Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS)

What is ALS (amyotrophic lateral sclerosis) or MND (motor neuron disease)?

ALS (amyotrophic lateral sclerosis) is pronounced “a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis”, which is also known as motor neuron disease (MND). ALS is actually a type of motor neuron disease. Some refer to ALS as Lou Gehrig’s disease after a famous American baseball player, Lou Gehrig, was the first to be diagnosed with the condition in the 1930s.

The condition is a progressive and fatal disease affecting the central nervous system and brain. ALS destroys the nerve cells in the body which leads to disability and paralysis in the patient during the late stages of the disease.

Motor neurons are nerve cells that are found in the spinal cord and central nervous system. Each neuron contains a specialised fibre that projects, or rather, communicates to other organs outside of the spinal cord in order to indirectly or directly control muscles and glands. These neurons conduct impulses, simply put, they convey impulses from the central nervous system to a gland, muscle or effector tissue, transmitting signals from the spinal cord to the muscles which aid in coordination and movement, telling the systems in the body how to function. In ALS, these neurons are slowly destroyed which prevents them from working effectively. The slow deterioration of the neurons is known as neurodegeneration.

The motor neurons control vital muscle activities such as:

  • Walking
  • Gripping
  • Speaking
  • Breathing
  • Swallowing

As ALS progresses and destroys motor neurons, resulting in the brain no longer being able to effectively give commands to the body, these functions will become increasingly difficult, and will eventually become impossible to perform over time.  This is why those with late stage ALS usually become paralysed.

It is not yet clear what the exact cause of neurodegeneration is (i.e. ALS does not have a cure). In some of the patients with the condition, roughly 5% have a family history of the disease or a family member with another condition known as frontotemporal dementia, meaning that genes may have a significant role to play. 

Frontotemporal dementia, also known as FTD is a progressive disease that results in damage to the frontal lobes of the brain. The typical symptoms are issues with speech, compulsive behaviour, memory issues and mood changes.

ALS is referred to as a motor neuron disease that is familial. In the majority of inherited cases, faulty genes have previously been identified as being a significant contributing factor to the condition developing.

There is no single diagnostic test or procedure for ALS to be accurately diagnosed. Typically, a nervous system and brain specialist known as a neurologist will conduct the diagnostic process. 

What is the difference between ALS and MS (Multiple Sclerosis)?

There is often some confusion between ALS (amyotrophic lateral sclerosis) and MS (multiple sclerosis). Both conditions are diseases that target and affect the individual’s central nervous system. Those who suffer from ALS and MS may also suffer from cognitive ability (i.e. knowing and perceiving) and memory issues. Those with MS may suffer more from mental impairment as opposed to those who have ALS. ALS is more typically defined by a lack of physical capabilities.

ALS and MS are both known as neurodegenerative diseases as they attack the muscles and nerves of the body. The two diseases are very similar in a number of ways, however, there are key differences that will determine the course of treatment and the patient’s overall outlook.

MS is an autoimmune disease that destroys the protective covering of the nerves (known as the myelin sheath) which results in the functions of the brain being impacted, and this impact affects the sufferer’s motor functions. MS more commonly results in mild symptoms for a number of years without causing severe debilitation.

ALS results in the neurons being destroyed completely over time resulting in the brain not being able to give any instructions to the body, this causes debilitation.   

These key differences between the two conditions are:

ALS

  • Targets the central nervous system and brain
  • The late-stage of this disease will often leave patients paralysed
  • Patients suffer from more physical problems and difficulties
  • This is NOT an autoimmune disease (i.e. a disease where the immune system will attack the body’s healthy cells) – The causes of ALS are largely unknown and can include a number of factors.
  • Is seen more commonly in men
  • People are mostly diagnosed with this condition between the ages of 40 and 70 years old
  • There is no known cure
  • This condition can be debilitating and fatal

MS

  • Targets and spinal cord and the brain
  • Individuals in the late-stage of this condition can have their mobility impacted, but they will rarely be left completely debilitated
  • Patients suffer from more mental problems and difficulties than those with ALS
  • This IS an autoimmune disease (a disease where the immune system will attack the body’s healthy cells) – MS is a result of the immune system viewing myelin, which is the protective nerve cell coating, as an invader and attacking it.
  • Is seen more commonly in women
  • People are mostly diagnosed with this condition between the ages of 20 and 50 years old
  • There is no known cure
  • This condition is rarely severely debilitating or fatal

Multiple Sclerosis

What are the different types of MND (motor neuron disease)?

There are four different types of MND, each one of them has different effects, however, they can also overlap each other in a number of ways.

The following is a brief outline on the four main types of MND:

Amyotrophic Lateral Sclerosis (ALS)

This is known as the most common type of MND and is the focus of this article. This condition involves both the lower and upper neurons in the central nervous system. The characteristics of this condition are defined by the wasting and weakness of the limbs due to nerve cell deterioration (i.e. neurodegeneration). The average life expectancy is between two and five years from the onset of the initial symptoms.

Upper motor neurons originate in the cerebral cortex or in the brain stem and transport motor information to the lower motor neurons. The lower motor neurons stem from the spinal cord to specific muscles.

Progressive Bulbar Palsy (PBP)

This condition affects both the lower and upper motor neurons. The symptoms are often those of slurred speech and difficulty in swallowing. The life expectancy of a sufferer is between six months to three years from the onset of the initial symptoms. 

Progressive Muscular Atrophy (PMA)

The condition only affects a small amount of people and mainly results in damage to the lower motor neurons. The early symptoms are often seen in the affected person acting exceptionally clumsy or experiencing weakness in their hands. The life-expectancy for those with this condition is normally more than five years.

Primary Lateral Sclerosis (PLS)

This is a very rare type of MND and only involves the upper motor neurons. The condition results in a weakness in one’s lower limbs and some people also suffer from weakness in their hands and speech issues. The sufferer’s life span is typically normal, however, this condition can also develop into ALS which will affect the outcome significantly.

What are the two types of ALS?

ALS can either be classified as familial ALS or sporadic ALS. These are explained as follows:

  • Familial ALS (fALS) – This type of ALS runs in families and is very rare. Roughly 5-10% of patients have familial ALS, meaning they have inherited it from their parent due to a gene mutation in their DNA.
  • Sporadic ALS – This is ALS that is not inherited and roughly 90% of patients have this kind, meaning they are the only one in their family with the disease. The exact cause is not yet understood, however, researchers believe that it may be a combination of genetic and environmental risks.

What are the symptoms of ALS?

The initial symptoms of ALS will typically affect the specific areas of the body and then progress to be more widespread. The vital thing is that an individual should contact their doctor if they notice any of the early symptoms of ALS. Early detection has shown evidence of helping to improve the overall survival of the patient.

The following are often early symptoms and signs of ALS:

  • Difficulty performing daily activities such as walking
  • Tripping over things and falling
  • Weakness in ankles, feet or legs
  • Weakness and clumsiness in hands
  • Trouble swallowing or slurred speech
  • Twitching of the hands, arms, tongue or shoulders and muscle cramps
  • Difficulty in keeping posture and keeping the head up

ALS typically begins in the feet, hands or limbs and will then progressively spread to other parts of the body. As the condition advances, more nerve cells are destroyed resulting in the muscles weakening. This ultimately affects the patient’s ability to swallow, speak, breathe, walk and grip.

ALS generally doesn’t affect the bladder, bowel control or the patient’s cognitive abilities.

The signs and symptoms of ALS will normally follow a specific pattern of three stages:

  • Initial stage symptoms
  • Advanced stage symptoms
  • End stage symptoms

Initial symptoms

The initial symptoms of ALS tend to develop and progress slowly and very subtly. It is easy to confuse these symptoms with those of other conditions that are unrelated but impact the nervous system.   

Limb-onset disease

In roughly 75% of those suffering from ALS or motor neuron disease, the initial symptoms can be seen in one of the patient’s legs or arms. This is what is known as limb-onset disease and the symptoms include the following:

  • Suffering from a weak grip which makes picking up or holding objects difficult
  • Weakening of the shoulder which makes it difficult to lift one’s arm over their head
  • Tripping over objects due to a weakness of the hip or ankle

These initial symptoms are typically painless and are often accompanied by widespread muscle twitches or cramps, there may also be a visible wasting of the muscles present or a significant loss of weight.

Bulbar-onset disease

In roughly 25% of patients with this condition, problems will initially impact the muscles that are used for swallowing and speech. This results in the patient suffering from increasingly unclear or slurred speech, known as dysarthria, and this is normally the first sign of this type of ALS and is often misdiagnosed as doctors may initially think that the patient is having a stroke.

As ALS progresses, swallowing may become more difficult, this is known as dysphagia and is sometimes misdiagnosed as a throat blockage.

Respiratory-onset disease

In very rare cases, ALS (amyotrophic lateral sclerosis) can begin by first affecting the lungs in the initial phases of the condition as opposed to affecting them towards the final stages of the disease. This is known as respiratory-onset disease.

In some cases, the initial symptoms are more obvious, such as a shortness of breath or battling to breathe. In a number of other cases, the symptoms can be less obvious and include the patient waking up on a constant basis during the night due to their brain being temporarily deprived of oxygen when they lie down. This often results in the individual feeling extremely tired and being left with a painful headache due to oxygen deprivation.

Advanced symptoms

As ALS progresses, the differences between the types of the condition become less noticeable due to increasing functions and areas of the body being affected. The rate that the disease spreads will vary, however, it will often remain at a similar pace for the individual, meaning once the person has been diagnosed, it is unlikely that the condition will speed up or slow down.

Muscular symptoms

The limbs will gradually weaken and the patient’s muscles will also start to appear as though they are wasting progressively. Because of this, the patient may find movement of his or her limbs more and more difficult. The muscles can also stiffen, which is a condition known as spasticity. Both the wasting of muscles and stiffness may also result in joint pain.  

Swallowing and speaking difficulties

Roughly 75% of those who are diagnosed with ALS or motor neuron disease have issues with swallowing and speaking which progress as the condition advances. Bear in mind that while episodes of choking are often distressing, they are unlikely going to be the cause of death.

Saliva issues

Issues with swallowing result in a reduction of the action and in turn excess saliva can lead to drooling. Thicker saliva can make it difficult for the patient to clear their chest due to the muscles that control coughing being weakened.

Yawning excessively

In some cases of ALS, the patient may suffer from episodes of uncontrollable and excessive yawning, regardless of whether they are tired or not. This can sometimes result in a painful or even a dislocated jaw. This yawning is a reflex of the nerves deteriorating in the jaw area as they begin to malfunction.

Emotional changes

ALS can result in a number of emotional changes in an individual and can impact their ability to have control over their emotions, specifically when the muscles controlling speech and swallowing are weakened.

Particularly common signs in someone with ALS are episodes of uncontrollable crying, and more rarely, uncontrollable laughter. This is sometimes referred to emotionality, the pseudobulbar affect or emotional lability. The patient may often act out irrationally or feel unnecessarily sad or depressed. It is thought that the cause has something to do with the neurological pathways responsible for regulating the one’s external expression of emotion being affected.

Mental ability changes

In some cases, those with ALS may suffer from issues with their planning, concentration or language use. These are known as cognitive changes. These deviations from one’s normal behaviour often overlap with another condition, frontotemporal dementia. This is an uncommon kind of dementia that significantly affects the sides and front of the brain, known as the temporal and frontal lobes, and results in issues with language and behaviour.

These initial changes in ALS patients are often subtle, making it difficult to differentiate them from the normal process of aging. They do not typically impact the person’s ability to make decisions.

Roughly 15% of those with ALS may develop a more profound form of frontotemporal dementia, this is normally soon after and sometimes before the first symptoms that are muscle-related appear. The patient is not always aware that they have problems affecting their personality and behaviour.

Breathing issues

In those with ALS, the muscles and nerves responsible for controlling the lungs will deteriorate progressively, this makes it difficult for the person to breathe properly.

This often leaves the patient out of breath when performing everyday tasks such as taking out the trash or even going for a short walk. In time, the person may even suffer from a shortness of breath when they are resting.

Shortness of breath often becomes an issue at night as it is often harder to breathe when lying down. As ALS progresses, the patient may have to wear a non-invasive mask for breathing.

End-stage symptoms

As ALS (amyotrophic lateral sclerosis) / motor neuron disease progresses to the final stage, the patient is likely to suffer from:

  • An increase in body paralysis, this may mean they may need help with completing daily activities.
  • Significant breathing issues and shortness of breath.

As mentioned, the patient will eventually need a non-invasive breathing device to help compensate for their loss of lung function. During this stage, the majority of patients with ALS will become more and more drowsy, and will eventually fall into a deep sleep and pass away peacefully.

Secondary symptoms

Additional symptoms have also been seen in some cases of ALS as a result of the impact of living with the condition. These often include:

  • Insomnia
  • Anxiety
  • Depression

What are the causes of ALS?

In about 5-10% of ALS patients, the condition is inherited. The rest of the cases do not have a known cause. There are a number of possible causes being studied, these include:

  • Gene mutation – There are a number of different genetic mutations that can lead to ALS that is inherited. These mutations also cause symptoms that are very similar to the forms of ALS that are not inherited. However, not all the gene mutations responsible for ALS have been researched and identified. Bear in mind that only 5% of cases are familial and have been inherited. Those who are the only family member with the disease are known as cases of ALS that are sporadic (i.e. sporadic ALS). Those who have sporadic ALS and those who have familial ALS have very similar symptoms. ALS is only classified as familial if two or more blood relatives have had the condition.
    • **My Med Memo: It is important to know the difference between hereditary and genetic. Genetic disorders and conditions are ones that are related, in part, to the individual’s genes and can be caused by environmental factors such as exposure to radiation. Changes in the genes, such as mutations, can result in a number of medical issues. On the other hand, some medical issues are hereditary, this means that they are a result of gene mutations that have been inherited, or rather, passed down from a parent to a child in their genome. It is often the case that a parent has been exposed to high levels of radiation which has caused his genes to mutate, this gene mutation, which is a permanent change to the individual’s DNA sequence, then becomes hereditary as it can be passed down to the child.
  • Chemical imbalance – Those who suffer from ALS tend to have levels of glutamate that are higher than they should be. Glutamate is one of the brain’s chemical messengers, or rather, neurotransmitters that send messages within the brain and the nerves throughout the body. It is found in the membranes of nerve cells and enables them to send messages to other nerve cells. Too much of this chemical messenger can be dangerous and toxic for some of the body’s nerve cells as this results in the individual’s nerve cells being overstimulated, leading to inflammation and even cell death.
  • Protein mishandling – Proteins are found within nerve cells, if these are mishandled or incorrectly processed, this action may result in abnormal formations of proteins accumulating gradually inside the person’s nerve cells, this can destroy the cells.  
  • Disorganised immune response – In some cases, the patient’s immune system may begin to attack some of the healthy cells in the body, this often leads death of the body’s nerve cells.

ALS explained

What are the risk factors of ALS?

The risk factors that have been established for ALS are the following:

  • Heredity – Roughly 5-10% of those who have ALS have inherited the condition, this is known as familial ALS. These people who have inherited ALS have a fifty-fifty chance of their children developing the condition.
  • Genetics – There have been some studies conducted that examined the human genome entirely (i.e. genome-wide association research), and found a number of similarities within the gene variations in those who have familial ALS as well as a few of those who have non-inherited ALS. These variations of genes may result in certain people being more at risk of developing ALS.
  • Age – The risk of ALS developing will increase with age and is most commonly seen in those who are in their 40s and older.
  • Gender – It has been noted that those who have the condition and are younger than 65 are more likely to be men than women. This sexual difference tends to disappear in patients over 70 years old.

It has also been documented that environmental factors may have a role to play in the risk factors of ALS. These risk factors include:

  • Exposure to environmental toxins – There has been some evidence that suggests the exposure to toxins such as lead in the home or workplace may have a link to the development of ALS. There have been a number of studies conducted to try and identify the exact kind of toxin exposure that increases one’s risk of ALS, but not one single chemical is yet to be identified.
  • Smoking – Smoking cigarettes is likely to be an environmental risk for ALS, this risk is especially greater for women and also increased after menopause.
  • Military service – There has been recent evidence that suggests that those who have completed military service could have an increased level of risk in developing ALS. It is still unclear as to why this link exists, although some research suggests that those in the military may be exposed to a number of chemicals, viral infections, traumatic injuries or undergo intense physical exertion that can play a role in disease development.

What are the complications of ALS?

As ALS progresses, those who suffer from it may experience some of the below complications:

Breathing issues

In time, ALS will progress and paralyse the muscles needed for breathing. At this stage, the patient may require a breathing device that helps them to breathe during the night. This air can be given through a BiPAP (bi-level positive airway pressure) or via a CPAP (continuous positive airway pressure). Both of these devices are non-invasive pressure support ventilation systems that make use of a flow-cycled or time-cycled process that alternates between the two mechanically applied levels of positive airway pressure.

Patients who suffer from advanced ALS may opt for having a tracheostomy (also known as a tracheotomy) conducted, this is a surgery that will create a hole in the neck that leads to the trachea (windpipe) in order for a full-time respirator to be inserted. This respirator will inflate and deflate the lungs.

Respiratory failure is the most commonly attributed cause of ALS deaths which tend to occur between three and five years post initial symptoms onset.

Speech issues

The majority of patients who suffer firm ALS will start to have issues with their ability to speak once the condition begins to progress over time. Speech problems tend to begin as mild and occasional slurs of words, however, these can develop further to become far more severe. The patient’s speech will eventually become more and more difficult to understand which often results in the individual needing communication technologies in order for them to be understood and communicate. There are some computers and mobile phone applications that allow for patients to type in what they wish to say and the program will read it out loud. Certain phrases can also be selected in order for the patient to not have to type everything out.

More advanced programs are able to communicate for the patient without the patient having to type or try to speak, these work on Brain-Computer Interfaces that allow for the patient to control the computer through their brain waves, these require no movement and are often used for patients with advanced ALS suffering from paralysis.

Eating issues

Those with ALS may develop dehydration and malnutrition due to the damage done to the individual’s muscles needed for swallowing. These individuals also have an increased risk of developing pneumonia as liquids, food and saliva can get into their lungs as a result of their inability to swallow properly (this is known as aspiration). Thus, the introduction of a feeding tube may be necessary to aid in reducing these risks. This can also ensure that the patient does not suffer from dehydration and malnutrition.

Feeding tube

Dementia

Some patients who have ALS may suffer from memory issues and battle to make decisions. This can lead to some of these individuals being diagnosed with frontotemporal dementia. Just to recap, this is a progressive disease that results in damage to the frontal lobes of the brain.

How is ALS diagnosed?

ALS (amyotrophic lateral sclerosis) is a disease that is complicated to diagnose in the early stages as the condition is able to mimic a number of other neurological disorders. Some of the tests that are used to eliminate the possibility of any other conditions include:

  • Magnetic resonance imaging (MRI) – An MRI is a type of scan that makes use of powerful magnetic fields as well as radio waves in order to produce extremely detailed visuals/images of the spinal cord and brain. This will allow for the doctor to detect any spinal cord tumours or other issues that might be the cause of the symptoms, such as a herniated disc (a condition where the soft centre of the spinal disc has pushed through a small crack in the exterior casing) in the patient’s back or neck.
  • Electromyogram (EMG) – This a procedure wherein the doctor will assess the health of the nerve cells and muscles by inserting an instrument known as an EMG needle electrode into certain muscles. This will then be able to evaluate the level of muscle activity when they are at rest and when they contract. If any abnormalities are detected during an EMG, this can aid the doctor in diagnosing ALS or determining whether there is a different nerve or muscle condition that is the cause of the symptoms. These findings will guide the chosen route of therapy.
  • Muscle biopsy – This test is conducted is the doctor suspects that the patient has a muscle disease other than ALS. While the patient is sedated under local anaesthesia, a small part of their muscle will be removed and then sent to the lab for testing. 
  • Nerve conduction velocity (NCV) test / Nerve conduction study (NCS) – This is a test that measures the speed of conduction (transmission of the impulses along a nerve) of an electrical impulse that is sent through the nerve. The nerve will be stimulated during this procedure, normally through the use of electrode patches that have been attached to the patient’s skin. This will enable to doctor to detect any nerve damage.
  • Urine and blood tests – In these tests a specialist will examine urine and blood samples in a lab in order for other causes of the symptoms to be ruled out.
  • Lumbar puncture (spinal tap) – In some cases, the specialist may take a spinal fluid sample to be sent for analysis. To do this, a very small needle will be inserted directly between the two vertebrae found in the lower back, from here the needle will be used to remove some of the cerebrospinal fluid in order for it to be sent to a lab for testing. This procedure can aid in diagnosing subarachnoid haemorrhage (bleeding around the brain) or other abnormalities of the central nervous system. 

What is the treatment for ALS?

The treatments for ALS are unable to reverse any of the damage done by the condition, however, they are able to slow down the rate at which the symptoms progress, prevent some of the complications and allow for the patient to be more independent and comfortable.

Effective treatment may require a cooperative team consisting of various doctors and specialists that are trained in a number of different areas as the treatment is often complex, it does, however, prolong the patient’s life and the quality thereof.

The following is a list of treatment options. The team of specialists involved will work towards finding the most effective means and combination of treatment for the individual.

Medications

There are two medications that have been FDA approved for the treatment of amyotrophic lateral sclerosis or motor neuron disease:

  • Riluzole (Rilutek) – This is a drug that has shown to slow down the progression of ALS in some patients. This can be due to the levels of glutamate (chemical messenger found within the brain) being reduced by this drug. This chemical, as previously mentioned, often exists in higher quantities in those with ALS which leads to nerve cell damage. Rilutek is taken in the form of a pill and can lead to changes in liver function, dizziness and gastrointestinal conditions.
  • Edaravone (Radicava) –  This is an FDA approved drug that is administered intravenously over a period of 10 to 14 consecutive days or once a month. Gait disturbances (this is a deviation from walking normally), swelling, hives and a shortness of breath are some of the side effects. Patients with a sulfite sensitivity may also have an allergic reaction to this drug as it contains sodium bisulfite.

The doctor is likely to also prescribe some of the below medications in order for some relief to be provided from the symptoms. These include medications for:

  • Muscle spasms and cramps
  • Spasticity
  • Excessive salivation
  • Uncontrollable outbursts of crying or in some rare cases, crying
  • Fatigue
  • Pain
  • Sleep problems
  • Constipation
  • Depression
  • Excessive phlegm

Therapies

  • Physical therapy – This kind of treatment involves a physical therapist who is able to address and assist with the pain associated with mobility and walking and provide the individual with equipment to aid them in staying somewhat independent. Walkers, wheelchairs and braces can be adjusted for the patient. As well as this, ramps may also be suggested for the individual’s home to make moving around more accessible. A PT (physical therapist) is able to assist the patient in practising some low-impact stretches and exercises in order for them to maintain their cardiovascular fitness, motion range and muscle strength until the condition progresses into complete paralysis.  
  • Breathing care – Breathing will become increasingly difficult as the disease progresses and the muscles needed for breathing weaken. Because of this, the patient’s breathing may be regularly tested and he or she may be given non-invasive devices to help them to breathe at night. As previously mentioned, mechanical ventilation may be needed. This requires a tube to be inserted through a hole in the patient’s neck that leads to their windpipe. This tube will be connected to the respirator. A respirator is a machine that induces artificial respiration (breathing).
  • Occupational therapy (OT) – This form of treatment is done through an OT (occupational therapist) who can help the patient to find ways that they can remain as independent as possible, regardless of their arm and hand weakness. An OT can also aid in adapting the patient’s home to modify it to suit their needs of mobility. They can also implement adaptive equipment to perform their daily activities which include grooming, dressing, bathing and eating. Adaptive equipment or technologies are devices that can be adapted to suit the needs of the individual in order to enhance their mobility such as adjustable beds that help the patient to sit up. In addition, OTs possess a thorough understanding of assistive computers to help with speech issues.
  • Nutritional support – The medical team will work with the patient and their family to ensure that the individual is meeting their nutritional needs. Eventually, a tube for food to enter the body (feeding tube insertion) will be introduced as swallowing becomes an issue.  
  • Speech therapy – Due to ALS affecting the muscles needed to speak, the communication for a patient with advanced ALS can become an issue. Through seeing a specialist in speech therapy (speech therapist), the patient will be able to learn adaptive techniques and technologies to help them communicate more clearly. A patient can also speak to their therapist about renting or borrowing assistive devices including computers or tablets that have text-to-speech programs as this can be a less expensive option than some of the more advanced Brain Computer Interface devices.
  • Social and psychological support – Someone to help with the overall wellbeing of the patient and their loved ones is known as a clinical social worker. This person may also be needed to aid with any financial issues, sourcing and purchasing equipment and insuring it. Psychologists can also help in providing counselling and emotional care and support for the patient and their family. 

Paralysed ALS patient

Clinical studies

There have been a number of clinical studies that have promising results regarding future treatment and medication options for ALS. A patient’s eligibility for joining one of these studies will depend on a number of aspects related to their condition. Bear in mind that although some studies have positive results, their outcomes are not guaranteed. It is best that a patient speaks to their doctor regarding any clinical studies they may have heard about or want to partake in. 

How to cope with ALS

Finding out someone has ALS is often an emotional ride for both the patient and their loved ones. The below tips are some coping techniques that may be able to help in dealing with ALS:

  • Take the time that is needed to grieve – Finding out about a fatal disease that will, in turn, reduce the patient’s mobility and their independence is a difficult experience. After diagnosis, it is healthy to take the time that is needed to discuss it with friends and family and come to terms with the issue at hand.
  • Be positive – The medical team will often help the patient to focus on their abilities rather than their disabilities and will teach them a number of coping techniques through assistive devices and other forms of treatment. Although the typical life expectancy for someone with ALS is between three and five years after the symptoms appear, there have been a number of people that live for longer than 10 years. Remaining hopeful, but with an aspect of realism, can also help improve the quality and enjoyment of a patient’s life.
  • Don’t let the physical limitations be a setback – Relatively speaking, the fact that the patient will suffer from paralysis and muscle weakening does result in their mobility being significantly hindered, however, this does not have to define them or their family. Many people suffering from ALS are able to lead rewarding lives.
  • Attend support groups – Comfort is often found through joining a local support group for those who are suffering from ALS. Friends and family can also attend these and learn more about caring and helping their loved one with the condition.
  • Find out about medical insurance and care – ALS is a condition that requires a lot of care and specialised attention, it is therefore best to find out about what one’s current insurance covers and possibly look into getting added insurance that has the most amount of benefits for someone with ALS. It is also a good idea to find out about future care options for the advanced stages of the condition such as a special care part or a full-time home nurse.

What is the outlook for ALS?

The symptoms of ALS tend to develop relatively quickly and there is currently no cure for the condition. The number of patients who live for longer than the five years survival rate is 20%. Roughly 10% of those patients will live longer than 10 years. Regardless, ALS is a fatal condition and should be treated as such.

ALS or motor neurone disease is a severe condition that will greatly impact the patient’s life and their loved ones. It can be extremely challenging to live with, but it is not as bleak as many people would imagine it to be.

Through specialist support and a strong community, a number of people are able to maintain various aspects of their independence for a large part of the course of the condition and can experience a better quality of life than they initially imagined.

The disease and life of the patient usually come to an end in a setting that is typically calm and often in their own home. They will typically pass away in their sleep from the progressive weakening of the muscles needed for breathing.

It is important to note that in the days leading up to this, the patient’s brain is often still working well and they can still hear and understand what their loved ones are saying to them. Because of this, loved ones should be a pillar of support for patients and speak to them as if they were still healthy individuals so as to keep their spirits up and their mind alert right until the end.

Disclaimer - MyMed.com is for informational purposes only. It is not intended to diagnose or treat any condition or illness or act as a substitute for professional medical advice.