THEME 9 RESPIRATORY AND NUTRITIONAL MANAGEMENT

P162 DOES TASTE PERCEPTION CHANGE WITH ALS?

PELLETIER C¹, ABOU-ZEID E¹, BARTOSHUK L², FEWELL D¹, RUDNICKI S¹

¹University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States, ²University of Florida, Gainesville, Florida, United States

E-mail address for correspondence: [email protected]

Keywords: ALS, taste, MND

Background: The traditional concept that patients with ALS have disease limited to the motor system has been challenged in recent years. It is well known that ALS patients frequently lose weight, attributed to muscle atrophy as well as dysphagia. However, some subjects also report less interest in food and/or taste changes. It is also known that focal taste damage may not be perceived with whole mouth tasting (as in eating) due to disinhibition of the taste cranial nerves [1], and that aging and gender affect taste perception [2],[3].

Objective: It was hypothesized that focal taste damage may occur in ALS patients compared to healthy controls.

Methods: Two age-gender matched adult groups were recruited (n = 22; 11 ALS and 11 healthy adults). All ALS patients received a clinical swallowing examination prior to participation. The Spatial Taste Test was administered using 1.0M NaCL, 1.0M sucrose, 0.032M citric acid, 0.001M quinine hydrochloride diluted in deionized water [4]. Taste identification, whole mouth and bilateral taste intensity ratings on the fungiform and circumvallate papillae were conducted using the generalized Labeled Magnitude Scale, which has been shown to have validity across groups [5]. Independent 2-sample t-test analyses were performed between groups for all tastes, laterality and papillae including whole mouth taste intensity. P-values ≤0.05 were considered significant.

Results: Compared to healthy age/gender matched volunteers, ALS patients exhibited significantly lower intensities for sweet and sour tastes in the fungiform papillae bilaterally. No significant differences were observed in the circumvallate papillate or whole mouth taste intensities. A trend of lower intensities across all tastes and papillae was observed in ALS patients vs healthy volunteers. Five of the 11 ALS patients reported taste changes; 3/5 had tongue fasciculations.

Discussion: Results of this small pilot study suggest ALS may alter taste perception with focal damage to the chorda tympani which innervates the fungiform papillae and may include the glossopharyngeal which innervates the circumvallate papillae. Although the hypothesis was confirmed, caution must be exercised when interpreting the results due to the small sample size. The mechanism for taste loss or changes with ALS is unknown. It is not known whether abnormal movement of a taste bud due to fasciculations will alter taste perception.

P163 A CARE PATHWAY FOR PEOPLE WITH MOTOR NEURONE DISEASE NEEDING NUTRITIONAL SUPPORT

GIBBS H¹, JOYCE J², FEATHERS L²

¹Leicestershire Nutrition and Dietetic Service, Leicester, ²University Hospitals of Leicester NHS Trust/ LOROS, Leicester, United Kingdom

E-mail address for correspondence: [email protected]

Keywords: nutrition, pathway, gastrostomies

Background: It is widely accepted that for patients with Motor Neurone Disease (MND) with bulbar symptoms, the early siting of an enteral feeding (EF) tube i.e. PEG (percutaneous endoscopic gastrostomy) or RIG (radiologically inserted gastrostomy) optimises care compared to later placement when a patient may be malnourished, dehydrated, or have respiratory insufficiency.

As part of a project to improve the care of people with MND, a multi-disciplinary team (MDT) of professionals examined the care of people needing nutritional support (NS): particularly when and how to broach the subject of NS with the patient; decision making over PEG vs RIG vs NGT (nasogastric tube) placement; and streamlining the process.

Objectives: To devise a care pathway to expedite the processes of referral and assessment for the appropriate EF tube. To improve the quality of life for patients with bulbar symptoms.

Methods: A task and finish group of MDT members (dietitians, nurse specialists in MND, doctors in palliative care, respiratory medicine, gastroenterology and radiology) examined current practice, and a literature search about best practice informed the new pathway. It was then refined as patients used it. Training needs were identified to enhance the assessment of respiratory function pre-procedure and facilitation of NG tube placement in the community. Documentation of timing and type of tube placement, use of tube (i.e. initially for hydration or immediately for total nutritional support) and the post-placement morbidity was recorded on a database. Patients and carers were asked about their experiences.

Results: An evidence-based care pathway has been developed. Prior to this pathway, PEGs were the only EF tube offered. The pathway has allowed the appropriate use of RIG and NG tubes. Measurement of sitting and lying forced vital capacity has provided a more objective assessment to inform decision making on the most appropriate EF tube.

Discussion and Conclusions: This care pathway has drawn together best practice for patients with MND needing nutritional support. We have discussed NS with patients earlier, so that timely, informed decisions can be made, with verbal feedback from patients and carers that this has empowered them and improved quality of life. A DVD resource is being developed to collate patient/carer comments so future patients can gain further insights. Training for the Home Enteral Nutrition Dietitian on NG tube placement for either short-term hydration purposes prior to PEG/RIG placement or to prevent acute hospital admissions at end of life has further improved overall choice. RIG placements are now appropriately offered and the process by which any EF tube is placed has been expedited. This initiative is already being examined by other specialist groups within our trust as an example of good practice.

P164 RESTING ENERGY EXPENDITURE (REE) IN PATIENTS WITH ALS

WINLAND J, SUFIT R, SIDDIQUE T, AJROUD-DRISS S, HELLER S, WOLFE L, LU B, ARMSTRONG J, CASEY P

Northwestern University, Chicago, IL, United States

E-mail address for correspondence: [email protected]

Keywords: metabolism, calorimetry, nutrition

Background: Nutrition is a prognostic factor for survival in patients with ALS. Known factors that contribute to poor nutrition include dysphagia, anorexia, fatigue, and constipation. In addition, research has shown that patients with ALS exhibit an increase in metabolism [1]. Sherman, et al. showed that standard equations are not accurate in determining REE in patients with ALS [2]; therefore, more accurate caloric needs should improve patient care. We propose that the best method to determine energy expenditure in the ALS population is indirect calorimetry.

Objectives: i) To understand better the metabolic requirements and nutritional needs of patients with ALS. ii) To Demonstrate the robustness of indirect calorimetry in ALS patients in an outpatient setting. iii)To Compare REE to gender, age, height/weight/BMI, FFM, FVC, VO2, diet status, ambulatory status, and disease onset.

Methods: 77 patients (43 male/34 females) with ALS (56 spinal/21 bulbar onset) were measured for REE using indirect calorimetry (KORR MetaCheck Metabolic Analyzer Model 7100.) Standard procedures were followed for the MetaCheck, except we used a face mask rather than the provided mouthpiece to ensure adequate seal.

Results, Discussions and Conclusions: 72 of the 77 patients completed the MetaCheck analysis. Five were excluded on account of low tidal volumes. REE was found to be higher in the ambulatory and oral feeding patients as compared to the wheelchair-bound and tube-fed patients. REE decreased as age increased. Patients with lower FVC levels had a lower REE compared to higher FVC levels. Bulbar patients had a lower REE than spinal patients. Indirect calorimetry successfully estimated REE. Different patient populations (e.g. spinal vs. bulbar) require different calorie intake to maintain nutrition.

P165 INDIRECT CALORIMETRY IN THE OUTPATIENT ALS SETTING

WINLAND J, ARMSTRONG J, SUFIT R, SIDDIQUE T, HELLER S, AJROUD-DRISS S, WOLFE L, LU B, CASEY P

Northwestern University, Chicago, IL, United States

E-mail address for correspondence: [email protected]

Keywords: metabolism, indirect calorimetry

Background: Research has shown that patients with ALS exhibit an increase in metabolism [1]. Sherman, et al. showed that standard equations are not accurate in assessing REE in patients with ALS [2]. Therefore, determining accurate caloric needs should improve the standard of patient care.

Indirect calorimetry is considered the only accurate and clinically feasible method of measuring energy expenditure. We propose that the best method to determine energy expenditure is indirect calorimetry and can be a routine test in the outpatient ALS clinic.

Objectives: Demonstrate the use of indirect calorimetry in ALS patients in an outpatient setting. Obtain accurate results with good compliance to the test procedures. Determine whether indirect calorimetry is cost effective, patient friendly, as well as easy for staff to use in the outpatient clinic.

Methods: 96 patients (53 male/43 females) with ALS were measured for REE using indirect calorimetry (KORR MetaCheck Metabolic Analyzer Model 7100.)

All patients were instructed to fast for 4 hours, avoid all caffeinated beverages, and avoid exercise on the day of the Metacheck. Patients were seated at rest for 15 minutes prior to the procedure. The test was adapted to use a face mask rather than the provided mouthpiece to ensure an adequate mouth seal. The testing time is 10 minutes of breathing at a normal rate through the face mask.

Results, Discussions and Conclusions: Goal of the clinic is to obtain a Metacheck for every patient. This would require all patients to fast for 4 hours prior to their clinic visit. Initial clinics proved this to be too difficult due to the patient load and prolonged fasting period. Standard procedures are being developed for easier patient compliance and improved patient comfort. This would include scheduled Metacheck times for pre-determined patients at different intervals during clinic. Team members were surveyed in the multidisciplinary clinic on the use of the Metacheck. Feedback revealed that the Metacheck did not impair others from seeing patients or prolong their visit time. The Metacheck was determined to be easy to use, required no special training for operation, and cost effective. Outcomes showed good compliance with the simple 10 minute test and the minimum 4 hour fasting period. 90 of the 96 patients were able to complete the MetaCheck analysis. Tidal volumes were too low to register in 5 patients and 1 patient was too uncomfortable to complete the test.

P166 MODELING ENERGY EXPENDITURE IN ALS PATIENTS

MENDIONDO MS, KRYSCIO RJ, KASARSKIS EJ, TANDAN R, MATHEWS DE, POTTER C, WELLS S, ALS NUTRITION/NIPPV STUDY TEAM

University of Kentucky, Lexington, KY, United States

E-mail address for correspondence: [email protected]

Keywords: total daily energy expenditure, prediction equations, modelling

Background: A clinical trial of 80 ALS patients underwent a protocol using doubly labelled water (DLW) to determine total daily energy expenditure (TDEE). DLW measurements were taken at days 1, 7 and 10. In addition, on each of these days measurements of fat free mass were obtained by both DEXA and BIS. On days 7–10, detailed dietary information was taken in addition to measures of physical activity determined by Actical accelerometers.

Objective: Determine how well established TDEE formulas predict actual TDEE values obtained from DLW measurements in ALS patients and to assess how to improve the predictive ability of such formulas by the inclusion of patient demographics, fat free mass, forced vital capacity measurements, ALS-FRS baseline score, and ambulatory status predict TDEE.

Methods: Multiple regression models were used to make the predictions. Various models based on the Harris Benedict equation [1], the Wang equation [2], the Columbia equation, and the Vermont equation were used as starting points. Models were supplemented by adding in patient demographics, forced vital capacity measurements (erect and supine), ALS-FRS score, and ambulatory status.

Results: These patients are 35% female, have a mean age of 59±11.7 years, and a mean of 14.26±2.88 years of education. At baseline, these patients had a mean ALS-FRS of 36.1±5.82 and mean%FVC of 76.13±15.85. The mean time from disease onset to entry in the trial was 23.8±19.67 months, and 69.6% had some ambulatory deficiency. Baseline TDEE measurements ranged from 1199 to 3614 kcal/day with a mean of 2276±600. For the 63 participants with available DLW results, all of the above prediction equations underestimate the true energy expenditure by 689, 957 calories/day at baseline.

Discussion: TDEE as measured by DLW is substantially higher than the values predicted by the established formulas, and suggests that ALS patients may expend many more calories than standard methods of estimation would indicate. Since maintaining the appropriate energy balance is important for ALS patients, models that accurately predict TDEE need to be developed for this special population.

P167 DIETARY INTAKE OF PATIENTS WITH MOTOR NEURONE DISEASE /AMYOTROPHIC LATERAL SCLEROSIS (ALS)

CELIS LEME TORINO V, STANICH P, SALVIONI C, YAMAMOTO UE, CHIEIA MA, OLIVEIRA SB, ACARY SB

¹Federal University of Sao Paulo, São Paulo, Brazil, ²Brazilian Association of Lateral Sclerosis Amiotrófica-ABRELA, São Paulo, Brazil

E-mail address for correspondence: [email protected]

Keywords: dietary,fibers,fats

Introduction: Motor Neuron Disease (ALS) represents a group of neurodegenerative disorders caused by impairment of motor neurons, leading to progressive muscle weakness and muscle atrophy. Respiratory failure, dysphagia and body composition alterations are present in patients, especially in those with bulbar involvement.

Objective: The purpose of this study is to analyze the dietary intake of patients with MND/ALS.

Method: The diet of 44 ALS patients was analyzed, from May/07 to November/07 through ALS patients recalling their food intake over a 24 hour period to the nutritional services. Support to the Nutrition Data System version 2.5a- DIS- UNIFESP/EPM software, was used in this study. A nutritional classification was performed by the body mass index (BMI) by WHO (1989). For patients who had a BMI below 20.9m²/kg, the adjustment for women was to 21kg m²/kg and for men, 22 m²/kg. For the patients that had BMI above 25 m²/kg, this value was adjusted to 24.9 m²/kg. The basal metabolism was calculate by Harris Benedict equation (1919) and was accreted by the factor of 1.4, to find the total energy necessary. The dietary fiber was adjusted by using the FDA (Food Drugs Administration). For the fat percentage, the range of 25–30% of total caloric value and the relationship of 1:1 saturated fat and unsaturated was used. The reference to the percentage of saturated fat is 10% of lipids totals. The reference standard for water intake (expected) was the consumption of 35 ml/kg/day. The classification of the intestinal function was normal or constipated.

Results and Discussion: The average of patients′ age was 57 years old; there were 23 male and 21 female patients. As for the nutritional status 8 patients (18.18%) showed under nutrition, 3 patients (6.81%) severe degree III and 5 (11.36%) degree I, 25 (56.81%) had atrophy in 11 (25%) showed excess weight, and 10 (22.72%) with overweight and 1 (2.27%) as Obesity degree I. Patients of both sexes reporting the caloric intake values of 1.888 Kcal, when the recommended value was 1.954 Kcal. The average fiber rate in the diet, for both sexes, was 12 grams in 1888 calories below the recommended; 62% of the patients presented with constipation. The fat total value present in the diet, for both sexes, exceeded the upper limit of 30%, presenting 36%. The saturated fats proportion in the diet had the average of 1:1,8 above the benchmark. The percentage of saturated fats on the total lipids in the diet, for males was, on average, by 10% and for females was 24%. As for the liquid intake there was an average of 911 ml, and the recommended average is 2131 ml, showing the patients consumed only 43% of the necessary.

Conclusion: The dietary consumption by patients with MND/ALS showed that the energy consumed in the average is below needs, is low in fiber, high in fat and low liquid consumption, resulting in constipation and conditions favourable to inflammatory processes by the high intake of saturated fats. Patients are atrophic, in the majority, with similar parts of undernourished and overweight.

P168 CORRELATION AMONG BODY MASS INDEX AND SURVIVAL AFTER PEG INSERTION IN AMYOTROPHIC LATERAL SCLEROSIS PATIENTS

NARDI K¹, BONGIOANNI P², EVANGELISTI I¹, RIVI P¹, AZADEGAN M¹, TUCCIO MC², PEPE G², ROSSI B²

¹Nutritional Service, Azienda Ospedaliero-Universitaria Pisana, ²Neurorehabilitation Unit, Neuroscience Department, Azienda Ospedaliero-Universitaria Pisana; NeuroCare-onlus, Pisa, Italy,

E-mail address for correspondence: [email protected]

Keywords: BMI, PEG, survival

Background: Patients suffering from amyotrophic lateral sclerosis (ALS) often have to face progressive nutritional problems leading at last to percutaneous endoscopic gastrostomy (PEG) placement. The body mass index (BMI), or Quetelet index, is a statistical measure of person's weight scaled according to height: BMI is defined as the individual's body weight divided by the square of height.

Methods: We evaluated 14 spinal-onset or bulbar-onset ALS patients (5 men and 9 women; mean age±SD: 63±11 yrs) with PEG. Many data (BMI, phase angle and other soft tissue analysis parameters) were collected just before PEG insertion and correlated with survival after PEG insertion. Patients were divided into 3 groups: A group with BMI ≤ 18.5; B group with BMI between 18.5 and 25; and C group with BMI ≥ 25.

Results: By comparing A (mean survival (days)±SD: 197±272; mean resistance±SD: 816±154; mean reactance±SD: 58±14; mean phase angle±SD: 4.3±1.8) with B (mean survival (days)±SD: 370±294; mean resistance±SD: 656±115; mean reactance±SD: 54±11; mean phase angle±SD: 4.9±1.1), or C group (mean survival (days)±SD: 392±248; mean resistance±SD: 545±60; mean reactance±SD: 55±2; mean phase angle±SD: 5.9±0.9), we found significantly (p < 0.05) enhanced survival values in B and C groups as compared to A group; whereas B and C groups did not significantly differ from each other.

Discussion and Conclusions: Our results support the concept that early PEG placement could enhance survival in ALS patients.

P169 PERCUTANEUS ENDOSCOPIC GASTROSTOMY IN PATIENTS WITH MOTOR NEURONE DISEASE/ AMYOTROPHIC LATERAL SCLEROSIS

STANICH P, SHIGUEO NAKAO F, SALVIONI C, CELIS LEME TORINO V, DA SILVEIRA ROHR MR, QUADROS A, DELLA LIBERA E, BERTO HAYASHI MC, FERRARI ÂNGELO P, GEOCZE S, SOUZA BULLE OA

University Federal of São Paulo, São Paulo, Brazil

E-mail address for correspondence: [email protected]

Keywords: Percutaneus Endoscopic Gastrostomy, nutritional status, survival

Background:Amyotrophic Lateral Sclerosis (ALS) is a degenerative, progressive and irreversible neurological disease, that affects motor neurons. Among the symptoms, we have reduction of forced vital capacity (FVC), dysphagia, weight loss and compromised nutritional status. Nutritional and respiratory support is the most important therapeutic interventions, with use of BiPAP and Percutaneus Endoscopic Gastrostomy (PEG) (a technique developed in 1980 as an alternative to surgical gastrostomy). It is suggested in patients with high surgical risk, and it can be done in outpatient department, under sedation and with decrease of morbility and absence of complications in more than 95% of cases.

Objective:To describe the evolution of nutritional status after PEG and its relation with survival.

Methods: From February/2000 to May/2007, 111 patients with ALS by El Escorial modified criteria were enrolled in a prospective non-contestant study. PEG was performed in these patients when a modified criterion from the proposed ones by Silani in 2000 was observed. Sedatives were midazolam 0.035 to 0.07mg/kg and fentanil 0.05 to 0.1 mg/kg. We evaluated nutritional status by body mass index (BMI) before and after 30 days of PEG insertion. FVC was measured before PEG insertion and BiPAP was used by Kleopa et al criteria.

Results: Mean age was 58.3 years (11.7); there were 39 males (66.1%) and 20 females (33.9%) with ALS and 18 males (34.6%) and 34 females (65.4%) with bulbar form. Before PEG insertion, classification of patients’ nutritional status was: 51.3% atrophy, 22.5% overweight and 26.2% malnutrition. FVC at the moment of PEG insertion varied between 13% and 86%, with mean of 41% (11.91). Good BiPAP tolerance was observed in 42.6% of patients, poor BiPAP tolerance in 31.7% and patients without BiPAP despite necessity were 16.8%. After PEG insertion nutritional status was: 49.5% was atrophy, 20.7% overweight and 29.7% malnutrition. Patients with bulbar form had high predominance of disturbance. Mean survival in ALS was 2190 days from the beginning of the first symptoms (IC 95%: 1226.0–3154.0), and in the bulbar form it was 1260 days (IC 95%: 904.5–1615.5) (p = 0.009, for the Long Rank Test). Death occurs usually by respiratory failure. Three patients died in the first 30 days after PEG insertion, all with FVC from 47% to 54%. Though the PEG insertion in some cases with FVC below the recommended one, in these patients the mortality was low in the first 30 days.

Conclusions: 1) Mortality probably is not related to PEG insertion; 2) PEG can be used even in patients with serious respiratory failure; 3) PEG allows improvement of nutritional status; 4) Despite nutritional support, patients with bulbar form have less survival, perhaps by higher level of respiratory failure and aspirative pneumonia.

P170 NON-INVASIVE MEASUREMENT OF MOUTH TO COLON TRANSIT TIME IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS (ALS)

RUSSO G, CHEN J, SAADIQ R, BARR C, DEBOO A, HEIMAN-PATTERSON T

Drexel University College of Medicine, Philadelphia, PA, United States

E-mail address for correspondence: [email protected]

Keywords: Amyotrophic Lateral Sclerosis, Autonomic, Gastrointestinal motility

Background: Amyotrophic Lateral Sclerosis (ALS) is generally considered a motor system disease. However, recent evidence exists for autonomic involvement outside of the motor system including cardiac, sudomotor and gastrointestinal (GI). While there have been studies demonstrating colonic involvement, small bowel motility has not been investigated [1]. GI complications are especially important given the frequent complaints of constipation and abdominal distention, the use of medications with anticholinergic side-effects, and the management of nutritional needs through the use of tube feedings.

Objective: To examine autonomic involvement of the small bowel in ALS by quantifying gastric motility in patients with ALS compared to healthy controls.

Methods: GI motility of the small bowel was evaluated using the mouth to colon transit time (MCTT) or gastric to colon transit time (GCTT) measured via the noninvasive hydrogen breath technique [2]. Seven healthy controls and six patients diagnosed with probable or definite ALS were examined. Patients were instructed to hold their medicines from the previous night. After an 8 hour fast, the baseline hydrogen content of their expired air was measured. This was followed by the administration of a measured caloric load containing 20 grams of lactulose. Hydrogen breath measurements were taken every ten minutes until there were two consecutive increases in hydrogen gas concentration, indicating the bolus had reached the colon. The time of the second increase in hydrogen gas concentration was recorded as the MCTT or GCTT.

Results: The mean MCTT/GCTT in individuals with ALS was significantly prolonged compared to controls (121 vs. 74 minutes, p = 0.002). MCTT/GCTT increased as ALS-FRS-R scores decreased (R= -0.72) indicating progressing small bowel involvement as the disease worsens.

Conclusions: These results indicate autonomic involvement of the small bowel that may increase as the disease progresses, likely contributing to some of the symptoms observed in patients with ALS. This has importance in the management of tube feedings and the use of medications that can further alter GI motility.

P171 UNSEDATED PERCUTANEOUS ENDSCOPIC GASTROSTOMY USING AN ULTRATHIN TRANSNASAL ENDOSCOPE FOR AMYOTROPHIC LATERAL SCLEROSIS WITH RESPIRATORY FAILURE

NONAKA M¹, YAMAUCHI R¹, ODA M¹, TOYOSHIMA T¹, TAKAHASHI N², HISAHARA S¹, IMAI T¹, SHIMOHAMA S¹

¹Department of Neurology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan, ²Department of Nursing, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan

E-mail address for correspondence: [email protected]

Keywords: PEG, transnasal endoscope, respiratory failure

Background: In amyotrophic lateral sclerosis (ALS) with dysphagia, percutaneous endoscopic gastrostomy (PEG) provides a survival benefit, and improves quality of life. However, due to the potential hazard of PEG for ALS with respiratory muscle weakness, PEG is recommended when the percent predicted forced vital capacity (%FVC) exceeds 50%. On the other hand, many patients who require PEG have already lowered respiratory muscle strength with FVC below 50%. A safe PEG method that can be used in patients with respiratory failure is required.

Objectives: Conventionally PEG is performed by the Pull method using a perioral endoscope. The Direct method using an ultrathin transnasal endoscope is less invasive and has no need for conscious sedation. We evaluated the safety and usefulness of this method in ALS patients with dysphagia.

Methods: Five ALS patients with dysphagia were studied. The nasal route was prepared before the procedure by applying a topical vasoconstrictor (naphazoline nitrate) and local anaesthetic (lidocaine hydrochloride). The GIF-XP260N endoscope (Olympus Optical Co.), which had a distal-end diameter of 5.0 mm, was inserted transnasally without conscious sedation. After air insufflation into the stomach, two-point fixation was conducted using the Funada's gastropexy device (Create Medic Co.). A button type catheter was inserted by the Direct method using a Kangaroo Seldinger PEG kit (Nippon Sherwood Medical Industries Co.).

Results: All 5 procedures were successful. The average duration of the procedure was approximately 15 min. The procedures were well tolerated by all the patients, with none requiring any sedation. There was no major complication, with no lowering of oxygen saturation and no abnormal increase of pulse rate. There was little pain associated with transnasal endoscope insertion and conversation was possible during the procedure. No peristomal infection was observed and sutures were removed after 1 week. In a patient who was receiving non-invasive ventilation (NIV), the procedure was conducted safely with simultaneous NIV support using a nasal mask with a channel created for the endoscope.

Discussion: The Direct method applying the Seldinger technique is an improved Introducer method. With the Direct method, the role of the endoscope is only for insufflations of air into the stomach, and observation of the procedure. There is no need for peroral insertion of a conventional endoscope. Recently developed ultrathin transnasal endoscopes are very fine, with external diameter of 5.0 mm. Therefore, conscious sedation is not required during the procedure. This point is very important for PEG in ALS patients with respiratory failure. Furthermore pharyngeal anesthesia is not needed, and the risk of aspiration of saliva or gastric fluid is reduced.

Conclusions: The Direct method using an ultrathin transnasal endoscope without sedation is less invasive and less painful than the Pull method using a peroral endoscope. We recommend using this safe method in PEG for ALS patients with respiratory failure.

P172 APPLICATION OF NON-INVASIVE VENTILATION DURING PERCUTANEOUS RADIOLOGIC GASTROSTOMY IN ALS PATIENTS WITH LOW VITAL CAPACITY

KANG SW¹, PARK JH¹, CHOI YC¹, IM SH², HAN SJ³

¹Yonsei University College of Medicine, Seoul, Republic of Korea, ²Cheju National University College of Medicine, Cheju, Republic of Korea, ³Ewha Womans University College of Medicine, Seoul, Republic of Korea

E-mail address for correspondence: [email protected]

Keywords: PRG, NIPPV, Nutrition

Background: Nutritional aspect in ALS patients is important in their survival and quality of lives. Percutaneous endoscopic gastrostomy (PEG) is recommended when forced vital capacity (FVC) in seated erect position is 50% of predicted value or greater. In general, PEG is a relatively safe procedure, but the morbidity and mortality rise as the FVC falls. When performing PEG, mild sedation is needed, which might be a hazard of respiratory compromise in patients with a FVC < 50% and there is a risk of aspiration as the pharynx is transiently anesthetised. Some studies reported successful PEG placement in ALS patients whose predicted FVC < 50% using noninvasive positive pressure ventilation (NIPPV) support by nasal mask during PEG procedure. But, respiratory compromise, insufficient ventilation and secretion care during procedure are unsolved problems. Percutaneous radiologic gastrostomy (PRG) is a good alternative method as it does not need sedation and an endoscopic tube, but, performing PRG to the patients with very low FCV is also a great burden to interventional radiologists.

Objectives: We describe the concomitant use of NIPPV during PRG placement in ALS patients with low FVC and discuss the safety and feasibility.

Methods: Twenty-five ALS patients (13 men, 12 women; mean age 55.5 years; range; 40–73 years) who had dysphagia and using NIPPV were recruited. Their pulmonary function tests were performed. During the PRG procedure, the patients used their NIPPV via a nasal mask. For pre-medication, no sedatives and narcotics were used. Under fluoroscopic guidance, PRG tube placement was performed by an interventional radiologist using gastropexy with 1∼2 T-fastener.

Results: The mean FVC in intermittent NIPPV using group (n = 16) was 1408.8±694.3 ml (35.9±17.7% of predicted value) in sitting position, and 1074.4±469.2 ml (27.3±11.1%) in supine. The mean PCF was 175.7±37.3 L/min. The FVC and PCF could not be evaluated in 24 hours NIPPV using group (n = 9) because the patients rejected to be weaned from their ventilators. The PRG placement showed 100% success rate technically. There were no major complication such as aspiration, panperitonitis, and respiratory arrest. However, several minor complications happened: pnemoperitoneum (4%), peri-gastrostomy tube leakage (8%), skin irritation and suture breakage (36%), and would infection (8%). Mean survival of 25 ALS patients was 32.1 months (Keplan-Meier method, SE, 5.0, median 20.0 months). There was no difference of survival between intermittent NIPPV using group and 24 hour NIPPV using group (log-rank test, p = 0.82).

Discussion and Conclusions: Application NIPPV during PRG is successful and safe method for nutritional care of ALS Patients with low FVC.

P173 AN INVESTIGATION FOR THE USE OF NON-INVASIVE POSITIVE PRESSURE VENTILATION AND PERCUTANEOUS ENDOSCOPIC GASTROSTOMY IN CHINESE ALS PATIENTS

WANG L, ZHANG X, ZHANG N, ZHANG J, FAN D

Department of Neurology, Peking University Third Hospital, Beijing, China

E-mail address for correspondence: [email protected]

Keywords: NIPPV, PEG, respiratory & nutritional managements

Backgrounds: In China, a few patients with amyotrophic lateral sclerosis (ALS) can get effective respiratory and nutritional managements in their whole disease course.

Objectives: To investigate the use of non-invasive positive pressure ventilation (NIPPV) and percutaneous endoscopic gastrostomy (PEG) in Chinese ALS patients, and analyze the potential influential factors of their current status.

Methods: Total 472 Chinese patients with ALS diagnosed from 01-01-2005 to 31-12-2007 were investigated using a specially designed questionnaire which contained 24 issues pertaining to information about the use of NIPPV and PEG.

Results: The overall percentage of patients presently using NIPPV and PEG was 16.3% and 4.4%, respectively. The percentages of use of NIPPV and PEG year by year were 3.7% and 1.9% in 2005, 10.0% and 1.7% in 2006, and 21.1% and 6.0% in 2007, respectively. Among the patients who received NIPPV therapy, the time from symptom onset to beginning of use was 0–1 years (20/77, 26.0%), 1–2 years (26/77, 33.8%), 2–3 years (14/77, 18.2%), and beyond 3 years (17/77, 22.0%), respectively. 76.2% of the patients who received PEG were operated in 3 years. The main reasons impelling ALS patients to use NIPPV included doctors’ advice (96.1%), propagating brochures (67.5%), communication among patients (31.2%), persisting dyspnea (30.0%) and education meeting (15.6%). In contrast, the main causes obstructing the patients to choice NIPPV were lack of doctors’ advice (47.3%), poor income (45.8%) and incomprehension to NIPPV (45.3%). Also, the main factors that made patients receive PEG were related to doctors’ advice (90.5%), dysphagia (71.4%) and propagating brochures (47.6%). The key points to obstruct patients from PEG were absence of doctors’ advices (50.6%), incomprehension to PEG (39.5%), dread of operation (39.5%), and thought of the inconvenience caused post-PEG (39.2%).

Conclusions: The percentages of use of NIPPV and PEG in ALS patients in China were much lower than those of patients with ALS in developed countries, but the proportion has been gradually promoting in recent years. The main influential factors to extend NIPPV and PEG in Chinese patients were doctors’ advice and patient education. The restrictive ones include absence of doctors’ advice, lack of knowledge about NIPPV and PEG, and lower level of income.

P174 INDICATORS OF EARLY RESPIRATORY FAILURE IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS

YAMAUCHI R, NONAKA M, HISAHARA S, IMAI T, SHIMOHAMA S

Sapporo Medical Univercity, Sapporo, Hokkaido, Japan

E-mail address for correspondence: [email protected]

Keywords: SNIF, diaphragmatic compound muscle action potential, NIV

Background: It has been shown that non-invasive positive pressure ventilation (NIV) may be effective in improving prognosis and maintaining QOL in patients with amyotrophic lateral sclerosis (ALS). However, it is difficult to determine the optimal timing of NIV initiation because the modes of onset and progression vary widely in ALS.

Objectives: The aims of this study were: (1) to elucidate a possible role of sniff nasal-inspiratory force (SNIF), diaphragmatic compound muscle action potential (DCMAP) and conventional pulmonary function parameters as indicators of early respiratory failure in patients with ALS; (2) to establish an appropriate protocol to predict the optimal timing of NIV initiation.

Methods: 30 patients who were diagnosed as probable ALS (El Escorial criteria) were subdivided into 4 groups according to the site of onset as follows: group A; upper limb onset, group B; lower limb onset, group C; bulbar onset, and group D; respiratory muscle onset. Diaphragmatic functions were evaluated using SNIF measurements and DCMAP recordings by electrical phrenic nerve stimulation at the neck. Blood gas analysis, forced vital capacity (%FVC), DCMAP, chest XP at inspiration/expiration, and SNIF were measured every four months for two years.

Results: There were 16 patients (53.3%) in group A, 6 patients (20.0%) in group B, 7 patients (23.3%) in group C, and 1 patient (3.3%) in group D. In group A (upper limb onset), onset was on the right in 13 patients (81.3%), on the left in 3 patients (18.8%). In group B (lower limb onset), onset was on the right in 3 patients (50.0%), on the left in 2 patients (33.3%), and bilateral in 1 patient (16.7%). The mean (range) disease duration was 38.5 (16–101) months in group A, 44.7 (13–122) months in group B, 23.9 (10–48) months in group C, and the duration was 17 months in group D. When patients had common cold symptoms or intermittent subjective dyspnea, the abnormal values were detected in the following order: SNIF > DCMAP =%FVC > diaphragm movement on chest XP > blood gas analysis. There were some patients who did not perceive dyspnea even though diaphragm movement had disappeared. NIV was initiated during the study period in 9 patients in group A (4 patients for 24 h and 5 patients only at night time), 1 patient in group B (for 24 h), and 1 patient in group D (for 24 h).

Discussion and Conclusions: Previous reports have already shown that%FVC is inadequate as the criterion of initiating NIV. In the present study, abnormalities in SNIF were detected at the early stage of respiratory failure, indicating that they may serve as indicators of NIV initiation.

P175 TRANSCUTANEOUS PCO₂ MEASUREMENT IN ALS

NAKAMURA Y, MORITA M, NAKANO I

Jichi medical University, Yakushiji, Shimotsuke-shi, Tochigi-ken, Japan

E-mail address for correspondence: [email protected]

Keywords: transcutaneous PCO²,

Background: Respiratory insufficiency is critical for ALS patients, and spirography is usually used as the main method to investigate the respiratory function. It cannot, however, be applied so frequently, and sometimes fails to provide us with reliable data for accurate estimation of the breathing status in ALS patients with bulbar symptoms or elder patients.

Objective: An evaluation of the usefulness of transcutaneous PCO₂ (tcPCO₂) analysis for the assessment of respiratory function in ALS patients.

Methods: Nine patients with ALS were investigated. We measured tcPCO₂ and SaO₂ for 8 hours while the patients were asleep at night, and the examinations were repeated several times for each patient. Spirography and arterial blood gas analysis were also performed.

Results: Mean tcPCO₂ tended to increase with disease progression in 7 patients, and the fluctuation patterns of tcPCO₂ well corresponded to PaCO₂ on arterial blood gas analysis. In one patient on NIPPV, tcPCO₂ and SaO₂ improved after NIPPV was started. In the other patients, mean SaO₂ deteriorated, while mean tcPCO₂ improved.

Conclusions: With some dissociation between tcPCO₂ and SaO₂, tcPCO₂ seemed to generally reflect the respiratory functions in the ALS patients. As tcPCO₂ examination can be performed noninvasively for ALS patients in any condition, it seems to be a useful method to complement spirometric examination.

P176 EFFECTIVENESS OF MONITORING SNIFF NASAL INSPIRATORY PRESSURE (SNIP) AND TRANS CUTANEOUS PCO₂ TO FORESEE PROGNOSIS OF PATIENTS WITH ALS

OGINO Y¹, OGINO M², KAMIDE N³, SUMIDA S³, SAKAI F²

¹Toshiba Rinkan Hospital, Sagamihara, ²Kitasato University School of Medicine Department of Neurology, Sagamihara,, ³Kitasato University East Hospital, Sagamihara, Kanagawa, Japan

E-mail address for correspondence: [email protected]

Keywords: SNIP, transcutaneous PCO² monitor, predict of prognosis of ALS

Background: Forced vital capacity (%FVC) is considered as the standard method to evaluate respiratory function of ALS patients. However, as the disease advances, technical difficulties increase to make it unreliable. We reported the effectiveness to monitor SNIP to foresee the ALS prognosis at this symposium last year. Meantime, trans cutaneous PCO₂ monitor has become available which is likely to serve the purpose.

Objectives: In this study we aim to [1] investigate effectiveness to monitor Trans cutaneous PCO₂ (TcPCO2) to foresee the prognosis, and [2] to compare SNIP and TcPCO₂ with the longitudinal observation.

Methods: 36 ALS patients were enrolled in the present study, whose SNIP was evaluated every 2∼3 months. Plug was inserted into nostril while contralateral nose was not occluded (opening nose method). In case of patients whose SNIP less than 30 cmH₂O, it was done while contralateral nose was occluded (occluding nose method). 6 healthy volunteers and 11 ALS patients were involved with TcPCO₂ monitor.

Results: During the study period, 11 patients had to be permanently ventilated (TPPV) or died. The logistic regression model shows that only average decrease of SNIP value (monthly decrease value /baseline SNIP) and baseline SNIP value contributed to poor prognosis (death or TPPV management) significantly whereas age, time since the onset and bulbar symptom did not. In event the baseline SNIP goes down less than 26.5 cmH₂O (81.8% for positive rate and 65.4% for negative rate) and/or the changes of SNIP value/month takes place to go down more than 7.05%,( 72.7% for positive and 69.2% for negative) death or permanent ventilation results in most cases. (Kaplan-Meier survival curve and log rank test). TcPCO₂ suggests an increase of nocturnal pCO₂ in most cases who have low SNIP value. In a single case, SNIP is in normal range as well as daytime pCO₂ is normal but TcPCO₂ revealed CO₂ retentions in night time.

Discussion and Conclusion: Monitoring the baseline SNIP and changes of SNIP /month is the effective way to foresee prognosis. TcPCO2 is possibly more accurate to foresee prognosis.

P177 APPLICATION OF POLYSOMNOGRAPHIC MONITORING IN PATIENTS WITH ALS

ZHANG Y, SHEN Y, GAO F, YANG Q, ZHANG N, ZHANG J, FAN D

Department of Neurology, Peking University Third Hospital, Beijing, China

E-mail address for correspondence: [email protected]

Keywords: polysomnograms, oxygen desaturation, Sleep

Objectives: To evaluate the application of polysomnograms (PSG) in patients with ALS.

Methods: Twenty-one cases of ALS patients (9 females, 12 males), with age range of 24 to 78 years old (50.2±10.8 years), were carried out PSG monitoring. The patients were divided into 1) bulbar group (9/21) and 2) limb group (12/21) according to the site of onset. The disease duration from the onset to the time of PSG ranged from 3 to 69 months (20.6±17.9). PSG parameters included the maximum and minimum oxygen saturation in stages of NREM and REM. Sleep-related oxygen desaturation was determined by a dip in SpO₂ by more than 4% from the overnight baseline value. Polygraphic recording includes EEG (C3/A2, C4/A2), electrooculogram, chin and leg electromyogram. The respiration was monitored and measured by oronasal thermistors, uncalibrated inductive plethysmography indicating thoracic and abdominal efforts, finger pulse oximetry, and snoring sounds through a calibrated microphone.

Results: The results of arterial blood gases, pulmonary function tests, and sleep-related oxygen desaturation which was determined by a dip in SpO₂ by more than 4% from the overnight baseline value were classified and analyzed according to the site of onset of ALS. It showed that: 1) 3 patients had abnormal arterial blood gases in 21 patients (14.3%), the bulbar group had 1 patient (11.1%) and the limb group had 2 patients (16.7%). 2) 7 patients had their oxygen desaturation dropped more than 4% (33.3%) during the NREM sleep, among whom the bulbar group had 2 patients (22.2%) and the limb group had 5 patients (41.4%). 3) 11 patients had their oxygen desaturation dropped more than 4% (52.4%) during the REM sleep, among whom the bulbar group had 4 patients (44.4%) and the limb group had 7 patients (58.3%).

Conclusions: Our preliminary results showed that PSG could detect the ventilation insufficiency happened in the absence of any symptoms of respiratory difficulty and abnormal arterial blood gases in ALS patients. Since it is suggested that abnormal response of motor neurons to hypoxia may participate in pathogenesis of ALS, initiation of respiratory care at an adequately early stage might improve the survival and the quality of daily life in ALS patients. Therefore, PSG should be included in a routine evaluation for ALS patients at an early stage to predict respiratory impairment.

P178 HELPING PATIENTS TO DECIDE ON THE BORDER OF LIFE: A VIDEO FILM ABOUT RESPIRATION, NONINVASIVE AND INVASIVE VENTILATION FOR ALS PATIENTS

WINTERHOLLER M¹, HECHT MJ²

¹Department of Neurology, Krankanhaus Rummelsberg, and University of Erlangen, Centre for Neuromuscular Diseases, Schwarzenbruck and Erlangen, Germany, ²Department of Neurology, Bezirkskrankenhaus Kaufbeuren, and University of Erlangen, Centre for Neuromuscular Diseases, Kaufbeuren and Erlangen, Germany

E-mail address for correspondence: [email protected]

Keywords: ventilation, decision, video

Background: In ALS, respiratory deterioration can be treated by different methods: Non invasive ventilation (NIV), invasive ventilation via tracheotomy or palliative care with oxygen. Patient's autonomy should be supported by shared decision making. However, respiratory physiology and different types of ventilation support are difficult to explain to patients. We hypothesized that the additional use of a video clip may help to inform ALS patients better without relevant increase of anxiety.

Patients and Methods: We developed a 23 minute video clip explaining physiology and pathophysiology of respiration, non-invasive and invasive ventilation as well as palliative care in ALS. Film production was done by professionals. We tested the patients knowledge about ALS, respiratory failure and therapy a 25 item questionnaire which was answered before (Q1) and after (Q2) watching the movie. After the movie, we added questions about patients opinion about the video and about the probable influence of the video on the patient's decision.

Results: 55 ALS patients (mean age 56 (36–84) years) watched the video clip and completed Q1 and Q2. Knowledge about ALS and the management and effects of NIV and tracheotomy was improved in 49 patients. The remaining 6 patients were well informed before and completed Q1 without mistake. All patients judged the video clip to be informative or very informative. 8 of 55 patients felt anxious or fearful after seeing the video clip. Younger patients tended to experience the video more informative and less frightening. 30 of 55 patients thought the video to influence their further decision making. The majority of patients recommended to include the video clip into informed decision making about NIV, tracheotomy and palliative care in ALS.

Conclusion: Video supported information seems to be helpful for ALS patients to decide about the treatment of respiratory deterioration and terminal care. We propose to include such media into clinical practice in ALS.

P179 PERSPECTIVES OF PATIENTS WITH ALS ON THE IMPACT OF LUNG VOLUME RECRUITMENT THERAPY ON THEIR HEALTH AND QUALITY OF LIFE

CLEARY S¹, WHEELER S², KENDALL S¹, KALRA S¹, JOHNSTON W¹

¹University of Alberta, Edmonton, ²Caritas Health Group, Edmonton, Alberta, Canada

E-mail address for correspondence: [email protected]

Keywords: rehabilitation, respiratory management, quality of life

Background: Lung volume recruitment (LVR) is a manual insufflation and cough augmentation technique used to help patients with ALS clear secretions. Although clinicians report positive results of LVR in terms of increasing lung volumes and improving cough effectiveness, limited disease-specific research data are available to support its use [1] and LVR has become a standard practice in several ALS clinics in Canada. In addition to the need for quantitative data on physiological outcomes, data on patient perspectives on the technique are necessary to inform clinical practice and quality of care.

Objectives: The purpose of this study was to examine patients’ perspectives on the impact of LVR therapy on respiratory function and personal well-being utilizing a multi-item self-report questionnaire and a semi-structured interview.

Methods: Ten patients (8 with ALS and 2 with other motor neuron disorders) who had been doing LVR therapy for an average of seven months (SD= 5.3 months). The ALSFRS-R and the SWAL-QOL were used to characterize participants’ functional status and quality of life related to swallowing and airway protection. Participants completed a 14-item, five-point Likert scale questionnaire related to airway protection and respiratory status. They also answered interview questions about their experiences with LVR. Questionnaire data were analyzed descriptive quantitative methods. Interviews were recorded, transcribed, and analysed using QSR Nvivo7 software, while following an interpretive description approach.

Results: Questionnaires: The majority of respondents (n = 10) agreed that LVR helped them to clear thick and thin secretions from their throat (77.8%). A large percentage of respondents (55.6%) felt that LVR helped them to keep their airway clear, to feel less anxious about their breathing, and to manage excess secretions. Five of the nine respondents indicated a positive change in cough strength after LVR, while 4 of the nine respondents indicated a positive change in voice quality after LVR. Interviews: Several themes emerged from the interview data and were consistent with questionnaire responses. Positive perceptions of LVR on physiological function were reported by n = 6, many of whom described their experiences in terms such as “good” or “excellent” and described improvements in secretion clearance and increased cough strength after LVR. Several participants (n = 4) reported feeling more in control of their respiratory health status and more capable of coping with episodes of respiratory infection. Positive perceptions related to personal well-being were also reported. Several participants (n = 5) also noted that LVR reduced their level of frustration related to their respiratory health status. The vast majority (n = 9) of interview participants agreed that LVR was worth their time and energy.

Discussion and Conclusions: Strong clinical and theoretical rationales exist for evaluating patients’ perspectives to complement other outcome measures in the end-of life airway management of individuals with ALS. The findings of this study suggest positive effects of LVR on well-being and respiratory function from the patient perspective. Further research should include analysis of patients’ perspectives in relation to other outcomes of LVR and should include the perspectives of family members who are primary care providers.

P180 THE EFFECTS OF MANUAL INSUFFLATION THERAPY ON THE SPEECH AND VOICE OF PATIENTS WITH ALS

CLEARY S¹, WHEELER S², KALRA S¹, KENDALL S¹, JOHNSTON W¹

¹University of Alberta, Edmonton, ²Caritas Health Group, Edmonton, Alberta, Canada

E-mail address for correspondence: [email protected]

Keywords: Cough augmentation, speech, voice quality

Background: Over the past few years a simple and inexpensive treatment approach, lung volume recruitment (LVR), has been used to improve coughing and airway clearance of patients with ALS and other neuromuscular diseases. LVR is a manual breath stacking technique that involves the use of a resuscitator bag that has been retrofitted with a one-way-flow-valve and mouthpiece. Patients and/or their caregivers are taught to manually compress the resuscitator bag in a series of breath-stacking manoeuvres until the patient is maximally insufflated. Once patients reach maximum insufflation capacity, they are able to cough with sufficient force to clear their lungs and airways of mucus and other secretions. In ALS clinics where the LVR technique is frequently used, many patients have reported more normal voice quality and improved speech immediately after LVR treatment sessions.

Objectives: The purpose of this study was to assess the short-term effects of LVR on voice quality and to evaluate claims that LVR improves respiratory support for speech and “increases speaking volume.” [1].

Methods: Eight patients with ALS previously trained in LVR were recruited to this pilot study. The research design was a one-group, pre-post test design (i.e., OXOO). Sustained phonation and connected speech samples were collected before and 30 minutes after a typical LVR treatment session. High-fidelity audio recordings were made with a MircoTrack 24/94 professional digital recorder (i.e., sampling rate of 44.1KHz with 16-bit resolution) and WAV files were analyzed using a KayPENTAX Sona-Speech II software package. A standard assessment of voice production (i.e., mean fundamental frequency, jitter, shimmer and mean decibel level) maximum sustained phonation and mean number of syllables per breath group was conducted. Changes in pre-post treatment were examined and comparisons to age-matched normative data were made. Inter-rater reliability judgments were performed on 30% of the overall sample.

Results: No changes were noted in overall pitch and loudness levels across the experimental conditions; however, a large effect was observed in post treatment jitter scores (i.e., a cycle-to-cycle measure of pitch variation that is considered the primary acoustical correlate of the perception of voice quality). Jitter scores improved from a mean baseline of 1.33% to 7.14% (as compared to norms of 21–27%). Maximum sustained phonation improved from a mean baseline of 13.03 seconds to 18.63 seconds (as compared to norms of 24.6 seconds). The average number of syllables per breath group produced while reading a standard passage improved from a mean of 11.73 syllables per breath group to 14.48 syllables per breath group (as compared to norms of 18.2 syllables). Overall inter-rater reliability was 88%.

Discussion and Conclusions: Preliminary findings of the effects of LVR on the acoustic and aerodynamic properties of speech and voice are positive. LVR may offer a short-term improvement in voice quality and respiratory support for speech. However, ffurther investigation of this therapeutic effect in a more controlled research experiment is necessary.

P181 PROLONGED AND INTENSIVE MONITORING AFTER STARTING NON-INVASIVE VENTILATION IMPROVES TOLERANCE IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS

VOLANTI P¹, SARVÀ M¹, DE CICCO D¹, PICCOLI F², LA BELLA V²

¹Neuroriabilitazione Intensiva, Fondazione Salvatore Maugeri IRCCS, Mistretta, Italy, ²ALS Clinical Research Center, Palermo, Italy

E-mail address for correspondence: [email protected]

Keywords: amyotrophic lateral sclerosis, respiratory failure, tolerance to NIV

Background: The most frequent cause of death in patients with amyotrophic lateral sclerosis (ALS) is respiratory failure (RF) secondary to impairment of the respiratory musculature. In recent years, some studies have indicated that non invasive ventilation (NIV) improves survival and quality of life of ALS patients with RF. One restricting factor to the efficacy of NIV is bulbar involvement, but little is known about predictors of NIV adaptation and tolerance.

Objective: To evaluate the effect of prolonged and intensive monitoring in a multidisciplinary ALS clinic setting on tolerance to NIV in ALS patients with RF.

Patients and Methods: We prospectively monitored all consecutive ALS patients with RF who attended our ALS Center since June 2006. NIV was offered to 20 eligible patients (11 M and 9 F) according to current guidelines and it was initiated during a hospital stay in our ALS Center. Nine patients presented with severe (n = 5) or mild-moderate (n = 4) bulbar impairment. During the whole hospitalization period, all patients underwent a respiratory kinesitherapy program. Patients were assessed by: i) pulmonary function; ii) disability; iii) nutritional status; iv) quality of life. After discharge, all patients were regularly followed-up at three-months interval. According to accepted criteria, tolerance to NIV was defined as the ability to use the ventilator for more of 4 hours/day, for at least 60 consecutives days. NIV settings were adjusted as necessary during follow-up.

Results: The mean interval time for adaptation to NIV was 5±2 days but the patients were allowed to prolong their hospital stay for an average extended period up to 3 weeks, during which a careful monitoring of NIV was performed. We observed that 19 of the 20 patients who initiated NIV, even those with severe bulbar impairment, remained tolerant at twelve months follow-up. One patient, with a moderate bulbar impairment, became non-tolerant to NIV three months after adaptation because of persistent airways mucus accumulation. Of the 19 tolerant patients, 11 (57.9%) had at least one change in NIV settings during the first year of follow-up.

Conclusions: Our study show that an intensive and prolonged monitoring in a hospital setting after NIV adaptation increases tolerance, even in patients with severe bulbar impairment. Moreover, a respiratory kinesitherapy during the NIV training and the NIV settings adjustments appear to further increase the tolerance.

P182 EVALUATION OF HOMECARE PROBLEMS IN HOME MECHANICAL VENTILATION USERS WITH ALS IN JAPAN

NAKAYAMA Y¹, OGURA A¹, KAWAMURA S², AMAMOTO H³, UENO K⁴, OGAWA S⁵, KAKUNO F⁶, KAMIIZUMI K², KONISHI K⁷, SATO M⁸, HIRABAYASHI K⁹, MURATA M¹⁰, ITAGAKI Y¹, MATSUDA C¹

¹Tokyo Metroplitan Institute for Neuroscience, Tokyo, Japan, ²Aomori University of Health and Welfare, Aomori, Japan, ³The Japan Medical Association, Tokyo, Japan, ⁴The National Association for Home-Visit Nursing Care, Tokyo, Japan, ⁵Japanese Nursing Association, Tokyo, Japan, ⁶Higashi-oumi Public Health Center, Shiga, Japan, ⁷Showa University, Tokyo, Japan, ⁸Japan Visiting Nursing Foundation, Tokyo, Japan, ⁹Kokugakuin University, Grauate School of Law, Tokyo, Japan, ¹⁰Department of Health and Welfare, Ibaraki, Japan

E-mail address for correspondence: [email protected]

Keywords: Home mechanical Ventilation, Homecare Problems, sufficient education

Background: The number of Home Mechanical Ventilation (HMV) users with ALS is increasing in Japan, but it is unknown about their problems with care at home.

Objectives: The purpose of this study was to evaluate the use of ventilators and accessory devices at home and identify problems in the management of care for HMV users with ALS.

Methods: Data were collected by requesting 684 organizations, including health centers all over Japan, to interview home-care “patients needing assistance for suctioning,” and who consented to cooperate with the study. The interviews involved a patient profile, evaluation on the use of the respirator and accessory devices, and problems during the past year. Simple statistical processing of the data was performed, and the contents of the problems were analyzed.

Results: The survey covered 1331 consenting patients, including 749 home-cared ALS patients using a ventilator (56.2%). At the time of the survey, the mean period of ventilator use was 4.43±3.91 (0–25) years, and the mean HMV period was 4.05±3.69 (0–24) years, in the 749 patients. The method of ventilator use was TPPV in 687 (91.7%) and NPPV in 63 (8.4%), with 24-hour ventilation control in 672 (89.7%). Concerning accessory devices, 539 (72.0%) had an external battery, 733 (97.9%) had a suction machine, and 680 (90.7%) had a resuscitation bag. Problems occurred in 353 patients (47.1%), which were due to changes in the patient's condition in 201 (56.9%), and medical instrument malfunction in 144 (40.8%).

Discussion: The percentage of those undergoing NPPV was low in the 749 patients, probably because the patients were registered with public organizations, including health centers, which listed the patients in a serious state and the “need for suctioning.” Duration of ventilator use was defined as the time after initiation of mechanical ventilation until HMV had been protracted. The finding that nearly half of the patients had experienced problems suggests the inadequacy of home care and the importance of sufficient education from the initiation of HMV and for as long as HMV is used.

Conclusion: This study revealed some aspects of HMV care in Japan. Since nearly half of the patients had experienced trouble during the past year, improvements in the home care environment for the prevention and early management of problems are considered to be vital.

P183 NON-INVASIVE VENTILATION AT THE END-OF-LIFE

JOHNSTON W, WHEELER, S, LYNCH M, HEULE M, ROKOSH E, KALRA S

¹University of Alberta, ²Misericordia Hospital, Caritas Health Group, Edmonton, Alberta, Canada

E-mail address for correspondence: [email protected]

Keywords: non-invasive ventilation, end-of-life decisions, outcomes

Background: Non-invasive ventilation (NIV) has been accepted as the standard of care for patients with Amyotrophic Lateral Sclerosis (ALS) with respiratory insufficiency. While indications for initiation of NIV have been published, management of NIV and its withdrawal at end-of-life has not been evaluated.

Objectives: To determine whether NIV use persisted to time of death in patients followed at the ALS clinic at the University of Alberta who died in the period January 1 2004 to March 31 2008.

Methods: Deaths in this period were identified from the ALS clinic database.

Results: Of 133 deaths in this period, 54 (40.6%) died without attempting NIV, 9 (6.8%) were titrated, but did not continue, and 71 (54.1%) were titrated and used NIV. The NIV was not tolerated or abandoned by 5 (7%), withdrawn electively when death imminent in 6 (8.5%) and continued until death in 60 (84.5%)

Conclusions: We conclude that that the majority of those who initiate NIV continue its use until death, and do not withdraw before death. Optimal management of patients using NIV at end of life is not yet reported.