Resources
Resources for ALS Patients and Caregivers
Dyspnea (shortness of breath): As the diaphragm and respiratory muscles weaken, breathing will require more effort. Breathing while lying flat may become difficult as well. Cough flow weakens, and it may be difficult to expel mucus. There is equipment that can assist with these symptoms. Report shortness of breath, difficulty coughing, and headaches upon waking to your neurology team or respiratory therapist.
Dysphagia (difficulty swallowing): As the tongue, cheek, and esophageal muscles weaken, chewing and swallowing become more difficult. A major risk associated with this is aspiration, or inhaling food or liquid into the lungs, which can cause severe pneumonia. Coughing and choking during meals should be reported to the neurology team, speech therapists, or dietitian.
Dysarthria (difficulty speaking): The muscles that support speech also weaken as ALS progresses. There are many alternatives depending on your individual needs and abilities. Please notify your speech therapist and neurology team as your communication becomes impaired so that we can ensure you have the technology and training you need to effectively communicate.
Weakness and reduced mobility: ALS causes progressive weakness of the arms and legs. This results in need for equipment such as orthotics, assistive devices (i.e. Cane, walker), and eventually a power wheelchair. Please relay difficulties with walking, standing, and transferring to your physical therapist and ALS team.
Cramping: Although not all patients experience it, many patients with ALS have painful cramping of the arms or legs. If you have cramping that is persistent, severe, or disturbing your sleep or quality of life, muscle relaxants can be very helpful in reducing cramping to a manageable level. Please report cramping to your neurology team.
Fasciculations: Fasciculations are painless twitches of the muscle that commonly occur in patients with ALS. There is no medication that is effective in reducing fasciculations, but if fasciculations are impairing your ability to sleep, please report this to your neurology team for a sleep aid.
Pseudobulbar affect (uncontrollable crying or laughter): Pseudobulbar affect (PBA) is a symptom of uncontrollable or inappropriate outbursts of crying or laughter. It does not occur in all patients with ALS, but PBA can sometimes be distressing for patients that do experience it. Nuedexta is an effective treatment for PBA and can be prescribed for most patients if PBA is disturbing their quality of life. Report symptoms of PBA to your neurologist.
Sialorrhea (excessive saliva): Excessive saliva or mucus occurs frequently in patients with ALS. Suction devices can be helpful, particularly in patients with difficulty swallowing, but there is also a variety of medications that can be used to proactively prevent excessive saliva. There are also procedures, such as Botox injections or salivary gland irradiation, which can be done for patients who do not respond to or who cannot tolerate medications.
Cognition: ALS can cause changes in cognitive and behavior. Most of these changes are mild, but some cases of ALS are associated with more significant dementia. Cognition will be screened at ALS Clinic visits and if significant abnormalities are identified you may be referred for additional neuropsychiatric testing.
Although there is no cure for motor neuron disease, several medications help to treat the symptoms and potentially slow the progression of the disease.
| Medication | What is it? | How does it work? | Dosage | Common Side Effects |
| Riluzole |
Riluzole is FDA approved and used to prolong the time to a ventilator in ALS. It has been demonstrated to slow disease progression by 10%.
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It decreases level of glutamate. Excessive exposure to glutamate may be toxic to nerve cells causing motor neuron death.
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One 50mg tablet by mouth every 12 hours:
• Take on empty stomach
• Take one hour before or two hours after meals
• To gain most benefit do not skip a dose. Take the dose as soon as you remember, on an empty stomach. If it is close to the next dose, skip the missed dose and go back to normal schedule.
• Do not take two doses at the same time.
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•Upset stomach Riluzole may cause liver function impairment. Blood work will be done prior to initiating treatment and 4 weeks after starting the medication.
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| Nuedexta |
Nuedexta is FDA approved to treat Pseudobulbar affect (PBA). ALS and some neurologic conditions may affect areas of the brain involved in crying and laughing. This causes frequent, involuntary episodes of crying and/or laughing, which do not match the feeling the person has. Anecdotal reports of improved speech and swallowing have also been studied.
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The exact way that Nuedexta works to treat PBA is unknown. This medication is a combination of dextromethorphan and quinidine. Dextromethorphan is a cough suppressant.
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• 1 capsule by mouth once daily on Day 1-7
• Days 7 onward, 1 capsule every 12 hours. May be administered with or without food.
• If you forget, take the dose as soon as you remember. If it is close to the next dose, skip the missed dose and go back to normal schedule.
• Do not take two doses at the same time.
• Avoid grape juice and tonic water.
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• Diarrhea
• Dizziness
• Cough
• Peripheral edema.
EKG prior to initiating treatment may be required due to potential risk of QT prolongation.
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Edaravone (Radicava)
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Radicava is an FDA approved medication that demonstrated efficacy in slowing ALS disease progression by 33% in select individuals with ALS. It does not restore function or cure ALS and there are no typically perceptible differences in day to day activities when taking Radicava. |
Radicava is an ongoing infusion treatment thought to mitigate the effects of oxidative stress, which plays a role in cell death. | Medication taken by mouth |
• Headache
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There are many, many over the counter or alternative off-label supplements and treatments on the market for ALS.
Please be wary of supplements that:
1. Promise a cure
2. Are expensive, out-of-pocket and experimental (you don’t pay to be in research studies)
3. Are not delivered by an experienced medical professional.
Below is a very short list of supplements that are very commonly inquired about. For additional, updated information on supplements, what they do and if they may be effective for ALS, please visit www.alsuntangled.com.
| Supplement | What is it? | How does it work? | Level of Evidence (per ALS Untangled) |
Side Effects |
| L-serine |
A nonessential amino acid; exists in two forms: L-serine and D-serine. Both forms can enter and support the central nervous system. It can improve brain function and body strength.
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Some investigators agreed that cases of sporadic ALS may be due to exposure to a chemical called BMAA (B-Methylamino-L- alanine). L-serine can impede the toxic effects of BMAA in the brain cells and the nervous system. It prevents damage caused by BMMA exposure. | Grade D for both categories: cases and trails. There is not enough evidence that L-serine is effective in ALS, nor that it can reverse damage that has already occurred. However, patients with ALS that took 30g daily self-reported improvements | GI side effects were reported, including bloating, nausea, and loss of appetite. No other side effects were reported. |
| Curcumin | Curcumin is a derivative of the spice turmeric. It has anti-inflammatory properties and it increases the antioxidant capacity of the body. | Curcumin is inhibits nuclear factor kappa B (NF-κB), acting as an anti-inflammatory and potentially slowing the progression of ALS. Curcumin has antioxidant effects neutralizing free radicals, which may cause cell damage in ALS. |
Cases: Grade B. Only one patient out of eight patients experienced "slight" improvement.
Trials: C. There is one small study that showed some benefits of curcumin. Clinical trials have been conducted with oral curcumin at doses up to 12g daily.
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Minimal side effects were reported. Most common side effects include abdominal pain, nausea, indigestion, constipation, diarrhea and hot flashes.
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Methylcobalamin (B12)
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Methylcobalamine is a form of vitamin B12, which is important in DNA synthesis and regulation, metabolism, blood formation and nervous system function. Deficiency can lead to fatigue, anemia, impaired memory, peripheral neuropathy and spinal cord degeneration. |
Methylcobalamine has shown to reduce oxidative stress, glutamate toxicity and cell death. It may also reduce homocysteine levels, which can be toxic to motor neurons.
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Grade D: only few patients reported effectiveness, most patients did not find it beneficial. Doses ranged from 2500mcg to 25mg daily. The optimal dose and route of administration has not been determined.
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Pain and bruising at injection site. Some of over-the-counter B supplements contain B12 and also B6; at larger doses can be harmful to the nervous system.
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| Basis | Basis is a dietary supplement that contains two main ingredients: nicotinamide riboside and pterostilbene. Basis promotes cellular health. |
Nicotinamide riboside affects metabolism and regulates response to oxidative stress. Pterostilbene is a natural antioxidant. |
Grade U: due to absence of trials. The recommended dosage is two capsules daily.
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No adverse effects reported. Patients taking high doses may experience mild diarrhea.
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| Sodium Phenylbutyrate + TUDCA (AMYLX0035) |
Sodium Phenylbutyrate is a prescription drug used in treatment of metabolic disorders. TUDCA is a water-soluble bile acid that is naturally produced in small amounts in the body.
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The combination of Sodium Phenylbutyrate and TUDCA is thought to reduce neuronal death to slow disease progression. Sodium Phenylbutyrate works in the Endoplasmic Reticulum (ER) and TUDCA works in the Mitochondria. These are important structures of a cell. The ER is the part of the cell that makes protein, and mitochondria is the part of the cell that makes energy. |
This was a phase 2 safety trial. Typically, a phase 3 trial would be necessary to determine the effectiveness of Sodium Phenylbutyrate and TUDCA.
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Gastrointestinal side effects occur:
• Nausea
• Abdominal Pain
• Diarrhea
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ALS Prevalence
According to the ALS National Registry, ALS was most common among whites, males, and persons aged 60–69 years. The age groups with the lowest number of cases were people aged 18–39 years. Males had a higher prevalence than females overall and across all data sources.
Living with ALS: What is ALS?
Managing ALS Multidisciplinary Care (MDC) – who is in it ? One Stop Shop
When treating those with ALS, many different specialists are often needed to treat various organ systems or symptoms that may arise. This is done by forming a Multidisciplinary Care (MDC) team, which may contain your ALS Neurologist/ ALS Nurse Practitioner. Respiratory Therapists, Speech Language Pathologist, Nurse, Physical and Occupational therapists, Palliative Care, Neuropsychologist, Social Worker, ALS Liason, and more depending on your symptoms. Your MDC team is to act as a “one-stop shop” for evaluations and treatment of the symptoms that you may experience living with ALS.
Why Multidisciplinary Care?
According to a recent study published in 2019, “Multidisciplinary care increased the survival of ALS patients in our study population. Timely use of respiratory support and gastrostomy are fundamental aspects of this benefit”(Healthcare.J,2019)
Living with ALS: Adjusting to Swallowing Changes and Nutrition Management
What is a Feeding Tube: Information Guide
Current ALS Trials at Holy Cross Health
Holy Cross Health believes patients deserve the chance for a cure. Holy Cross is home to multiple ALS studies. They are also an offical affiliated site of the Healy Platform Trials which is one of the largest ALS trials done today. For a list of current trials, visit our Clinical Trials page.
Reliable Websites
Caregivers are individuals who provide physical, emotional, psychological support to persons with ALS (pALS). A caregiver can be a significant other, spouse, partner, child, hired aide, etc. Caregivers dedicate their time and energy to sustain and support quality of life for people diagnosed with ALS. Caregivers’ ability to cope with caring for a person with Motor Neuron Diseases/Amyotrophic Lateral Sclerosis oscillates between positive and negative aspects of caring, being at times active, at times passive (Weisser.F, 2015).”
When considering the emotional and physical toll of a caregiver, it is imperative to understand that caregiver grief and burnout is common. It is okay to ask for and to accept help. We encourage our caregivers to reach out for support. Holy Cross and the ALS Association offer ALS Virtual Caregiver Support Groups to meet other caregivers in a comparable situation. The ALS Team at Holy Cross believes that emotional support to caregivers is integral to comprehensive ALS care.
Caregiver Burnout
Caregiver burnout occurs when a caregiver takes on the emotional, physical and psychological toll of the pALS. Caregivers tend to neglect themselves and their own health due to their dedication to their loved one. The stress of this lifestyle leads to the caregiver burning out.
Symptoms of Burnout
Symptoms of caregiver burnout may include:
- Alcohol/drug abuse
- Depression
- Panic attacks
- Anxiety
- Lack and loss of self-esteem
Remember: You are not alone and it is okay to ask for help.
Donate
We encourage you to support the Institute and ALS Clinic as part of the larger Holy Cross comprehensive fundraising campaign. Your donation can make an impact on the previously mentioned areas as well as capital and equipment for renovations and innovations within the Phil Smith Neuroscience Institute.
For more funding and naming opportunities, please do not hesitate to contact us! If you would like to make a charitable gift, visit HolyCrossDonations.com or for more information, contact director of development Marcie Hall at Marcie.Hall@holy-cross.com.