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
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CONDITIONS & DISORDERS Table 1: Causes of malnutrition Dysphagia Problems with salivary secretion and mastication Loss of dexterity of the upper limbs affecting ability to prepare and eat foods Hypermetabolism
Cognitive impairment Depression
Respiratory insufficiency
Constipation
Fatigue
Polypharmacy
patients with MND8 and is a major cause of morbidity and mortality.9 Dysphagia results from damage to either corticobulbar pathways or brainstem motor nuclei, causing spasticity and weakness to the tongue, lips, facial muscles, pharynx and larynx, as well as impacting upon the management and clearance of saliva and respiratory secretions. This difficulty in swallowing can lead to prolonged and effortful mealtimes, distressing episodes of coughing and choking, increased chest infections caused by aspiration pneumonia, dehydration, weight loss and malnutrition.10-11 The clinical assessment of bulbar symptoms requires a multidisciplinary approach, comprising of history taking, evaluation of weight and BMI, respiratory function assessment and clinical examination of swallowing and speech. Hypermetabolism Hypermetabolism is now widely considered a feature of MND, although the exact origins are not yet clear. Some studies suggest it exists as a result of the increased effort of breathing and metabolic demands on the remaining functional muscles, thus increasing energy requirements.10 More recent research, however, suggests that a number of other factors correlate with a hypermetabolic state, including fat-free mass, age, sex and weight, as well as the possibility that the sympathetic nervous system and mitochondrial derangements (causing excessive heat production and energy deficit) may also play a role.12 Further research is clearly required; however, regardless of its origin, hypermetabolism itself poses a further challenge in meeting the nutritional requirements of patients with MND. THE PREVALENCE OF MALNUTRITION
Malnutrition is a major concern in MND, occurring in approximately 55% of patients13 and several 14
Anxiety Embarrassment when eating
studies demonstrate that nutritional status is an independent prognostic factor for survival, with malnourished patients having an eightfold increased risk of premature death.14-15 Notably, whilst patients with dysphagia may have a poorer prognosis, they are not considered any more likely to be malnourished than those without.14,16-17 As discussed previously, MND is associated with an altered nutritional state, energy intake and expenditure; 70% of patients have a decreased calorific intake and consume less than the recommended dietary allowance for energy.16,18 THE MANAGEMENT OF NUTRITIONAL STATUS
In the absence of a cure, symptomatic and palliative treatment is the priority for optimising quality of life.21 Management of nutritional status throughout the course of disease progression is pivotal for enhancing quality of life and optimising the timing of appropriate interventions.22 It is recommended that nutritional assessment should commence as soon as the diagnosis of MND is suspected23 and be reviewed on a three-monthly basis through measurement of body weight.9 Involvement of a speech and language therapist and a dietitian is essential from an early stage to assess, monitor and review the individual’s nutritional intake so that practical oral and non-oral dietary advice can be given to enable nutritional needs to be met. Nutritional insufficiency may evolve gradually and asymptomatically and, therefore, a proactive approach for early recognition and intervention may delay or minimise associated complications.24 It is important that patients with MND are not screened for nutritional risk based solely on the Malnutrition Universal Screening Tool (MUST) score. These patients are at high risk of rapid deterioration in nutritional status and will benefit from early discussions regarding
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Table 2: Members of the MDT29 Core MDT members
Other specialists
Neurologist Neurology specialist nurse Palliative care specialist Dietitian Physiotherapists Occupational therapists Respiratory physiologist Speech and language therapist
Psychology and counselling services Social care Respiratory medicine Gastroenterology Community neurology teams Equipment services
preferences for treatment as the condition progresses and early interventions to avoid crisis situations. There are a number of strategies that can be implemented to optimise nutritional intake, including the use of adaptive cutlery and posture support, modified texture diets and safer swallowing techniques, such as a chin tuck swallow, increasing the frequency of meals and fortifying the energy density, as well as using ONS.10,21,22 However, due to the progressive nature of the disease, over time these measures usually become insufficient and enteral feeding is then indicated.9 THE ROLE OF THE MULTIDISCIPLINARY TEAM (MDT)
The MDT is widely considered a fundamental part of the management of MND due to the diverse and interlinking symptoms.26 Evidence suggests that multidisciplinary care can show improvements to both quality and quantity of life, by not only improving timely access to supportive interventions such as gastrostomy and non-invasive ventilation (NIV), but by providing tailored support and education to families and carers.27,28 Table 2 shows the recommended core practitioners for an MDT, along with other specialists that may need to be accessed for additional support. The MDT can provide early holistic assessment of nutritional status and supportive measures that can optimise intake. This may prolong the time period before more invasive supportive measures are required. GASTROSTOMY FEEDING - WHEN IS THE RIGHT TIME?
For both professionals and many patients discussions around enteral feeding can be a source of intense discomfort and considering this at an early stage can be difficult due to uncertainty
about disease course. Discussions can bring to the fore the reality of the progressive nature of the condition and patients are frequently reluctant to consider further deterioration at an early stage. Gastrostomy feeding is recommended to provide long-term nutritional support for patients with amyotrophic lateral sclerosis with severe dysphagia.9 The European Federation of Neurological Societies (EFNS) guidelines26 recommend early insertion of gastrostomy based on considerations of the quality of bulbar function, weight loss (>10% of premorbid), respiratory function and general condition of the patient. As MND progresses, there is an increased risk of developing respiratory compromise, making gastrostomy insertion a more risky procedure. This is supported by the American Academy of Neurologists (AAN) recommending gastrostomy insertion when forced vital capacity (FVC) is greater than 50% to reduce procedural risks.9 This means that the opportunity for safe placement of gastrostomy should be seized at the earliest opportunity and this necessitates early robust discussion about future management of nutritional needs. An important part of this process is to encourage the patient to take an active role in the management of their condition and limit situations in which the patient must make difficult decisions for themselves under pressure of rapidly deteriorating clinical status. It is useful to consider the following points when supporting discussions around gastrostomy: • The patient is given sufficient background information to help in the decision. • Information is provided in a sympathetic and empathic way, with both sides of the argument given. • Time is spent explaining the various points and aims of any interventions or treatments.
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CONDITIONS & DISORDERS • The person is supported, if they wish, by family or friends. • Written information may be of help to allow further consideration.30 It is important to dispel any myths surrounding gastrostomy, so that patients are aware that the main function of the tube is to improve quality of life. Conversations about gastrostomy insertion can be particularly distressing for patients who are in the early stages of their disease and still eating and drinking well. The gastrostomy, when used appropriately, can be significant in reducing episodes of distress associated with eating and drinking, such as fears of choking, optimising nutrition and hydration and improving social isolation experienced from prolonged mealtimes.31 The www.mytube.mymnd.org.uk website32 is a fantastic resource to assist patients through the decision-making process. It allows them to access safe and reliable information about gastrostomy placement and management within the comfort and privacy of their own homes. Many patients find it easier to process this information in their own time and this enables them to be more open in later discussions. There are three main methods of gastrostomy insertion currently used in patients with MND: • percutaneous endoscopic gastrostomy (PEG) • radiologically inserted gastrostomy (RIG) • per-oral image-guided gastrostomy (PIG)33 The ProGas study34 is the most recent large scale prospective cohort study that has looked at the optimum timing and method of gastrostomy in patients with ALS. The study made a number of key findings and recommendations that may be used to influence and guide future practice. Firstly, the study recommends that it may be more beneficial to consider placement of gastrostomy at approximately 5% weight loss. This was based on a number of factors: • The odds for 30-day mortality were greatest for patients who had lost >10% of their prediagnosis weight. • Gastrostomy feeding only prevented further weight loss in approximately half of the study participants. 16
• In those patients who gained weight, these gains were small and the clinical benefit was not clear. • The greater the percentage of weight loss at the time of gastrostomy from diagnosis, the less likely it was for patients to recover this loss after gastrostomy. This was most evident in the subgroup of patients who had lost >10% of their diagnosis weight. • Those patients whose weight loss continued following gastrostomy had poorer survival rates. The study also recommends that PEG is the optimal method of placement in patients where respiratory function is largely unimpaired, followed by PIG when respiratory function is significantly compromised. These recommendations relate primarily to the fact that patients who underwent RIG experienced a higher rate of gastrostomy tube-related complications such as displacement/leakage. Some patients choose to decline additional supportive options such as gastrostomy. In these cases, it is important to ensure that any misconceptions are corrected, but if ultimately the decision remains the same, it is essential that they are given reassurance that supportive care will continue to be provided, with the ongoing aim of managing symptoms and minimising any discomfort or distress. SUMMARY
Patients with MND are at high nutritional risk and poor nutritional status is an independent prognostic factor for survival. Early assessment and ongoing review are essential to optimise the timing of interventions to support nutritional needs, with the role of the MDT being essential. Gastrostomy is recommended to provide long-term nutritional support for patients with MND. It may be more beneficial to consider placement of gastrostomy at approximately 5% weight loss from pre-diagnosis weight. PEG is the optimal method of placement in patients where respiratory function is largely unimpaired, followed by PIG when respiratory function is significantly compromised. For patients who decline interventions, they must be reassured that supportive care will continue.
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