ALS at 27: Baseball Player With Lou Gehrig's Disease Strikes Back - ABC News ABC News ALS at 27: Mass. Baseball Player Strikes Back By Katie Moisse Pete Frates, 27, was diagnosed with Lou Gehrig's disease in March. 0 Shares Pete Frates , a 27-year-old baseball player from Beverly, Mass., was in the best shape of his life. But a wayward pitch to the wrist and the lingering weakness that followed marked the start of a tragic turn. "There were no broken bones, so they started to look for nerve damage," Frates said. Six months of tests would reveal something far worse than a damaged nerve. He discovered in March that he has ALS , a deadly neurological disease that usually strikes people in their 50s and 60s. "The man upstairs, this is his plan for me," said Frates, whose sculpted muscles are destined to waste away, trapping him inside a paralyzed body. ALS, better known as Lou Gehrig's disease after the Yankee slugger, kills the nerves that control movement, relentlessly robbing its victims of the ability to walk, speak, eat and even breathe.

But Frates, a team captain his whole life, is ready to play ball. "Instead of wasting energy on the negative, let's use the energy in a positive way," he said. "Let's raise some awareness; raise some funds." About 30,000 Americans have amyotrophic lateral sclerosis, landing the condition in a category of rare and often underfunded "orphan" diseases. But experts say the number is deceiving because each new diagnosis is offset by someone's untimely death. "One in 500 people will die from ALS," said Dr. Robert Brown, chair of the Department of Neurology at the University of Massachusetts Medical School in Worcester and president of the ALS Therapy Alliance . "And we call it an orphan disease?" It is rare, however, to see ALS in someone as young as Frates, who is statistically unlikely to live past 32. "The sense of tragedy looms even larger when someone is so young," said Brown, who has seen patients as young as 11.

"The irony is that at a time when their muscles are wasting away, we see extraordinary courage and motivation, and what can only be called strength." Frates hopes to use his youth and every ounce of energy to change the face of ALS, and convince the country a cure is worth fighting for. "We need more funding at a federal level," he said, adding that the one treatment approved to slow disease progression offers some patients a few extra months, at most. "If that's all people with ALS have to lean on, it's unacceptable. We need to do more." Frates' father, John Frates, said he can't think of a better person to rally resources for people with ALS, many of whom have been silenced by the disease. "He's always been a leader; a captain," he said, his voice shaking. "It's just the guy he is. It's always about the team." Frates has always been a driven athlete, playing baseball since he was 3. "If he cried, we'd stop [playing]," John Frates said. "So he just never cried." Frates was team captain for Boston College, and even tried out for the majors. "That's the most challenging part of this," John Frates said.

"Watching his body let him down. He played by the rules: no smoking, no drugs, always worked out." But ALS doesn't discriminate. Indeed, some studies suggest that it might even disproportionately affect athletes. "The concept that this disease could be overrepresented among athletes has been in the medical literature for a long time, and no one underscores that concept more than Lou Gehrig," neurologist Brown said. "But the interesting question is: Does athleticism set the stage for motor neuron degeneration, or does that same property that makes a person a great athlete also make them susceptible to the disease?" Young Athlete Strikes Back at ALS In the 11 weeks since Frates was formally diagnosed, he has seen evidence of the disease's swift progression. "My calf muscles and ankles seem to be slowing first, making me a little clumsier than usual," he said. "I'm definitely not running or throwing the ball around as much." And Frates' left hand below the wrist smacked with a speeding ball last summer has lost 90 percent of its function. Some experts think such injuries may act as triggers for a disease that lies dormant in the nervous system.

"It may seem like an everyday encounter, so to speak, but in the right setting it may trigger motor neuron degeneration," said Brown. Or, he added, injuries might signal the earliest symptoms of the disease. "If an ankle gets weak, people might catch their toe when stepping up on a curb and fall," said Brown. "It's still an open question, which came first: the fall or the weakness." Either way, however, Frates was destined to be afflicted with ALS. Researchers have started to uncover the biological roots of the disease, opening doors for new treatments. "I'm cautiously upbeat about where things are going," said Brown, who has long been studying the genetic causes of ALS. "When we understand the cause, we can see how the molecular dominos fall. It turns out there are a number of ways a cell can fall apart, so at least we have the targets." But Frates is short on time and eager for a breakthrough . He hopes more funding will accelerate the drug-discovery process, and he's ready to do his part. Less than two months after his diagnosis, "Team Frate Train," a group of Frates' family and friends, raised $27,000 for New York City's Walk to Defeat ALS.

"I hope it's a microcosm of what will happen in the greater population," he said. "The support I've seen has been overwhelming, and I can't thank everyone enough." Determined to get the word out on ALS across the country, Frates threw the first pitch for the Boston Red Sox at Fenway Park Wednesday as part of Major League Baseball's ALS Awareness Day. "Hopefully, people will see the juxtaposition of me hitting home runs and making diving catches just last year, to limping out there and throwing a pitch from 40 feet instead of the mound," he said. "If they see a 27-year-old guy in the prime of his life dealing with this, maybe it'll get them thinking about what they can do to help." Donations to help fund ALS research can be made through the Pete Frates #3 Fund or through CVS/ALS Therapy Alliance . 0 Shares En español ALS was first found in 1869 by French neurologist Jean-Martin Charcot, but it wasn’t until 1939 that Lou Gehrig brought national and international attention to the disease. Ending the career of one of the most beloved baseball players of all time, the disease is still most closely associated with his name.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. ALS: What Are the Types and Causes? What Causes ALS? ALS is short for amyotrophic lateral sclerosis. You might also have heard it called Lou Gehrig's disease, after the baseball player who was diagnosed with it in the 1930s. A French doctor named Jean-Martin Charcot discovered the condition in 1869. ALS is a progressive disease, which means it gets worse over time. It affects nerves in your brain and spinal cord that control your muscles. As your muscles get weaker, it gets harder for you to walk, talk, eat, and breathe . ALS and Motor Neurons It’s a disease that affects your motor neurons.

These nerve cells send messages from your brain to your spinal cord and then to your muscles. You have two main types: Upper motor neurons: Nerve cells in the brain . Lower motor neurons: Nerve cells in the spinal cord. These motor neurons control all your voluntary movements -- the muscles in your arms, legs, and face. They tell your muscles to contract so you can walk, run, pick up your smartphone, chew and swallow food, and even breathe. ALS is one of a few motor neuron diseases. Some others include: What Are the Main Types? There are two types of ALS: Sporadic ALS is the most common form. It affects up to 95% of people with the disease. Sporadic means it happens sometimes without a clear cause. Familial ALS (FALS) runs in families. About 5% to 10% of people with ALS have this type. FALS is caused by changes to a gene. Parents pass the faulty gene to their children. If one parent has the gene for ALS, each of their children will have a 50% chance of getting the gene and having the disease. What Causes ALS? Researchers still don't know exactly what causes motor neurons to die with ALS.

Gene changes, or mutations, are behind 5% to 10% of ALS cases. More than 12 different gene changes have been linked to ALS. One change is to a gene that makes a protein called SOD1. This protein may be toxic to motor neurons. Other gene changes in ALS might also damage motor neurons. Environment could also play a role in ALS. Scientists are studying whether people who come into contact with certain chemicals or germs are more likely to get the disease. For example, people who served in the military during the 1991 Gulf War have gotten ALS at higher rates than usual. Scientists are also looking at these other possible causes: Glutamate. This chemical sends signals to and from the brain and nerves. It's a type of neurotransmitter. With ALS, glutamate builds up in the spaces around nerve cells and may damage them. The only medication that's approved to treat ALS, called riluzole ( Rilutek ), works by lowering glutamate levels. Immune system problems. Your immune system protects your body from foreign invaders such as bacteria and viruses. In your brain, microglia are the main type of immune cell. They destroy germs and damaged cells.

With ALS, microglia might also destroy healthy motor neurons. Mitochondria problems. Mitochondria are the parts of your cells where energy is made. A problem with them might lead to ALS or make an existing case worse. Oxidative stress . Your cells use oxygen to make energy. Some of the oxygen your body uses to make energy may form into toxic substances called free radicals, which can damage cells. Researchers learn more about ALS every day. What they discover will help them develop medications to treat symptoms and improve the lives of people who have this disease. WebMD Medical Reference Reviewed by Neil Lava, MD on November 06, 2016 Sources What's Als? | So Much So Fast | FRONTLINE | PBS What is ALS and how does it affect the body? A motor neuron at a magnification of 250x (© Visuals Unlimited/Corbis) ALS -- amyotrophic lateral sclerosis -- is a progressive disease that primarily affects motor neurons, the nerve cells that connect the brain to muscles throughout the body. For reasons that remain unknown, the motor neurons of people with ALS degenerate and eventually die , causing the individual to lose the ability to control voluntary muscles.

With some, the condition first weakens and disables muscles in the arms or legs; with others, symptoms first appear in the throat area. In most cases, ALS develops into complete paralysis, including the loss of speech. Because ALS primarily affects motor neurons, most of the body's involuntary functions, such as the heart, the digestive system and the senses, are generally unaffected. The brain's function also remains largely unchanged, though patients may experience mild behavioral effects, such as changes in their reasoning and social interaction skills. ALS ultimately affects an individual's ability to breathe and swallow. ALS typically develops in individuals aged 40 to 70, but it can affect individuals in their 20s or 30s. About 5 to 10 percent of cases, known as familial ALS (FALS), are the result of an inherited genetic defect. But in more than 90 percent of ALS patients -- including Stephen Heywood, profiled in the film So Much So Fast -- the disease develops seemingly at random, with no apparent risk factors. This form is known as sporadic ALS (SALS).

In North America, ALS is commonly referred to as Lou Gehrig's Disease, after the famous New York Yankees baseball player who left the team after being diagnosed with ALS in 1939. He succumbed two years later. Watch a short video from Project ALS illustrating how ALS affects the body. The ALS Association (ALSA) has a good Q&A about familial ALS. How many people have ALS? It is estimated that 30,000 people in the U.S. have ALS at any given time, with approximately 5,600 new cases diagnosed annually. More than 5,000 people die from the disease each year. How is ALS diagnosed? What are the early symptoms? There is no specific test or procedure to diagnose ALS. Rather, doctors generally reach a diagnosis by using a battery of tests to eliminate other possibilities. These tests include: blood and urine sampling; spinal taps; neurological examinations; muscle and/or nerve biopsies; imaging technologies, including MRIs, x-rays and myelograms (an x-ray examination of the spine); and electrodiagnostic tests that measure muscles' and nerves' response to electrical currents.

Doctors will also take a family history in order to help determine if an individual has familial ALS. There is a genetic test to look for the gene mutation linked to FALS; however, only 20 percent of individuals with FALS actually display the mutation, so the genetic test is not in itself conclusive. The most common early symptom of ALS is muscle weakness in the arms, legs, or throat muscles. Individuals may experience difficulty speaking clearly or swallowing. They may be unable to complete tasks that require fine motor skills or may find themselves tripping or abnormally clumsy. Some individuals also experience muscle fatigue, cramps or twitching. Among older ALS patients, the first signs of the disease are often mistaken for the common effects of aging. What is the average life expectancy? ALS is a fatal disease: Most ALS patients die within two to five years of diagnosis. Common causes of death are respiratory failure and/or cardiac problems related to insufficient oxygen. Patients may also suffer respiratory infections, such as pneumonia. Up to 10 percent of ALS patients live for 10 years or more after diagnosis. Some of these are patients who have opted to use a ventilator to assist breathing.

Physicist Stephen Hawking has lived more than 40 years with the disease, though he does not use a respirator. Read more of Hawking's story. Is there a cure? What are the existing treatments? There is currently no cure for ALS and only one FDA-approved drug treatment. The approved drug -- riluzole, approved in 1995 and sold under the brand name Rilutek -- is a twice-a-day pill that targets glutamate, an amino acid made in each cell in the body that carries signals along motor neurons. Though the exact cause of ALS is still unknown, some scientists theorize that people who develop ALS produce an overabundance of glutamate that proves toxic to the motor neurons. Researchers believe that Rilutek may help protect neurons from glutamate, but the drug's exact effects are not fully understood. In the first year of clinical trials, Rilutek appeared to impede the disease's progress and prolong life by an average of three months. However, by the end of the trial's five-year run, there was no significant difference in life extension between patients who received Rilutek and those who received a placebo.

Drug development and clinical trials seeking more and better treatments for ALS are ongoing. Organizations like the ALS Therapy Development Institute , featured in So Much So Fast, are applying new models to the process of drug development in the hopes of speeding up research that could lead to identifying new treatment options for ALS. What devices and technologies are available to help people with ALS? In the beginning stages of ALS, patients may use walkers or canes before progressing to a wheelchair. There are power wheelchairs available that can be controlled by the head or chin or by a "sip-and-puff" mouth device. Transfer boards and patient lifts are also available to help maneuver the patient's body. And there are a multitude of devices -- from elastic shoelaces to silverware aids -- available to help with daily tasks. When swallowing becomes too difficult, patients may opt to have a feeding tube placed directly into the stomach. Through the tube, they are given water and special liquid supplement formulas designed to meet all nutritional needs. After the feeding tube is inserted, patients can still eat orally, so long as they can safely chew and swallow. Some individuals, including Stephen Heywood, make this decision proactively, as weight loss can exacerbate other effects of the disease.

Ventilators are typically used by ALS patients having difficulty breathing. There are several kinds of non-invasive ventilators, such as small devices that fit over the nose and mouth. Patients may also opt for a tracheostomy, a surgical procedure in which a tube is placed into the throat. A mechanical ventilator can be attached to the tube, and the tube can also be used to suction out secretions. Because it is an invasive procedure, some patients opt not to pursue a tracheostomy. More information on the above technologies can be found in the ALSA manual " Living with ALS ." There are many technologies available for patients having problems communicating, from software and adaptations for personal computers (for example, switches or mouth sticks to aid in typing) to dedicated communication devices, which operate through typing or visual scanning. In the film So Much So Fast, Stephen Heywood is seen communicating through a computer program called EZ Keys, which scans through letters and words. By clicking, he can build phrases; a voice synthesizer can then turn the words into speech. Stephen used the device for everything from instant-messaging and e-mail to telling his son Alex when it was time for bed.

Stephen also took part in a clinical trial for a new technology called BrainGate, developed by Cyberkinetics, in which a sensor is placed on the brain's motor cortex (the part that controls movement). The sensor records the brain's electrical signals and then transmits them to a device outside the body, where they are interpreted by special software and fed into a computer. The goal of the study that Steven took part in is to evaluate the quality and usefulness of the participant's ability to move a cursor on the computer screen using their thoughts. V I D E O · View a short video of Stephen using BrainGate. Another explanatory video can be found on Cyberkinetics' Web site. Who else is doing ALS research? While the film So Much So Fast profiles the ALS Therapy Development Institute, founded by Jamie Heywood, there are a number of other research and advocacy organizations: The Muscular Dystrophy Association has a division devoted to ALS research and its Web site has a list of clinical trials currently in production. Though the International Alliance of ALS/MND Associations does not conduct research itself, it maintains a list of international organizations who do. In addition to patient and caregiver education, the ALSA Web site is a good place to find the latest research news .

Project ALS was founded by theater producer Jenifer Estess, her friends and family, after she was diagnosed with ALS at age 35. Eighty percent of the funds raised by Project ALS are directed to research and education, with four main target areas: genetics , drug discovery , stem cell research and disease pathways (the study of how ALS progresses). The Day Laboratory for Neuromuscular Research , run by Dr. Robert Brown, studies the underlying causes and possible treatments for ALS. Scientists there are exploring approaches including gene therapy and stem cell therapy, as well as testing existing drugs for efficacy and looking for biomarkers to help understand how the disease progresses. Researchers at The Robert Packard Center for ALS Research at Johns Hopkins also focus on understanding ALS's underlying causes and developing therapies. Their current approaches include isolating, identifying and cloning genes and mutations that may control the disease's progression. The ALS Center at the University of California, San Francisco is involved in several drug trials and is also working to help establish a national gene bank with blood drawn from ALS patients. Scientists there are also studying possible environmental and genetic risk factors. Why is ALS considered an "orphan disease"? What are others? What are the added obstacles to finding cures for these diseases?

Orphan diseases, also called rare diseases or rare disorders, are conditions that affect fewer than 200,000 people in the U.S. There are approximately 6,000 orphan diseases -- some of the better known ones are cystic fibrosis, Huntington's Disease, and Tourette's Syndrome -- and more are discovered all the time. Although an estimated 25 million Americans have an orphan disease, the drug discovery process is a challenge: The numbers are so few per disease that pharmaceutical companies are unlikely to make a profit after investing in costly research and development. In 1983, Congress passed the Orphan Drug Act , offering tax credits, exclusive marketing rights and other financial incentives to encourage research. Though the act has helped -- in February 2007, the Pharmaceutical Research and Manufacturers of America (PhRMA), the Genetic Alliance and the National Organization for Rare Disorders released a report listing 303 drugs for orphan diseases in clinical trials or awaiting FDA approval -- some private foundations, such as the ALS TDI, have stepped in to aggressively speed up the process.

Lou Gehrig's Disease (ALS) Lou Gehrig's Disease (ALS) Esclerosis lateral amiotrófica What Is Lou Gehrig's Disease? Lou Gehrig's disease is a disorder that's also called amyotrophic lateral sclerosis (say: ah-my-uh-TRO-fik LA-tuh-rul skluh-RO-sis), or ALS. The official name comes from these Greek words: "a" for without "lateral" for side (of the spinal cord) "sclerosis" for hardening or scarring So, amyotrophic means that the muscles have lost their nourishment. When this happens, they become smaller and weaker. Lateral means that the disease affects the sides of the spinal cord, where the nerves that nourish the muscles are located; and sclerosis means that the diseased part of the spinal cord develops hardened or scarred tissue in place of healthy nerves. ALS is often called Lou Gehrig's disease after Lou Gehrig, a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s. People in England and Australia call ALS motor neurone disease (MND).

The French refer to it as maladie de Charcot, after the French doctor Jean-Martin Charcot, who first wrote about ALS in 1869. Lou Gehrig's disease damages motor neurons in the brain and spinal cord. Motor neurons are nerve cells that control muscle movement. Upper motor neurons send messages from the brain to the spinal cord, and lower motor neurons send messages from the spinal cord to the muscles. Motor neurons are an important part of the body's neuromuscular system. The neuromuscular system enables our bodies to move and is made up of the brain, many nerves, and muscles. Things that we do every day — like breathing, walking, running, lifting stuff, and even reaching for a glass of water — are all controlled by the neuromuscular system. Here's how the neuromuscular system works: If you want to make a fist, your brain first sends signals through upper motor neurons to the area in your spinal cord that controls your hand muscles. Then lower motor neurons in your spinal cord signal the muscles in your hand to move and make a fist.

Over time, Lou Gehrig's disease causes these motor neurons in the brain and spinal cord to shrink and disappear, so that the muscles no longer receive signals to move. As a result, the muscles become smaller and weaker. Gradually the body becomes paralyzed, which means that the muscles no longer work. However, someone with ALS, even at an advanced stage, can still see, hear, smell, and feel touch. The nerves that carry feelings of hot, cold, pain, pressure, or even being tickled, are not affected by Lou Gehrig's disease. In some people with ALS, the parts of the brain that allow us to think, remember, and learn also are affected by the disease. continue Rare Among Kids Although this disease can strike anyone, it is extremely rare in kids. According to the ALS Association, most people who develop Lou Gehrig's disease are adults between 40 and 70. Only 2 out of every 100,000 people will get the disease each year. Because it is not contagious, you can't catch ALS from someone who has the disease. Among ALS cases in the United States, 5% to 10% are hereditary, which means the disease runs in certain families.

This is called familial (say: fuh-MEE-lee-ul) ALS. At least 90% of cases are not inherited; this is called sporadic (say: spuh-RAH-dik) ALS. How Is the Disease Diagnosed? Lou Gehrig's disease doesn't always begin or become worse in the same way. The disease is different for every person who has it. In general, muscle weakness, especially in the arms and legs, is an early symptom for more than half of people with ALS. Other early signs are tripping or falling a lot, dropping things, having difficulty speaking, and cramping or twitching of the muscles. As the disease gets worse over time, eating, swallowing, and even breathing may become difficult. It may take several months to know for sure that someone has Lou Gehrig's disease. The illness can cause symptoms similar to other diseases that affect nerves and muscles, including Parkinson's disease and stroke. A doctor will examine the patient and do special tests to see if it might be one of those other disorders.

(It's like using the process of elimination to figure out the answer to a multiple-choice question on a test.) One of the tests, an electromyogram (say: eh-lek-tro-MY-uh-gram), or EMG, can show that muscles are not working because of damaged nerves. Other tests include X-rays, magnetic resonance imaging (MRI), a spinal tap, and blood and urine evaluations. Sometimes a muscle or nerve biopsy is needed. A biopsy is when a doctor takes a tiny sample of tissue from the body to study under a microscope. Examining this tissue can help the doctor figure out what's making someone sick. previous continue How Is the Disease Treated? Currently, there's no way to prevent or cure Lou Gehrig's disease, but a number of treatments are available to people with the disease. Medicines can control symptoms, such as muscle cramping and difficulty swallowing, and other drugs can slow the development of the disease. Physical therapy can help people with ALS cope with muscle loss and breathing problems. Special equipment is also provided when it becomes necessary. For instance, a power wheelchair can enable a paralyzed person with ALS to get around.

A machine called a ventilator (say: VEN-ti-lay-ter) can help a someone breathe. In addition, a nurse or other health assistant may come to the person's home to provide care that the family cannot handle alone. It's normal for family members to feel upset, overwhelmed, and sad if a loved one has ALS. Counseling, as well as support from other family members and friends, can make it easier to deal with the challenges they face. Living With Lou Gehrig's Disease According to the ALS Association, about half of all people with ALS live at least 3 years after they find out they have the disease, and 20% (or 1 in 5) live 5 years or more. As many as 10% will survive more than 10 years. Stephen Hawking has been living with Lou Gehrig's disease for about 50 years — ever since his diagnosis at age 21. He is the most famous long-term survivor of the disease. Born in England, Hawking is a famous physicist who furthered our understanding of the universe. He has written a lot of books, including the bestseller A Brief History of Time.

He has done these things despite being confined to a wheelchair for many years, being able to move only a few fingers, and needing a voice synthesizer and special computer to speak and write. Hawking, who has a wife and three children, once said, "The prospect of a short life made me want to do more. I realized life was good, and there was a great deal I wanted to do." Living with Lou Gehrig's disease is physically difficult, but it is reassuring to know that the mind is not affected. People with the disease can think as clearly as ever, are able to maintain relationships with friends and family, and should be treated respectfully and normally. Communication can be difficult because the disease affects the person's breathing and the muscles needed for speech and arm movement. With patience, the families of patients with ALS can learn to communicate effectively with their loved one. Researchers continue to study ALS as they try to understand why it happens, and how the disease damages the motor neurons in the brain and spinal cord. As they learn more about the disease, researchers can continue to develop new and better treatments. Professor Hawking said, "[ALS] has not prevented me from having a very attractive family, and being successful in my work . . .

I have been lucky that my condition has progressed more slowly than is often the case. But it shows that one need not lose hope." History of ALS History of ALS Jean- Marie Charcot (1825-1893) noted the first reports of the characteristics of ALS in 1874, and named the fatal syndrome based on what he found. He was a noted French neurologist who has been called ï¿½the Father of Neurologyï¿½, and explained how the central nervous system works. Damage to upper motor neurons, which begin at the top of the brain, results in muscle weakness, stiffness, and augmented reflexes. Lower motor neurons start at the base of the brain and spinal cord; injury to these neurons causes muscle atrophy, twitching, weakness, and reduced reflexes. When motor neurons in the brain are affected, muscles for talking, chewing, and swallowing weaken. When lower spinal cord motor neurons are involved, movement is lost in the limbs, neck, and trunk. ALS weakens skeletal (voluntary) muscles. It is usually considered painless. The body is ultimately fully paralyzed; patients must use respirators to breathe.

For some, ALS affects the limbs first, making it hard to move; this is called limb- onset ALS. In others, the symptoms affect the facial muscles, speech, and swallowing. This condition is called bulbar- onset ALS. Many have muscle spasms, cramps, and atrophy. Since ALS is a progressive condition, most will suffer from all of these symptoms. In the US, approximately 5,000 people are diagnosed yearly with ALS. Most are between the ages of 45-65, although many are not. Presently in the United States, about 30,000 people are victims of ALS; most will die within 3-5 years of contracting the fatal syndrome. Ten percent of all known cases are hereditary. Although males are more prone to ALS, the condition has no racial or ethnic boundaries. Drs. Scarmeas, Shih, Stern, Ottoman, and Rowland recently published a scientific article, concluding that subjects with motor neuron diseases were more likely to be slim, or had once been serious athletes. Many famous people in US history have had ALS. For example, Lou Gehrig was a very famous Yankee baseball player and Ezzard Charles was a heavyweight-boxing champion.

We found there were many other such people, such as Catfish Hunter, another baseball player, and many football players as well. Senator Jacob Javits was an avid tennis player. David Niven was a competitive sailor. Even some in the musical field were subjected to ALS; Dimitri Shostakovitch and Charles Mingus both vigorously practiced musical instruments. Stephen Hawking, the famous physicist, has ALS as well. Donate What is ALS? ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. A-myo-trophic comes from the Greek language. "A" means no. "Myo" refers to muscle, and "Trophic" means nourishment – "No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening ("sclerosis") in the region. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body.

The progressive degeneration of the motor neurons in ALS eventually leads to their demise. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe. The motor nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle control. Examples of voluntary movements are making the effort to reach for a smart phone or step off a curb. These actions are controlled by the muscles in the arms and legs. There are two different types of ALS, sporadic and familial. Sporadic which is the most common form of the disease in the U.S., is 90 – 95 percent of all cases. It may affect anyone, anywhere. Familial ALS (FALS) accounts for 5 to 10 percent of all cases in the U.S. Familial ALS means the disease is inherited. In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease. French neurologist Jean-Martin Charcot discovered the disease in 1869.

During the 2014 ALS Ice Bucket Challenge, millions of people started talking about ALS. Keep the conversation going to increase understanding of the disease. View Infographic Recent years have brought a wealth of new scientific understanding regarding the physiology of this disease. There is currently one FDA approved drug, riluzole, that modestly slows the progression of ALS in some people. Although there is not yet a cure or treatment that that halts or reverses ALS, scientists have made significant progress in learning more about this disease . In addition, people with ALS may experience a better quality of life in living with the disease by participating in support groups and attending an ALS Association Certified Treatment Center of Excellence or a Recognized Treatment Center. Such Centers provide a national standard of best-practice multidisciplinary care to help manage the symptoms of the disease and assist people living with ALS to maintain as much independence as possible for as long as possible. According to the American Academy of Neurology’s Practice Paramater Update , studies have shown that participation in a multidisciplinary ALS clinic may prolong survival and improve quality of life. To find a Center near you, visit .

ALS usually strikes people between the ages of 40 and 70, and approximately 20,000 Americans can have the disease at any given time (although this number fluctuates). For unknown reasons, military veterans are approximately twice as likely to be diagnosed with the disease than the general public. Notable individuals who have been diagnosed with ALS include baseball great Lou Gehrig, theoretical physicist, cosmologist, and author Stephen Hawking, Hall of Fame pitcher Jim "Catfish" Hunter, Toto bassist Mike Porcaro, Senator Jacob Javits, actor David Niven, “Sesame Street” creator Jon Stone, boxing champion Ezzard Charles, NBA Hall of Fame basketball player George Yardley, golf caddie Bruce Edwards, musician Lead Belly (Huddie Ledbetter), photographer Eddie Adams, entertainer Dennis Day, jazz musician Charles Mingus, former vice president of the United States Henry A. Wallace, U.S. Army General Maxwell Taylor, and NFL football players Steve Gleason, O.J. Brigance and Tim Shaw.

In ALS fight, Pete Frates' message is loud and clear in Ice Bucket Challenge - darren rovell (@darrenrovell) August 20, 2014 "It doesn't surprise me at all that Pete has been able to bring this kind of energy and enthusiasm to the fight against ALS," said Marblehead native Cory Schneider, the New Jersey Devils goaltender who knew Frates during his three-year career at Boston College. "The Ice Bucket Challenge has been truly amazing. What most people don't know is Pete has worked even harder in the two years prior to create attention and increase efforts to fight this terrible disease. "These past few weeks have simply been the culmination of the hard work by his friends, family, supporters and, most importantly, Pete and his wife, Julie," said Schneider, who posted his own challenge video Aug. 4. "It is extremely gratifying to see, but the fight is nowhere near over." Frates is credited with creating the challenge along with friend and fellow ALS patient Pat Quinn of Yonkers, New York. For many of Frates' friends, the Beverly native was the right man at the right time to take up the gauntlet.

When he was first diagnosed with ALS, commonly known as Lou Gehrig's disease, in the spring of 2012, Frates said the battle against the disease "is everything now. It's my life's work. It's what I believe the big man upstairs has put me here to do." "That's just been my main focus since this started happening, to be a positive influence for other people who have the disease, be a positive influence for family and friends," said Frates. "At the end of the day, I want to be the cliché game-changer. I want to be the guy who shifts everyone's thinking and shifts where the funds are going. Selfishly, I want to give myself a chance but also give a lot of other people opportunity as well." He has never wavered from that commitment. "That's where my wife and I are the most proud of him, but we can't be surprised," said John Frates. "He's always been the leader. He's always been the team captain. He's always put himself before others." NHL left winger John McCarthy said Frates took him under his wing when the two played hockey at St. John's Prep in Danvers.

"Pete's a great guy, and he always looked out for me," said the 28-year-old McCarthy, an Andover native. "Now you see his network of friends and how everyone is so supportive. That speaks to the kind of person Pete is." Steve Gath, organizer of the Young Faces of ALS Corntoss Challenge fundraiser, says Frates has inspired him to help others. "You have to get involved, put yourself out there and do your part. Pete laid that groundwork for me," he said. Brion O'Connor Frates' story is a difficult one to process in its entirety. ALS doesn't recognize courage, willpower or good intentions. It is fatal. There is no known cure and no effective treatment. Frates has had to come to grips with the same prognosis Gehrig faced 75 years ago. He is now confined to a wheelchair, requiring near-constant care. "Am I surprised about the progression of the disease? Of course I am," said John Frates. "I thought a 6-foot-2, 220-pound, ripped, powerful guy could keep this thing at bay while they figured it out.

But what he's done for this movement, in one fell swoop, in one two-week period, has fast-forwarded this disease 75 years. It's incredible." On Saturday, the ALS Therapy Development Institute in Cambridge hosted a Young Faces of ALS Corntoss Challenge to raise funds. First conceived in November, it likely would have been a small-but-worthwhile fundraiser for ALSTDI, a nonprofit research firm founded 13 years ago. Less than a month ago, fewer than 20 teams had signed up. But the event got an unexpected bump from the Ice Bucket Challenge, and by the time the beanbags started flying, it had sold out all 65 slots with 130 competitors. More than 200 people attended the festivities, raising an estimated $25,000. "This is what Pete tried to instill in us," said Gath, a 29-year-old commercial loan officer from Reading who helped organize the Boston Corntoss Challenge. "You have to get involved, put yourself out there and do your part.

Pete laid that groundwork for me." The obstacles facing ALS researchers and fundraisers are formidable, in part because the population suffering from the disease at any given time is relatively small. Currently, roughly 30,000 people in the United States are suffering from ALS in various stages. That number is a testament to just how ruthless the disease is. The average life expectancy of a person with ALS is two to five years after diagnosis, said Carol Hamilton, director of development for ALSTDI. As a result, ALS sufferers don't have a chance to build the community required to make a strong, consistent argument for increased funding. The ALS Ice Bucket Challenge, however, has turned that paradigm on its head. First is the funding component, which always drives research. More importantly, though, is that the challenge is stripping away the mystery that surrounds the disease. According to Dr. Fernando Vieira, director of research operations at ALSTDI, the Internet phenomenon has triggered interest, which has led to increased awareness. "It's been almost surreal to see the world stop, look up and take notice of ALS," he said. "It's been a dream come true." ALS patient Steve Saling of Chelsea watches the corntoss action at Saturday's fundraiser.

Brion O'Connor Arthur Cronin, Frates' uncle, said the Boston professional sports fraternity has also stepped up, with the Bruins, Red Sox, Patriots and Celtics all lending a hand. Die-hard Boston fans, like Andrew Holden, a former South Boston neighbor of Frates, aren't surprised. "The sports community in Boston is massive," said the 29-year-old Holden. "And when they get behind something, remarkable things can happen." The Ice Bucket Challenge has reached far beyond the sports world, with celebrities including Justin Timberlake and Oprah Winfrey and billionaires like Bill Gates dousing themselves in ice water. ALS researchers are encouraged by the outpouring of support that the challenge has generated, but they are also pragmatic. Vieira said that while the viral nature of the Ice Bucket Challenge isn't sustainable, it has succeeded in promoting an understanding of the full scope of ALS. "Now people notice us, and groups like ours, trying to make a difference," he said. "I think we'll have more people engaged over the long haul." Vieira compared the ALS fight to the battle against multiple sclerosis. Once researchers and doctors began to make inroads against MS, patients started to live longer, which in turn provided more voices.

Through the Ice Bucket Challenge, Frates and Quinn are doing the same for ALS, Vieira said. "More people" translates to creating a larger community. A team, if you will. Justin Silvestro of Waltham, who plays third base for the 2013 Intercity Baseball League champion Lexington Blue Sox, recalled a pump-up speech that Frates gave the team last year before a game against Reading at Endicott College. "Pete told us, 'Baseball is a great game, but the best thing you can do is spend time with your teammates, creating that unbreakable bond,'" said the 21-year-old Silvestro. "He said, 'If you get to know the people you play with, you'll be unbeatable.'" Those who have heard Pete Frates' voice have memories that won't fade with time. For those who haven't, they can find his message being repeated by his ever-widening support network throughout the Internet, loud and clear.

Question: What baseball player was diagnosed with ALS disease?
Answer: Lou Gehrig