Amyotrophic Lateral Sclerosis (ALS/Lou Gehrig’s Disease) and Medicare
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects the body mentally and physically by attacking nerve cells in the brain and spinal cord.
It affects the motor neurons in the brain and is characterized by stiff muscles, gradually worsening weakness and muscle twitching, among other symptoms.
ALS is commonly referred to as Lou Gehrig’s disease, so named because of the New York Yankee’s Hall of Famer, who was famously diagnosed with the disease in the 1930s. At times, it is also known as motor neuron disease (MND) or Charcot’s disease.
The condition is one of several motor neuron diseases that are caused by the gradual deterioration and death of motor neurons. Motor neurons are nerve cells that extend from the brain to the spinal cord and muscles throughout the body. They provide vital communication links between the brain and muscles.
With ALS, the upper motor neurons and the lower motor neurons degenerate or die, and stop sending messages to the muscles. When messages can’t get through, muscles can’t function and gradually weaken. They start to twitch and atrophy.
The disease occurs most often in people between 40 and 70 years old. All told, about 5,000 Americans are diagnosed with ALS each year. Overall, about 15-20,000 people may suffer from ALS at any one time. Of these, about 5-10% of diagnosed cases are inherited from a patient’s parents. The exact cause of the other 90% of cases is not known.
Different types of ALS are classified by which motor neurons are affected.
- Classic ALS is the most common and accounts for about 70% of all diagnosed cases. It affects neurons in the brain and spinal cord. Classic ALS can be further subdivided into spinal-onset ALS and bulbar-onset ALS.
- Primary lateral sclerosis (PLS) affects upper motor neurons in the arms and legs and accounts for about 5% of all cases.
- Progressive muscular dystrophy (PMA) affects lower motor neurons in the brain and also accounts for about 5% of all cases.
- Variations of ALS produce symptoms that are limited to single regions in the body and progress more slowly than Classic ALS, accounting for the balance of the remaining cases.
Because of the severe symptoms and high mortality rate of ALS, Medicare has special rules in place to provide health insurance coverage for people impacted by this disease.
ALS is a progressive disease, and there is no cure. But the disease can be slowed through medication, and treatments are available to help lessen the symptoms.
Medicare eligibility for ALS and ESRD patients
How do I sign up for Medicare with ALS?
What is the life expectancy of someone diagnosed with ALS?
How much does ALS treatment cost?
Where can I get more information on ALS?
ALS and Medicare
If you’re 18 or older and have been diagnosed with ALS, you should qualify for Social Security Disability Insurance (SSDI) benefits. Social Security has established specific criteria for determining if someone has ALS, and if those criteria are met, the patient is automatically eligible for SSDI benefits.
There is no single specific test that establishes if someone has ALS. The diagnosis must be made following accepted practices and clinical procedures. Your medical records must reflect this and include a specific ALS diagnosis.
Social Security will want a history of your symptoms and neurological test results that are consistent with an ALS diagnosis. They will also want to see the results of electrophysical and neuroimaging tests to rule out other conditions, and electromyography or nerve conduction studies that may also support your diagnosis.
When you are eligible for SSDI benefits due to ALS, you automatically become eligible for Medicare benefits as well.
With SSDI benefits, you have to wait five months to start collecting benefits. But your diagnosis can be fast-tracked under Medicare’s Compassionate Allowances program, and you should have a decision on benefits within a few short weeks.
ALS is also a qualifying condition for a Chronic Special Needs Plan or CSNP. CSNPs provide additional perks like low out-of-pocket costs and the ability to change plans during any time of year with a Special Enrollment Period.
What does Medicare cover?
Everyone who enrolls in Medicare will begin with Part A and Part B coverage known as Original Medicare. Part A includes hospital, hospice, skilled nursing, home health.
Part B includes preventative services, mental health, lab tests, x-rays, emergency transportation, medical equipment.
After you have enrolled in Original Medicare, you can also enroll in Medicare Advantage (Part C). These plans can include physical fitness, extra home health benefits, non-emergency medical transportation, meal delivery, and Prescription Drug coverage (Part D) to help pay for needed medications.
Does Medicare Cover Home Health Care for ALS patients?
Yes. Medicare benefits coverage can include skilled nursing, physical therapy, speech therapy, occupational therapy, home health aides, medical social services, and medical supplies up to 35 hours per week as long as qualifying conditions are met.
Those conditions include:
- A patient must have needs that are “medically reasonable and necessary” and “intermittent.” Intermittent means “you need care at least once every 60 days and at most once a day for up to three weeks.”
- Home care services must be ordered through a written plan of care by a physician.
- The patient must be homebound, meaning they don’t have the ordinary ability to leave home without assistance.
- Care must be provided by a Medicare-certified home health agency.
- The care plan must be recertified every 60 days.
Medicare eligibility for ALS and ESRD patients
Patients with ALS or end-stage renal failure (ESRD) can receive Medicare benefits regardless of age, and without waiting for 24 months of disability payments before Medicare benefits begin, which is the case for other kinds of disability-related Medicare eligibility.
You are eligible for Medicare benefits on the first day of the fourth month you receive dialysis (or immediately if you receive home dialysis) or as soon as you’re hospitalized for a kidney transplant. However, if you stop a self-dialysis program and start going to a dialysis center instead, your Medicare benefits will stop. You will have to wait until you complete four months of dialysis treatment to receive benefits again.
Most Medicare Advantage plans are currently not available for ESRD patients, but this will change in 2021 as part of the 21st Century Cures Act. At that time, Medicare Advantage plans will be guaranteed issue for all Medicare beneficiaries, including patients with ESRD.
However, no rules are limiting Medicare Advantage access for people with ALS. If you have ALS and you’re enrolled in Medicare Part A and Part B, you can enroll in an available Medicare Advantage plan in your area.
Also, for patients under 65 who have ALS or ESRD, Medigap plan availability varies from state to state.
There are 27 states that require Medigap plans to be guaranteed-issue for ESRD patients when they first become eligible for Medicare. Premiums are higher than they would be if a person were enrolling in Medicare because they were turning 65.
In the other 23 states, a Medigap insurer can reject an under-65 applicant who has become eligible for Medicare due to ESRD.
For ESRD patients who have private health insurance in addition to Medicare, private insurance is the primary payer for the first 30 months. After that, Medicare will become the primary payer.
How do I sign up for Medicare with ALS?
To apply for SSDI benefits, call the Social Security Administration at (800) 772-1213.
If you qualify, you can also apply for a railroad disability annuity by calling your local Railroad Retirement Board field office.
You will make an appointment to complete an application by telephone or at your local SSA office. You can also apply for SSDI benefits online. You should make contact as soon as you are diagnosed with ALS.
Social Security has an expedited process for processing SSDI and SSI benefits for terminal illness cases like ALS. This is known as the Terminal Illness Program (TERI).
An applicant does not need to state on their application that they have a terminal illness. A field office representative who reviews the application will send the claim to the TERI program for quick and compassionate processing when a claimant applies for disability due to ALS or AIDS.
TERI treatment also applies in with other terminal illnesses, including several types of cancer, chronic heart failure, comatose for 30 days or longer, and patients waiting for various organ transplants.
If you’re approved for SSDI benefits, or you get a Railroad Disability annuity check based on ALS, your eligibility for Medicare will begin immediately. You will automatically get Medicare Part A, and Part B benefits the month your disability benefits begin.
When you get Original Medicare coverage, you can also choose to enroll in a Medicare Advantage Part C plan and a Part D prescription drug plan.
ALS Symptoms
Initial ALS symptoms can be subtle at first. Some of those early symptoms will include:
- fasciculations (muscle twitches) in the arm, leg, shoulder, or tongue
- muscle cramps
- tight and stiff muscles
- weakness of the muscle that affects an arm, leg, neck or diaphragm
- slurred and nasal speech
- difficulty chewing or swallowing
- loss of tongue mobility
- overactive gag reflex
Symptoms may first appear in a hand or arm and will cause someone difficulty in performing simple tasks like buttoning a shirt or turning a key in a lock.
When symptoms begin in the arms or legs, it is known as limb onset ALS.
When people first notice problems swallowing or with speech, this is known as bulbar onset ALS.
In most cases, the disease begins in the hands, feet, arms, and/or legs before spreading to other parts of the body.
Symptoms will gradually get worse and develop into more obvious signs of weakness or atrophy. Near the end of ALS’s progression, people have problems chewing, swallowing, speaking, and breathing, tripping, and falling start to take place routinely.
Many patients will begin repeating phrases or gestures, show apathy, and loss of inhibition. Language, social cognition and verbal memory problems are common as well.
Half of ALS patients also suffer from emotional lability, which is crying or laughing for no reason.
Neuropathic pain caused by nerve damage can produce spasticity, muscle cramps, muscle contractures (permanent shortening of a joint or a muscle).
Sensory nerves are generally not affected, meaning that most ALS patients maintain hearing, sight, touch, smell, and taste.
People with ALS burn calories faster rate than normal. Because of this, they tend to lose weight rapidly and can become malnourished.
Ultimately, a person will lose the ability to initiate and control all voluntary movement. Bladder and bowel functions and eye muscles are spared until the final stages of ALS.
Towards the end of the disease’s progression, people will often have difficulty chewing, swallowing, speaking, and even breathing.
In later stages, because eating is difficult, choking, or aspirating food into the lungs is a danger. Aspiration pneumonia can develop, and the insertion of a feeding tube to maintain body weight may be required. Memory loss and dementia may become frequent.
As muscles around the diaphragm weaken, lung function will decrease. As a result, most people with ALS die from respiratory failure or pneumonia within three to five years after symptoms first appear.
How is ALS diagnosed?
ALS can be challenging to diagnose because early symptoms can point to several possible diseases. As a result, diagnosing ALS often starts by ruling those other diseases out.
In addition to an extensive medical history, a suspected ALS patient initially may be given a neurological exam and x-rays. Other diagnostic testing may include nerve and muscle biopsies, spinal taps, and electrodiagnostic tests.
A cervical spine myelogram may also be performed, allowing a doctor to use x-rays to look closely at the spinal lining, spinal cord, and nerve roots. A spinal tap involves an injection into the spine to remove fluid that is tested for nerve disorders and infections.
An electrodiagnostic test records electrical activity in the muscles and nerves to reveal any nerve damage. A nerve conduction study (NCS) measures the electrical activity of the nerves and muscles by assessing the nerve’s ability to send a signal to other nerves or muscles.
A magnetic resonance imaging (MRI) test using a magnetic field and radio waves to produce detailed images of the brain and spinal cord are also common tests for people suspected with ALS.
An MRI can reveal other problems that may be causing ALS-like symptoms. Those might include a spinal cord tumor, a herniated disk in the neck that compresses the spinal cord, a cyst in the spinal cord, or abnormal wear affecting the spine in the neck.
Some infectious diseases can also produce symptoms that mimic ALS. HIV, human T-cell leukemia virus (HTLV), polio, and West Nile virus sometimes cause ALS-like symptoms. Other neurological disorders such as multiple sclerosis, post-polio syndrome, multifocal motor neuropathy, and spinal and Kennedy’s disease also produce similar symptoms.
When these diagnostic services are considered necessary and viable, you will be covered by Medicare Part B. You could be responsible for a 20% copayment. Still, if you have a Medicare Advantage plan, it may cover some or all of the 20% portion you would otherwise be responsible for.
Neurologic tests will be given at regular intervals to compare results against an initial baseline, checking of progressive muscle weakness, muscle atrophy, and spasticity.
Also, because an ALS diagnosis can be difficult, getting additional neurological opinions are common.
Is ALS genetic?
In some cases, yes. But only about 5-10% of ALS cases are considered “familial,” meaning a mother or father with ALS will pass a mutated gene to their children. A doctor will study your family history and your DNA to better determine if you are at an increased genetic risk for developing ALS.
Familial ALS usually only requires one parent to carry a mutated gene responsible for the disease. Overall, more than a dozen mutated genes have been shown to cause ALS.
Mutations in more than a dozen genes have been found to cause familial ALS. Among these, the most common gene related to ALS is “chromosome 9 open reading frame 72,” or C9ORF72. This same gene has also been linked to frontotemporal dementia (FTD).
The vast majority of cases are considered “sporadic.” That means environmental factors may be the cause for those who contract ALS.
What causes ALS?
In addition to a possible genetic cause for ALS, researchers believe there are three other primary ways that a person can develop the disease. They are:
- Higher-than-normal levels of glutamate, a neurotransmitter in our brains
- An overactive immune system that attacks its own cells
- Proteins that the body has not processed correctly. Proteins serve vital functions in cells, and when they are abnormal, they can damage or kill nerve cells and cause ALS.
Certain populations are at a higher risk for developing ALS. They include:
- Symptoms most commonly develop between the ages of 55 and 75, although ALS can develop at any age.
- Men are slightly more likely to develop ALS, but that difference disappears the older a person becomes.
- Caucasians and non-Hispanics and the most likely to develop ALS.
Some studies indicate that military veterans are as much as twice as likely to develop ALS. Scientists theorize that exposure to pesticides, lead, and other toxins may be the reason. The U.S. Department of Veterans Affairs recognizes ALS as a service-connected disease.
What is the life expectancy of someone diagnosed with ALS?
Part of the life expectancy of someone with ALS depends on how far along the disease has progressed with a diagnosis is first made. In general, a person will live from two to five years after the initial diagnosis. Specifically, ALS has a mortality rate of 50% within 3 years.
There are exceptions, with about 10% of those diagnosed who live 10 years or more. The downside is that quality of life will diminish quite a bit during the latter stages of ALS.
The most famous and unusual survivor of ALS was Cambridge University physicist and cosmologist Stephen Hawking who lived for 55 years following his diagnosis.
When an early diagnosis is made, it improves the quality of life and extends survival time because of the increased efficacy of prescription drugs.
Finding a cure for ALS
There is no cure for ALS, but research has continued an intensified in recent years.
In 2017, edaravone and radicava became the first FDA-approved drugs that slow down ALS progression.
ALS has also gotten more attention aside from notable sufferers like Lou Gehrig and Stephen Hawking.
In 2014, the Ice Bucket Challenge promoted by the ALS Association encouraged people to pour a bucket of ice water over themselves and share a video of the results. The campaign went viral and let to a large influx of donations and attention to finding a cure for ALS.
Scientists are looking for answers to help them understand why some factors selectively trigger motor neurons to degenerate in ALS. From this, they hope to discover effective treatments to halt the processes that lead to cell deaths.
Research is also continuing on ALS gene mutations that also lead to the degeneration and destruction of motor neurons. Focusing on mutated and cellular defects, stem cell research, and how proteins interact on a cellular level, are also making strides towards a cure.
Epigenetics, which is the study of switching genes “on and off,” is also showing promise as well. But there is still a long way to go.
ALS treatment options
There is no cure for ALS, but there are treatment options.
Four drugs are currently FDA-approved to treat ALS. They are Riluzole, Nuedexta, Radicava, and Tiglutik
The most common treatment is prescribing the drug Riluzole, which slows ALS’s progression and helps to control symptoms. Although it has been approved by the FDA, unfortunately, it is expensive and does not always work. Clinical trials indicate that Riluzole prolongs survival by a few months.
The FDA has also approved Edaravone, but it may not be effective in the later stages of ALS.
Other drugs are used to treat specific symptoms—for example, Baclofen and Diazepam control muscle spasms. Gabapentin is prescribed to help control pain.
Because breathing and feeding are problematic, feeding tubes are often used, along with drugs like Trihexyphenidyl and Amitriptyline. Doctors may also prescribe a breathing device or perform a tracheostomy to improve breathing.
Drugs are also commonly prescribed to help people with stiffness, excess saliva and phlegm, pain, depression, sleep disturbances, and constipation.
Physical therapy using range-of-motion exercises and low-impact aerobics, such as walking or swimming, helps to strengthen unaffected muscles and improve cardiovascular health. Patients are also often given braces, walkers, and wheelchairs that help them stay mobile and conserve energy.
As ALS progresses, speech therapy may provide a benefit by teaching adaptive strategies and using computer-based speech synthesizers. As an adjunct to this, some patients also use voice banking while they can still speak. This involves storing their own voices that can later be used in speech synthesizers.
Nutrition is a critical part of treatment as well. Since those with ALS burn more calories than normal, a proper diet is essential to maintaining weight and eating foods that are easy to swallow. At some point, a feeding tube may also be introduced to provide adequate nourishment and reduce the risk of choking and pneumonia.
When lungs and muscles weaken, a noninvasive ventilation (NIV) apparatus may be introduced. NIV is usually a mask that covers the mouth and nose to maintain normal oxygen and carbon dioxide levels. Later, a mechanical ventilator may be introduced to help inflate and deflate the lungs.
How much does ALS treatment cost?
For most people, Medicare Part A is free. The premium for Medicare Part B is $144.60 for 2020. Keep in mind that Original Medicare only covers 80% of treatment costs. There will also be copays and coinsurance costs as well.
However, with ALS, the treatment costs can be much higher.
One study that followed a single patient’s direct costs over the 10-year disease duration showed accumulated costs were $1,821,245 (adjusted for the 2013 consumer price index). In this case, total direct costs were paid through a combination of insurance, out-of-pocket by patient/family, and charity.
Every case of ALS is different, and as the disease progresses, costs will go up. This study was in line with the average annual estimated costs of between $150,000 and $200,000 per patient based on another study.
To offset these costs, patients often look to a combination of private health insurance, Medicare Part C and Part D coverage, and in some cases, Medicaid coverage.
Prescription drug coverage is critical because the cost for Riluzole alone can range from about $2,000 to $9,000 or higher.
The cost for a single infusion of edaravone is about $1100 for a single infusion, so the annual cost for this drug is approximately $150,000 per patient per year.
In other words, from the time of ALS onset until death, the costs for treatment, palliative care, and other medical expenses are substantial.
Where can I get more information on ALS?
For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute’s Brain Resources and Information Network (BRAIN) at:
Brain Resources and Information Network (BRAIN)
P.O. Box 5801
Bethesda, MD 20824
800-352-9424
The ALS Association
275 K Street N.W., Suite 250
Washington, DC 20005
202-407-8580
ALS Therapy Development Institute
300 Technology Square, Suite 400
Cambridge, MA 02139
617-441-7200
Les Turner ALS Foundation
5550 West Touhy Avenue, Suite 302
Skokie, IL 60077-3254
847-679-3311
Prize4Life
P.O. Box 5755
Berkeley, CA 94705
617-545-4882
Project ALS
801 Riverside Drive, Suite 6G
New York, NY 10032
212-420-7382
855-900-2257
Muscular Dystrophy Association
222 S. Riverside Plaza, Suite 1500
Chicago, IL 60606
800-572-1717
U.S. National Library of Medicine
National Institutes of Health/DHHS
8600 Rockville Pike
Bethesda, MD 20894
301-594-5983
888-346-3656
National ALS Registry and the National ALS Biorepository
The National ALS Registry collects, manages, and analyzes data about people with ALS in the United States. Developed by the Center for Disease Control and Prevention’s Agency for Toxic Substances and Disease Registry (ATSDR)
NIH NeuroBioBank
The National Institute of Health NeuroBioBank involves several brain banks across the U.S. that supply scientists and researchers with tissue from people with neurological and other disorders.
