance, and other areas that impact the lives of people who are living with ALS. Many legislators don’t know about the experiences of someone who is living with ALS or how the disease can impact a family. That’s why your involvement is crucial – as an advocate, you can help educate the decision-makers who are making determinations about the lives of people with ALS.
Not sure where to start? There’s no special training needed; most of what a good advocate does is intuitive and grows out of a few basic principles. Here are a few tips to help you change the laws and policies for people affected by ALS:
Write, email or call your legislator. Express your feelings about the issue; use your own words and include your personal experience. Speak or write from the heart, and ask for a response. One personal story can change a person’s mind and heart.
Utilize the media. Write a letter to the editor of your community newspaper or contact your local anchor. Post something to your social media accounts and spread the word that help is needed on behalf of people battling ALS.
Sign up to become an advocate on our Chapter website, www.alsa-stl.org. Join a community of advocates who have realized significant accomplishments in our nation’s capital through active participation in the process.
Contact our office at 314-432-7257 or by email at firstname.lastname@example.org. We will be happy to help guide you and answer any questions you might have about ALS advocacy.
By continuing to share the ALS story with legislators and putting a face on this disease, advocates can advance legislative and regulatory changes that affect families living day to day with an ALS diagnosis. Decision-making centers around you, the voters. At some point in the legislative process, there will be one letter or one call that breaks the camel’s back and affects change. Make your voice heard and make a difference for people with ALS.