Neuromuscular Disorders
Volume 12, Issue 10 , Pages 970-974, December 2002

Workshop: Management of adults and children with feeding difficulties secondary to chronic muscle disease, 22nd March 2002, Sheffield, UK

  • Marguerite Hill

      Affiliations

    • Institute of Clinical Neurosciences, Frenchay Hospital, Bristol BS16 1LE, UK
    • Corresponding Author InformationCorresponding author. Tel.: +44-117-918-6762; fax: +44-117-918-6672
    • ,
  • Tom Hughes

      Affiliations

    • Department of Neurology, University Hospital of Wales, Heath Park, Cardiff CF14 4XN, UK

Article Outline

 

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1. Introduction 

People with chronic muscle disease frequently experience problems maintaining an adequate nutritional intake. The reasons for this are complex and stretch far beyond the mechanics of swallowing. In patients with a swallowing problem the lack of informed carers and of suitably prepared food combined with coexistent impairments of respiratory function, cough, posture control, motivation, appetite, control of secretions and chewing may contribute as much or more to failure of oral feeding as defects in the mere swallowing process. Furthermore, while there are clear physical consequences following from inadequate swallowing and nutrition such as failure to thrive and recurrent chest infections, it is also important to appreciate the impact that such difficulties have on family dynamics, social interactions and self esteem. Yet despite the manifest importance of maintaining an adequate nutritional intake in people with muscle disease, the evidence for optimal assessment and management is extremely poor and largely anecdotal. This workshop was convened under the auspices of the Muscular Dystrophy Campaign (MDC) of Great Britain to review the evidence for the optimal assessment and management of eating difficulties in people with muscle disease, to suggest some basic management guidelines and highlight areas for future research. The findings of this workshop were presented and discussed at the Annual Symposium of the MDC on 22nd March, 2002. This paper is a summary of the findings of this workshop.

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2. The extent of the problem 

The workshop limited itself to the issue of feeding difficulties in people with chronic progressive muscle disease. Dysphagia associated with relapsing inflammatory disease of muscle or the neuromuscular junction was explicitly excluded as it can generally be effectively treated using immunosuppressant therapy.

A normal swallow is divided up into three phases; oral, pharyngeal and oesophageal. During the oral phase the food is macerated by the teeth to form a bolus and the tongue then elevates to propel the bolus over the lingual surface to the posterior pharyngeal wall. During the pharyngeal phase, the bolus is propelled by the pharyngeal musculature to the oesophagus and the larynx is pulled upwards to protect the airway. The upper oesophageal sphincter relaxes and the food is propelled towards the stomach by the peristaltic action of the oesophageal muscles. Tom Hughes described the common swallowing abnormalities in chronic muscle disease, which are primarily due to weakness as neural input is preserved. Weakness of the tongue, face and jaw may impair the ability to adequately prepare a bolus and to retrieve bolus particles. Jo Philpot commented that abnormal mouth architecture is a common problem in children with congenital muscular dystrophy [1]. Whilst palatal weakness may increase the chances of nasal regurgitation and pharyngeal weakness will impair the pressure applied to the tail of the bolus, it is weakness of the anterior and posterior suprahyoid musculature which can be particularly disabling. This is because the hyoid and larynx are pulled upwards and forwards by these muscles and this serves to pull open the relaxed upper oesophageal sphincter; the associated fall in sphincter pressure exerts a downwards pull on the bolus. If these muscles are weak the upper oesophageal sphincter does not open adequately, the bolus is not pulled down into the upper oesophagus effectively and pooling of bolus occurs in the pharynx. If there is associated weakness of laryngeal closure, impaired respiratory function and poor posture control the patient is at very high risk of developing chest problems.

The prevalence of swallowing difficulties in people with muscle disease is unknown and is difficult to determine, due in part to different definitions of the problem. Dysphagia can be said to be present (or dysphagia can be diagnosed) on the basis of a symptom, a clinical sign or a radiological sign, or when it is uncovered by the occurrence of nutritional or respiratory complications. In some conditions such as oculopharyngeal muscular dystrophy, dysphagia forms part of the symptom complex and is therefore predictable. In more generalised dystrophies, feeding difficulties are one aspect of often complex disabilities and as such are frequently overlooked and under-reported. A questionnaire based study of people with a range of neuromuscular disorders, both inflammatory and progressive, estimated a prevalence of 34.9% [2] overall. Even respondents with FSH, a dystrophy not normally associated with swallowing difficulties, reported occasional choking episodes. This was a questionnaire based study dependent on accurate self-reporting and therefore it is likely that this is an underestimate of the true figure. Feeding problems often develop insidiously so that the patient gradually adapts and develops coping mechanisms, therefore their method of feeding may be normal and not a problem to them despite the fact that it is abnormal when compared with their peers and inadequate in nutritional terms. Indeed Jo Philpot reported that observation of children with merosin deficient congenital muscular dystrophy while feeding was more informative than direct questioning [1]. Research into the problem is urgently needed as it is likely to have a direct impact on the provision of services for people with chronic muscle disease.

There is even less data available on the impact of eating difficulties on the physical, social and psychological well-being of people with muscle disease and their families. From the physical point of view, chronic malnutrition must have a negative impact on the function of diseased muscle and will delay the development of puberty in teenagers. Recurrent chest infections secondary to aspiration, often attributed to respiratory muscle weakness, may result in permanent lung damage. One study reported that out of 11 patients assessed for swallowing dysfunction, four died over the period of follow-up (25 months) directly as a result of these problems [3]. Fear of choking or nasal regurgitation and prolonged feeding times can lead to social isolation and have adverse effects on family dynamics; parents may feel guilty because their children are failing to thrive despite their best efforts to feed them. Despite this Jo Philpot reported that when asked about feeding difficulties many families said that it was the first time the issue had been discussed. However, she also noted that managing children and adults of low weight may be easier for carers, and significant weight gain may compromise their ability to be cared for at home.

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3. Clinical assessment of eating difficulties in people with chronic muscle disease 

Sally Darby and Sue Tyres reported on the initial assessment of people with muscle disease. They both made the point that eating difficulties need to be recognised as part of the symptom complex of muscular dystrophy and patients need to be routinely questioned about them rather than waiting for them to volunteer the information. They recommended that all patients are asked about changes in diet, coughing and choking, nasal regurgitation, frequency of chest infections, time taken to eat meals and the amount of food actually eaten. Sue Tyers considered the possibility that some of this information could be elicited by questionnaire. In her experience most of the questionnaires developed for this purpose were too long to be clinically useful. However, a short, well validated questionnaire may be useful for screening. At the moment there does not appear to be a questionnaire that fulfils these requirements.

Sally Darby recommended that each person attending a muscle clinic should be weighed at each visit so that progressive weight loss in adults or failure to thrive in children can be detected early. She noted that there are reports that some individuals with feeding difficulty could gain weight if they altered their diet to increase their intake of calorific but easily swallowed foods such as milk based products [4], but felt this to be an uncommon problem in clinical practice.

If a possible problem with eating is identified during a routine assessment, the individual should be referred for more detailed assessment. Indeed it could be argued that patients in whom dysphagia is an almost invariable feature of the disease should be under routine follow-up by an appropriate professional with an interest in swallowing difficulties so that problems can be identified early. Sue Tyers described the assessment provided by a Speech And Language Therapist with an interest in dysphagia, which should include a full clinical history, assessment of oral anatomy and observation of a controlled swallow using foods of different consistencies. Sally Darby reported that the dietetic assessment should include assessment current diet, consistency of food eaten and calorific intake and the time taken to complete meals.

Tom Hughes noted that not all the difficulties with feeding in people with muscle disease were necessarily due to muscle weakness and the clinician should also explore other factors that may directly or indirectly have a negative impact on the individual's ability or willingness to feed. Examples of such factors include depression, anorexia nervosa, cognitive decline, respiratory insufficiency, excessive daytime sleepiness, dry mouth or excessive salivation, oesophageal reflux, poor dental hygiene, inability to feed efficiently because of arm weakness and failure of carers to provide adequate assistance for feeding. Addressing such issues directly may have a significant impact on the individual's nutritional status.

Chris Milford and Stephen Golding reviewed the techniques currently available for more detailed assessment of the mechanism of dysphagia namely endoscopic examination, oesophageal manometry and video fluoroscopy (modified Barium swallow). Endoscopic examination of the larynx and hypopharynx provides a direct view of the larynx and pharynx during swallowing and can identify problems such as reduced movement of the vocal cords (unilateral or bilateral), pooling of secretions within the hypopharynx and secretions ‘spilling’ over into the larynx [5]. It is a relatively straightforward procedure in experienced hands that can be performed under local anaesthetic in the outpatients department. Oesophageal manometry measures the pressure of the upper oesophageal sphincter and has been advocated by some in the assessment of dysphagia but Chris Milford felt that technical problems limits its usefulness and it does not provide useful information regarding aspiration [6].

Stephen Golding reported that videofluoroscopy (modified Barium swallow) has long been thought to be the ‘gold standard’ of swallowing assessment. It allows accurate assessment of all phases of swallowing from the mouth to the oesophagus and detects silent aspiration more accurately than clinical assessment [7]. It can also permit assessment of the efficiency of various therapeutic measures designed to improve the efficiency and safety of swallowing. He felt that it is crucial that all videofluoroscopies are interpreted by an experienced radiologist.

There was some discussion in the workshop about the value of videofluoroscopy in the management of dysphagia due to muscle disease. While the examination may provide the most accurate demonstration of the abnormal swallow, there was a concern that clinician may be tempted to institute therapy solely on the basis of the videofluoroscopy rather than treating the patient as a whole. A recent report looking at neurogenic dysphagia came to the conclusion that videofluoroscopy did not change patient management [8]. There are no well conducted prospective studies looking at the role of videofluoroscopy in the assessment of a patient with muscle disease that not only consider outcome in videofluoroscopy terms but also quality of life and patient satisfaction over the medium and long term. In view of this the workshop participants came to a consensus view that videofluoroscopy is a useful adjunct to thorough clinical assessment of an individual with swallowing problems secondary to muscle disease in the following situations:

•if the underlying cause of the swallowing problem is unclear,

•if surgical intervention is being contemplated,

•to reinforce the nature of the problem to the patient,

•for research purposes (subject to ethical approval).

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4. Management 

Sue Tyers and Sally Darby reported that in most cases the initial detailed assessment may reveal the nature of the problem and strategies to improve the safety and efficiency of swallowing and feeding may be sufficient. Strategies include changing in posture during swallowing [9], [10], [11], [12], [13], manoeuvres including effortful swallowing and double swallows [12], [13], enriching current food and utilising appropriate dietary supplements [14], [15], [16] and altering the consistency of food and liquids. However, most of the evidence has been obtained from studies of neurogenic dysphagia and it is assumed rather than proven that the same interventions will be effective in muscle disease. Furthermore, one study has shown that 21% of subjects never complied with advice on safe swallowing, and this is particularly true for young people living at home which account for a significant proportion of people with muscle disease [17]. Jo Philpot noted that it is not clear if early intervention with dietary supplements, for example, can prevent problems later, particularly in children, and this was felt to be a key area for future research.

Tom Hughes recommended that other key issues are addressed at this stage to aid in the feeding process. Xerostomia may be improved by provision of artificial saliva while excess salivation may respond to hyoscine, oral atropine or glycopyrronium. Attention should be paid to the possibility of oesophageal reflux, especially in children who may need proton pump inhibitors or, in some cases, fundoplication.

Chris Milford described the three main surgical procedures used in the treatment of dysphagia, namely cricopharyngeal myotomy (CPM), oesophageal dilatation and botulinum injection of the cricopharyngeus muscle. Videofluoroscopy often demonstrates an apparent hypertrophy or spasm in the cricopharyngeus muscle although felt that it is possible that this appearance could be due to inadequate hyolaryngeal movement rather than true hypertrophy. This observation has made this muscle a target for interventional therapy aimed at relieving the apparent obstruction. Myotomy of the muscle has gained popularity as a treatment for a range of swallowing disorders, although the evidence that it is of benefit is based on retrospective reports of small numbers of people with a range of disorders followed up for a relatively short period of time [4]. The assessments used to monitor outcome are not consistent between studies making it difficult to make direct comparisons of efficacy and it is likely that there is a bias towards reporting positive results [18], [19]. Overall Chris Milford felt that CPM may be indicated in a small group of patients with otherwise good oral and pharyngeal phase swallowing. Appropriate selection criteria are as yet unclear but patients with severely impaired pharyngeal propulsion are unlikely to benefit from the procedure [3]. There is a clear need for well designed prospective trials of this procedure in a well evaluated population of patients with muscle disease.

More recently botulinum toxin injected directly into the cricopharyngeus muscle to temporarily weaken the muscle has been advocated as a treatment for dysphagia. Anecdotally, this appears to be most successful in patients with isolated cricopharyngeal or Xth nerve dysfunction, as opposed to more global problems [20]. There are no trials of its use in muscle disease and there was concern about the possible complications of intentionally paralysing already weakened muscles. Currently, it was felt that there was no evidence to support its use in patients with dysphagia secondary to chronic muscle disease.

Sue Tyers reported that in her experience balloon dilatation of the upper oesophageal sphincter is a useful procedure in OPMD. Others have reported a modest improvement in dysphagia in 55.7% in OPMD with moderate to severe dysphagia, maintained for at least 18 months. Interestingly, radionucleotide and manometric studies did not appear to predict a positive outcome [21]. Further studies are needed to evaluate this procedure in a range of patients to clarify the indications for the procedure and long term outcome.

Sally Darby reported that enteral feeding may be considered for a number of reasons: recurrent aspiration, inability to maintain adequate oral intake or for administration of medication and fluids. The preferred method is insertion of a percutaneous endoscopic gastrostomy tube (PEG) because it is discrete and may be more effective in reducing aspiration than a nasogastric feeding tube. However, it was noted that evidence for the superiority of PEG over NG in preventing aspiration is based on studies of dysphagia following stroke [22] where a significant proportion of patients had a reduced conscious level and therefore these results are not directly transposable to the population currently being considered. Tom Hughes commented that he had patients who preferred to use NG feeding to supplement their diet and became proficient in self-insertion of the tube.

There is little published evidence that allowed the group to assess the value of PEG feeding in patients with muscle disease. Jo Philpot noted that in her study all of the children with feeding problems in her study who had a PEG inserted increased their weight from below the third centile up to the tenth centile. However, she also noted that a number of children and their parents were resistant to the idea of PEG feeding, and a few refused this intervention, although those who had the PEG inserted were generally pleased that they had done so. Sally Darby felt that part of the problem might be the perception that insertion of a feeding tube is a ‘terminal event’ and that once inserted it is permanent and that the individual can never feed orally again. It is important that all issues of PEG placement are thoroughly discussed with the patient, including management at home. Respiratory function should be assessed when considering insertion of a feeding tube as significant respiratory compromise adds to the risk of the procedure. In such patients a PEG can be inserted radiographically. She felt that it was crucial that once a PEG has been inserted the patient is regularly assessed by a specialist team to ensure adequate nutrition and to detect potential problems.

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5. Conclusions 

Feeding difficulties are a common complication of chronic muscle disease, and are associated with significant physical, psychological and social morbidity. However, this problem is under-recognised and the true prevalence is unclear. Issues relating to eating should be specifically addressed on a regular basis in this patient population, and if there is a suggestion that there may be problems, the individual needs to be formally assessed by experienced clinicians and therapists. The value of invasive investigations in patient management is a subject of debate as the evidence base demonstrating benefit is poor. Furthermore, the role of surgical intervention is not clear; well designed studies in this area would be extremely helpful when deciding on optimal management of such patients.

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Acknowledgements 

This workshop was made possible by the financial support of the Muscular Dystrophy Campaign of Great Britain.

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Appendix A. Workshop participants 

Sally Darby (Bristol, UK)

Stephen Golding (Oxford, UK)

Marguerite Hill (Bristol, UK)

Tom Hughes (Cardiff, UK)

Chris Milford (Oxford, UK)

Jo Philpot (Frimley Park, UK)

Jane Stein (Oxford, UK)

Sue Tyers (Salisbury, UK).

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References 

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Neuromuscular Disorders
Volume 12, Issue 10 , Pages 970-974, December 2002

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