What is the life expectancy of someone with motor neurone disease? – A person with MND will usually die between two to three years after diagnosis, but this can vary from person to person. Some people live many years after their diagnosis.
- 0.1 What are the stages of MND progression?
- 1 How long do MND sufferers live?
- 2 How do you know when an ALS patient is near death?
- 3 How painful is MND?
- 4 Why is MND on the rise?
- 5 Which type of motor neuron disease is fatal?
- 6 Is MND palliative?
- 7 Has anyone ever recovered from MND?
- 8 Is there any hope for MND sufferers?
- 9 What is palliative treatment for MND?
- 10 What is the MND cure 2023?
- 11 How fast do you deteriorate with ALS?
- 12 What is the final stage of terminal illness?
What are the final stages of death in MND?
The final stages of MND will usually involve gradual weakening of the breathing muscles and increasing sleepiness. This is usually the cause of death, either because of an infection or because the muscles stop working. Specialist palliative care supports quality of life through symptom control.
What are the stages of MND progression?
Motor neuron disease (MND) refers to a group of rare but severe neurodegenerative diseases in which motor nerves in the spine and brain lose function over time. Early signs include weakness and slurred speech. Motor neurons are nerve cells that send electrical output signals to the muscles, affecting the muscles’ ability to function.
Motor neuron disease (MND) can appear at any age, but the symptoms usually appear after the age of 50 years, It affects more males than females. The most common type of MND is amyotrophic lateral sclerosis (ALS), The renowned English physicist Stephen Hawking lived with ALS for many decades until his death in March 2018.
The American baseball player, Lou Gehrig, also had ALS. This resulted in people calling it Lou Gehrig’s disease, There are several types of MND. Doctors classify them according to whether they are hereditary or not, and which neurons they affect. The types include:
ALS: This is the most common type of MND. It affects both the upper and lower motor neurons — the neurons in the brain and spinal cord — which then affects the muscles of the arms, legs, mouth, and respiratory system. On average, people with ALS live for 3–5 years after receiving the diagnosis, but with supportive care, some people live for 10 years or longer. Primary lateral sclerosis: This affects the neurons in the brain. It is a rare form of MND that advances more slowly than ALS. It is not fatal, but it can affect the quality of life. Juvenile primary lateral sclerosis occurs in children. Progressive bulbar palsy (PBP): This involves the brain stem, and people with ALS often have PBP, too. It causes frequent choking spells and difficulty speaking, eating, and swallowing. Progressive muscular atrophy: This type of MND is rare. It affects the lower motor neurons in the spinal cord and causes slow but progressive muscle wasting, especially in the arms, legs, and mouth. Spinal muscular atrophy (SMA): This inherited MND develops in children. There are three types, all caused by a genetic change known as SMA1. It tends to affect the trunk, legs, and arms. The long-term outlook depends on the type.
Motor neurons instruct the muscles to move by passing on signals from the brain. They play a role in both conscious and automatic movements, such as swallowing and breathing. Experts believe that around 10% of MNDs are hereditary. The other 90% develop randomly.
What is the cause of death in MND patients?
Introduction – Motor neurone disease (MND; also known as amyotrophic lateral sclerosis ) is a progressive neurological disease which leads to increasing paralysis of arms and legs together with speech and swallowing and breathing problems, due to progressive muscular weakness.
The aetiology is unknown, although 5–10% of patients have a family history, and for these patients an abnormal gene mutation can be found in up to 60%.1 In patients, with no family history, the same mutations are found in 10–20% and there is increasing evidence that there is a genetic component, with unknown environmental factors, that may lead to the disease.1 The prognosis for most patients is 2–3 years, although 25% of patients are alive at 5 years and 5% at 10 years.2 The progression of the disease is very individual, but there is usually progressive loss of muscle power, which may affect the limbs, the bulbar area – affecting speech and swallowing – and the respiratory muscles of the chest wall and the diaphragm.
The usual cause of death is respiratory failure, often associated with infection. There are now two drugs licensed for MND – riluzole, which has been shown to slow the progression in some patients, 3 and edaravone, which has been shown to help certain patient groups and is licensed in the United States.4 As there is, at present, no cure, palliative care may be seen as appropriate from the time of diagnosis, particularly as the prognosis is poor.
Many patients may not be diagnosed for up to 12 months after their first symptoms and thus may have developed severe disability and may have a short prognosis. There are many new developments and challenges in the care of a person with MND, and their family – in the communication about the disease, the use of interventions in the management of symptoms, the issues of exercise and physiotherapy, the psychosocial issues faced by patient and family and care at the end of life.
The issues discussed below are of particular significance for patients and families and have been highlighted within national and international guidelines on the provision of care in MND.
How long do MND sufferers live?
Amyotrophic lateral sclerosis (ALS): – This is the most common form of MND, with weakness and wasting in the limbs, muscle stiffness and cramps. Someone may notice they are tripping when walking or dropping things. Life expectancy is usually two to five years from the onset of symptoms.
How do you know when an ALS patient is near death?
Symptoms Of End Stages of ALS Some of the more common symptoms in the end stages of the disease include: Paralysis of voluntary muscles. Inability to talk, chew and drink. Difficulty breathing.
Do people with MND sleep more?
Getting enough sleep can help us feel rested in mind and body, and generally feel better as we get through each day 1, But what happens to sleep for people living with motor neurone disease (MND)? Can the symptoms of MND be better managed to help with sleep? Research finds that getting enough sleep can be very important for people living with MND.
- Sleep helps to provide relief and improve quality of life with MND 2,
- MND may make sleep difficult, however.
- For many people with MND, as the disease and consequent muscle weakness progresses, they experience problems that disrupt their sleep 3, 6,
- Waking up frequently or having trouble getting to sleep then creates other sorts of strains and pressures, adding to the burden of the disease 4,
Problems with sleep from MND affects carers and loved ones, too. Studies show that sleep plays a major role in the wellbeing and support needs of family, loved ones and others caring for someone living with MND 5, With the New Year well and truly underway, in our first blog for 2021 we looked at the evidence of what might help with sleep for people affected by MND.
How painful is MND?
Is MND painful? Motor neurones do not transmit or modify pain signals, so the disease itself is not inherently painful.2 However, pain may be experienced as the disease progresses. Pain may occur at any stage of MND, including early on, with no relationship between pain intensity and length of time since diagnosis.
Why is MND on the rise?
Discussion – In this study, we used multiple independent data sources to provide comprehensive health surveillance estimates of MND in the Netherlands from 1998 to 2017, resulting in an average estimated incidence of 2.64 per 100,000 person-years, a prevalence of 9.5 per 100,000 persons, and a lifetime MND risk of 1 in 323.
Mean diagnostic delay did not significantly change while median survival time improved by 3 months from 2006 through 2017, possibly as a result of improved multidisciplinary care. This observed increase in median survival time could imply that the increase in MND incidence may be larger than the observed increase in MND mortality rates since 1998.
After age and sex adjustment, MND mortality rates increased by 14.1% over the last 2 decades in both men and women. The 53.2% increase in unadjusted MND mortality rates could have significant consequences for planning of future health care services in that it may indicate a doubling of MND prevalence by the year 2050.
- Our results suggest that either genetic or potentially preventable environmental risk factors are driving MND risk, and urgent identification is needed, which may be aided by detailed geographic studies of MND risk.
- We found a different effect of age when comparing MND and Alzheimer risk.
- The decline of MND risk in the oldest age groups contrasts with the risk of Alzheimer and suggests that there is a time period of maximal susceptibility and that MND is not only a result of aging.
Short survival in cases of MND compared to cases of Alzheimer may also play a role. MND may be exclusive to a small susceptible subpopulation, the majority of whom are deceased by 70 to 80 years of age as a result of either MND or other unrelated causes.
There is evidence that loss of competing risks and longevity of this susceptible subpopulation could also be driving the increase in MND mortality rates.15 Indeed, we found that the risk of death due to causes other than MND decreased during the study period. Our findings of a reduction of the size of a susceptible subpopulation and larger increase of MND mortality rates among younger age groups, however, contrast with this hypothesis.
Nevertheless, we cannot rule out that longevity of a susceptible subpopulation might had affected the increase of MND mortality rates. Aging of the general population is an important driver of the increasing incidence of neurodegenerative diseases.20 An earlier study found no statistically significant change in MND incidence in Minnesota in the United States in the period of 1925 to 1998.21 In contrast, several studies have indicated that MND incidence has increased in the last 2 decades.3, 4, 22 For example, in an epidemiologic study in Northern Italy, MND incidence increased by 14% from 1995 to 2014, mostly in women.4 A possible explanation for the larger increase in MND incidence in more recent years may be that those born during the baby boom after World War II reached ages with highest incidence in the period of 2005 to 2017.
Most studies were based on registry data only while using capture-recapture methodology to correct for underascertainment, which can underestimate or overestimate the total number of cases when data sources are not independent.23 In our study, we have therefore performed both capture-recapture and simulation-based sensitivity analyses with 3 independent data sources, including unselected data from a national compulsory health database.
In absolute terms, the number of cases per year increased from 298 in 1998 to 466 in 2017. If this increase continues, the lifetime risk of MND would increase to 1 in 205 by 2050. This observed increase is not driven solely by aging of the general population, which could explain only ≈75% of the increase.
Increased awareness and timelier referral of patients suspected of having MND since the start of the Netherlands MND Center in 2003 may play a role, although several environmental risk factors that have been linked to MND could be involved such as exposure to organic dust, agricultural pesticides, air pollution, and other occupational hazards.24, –, 27 To help develop hypotheses as to which risk factors are currently driving MND risk, we mapped geographic MND risk from 1998 through 2017 to identify high-risk areas.
The use of national data is particularly advantageous because assessing geographic risk in a limited area is an approach known to exaggerate the likelihood of identifying high-risk areas.8 There was considerable spatial variation in MND risk, which indicates that associating spatial risk with characteristics of that particular area may be worthwhile (i.e., identify factors that explain spatial variation in risk).
For example, by quantifying risk factors such as lifestyle, physical activity levels, and environmental exposures in well-defined geographic areas, 27, –, 29 we may be able to efficiently identify risk factors. We have illustrated its potential use by associating MND risk with population density, which may act as a proxy for other risk factors.30 Although we found no direct correlation of population density with MND risk, its simplicity could stimulate etiologic research in MND.
For example, air pollution could be more severe in cities; by combining spatial risk estimates with exposure levels, on both an individual and a geographic level, one may increase power to find potential associations. A similar approach may hold true for exposure to agricultural pesticides, which are more common in rural areas.
An illustrative example of the latter is the increased risk of Parkinson disease in the Netherlands associated with living in the vicinity of agricultural fields where pesticides are used.31 Furthermore, genetic risk factors may also cause geographic variability in MND risk.7 Previously, studies found that rare genetic variants appear to be important drivers of MND risk 32 and that genetic variation can be localized geographically, meaning that there is relatively little migration in the Netherlands.33 As a result, local MND incidence may increase via a genetic founder effect when new genetic variations occur.
We hypothesize that both environmental risk factors, unique to each area, and local genetic admixture may thus explain geographic variation in MND risk. Quantification of a broad spectrum of risk factors, both on an individual level as per geographic area, remains paramount.
Due to privacy regulations, our study was limited in that we could not confirm a clinical diagnosis in the DPR database for individual cases. Therefore, we cannot rule out some misclassification in the DPR database. Nevertheless, we considered the DPR database to be of a high standard for several reasons.
Because registration is mandatory, the DPR database includes all Dutch residents. Furthermore, it is likely that patients with MND will visit a neurologist at least once because the Dutch public healthcare system ensures that there are no financial or physical hurdles to receiving health care.
- As a result, it is probable that if a patient receives an MND diagnosis, this will also be communicated to that individual’s general practitioner or nursing home physician.
- Dutch law requires that a physician (usually the general practitioner or nursing home physician) list all possible contributing causes of death on the death certificate.
In addition, we found that both age at death and male:female ratio were stable, suggesting that case ascertainment has not changed during the study period. Moreover, the incidence and prevalence estimates that we found are in line with estimates found in other North European countries with compulsory national health databases.
In Denmark, Norway, and Sweden, incidence and prevalence estimates ranged from 2.47 to 3.54 per 100,000 person-years and 8.0 to 8.7 per 100,000 persons, respectively.34, –, 40 Last, we found similar prevalence estimates using capture-recapture methodology and a simulation-based approach using data from independent sources, thus further confirming the accurate case ascertainment of the data sources used.
Using the national DPR database enabled us to assess that 72.0% of all Dutch patients with MND were identified by the Netherlands MND registry. This coverage rate is comparable to or higher than other large population-based registries.41, –, 43 The higher mean age of patients and higher proportion of female patients in the DPR database suggest that older female patients may be underrepresented in the Dutch population–based MND registry.
This unintended selection of younger male patients with, on average, longer survival was also observed when trial participants were compared to the eligible MND population.17 It is important to recognize this unintended selection of patients because it can lead to biased effect sizes in population-based studies when investigating etiologic risk factors or genes.29, 44 To resolve unintended selection in population-based studies, future studies that investigate reasons for nonparticipation in observational studies are needed to identify these as-yet unknown patient factors.
We found a national 53.2% increase in MND mortality rates from 1998 through 2017 in both men and women. This large increase in the last 2 decades underlines the impact that the care of patients with MND will have on health services in the future. There was geographic variation in MND risk, which could be related to local genetic admixture or complex environmental risk factors.
Which type of motor neuron disease is fatal?
What are motor neuron diseases? – Motor neuron diseases (MNDs) are a group of progressive neurological disorders that destroy motor neurons, the cells that control skeletal muscle activity such as walking, breathing, speaking, and swallowing. This group includes diseases such as amyotrophic lateral sclerosis, progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, spinal muscular atrophy, Kennedy’s disease, and post-polio syndrome.
- Messages or signals from nerve cells in the brain (upper motor neurons) are typically transmitted to nerve cells in the brain stem and spinal cord (lower motor neurons) and then to muscles in the body.
- Upper motor neurons direct the lower motor neurons to produce muscle movements.
- When the muscles cannot receive signals from the lower motor neurons, they begin to weaken and shrink in size (muscle atrophy or wasting).
The muscles may also start to spontaneously twitch. These twitches (fasciculations) can be seen and felt below the surface of the skin. When the lower motor neurons cannot receive signals from the upper motor neurons, it can cause muscle stiffness (spasticity) and overactive reflexes.
This can make voluntary movements slow and difficult. Over time, individuals with MNDs may lose the ability to walk or control other movements. MNDs are classified according to whether the loss of function (degeneration) is inherited (passed down through family genetics); sporadic (no family history); and whether they affect the upper motor neurons, lower motor neurons, or both.
In cases where a motor neuron disease is inherited, it is usually caused by mutations in a single gene. These conditions are usually inherited in one of several patterns:
- Autosomal dominant means that a person needs to inherit only one copy of the defective gene from one parent with the disorder to be at risk of the disease. There is a 50 percent chance that a child of an affected person will inherit the abnormal gene and develop the disease.
- Autosomal recessive means a person must inherit a copy of the defective gene from each parent. These parents are likely to be asymptomatic (without symptoms of the disease). Autosomal recessive diseases often affect more than one person in the same generation (e.g., siblings).
- X-linked inheritance occurs when the female parent carries the gene on one X chromosome and passes the disorder along to male children. Male children inherit an X chromosome from female parent and a Y chromosome from their male parent. Male children have a 50 percent risk of inheriting the abnormal X chromosome and developing the disease. Female children inherit an X chromosome from each parent. Female children have a 50 percent chance of inheriting their female parent’s X chromosome and a safe X chromosome from their male parent, which usually makes them asymptomatic carriers of the mutation.
Though there are several types of MNDs, they all cause muscle weakness that gradually worsens over time and leads to disability. In some cases, these diseases are fatal. Respiratory insufficiency, a condition in which the lungs cannot properly take in oxygen or expel carbon dioxide, is a feature of most MNDs.
- Symptoms may include breathlessness, shortness of breath that occurs while lying down, recurrent chest infections, disturbed sleep, poor concentration and/or memory, confusion, morning headaches, and fatigue.
- Common motor neuron diseases Amyotrophic lateral sclerosis (ALS), also known as classical motor neuron disease, affects both the upper and lower motor neurons.
It causes rapid loss of muscle control and eventual paralysis. Many doctors use the term motor neuron disease and ALS interchangeably. Early symptoms of ALS usually include muscle weakness or stiffness in a limb or muscles of the mouth or throat (so-called bulbar muscles).
Gradually almost all the muscles under voluntary control are affected, and individuals lose their strength and the ability to speak, eat, move, and even breathe. Most people with ALS die from respiratory failure, usually within three to five years from the onset of symptoms. However, about 10 percent of people with ALS survive for 10 or more years.
Progressive bulbar palsy (PBP), also known as progressive bulbar atrophy, attacks the lower motor neurons connected to the brain stem. The brain stem (bulbar region) controls the muscles needed for swallowing, speaking, chewing, and other functions. Many ALS experts consider PBP within the spectrum of ALS because the majority of individuals who begin with this form of the disease eventually develop more widespread MND.
Indeed, many clinicians believe that PBP by itself, without evidence of abnormalities in the arms or legs, is extremely rare. Symptoms, which worsen over time, include trouble chewing, speaking, and swallowing. Individuals may also have weakness in their tongue and facial muscles, twitches, and a reduced gag reflex.
They may also experience weakness in their arms or legs, but it is less noticeable than other symptoms. Because they have difficulty swallowing, individuals are at risk of choking and inhaling food and saliva into the lungs. People can also have emotional changes and may begin to laugh or cry at inappropriate times (called pseudobulbar affect or emotional lability).
Some symptoms of stroke and myasthenia gravis are similar to those of progressive bulbar palsy and must be ruled out prior to diagnosis. Primary lateral sclerosis (PLS) affects only the upper motor neurons, causing the movements in the arms, legs, and face to be slow and difficult. The disorder often affects the legs first, followed by the torso, arms and hands, and, finally, the muscles used for swallowing, speaking, and chewing.
The legs and arms become stiff, clumsy, slow, and weak, making it difficult to walk or carry out tasks requiring fine hand coordination. Speech may become slowed and slurred. Individuals may have difficultly balancing, increasing the risk of falls. Affected individuals may also experience emotional changes and become easily startled.
Progressive muscular atrophy (PMA) is a rare disease marked by slow but progressive damage to only the lower motor neurons. It largely affects men, and usually at a younger age than most other adult-onset MNDs. Weakness is typically seen first in the hands and then spreads into the lower body, where it can be severe.
The torso muscles and breathing may become affected. Exposure to cold can worsen symptoms. Other symptoms may include:
- Muscle wasting (shrinking)
- Clumsy hand movements
- Muscle cramps
Spinal muscular atrophy (SMA) is an inherited disease that affects lower motor neurons. It is the most common genetic cause of infant mortality. Defects in the SMN1 gene result in a loss of the SMN protein, which causes the lower motor neurons to deteriorate, producing muscle weakness and wasting.
- This weakness is often worse in the proximal muscles, which are closer to the center of the body (e.g., torso, thigh, and arm), than distal muscles which are further away (e.g., hands and feet).
- SMA is classified into three main types—based on age of onset, severity, and progression of symptoms.
- Generally, the earlier symptoms start to appear, the greater the impact on motor function.
All three main types are caused by defects in the SMN1 gene.
- SMA type I (also known as Werdnig-Hoffmann disease) is evident by the time a child is 6 months old. Symptoms may include poor muscle tone, lack of reflexes and motor development, twitching, tremors, and difficulties swallowing, chewing, and breathing. Some children also develop scoliosis (curvature of the spine) or other skeletal abnormalities. These children never sit independently and, before the availability of genetic therapies, most died by one year of age.
- SMA type II usually begins to appear between ages 6 and 18 months. Children may be able to sit but cannot stand or walk without help and may have difficulty breathing.
- SMA type III (also known as Kugelberg-Welander disease) usually appears between 2 and 17 years of age, with symptoms that include abnormal gait (problems walking); difficulty running, climbing steps, or rising from a chair; and a slight tremor in the fingers. The lower limbs are most often affected. Complications include scoliosis and chronic shortening of muscles or tendons around the joints (contractures), which prevents the joints from moving freely. Individuals with SMA type III may be prone to respiratory infections.
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a rare, genetically distinct form of SMA. The disorder is caused by mutations in the IGHMBP2 (immunoglobulin helicase μ-binding protein 2) gene. Symptoms appear during infancy, between ages 6 weeks and 6 months.
Children with SMARD1 suddenly may be unable to breathe due to diaphragm paralysis and may develop weakness in their distal muscles. Congenital SMA with arthrogryposis is a rare disorder that appears at birth. Symptoms include severe muscle contractures, making babies unable to extend or flex the affected joints.
In the majority of cases, both the arms and legs are affected. Other symptoms include scoliosis, chest deformity, respiratory problems, unusually small jaws, and drooping of the eyelids. Kennedy’s disease (also known as spinal and bulbar muscular atrophy, bulbo-spinal muscular atrophy, X-linked spinal and bulbar muscular atrophy) is an X-linked recessive disease that affects men.
It is caused by mutations in the gene for the androgen receptor. Daughters of individuals with Kennedy’s disease are carriers and have a 50 percent chance of having a son affected with the disease. The onset of symptoms varies but most commonly the disease is first recognized between 20 and 40 years of age.
Generally, the disease progresses very slowly. Early symptoms may include:
- Tremor of outstretched hands
- Muscle cramps during physical activity
- Muscle twitches
- Weakness of the facial, jaw, and tongue muscles, leading to problems with chewing, swallowing, and speaking
Over time, individuals develop weakness in the arms and legs, often beginning in the pelvic or shoulder regions. They also may develop pain and numbness in the hands and feet. However, individuals tend to retain the ability to walk until the later stages of the disease, and many have a normal lifespan.
- Post-polio syndrome (PPS) can strike polio survivors up to four decades after they have recovered from the initial illness, which can cause major damage to motor neurons.
- Symptoms include fatigue, muscle and joint weakness, and pain that slowly gets worse over time, muscle atrophy and twitches, and decreased tolerance to cold.
These symptoms appear most often among muscle groups affected by the initial polio illness. Other symptoms include difficulty breathing, swallowing, or sleeping. Older people and those individuals most severely affected by the earlier disease are more likely to experience symptoms.
Does MND affect the brain?
Motor neurone disease (MND) is a rare neurological condition that causes the degeneration (deterioration and loss of function) of the motor system (the cells and nerves in the brain and spinal cord which control the muscles in our bodies). This results in weakness and wasting of the muscles.
Is MND palliative?
Abstract – The palliative care of a patient with motor neurone disease (MND) starts even at the time of diagnosis, and this care throughout the disease process is crucial to management in the later stages. Palliative care is defined as: ‘The active total care of patients whose disease is not responsive to curative treatment.
Control of pain, of other symptoms, and of psychological, social and spiritual problems is paramount. The goal of palliative care is achievement of the best possible quality of life for patients and their families.’ (World Health Organization 1990). As there is, at present, no cure for MND, and Riluzole, at best, only slows the progression of the disease.
Therefore, the care of patients with MND is palliative and this approach will enable them, together with their families, to retain as good a quality of life as possible. During the disease progression, there are times when there are particular
Has anyone ever recovered from MND?
Motor neurone disease (MND) is an uncommon condition that affects the brain and nerves. It causes weakness that gets worse over time. There’s no cure for MND, but there are treatments to help reduce the impact it has on a person’s daily life. Some people live with the condition for many years. MND can significantly shorten life expectancy and, unfortunately, eventually leads to death.
Is there any hope for MND sufferers?
We led a Phase 3 clinical trial of an investigational drug for a type of motor neurone disease. The results of the trial showed significant improvements in patients’ symptoms, with one patient in a wheelchair later able to walk without sticks. Motor neurone disease (MND) affects around 5,000 people in the UK every year.
- There is currently no cure.
- An international clinical trial led by Professor Dame Pamela J Shaw in the UK has been shown to slow down progression of a type of motor neurone disease and could be a turning point for patient care.
- The results of a Phase 3 clinical trial showed significant improvements in patients’ symptoms after 12 months, with one patient in a wheelchair at the start of the trial now able to walk without sticks.
While the treatment only applies to patients with a subtype of MND caused by a faulty SOD1 gene (approximately 2 per cent of all MND patients) this work has the potential to change the future of trials for all MND patients.
What is the terminal stage of MND?
Palliative and end of life care are often sensitive topics for people and need to be introduced carefully and preferably when initiated by the person with MND and/or their family. Introducing the concept of a palliative approach to care early on may help people to better understand the difference between palliative care and end of life care.
Establishing links with a specialist palliative care service, if available, may also help establish relationships to support choice and control and optimal end of life care. The person with MND is likely to have fears and questions about death and about how they will die and be cared for in the very final phase of the disease.
The person with MND and their family may also have questions about when to go to hospital or a hospice, or whether they can stay at home for as long as possible, or be supported to die at home. Addressing these concerns through ongoing discussion, assistance with completing an advance care plan, appropriate support and the provision of evidence based and quality information will help allay fear and support people to plan ahead.
- It is very important to be attuned to the patient’s readiness to have these discussions as well as their style of decision-making.
- The MND Australia publication “End of Life Care: A guide for people living with MND” is available to help make these sensitive and important conversations easier.
- Health professionals and providers may find the guide a useful resource to share when the topic of end of life care and MND comes up in conversation.
Having the guide on hand and ready to be shared in a discrete and organic way, rather than directly, can help to better support someone learning about end of life care on their terms, and when they feel ready. There are five parts in the guide. Each part has individual sections that provide specific information and advice on a common theme.
- For example, Part 1: What Do I Need To Know? introduces some of the most important concepts, like the benefits of being organised, the need to have plans in place for medical, financial, legal and family needs, and when is a good time to start talking about end of life care.
- Later in the guide, Part 3: What my family, friends and others close to me need to know explores ways to release tension and work through emotions through conversations with family and children.
The five parts are: 1: What do I need to know? 2: Things I’m likely to experience 3: What my family, friends and others close to me need to know 4: What else might I need to consider? 5: How do I get more help? Call the MND Info Line (Freecall 1800 777 175) to speak to the MND Association in your state or territory about the guide, ordering copies and what may help when sharing it with patients.
Initiating discussions about end of life is often also very difficult for health professionals and providers. It is therefore important for the team to meet regularly to coordinate care, discuss how and when to introduce discussions regarding end of life care and advance care planning, and to provide peer support.
The availability of professional supervision is also very important for health professionals and service providers involved in the care of people living with MND. As MND has no curative treatment and has a prognosis for many of only 2–3 years, end-of-life issues may need to be considered early in the disease progression, particularly as there may be delays in diagnosis so that the disease has progressed by the time the diagnosis has been made.
- There is evidence that patient and families do appreciate the discussion of end-of-life issues and the NICE guideline recommended that end of life should be discussed if the patient or family ask and when considering and commencing new interventions, such as gastrostomy and NIV.
- However, there is often reluctance to discuss the issues of dying and death, although many patients and families have great concerns about the future.
There are many myths that dying of MND is ‘distressing’ and ‘horrendous’ and is ‘due to choking’,These myths need to be confronted and evidence provided that death can be peaceful (Oliver, 2019). End of life conversations can be a source of great anxiety and discomfort for the patient, their families and health care professionals, and as a result such discussions may be avoided.
The issue of advance directives should be raised soon after the diagnosis (Jackson et al 2015). The experience at the end of life can have a significant impact on patients and their families. Due to the complexities of management of advanced ALS it is recommended that specialist palliative care services are involved early and throughout the disease.
Advanced care planning allows patients and their families to document their attitudes towards future events but enactment of these wishes may not always be straightforward and decisions should be reviewed regularly (Hobson and McDermott 2016). Goals of treatment should be discussed regularly with the person living with MND and their carer.
As MND progresses to the end of life phase, the goal of palliative care changes from maximising function to providing compassionate, person centered care according to the person’s beliefs and preferences. Good symptom management and access to psychosocial, emotional and spiritual support, based on the needs of the individual, is essential in order to achieve a peaceful and dignified death and to support the carer and family.
Refer to Psychological support for people living with MND section, As end of life approaches it is vital that the health professionals involved regularly check symptoms and ensure appropriate treatment and support. Refer to the Managing Symptoms section.
- Support services in the home may be need to be increased as end of life approaches, particularly if the person with MND has expressed a wish to die at home.
- An urgent review of the person’s NDIA plan or aged care package and referral to community nursing service should be considered.
- In addition assistive technology needs should be reviewed to ensure access to appropriate aids and equipment to support comfort and carer safety.
Aids and technology used to assist communication may also need to be reviewed. Some people may express a need or desire for spiritual counselling and appropriate information and referrals should be made. It may be appropriate to explore the use of complementary therapies such as massage, music therapy, aromatherapy and other relaxation techniques.
opioid analgesics – reduce cough reflex, relieve dyspnoea, control pain and help to reduce fear and anxiety anti-cholinergics – such as hyoscine hydrobromide and glycopyrrolate reduce saliva and lung secretions sedatives – such as diazepam, midazolam, clonazepam and chlorpromazine reduce anxiety oxygen – to relieve the sensation of breathlessness haloperidol – for terminal restlessness
Dosage and modes of administration of medications should be discussed with the palliative care physician. Carers may be trained and supported to provide and administer medications. The terminal stage is recognised as progressive weakness and often a sudden deterioration over a few days or hours.
- The most common cause of death is respiratory failure, usually following upper respiratory tract infection.
- The terminal stage may be preceded by reduced chest expansion, a quietening of the breath sounds, use of accessory muscles (if any are left) and morning headache from CO2 retention overnight.
- Signs may be noticed by the carer or a member of the care team and it is important to prepare the family for the coming days and the patient’s imminent death (often after several years of disease).
Preparing the family and providing additional support may help prevent the shock of an ‘unexpected’ death (with the risk of more severe bereavement) or the family inadvertently ringing an ambulance after the patient has died at home. Note: It is important to reassure patients and carers that death from choking is rare.
The end-of-life phase in MND is variable from one patient to another. This phase is marked by any combination of significant functional decline, respiratory failure, dysphagia, marked weight loss, recurrent infection and cognitive decline. Symptoms such as dyspnoea can be managed effectively at the end-of-life phase with appropriate pharmacotherapy, such as subcutaneous morphine (Lau et al 2018).
The value of laycarers in helping palliative care patients to remain at home cannot be underestimated. Laycarers provide psychosocial and physical care, but it needs to be remembered that they have real needs of their own. Palliative care service providers need to support laycarers so that they can be successful and robust in their caring role.
This study demonstrates that skilling laycarers to provide subcutaneous injections gives them confidence and that this confidence increases significantly with experience. Embedding laycarers as part of the multidisciplinary community palliative care team, by teaching them to prepare and administer medications for breakthrough symptoms and monitor symptom relief, is likely to further empower them in their caring role (Healy et al 2018).
Anticipatory prescribing of medications to treat symptoms at the end of life may enable informal carers to administer medication whilst subcutaneous infusions may be required for on-going symptoms (Table 3). The Motor Neurone Disease Association has developed a Just-in-case box in which medication for carers or clinicians (ambulance service or nurses) to use to treat terminal symptoms can be stored and recorded (Hobson and McDermott 2016).
- Dyspnea, despite liberal use of NIV, can be treated with morphine starting at 2.5–5 mg every 4 hours and increased in dose and frequency as needed.
- Anxiety and restlessness can be treated with lorazepam 0.5–2 mg every 4 hours as needed.
- 88 Rocha) Opiates and anxiolytics doses should be increased if they are not providing satisfactory control (Jackson et al 2015).
MND causes progressive loss of independence and an increased need for help with activities of everyday life. Subsequently carers will progressively increase the time they devote to caring. Identifying and addressing the physical, emotional, social and psychosocial needs of the carer is therefore integral to end of life care in MND.
- The progression of MND is unpredictable and individual.
- Death may occur more quickly than anticipated and it is therefore very important that carers and family members are prepared and provided with ongoing practical and emotional support.
- It is important that care plans and information is shared by all members of the care team, and adequate nursing cover needs to be maintained at the home, hospice, residential aged care facility or hospital.
An out of hours contact number for the carer and information on what to do in an emergency is vital. Regular reassurance that all will be done to manage symptoms as they arise to ensure a peaceful death is imperative. The carer should be encouraged to seek and ask for additional support.
The health professionals and service providers involved in providing support and services in the home should review needs regularly and make referrals to increase services or access assistive technology as, when and if available. Loss of speech and the impact on the person’s ability to communicate with loved ones may be particularly distressing for carers.
Regular assessment and review of AAC devices to help maintain effective communication as the person nears end of life will help to minimise carers social and emotional isolation. For some people maintaining care at home may become too difficult and admission to a hospice or palliative care facility, hospital or a residential aged care facility may need to be organised.
- It is important that the carer and family members are supported through the decision making process and reassured that their loved one will be well cared for whatever the setting.
- Our study highlighted a lack of death preparedness amongst bereaved carers of patients with MND.
- Death preparedness can be defined as the readiness for death experienced by the patient and their carer.
A contributing factor to being prepared for death is also an awareness of dying, however carers portrayed that they were unaware the patient with MND was going to die. This finding confers with previous research demonstrating a lack of death preparedness amongst patients and carers with other forms of non-malignant conditions, such as non-malignant respiratory disease.
- The present study also aligned with the theory of closed awareness which refers to the patient, and potentially the carer, being unaware of the patient’s poor prognosis however the healthcare professionals involved in their care are aware and have not shared this information.
- This demonstrates the importance of effective information sharing between healthcare professionals, patients with MNDs and their carers.
Additionally, this particular finding may provide further insight into why less than half of patients in the present study had completed an ADRT (McVeigh et al 2019). With most of the care and support occurring at home, family carers of PwMND endure adverse effects on their physical, emotional and social wellbeing and they often describe their caring experiences as unrelenting due to the progressive nature of the disease and the relative hopelessness with respect to recovery.
- To ameliorate some of the negative effects of caregiving, family carers need support in their dual role: support to enable them to care for the patient (their ‘coworker’ role) and also direct support for their own health and wellbeing (their ‘client’ role),
- As Miles and Asbridge described family carers, they are vital partners in increasing the person-centeredness of health and social care systems (Aoun 2018).
Common fears of patients faced with imminent death are choking and respiratory distress. Having early discussions with the patient and their carers regarding what is to be expected at various phases of the disease, particularly the terminal phase, can allay these fears.
Throughout the illness, the role of the carers is pivotal. A significant role for the polyclinic is the physical and emotional support of carers (Lau et al 2018). Results : the needs of patients and carers are not being adequately met in the final stages of MND and there appears a need for increased, co-ordinated support from palliative care services.
The use of advance care planning tools is regarded as beneficial for patients and carers, but health professionals demonstrate a limited understanding of them. Anxiety and distress in patients, carers and bereaved carers is heightened during this period.
Carer burden is excessive and may exacerbate patient distress and desire for hastening death (Whitehead et al 2012). Carers, family and friends will likely experience feelings of grief and loss throughout the disease trajectory. The progressive nature of the condition also means that the carer and family will have provided increasing care and support during the course of the disease.
This can take its toll on physical and psychological health and wellbeing particularly for the primary carer. Provision of person centred services and support, for both the person with MND as well as their carer and family, is vital in helping them to cope with grief and loss and to maintain their own health and wellbeing.
- Refer to Wellbeing and support section,
- Bereavement can be very distressing and isolating.
- Optimal care and support from health professionals, and all those involved in providing care, during the end of life period will help to prepare the carer and family for the death of their loved one.
- Feeling prepared and well supported is important in helping to ameliorate the impact of bereavement.
For many carers there will have been weeks and maybe even months of intense caring and support from a range of providers, family and friends. Following death this may suddenly stop. It is therefore very important that follow up is embedded into practice, based on the needs and wishes of the individual.
Bereavement counselling and support should be offered to all carers. Some people living with MND may wish to create a digital legacy, written stories or messages for their loved ones to read and reflect on following their death. These may provide comfort for the family, particularly children. Health professionals and providers may like to provide information and an opportunity to discuss this option with the person with MND and their carer early on in the disease trajectory.
If interest is expressed then a health professional, service provider or volunteer could be identified to support the person to develop their digital legacy. Creating, or using, a digital legacy by people affected by motor neurone disease generates not only challenges and barriers but also benefits.
- It provides a way for people living with the disease to capture their identity and biography enabling a continuation of existence after their death, in a digital form.
- Similarly, bereaved young people gain a sense of comfort from using a digital legacy, to see, hear and learn about the deceased (Clabburn et al 2019).
The last cornerstone of a palliative approach is bereavement support. Nevertheless, one of the modern public health paradoxes is the neglect of the bereaved (though bereavement is a universal experience). Palliative care standards propose that bereavement support should be matched to risk and need.
What is palliative treatment for MND?
Palliative care for MND Palliative care is active holistic care of people with advanced progressive illness. It involves: management of pain and other symptoms. psychological support.
What is the MND cure 2023?
What is MND SMART? – MND SMART (Motor Neurone Disease – Systematic Multi-Arm Adaptive Randomised Trial) is ‘multi-arm’ meaning more than one treatment will be tested at the same time.The trial will initially test medicines that are already approved as treatments for other disorders in the UK.
- Trial participants taking the different treatments will be compared with a single group who receive a placebo (a dummy drug).
- This means that people in MND-SMART are more likely to receive an active treatment when compared to standard clinical trials where half of participants receive the placebo and half the active treatment.
The trial started out with 3 arms; drug 1 (Memantine Hydrochloride – phase 2), drug 2 (Trazodone Hydrochloride – phase 3) and placebo (dummy drug). This allows the evaluation of drug 1 versus placebo and separately drug 2 versus placebo. Participants will be randomly allocated to either drug 1, drug 2 or placebo.
- Medicines being tested are already approved for use in other conditions.
- Currently recruiting in trial centres across the UK.
- A new drug, called Amantadine Hydrochloride, was added to the trial in April 2023.
- New drugs will be selected for investigation in MND-SMART based on continuous review of constantly updated scientific evidence as well as findings from state-of-the-art human stem cell based drug discovery platforms.
You can find out more about the trial here,
How long does ALS last before death?
Understanding ALS Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. They govern voluntary movements and muscle control. ALS causes these motor neurons to degenerate over time until they eventually die. When the motor neurons die, the brain can no longer initiate and control muscle movement.
When voluntary muscle action is progressively affected, people may lose the ability to speak, eat, move and breathe. ALS was identified in 1869 by French neurologist Jean-Martin Charcot, but it became more widely known internationally in 1939 when it ended the career of one of baseball’s most beloved players, Lou Gehrig.
For many years, ALS was commonly known as Lou Gehrig’s Disease. “Amyotrophic” comes from the Greek language. “A” means no. “Myo” refers to muscle. “Trophic” means nourishment. So, amyotrophic means “no muscle nourishment,” and 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.
- ALS is a disease that typically involves a gradual onset.
- The initial symptoms of ALS can be quite varied.
One person may have trouble grasping a pen or lifting a coffee cup, while another may experience a change in vocal pitch when speaking. The rate at which ALS progresses can be quite variable, as well. Although the mean survival time with ALS is two to five years, some people live five years, 10 years or even longer.
- Symptoms can begin in the muscles that control speech and swallowing or in the hands, arms, legs or feet.
- Not all people with ALS experience the same symptoms or the same sequences or patterns of progression.
- However, progressive muscle weakness and paralysis are universally experienced.
- Every 90 minutes, someone is diagnosed with the disease, and someone passes away from it.
Most people who develop ALS are between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. However, cases of the disease do occur in people in their twenties and thirties. ALS is 20% more common in men than women. However, with increasing age, the incidence of ALS is more equal between men and women.
About 90% of ALS cases occur without any known family history or genetic cause. The remaining 5-10% of ALS cases – known as familial ALS (FALS) – are inherited through a mutated gene with a known connection to the disease. For unknown reasons, military veterans are more likely to be diagnosed with the disease than the general public.
: Understanding ALS
How fast do you deteriorate with ALS?
ALS progression timeline – There can be substantial variation from person to person in how quickly ALS progresses. While the average survival time among people with ALS is up to five years from the onset of the disease, about 1 in 5 patients will live at least five years, and 1 in 20 will survive two decades or longer.
While there is no cure for ALS, and no therapies can reverse symptoms that have already accrued, several treatments can help to slow the decline in function over time for people living with ALS. Some also can extend survival by a few months. This means that ALS stages will have variable duration among patients.
While it’s difficult to determine how long patients will remain in each of the four general stages of ALS disease progression, studies examining more formal disease stages can provide a general idea. One of the formal scales more commonly used in studies to document ALS progression is the King’s College staging system.
Stage 1, representing the early stage of disease, typically lasts from about nine months up to 1.5 years. In general, patients with limb-onset disease and those who receive ALS treatment early in the disease course will stay longer in this stage, Stages 2 and 3, which can be generally thought of as the middle stage of ALS, together last anywhere from about nine months to just longer a year for most patients. Stage 4, typically corresponding to the late stage, usually lasts about four to seven months for most individuals, though it may be longer depending on what kinds of life-supporting treatments are given. The time to stage 5, which can be seen as the duration of the end stage, is usually about three months for most patients.
The MiToS system is another commonly used formal scale that assesses whether patients have impairment in four main areas of functionality: movement, swallowing, communication, and breathing. It stages patients on a scale of 1 through 4 based on how many of these areas are impaired.
A stage 0, indicating no impairment, is included in the MiToS system, as is a fifth stage representing death from ALS. As the King’s College and MiToS systems measure different but complementary information, they are often used in combination to help track patients’ progression. ALS News Today is strictly a news and information website about the disease.
It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.
What is the most indicative of imminent death for someone with ALS?
ALS Signs of Impending Death – Hands, feet, and limbs are the most common places where ALS death symptoms begin to show before spreading across the body. Most ALS patients succumb to respiratory failure, which occurs when they are unable to obtain enough oxygen from their lungs into their bloodstreams.
What is the final stage of terminal illness?
Physical changes – The body begins its natural process of slowing down all its functions. How long this takes varies from person to person. It might take hours or days. The dying person will feel weak and sleep a lot. When death is very near, you might notice some physical changes such as changes in breathing, loss of bladder and bowel control and unconsciousness.
- It can be emotionally very difficult to watch someone go through these physical changes.
- But they are part of a natural dying process.
- They don’t mean that the person is uncomfortable or in distress.
- The doctors and nurses looking after the person will regularly check for these changes.
- They will do all they can to make your relative or friend as comfortable as possible during their death.
If you are looking after someone at home while they are dying, you should have support from a specialist community nurse, district nurses and the GP. They can answer your questions and help make home nursing easier for you.
What happens when a motor neuron dies?
NEW YORK, NY (February 6, 2014) — In most cases of amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease, a toxin released by cells that normally nurture neurons in the brain and spinal cord can trigger loss of the nerve cells affected in the disease, Columbia researchers reported today in the online edition of the journal Neuron.
The toxin is produced by star-shaped cells called astrocytes and kills nearby motor neurons. In ALS, the death of motor neurons causes a loss of control over muscles required for movement, breathing, and swallowing. Paralysis and death usually occur within 3 years of the appearance of first symptoms. The report follows the researchers’ previous study, which found similar results in mice with a rare, genetic form of the disease, as well as in a separate study from another group that used astrocytes derived from patient neural progenitor cells.
The current study shows that the toxins are also present in astrocytes taken directly from ALS patients. “I think this is probably the best evidence we can get that what we see in mouse models of the disease is also happening in human patients,” said the study’s senior author, Serge Przedborski, MD, PhD, the Page and William Black Professor of Neurology (in Pathology and Cell Biology), Vice Chair for Research in the department of Neurology, and co-director of Columbia’s Motor Neuron Center,
The findings also are significant because they apply to the most common form of ALS, which affects about 90 percent of patients. Scientists do not know why ALS develops in these patients; the other 10 percent of patients carry one of 27 genes known to cause the disease. “Now that we know that the toxin is common to most patients, it gives us an impetus to track down this factor and learn how it kills the motor neurons,” Dr.
Przedborski said. “Its identification has the potential to reveal new ways to slow down or stop the destruction of the motor neurons.” In the study, Dr. Przedborski and study co-authors Diane Re, PhD, and Virginia Le Verche, PhD, associate research scientists, removed astrocytes from the brain and spinal cords of six ALS patients shortly after death and placed the cells in petri dishes next to healthy motor neurons.
- Because motor neurons cannot be removed from human subjects, they had been generated from human embryonic stem cells in the Project A.L.S./Jenifer Estess Laboratory for Stem Cell Research, also at CUMC.
- Within two weeks, many of the motor neurons had shrunk and their cell membranes had disintegrated; about half of the motor neurons in the dish had died.
Astrocytes removed from people who died from causes other than ALS had no effect on the motor neurons. Nor did other types of cells taken from ALS patients. The researchers confirmed that the cause of the motor neurons’ death was a toxin released into the environment by immersing healthy motor neurons in the astrocytes’ culture media. The presence of the media, even without astrocytes, killed the motor neurons.
- How the Toxin Triggers Motor Neuron Death The researchers have not yet identified the toxin released by the astrocytes.
- But they did discover the nature of the neuronal death process triggered by the toxin.The toxin triggers a biochemical cascade in the motor neurons that essentially causes them to undergo a controlled cellular explosion.
Drs. Przedborski, Re, and Le Verche found that they could prevent astrocyte-triggered motor neuron death by inhibiting one of the key components of this molecular cascade. These findings may lead to a way to prevent motor neuron death in patients and potentially prolong life.
But the therapeutic potential of such inhibition is far from clear. “For example, we don’t know if this would leave patients with living but dysfunctional neurons,” Dr. Przedborski said. The researchers are now testing the idea of inhibition in animal models of ALS. New Human Cell Model of ALS Will Speed Identification of Potential Therapies The development of new therapies for ALS has been disappointing, with more than 30 clinical trials ending with no new treatments since the 1995 FDA approval of riluzole.
The lack of progress may be partly because animal models used to study ALS do not completely recreate the human disease. The new all-human cell model of ALS created for the current study may improve scientists’ ability to identify useful drug targets, particularly for the most common form of the disease.
- Although there are many neurodegenerative disorders, only for a handful do we have access to a simplified model that is relevant to the disease and can therefore potentially be used for high-throughput drug screening.
- So this model is quite special,” Dr.
- Przedborski said.
- Here we have a spontaneous disease phenotype triggered by the relevant tissue that causes human illness.
That’s one important thing. The other important thing is that this model is derived entirely from human elements. This is probably the closest, most natural model of human ALS that we can get in a dish.” The paper is titled: “Necroptosis drives motor neuron death in models of both sporadic and familial ALS.” Other contributors are: from CUMC, Changhao Yu, Kristin Politi, Sudarshan Phani, Burcin Ikiz, Lucas Hoffman, Tetsuya Nagata, Dimitra Papadimitriou, Peter Nagy, Hiroshi Mitsumoto, Shingo Kariya; Martijn Koolen (CUMC and University of Amsterdam); Mackenzie Amoroso, Hynek Wichterle, and Christopher Henderson (CUMC and Project A.L.S.).
The research was supported by the NIH (grants U42RR006042, NS062180, NS064191, NS042269, NS072182, NS062055, NS078614, ES009089, TR000082, and ES016348 ), the U.S. Department of Defense (W81XWH-08-1-0522 and W81XWH-12-1-0431), Project A.L.S., P2ALS, the ALS Association, the Muscular Dystrophy Association/Wings Over Wall Street, the Parkinson’s Disease Foundation, Midwinter Night’s Dream Summer Research Program, the NIEHS Center of Northern Manhattan, and the Philippe Foundation .
The authors declare no financial or other conflicts of interest. #### Columbia University Medical Center provides international leadership in basic, preclinical, and clinical research; medical and health sciences education; and patient care. The medical center trains future leaders and includes the dedicated work of many physicians, scientists, public health professionals, dentists, and nurses at the College of Physicians and Surgeons, the Mailman School of Public Health, the College of Dental Medicine, the School of Nursing, the biomedical departments of the Graduate School of Arts and Sciences, and allied research centers and institutions.
What happens in end stage ALS?
Most deaths in ALS occur very peacefully. As the disease progresses, the diaphragm, the major muscle involved in breathing, becomes weaker. Therefore, it becomes more difficult to breathe. Noninvasive ventilators assist breathing and they can be effective for very long periods of time.