Motor Neurone Disease is a chronic, degenerative condition which affects the nerve cells that control movement.

What is Motor Neurone Disease

Motor Neurones are the nerve cells in the brain and spinal cord that are responsible for movement control. In Motor Neurone Disease, these nerve cells become damaged, and this damage is irreversible and worsens over time. Without healthy motor neurones, we lose our ability to control movement.

At the start of the disease, this may have a minimal effect on people. In about two thirds of people, the first symptoms are some weakness in the arms or legs. Over time, MND begins to affect all movement. This includes all movement of the limbs, and progresses to restrict a person’s ability to speak, swallow, and breathe.

Everyone’s experience of MND is a little different, and the pattern of disease progression may be slightly different from one person to another. The speed at which the disease progresses can also vary significantly – some people have very swift disease progression, and reach the end of their lives within only a few months.

Others may have very slow disease progression, and sometimes find that their symptoms plateau for periods of time. The average life expectancy after diagnosis of MND is around 3 years[i]. There are a few people who have lived for many decades after a diagnosis of MND, with some supportive care and life adjustments.

Symptoms of Motor Neurone Disease

Motor Neurone Disease is a disorder that affects movement. In the early stages, it can present as weakness of the muscles. Some of the first symptoms someone with MND might notice include:

  • Weakness in the ankles and legs – being more likely to trip or fall.
  • A weak grip – in the beginning, finding it difficult to perform tasks which require strong hands or fine motor skills, such as opening jars or gripping cutlery.
  • Loss of muscle mass – particularly noticing that arms or legs are getting thinner and weaker.
  • Speech difficulties – speech may become slurred.

As the disease progresses, the symptoms worsen. Over time, weakness progresses to total lack of control over movement. People with MND lose the ability to walk, talk, and look after themselves without considerable help and support.

The muscles that control breathing and swallowing are affected just as much as those that control mobility. The effect of the disease on these functions is usually the cause of decline at the end stage of MND. Without effective control over breathing, coughing and clearing the throat, respiratory problems can develop quickly. With a compromised ability to actively or reflexively swallow, nutrition and hydration can become a serious issue. Swallowing difficulties combined with reduced lung function make a person at very high risk of aspiration pneumonia.

Who is at Risk of Developing Motor Neurone Disease?

We know that some – around 10%[ii] – of people who develop MND have a genetic predisposition. People with this form of MND in the family may be offered genetic counselling – this means they can be offered a test to look for genes known to be associated with the familial form of MND. They will receive support and counselling for themselves and their family if they are found to be at high risk of developing MND.

For the 90% of cases of MND that don’t seem to follow a genetic pattern, it isn’t usually possible to anticipate the development of the disease. There are lots of theories and research is ongoing into the different effects that some viruses, inflammatory responses, environmental toxins and other possible culprits might have on the motor neurones. There is not, however, a single clear explanation as to why some people develop MND.

MND is more likely to develop as we get older, and most cases are diagnosed when people are in their 50s or 60s; the average age of diagnosis is 55[iii]. People with familial MND usually begin to develop symptoms a little younger, in their 40s to 60s.

Motor Neurone Disease Diagnosis

MND - Healthy vs. Damaged Neurone

Some of the earliest symptoms of Motor Neurone Disease can overlap with the symptoms of other conditions, so some investigations are needed before a definite diagnosis is made.

There is no one simple test that gives a definitive diagnosis of MND, so a diagnosis is reached by collating different pieces of information[iv]. This may include:

  • A description of symptoms, particularly a person’s description of the way symptoms have developed.
  • Physical examinations, which may look for evidence of weakness or reduced motor control.
  • Blood tests, to look for signs of muscle wasting, inflammatory response, and other problems that can accompany MND
  • Nerve conduction studies – these are tests where a tiny electrical impulse is used to stimulate a nerve, and the way this signal is passed through the nerve is measured.
  • Electromyography – this is a way of measuring the way the muscles respond to small electrical impulses.
  • Lumbar puncture (LP) – an LP involves taking a small sample of the fluid around the spinal cord with a fine needle.

Lumber Puncture

This can look for other causes of MND-type symptoms, like infections or inflammation affecting the brain and spinal cord.

  • Magnetic Resonance Imaging (MRI) – this is a very detailed scan to look for some other causes of symptoms which may mimic MND. An MRI can show some kinds of damage and inflammation around the brain, spinal cord, and nerves.

Treating Motor Neurone Disease

There is no cure for Motor Neurone Disease. It is a progressive disease which gets worse over time, and it is eventually fatal. The progression of the disease cannot be reversed, so the main aim of treatment for MND is to promote good quality of life for as long as possible throughout the disease, and to control symptoms. Having discussions early about what treatment would be appropriate or desirable as the disease progresses can help loved ones make difficult decisions.

Any disorder which significantly impairs a person’s ability to move, communicate, eat and drink, and independently respond to their body’s own needs comes with a high risk of causing certain other problems and conditions. Treatment for MND largely takes the form of symptom management, good nursing care, and the prevention of associated problems. Some of the issues that a person with MND might be at high risk of include:

  • Skin and pressure area damage: People unable to mobilise and to reposition themselves are at risk of developing damage caused by pressure to the skin and underlying tissues. A person’s own weight can cause damage to any parts of their soft tissue subject to these kinds of pressures. Good nursing care, with regular positional changes and pressure-relieving equipment like air-flow mattresses and cushions can help mitigate this risk.
  • Continence: Reduced mobility is also associated with issues around continence – this includes the practicalities of being unable to get to the toilet independently, reduced control over the muscles that support the bladder and bowels, and difficulty in communicating one’s own needs. Constipation is also a potentially serious problem associated with reduced mobility and bowel motility.

 

  • Nutrition and hydration: as MND progresses, the ability to swallow becomes impaired. This can cause problems with staying nourished and hydrated, and some people might consider alternatives to oral nutrition, such as having a PEG – a tube into the stomach for feeding, water, and medications. Having a reduced ability to control swallowing also makes us more prone to having food or fluid enter the lungs, causing aspiration pneumonia. The simple, practical loss of ability to express needs and feed oneself is a huge risk factor for malnourishment and dehydration in MND.
  • Depression: a combination of a terminal disease diagnosis, impact on home and family life, ability to work and pursue the things a person enjoys in their life, increased dependence on others, and lots of other worries and problems can lead to serious mood and emotional problems in MND. Concerns over finances and worrying about what will happen to their loved ones after the person with MND reaches the end of their life are also common. Communication is always key in helping tackle these kinds of problems, and support from clinical psychologists and counsellors is invaluable – for everyone affected by one person’s MND.
  • Breathing: as MND progresses to the later stages, control over breathing becomes impaired. It may be necessary and appropriate to use a mechanical ventilation machine to support breathing. As MND is progressive, the muscles that control breathing as well as all other body movements will become weaker and weaker, and there may be a point where a person with MND cannot breathe without a ventilator.

Having a ventilator will not stop or slow the progression of the disease, but can help to bypass some of the breathing problems that come with MND, controlling some symptoms and supporting quality of life for as long as possible. It’s a good idea to have discussions about what level of breathing support would be appropriate and acceptable at the latter stages of MND.

  • Pain: muscle cramps, muscle wasting, problems with joints associated with immobility, and sometimes pain which doesn’t have a clear origin is common in MND. Chronic pain seriously impairs quality of life and needs to be carefully managed by specialists.

In the later stages of MND, care may be referred to as ‘palliative care’; this means that the aim is symptom control, comfort, and support for the person with MND and those close to them. Any symptoms a person experiences at the end stage of a terminal disease can be well controlled so they remain comfortable and calm as they reach the end of their life.

Challenges of Motor Neurone Disease

Receiving a diagnosis of Motor Neurone Disease can be overwhelming; it means a huge change in the way we live and in the lives of the people closest to us. A diagnosis of a terminal disease raises uncertainties and questions – from the emotional, psychological and physical changes to expect, to the practicalities of reaching the end of a person’s life.

Conversations about the later stages of MND can be difficult to begin. It is hard to talk about terminal illness, death, and the reality of living with a disease that will become so completely limiting. It can be very helpful for everyone involved, however, if a person’s wishes surrounding their care and treatment in the later stages of the disease are shared and understood[v].

Living with Motor Neurone Disease

Motor Neurone Disease is a terminal disease which severely limits a person’s function, independence, lifestyle, and longevity. The kind of care and support a person with MND needs will change over the course of the disease. Generally, at some point after diagnosis, someone with MND will begin to need some minimal assistance with their symptoms; this might be mobility or communication support. As the disease progresses, and towards the later stages, carers – whether family, formal carers, or a mix of both – will be needed for support with all activities of daily living.

People with MND can expect to have ongoing support from teams made up of specialist doctors; nurses; dieticians; occupational, physical, and speech and language therapists; clinical psychologists and counsellors, and more.[vi] In the later stages of the disease people with MND usually have significant or total care needs, and a formal care package is usually necessary. The complications that can arise with movement disorders – continence issues, risk of pressure damage, nutrition and communication challenges – require very specialised care, and some people need support round-the-clock to help keep them safe, comfortable, and content in the later stages of their disease.

Family members and close friends often take on an increasingly demanding role in the care of a person with MND – the gradual progressive nature of the disease means that it can be difficult to pinpoint the exact time when an informal carer needs more support. Arranging a care package early helps ensure that no one reaches a crisis point before they receive the support they need. The difference a supportive full-time care package can make to quality of life for everyone affected by MND is immeasurable.

[i] Turner MR, Parton MJ, Shaw CE, et al (2003) Prolonged survival in motor neuron disease: a descriptive study of the King’s database 1990–2002 Journal of Neurology, Neurosurgery & Psychiatry;74:995-997. https://jnnp.bmj.com/content/74/7/995

[ii] Shaw P J. (1999) Motor neurone disease BMJ 318 :1118 https://doi.org/10.1136/bmj.318.7191.1118

[iii] Azam, S & Leigh, P.N. (2007) Motor Neuron Disease and the Elderly. Geriatric Medicine https://www.gmjournal.co.uk/media/21414/mar07p61.pdf

[iv]McDermott C J, Shaw P J. (2008) Diagnosis and management of motor neurone disease BMJ; 336 :658 https://doi.org/10.1136/bmj.39493.511759.BE

[v] McLeod, J. E., & Clarke, D. M. (2007). A review of psychosocial aspects of motor neurone disease. Journal of the neurological sciences, 258(1-2), 4-10. https://doi.org/10.1016/j.jns.2007.03.001

[vi] Leigh PN, Abrahams S, Al-Chalabi A, et al (2003) The management of motor neurone disease. Journal of Neurology, Neurosurgery & Psychiatry74:iv32-iv47. http://dx.doi.org/10.1136/jnnp.74.suppl_4.iv32

Brain injuries come in many forms. Some are present from birth: these can be congenital, meaning a person is born with them; inherited, meaning they are passed on from one or both parents; or can happen from complications in pregnancy, or during or around the time of birth. Acquired brain injury (ABI) is a brain injury that is not present at birth, but which happens at a later stage in a person’s life.

Acquired brain injury is not uncommon, with around 350,000 people admitted to hospital in the UK every year with an ABI, usually related to a traumatic head injury or stroke.

What is an Acquired Brain Injury? 

An ‘acquired brain injury’ refers to any injury to the brain that happens after birth. They are ‘acquired’ – meaning that they happen to a person, usually with a clear specific cause. The effects of an ABI can range from extremely mild to completely debilitating, and the experience of an ABI varies from one person to another.

The brain is the part of the body that interprets signals and sends instructions to the rest of the body. Every sense we have – sight, smell, touch, taste, hearing, pain, our thoughts and emotions, knowing when we need to go to the toilet or recoil from a hot surface – relies completely on the brain functioning well. Every way we have of understanding the world is controlled by the brain. This means that damage to the brain can affect any part of the rest of the body, and can affect the body functions we aren’t aware of as well as those we are.

Different parts of the brain control and respond to different parts of the body, so the placement and amount of damage has a big impact on the way a brain injury presents and the effect it has on a person. Injuries to the front, back, left or right of the brain all have very different effects, and within the brain are lots of complex, subtle parts which can be damaged and cause problems.

For example, people with certain kinds of damage to the hypothalamus – a part of the brain which regulates lots of hormones and body processes – can experience feelings of constant hunger which is never satisfied, no matter how much someone eats; this can lead to depression, obesity and related diseases[i].

Hypothalamus

Changes in behaviour and personality are common in people with certain sorts of brain injury, and there are even a few examples of people who, after injury to part of their brain, develop unsuspected talents[ii].

Brain injuries commonly result in some degree of cognitive impairment, affecting memory function, thought processes, attention, understanding and communication. In some cases, this may resolve partly or fully. Sometimes, changes in creative thinking or memory function aren’t always obvious at first, but become more noticeable when someone goes back to their normal environment and activities after the injury.

Causes and Types of Acquired Brain Injury

The causes of acquired brain injury are very varied, but may be broadly grouped into traumatic[iii] – i.e. resulting from an accident or injury inflicted on the head and brain, or atraumatic – i.e. resulting from something internal. Some of the causes of ABI can include:

  • Stroke – a bleed, blockage, or narrowing in a blood vessel in the brain can cause serious and extensive damage to the brain. A narrowing in a blood vessel supplying the brain restricts blood flow and oxygen perfusion. Any tissues in the body very quickly become damaged and cells die without adequate perfusion, which is why time is of the essence when someone has a suspected stroke.
  • Traumatic head injury – the brain is well-protected within the skull from many forms of injury or accident, but the kind of accident where the brain is effectively thrown against the inside of the skull can cause bruising, bleeding, and swelling to the brain. This in itself can constitute a serious injury, but the effects of a bleed or significant swelling within the encased skull can put pressure on the whole brain and even damage parts of it which weren’t immediately impacted by the injury.

Close,Up,Hand,Doctor,Point,Mra,Brian,Scan,Image,Of

Road traffic accidents are the most common cause of traumatic head injuries, followed by sporting accidents. Injuries to the head do not necessarily mean that the brain has been affected at all.

  • Toxicity – i.e. poisoning, which could be through drugs, alcohol, or even occupational exposure.
  • Cancer or other diseases – brain tumours or cancers which metastasize (spread) to the brain can cause ABI. Other diseases which affect the brain, such as forms of dementia, can be considered a form of ABI, although they’re usually grouped separately as the management and trajectory is so different from acute acquired brain injury.
  • Hypoxic or anoxic brain injury – this is a brain injury caused by lack of blood supply to the brain. Hypoxic means there has been too little oxygen supplied to the brain; anoxic means there has been no oxygen supplied to the brain. A stroke is a hypoxic brain injury, but the term may be used more broadly to describe an ABI caused by a circulation problem that is external to the brain itself. This usually causes more universal damage, whereas a stroke is generally more regional within the brain. A cardiac arrest – where a person’s heart stops pumping blood around the body, drowning, or carbon monoxide poisoning are some of the most common causes of hypoxic brain injury.

Treating Acquired Brain Injury

The initial treatment of a brain injury depends on the cause and nature of the injury itself. Depending on the nature and extent of the injury, and the extent of the effect on the rest of a person’s function, an ABI may require an extended stay in hospital and a long period of rehabilitation. The prognosis varies from full recovery or minimal impact on a person’s abilities, to being almost entirely debilitating, affecting almost all voluntary and much involuntary brain function.

Diagnosis

Diagnosing a brain injury takes a combination of medical assessments and investigations. Medical imaging like computed tomography (CT) or magnetic resonance imaging (MRI) scans are used to create a picture of the brain and visualise areas of damage or abnormalities. Experienced radiologists using MRI scanners are able to detect even very subtle changes in the tissues of the brain, but the delicate brain tissues can be affected even by microscopic damage.

Sometimes, someone with a brain injury might have to have a series of scans at set intervals to look for ongoing changes. Doctors attending someone with a suspected brain injury will need to know about the history of the injury and the person’s symptoms, and will do a physical examination which will include shining a torch in their patient’s eyes, assessing their consciousness, cognition, and control over movement and sensation in different areas of the body.

Treatment

Treatment for an ABI includes treatment for the injury itself, and, where possible, treatment of the underlying cause. An atraumatic ABI is more likely to have an underlying cause that needs to be managed – a medical condition which has caused an injury to the brain.

Treatment for an ABI will usually involve assessment and treatment in an acute hospital setting. This may be in intensive care unit, stroke unit, or specialist brain injury department. Some brain injuries can be managed on more general medical wards. Some injuries may require a medical procedure or surgery, and some may be managed more conservatively with medications and monitoring; no two brain injuries are the same.

Rehabilitation

Rehabilitation is an incredibly important part of care after an ABI; the aim of rehabilitation is to help people get as independent and well as they can be after their injury, and then to ensure that any ongoing support they might need will be available to them.[iv] It isn’t always possible to anticipate the length of time it takes to rehabilitate to a stable condition and level of function; with a severe brain injury it can take many months. There can also be wide variances in the extent that the brain itself will recover – some kinds of tissue damage are irreversible, whereas some can resolve to some degree over time.

Female hand trying to connect a missing jigsaw puzzle of human brain on gray background. Creative idea for solving problem, memory loss, dementia or Alzheimer's disease concept. Mental health care.

Rehabilitation after an ABI may incorporate:

  • Physiotherapy, especially where movement or mobility has been affected.
  • Occupational therapy, with an aim to adapt or relearn functional living skills.
  • Speech and language therapy, to support changes in communication needs, and also when swallowing reflexes are affected.
  • Counselling or input from a clinical psychologist.
  • Consultant-led medical care for ongoing optimisation of treatment, or to manage and monitor related conditions.

Some people with ABI need to have long-term follow up with consultants or specialist brain injury support teams including nurses, doctors, and other healthcare professionals. Some rehabilitation activities can continue at home.

The Impact of an Acquired Brain Injury

Sustaining a severe acquired brain injury is likely to have a very large impact on a person’s life. It also affects the people closest to them, and can change everybody’s life in significant and important ways.

As a family member

Learning that a loved one has sustained a serious injury is always devastating, and when that injury involves the brain, it can be even more overwhelming. Injuries involving the brain can be unpredictable, they can impact on every aspect of a person’s life, abilities, and even personality.

When someone has a severe brain injury, the rest of their close family and household may have serious concerns over income, expenses, their home, future care of the affected person, the impact on children in the family, and more – all while their loved one is critically ill[v].

This combination of worries, both practical and uncontrollable, is acutely distressing. Having support from others, whether friends and family, the medical and rehabilitation teams, or specialist support organisations, can be invaluable.

As a person with an ABI

Someone with a serious ABI may fully understand the extent of their injuries and the impact it will have on their, and their loved ones’ lives. On the other hand, it may be difficult to comprehend – some severe brain injuries have a serious and permanent impact on a person’s ability to process and remember information.

When someone recovers enough from a serious brain injury to understand their condition and the potential consequences, it can be very distressing, even depressing[vi]. Any impact on brain function is an emotionally charged issue – to learn that you have sustained an injury that might affect your ability to communicate, interact with your family in the same way, to work, simply to go about your usual life independently is overwhelming.

Sometimes, brain injuries themselves can cause personality and mood changes – and sometimes living with brain injuries can cause these changes too. It’s important to know that even with the most severe brain injuries, you are important to your family and to society, and that there’s a reason that there is support available – it is because you are needed.

Living with Acquired Brain Injury

Rehabilitation following an ABI can continue for as long as it is helpful – being medically stable is only the beginning of life after an ABI. Input from healthcare professionals, social support and carers can help get people with ABIs back to their normal life.

Sometimes, someone might need a lot of adjustments in their living conditions when they leave hospital – a home that is adapted for access and safety, and they may need a package of care to ensure that someone is always with them to support physical or cognitive needs.

Depending on the extent of impact from the ABI, getting back to work, education, social or recreational activities may be possible with time and adaptation. Any recovery takes time, and the nature of an injury to the brain; an injury which can cause a range of cognitive as well as physical effects, can be particularly challenging. Organisations like The Brain and Spine Foundation and Headway, which provide practical advice as well as emotional support can be an essential resource. Everyone who is affected by an acquired brain injury – whether to themselves, a friend or family – might need support.

[i] Ahima, R. S., & Antwi, D. A. (2008). Brain regulation of appetite and satiety. Endocrinology and metabolism clinics of North America37(4), 811–823. https://doi.org/10.1016/j.ecl.2008.08.005

[ii] Afra, P., Funke, M., & Matsuo, F. (2009). Acquired auditory-visual synesthesia: A window to early cross-modal sensory interactions. Psychology research and behavior management2, 31–37. https://doi.org/10.2147/prbm.s4481

[iii] Greve, M. W., & Zink, B. J. (2009). Pathophysiology of traumatic brain injury. Mount Sinai Journal of Medicine. A Journal of Translational and Personalized Medicine. A Journal of Translational and Personalized Medicine, 76(2), 97-104. https://doi.org/10.1002/msj.20104

[iv] Turner‐Stokes, L., Pick, A., Nair, A., Disler, P. B., & Wade, D. T. (2015). Multi‐disciplinary rehabilitation for acquired brain injury in adults of working age. Cochrane database of systematic reviews, (12). https://doi.org/10.1002/14651858.CD004170.pub3

[v] Degeneffe, C. E. (2001). Family caregiving and traumatic brain injury. Health & social work, 26(4), 257-268. https://doi.org/10.1093/hsw/26.4.257

[vi] Juengst, S. B., Kumar, R. G., & Wagner, A. K. (2017). A narrative literature review of depression following traumatic brain injury: Prevalence, impact, and management challenges. Psychology Research and Behavior Management, 10, Article 175-186. https://doi.org/10.2147/PRBM.S113264

One condition that can mean a person develops complex care needs is Duchenne Muscular Dystrophy (DMD).

What is Duchenne Muscular Dystrophy?

Muscular dystrophy is an umbrella term that encompasses a range of conditions, all of which have certain common features. Duchenne Muscular Dystrophy, or DMD is the most common form of muscular dystrophy. It mainly affects boys and men – around 1 in 4700 boys[i], and only about 1 in 50 million girls. It first appears in early childhood, and the average age at diagnosis of DMD is 4 years.

Symptoms of Duchenne

Duchenne is characterised by muscle weakness and wastage. As a genetic condition, the gene for Duchenne is always present, but the symptoms don’t fully appear until a child is around 2 years old.

Duchenne is one of the conditions that might be suspected if a boy doesn’t meet the usual physical milestones children reach by around age 2 or 3. There are some specific signs which children with Duchenne show – a particular gait, delay in walking and difficulty running or jumping, and using the ‘Gower Manoeuvre’[ii] to get up from the floor – this is where the large muscles in the hips and legs are weaker than usual so a child uses their arms to ‘walk’ themselves up from the floor.

As Duchenne progresses, more and more muscle groups are affected. There can be effects on the skeleton, with scoliosis (curvature of the spine) fairly common among boys with Duchenne. Duchenne eventually affects the muscles that control breathing, and more complex care input is needed. Duchenne can affect every muscle of the body, including the heart muscles.

Stages of Duchenne

Duchenne can be described in four different stages, though progression through these stages isn’t always the same for everyone.

Stage 1: Early Ambulatory. Duchenne first becomes obvious as children begin to move more, when they learn to walk and run. Children with Duchenne don’t meet the same physical developmental milestones as children without, and struggle with jumping and running at this stage. Some muscles will be affected more than others, and the calf muscles may appear enlarged. The  Duchenne can also affect speech development.

Children with Duchenne may or may not show some degree of learning difficulties, ranging from mild to severe.

Stage 2: Late Ambulatory. Children at this stage can still walk and move independently, but it becomes more difficult, they seem physically weaker than other children their age and become fatigued very easily. Balance and gait are affected, and children with this stage of Duchenne often seem to walk balancing on their tiptoes.

Stage 3: Early Non-Ambulatory. At this stage of DMD, children lose the ability to walk, and need to use a wheelchair for getting around. They may be able to use their arms to propel the wheelchair, but those muscles will also weaken over time. The muscles which control breathing become weaker and people at this stage may need to begin to use or consider some extra respiratory support. This includes chest physio with respiratory physiotherapists, non-invasive ventilation, and devices designed to assist with coughing and clearing the chest and airways.

This is the stage where it may become clear that the heart muscles are affected – this is called cardiomyopathy, and should be monitored by a cardiology team. Cardiomyopathy is diagnosed with an ‘echo’ test – an echocardiogram is an ultrasound scan of the heart which shows whether there are structural and functional changes in the heart.

Stage 4: Late Non-Ambulatory. At the later stages of Duchenne, lots of support will be needed for respiratory function, and mechanical ventilation might be necessary some or all of the time. Swallowing difficulties are also more profound at this stage, and a nutritional support and a feeding tube might be required. People with late stages of Duchenne are usually still able to speak, though this might be somewhat restricted by their breathing difficulties, and there’s not usually any impact on understanding and cognitive abilities – people with even very complex physical needs due to Duchenne can still have an active role in society, relationships, social and work lives. At this stage, people with DMD usually need full support with all their care needs, and require help with continence needs, eating and drinking, and positioning in a bed or chair.

What Causes Duchenne Muscular Dystrophy?

Duchenne muscular dystrophy is caused by a genetic mutation[iii]. Genetic conditions are complicated; what we do know is that the gene for Duchenne is on the X chromosome. It is the gene that enables us to create the protein that protects our muscles from damage. As girls have two X chromosomes – one from each parent – a girl with the Duchenne gene will normally have one X chromosome with the mutation and one without, which means that girls are still able to produce the muscle-protecting protein and very rarely develop DMD.

Boys with the mutation on the X chromosome only have one X chromosome – from their mother – and a Y chromosome – from their father. If a boy has the genetic mutation for DMD on their X chromosome, they will develop DMD. Although full-blown DMD is rare in girls, around 8% of female carriers have some symptoms, and may particularly have cardiac problems[iv].

Dystrophin[v] is the protein our bodies produce to protect our muscles from damage – without enough dystrophin, even simple movements begin to cause the muscles to weaken.

The gene mutation which causes DMD sometimes happens spontaneously; it can happen even when there’s no one in the family with the same mutation. More commonly, it is passed down in families, and as it is on the X chromosome, a boy with DMD will have inherited it from his mother’s side. People with DMD in their family can access genetic counselling – information and tests to determine the likelihood of passing DMD on to their descendants, and what their options are.

Diagnosing Duchenne

To diagnose Duchenne, doctors ask about the signs and symptoms and family history of the child with suspected Duchenne. Blood tests are also taken which look for evidence of specific muscle damage. If the findings are in line with Duchenne or a similar condition, genetic testing – again by taking a blood sample – can confirm the diagnosis. Sometimes, doctors might take a muscle biopsy – this is a small sample of the muscle tissue – for examination. This is done under a local anaesthetic (numbing the area where the biopsy will be taken) and can help work out the amount of dystrophin in the muscle.

Some different muscle conditions can have similar symptoms to Duchenne so it’s important to have a proper diagnosis so that the right treatment can be given. With a diagnosis of Duchenne, the medical team and family can begin to plan the future management of the condition.

Treatments for Duchenne Muscular Dystrophy

Duchenne cannot be cured – there is currently no way to change a person’s genetic code if they have the genetic mutation for DMD. There is, however, ongoing research into treatment for Duchenne. Researchers are looking at ways to repair or replace genetic mutations[vi], and ways to enable the body to use different proteins genes to protect the muscles in the same way as dystrophin[vii].

There are lots of different forms of treatment and management of Duchenne muscular dystrophy[viii], and will involve strategies such as:

Physiotherapy: physiotherapists can help people with Duchenne learn the best ways to manage their movements and mobility. Good physiotherapy in muscular dystrophy can help people preserve their physical function and range of movement for as long as possible[ix]. Physiotherapists can also fit orthotic devices like braces to help support muscles and bones.

Occupational Therapy: Occupational therapists can help with adaptations to support home living, like grab rails, accessible bathroom equipment, and aids for independent living.

Gentle exercise: very gentle, low impact exercise like swimming can help people with DMD stay mobile as long as possible.

Medications: There are a few different medication options in DMD, and evidence for different treatments is improving all the time[x]. Corticosteroids in particular can help preserve muscle strength for a limited amount of time.

A healthy diet: Following a healthy diet[xi] is important for helping maintain the best possible general health.

healthy food concept

Some of the medications used for muscular dystrophy mean it’s extra important to focus on a nutritious diet; corticosteroids can make people gain weight and can also impair the absorption of calcium and affect bone structure. In later stages, swallowing can be a problem and careful management is needed to prevent malnutrition and dehydration[xii] – this might include fitting a feeding tube through the wall of the abdomen into the stomach (Percutaneous Endoscopic Gastrostomy or PEG).

Speech and language therapy (‘SALT’): speech and language therapists can help overcome the communication difficulties that can present with DMD especially as the muscles controlling breathing weaken and speech becomes difficult. Becoming dependent on mechanical ventilation can cause particular communication challenges, which speech therapists can help manage. A SALT (sometimes ‘SLT’) team are also the healthcare specialists who support people with swallowing difficulties, recommending management strategies like pureed diets and thickened fluids.

Cardiology input: DMD can affect heart function, causing reduced tone of the heart muscle and heart rhythm disturbances. Input from a cardiology team can help manage reduced heart function and arrhythmia risk. Permanent pacemakers are sometimes needed to manage heart rhythm problems.

Respiratory input: As the muscles which control breathing get weaker in the later stages of Duchenne, respiratory support[xiii] becomes more important, and more and more input is needed as time goes by.

Reduced ability to cough and clear the lungs makes a person more prone to chest infections and pneumonia, particularly when swallowing becomes compromised. Physiotherapists can help with coughing, and cough assist devices are available to help clear the lungs of fluid and mucous. As Duchenne progresses, alternatives to independent breathing may be necessary; non-invasive mechanical ventilation, often using a tracheostomy may become essential.

Home care: Some of the care needs a person with Duchenne will develop can most easily be managed by dedicated professional carers. Becoming less mobile makes a person more at risk of developing pressure damage, of having trouble with continence and constipation, and simply need more support to stay as active as possible and live the fullest life they can with the restrictions they’re developing.

Carers and nursing teams who specialise in complex care needs can also provide essential respiratory and nutritional support such as tracheostomy care, mechanical ventilation management, and PEG feeding. Families and informal carers can be as involved as they wish or can leave complex care to professionals and maintain a more traditional family role.

Living with Duchenne Muscular Dystrophy

Duchenne muscular dystrophy is a severely life-limiting condition. People with Duchenne usually only live into their 20s or 30s. Although life expectancy is shortened, there are ways to continue to have quality of life, and the right kind of support can enable people with Duchenne and their families to live the fullest possible life.

In the earlier stages, a family may be able to manage Duchenne at home independently with medical and nursing input for specific problems as they arise. As the child gets older, they move through the stages of Duchenne and are more affected by the muscle wastage. Their care needs will increase, and they will grow into adolescence and adulthood, making it more difficult to manage their physical needs without formal care input.

In the later stages of DMD, the affected person may begin to experience trouble breathing independently. At this point, they will need complex respiratory input like ventilators or devices which help them clear their lungs when the muscles that control coughing are affected.

Swallowing can also become a problem, making people with DMD at risk of aspiration pneumonia (a condition where food or drink enters the lungs), and there are some options for staying well fed and hydrated without the ability to swallow safely. Staying at home at that stage means having support with all their personal care needs and more complex support for nutrition and respiratory needs.

Resources

Living with Duchenne Muscular Dystrophy means making some major adjustments, and Duchenne affects more than just the individual with the condition.  If you or a family member has Duchenne, there are organisations and groups which can offer life-changing support.

UK based groups supporting people with Duchenne and other sorts of muscular dystrophy:

https://www.duchenneuk.org/

https://www.myotonicdystrophysupportgroup.org/

https://www.actionduchenne.org/

https://www.musculardystrophyuk.org/

https://www.musclehelp.com/

Like to know more?

 

[i] Dooley, J., Gordon, K. E., Dodds, L., & MacSween, J. (2010). Duchenne Muscular Dystrophy: A 30-Year Population-Based Incidence Study. Clinical Pediatrics49(2), 177–179. https://doi.org/10.1177/0009922809347777

[ii] Shrestha S, Munakomi S. Gower Sign. (2020). StatPearls Publishing https://www.ncbi.nlm.nih.gov/books/NBK540973/

[iii] Emery, A. E., Muntoni, F., & Quinlivan, R. C. (2015). Duchenne muscular dystrophy. OUP Oxford. https://doi.org/10.1016/j.ejpn.2004.05.004

[iv] Song, T. J., Lee, K. A., Kang, S. W., Cho, H., & Choi, Y. C. (2011). Three cases of manifesting female carriers in patients with Duchenne muscular dystrophy. Yonsei medical journal52(1), 192. http://doi.org/10.3349/ymj.2011.52.1.192

[v]Hoffman, E. P., Brown Jr, R. H., & Kunkel, L. M. (1987). Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell, 51(6), 919-928.  https://doi.org/10.1016/0092-8674(87)90579-4

[vi] Guiraud, S., Chen, H., Burns, D. T., & Davies, K. E. (2015). Advances in genetic therapeutic strategies for Duchenne muscular dystrophy. Experimental physiology100(12), 1458-1467. https://doi.org/10.1113/EP085308

[vii] Gillis, J. M. (2004). Utrophin, a way to cure Duchenne muscle dystrophy. Medecine Sciences:https://doi.org/10.1051/medsci/2004204442

[viii]Bushby, K., Finkel, R., Birnkrant, D. J., Case, L. E., Clemens, P. R., Cripe, L., … & DMD Care Considerations Working Group. (2010). Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. The Lancet Neurology, 9(1), 77-93. https://doi.org/10.1016/S1474-4422(09)70271-6

[ix] Gianola, S., Pecoraro, V., Lambiase, S., Gatti, R., Banfi, G., & Moja, L. (2013). Efficacy of muscle exercise in patients with muscular dystrophy: a systematic review showing a missed opportunity to improve outcomes. PloS one8(6), e65414. https://doi.org/10.1371/journal.pone.0065414

[x] Strober, J.B. Therapeutics in Duchenne muscular dystrophy. NeuroRX 3, 225–234 (2006). https://doi.org/10.1016/j.nurx.2006.01.005

[xi]Davidson, Z. E., & Truby, H. (2009). A review of nutrition in Duchenne muscular dystrophy. Journal of human nutrition and dietetics, 22(5), 383-393. https://doi.org/10.1111/j.1365-277x.2009.00979.x

[xii]Michel Toussaint, Zoe Davidson, Veronique Bouvoie, Nathalie Evenepoel, Jurn Haan & Philippe Soudon (2016) Dysphagia in Duchenne muscular dystrophy: practical recommendations to guide management, Disability and Rehabilitation, 38:20, 2052-2062 https://doi.org/10.3109/09638288.2015.1111434

[xiii] Sheehan, D. W., Birnkrant, D. J., Benditt, J. O., Eagle, M., Finder, J. D., Kissel, J., … & Wolfe, L. F. (2018). Respiratory management of the patient with Duchenne muscular dystrophy. Pediatrics142(Supplement 2), S62-S71.  https://doi.org/10.1542/peds.2018-0333H

 

 

 

 

Huntington’s Disease, sometimes called Huntington’s Chorea or just Huntington’s, is a genetic disorder which affects the brain. The name ‘Huntington’s Chorea’ describes the kind of involuntary fidgety movements people with Huntington’s disease experience. Huntington’s disease is a neurodegenerative disease, which means that it causes gradual degradation to the nerve processes of the brain. Huntington’s causes progressive deterioration and is severely life-limiting.

What causes Huntington’s Disease?

Huntington’s disease is caused by a defective gene in a person’s DNA. Someone with the gene for Huntington’s will at some point develop Huntington’s disease. It is almost unknown in people who have no family history of Huntington’s disease, and where people do develop it without having a parent who was known to have had it, that is nearly always thought to be simply because that parent died of another cause before developing it.

Huntington’s is an inherited genetic disorder. If one parent has Huntington’s disease, each of their children has a 50% chance of inheriting the gene. Each child with the gene has a 50% chance of passing it on to each of their own children, and so on. People who do not have the gene for Huntington’s disease cannot pass the disease on to their children, even if they have other close family members with the disease. Huntington’s disease is much more common in people of white European descent than any other population in the world. It can affect men and women equally.

People with the Huntington’s gene defect usually begin to show symptoms between the ages of 30 and 50, although sometimes onset can be much later in life. Occasionally Huntington’s symptoms begin earlier in life. If it develops in someone aged 20 or under it is referred to as juvenile Huntington’s disease.[i]

Symptoms of Huntington’s Disease

Once Huntington’s Disease has been diagnosed and begins to show symptoms, the symptoms get progressively worse over time. In the early stages, symptoms include:

  • Mood changes, particularly depression and difficulty in finding joy or interest in things.
  • Memory problems which are significantly worse than normal forgetfulness.
  • Altered gait, seeming clumsy or bumping into things when walking.
  • Reduced ability to focus or concentrate.

Huntington’s ‘chorea’ develops in the early to intermediate stages of the disease; this is the characteristic involuntary movement of the head, arms, legs, and torso.

Depression is a very common symptom of Huntington’s; this is partly due to the physical changes in the brain, and often partly due to the impact of the disease on a person’s life and lifestyle as well as the symptoms and anticipation of a degenerative disease.

As the disease progresses, it affects more and more of a person’s cognitive function and control over almost all physical functions. Compromised swallow can make people with Huntington’s disease prone to malnutrition, dehydration, and aspiration pneumonia, which contribute to the most common causes of death for people with Huntington’s Disease.

The Five Stages of Huntington’s Disease

Huntington’s disease is often described by ‘stages’[ii], which follow a fairly predictable pattern of progression. The length of time it takes to progress through the stages after diagnosis can vary from person to person, but life expectancy after diagnosis is rarely more than 20 years. The stages we describe start from the time of the first symptoms, though the gene is present from the earliest stages of foetal development, and the changes in the brain which cause the disease begin long before symptoms appear.

Stage 1: Early Stage

We refer to the first symptoms of Huntington’s as ‘stage 1’. Symptoms may be mild but with a family history or positive genetic test for Huntington’s disease, anyone beginning to have symptoms of memory loss, confusion or altered gait should have a low threshold for seeking medical advice.

Some early symptoms which could be due to Huntington’s disease might also be symptoms of other conditions, so it’s important to see a doctor for an accurate diagnosis and appropriate treatment.

Stage 2: Early Intermediate Stage

Problems with memory, cognitive ability, mood and behaviour mean that some support may begin to be required for normal activities. Sometimes a little prompting for memory problems or having general support around can be helpful, and some assistance with mobility and movement might be needed. Depending on the nature of the job, some people are able to continue to work in the early intermediate stage, if adjustments can be made to support them.

Stage 3: Late Intermediate Stage

Changes in mobility and involuntary movements usually become more pronounced in the middle stage of Huntington’s. Sometimes this can begin to affect swallowing ability, and the combination of lots of uncontrollable movements burning calories and worsening swallowing difficulties can cause significant weight loss. A speech and language therapist can help find the right texture and techniques for managing food and drink, and a dietitian can give recommendations on the kind of high-calorie foods that might be best from this stage on.

 

Stage 4: Early Advanced Stage

Moving into the advanced stages of Huntington’s disease usually means becoming more reliant on others for more and more of the things that would normally be done independently. Washing, dressing, and other self-care activities may need some support, and deficits in functional ability will become more pronounced. People entering the advanced stages of Huntington’s will need help with household tasks such as shopping, cooking, cleaning, and managing finances.

As the early advanced stage of Huntington’s progresses, it’s important to think about the future, and to anticipate the kind of increasing care needs that will be required as time goes on. There may be a time when having care from family members or drop-in carers is no longer enough, and decisions have to be made about having full-time care.

Stage 5: Advanced Stage

In the most advanced stage of Huntington’s disease, people usually have almost no control over their movements and body functions and have significant cognitive impairment. This means they may be unable to effectively communicate at all; they may have serious problems swallowing and be prone to conditions associated with immobility, malnutrition, and dehydration. The last stages of Huntington’s disease can last months or years, during which time the person with the disease will need full time nursing care. Sensitive care and good management of symptoms to maintain comfort and dignity is the main aim of any interventions at this stage.

A person with advanced Huntington’s will be expected to reach the end of their lives from simple disease progression or complications. Pneumonia is the most common cause of death for people with Huntington’s disease.[iii]

Treatment for Huntington’s Disease

There is currently no cure for Huntington’s disease, and no way to stop the progression of the disease. Many of the symptoms of Huntington’s can be managed and the aim of treatment for people with Huntington’s disease it to preserve quality of life for as long as possible.

Medication

People with Huntington’s disease may take antidepressants and mood stabilisers for some of the mood and behavioural changes that can accompany the disease. Medications are also available which can help reduce the involuntary movements that people with Huntington’s develop. Other health problems that can arise with or without Huntington’s disease also need to be managed alongside the condition.

Physiotherapy

Huntington’s causes movement disorders, and physiotherapists can recommend exercises, techniques, and mobility aids to support people with Huntington’s disease to stay as independent as possible for as long as possible. Physiotherapists also help to manage chest conditions in later stages of the disease when it becomes difficult to control swallowing, coughing, and deep breathing.

Occupational therapy

Occupational therapists help people find adaptations to continue living independently or to find the right support for their progressing disease. Examples of aids provided by occupational therapists could be grab rails around the house for mobility support in early stages, or in the later stages of the disease, a pressure-relieving and position-changing hospital bed.

Lifestyle

Although the condition is always progressive and degenerative, there is some evidence that staying otherwise fit and active helps symptoms stay milder for longer.[iv]

Nutrition

A healthy diet is always recommended for prevention of many serious conditions. With Huntington’s disease in particular, rapid and uncontrollable weight loss is very common due to the near-constant involuntary movements. People with Huntington’s can burn up a lot more calories in a day that the average person, so a high calorie diet is essential[v]. In the later stages swallowing can be affected, and a speech and language therapist can recommend different textures and types of food and drink to reduce the risk of aspiration pneumonia, malnutrition, and dehydration.

Genetic Counselling for Huntington’s Disease

We know that Huntington’s Disease is passed on from parents to their children, and there is a 50% chance of inheriting the disease if a person has one parent with Huntington’s. There are now tests which can show whether a person has inherited the gene for Huntington’s Disease, and most people in the UK with a close family member who develops Huntington’s Disease will be offered this test.[vi] Having a test which shows the likelihood of developing a serious, life-limiting disease is naturally a highly emotive and possibly life-changing process, and clinical counselling should be offered alongside diagnostic tests.[vii]

Not everyone at risk of Huntington’s Disease will decide that they want to have a test, but some people find that it helps them to plan for their future life. It’s also possible to choose to have the test at a later date, perhaps when considering a pregnancy or if they become worried about symptoms.

People who have the gene for Huntington’s disease and who are planning a family have several options to ensure that the gene isn’t passed on

Living with Huntington’s Disease

After a diagnosis of Huntington’s disease, the prognosis is fairly clear; from the onset of symptoms there is normally a life expectancy of 10 to 20 years. Some people progress through the stages faster than others, but anyone with Huntington’s disease should expect to need full time care at some stage of their disease.

Choosing an appropriate care setting is important, and many people try to stay at home for as long as possible. For most, this is only possible with round-the-clock carers, and full time care packages are available for people with extensive care needs at home. Other care settings may be considered, and if considering care homes it’s important to understand the level of care they can provide; care homes offering residential care offer significantly different services and have a much lower ratio of staff to residents than a care home providing full nursing care.

To stay at home in the late stages of Huntington’s disease, significant adaptations need to be made to enable good nursing care to prevent complications such as pressure damage from immobility or aspiration pneumonia from compromised swallow.

After a diagnosis of Huntington’s, it is reasonable to expect that there will come a time when that person isn’t able to express their preferences or where they may be unable to make informed decisions about their care and medical treatment. Choosing someone who can act as advocate can be useful, as can an advanced statement of choices about treatment, or a living will. The sorts of treatment that will be thought appropriate might change over the course of the disease, so any advanced decisions can be flexible and updated.

Having good care at the end stages of Huntington’s disease can make a world of difference both to the person affected and those close friends and family members around them. Full time care can enable relationships to stay as normal and stable as possible, and mean support through the hardest times. Full-time care is essential to reduce the impact of serious complications of the illness, and to support everyone involved.

http://Like to know more?

[i] Quarrell, O., O’Donovan, K. L., Bandmann, O., & Strong, M. (2012). The Prevalence of Juvenile Huntington’s Disease: A Review of the Literature and Meta-Analysis. PLoS currents  https://doi.org/10.1371/4f8606b742ef3

 

[ii] Kirkwood, S. C., Su, J. L., Conneally, P. M., & Foroud, T. (2001). Progression of symptoms in the early and middle stages of Huntington disease. Archives of neurologyhttps://jamanetwork.com/journals/jamaneurology/article-abstract/778574

 

[iii] Heemskerk A, Roos RAC. (2010) Causes of death in Huntington’s disease

Journal of Neurology, Neurosurgery & Psychiatry http://dx.doi.org/10.1136/jnnp.2010.22638.4

 

[iv] Garcia-Gorro, C., Garau-Rolandi, M., Escrichs, A., Rodriguez-Dechicha, N., Vaquer, I., Subira, S., … & Muñoz, E. (2019). An active cognitive lifestyle as a potential neuroprotective factor in Huntington’s disease. Neuropsychologia122, 116-124. http://brainvitge.org/website/wp-content/uploads/2019/03/Garcia-Gorro_Neuropsychologia_2019.pdf

 

[v] Marder, K., Zhao, H., Eberly, S., Tanner, C. M., Oakes, D., Shoulson, I., & Huntington Study Group (2009). Dietary intake in adults at risk for Huntington disease: analysis of PHAROS research participants. Neurologyhttps://doi.org/10.1212/WNL.0b013e3181b04aa2

 

[vi] Huntington’s Disease Association (2020) Huntington’s Disease: A Genetic Testing Guide.  https://www.hda.org.uk/media/2938/a-genetic-testing-guide-resource-pages.pdf

[vii] Myers, R.H. Huntington’s disease genetics. Neurotherapeutics 1, 255–262 (2004). https://doi.org/10.1602/neurorx.1.2.255

Autonomic dysreflexia[i] (AD) is also known as autonomic hyperreflexia. It describes a situation where the body exhibits an exaggerated reflex response to a problem or stimulus. A person with a high spinal cord injury is likely to be unable to consciously sense and respond to a damaging or irritating stimulus.

The body’s unconscious systems – the autonomic nervous system – respond to the problem, leading to rising blood pressure and other physical responses that can result in rapid deterioration, serious illness, seizures, or stroke. AD occurs in people with high spinal cord injuries – typically at T6 or higher – when the autonomic nervous system cascades this exaggerated and uncontrolled response to a stimulus.

If someone is suspected to have autonomic dysreflexia, this should be treated as a medical emergency, and finding the cause and treating the symptoms should be an immediate priority.

Why does AD happen?

People with spinal cord injuries at T6 or higher are most at risk of developing AD, the higher the injury, the higher the risk of AD.

People with high spinal cord injuries can sometimes develop serious physical problems without becoming directly aware of them or able to locate the site of the issue. The autonomic nervous system[iii] is the system which controls the unconscious activities of our body, things like heart rate, digestive processes and response to illness or irritants.

The autonomic nervous system has two branches which are involved in the development of AD: the sympathetic and parasympathetic nervous systems. They complement and oppose each other to balance the body’s need to respond to stimuli while remaining stable and able to relax appropriately.

The sympathetic nervous system is sometimes referred to as the ‘fight or flight’ system; it responds quickly to stimuli and helps us immediately in situations or, for example, danger or pain. The parasympathetic nervous system – the ‘rest and digest’ system – allows us to temper that response and maintain appropriate calm, enabling rest and normal function.

In people with spinal cord injuries, the balance between these two systems can be impaired and this is the cause of some of the symptoms and syndromes people with spinal cord injuries experience. Nervous system responses to stimuli below the site of injury are not relayed or responded to by the higher nervous system – i.e. the brain – and so can quickly become unbalanced. Add this to a person who can develop physical problems below the site of their injury which they cannot consciously sense or respond to, and we start to see seriously imbalanced autonomic responses – ‘autonomic dysreflexia’.

Autonomic dysreflexia causes uncontrolled hypertension – a dangerously high blood pressure – and can lead to seizures, strokes, and cardiac arrests.

Image courtesy of National Rehabilitation Hospital, Ireland

Causes of autonomic dysreflexia

AD occurs when the body reacts to a problem or stimulus and is not able to temper that reaction by appropriate balance within the autonomic nervous system. It occurs in people with high spinal cord injuries because they are not usually able to consciously identify or locate these issues, and because their autonomic nervous system can be disordered and disrupted by its own response to stimuli.

People with high spinal cord injuries are at direct risk of AD because of their neurological injury, and are also, indirectly, at higher risk of AD because of the results of their neurological injury – a higher likelihood of bowel or bladder dysfunction, for example.

Common triggers for AD[iv] include:

  • Bowel problems – commonly a distended bowel, pressure on the rectum from hard-to-pass stool, or more severe faecal impaction.
  • Bladder dysfunction – such as urinary retention, a blocked catheter, or a urinary tract infection.
  • Injury – which could be from relatively common problems like pressure damage or moisture lesions. Physical trauma such as a fall could also trigger AD, even with no signs of visible injury.
  • Tight clothing or straps – Sometimes loosening a restrictive waistband is enough to remove that irritating stimulus that triggers AD.

AD can also develop as a result of the stimulation of sexual intercourse and can be due to menstruation. It is common in people with high spinal cord injuries during pregnancy and childbirth. Pregnant women with high spinal cord injuries[v] and the people close to them should be extra vigilant over AD symptoms and early signs of labour.

The single biggest risk factor for autonomic dysreflexia is a high spinal cord injury, particularly at T6 or higher.

Some other neurological conditions can make people prone to AD, such as multiple sclerosis or some forms of brain injury.

The best way to reduce a person’s likelihood of developing AD is to identify and monitor their personal risk factors. Good nursing care with attention to bowel, bladder and skin care can eliminate some of the most common risks for AD. Having individually devised care plans identifying specific risk mean that everyone involved can identify risks, triggers, and early signs of AD.

Autonomic Dysreflexia symptoms

Often, the first symptom that a person with autonomic dysreflexia will report is a severe headache or describe a non-specific feeling of illness. As this can be an early warning sign of AD, it should be taken seriously and investigated further. Other signs to look for in AD include a high blood pressure, accompanied by a low heart rate.

People with T6 or higher spinal cord injuries often have lower than average baseline blood pressure. Blood pressure that would not be out of range for other people could be significantly out of range for someone at risk of AD. It is important, therefore, to know what someone’s baseline blood pressure is to help identify a blood pressure that is abnormal for an individual. A systolic blood pressure – the first, higher number in a blood pressure reading – which is 20 – 40 mmHg or more higher than usual is a serious concern.

As people with a spinal cord injury typically have no neurological damage above the site of the injury, there can be a sharp demarcation between the appearance of the body above and below the site of injury. More usual signs of illness, such as heat and flushing to the skin, may occur above the site of injury. Conversely, below the site of injury, the skin may become cold and clammy, with goosebumps and possibly mottled skin.

Sometimes the ‘soft signs’ of illness are enough to identify a problem; feeling anxious or lethargic, confused or just generally unwell. The value of having family members or carers present who get to know the individual they are helping look after cannot be understated; knowing a person well means being able to spot when something isn’t right.

Preventing autonomic dysreflexia

The most common causes of AD can usually be prevented by good nursing care. Creating individualised plans of care can help identify risks and alert care teams and close friends and family members to early signs of problems.

Knowing the most likely risk factors for AD for an individual can help everyone to anticipate and avoid problems. Difficulties with the bladder and bowels are the most common causes of AD. This means tackling areas of risk such as:

  • Bladder emptying – People with spinal cord injuries may have a urinary catheter or may rely on other methods of continence care. Noticing when someone’s urine output tails off, if it’s been a long time since they passed urine, or if they have an unusually distended lower abdomen are all signs that formal or informal carers can look for to identify issues before they become serious problems. Catheters can become blocked and may need to be irrigated. Catheter and bladder care should be part of a training programme for anyone who helps care for someone with a high spinal cord injury.
  • Bowel emptying – People with high spinal injuries need careful bowel management; this may include a combination of laxatives and stool softeners, physical bowel management through manual evacuation of stool and good continence care. Monitoring the frequency of bowel movements can help to avoid serious problems such as faecal impaction, painful haemorrhoids, and bowel distension. Bowel problems are such a common cause of AD that NHS England released a patient safety alert[vi] in 2018 to promote understanding of the importance of bowel care in people at risk of autonomic dysreflexia.
  • Skin and pressure area care – People who are unable to make positional changes themselves, and who would be unable to feel developing damage to the skin or tissues need extra care to avoid pressure damage. Pressure damage – ‘bed sores’ – can occur anywhere where pressure acts upon soft tissues – commonly on the buttocks, sacrum, hips or even the bony prominences of the spine. Damage can also occur from tight straps from airway management devices or seatbelts, or even from the action of a catheter rubbing inside the urethra. Poor continence management also makes skin damage more likely. Damaged skin and underlying tissues, infection and fluid loss through wounds can all cause serious illness and trigger AD.
  • Preventing or treating infection – urinary tract infections (UTIs) are common and, again, good catheter care can go a long way towards helping prevent them. Being able to identify and treat a UTI in the early stages can prevent more serious problems, and carers can look out for signs such as an altered odour or colour of the urine. Wound care and management of any other indwelling devices is also an essential part of the caring role.

Treatment for autonomic dysreflexia

AD is a medical emergency. It’s important to identify and treat the cause, and that might actually be quite simple – irrigation of a blocked catheter or removal of some hard stool from the rectum may be enough to completely eradicate the irritating stimulus that has triggered the AD. Even if the cause can be easily identified and treated at home, however, the person experiencing AD symptoms still needs emergency medical care. Once the autonomic nervous system has been triggered, the symptoms go beyond the cause and may not resolve without treatment. People with AD need close observation, including ongoing blood pressure and heart rate monitoring.

Treatment for AD[vii], aside from identifying and removing the trigger, is to manage blood pressure. This may be through such simple methods as positional changes – going from a lying to sitting position can be enough to drop the blood pressure and improve the outlook. It may be necessary to use medications to bring the blood pressure down, and management of a very slow heart rate can include changes in medications. Sometimes a doctor may recommend a cardiac pacemaker[viii] if a profoundly slow heart rate is causing further problems.

Sometimes it is harder to identify the initial trigger, and in some people a trigger can be as simple, but easily overlooked, as restrictive clothes. At times, the trigger is less simple, such as the physical changes in pregnancy, and will require careful management and the input of specialists.

Living with the risk of autonomic dysreflexia

Monitoring for changes and managing some of the features of AD can be life-saving, and everyone who takes part in the care of someone with a high spinal injury should be able to identify risks and early signs of AD. Informal carers and the members of a formal care package can all help to prevent or manage this serious condition. Early identification and prompt treatment of the causes and features of autonomic dysreflexia saves lives.

There are now lots of resources online to help people learn more about autonomic dysreflexia, along with support groups and specialist services for people with spinal cord injury. These can be a helpful place to start. The best way to avoid and manage serious complications after a high spinal cord injury is through nursing and care teams with specialist training in the care of people with spinal cord injuries.

Having a full time care team who are dedicated to one individual client means that everyone involved is able to identify anything out of the ordinary and treat problems early. A care provider which enables their client to have a dedicated, full time team can provide a package of training tailored to the needs of that individual, meaning the most specialised possible care. Continuity of care and the chance to develop strong, long-term relationships are key in the prevention of serious problems, as well as overall quality of life for everyone involved.

Like to know more?

[i]Royal National Orthopaedic hospital. Autonomic Dysreflexia. rnoh.nhs.uk https://www.rnoh.nhs.uk/services/spinal-cord-injury-centre/medical-management-advice/autonomic-dysreflexia

[iii]Sharif, H., & Hou, S. (2017). Autonomic dysreflexia: a cardiovascular disorder following spinal cord injury. Neural Regeneration Research12(9), 1390. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5649450/

[iv] Allen, K. J., & Leslie, S. W. (2019). Autonomic dysreflexia. In StatPearls [Internet]. StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK482434/

[v] Soh, S. H., Lee, G., & Joo, M. C. (2019). Autonomic dysreflexia during pregnancy in a woman with spinal cord injury: a case report. Journal of International Medical Research47(7), 3394-3399. https://dx.doi.org/10.1177%2F0300060519853659

[vi]NHS Improvement (2018) Resources to Support Safer Bowel Care for Patients at Risk of Autonomic Dysreflexia. NHS Improvement Patient Safety Alert.  https://www.england.nhs.uk/wp-content/uploads/2019/12/Patient_Safety_Alert_-_safer_care_for_patients_at_risk_of_AD.pdf

[vii] Gall, A., Turner-Stokes, L., & Guideline Development Group. (2008). Chronic spinal cord injury: management of patients in acute hospital settings. Clinical medicine8(1), 70. https://www.ncbi.nlm.nih.gov/pubmed/18335674

[viii]Oh, Young-Min, and Jong-Pil Eun. “Cardiovascular dysfunction due to sympathetic hypoactivity after complete cervical spinal cord injury: a case report and literature review.” Medicine vol. 94,12 (2015): e686. https://dx.doi.org/10.1097%2FMD.0000000000000686

 

 

 

 

 

Anterior cord syndrome is a condition affecting the spinal cord. ‘Anterior’ refers to the part of the cord affected; in this case, the aspect of the cord facing the front of the body. It is an ischaemic condition, meaning it is caused by lack of blood flow ‘feeding’ oxygen and nutrients to the spinal cord, resulting in injury to some of the tissues.

The anterior spinal cord is perfused by – receives its blood supply from – the anterior spinal artery; as an injury to the central nervous system caused by blood supply problems, it is most usefully compared to a stroke. It differs from what most people think of as a typical stroke, as it occurs further along the bundle of nerves comprising the spinal cord so doesn’t directly affect the brain.

The outlook for someone with an incomplete spinal cord injury like anterior cord syndrome is much better than that for someone who has had a cerebral stroke. The functional effects of an injury affecting the spinal cord are usually only found below the level of injury, though a spinal cord injury can impact on the whole body through effects on blood pressure and the autonomic nervous system – the system which controls the body functions that we don’t have any conscious control over.

Anterior cord syndrome is rare with around 40 cases per million people, according to one study in the United States. It can vary in severity from being extremely debilitating in the long term to being associated with only very mild or insignificant long-term effects.

Causes

To understand the causes, it’s essential to have some understanding of the anatomy of the spinal cord. Looking at a cross-section of the spinal cord, different areas of the cord relate to different regions of the body. They are also supplied by different blood vessels, so an occlusion – a blockage or narrowing – in one of the arteries supplying blood to the spinal cord might only impact on one of these areas. The ‘anterior’ cord is the way we describe the two-thirds of the cord facing the front of the body.

The blood supply to the anterior spinal cord is provided by the anterior spinal artery, and problems can occur for a few different reasons. Generally, vascular – blood supply – injuries to the anterior spinal cord can be caused by traumatic events or atraumatic (non-traumatic) events.

Traumatic events include injuries sustained in car crashes or other serious accidents; any injury which causes disruption to the blood supply to the anterior spinal cord, usually due to compression or rupture of those blood vessels.

Non-traumatic events include spinal artery thrombosis – a clot in the blood supply to the spinal cord, or interrupted blood supply due to a bleed such as in the case of a ruptured aortic aneurysm.

Interrupted blood supply causes an area of ischaemia – tissues that have been damaged by a lack of oxygen due to restricted blood supply. This area of damage means that some of the signals that would normally be passed through the nerves at that point cannot be transmitted properly.

This means that the information being passed to and from the brain to any part of the body supplied by those affected nerves is disrupted. This disruption can affect movement and motor control, sensation of touch, pain, and changes in temperature, and sometimes affects the action of the muscles controlling the bladder, bowels, and reproductive organs.

Diagram of cross section of spine showing anterior cord syndrome

The characteristic ischaemic areas in anterior cord syndrome

Damage to different parts of the bundles of nerves in this imaginary cross section of the spinal cord affects different functions and areas. The position along the length of the spine where damage occurs also has an effect on the result of the injury. Generally, movement and sensation are only affected below the point on the spine where the damage occurs.

It is not always possible to tell straight away how much someone will recover their abilities; some people completely recover their ability to walk, whereas others do not regain good muscle control. Early tests, treatment and rehabilitation can help predict the path of recovery.

Risk Factors

There are several different conditions which can lead to anterior cord syndrome, and the risk factors for these vary. Some conditions leading to vascular spinal cord injuries are the same kinds that cause any other problems with blood supply around the body – such as hardening of the arteries (arteriosclerosis or atherosclerosis), thrombosis (blood clots).

Adequate blood supply can also be interrupted by aortic aneurysm – related to heart disease, hardened arteries, and uncontrolled hypertension (high blood pressure), or by very severe hypotension (low blood pressure), such as that associated with significant blood loss.

Anterior cord syndrome can be caused by trauma, such as during a violent injury, or is listed as one of the very rare complications associated with certain spinal cord surgeries. Other conditions which can cause damage and insufficiency of the anterior spinal cord include tumours causing local pressure, or extreme cases of cervical spondylosis.

Signs and Symptoms

Anterior cord syndrome is an acute onset condition, meaning that it happens very quickly and suddenly. It may occur with no warning at all, and even at rest – people report sitting down and suddenly losing all sensation or movement in their legs. Both sides of the body are affected.

The symptoms include sudden loss of muscle strength and movement control. Some sensitivity to pain or heat is also lost, but people with anterior spinal cord syndrome, normal touch perception is usually retained. Proprioception – the sensation which lets us understand intuitively our body’s position, placement and movement – is usually unaffected by anterior cord injury. Some people lose tone and sensation to their bowels and bladder control.

The symptoms of anterior cord syndrome are enough for doctors to make a working assumption of the condition in an emergency setting, but an early MRI scan will give more indication of the exact location and severity of the condition, and will guide the treatment for the best possible prognosis.

Management

The acute phase of any spinal cord injury needs to be managed with immediate hospital attention, and from there the path to recovery is variable from person to person. The extent and location of the injury have a huge bearing on the amount of sensation and movement control which will be regained, and early, effective physiotherapy can also make a difference.

The main treatment in the initial phase of anterior cord injury is to treat the underlying cause. This may take the form of medications such as steroids and blood thinners, and some conditions causing spinal cord damage will need surgical treatment.

Someone with an anterior spinal cord syndrome is likely to have to take medications for life. These will depend on the initial cause of the problems, but may include blood thinners and treatments to manage disease affecting the blood vessels, such as treatments for cholesterol or high blood pressure.

Most people with spinal cord injuries will require a period of rehabilitation to help recover as much of their functional ability as possible in a safe, supportive environment. Long or short stays in spinal injury centres or on specialist rehab wards may be beneficial, but some people are able to live at home with provisions to meet their care needs, and can attend rehab centres through the day, or have home physiotherapy in some circumstances. The extent and success of rehabilitation prior to discharge from hospital is a strong indicator of the ongoing care needs for that person.

Ongoing needs

The severity of anterior cord syndrome can vary widely, and so any ongoing care needs must be assessed on an individual, case-by-case basis. Physiotherapy plays a huge part in improving function, particularly in the first few weeks after the onset of symptoms. People who are admitted to hospital with anterior cord syndrome tend to have longer hospital stays than those with other incomplete spinal cord injuries, but often show lots of functional improvement with extensive physical and functional therapies.

As the sensation of pain is so often impaired after an anterior spinal cord injury, an increased awareness and mindfulness of the surroundings is essential – many people retain the sense of touch to the areas affected by injury only to the anterior part of the spinal cord, but might not be able to tell the difference between normal touch and, for example, a serious burn or cut. People with loss of sensation – particularly pain sensation – should be extra cautious when it comes to any injury or danger.

The degree of muscle weakness and the extent of reduced mobility will be the main factor determining ongoing needs, and the greater the reduction in ability compared to the person’s pre-injury state, the more consideration needs to go into maintaining their standard of living and pursuing normal activities. People with any spinal cord injuries may have increased mobility needs, requiring wheelchair use, accessible housing, specialist equipment in the home, and sometimes a change of location. These needs should be taken into consideration prior to discharge from hospital, and a comprehensive occupational health assessment should be undertaken, with adaptations and equipment as required.

Other needs to consider include continence care, skin and pressure area care, meeting nutritional needs, and maintaining a healthy lifestyle, social life, and good mental health. A life-changing medical condition or care need can have a serious impact on mental health and emotional wellbeing and so planning for ongoing care needs must take this into account.

Care in the Community

For those who have ongoing, long-term extra needs, whether personal care such as help with washing and dressing, household tasks and normal day-to-day activities, there will be an assessment of your ongoing care needs. This may initially take the form of a multi-disciplinary team meeting in a rehabilitation centre, to work out the best way to get you home safely and with all the necessary assistance available.

Choosing a care provider who specialises in clients with spinal cord injuries means you’ll get the best care for yourself and your condition. At Total Community Care, we work with our clients to build exactly the right team and package of care for the individual, acting as facilitators for our clients to choose their own carers. We believe that you should have total control over the people you allow into your home and your life.

Lady in wheelchair with family and friends standing around her

The extent of your ongoing needs will inform the kind of care you choose – Further down the line, it may be that your care needs at home change, and that’s when it’s important to have a dedicated care manager overseeing a team of professionals and experienced carers who can provide continuous, ongoing assessments of any changing needs.

Again, the importance of emotional wellbeing cannot be overstated, at a time of great change, with the challenges of a new long-term medical condition, and when it might seem like previously easy and enjoyable activities, work, family, and social life take a lot more planning and effort than previously. Having a team dedicated to helping you maintain the kind of life you want to continue leading can make all the difference, and a care provider who values your choices and quality of life is essential.

At Total Community Care, we specialise in helping clients with significant care needs, setting up 24-hour-a-day care at home. We help you find the right team for you, taking into account the kind of lifestyle you want to lead. Our staff can travel with you, enable you to work and continue your family and social life as normal. We believe that when you work that closely with someone, you should have a chance to meet them beforehand, and assemble a team of people you enjoy spending time with, who are also experienced and exemplary professionals with a background and particular interest in medical and social care for people with long term needs.

If you’d like to discuss your home care needs and desires with a specialist spinal injury care provider, get in touch with us at Total Community Care.

Reference Material

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2031952/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4264724/

TCC supports individuals with Huntington’s Disease and are developing relationships with the Huntington’s Disease Association (HAD) https://www.hda.org.uk/huntingtons-disease).

An Overview of Huntington’s Disease

It is estimated that some 8,000 individuals in the UK display the symptoms of the disease but a further 32,000 carry the faulty gene.

Huntington’s disease is an illness caused by a faulty gene in your DNA (the biological ‘instructions’ you inherit which tell your cells what to do). If you have Huntington’s, it affects the body’s nervous system – the network of nerve tissues in the brain and spinal cord that co-ordinate your body’s activities.

Huntington’s can cause changes with movement, learning, thinking and emotions. Once symptoms begin, the disease gradually progresses, so living with it means having to adapt to change, taking one day at a time.

Some facts about Huntington’s:

  • Huntington’s is not something you can catch; it is inherited. Every child conceived naturally to a parent who carries the Huntington’s gene has a 50% chance of inheriting it.
  • A genetic test can find out if you have the faulty gene.
  • You can live with the faulty gene for years without symptoms, but if you do have it, at some stage you will develop symptoms. Doctors cannot tell you when this will be.
  • Huntington’s disease affects men and women. It usually develops between the ages of 30 and 50, but can start at any age. If you develop symptoms before the age of 20, this is known as Juvenile Huntington’s disease.

Living With Huntington’s Disease

Although there is, as yet, no cure, you can learn to manage symptoms more effectively to improve quality of life.

TCC believe that personalised care support packages can enable individuals to maintain their independence at home.

Reg Perrins—Commercial Director

The key to getting home and staying home after a brain injury is effective rehabilitation, good planning, and a bespoke team of carers and professionals to meet your ongoing care needs.

The brain is incredibly complex, and the results of any injury to the brain can range from mild changes to catastrophic, life-changing events.

Brain injury survival rates are improving all the time with advances in medical science and understanding. As important as survival rates are recovery rates, and this is more complex[i]. The complexity and range of the physical and functional brain mean that there’s huge variety in the effect and impact of a brain injury. The extent of recovery also varies, and isn’t always entirely predictable – the brain’s ability to ‘heal’ is limited, but people can make remarkable changes to compensate for injury.

Although injury to the brain can be catastrophic and wide-ranging in effect, improved care and rehabilitation services are improving outcomes for people with brain injuries all the time[ii]. Neurological rehabilitation is an area which remains consistently at the forefront of neuro and rehabilitative medicine.

Any kind of injury or condition that affects the brain can have a profound effect on a person’s abilities and a lasting impact on their life, and on the lifestyle and needs of both the injured person and the people around them.

Brain Injuries

Brain injuries are broadly divided into ‘acquired’ or ‘traumatic’.

Acquired brain injuries are usually a secondary result of a medical problem – for example, as a result of a stroke, or a ‘hypoxic’ brain injury – such as might result from lack of oxygen flow to the brain after a cardiac arrest.

Traumatic brain injuries are those usually sustained through direct force action to the skull, like in a car accident or a serious sports-related injury.

Whatever the cause of the brain injury, the impact can be wide-ranging, and the effects can be widely varying. The brain is the essential organ responsible for all conscious – and most unconscious – body processes, sensations, understanding, control, and thoughts. This means that it’s difficult to describe the effects of a brain injury – it can range from no noticeable effect, through to some memory loss, all the way through to profound disability.

Our team at Total Community Care understand that anyone requiring care at home after sustaining a brain injury is an individual, with specific needs and desires; no two people and no two conditions are the same. This is why a personalised, bespoke care package is essential to meet the needs of the person, not just the condition.

Having a hand-picked, dedicated team for your ongoing care means that you have control over the people who are coming to your home and helping you with all those things you might need help with. The way we put our teams together also means that you have consistency, continuity of care, and that we can offer our staff targeted training and support to help them look after you

Getting Home

When planning discharge home from hospital or a rehabilitation setting, it takes a whole multidisciplinary team to come together to make this transition as easy as possible[iii].

This is as much about you and the setting you’re coming home to, as it is about your functional ability and medical needs. To prepare for returning home you will need:

  • To be medically stable – time with physiotherapists, speech and language therapists, specialist doctors, nurses, and other rehabilitation specialists will help you get to a point where your needs are consistent enough to be managed at home. This might involve any of your daily living needs, anything from helping you get independently mobile to stable mechanical ventilation.
  • A home that’s ready for you – occupational therapists are usually the people most involved with assessing any new needs you have and making sure that everything at home is safe and appropriate for discharge. This might include adaptations such as improved access like widened doorways, ramps, and a stairlift. An appropriate bathroom or wet room with an accessible toilet, grabrails and a shower chair might be necessary.
  • Careful consideration of the things that matter to you as an individual, and the best ways to meet your social needs and lifestyle as well as physical needs – being able to get out and about, even if that involves having special breathing apparatus, specially designed wheelchairs, or just someone standing by.
  • Having the right care in place – a dedicated team who can provide care round-the-clock and any help that meets your particular needs – people who you’re comfortable with to provide intimate personal care, who can assess the best way to ensure you’re safe at home and stay that way; who make the meals you like, get you out and about, and generally meet any of your needs to facilitate your absolute best quality of life after a brain injury.

Wet room bathroom wet roomsIt can take some time to arrange a safe discharge plan – this time allows for home adaptations, for getting you to your best, most stable condition, and for compiling the right team of carers and professionals for input at home.

Unfortunately, it is common to see people trying to do too much too soon. One of the priorities of a good care team, however, is to make sure you get the rest needed to maximise your rehabilitation benefits.

The aim of a robust discharge plan is to get you home and keep you there; anticipating any bumps in the road and never rushing. It’s natural to be very eager to get home, but it can take time to get everything set up; we want to avoid anyone being admitted to hospital just because their social care needs aren’t being well met.

Having the right care team at home helps to reduce the chance of hospital admissions, but having the consistency of a dedicated care team who you get to know well also means that they’re in a good position to recognise any change in your condition. Carers who know you will know when things aren’t right, in a way that ad hoc or drop-in carers couldn’t.

Sometimes, planning discharge home with big changes and complex care needs takes a trial run – Total Community Care can work with the discharge team in your rehabilitation setting, liaising closely with occupational therapists to facilitate discharge trials.

Life After a Brain Injury

The range of functional changes that can occur after a brain injury mean that there’s no standard blueprint for the kind of life someone will lead afterwards. It depends on the individual, the extent of impact the injury has had on them, their care needs, and – at least as important as any of the practical considerations – the kind of life they’d like to lead, within their limits.

Whatever the extent of your brain injury, if you’re considering long-term care then there will be significant changes to the way you live your life. When it comes to the day-to-day activities of living, though, quality is key. We believe that the best way to enable you to maintain the kind of lifestyle that you enjoy is with a small group of dedicated carers and specialists who can get to know you.

It can take time to get used to the changes that come after any significant injury or change in ability and needs, but there are still ways to continue to do the things you enjoy – sometimes with a little creativity and adaptation. This depends on the extent of your ability – but simply getting out, whether in a specially adapted wheelchair, with mechanically assisted breathing, or needing only a little support, Total Community Care can help you enjoy your life.

With a bespoke care package, you and the people close to you can meet the carers, choose the people you want in your life, and make a plan to enable you to continue to enjoy the things you want to enjoy. We specialise in – and take pride in – providing a quality care package to promote your quality of life.

How Total Community Care Can Help

Having a brain injury can affect more than the individual who sustained it; it can have profound effects on family and friends, and can mean significant changes to the dynamics of a family. A person can go from being a breadwinner or caring for their children to needing significant care themselves. The needs of the family cannot be overlooked when planning care for a person with new needs; we aim to work with the whole family to make the transition into this new phase of everyone’s lives as smooth and easy as possible.

Traditional, drop-in care packages can offer adequate services when it comes to basic care needs: personal care, washing and dressing, continence needs and skin care can all be met to some degree by a conventional care package. For people with more significant or variable needs, a complete package is necessary. For people with variable oxygen requirements, with unpredictable continence, and who are at risk of damage to their skin from immobility, pressure or moisture, carers coming in three or four times a day simply cannot meet their needs.

Pulmonary odema and brain injuy

Total Community Care specialise in long-term solutions. We work with you and your medical and rehab team to create the right care package, with 24 hour care in your own home. We provide a service to meet your needs, and you choose what you want and need. We help you choose your own care team who can support you with all activities of daily living, give timely and effective care and support, and empower you to continue life as you want it.

Our staff work with you to help you rebuild the life you want – from cooking a meal to travelling the world, we support you – reablement, empowerment, and an individualised service. To meet the complex needs associated with a brain injury, we appoint a dedicated care manager for seamless integrated support. Our teams work together with their own support networks to continually improve their practice, and we provide a high level of education and training for our staff, specially tailored to meet the needs of their individual client.

Your care manager works with you and your family to coordinate a service that both meets your needs and fits your desired lifestyle. Getting home after a serious illness or injury is a time of many emotions – joy, fear, relief excitement, trepidation, and a whole range of complicated feelings. Having a care provider that enables you to meet and choose your own team, who will take over the practicalities of getting you home safely goes a long way towards easing that path – we aim to take the worry out of going home with additional care needs, and to keep you at home safely.

Support for you, support for those around you

Having a complete care package takes a lot of pressure off everyone when imagining life at home with new care needs. As you can choose the people who are there to assist you, 24/7, we hope that our staff become like family to you. Your loved ones can be as involved or separate from your care needs as they like; we work with people in all kinds of families. We’ve found that some families want to help provide hands-on support, and some prefer to keep those care needs separate from their special relationships within the family. There’s no right or wrong way to approach care at home, and we will be led by you and your family.

One of the benefits of choosing your own care team is something that isn’t always acknowledged by care providers: it’s just natural to get on better with some people more than others. Meeting a range of carers and supportive professionals and choosing your own team; people you want to allow into your home and who you can rely on to work in tandem with your family and lifestyle is incredibly important.

For more information or advice on planning a package of care at home, get in touch with our advisors at Total Community Care – we can work together to facilitate safe and effective care at home, enhance quality of life, and provide peace of mind for everyone involved.

[i] https://www.upi.com/Health_News/2019/05/08/Revised-guidelines-improve-survival-rates-for-traumatic-brain-injury-patients/9101557342416/

[ii] https://www.uptodate.com/contents/management-of-acute-moderate-and-severe-traumatic-brain-injury?topicRef=13854&source=see_link

[iii] https://www.headway.org.uk/about-brain-injury/individuals/rehabilitation-and-continuing-care/rehabilitation/