Alan Rio

The management of motor neurone disease

The management of motor neurone disease (MND) has evolved rapidly over the last two decades. Although still incurable, MND is not untreatable. From an attitude of nihilism, treatments and interventions that prolong survival have been developed. These treatments do not, however, arrest progression or reverse weakness. They raise difficult practical and ethical questions about quality of life, choice, and end of life decisions. Coordinated multidisciplinary care is the cornerstone of management and evidence supporting this approach, and for symptomatic treatment, is growing.1–3 Hospital based, community rehabilitation teams and palliative care teams can work effectively together, shifting emphasis and changing roles as the needs of the individuals affected by MND evolve. In the UK, MND care centres and regional networks of multidisciplinary teams are being established. Similar networks of MND centres exist in many other European countries and in North America. Here, we review current practice in relation to diagnosis, genetic counselling, the relief of common symptoms, multidisciplinary care, the place of gastrostomy and assisted ventilation, the use of riluzole, and end of life issues. View this table: Table 1 Clinical syndromes of MND (ALS—amyotrophic lateral sclerosis) and related disorders (modified from Kato et al , 2003*, with permission) The average delay from onset of symptoms to diagnosis is about 14 months, about one third of expected survival. Occasionally, survival following diagnosis may be less than six months. The patient may already suspect the diagnosis …

Survival of patients with ALS following institution of enteral feeding is related to pre‐procedure oximetry: A retrospective review of 98 patients in a single centre

A retrospective review was carried out on the influence of pre‐procedure respiratory assessment on survival of patients with amyotrophic lateral sclerosis (ALS) requiring nutritional support with either a gastrostomy or a nasogastric feeding tube. Over a five‐year period 98 patients (49 male, 49 female; median age 61 years, range 26–86 years) with ALS were referred for enteral feeding with either radiological inserted gastrostomy (RIG), percutaneous endoscopic gastrostomy (PEG) or nasogastric tube (NG). Case notes review was performed to record patient age, sex, pre‐procedure respiratory assessment, method of enteral feeding and survival post‐procedure. Kaplan‐Meier survival curves were constructed for each group, with Cox regression analyses performed in order to establish the effect of each variable on outcome. Median survival (with 95% confidence intervals) following RIG, PEG and NG was 6.31 months (4.58–8.04 months), 7.13 months (4.81–9.45 months) and 0.95 months (0.00–2.77 months), respectively. The survival advantage between RIG and PEG was not statistically significant (p = 0.50), but for NG versus RIG and PEG groups combined, there was a significant difference (p = 0.03). For patients with normal overnight oximetry, median survival was 8.54 months (3.88–13.21 months), compared to 4.80 months (1.20–8.39 months) in the abnormal oximetry group (p = 0.03; relative risk 1.97). It is concluded that RIG and PEG are equivalent in terms of post‐procedure survival. Abnormal oximetry prior to the procedure is a significant indicator of post‐procedure survival.

Occurrence of refeeding syndrome in adults started on artificial nutrition support: prospective cohort study

Background Refeeding syndrome is a potentially life-threatening condition characterised by severe intracellular electrolyte shifts, acute circulatory fluid overload and organ failure. The initial symptoms are non-specific but early clinical features are severely low-serum electrolyte concentrations of potassium, phosphate or magnesium. Risk factors for the syndrome include starvation, chronic alcoholism, anorexia nervosa and surgical interventions that require lengthy periods of fasting. The causes of the refeeding syndrome are excess or unbalanced enteral, parenteral or oral nutritional intake. Prevention of the syndrome includes identification of individuals at risk, controlled hypocaloric nutritional intake and supplementary electrolyte replacement. Objective To determine the occurrence of refeeding syndrome in adults commenced on artificial nutrition support. Design Prospective cohort study. Setting Large, single site university teaching hospital. Recruitment period 2007–2009. Participants 243 adults started on artificial nutrition support for the first time during that admission recruited from wards and intensive care. Main outcome measures Primary outcome: occurrence of the refeeding syndrome. Secondary outcome: analysis of the risk factors which predict the refeeding syndrome. Tertiary outcome: mortality due to refeeding syndrome and all-cause mortality. Results 133 participants had one or more of the following risk factors: body mass index <16–18.5≥(kg/m2), unintentional weight loss >15% in the preceding 3–6 months, very little or no nutritional intake >10 days, history of alcohol or drug abuse and low baseline levels of serum potassium, phosphate or magnesium prior to recruitment. Poor nutritional intake for more than 10 days, weight loss >15% prior to recruitment and low-serum magnesium level at baseline predicted the refeeding syndrome with a sensitivity of 66.7%: specificity was >80% apart from weight loss of >15% which was 59.1%. Baseline low-serum magnesium was an independent predictor of the refeeding syndrome (p=0.021). Three participants (2% 3/243) developed severe electrolyte shifts, acute circulatory fluid overload and disturbance to organ function following artificial nutrition support and were diagnosed with refeeding syndrome. There were no deaths attributable to the refeeding syndrome, but (5.3% 13/243) participants died during the feeding period and (28% 68/243) died during hospital admission. Death of these participants was due to cerebrovascular accident, traumatic injury, respiratory failure, organ failure or end-of-life causes. Conclusions Refeeding syndrome was a rare, survivable phenomenon that occurred during hypocaloric nutrition support in participants identified at risk. Independent predictors for refeeding syndrome were starvation and baseline low-serum magnesium concentration. Intravenous carbohydrate infusion prior to artificial nutrition support may have precipitated the onset of the syndrome.

Nutritional factors associated with survival following enteral tube feeding in patients with motor neurone disease.

BACKGROUND Motor neurone disease (MND) is a progressive neurodegenerative disease leading to limb weakness, wasting and respiratory failure. Prolonged poor nutritional intake causes fatigue, weight loss and malnutrition. Consequently, disease progression requires decisions to be made regarding enteral tube feeding. The present study aimed to investigate the survival, nutritional status and complications in patients with MND treated with enteral tube feeding. METHODS A retrospective case note review was performed to identify patients diagnosed with MND who were treated with enteral tube feeding. A total of 159 consecutive cases were identified suitable for analysis. Patients were treated with percutaneous endoscopic gastrostomy (PEG), radiologically inserted gastrostomy (RIG) or nasogastric feeding tube (NGT). Nutritional status was assessed by body mass index (BMI) and % weight loss (% WL). Serious complications arising from tube insertion and prescribed daily energy intake were both recorded. RESULTS Median survival from disease onset was 842 days [interquartile range (IQR) 573-1263]. Median time from disease onset to feeding tube was PEG 521 days (IQR 443-1032), RIG 633 days (IQR 496-1039) and NGT 427 days (IQR 77-781) (P = 0.28). Median survival from tube placement was PEG 200 (IQR 106-546) days, RIG 216 (IQR 83-383) days and NGT 28 (IQR 14-107) days. Survival between gastrostomy and NGT treated patients was significant (P < or = 0.001). Analysis of serious complications by nutritional status was BMI (P = 0.347) and % WL (P = 0.489). CONCLUSIONS Nutritional factors associated with reduced survival were weight loss, malnutrition and severe dysphagia. Serious complications were not related to nutritional status but to method of tube insertion. There was no difference in survival between PEG and RIG treated patients.

Comparison of two percutaneous radiological gastrostomy tubes in the nutritional management of ALS patients.

Patient care and minimizing complications post gastrostomy have to date received little attention in ALS patients. We compare the complications associated with pigtail and mushroom type percutaneous radiological gastrostomy tubes in this patient group. Patients requiring PRG received either Wills‐OglesbyTM or the skin level EntristarTM. Retrospective review of the clinical notes was performed capturing demographic data, peristomal infection, tube displacement, tube failure, nutritional status, site of disease onset, and survival. Thirty‐five patients (Group 1) had the Wills‐OglesbyTM tube of which 14 (40%) tubes required replacement. The EntristarTM tube was inserted in 29 patients (Group 2) where 8 (28%) required replacement (NS). The incidence of infection was significantly lower with the EntristarTM tube, (p<0.001). The mean time to tube removal in Group 2 was 223 days (SD 147; range 71–494 days) due to ‘buried bumper syndrome’. We conclude that the Entristar TM skin level gastrostomy tube is associated with a reduction in peristomal infection, tube failure and blockage compared with the Wills‐OglesbyTM tube.

Clinical guidelines and enteral nutrition support: a survey of dietetic practice in the United Kingdom

Background:Artificial nutrition support is used in treating hospital patients and has been shown to reduce hospital stays. The NICE (National Collaborating Centre for Acute Care) guidelines are the first national consensus guidelines for dietetic practice in artificial nutrition. The aim of the current survey was to explore the influence of local and national guidelines, and clinical experience on enteral tube feeding practices in a large cohort of UK dietitians.Methods:A cross-sectional anonymous online survey of UK registered dietitians was performed.Results:A total of 681 responses were received. In all, 85% deemed ‘clinical experience’ to be of greatest influence when initiating a tube feeding regimen; the influence of ‘clinical experience’ was significantly associated with the number of years in practice (P=<0.001). A total of 70% of respondents were aware of a department feeding protocol with 67% of protocols using a start rate of 24–49 ml/h; furthermore, 65% of respondents reported most commonly using a feeding start rate of 24–49 ml/h and 75% of them reported that their department had a protocol for preventing refeeding syndrome; 23% had mandatory implementation of NICE guidelines.Conclusions:Enteral feeding practice varies among practitioners. Clinical experience and published clinical guidelines have a pivotal role when treating adult patients that require enteral tube feeding.