P135 EFFECT OF SWALLOWING PROBLEMS ON MOOD AND QUALITY OF LIFE IN MOTOR NEURON DISEASE
Burbidge AA & Ward CD
University of Nottingham Rehabilitation Research Unit, Derby, UK
E‐mail address for correspondence: [email protected]
Background: The experience of motor neuron disease is associated with anxiety and depression but evidence suggests that disturbed mood does not correlate closely with severity of physical impairment in disabled people. Swallowing problems may be expected to contribute to mood and quality of life (QOL), and gastrostomy feeding is sometimes justified on these grounds. This paper reports early results from an ongoing prospective study of swallowing disorders and nutritional management in MND.
Objectives: To explore the relationship between swallowing severity and anxiety, depression and quality of life in MND.
Methods: Patients with MND and their carers were followed‐up at 3–4‐monthly intervals using the following outcome measures: total disease severity and bulbar severity (ALS functional rating scale), swallowing symptom severity (SWAL‐QOL Outcomes Tool for Oropharyngeal Dysphagia), anxiety and depression (Hospital Anxiety and Depression scale) and quality of life (single‐item scale, physical well‐being, psychological and existential sub‐measures of the McGill Quality of Life questionnaire).
Results: Thirty‐two patients and 26 carers (mean age 62.3 and 62.4 years, respectively) were recruited. Sixteen patients commenced gastrostomy feeding during the study. Thirty‐one percent of patients and 42% of carers were found to have abnormal anxiety levels at entry to the study, while 22% of patients and 19% of carers scored abnormal for depression. At the visit prior to gastrostomy, the values for anxiety were 44% of patients and 62% of carers and those for depression were 31% and 31%, respectively. Paired t‐test showed no significant difference in anxiety or depression between patients and carers. No correlations were found between anxiety or depression and either bulbar severity or swallowing symptom severity in patients or carers at either time point. There was also no correlation with total disease severity. For quality of life (patients only), significant correlations were found between swallowing severity score and the McGill Physical well‐being score (r = 0.388, p = 0.028) at entry, and with the single‐item‐scale (r = 0.512, p = 0.044) at pre‐gastrostomy. No correlations were found between the bulbar score and quality of life.
Discussion: These data showed no consistent relationship between mood and swallowing problems, although certain aspects of quality of life did appear to be influenced by swallowing symptoms.
Conclusions: Our findings suggest that the sources of anxiety and depression are complex. It is unlikely that gastrostomy alone would have a major impact on anxiety or depression in a patient with MND.
P136 QUANTITATIVE SENSORY TESTING IN THE ASSESSMENT OF LARYNGEAL SENSATION IN AMYOTROPHIC LATERAL SCLEROSIS (ALS) PATIENTS
Harris DRH, Amin MRA, Patterson THP & Cassell SC
MDA/ALS Center Of Hope, Philadelphia, USA
E‐mail address for correspondence: [email protected]
Background: ALS is a progressive motor neuron disease of unknown etiology. Mortality in the population is frequently due to aspiration pneumonia. Although typically considered to be a disorder limited to motor neuron involvement, some investigators have indicated that decreased sensory function in ALS patients additionally contributes to the disease process.
Objective: The objective of this study was to evaluate laryngeal sensation in the ALS population in order to quantify the range of sensory deficits and correlate any abnormalities with demographic data to determine which patients are at risk for having sensory deficits.
Methods: We examined the sensation of the larynx in 27 patients with ALS to determine whether a sensory deficit was present. Following the completion of a dysphagia questionnaire and medical history, patients underwent flexible endoscopic evaluation of swallowing with sensory testing (FEESST) to evaluate sensory function. Threshold values were determined and recorded for initiation of the adductor reflex.
Results: The results of the sensory and swallowing function assessments performed on 27 patients demonstrate abnormal sensation in 44% of the rested population. Asymmetrical findings were noted in 66% of those patients. There was no correlation noted between the presence of sensory deficits and the severity or duration of the disease.
Conclusion: Progressive dysphagia in the ALS population has typically been attributed to muscle weakness. This study points to the presence of sensory deficits in the larynx, which can further affect proper swallowing function.
P137 NUTRITIONAL ASSESSMENT IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
Brugnani M1, Mazzini L2, Antonelli L1, Passera S1, Pastore I2 & D'Andrea F1
1Clinical Nutrition Unit, Maggiore della Carità Hospital, Novara, and 2Department of Neurology, Piemonte Orientale University, Novara, Italy
E‐mail address for correspondence: [email protected]
Background: Amyotrophic lateral sclerosis (ALS) is a degenerative disease of the nervous system that affects motor neurons; it is progressive and irreversible. Nutrition is an independent prognostic factor for survival: therefore the assessment of nutritional status is important because of the impact of suboptimal nutrition on function, quality of life and life expectancy.
Objectives: To evaluate the patients' nutritional state and nutritional problems.
Methods: We performed a nutritional assessment in all patients diagnosed with ALS evaluated in our centre by a multidisciplinary team, as from April 2004. Nutritional evaluation included: questionnaire about patients' ability to eat, chew and swallow; history of body weight and weight loss; dietary habits and food intake; anthropometry; height, weight and body mass index (BMI = weight [kg]/height [m]2), triceps skinfold thickness, mid‐upper arm circumference and mid‐upper arm muscle area (MAMA). Nutrition counselling and feeding strategies were provided to all patients and carers.
Results: We observed 72 patients (38 males, 34 females; mean age 52.67±13.62 years, range 20–79 years), of whom 25.0% had bulbar clinical presentation, at 30.12±20.35 months from diagnosis (range 2–82 months). We observed ability to autonomously eat in 45.8%, chewing difficulty in 27.5%, dysphagia in 18.1% (mainly for liquid foods) and occasional swallowing difficulties in 43.0% of cases; 4.2% had percutaneous endoscopy gastrostomy (PEG). Mean BMI was 23.85±4.43 (range 14.5–34.9), <18.5 in 16.7%, 18.5–24.9 in 48.6%, ⩾25 in 34.7%; 62.5% showed weight loss compared to usual body weight. MAMA was below 5° percentile in 51.9% of cases. Information about correct nutrition was provided to all patients able to eat by mouth; moreover in 26.4% a recommendation was made to modify the consistency of foods, 20.8% were educated in simple behavioural feeding techniques and 15.3% were referred to a speech and language therapist.
Conclusions: The symptoms and progression of ALS can affect the patient's nutritional status, limiting feeding ability (progressively weakening muscles, chewing and swallowing difficulties, dysphagia), leading to weight loss and malnutrition. Individualized nutritional assessment and monitoring should be a part of the treatment in ALS patients to develop an adequate nutritional support.
P138 NUTRITIONAL STATE EVOLUTION AND ALIMENTARY CONSUMPTION OF PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS: RANDOMIZED STUDY
Lambertucci AC, Stanich P, Ueta EY, Torino VCL, Nakao F, Quadros AAJ, Oliveira ASB & Gabbai AA
Universidade Federal de São Paul, Escola Paulista de Medicina, São Paulo, Brazil
E‐mail address for correspondence: [email protected]
Introduction: Amyotrophic lateral sclerosis (ALS) is an evolutionary disease, irreversible and incurable. Observable symptoms include a decrease of vital capacity, loss of corporal weight, an increase in nutritional needs and dysphagia. Some studies suggest that the indication of alternative nutritional support in patients with ALS can improve quality of life and minimize weight loss during the evolution of the disease.
Objective: To evaluate the nutritional state and the alimentary consumption of ALS patients and compare nutritional with alternative nutritional support in relation to patients without alternative nutritional support.
Methods: ALS patients were selected (15 patients with percutaneous endoscopic gastrostomy (PEG) in agreement with criteria proposed by Silani (2000) and 15 patients without PEG). The study was six months long and in this period nutritional evaluations were accomplished as anthropometric measures (corporal weight, arm circumference, arm muscular circumference and triceps skinfold) and trimestrial alimentary consumption using a 24 h alimentary record. The classification of the Nutritional State was based on the patterns of Frisancho (1990) for Arm's Circumference, Arm's Muscular Circumference and triceps skinfold; and the weight classification was obtained using the Body Mass Index (BMI) proposed by the World Health Organization (1998). The alimentary consumption analysis, in relation to calorie and protein intake, was accomplished by the CIS/EPM software.
Results and discussion: The patients' age varied from 31 to 74 years, with median of 53.3 years; 19 (63.3%) were male and 11 (36.7%) female. Analysis of the anthropometric measures indicated that the patients with PEG presented reduction in all measures (corporal weight, arm circumference, arm muscular circumference and triceps skinfold) compared to the group without PEG, where the measures remained unchanged. Protein and calorie ingestion was greater in the patients with PEG compared with the group without PEG. Despite the appropriate protein and calorie ingestion in the group with PEG, a nutritional state deterioration was observed. Those patients accomplished the PEG procedure tardily, as they had already presented with a 50% reduction in forced vital capacity in 50% of the foreseen, which in most cases is the decisive factor for the increase of nutritional demand, in addition to characterizing the advanced stages of the disease. The patients without PEG received nutritional orientation, mainly about the use of alimentary supplements, which possibly favoured the nutritional state maintenance. In conclusion, early indication of an alternative nutritional support in patients with ALS can attenuate the weight loss presented during the disease evolution.
P139 NUTRITIONAL STATUS AND BIOELECTRICAL IMPEDANCE ANALYSIS IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS (ALS)
Kuenhlein P1, Sperfeld AD1, Kurt A1, Waibel S1, Stippler D2 & Ludolph AC1
1University of Ulm, Ulm, and 2Pfrimmer Nutricia, Erlangen, Germany
E‐mail address for correspondence: [email protected]
Background: Nutritional deficits are an independent predictor of life expectancy for patients suffering from amyotrophic lateral sclerosis (ALS). This is caused by development of bulbar paralysis with difficulty in swallowing, hypermetabolism due to an increased effort to mechanically overcome pulmonary failure, and an intrinsic increase of energy consumption of unknown cause. The decline in muscle bulk is the basis of potential catabolism, and in addition many patients experience decreased gastrointestinal motility and also dehydration. Novel aspects of the disease process were recently described in animal experiments, in particular alterations of energy consumption which showed the importance of a sufficient calorie intake in experimental models. ALS is a prototype neurodegenerative disease in which the need for nutritional intervention is obvious, but currently it is not known how much nutritional intervention is needed.
Methods and results: We collected data on the nutritional status of 94 early ALS patients (mean age at disease onset 53.3±13.6 years; 30 females, 64 males) and performed bioelectrical impedance analysis (BIA) in the course of disease of 47 patients. During initial stages, there is no evidence for deficits of vitamins (B12, B6, B1, vitamin E and C). Furthermore, no abnormalities in body weight, body mass index, methylmalonic acid, homocysteine, haematocrit, creatinine, ketone bodies, parameters of lipid metabolism, albumin and prealbumin and fasting blood glucose were found. Only serum levels of CK were elevated. In contrast, performance of BIA showed distinct significance abnormalities in estimation of free fat mass and phase angle (PA) regardless of the underlying disease subtype. This indicates that an alteration in body composition of unknown mechanism is present in early ALS stages.
Conclusions: Our data on the nutritional status of ALS patients in early stages do not show changes of weight, vitamin deficiency or catabolism as measurable by laboratory tests or abnormalities in lipid metabolism, but there are changes of parameters obtained by BIA. BIA is supposed to be a valid test of the nutritional status in the course of the disease. However, for the future it is necessary to obtain follow‐up BIA data in the course of ALS to assess its value for monitoring nutritional status, as metabolic and caloric requirements of ALS patients, especially in the late phase, are not well understood at present.
P140 HYPERMETABOLISM IN ALS: CORRELATIONS WITH CLINICAL PARAMETERS AND SURVIVAL
Desport JC2, Torny F1, Lacoste M1, Preux PM3 & Couratier P1
1Centre SLA, Limoges, 2Unite Nutrition, Limoges, and 3Departement Biostatistiques, Limoges, France
E‐mail address for correspondence: [email protected]
Background: ALS is a neurodegenerative disorder characterized by upper and lower motor neuron degenerations leading to an extensive paralysis with muscle atrophy. Resting energy expenditure (REE) is linked to fat free mass (FFM), age and sex. Despite a reduction in FFM, a hypermetabolism has been reported with an average of 10% in ALS patients compared with a healthy population (1).
Objectives: To confirm the level of hypermetabolism in a larger group of ALS patients, to study variations over time and correlations with clinical and paraclinical parameters and survival.
Patients and methods: One hundred and sixty‐eight patients with probable or definite ALS were prospectively enrolled. REE level was determined by using indirect calorimetry. Difference with calculated value given by Harris and Benedickt equations determined a ΔREE. FFM was given by bioimpedance dual frequency (5–100 kHz). Phase angle, which is a physical parameter of cellular integrity was analysed in BIA evaluation. At each consultation, body mass index (BMI) was calculated. Neurological assessment was performed using MMT and ALSFRS scores. Forced vital capacity was evaluated by spirometry. Survival was analysed from first symptoms of ALS and from the first evaluation at T1 (mean delay from first symptoms: 556±524 days). Correlations with clinical and paraclinical parameters were studied on REE and ΔREE levels. Survival was analysed in univariate and multivariate analyses after adjusting for factors with a p value between 0.25 and 0.05.
Results: At T1, REE was significantly increased by an average of 14% compared with calculated value; 62.3% of ALS patients were considered as hypermetabolic (REE measured exceeding 110% of the calculated value). In univariate analysis, REE was correlated with age, sex, clinical form at onset, presence of a denutrition determined on BMI, weight, FFM, phase angle and ALS‐FRS. In multivariate analysis, REE was linked to age and FFM. ΔREE was correlated with sex, phase angle and MMT in univariate analysis. In multivariate analysis, age and sex remained significantly correlated. Over time, REE levels remained higher than calculated values with a trend to decrease at proximity of death, whereas FFM remained stable near the period of death. Survival from first symptoms was linked to age and clinical form at onset. From T1, survival in univariate analysis was linked to MMT, ALSFRS, FVC, REE and phase angle. In multivariate analysis, age, FVC and BMI were correlated.
Conclusion: We confirmed the existence of a hypermetabolic state in the ALS population. Hypermetabolism remains stable over time except at the proximity of death. It depends mainly on age, sex and FFM. REE is a prognostic factor for survival in univariate analysis. Its cause remains poorly understood. Some hypotheses including cytokine secretion, mitochondrial disturbances or sympathetic dysfunction have to be addressed.
Keywords: Hypermetabolism, Survival, Nutrition
References
- Desport et al. Am J Clin Nutr 2001; 74(3):328–24.
P141 PERCUTANEOUS DILATATIONAL TRACHEOSTOMY USING UNCUFFED SMALL CANNULA FOR PALLIATIVE CARE IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
Nonaka M, Imai T, Chiba S & Matsumoto H
Sapporo Medical University School of Medicine, Sapporo, Japan
E‐mail address for correspondence: [email protected]
Background: Patients with terminal amyotrophic lateral sclerosis (ALS), particularly the bulbar type, often have a high risk of upper airway obstruction due to aspiration of secretions, accompanied by the fear of “choking to death”. This problem occurs suddenly and is not reduced by the administration of opioids.
Objectives: Intra‐tracheal suction can be approached via the nose or mouth, but this unpleasant and blind technique is often unsuccessful. Frequent suction may exhaust patients. Percutaneous dilatational tracheostomy (PDT) using the Seldinger technique has a low incidence of complication compared with surgical tracheostomy and provides an access to the trachea for suction. The purpose of this study was to assess the safety and efficacy of PDT for palliative care in patients with ALS.
Methods: Three ALS patients in terminal phase received PDT. These patients were extremely distressed by the accumulated secretion and declined ventilation support via tracheostomy. PDT was performed using a Mini‐Track II minitracheostomy kit or Melker emergency cricothyrotomy catheter set. Placement of a small uncuffed cannula (inner diameter: 4 or 6 mm) was achieved at the cricothyroid membrane or between the cricoid and the first tracheal ring.
Results: In all cases, PDT was easily and safely performed without any trouble. There were no complications such as pharyngotracheal aspiration caused by the cannula and cricoid stenosis. Two patients were under local anesthesia, and the other was under light inhalation anesthesia during the PDT procedure. Intra‐tracheal suction was performed immediately when patients noted distress due to airway obstruction by secretion. Speech and swallowing functions were not affected by the cannula. Two patients were able to go home soon after receiving the procedure. All patients died peacefully without ventilation support. The calmness of their final days was maintained by intermittent suction via the cannula and administration of opioids. The cannula was retained for periods ranging from 2 to 3.5 months until death.
Discussion and conclusions: Standard tracheostomy usually requires general anesthesia and carries a high risk of ventilator dependence in ALS patients suffering from respiratory failure. In contrast, PDT with a small uncuffed cannula is less invasive and may contribute to maintain the general welfare of the patient. PDT is regarded as beneficial to ensure a peaceful end and increase the quality of life of ALS patients and their families.
P142 A POPULATION BASED PILOT STUDY OF RESPIRATORY FUNCTION IN MOTOR NEURON DISEASE PATIENTS IN TAYSIDE AND NORTH‐EAST FIFE: DEVELOPMENT OF A GUIDELINE/REFERRAL PROTOCOL
Colville SM1, Swingler RJ1, Grant IS2 & Williams FLR3
1Department of Neurology, Ninewells Hospital, Dundee, 2Home Ventilation Service, Western General Hospital, Edinburgh, and 3Department of Epidemiology and Public Health, Ninewells Hospital, Dundee, UK
E‐mail address for correspondence: [email protected]
Background: Respiratory failure is the most common form of death for a motor neuron disease patient, with respiratory insufficiency symptoms often insidious in onset, implying a larger problem than is currently realized. There is evidence that symptomatic management by the use of non‐invasive ventilation can improve a patient's quality of life; however, this form of treatment is not uniformly practised in the United Kingdom and the use of non‐invasive ventilation falls behind that of the United States and Europe with this patient group. To our knowledge there have been no population‐based studies to ascertain the level of the problem within a motor neuron disease population.
Objectives: To evaluate the level of respiratory insufficiency within the motor neuron disease population of Tayside and north‐east Fife and to develop a protocol/guideline for referral for symptomatic management of respiratory insufficiency.
Methods: A population based study carried out over a three‐month period on all patients with a diagnosis of motor neuron disease as classified by the El Escorial criteria within the Tayside and north‐east Fife area. Outcome measures used include spirometry, sniff nasal inspiratory pressure, nocturnal pulse oximetry measurements, venous bicarbonate and chloride levels, the Epworth Sleepiness questionnaire and the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale.
Results: Thirty‐one patients were included in the study. Standard questionnaires such as Epworth Sleepiness Scale and Amyotrophic Lateral Sclerosis Functional Rating Scale‐Revised identified 26% and 16%, respectively, of cases with respiratory insufficiency symptoms. However, with the use of more detailed questions, the level of the problem increased with 87% of the study population reporting at least one respiratory insufficiency symptom. Fifteen cases (58%) had a spirometry result below 70% predicted value (four missing cases), and nocturnal pulse oximetry results showed that 11 cases spent >5% of time below 90% oxygen saturation. Using the proposed guideline/protocol 10 patients (32%) met the criteria for referral to the respiratory physician for further respiratory assessment and initiation of non‐invasive ventilation if they chose this option; currently only one patient had been offered this symptomatic treatment.
Conclusions: The use of standard questionnaires is insufficient to detect respiratory compromise and more detailed questions were required in conjunction with respiratory function tests. The development of the guideline/protocol allows more effective monitoring of respiratory function with the possibility of use throughout Scotland. Through the use of the proposed guideline/protocol the quality of life for an estimated 84 people living with motor neuron disease in Scotland could be improved by referral for non‐invasive ventilatory support.
P143 AMYOTROPHIC LATERAL SCLEROSIS: CORRELATION BETWEEN MAXIMUM PHONATION TIME AND FORCED VITAL CAPACITY
Baggio L1, Sorarù G2, D'Ascenzo C1, Palmieri A1, Polo A2 & Angelini C1
1Department of Neurosciences, University of Padova, IRCCS S.Camillo, (Venezia), Venezia, and 2Division of Neurology, Piove di Sacco, (Venezia) , Piove di Sacco (PD), Italy
E‐mail address for correspondence: [email protected]
Background: Amyotrophic lateral sclerosis (ALS) is a neuromuscular disease in which progressive respiratory failure is usually the cause of death. Regular assessment of respiratory function is important in predicting disease progression. In particular, Forced Vital Capacity (FVC) is a useful prognostic index and its reduction below 50% of predicted value is often associated with the onset of respiratory symptoms.
Objectives and methods: In order to obtain a more simple, reliable and non‐stressful measurement to evaluate the progression of ventilatory impairment even in patients at home, we investigated the relationship between maximum phonation time (MPT) and FVC. We considered data from 40 ALS patients (28 males, 12 females) followed at our Neuromuscular Unit in the period February 2004–February 2005. A motor involvement was present in all patients whereas bulbar symptoms were found only in 25 patients. At each quarterly examination, FVC was assessed by a portable spirometer and MPT was obtained by asking the patients to take a single deep breath and to prolong the vowel sound ‘a’ as long and steadily as possible. All subjects were tested while in seated position. For each examination the best of three attempts was considered for both FVC and MPT assessment.
Results: A regression analysis between MPT and FVC was performed and a strong positive correlation was found between the two variables in ALS patients whereas no significant correlation was found in the ALS patients with bulbar signs.
Conclusions: This result indicates MPT as an index of ventilatory function in ALS patients without bulbar involvement.
P144 EARLY RESULTS OF LAPAROSCOPIC MOTOR POINT DIAPHRAGM PACING IN AMYOTROPHIC LATERAL SCLEROSIS: CAN EXOGENOUS ELECTRICAL STIMULATION IMPACT RESPIRATORY FAILURE?
Onders RP, Schilz R, Katirji B, Elmo MJ & Ignagni A
University Hospitals of Cleveland and Case Western Reserve University, Cleveland, USA
E‐mail address for correspondence: [email protected]
Background: Respiratory failure is responsible for the majority of deaths in patients with amyotrophic lateral sclerosis (ALS). Therapeutic electrical stimulation has been shown to maintain the strength of other peripheral muscles in ALS. Electrical stimulation may also maintain physiologic activity, contractile properties, calcium levels and promote collateral axon sprouting. We have shown that a laparoscopic diaphragm pacing system in spinal cord injured patients is a low‐risk, cost‐effective outpatient system that will support the full‐time respiratory needs of patients. We are currently studying the use of this technology in patients with ALS and respiratory insufficiency.
Objective: To evaluate, in this Phase I trial, the safety of implanting the diaphragm pacing system for use in conditioning the diaphragm of classic ALS patients. Our secondary objectives are to assess the system in slowing the respiratory decline and improve quality of life for implanted patients.
Methods: The principle inclusion requirements are a forced vital capacity above 50% at the start of the study and above 45% at the time of surgical implantation. Each patient is followed for three months pre‐implantation with a series of tests including: pulmonary function tests, speech phonation times, ultrasound analysis of diaphragm thickness, phrenic nerve conduction tests and quality of life tests. Patients undergo a 1–2 h outpatient laparoscopic mapping of their diaphragm to locate the phrenic nerve motor points with two electrodes implanted in each hemidiaphragm. Two weeks after surgery, the stimulus output characteristics of each electrode are determined and stimulus parameters are selected for conditioning. The patients then condition their diaphragm at home with five 30‐min sessions of therapeutic electrical stimulation per day. The conditioning sessions may be performed while they are carrying out their ordinary activities of daily living. Patients are assessed post‐operatively on a monthly basis with the same tests performed pre‐operatively to compare pre‐ and post‐implant trends.
Results: Three patients were successfully implanted. All three had an FVC of 53% or less at the time of surgery. The diaphragms at surgery presented with significant weakness in two patients. One patient had a radial banding appearance of her intact motor units. All have been able to tolerate training with the system. At early follow‐up the patients' FVC (without stimulation) has decreased but at the 2‐month follow‐up the first patient's FVC had increased to above the 1‐month follow‐up. In all patients the phonation times, muscle thickness and movement of diaphragm with stimulation under fluoroscopy have improved. All three patients have, anecdotally, reported feeling better.
Conclusion: The diaphragm pacing system can be safely implanted and utilized in patients with ALS. Whether there will be a long‐term improvement in ventilation leading to an increased survival or quality of life requires further follow‐up.
P145 RESPIRATORY AIDS IN PATIENTS WITH MOTOR NEURON DISEASE
Kareus SA, Moreland SD, Fewell DF & Rudnicki SA
University of Arkansas for Medical Sciences, Little Rock, USA
E‐mail address for correspondence: [email protected]
Background: Non‐invasive ventilation (NIV), portable suction (PS), exsufflator/insufflator (EI) and high frequency chest wall oscillator (HFCWO) are respiratory aids (RAs) used in patients with motor neuron disease (MND).
Objectives: To determine use of RAs in our clinic, benefits of NIV and HFCWO, and patient features predicting use of NIV.
Methods: Retrospective study of MND patients seen over the past two years for whom the recommendation of a RA was made.
Results: Thirty‐nine patients fit the criteria, and for all, NIV was the first RA recommended either alone or in conjunction with another RA. Thirty‐two (82%) agreed to try NIV, and seven refused. Of those who refused initially, one agreed three months later, and one used it terminally as palliation. There was no significant difference in gender, onset site, sleep score, ALS‐FRS‐R, orthopnea or dyspnea score for those who agreed to NIV initially and those who did not. In 16 patients with sleep scores recorded pre‐ and post‐NIV, it improved in 10, was unchanged in four, and worsened in two following NIV. There was no significant difference in the change in sleep score for patients who used NIV 0–4 h compared to those who used it longer. Of the 19 patients who were seen at least once after initiating NIV, at three months 12 (63%) were using NIV for 4–8 h per night. Of the seven using it less at three months, two ceased using it within a year and three subsequently increased use. Three months after starting NIV, patients who could identify specific improved symptoms were more likely to be using it 4–8 h per night (p<0.01). Nineteen patients had HFCWO recommended for weak cough, three refused, one agreed but died before starting, and 15 tried it. For the 10 who used it for more than a month, using a 0–5 point scale with 0 ‘strongly disagree’ and 5 ‘strongly agree’, they reported at one month: improved secretion clearance 4.1±0.6; less shortness of breath 3.9±0.8, and better sleep 3.9±0.7. NIV alone was used by 14, HFCWO and NIV, seven; suction alone, one; NIV and EI, three; NIV, suction, and HFCWO, three; NIV, suction, HFCWO, and EI three.
Conclusions: The percentage of our patients willing to try NIV and use it for more than 4 h per night was high. This is probably multifactorial, as two years ago a respiratory therapist joined our clinic, and we identified a home respiratory therapy company that provided excellent teaching and follow‐up, and changed the brand of the NIV machine. We could not identify factors that distinguished between patients willing to try NIV and those unwilling; identifiable symptomatic benefits were associated with longer nightly use. HFCWO may be helpful to some patients with MND, particularly regarding secretion clearance.
P146 PREDICTING OUTCOMES FOR PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS TREATED WITH NON‐INVASIVE POSITIVE PRESSURE VENTILATION USING A SALIVARY DYSPHAGIA SCORE
Cazzolli P1, Lewarski J2 & Chatburn R3
1Neuromuscular Education Project, Canton, 2Inogen Inc, SF, and 3University Hospitals of Cleveland, Cleveland, USA
E‐mail address for correspondence: [email protected]
Background: Non‐invasive positive pressure ventilation (NPPV) is often used to support breathing in patients with amyotrophic lateral sclerosis (ALS). However, patients with progressive bulbar paralysis may not tolerate NPPV due to their inability to swallow saliva. Thus, severe dysphagia threatens survival.
Objective: The purpose of this study was to develop a salivary dysphagia scoring system and evaluate the score's ability to 1) predict duration of survival after initiation of NPPV, 2) determine intolerance of NPPV, and 3) identify appropriate care interventions, including the need for end‐of‐life care, or when to start invasive ventilation.
Methods: Data were collected from 1990 to 2002 on 157 patients. Twenty‐two tried NPPV, but failed. The remaining 135 were treated with NPPV and closely followed through ongoing periodic home visits. An ordinal scoring system was defined as follows: score of 4 = no impaired ability to swallow saliva; 3 = small accumulations of saliva in the mouth; 2 = occasional pooling/drooling; 1 = frequent pooling/drooling and conscious effort to swallow; 0 = constant pooling/drooling plus the inability to clear secretions from the trachea. Survival was defined as the interval (months) from the start of NPPV until death or transition to TPPV. Intolerance of NPPV was defined as the inability to continue treatment without risk of aspiration. Median survival scores were compared with using Kruskal‐Wallis one‐way ANOVA. Post hoc comparisons were performed with Dunn's method. Differences associated with p values <0.05 were considered significant.
Results: All patients receiving NPPV had initial scores greater than zero. Median survival differed significantly between those with a score of 1 (eight months) compared to those with a score of 4 (27 months, p = 0.002). Fifteen patients with a score of 4 used NPPV for 24 h per day and survived 24 to 92 months. Seven of the 15 used NPPV for 5 to 7+ years. There was no difference in survival between patients with scores of 2 and 3. The median score for patients who became intolerant of NPPV and died was 1. The use of medications to dry secretions or mechanically assisted coughing was ineffective in patients with a score of 0 or 1.
Conclusions: The salivary dysphagia score was useful in predicting outcomes on NPPV. A score of 1 reliably signaled the intolerance of NPPV and/or the need for end‐of‐life care or transition to invasive ventilation. The score is easy to apply and may help to avoid inappropriate therapy, emergency hospitalizations or early mortality.
P147 SURVIVAL AND FACTORS PREDICTING SURVIVAL FOLLOWING NON‐INVASIVE VENTILATION IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
Peysson S1, Philit F3, van den Berghe N1, Bayle JY4, Vial C2 & Broussolle E1
1Neurologie C Hôpital Pierre Wertheimer, Lyon, 2Service d'ENMG et pathologies neuro‐musculaires Hôpital Neurologique Pierre Wertheimer, Lyon, 3Service de Réanimation Médicale et d'assistance respiratoire Hôpital de la Croix Rousse, Lyon, and 4Service d'Exploration Fonctionnelle Respiratoire Hôpital Cardiologique Louis Pradel, Lyon, France
E‐mail address for correspondence: [email protected]
Background: Amyotrophic lateral sclerosis (ALS) is characterized by a progressive decline of motor functions with involvement of respiratory muscles as a major predicting factor for survival. There is considerable evidence that non‐invasive ventilation (NIV) can treat and relieve symptoms of alveolar hypoventilation.
Objectives: To determine survival and factors predicting survival following NIV in 33 patients with ALS.
Methods: We conducted a retrospective study of 33 ALS patients with NIV, followed between 1996 and 2004. We determined mean survival since onset of the disease and since treatment by NIV. Statistical analysis was performed using the Kaplan‐Meier test. Cox proportional hazards models were constructed to adjust for potential confounding variables.
Results: Mean age was 60.4 years with 23 male and 10 female patients. Ten patients had bulbar onset, 22 patients had limb onset and one patient had initial (inaugural) respiratory signs. Mean initial and maximal diurnal total utilization of NIV were 10 and 14 h. Mean survival since ALS onset and since treatment by NIV were 34.2 and 8.4 months. Survival depends on age and clinical form. Survival after treatment by NIV is influenced by the use of a mechanical in‐exsufflation device.
Discussion: Prognosis depends on age and initial clinical form of the disease. Survival after treatment by NIV is not influenced by respiratory parameters or bulbar symptoms. The utilization of thermoformed nasal interfaces is accompanied by an excellent tolerance of NIV, independent of the clinical form.
Conclusion: NIV can treat sleep‐disordered breathing and nocturnal alveolar hypoventilation, also in bulbar onset ALS patients. Clarification for optimal timing for initiation of NIV and tolerance needs to be undertaken.
P148 THE RESPIRATORY SUPPORT OF ALS PATIENTS WITH TPPV IN TOKYO METROPOLITAN NEUROLOGICAL HOSPITAL FROM 1980 TO 2005
Kawata A, Hirai T, Shimizu T, Nagao M & Hayashi H
Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
E‐mail address for correspondence: [email protected]
Background: The clinical investigations of ALS patients using tracheostomy positive pressure ventilation (TPPV) in Tokyo Metropolitan Neurological Hospital (TMNH) have disclosed the total manifestation of ALS. In this study, we re‐examined various problems of ALS patients with TPPV in TMNH from 1980 to 2005.
Objectives and methods: To investigate the clinical course of ALS with TPPV, we examined the medical records of ALS patients admitted to TMNH from January 1980 to May 2005. Kaplan‐Meier analyses were used to estimate the disease prognosis.
Results: Of the 239 ALS patients who showed critical respiratory failure between 1980 and 2005, 98 patients (61 males and 37 females) were treated with TPPV (41%), while 141 patients died without ventilation support. In the early years (from 1980 to 1984), however, almost 100% of patients were treated with TPPV. The median interval from onset to respiratory failure was 28.6 months in the 98 patients with TPPV. Sixty‐one patients of the 98 patients have already died, and the median survival period of them was 128.6 months. In the 41 patients on TPPV for more than five years, severely impaired communication occurred, namely the totally locked‐in state (TLS) developed in seven (17.1%) and the minimal communication state (MCS) in 12 (29.3%). The causes of death were pneumonia (23 patients, 38%), heart failure (eight patients, 13%), sudden cardiac arrest (seven patients, 11%), renal failure (three patients, 5%), and others. Although 36 patients among 61 deceased patients were followed up at home by the neurologists from our hospital, only two patients died at home and others died in our hospital or other hospitals.
Discussion and conclusions: The high frequency of TPPV treatment in the first five years and gradual decrease to plateau level (around 40%) in TMNH might be partly attributed to a lack of provision of precise information to the patients in the early period, but afterwards we tried to disclose to the patients the diagnosis, and the information about the likely course of ALS based on the ‘the new view of ALS’. Although the ALS patients with TPPV and caregivers need to cope with various burdens due to aggravating motor impairments after respiratory failure, it has been confirmed that they could live for a long time beyond respiratory failure. About 50% of patients on TPPV showed severely impaired communication (MCS or TLS); however, many patients led active lives, expressing their thoughts using communication devices for many years. To improve prognosis further, we have to prevent pneumonia effectively and pay attention to the sympathetic hyperactivity which may cause circulatory collapse and sudden death. The low frequency of death at home in spite of the high percentage of follow‐up by neurologists at home may reflect Japanese socio‐cultural circumstances.