Motor neurone disease (MND) is a fatal, rapidly progressing disease that affects the brain and spinal cord. MND attacks the nerves that control movement so muscles no longer work. It does not usually affect senses, for example sight, sound and touch.
There is no cure for MND, but there are interventions such as non-invasive ventilation and gastrostomy that can help manage symptoms and the assistance of their carer or nurse.
Up to half of all people with MND will be affected by some degree of cognitive or behavioural change. A proportion will be diagnosed with front temporal dementia. Some patients may experience cognitive changes, or be diagnosed with FTD, before they are even diagnosed with MND. These changes can affect behaviour, decision-making and the way they use language.
How can MND affect people at the palliative and end of life stage?
MND affects each person differently, so symptoms, progression and what to expect at the palliative and end of life stage can vary from person to person. Some of the symptoms that may affect a person in the later stages of MND are listed below, but it’s important to note that these symptoms could occur throughout the course of the disease.
- Muscle weakness: Widespread muscle wasting will affect a person’s mobility and their ability to undertake daily tasks. Most people with MND eventually need to use a wheelchair and will require support from carers /nurses.
- Respiratory problems: Most people with MND will experience breathing difficulties late in the course of their disease, because their diaphragm and accessory muscles will be affected. Some people may choose to have assisted ventilation which is administered by their carer or nurse.
- Dysphasia: Weakness of the bulbar muscles (tongue, mouth, and throat muscles) can lead to swallowing problems. Eating and drinking can become more difficult, which may lead to episodes of coughing and sometimes to choking and aspiration. Some people may choose to have a gastrostomy.
- Saliva problems: Bulbar weakness can also lead to the pooling and drooling of thin saliva, or difficulty clearing thick, tenacious saliva.
- Dysarthria: Bulbar weakness may lead to slurred or quiet speech. In rare cases, the person may lose their speech completely. People with MND may use a range of alternative and augmentative communication strategies and devices such as electronic devices.
- Pain: Patients suffering with MND may experience pain, usually as a result of muscle cramps or spasticity.
What a carer or nurse should be aware of when treating a patient with MND at the palliative and end of life stage?
- Cause of death: It is important to provide reassurance to your patient and their loved ones that the cause of death in people with MND is very rarely choking. The usual cause is respiratory insufficiency. If symptoms are well managed by their carer or nurse, the majority of cases death can be peaceful.
- Posture and positioning: If a person has respiratory problems, they may feel breathless when lying flat. This may feel uncomfortable, scary and, in some cases, may be dangerous. It is vital for their carer or nurse to find positions that make it easier for their patient with MND to breathe. The ideal position is usually upright or slightly reclined, with the arms, back, head and neck supported.
- Oxygen: Although there are some exceptions, oxygen therapy generally can have a harmful effect in people with MND, reducing respiratory drive and worsening their condition.
- Emotional and psychological support: Patients suffering with MND and those close to them often experience considerable psychological and emotional distress. Being a carer or nurse for someone with MND can also be physically and emotionally challenging.
- Cognitive change: Up to half of all patients suffering with MND will experience change in thinking and behaviour. So a nurse would need to take care to ensure they are able to give informed consent during decision making. All patients diagnosed with MND should be screened for cognitive change using appropriate assessment tools. People with MND and front temporal dementia may lack mental capacity and care should be adapted accordingly.
Considerations should be made in approach to providing the proper carer or nurse for someone with MND?
- Early referral to specialist palliative care: MND can progress rapidly, support from specialist palliative care or nursing services should be available as soon as your patient feels it is appropriate for them. Specialist palliative care and nursing can provide a crucial source of support as part of the multidisciplinary team and can make a huge difference to quality of life for both patient and family.
- Timely access to equipment and support: Equipment, support and symptom management should be planned ahead and put in place early, where possible. However, this should be approached with care and sensitivity and discussed with their patient and their family first. Also, every effort should be made to prevent untimely case closures, as needs can change quickly.
