General awareness of motor neuron disease (MND) rose quickly after the Ice Bucket Challenge went viral in July-August 2014. To mark the one year anniversary of this fantastic campaign it is important to discuss advancements in the management of this disease.
To date, there is no known cure or effective treatment for motor neuron disease (MND), with two Australians dying from the disease every day. MND has many names, including amyotrophic lateral sclerosis (ALS) or Lou Gehrig’s disease. It is a progressive disease that attacks motor neurons in the body and eventually leads to muscles wasting away.
Muscle wastage results in simple limb movements, swallowing, talking, and even breathing being increasingly difficult. Stephen Hawking is one well-known sufferer of MND, yet despite his disease, his amazing courage and perseverance is certainly an inspiration to all individuals affected by this disease.
What Causes Motor Neuron Disease?
The cause of MND remains relatively unknown. Researchers have found hereditary factors account for around 5-10% of cases, but most causes of MND remain unidentified. Current research into this area is looking at not only genetic factors, but also nerve growth factors, the repair and ageing of motor neurons, immune-mediated damage, as well as viruses, toxins, and chemical interactions with nerve cell control and communication.
Risk Factors and Symptoms
MND affects both adults and children. With children, often the symptoms are present at birth or begin to appear before the child starts to walk. With adults the symptoms can begin appearing after the age of 40. Men are more susceptible to the disease than women.
The symptoms and the rate of progression of MND vary between individuals. Early symptoms may present as a mere weakness or tingling in the hands and feet. Individuals may have trouble holding objects and may notice they are stumbling more. Some people may notice they are beginning to slur their speech and may have difficulty swallowing. These weaknesses then progress to widespread muscle wastage.
Complications of Motor Neuron Disease
The average life expectancy of someone with MND is 27 months. Up to 50% of people with MND will also display changes in their cognition, language, behaviour, and personality as the disease progresses. They develop generalised paralysis, lose their capability to speak, and have difficulty swallowing. As such, they become dependent on others for all aspects of their day-to-day living—from mobility to eating. This can lead to the development of complications such as aspiration pneumonia and the development of pressure areas in addition to negative emotional and psychological effects.
The Nurse’s Role
The nurse’s role becomes increasingly important as the individual with MND progresses through the disease process. Patients will require increasing support and assistance for their day-to-day activities, although, this is often provided by a family carer. The nurse plays a number of vital roles, including both the provision of support to the MND sufferer and their carer while in the community. They will also be a member of the multidisciplinary team caring for the person if the individual is hospitalised.
As a nurse, you will need to be aware of potential complications of the patients’ disease progression—such as aspiration—and ensure care is targeted to prevent these complications eventuating. This could be as simple as ensuring the patient is sitting upright when eating. The carer’s of these individuals are also often experiencing both physical and emotional demands and stress from their role. So nurses must also be considerate to the needs of the patient’s carers and support them during the disease process.
Treatment of MND
Although motor neuron disease is currently incurable, some medications have been shown to be effective in helping sufferers remain in the milder stages of the disease for longer, ultimately prolonging their median survival by 2-3 months. Symptoms can also be managed by the help of a multidisciplinary healthcare team who provide assistance with interventions to improve quality of life during the disease process.
Current research focuses on a multitude of areas within MND—from what causes it, to how to treat it. Therefore, funding into MND is essential to ensure this research continues. No doubt we’ve all heard about, or perhaps even participated in the Ice Bucket Challenge. This particular initiative raised more than 164 million dollars (USD) for MND worldwide in 2014. Research funded by this campaign has already led to the identification of certain genes associated with a rare inherited form of MND, research into a certain protein mutation and toxicity to motor neurons, and even the use of a mathematical approach to predict MND.