COVER STORY
MOTOR NEURONE DISEASE: NUTRITIONAL MANAGEMENT Lucy Williams RD Specialist Neurosciences Dietitian: Salford Royal NHS Foundation Trust Lucy is a Specialist Dietitian working in the Greater Manchester MND Care Centre. She is Team Lead for Neurosciences at Salford Royal NHS Foundation Trust.
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This article gives an overview of the multidisciplinary approach to the management of motor neurone disease (MND) and timely placement of gastrostomy. MND is the name given to a group of closely related disorders characterised by progressive degeneration of lower (spinal and bulbar) and upper (corticospinal) motor neurones, leading to muscle atrophy, paralysis and death.1 The disease can be classified into four main types depending on the pattern of motor neurone involvement and the part of the body where symptoms begin. Amyotrophic Lateral Sclerosis (ALS) is the most common type, accounting for over 75% of the total MND cases.2 It is characterised by muscle weakness and stiffness, over-active reflexes and, in some cases, rapidly changing emotions. Initially, the limbs cease to work properly and later the muscles of speech, swallowing and breathing can become affected. Whilst the greater number of patients present with progressive weakness of the arms and legs, between 25-30% of all patients initially present with bulbar symptoms.3 When MND begins in the muscles of speech and swallowing, it is described as Progressive Bulbar Palsy (PBP). The limb muscles may also become affected in PBP and it generally carries a poorer prognosis. Progressive muscular atrophy (PMA) and Primary lateral sclerosis (PLS) are much rarer forms of MND. In their true form, they have a slower rate of progression which may not significantly reduce life expectancy, but levels of disability can be high. As of yet, there is no clear cause for MND, but it is likely to be due to a combination of environmental, lifestyle and subtle genetic factors. In combination, they may ‘tip the balance’ towards
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someone developing the disease. In rare cases, there is a familial link for MND that is passed down through generations. Research shows abnormalities in the gene C9ORF72 in up to 46% of familial cases and 23% in sporadic cases.4 There is currently no cure for MND and most people die within two to three years of developing symptoms.5 THE IMPACT OF MND ON NUTRITIONAL STATUS
As MND progresses, it causes loss to the upper and lower motor neurones, resulting in weakness and wasting to the muscles involved in movement, mobility, breathing, swallowing and speech. This has a profound impact on a person’s ability to eat and drink and, therefore, on their ability to meet and maintain their nutritional needs. As the condition deteriorates, so too does the risk to nutritional status, which in itself impacts further on the deterioration of the clinical status of the patient, causing a vicious cycle that, without appropriate intervention, worsens prognosis.6 There are a number of factors that can influence nutritional status in MND, as shown in Table 1. These include: swallowing difficulties, which preclude safe oral intake; physical difficulties, which affect the ability to prepare food and feed one’s self; physiological changes, which cause a hypermetabolic state; and psychological factors affecting appetite and enjoyment of meals, amongst others.7 Dysphagia Dysphagia is perhaps one of the most challenging symptoms to manage; it is a significant problem for 90% of