P20 ASSESSMENT OF HYPOVENTILATION IN AMYOTROPHIC LATERAL SCLEROSIS: MULTIMETHODOLOGICAL SIMULTANEOUS MEASUREMENT
Ogata K1, Suzuki M1, Miyatake S1, Okahashi S1, Yamamoto T2, Kawai M1
1NHO Higashisaitama Hospital, Saitama, Japan, 2National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan
E‐mail address for correspondence: [email protected]
Background: Pulmonary dysfunction resulting from weakness of the respiratory muscles is critical for patients with amyotrophic lateral sclerosis (ALS). Assessment of hypoventilation is important for medical management of ALS (1). Several methods for assessment of pulmonary function have been reported along with their advantages and disadvantages (2).
Objectives: To determine the correlation among various tests for ventilatory function in ALS.
Methods: We evaluated ventilatory function in 10 patients with ALS using spirometry, analysis of arterial blood gas (ABG) and nocturnal pulse oximetry (NPO). We measured VC, %VC, FVC, %FVC and peak flow with spirometry; PaCO2, PaO2 and pH with the analysis of ABG; mean and minimum value of SaO2 and mean pulse rate (PR) with NPO. We defined a summary index of NPO in an earlier report (3) as follows: nocturnal hypoxia index (NHI) = (% time of 95%⩾SaO2>90%)+(% time of 90%⩾SaO2>85%)×2+(% time of 85%⩾SaO2>80%)×3+… To analyse the correlation, we calculated Spearman's rank correlation coefficient among measured values.
Results: In one patient, NPO showed periodic hypoxemia, compatible with hypoventilation in REM sleep reported in ALS (4) and Duchenne muscular dystrophy (5). Significant correlation (p<0.05) was found between: PaCO2 and NHI; PaCO2/minimum SaO2 in NPO; and all values measured in spirometry. Scattergram showed that %FVC decreased earlier than elevation of PaCO2 and NHI.
Discussion and conclusions: Statistical analysis suggested different meanings of values in NPO. Analysis of ABG and NPO may be useful for evaluation of hypoventilation in advanced ALS with difficulties for spirometry. The relationship between PaCO2 and NPO was still ambiguous. Longitudinal study might make it clear.
References
- Winhammer JMC, Rowe DB, Henderson RD, et al. Lancet Neurol. 2005;4:229–38.
- Bach JR, Bianchi C, Aufiero E. Chest 2004;126:1502–7.
- Kawai M, Shintani M, Komiya T, et al. Rinsho Shinkeigaku 1995;35:1003–7.
- Ferguson KA, Strong MJ, Ahmad D, et al. Chest 1996;110:664–9.
- Ogata K, Kohno S, Komiya T, et al. Rinshi Shinkeigaku 1996;36:850–3.
P21 NOCTURNAL HYPOXIA IN EARLY MOTOR NEURON DISEASE IS NOT PREDICTED BY STANDARD RESPIRATORY FUNCTION TESTS
Winhammar JMC1, Joffe D1, Simmul R1, Schoeffel R1, Kiernan MC2, Rowe DB1
1Departments of Neurology and Thoracic Medicine, Royal North Shore Hospital, University of Sydney, Sydney, Australia, 2Multidisciplinary Motor Neuron Disease Service, Prince of Wales Hospital and Prince of Wales Medical Research Institute University of New South Wales, Sydney, Australia
E‐mail address for correspondence: [email protected]
Background: With increasing awareness of motor neuron disease (MND), the approach to respiratory management of patients with this disease will more commonly face the physicians who manage MND.
While respiratory failure in MND patients is generally a direct consequence of muscle weakness during disease end‐stage (1), a proportion of patients develop early and severe respiratory difficulties during sleep, termed nocturnal hypoventilation. Hypoventilation produces nocturnal hypoxia (NH), which is becoming increasingly recognized as a predictor of survival, independent of respiratory muscle weakness (2,3). It has recently been demonstrated that intervention with non‐invasive ventilation in MND patients with symptomatic respiratory failure improves survival (4).
Objective: The aim of this study was to determine if standard respiratory function tests could determine the presence of NH in MND patients without respiratory symptoms.
Methods: Respiratory function tests were used to examine daytime respiratory function and sleep studies were used to detect NH in 16 consecutive patients with MND, as well as nine healthy control subjects. Demographic data, clinical parameters, respiratory function tests and oximetry studies were obtained. Statistical analyses were performed using t‐tests and analysis of variance where appropriate.
Results: NH was detected in 50% of patients with MND with no hypoxic events detected in the control group. Standard respiratory function tests were not able to predict the presence of NH.
Conclusion: There was no correlation between respiratory function test parameters and the presence of NH. This study highlights the inability of standard respiratory function tests to predict nocturnal hypoxia that may arise early in the course of motor neuron disease.
References
- Oppenheimer EA. Treating respiratory failure in ALS; the details are becoming clearer. Journal of the Neurological Sciences 2003;209:1–4.
- Winhammar JM, Rowe DB, Henderson RD, Kiernan MC. Assessment of disease progression in motor neuron disease. Lancet Neurology 2005;4:229–38.
- Velasco R, Salachas F, Munerati E, Le Forestier N, Pradat PF, Lacomblez L, et al. Nocturnal oximetry in patients with amyotrophic lateral sclerosis: role in predicting survival. Rev Neurol. (Paris) 2002;158:575–8.
- Bourke SC, Tomlinson M, Williams TL, et al. Effects of non‐invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis. Lancet Neurology 2006;5:140–7.
P22 USEFULNESS OF SNIFF NASAL INSPIRATORY PRESSURE (SNIP) FOR JAPANESE PATIENTS WITH ALS
Kamide N1, Ogino M2, Ogino Y2, Fukuda M1, Sumida S3, Hiraga Y3, Sakai F2
1School of Allied Health Sciences, 2Department of Neurology, Kitasato University, Sagamihara, Japan, 3Kitasato University East Hospital, Department of Rehabilitation, Sagamihara, Japan
E‐mail address for correspondence: [email protected]
Background: As an assessment tool for respiratory function of patients with ALS, forced vital capacity (%FVC) is thought to be standard. However, it is sometimes difficult to measure %FVC in advanced stages of disease. Therefore, SNIP has been reported as a new assessment tool for respiratory function (1). However, SNIP data measured in Japanese patients are insufficient, and usefulness of SNIP is not clear yet in Japan.
Object: The purpose of this study was to investigate usefulness of SNIP in Japan.
Methods: Twenty‐five healthy controls (21–57 years of age), and 30 patients with ALS (41–79 years, disease duration 0.3–13 years) were enrolled in this study. As outcome measures, SNIP, maximal mouth inspiratory pressure (PImax) (healthy control only), %FVC and PaCO2 (ALS only) were collected.
Results: An average value of SNIP was 79.4±21.8 cm H2O in healthy men, 58.2±17.8 cm H2O in healthy women, 30.3±20.1 cm H2O in patients with ALS. High correlation was observed between SNIP and Pimax (r = 0.61), %FVC (r = 0.53) and PaCO2 (r = −0.49), respectively. In addition, SNIP and PaCO2 were correlated in ALS patients in whom measurement of %FVC was technically impossible (r = −0.61). A weak correlation was observed between SNIP and PaCO2 in patients with bulbar symptoms (r = −0.35).
Discussion and conclusions: Because SNIP values in healthy Japanese people and patients with ALS were lower than in Europeans and Americans (1,2) it is necessary that original standard values are established in Japan. Since correlation was observed between SNIP and Pimax, %FVC and PaCO2, it was confirmed that SNIP was reliable also as an assessment of respiratory function in Japanese people. It was suggested that SNIP was especially useful as a predictor of hypercapnia. In addition, SNIP can be measured in patients in whom measurement of %FVC was technically impossible. In patients with bulbar symptoms, although SNIP tended to be lower than in patients without bulbar symptoms, SNIP and PaCO2 were weakly correlated.
References
- Morgan RK, et al. Am J Respir Crit Care Med. 2005;171:269–74.
- Uldry C. Throrax 1995;50:371–5.
P23 RESPIRATORY MUSCLE STRENGTH AND COUGH CAPACITY IN AMYOTROPHIC LATERAL SCLEROSIS
Kang S‐W, Choi YC, Park JH, Lee SC, Choi WA, Kim W, Kim DH
Yongdong Severance Hospital, Rehabilitaton Medicine, Seoul, South Korea
E‐mail address for correspondence: [email protected]
Background: Ineffective cough is a serious problem in patients with amyotrophic lateral sclerosis (ALS). This makes the patients highly susceptible to potentially fatal respiratory infections.
Objectives: The purpose of this study was to investigate the relationship of voluntary cough capacity and respiratory muscle strength.
Methods: Twenty‐seven patients with ALS were investigated. The vital capacity (VC) in sitting and supine position, maximum inspiratory (MIP) and maximum expiratory pressure (MEP), and unassisted peak cough flow (PCF) were measured.
Results: The mean VC in sitting and supine positions was 35.09% (1330.00 ml) and 27.90% (1051.85 ml) of normal predicted value, respectively. VC in the sitting position was of significantly higher value than in the supine position. The mean PCF was 205.37 l/min. The mean MIP and MEP were 26.48% (21.48 cm H2O) and 27.96% (33.41 cm H2O) of normal predicted value, respectively. Both MIP and MEP were correlated with PCF.
Conclusions: For assisting cough effectively, it is important to analyse the correlation between cough capacity and the strength of respiratory muscles. Both MIP and MEP, which are the markers of respiratory muscle strength, should be taken into account in the study of cough effectiveness.
P24 RELATIONSHIP BETWEEN TIME FROM ONSET OF ALS SYMPTOMS AND INITIATION OF HOME MECHANICAL VENTILATION (HMV) IN BULBAR AND SPINAL ONSET ALS PATIENTS AND DIFFERENCES IN ADVANCE DIRECTIVES CONCERNING INVASIVE MECHANICAL VENTILATION (IMV)
Felding MF, Dreyer PD, Lorentzen CKL
Respiratory Centre West, Aarhus University Hospital, Aarhus, Denmark
E‐mail address for correspondence: [email protected]
Background: ALS is a progressively paralysing disease resulting in poor quality of life and death due to respiratory failure. HMV has been shown to improve quality of life and survival. The degree of progression in the loss of respiratory function has been shown to be related to the onset of symptoms, with a faster decrease in bulbar onset compared with spinal onset patients. These factors may have an impact on the time of initiation of ventilatory intervention and on the patient's ability to make an advance directive concerning IMV.
Objective: To determine the influence of bulbar and spinal onset in patients with ALS on the timing of HMV, and on the ability to make an advance directive concerning IMV.
Methods: Data were collected retrospectively from ALS patients cared for at our centre in 2005 and for whom HMV, non‐invasively (NIV) and invasively had been ordered. The patients had been diagnosed with ALS/MND by El Escorial revised criteria, and were referred for respiratory evaluation when patients presented symptoms of nocturnal hypoventilation and/or forced vital capacity (FVC) decreased to 70% of predicted. Mechanical ventilation was initiated after episodes of acute respiratory failure or after evaluation of polysomnographic studies. All patients were informed of the possible respiratory complications and the therapeutic options in an attempt to achieve early decision‐making. Onset of symptoms and time from start of symptoms to initiation of mechanical ventilation was recorded. Furthermore, onset of symptoms and time from start of symptoms to decision to make an advanced directive was recorded in the NIV group.
Results: Fifty‐two patients were identified, 27 (52%) with bulbar onset and 25 (48%) with spinal onset. Thirty‐eight were ventilated non‐invasively and 14 invasively.
The mean time from start of symptoms to initiation of HMV was, in the bulbar onset group, 23 months (range 10–60 months) and in the spinal group 34 months (range 9–84 months) (p<0.01).
In the bulbar group, 88% made an advance directive after a mean period of 30.7 (range 11–72) months, where 40% accepted and 48% refused IMV; 12% did not make an advance directive at all. In the spinal group, 66% made an advance directive after a mean period of 44.1 (range 11–72) months, where 38% accepted and 29% refused IMV; 33% did not make an advance directive at all.
Conclusions: In our population of ALS patients the onset of symptoms had a significant impact on the timing of initiation of HMV with the shortest time in the bulbar onset group. This knowledge may imply a more aggressive approach concerning respiratory monitoring and information, and in terms of advanced directives an early attempt in giving information to approach early decision‐making in this subgroup of patients. For the spinal group a large number of patients did not make an advance directive at all. This may imply a more careful practice in giving information to approach decision‐ making for all patients in this subgroup.
P25 DIFFICULTIES, PSYCHOLOGICAL SUPPORT, AND RELATIONSHIP WITH HOPE IN INVASIVE MECHANICAL VENTILATOR‐DEPENDENT ALS PATIENTS IN JAPAN
Hirano Y1, Yamazaki Y1, Shimizu J2
1The University of Tokyo, Tokyo, Japan, 2Tokyo Metropolitan University, Tokyo, Japan
E‐mail address for correspondence: [email protected]
Background: Most ALS patients who depend on an invasive mechanical ventilator (IMV) suffer from movement, speech, and swallowing deficits, while at the same time facing nearly insurmountable barriers in communication of their suffering. While it is crucial for such patients to depend on psychological support for a sense of hope, no research on these factors in IMV ALS patients has yet been performed.
Objectives: This research seeks to provide a basis for improving IMV ALS patient care by investigating their difficulties, sources of psychological support, their joys and happiness, and their desires and wishes. The relationships between these factors and patient attributes are explored, followed by assessment of relationship to hope as an indicator of successful adaptation to the illness experience (assessed by the Herth Hope Index).
Methods: We first performed interview surveys, then used the results from these to craft postal surveys using anonymous questionnaires. We interviewed 27 patients and their families, and obtained valid responses from 157 subjects for the written survey (recovery rate of 78.8%).
Results: Most patients suffered multiple kinds of physical, emotional and social difficulties, which were correlated with lower levels of hope. Patients with more severe physical symptoms also suffered from more types of difficulties. Ninety per cent of patients said that a particular person provided them with psychological support or some source of joy and happiness. Only 30% of patients claiming someone providing psychological support reported help from ‘friends or acquaintances’ and other ‘supporters’, while 60% of those claiming a source of joy and happiness described ‘learning or education’ and ‘activities’. Patients describing more categories had higher levels of hope. All patients described desires and wishes in some form, with health of family members ranking the highest. Patients using computer‐assisted communications tended to have more categories of people providing psychological support, joys and happiness, and desires and wishes.
Discussion and conclusions: These results show that care should focus both on alleviating difficulties and on providing effective psychological support. This double‐pronged approach has the potential to help patients maintain or recover a life‐sustaining sense of hope. Furthermore, improving support systems for families who care for IMV ALS people will help not only the families themselves, but also the patients who perceive themselves as burdens.
P26 SURVIVAL OF ALS PATIENTS ON HOME MECHANICAL VENTILATION
Laub M1, Midgren B2
1Respiratory Centre East, University Hospital, Copenhagen, Denmark, 2Department of Lung Medicine, University Hospital, Lund, Sweden
E‐mail address for correspondence: [email protected]
Background: Several studies have provided convincing evidence that non‐invasive ventilation can prolong survival in patients with ALS.
Objectives: We wished to present survival data in ALS patients on home mechanical ventilation (HMV) and compare the probability of survival with other patients on HMV.
Methods: From the nationwide Swedish Home Mechanical Ventilation Register we have prospective data on patients on HMV over nearly 10 years. The patients represent a broad diagnostic spectrum that enables us to compare data in a large number of patients. Also, we present two cases of patients on HMV who have survived more than seven years after disease onset.
Results: The survival of 1474 patients on HMV is displayed; ALS patients make up 148 (10%) of these cases. The survival after starting ventilation in the ALS group was significantly poorer compared to all other groups (p<0.001). ALS survival rates were 45% at 1 year, 19% at 2 years and 6% at 5 years. In contrast to what was seen in other groups, no specific patient‐related or healthcare system‐related factors contributed to a greater relative risk for death among ALS patients. The predominant cause of death was respiratory failure.
Discussion and conclusions: In ALS, previous studies have stated varying survival rates. In an early series of 101 patients, survival rates were 69% at two years and 33% at five years. These are considerably higher rates compared with more recent smaller studies and our report. Part of an explanation may be that all patients were ventilated via a tracheostomy, whereas this only applied to 5% of our ALS patients (at therapy launch). Trach‐ventilation per se may be easier to carry out in patients with severe paralyses, especially of bulbar nature. We also speculate that caregivers and relatives may be more reluctant to withdraw tracheostomy ventilation than non‐invasive ventilation. In another study including 122 patients, 38 used NIV more than four hours per day and had a survival rate after two years just below 20%, which is very close to the findings in our study. In a control group consisting of 52 patients who renounced ventilatory support, none were alive after two years.
In conclusion, even if HMV probably improves life expectancy in MND/ALS, survival is significantly poorer compared to all other patient groups on HMV.
P27 MANAGEMENT OF MND/ALS PATIENTS IN A SPECIALIZED RESPIRATORY CENTRE AND IN THE PRIMARY HEALTH SECTOR
Mortensen L, Laub M, Nyholm G
1Institution for Respiratory Disabled, Copenhagen, Denmark, 2Respiratory Centre East, University Hospital, Copenhagen, Denmark
E‐mail address for correspondence: [email protected]
Background: The number of ALS patients on respiratory support has been growing considerably during the last decade. After launch of non‐invasive or tracheostomy ventilation, the majority of the patients are discharged to their home. In some cases this is, however, not feasible. Care of ALS patients on long‐term mechanical ventilation in a hospital is for several reasons not an appropriate arrangement. Residence in a ventilator‐specialized institution appears to be a more satisfactory solution.
Objectives: We wish to describe the organizing initiatives and the step‐wise intensification in treatment leading to home mechanical ventilation (HMV). Furthermore, we wish to relate the collaboration with a specialized nursing home for long‐term mechanical ventilated patients.
Methods: We conducted a survey of the management of respiratory services in ALS in eastern Denmark covering a population of 2.7 million.
Results: Based on intermittent interdisciplinary meetings with local ALS teams, patients are referred to Respiratory Centre East. In recent years we have in this way received about 35 ALS patients annually. Initially all patients embark on intermittent day‐time continuous positive airway pressure (CPAP) and if the vital capacity is below 50% of expected, nocturnal cardio‐respiratory monitoring is performed. The ALS patients are encouraged to try nocturnal non‐invasive ventilation (NIV) when respiratory failure is imminent. Transition to tracheostomy ventilation when patients are ventilated more than 12–14 hours per day has become more common.
Discussion and conclusions: The social welfare system in Denmark differs from that in most other countries because almost all social benefits are free, as everything is covered via income tax. ALS patients have different options for assistance: aids and appliances, personal helpers, speech therapy and physiotherapy. Respite care or moving to a rest home, nursing home or hospice is another option.
If the patient has embarked on non‐invasive or tracheostomy ventilation the Institution for Respirator Patients (IRP) may be an option. The residence may be permanent or temporary until e.g. a team of personal helpers has been trained. The ALS patients in IRP are most often patients with a permanent residence.
In conclusion, respiratory services in ALS are challenging and may demand considerable resources. IRP in many cases relieves the pressure on more expensive beds in the hospitals and offers the patient an existence under homelike conditions.
P27A VARIABILITY IN RESPIRATORY PHYSICAL THERAPY IN ALS PATIENTS
VALERA F1, MARÍN S2, MINAYA FJ1, VALERA MA3, MASCIAS J2, SALAS T4, MORA J2
1Department of Physical Therapy; 2Department of Neurology; Neuromuscular Research Unit, Hospital Carlos III, Madrid, Spain; 3Department of Pedagogy, Institute of Education, Murcia, Spain; 4FUNDELA; Foundation of Development and Research in ALS, Madrid, Spain.
E‐mail address for correspondence: [email protected]
Background: Impairments related to ALS are defined by loss of strength, respiratory insufficiency, spasticity, loss of fine motor coordination, and speech and swallowing difficulties. Physical therapy can potentially maximize muscle and respiratory function prolonging survival, independence in ADL and quality of life. This study is part of a larger study of “Clinical Practice Guidelines based Evidence for patients with ALS/MND”.
Objectives: The purpose of the study was to analyze variability in respiratory physical therapy based on specific respiratory muscle training in ALS patients.
Methods:Study population. The inclusion criterion was: Physiotherapist (PT) with clinical experience in ALS patients. As heterogeneous a group as possible was formed, by taking urbanization, practice type, gender and age into account.
Qualitative Research. A telephone survey was used. Two trained interviewers conducted the semi‐structured interviews using open‐ended questions. An interview guide was used, which was developed and adapted through pilot interviews. The interviews focused on the type of specific respiratory muscle training given to ALS patients, frequency, time of session, treatments modalities (home exercise and supervised treatment). For example, questions from the PT interview guide were: what type of treatment do you give to ALS patients? Do you include specific respiratory muscle training at home? Do you use techniques of manual therapy?
Results: For this study, seven PT were interviewed (4 males and 3 females), mean age 28 (SD:4) years and four years of clinical experience in ALS patients. 85.7% always performed respiratory physical therapy in ALS patients. Of these, all physiotherapists carried out a supervised treatment and 71.4% also included a domiciliary treatment (home exercise).
In the supervised treatment, mean time dedicated to specific respiratory muscle was 19 minutes (mode 10 minutes). Thirty three percent performed the treatment three times a week and 66.6% twice.
In the home exercise programme, all of the physiotherapists recommended that respiratory exercise (expiratory and inspiratory) should be performed seven times a week, nevertheless a large variation exists as far as the number of times of day (14.3% recommend once; 28.6% twice and 57% three times) and mean time dedicated to specific respiratory muscle (mean 14 minutes; range 7–20 minutes).
Conclusions:
85.7% of physiotherapists performed specific respiratory muscle training in ALS patients but there was a large variation in the different parameters used.
It is necessary to include the parameters of the treatment of respiratory physical therapy in the Clinical Practice Guidelines.
P27B HOW IS RESPIRATORY PHYSICAL THERAPY TREATMENT USED IN ALS PATIENTS?
MARÍN S2, VALERA F1, MINAYA FJ1, VALERA MA3, MASCIAS J2, SALAS T4, MORA J2
1Department of Physical Therapy; 2Department of Neurology; Neuromuscular Research Unit, Hospital Carlos III, Madrid, Spain; 3Department of Pedagogy, Institute of Education, Murcia, Spain; 4FUNDELA; Foundation of Development and Research in ALS, Madrid, Spain.
E‐mail address for correspondence: [email protected]
Background: In a changing health care world with increasing emphasis on patient participation and shared decision making, patients' beliefs and ideas strongly influence the process and outcomes of treatments.
ALS patients' experiences may provide unexpected insights and may help in the understanding of clinical decision making and the various alternative and complementary treatment methods.
Objectives: The purpose of this study was to identify the respiratory physical therapy care, from point of view of ALS patients.
Methods:Study population. The inclusion criterion was: Diagnosis of probable or definite ALS (El Escorial diagnostic criteria) in initial phase. ALS patients from Hospital Carlos III Madrid were included in the study.
Qualitative Research. A telephone survey was used. Two trained interviewers conducted the semi‐structured interviews using open‐ended questions. An interview guide was used, which was developed and adapted through pilot interviews. The interviews focused on type of specific respiratory muscle training in ALS patients, frequency, time of session, treatments modalities (home exercise and supervised treatment). For example, questions from the patients interview guide were: Do you perform respiratory physical therapy? How is the specific respiratory muscle training? Who explained the respiratory physical therapy?
Results: In this study, 20 patients were evaluated (15 males and 5 females). Only 40% of the patients indicated that they carried out the respiratory physical therapy programme. Of these, they received a treatment based on techniques of manual therapy and supervised exercises by a physiotherapist. They also carried out a treatment of domiciliary exercises taught by their physiotherapist but they did not know exactly how to perform them. Sixty percent didn't even know the time of the treatment session supervised by a physiotherapist. The treatment of this type is once a week in 50% of the cases.
Sixty percent didn't know or didn't remember the length of time they they should carry out the programme of domiciliary exercises. They didn't know or didn't remember how many days a week they should be carried out or how many times a day. They indicated that they carried out inspiratory (40%) and expiratory (30%) exercises and exercises of upper limb (16.7%). Fifteen percent of people indicated that the exercises have not been modified since the beginning and that they get bored.
Conclusions:
The supervision of a physiotherapist is necessary to guarantee that the patient carries out the respiratory physical therapy and home exercise.
ALS patients do not carry out physiotherapy specific respiratory muscle training correctly. They do not know or do not remember the parameters.
P28 THE SIGNIFICANCE OF RESPIRATORY PHYSICAL THERAPY (RPT) FOR ALS PATIENTS WITH TPPV
Kasahara Y, Michiyama N, Hino H, Ogawa K, Nagao M, Kawata A, Hayashi H
Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
E‐mail address for correspondence: [email protected]
Background: In the course of ALS, in addition to limb muscle weakness, respiratory muscle weakness inevitably occurs, leading to ventilatory impairment.
In addition to the physical therapies for ALS patients to keep the power and the range of motion for limbs, we have performed respiratory physical therapy (RPT) for them to maintain the power and flexibility of the respiratory muscles and promote the removal of sputa even after they use tracheostomy positive pressure ventilation (TPPV). The RPT includes manual breathing assistance, the range of motion exercise of the thorax, posterior lifts, mobilization of the shoulder girdle and squeezing. To continue the RPT at home, we have taught the methods to the patient's caregivers, home helpers and visiting nurses.
Objectives: We performed this study to evaluate the significance of continuous RPT on ALS patients with TPPV.
Methods: A total of 21 patients (12 men and 9 women) with TPPV, who had taken RPT more than twice a week, were included in this study. We reviewed the medical records of the patients and also conducted a questionnaire survey of the patients and their family members.
Results: The clinical parameters of ALS patients with TPPV who took RPT were as follows: mean age, 63.2±10.4 years; duration of illness, 10.6±7.4 years (3–25.4 years); the duration of the use of TPPV, 5.6±5.2 years (0.4–19.6 years); the treatment period of RPT after TPPV, 4.0±3.1 years (0.2–11.0 years). The main performers of RPT were the visiting nurses sent from home nursing stations, followed by the physiotherapists. The patients' comments on RPT were as follows: it makes them feel comfortable, relaxed and makes it easy to remove phlegm. Eleven patients out of 14, who can be transferred to a wheel chair, went out from once a week to several times a year for a change or to attend some social activities.
Discussion and conclusions: Although we cannot show the effectiveness of RPT on ALS patients with TPPV by any clinical parameters, squeezing seems to promote the removal of sputa and respiratory muscle stretch gymnastics on chest wall mobility seems to maintain the flexibility of the chest wall even under TPPV care. In addition, RPT is a kind of relaxation method through direct touch of the patient's body, and may contribute to mental support, palliative care and the establishment of the confidential relationship between the performers and the patients. Moreover, the stabilization of mental and physical status by RPT may partly enable the patients to go out.
P29 RISK OF ASPIRATION PNEUMONIA POSED BY ORAL FOOD INTAKE FOR ALS PATIENTS WITH TRACHEOSTOMY AND ARTIFICIAL VENTILATION
Yakuwa H, Ohta A, Yarimizu H, Ikeno C, Mito M, Sasaki Y, Yoshino H
Yamagata Tokushukai Hospital, Yamagata, Japan
E‐mail address for correspondence: [email protected]
Background: Patients with ALS usually stop oral food intake and use PEG when they receive a tracheostomy, because swallowing becomes more difficult than before, and may lead to aspiration pneumonia. In addition, oral food intake may be a burden for caregivers.
Objectives: We assessed the risk of aspiration pneumonia posed by oral food intake for ALS patients with tracheostomy and artificial ventilation.
Methods: We enrolled nine hospitalized ALS patients with 24 hour artificial ventilation with tracheostomy from September 2004 to April 2006. Among these nine, three patients maintained oral intake even after tracheostomy, three stopped oral intake after tracheostomy, and the other three sometimes had oral intake only some of the time. Fever of more than 38oC and use of antibiotics due to respiratory inflammation were considered as an event. We compared the frequency of events during periods of oral food intake with periods when oral food intake was stopped.
Results: During a period of oral intake totalling 65 months, events were observed 14 times (0.22 per month). During the period when oral intake was stopped (72 months in total), events were observed 12 times (0.16 per month).
Conclusion: Although respiratory inflammation may happen slightly more frequently, the enjoyment of taking food makes patients feel happy. Oral food intake should be considered to improve quality of life for ALS patients with tracheostomy and artificial ventilation.
P30 USEFULNESS OF THE TRACHEOESOPHAGEAL DIVERSION AND LARYNGOTRACHEAL SEPARATION FOR INTRACTABLE ASPIRATION IN ALS
Mita S, Yamaguchi K, Samejima Y, Imamura S
Department of Neurology, Kumamoto Saishunso Hospital, Koshi, Kumamoto, Japan
E‐mail address for correspondence: [email protected]
Background: Swallowing difficulties sometimes result in aspiration pneumonia, which is a major life‐threatening problem for patients with ALS and those with other neurological diseases. Therefore, great effort is necessary to protect patients from aspiration pneumonia. Management of patients with aspiration requires discontinuation of oral intake. Traditional surgical management has often relied on tracheostomy. This medical management is usually effective, but requires significantly more care in controlling salivary secretion because it increases the risk of aspiration. Frequent suctioning with an aspirator by medical staff and caregivers is necessary and time‐consuming. This causes fatigue among patients, caregivers, and nurses alike. In cases where there is a failure to control aspiration, further surgical procedures may be necessary, such as laryngotracheal separation (LTS), which is a modification of tracheoesophageal diversion (TED), or laryngectomy. These procedures separate the airway and digestive systems and can effectively eliminate intractable aspiration. To most neurologists, little is known about LTS/TED in detail. Very few studies have evaluated the usefulness of LTS/TED or patients' and families' satisfaction with it. Indeed, as far as we could determine, it has not been recognized as a common procedure.
Objective: We investigated changes in medical management after LTS/TED had been performed, including feeding conditions and whether or not patients, family caregivers, and medical staff are truly satisfied with the outcome.
Methods: We performed LTS/TED for intractable aspiration in seven patients with ALS and nine patients with other neurological diseases. Most of the subjects had already received a tracheostomy, and all the patients with ALS had tracheostomy positive pressure ventilation. However, they remained at risk of intractable aspiration, had experienced frequent suctioning of aspiration material, and could not eat. After LTS/TED, we evaluated the efficacy of the therapy.
Results: In all cases, LTS/TED was performed safely within three hours and without any complications. After LTS/TED, there was no aspiration of saliva in any of the patients. In most subjects the frequency of suctioning by medical staff and caregivers was much reduced. The frequency of fever/infection by aspiration was also extremely reduced. Follow‐up study demonstrated that complete control over aspiration was achieved in all of the patients. Six of them were completely self‐sufficient in their ability to eat. Some of the other patients were at least able to enjoy taste. Nutritional status was significantly improved. All the patients, family caregivers, and medical staff involved in this study were satisfied with the outcome.
Conclusions: These results indicate that LTS/TED is a very useful procedure in several aspects: it benefits patients who have a fear of aspiration, it reduces the burden on patients and family caregivers, it promotes their quality of life and it limits the aspiration‐associated demands on medical staff.
P31 ANOTHER USEFUL TUBE FEEDING METHOD FOR PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS: INTERMITTENT ORAL CATHETERIZATION (IOC)
Nozaki SN1, Umaki YU1, Matsumura TM2
1Tokushima National Hospital, Tokushima, Japan, 2Toneyama Nationak Hoapital, Tokushima, Japan
E‐mail address for correspondence: [email protected]
Background: For tube feeding of ALS patients, we have used continuous nasal catheterization or percutaneous endoscopic gastrostomy in Japan. It has been highlighted that continuous nasal catheterization has some problems as a long‐term tube feeding method (1).
On the other hand, there are many complications in the management of percutaneous endoscopic gastrostomy (2).
IOC has been used as supplemental tube feeding for dysphagic children and cerebrovascular disease patients (3). We introduced IOC for patients with amyotrophic lateral sclerosis (ALS) for long‐term tube feeding.
Objective: To investigate the progress of ALS patients fed via IOC.
Methods: We introduced IOC to 17 patients with ALS (58–82 years of age). The duration between the onset of ALS and introduction of IOC was 7–59 months. Before the introduction of IOC, four patients were able to eat modified food, 13 patients found oral intake very difficult or impossible, and three patients had not been given a diagnosis at the introduction of IOC.
At every meal time, the patient or caregiver inserted the catheter orally to the stomach and pulled out the tube after feeding.
Results: IOC was introduced before the start of ventilation in 10 patients and after the start of ventilation in seven patients. Their forced vital capacity was 30–60%.
We could introduce IOC for patients under non‐invasive positive pressure ventilation or tracheostomy positive pressure ventilation. The patients continued IOC for 3–36 months. Five patients who died during follow‐up had continued with IOC nutrition until the end of their life. Three patients discontinued IOC because of tracheotomy, fatigue and increase of sputum. We found no complication related to IOC.
It took less than 10 sessions of training to master the skill of IOC. Almost all the patients and families felt comfortable about IOC.
Conclusion: We could introduce IOC to patients with mild to advanced stages of dysphagia, regardless of respiratory function and whether they had been informed of a diagnosis of ALS.
IOC is one of the useful tube feeding methods for ALS patients.
References
- Miller RG, Rosenberg JA, Gelinas DF, et al. Neurology 1999;52:1311–23.
- Gomes GF, Pisani JC, Macedo ED, et al. Current Opinion in Clinical Nutrition & Metabolic Care 2003;6:327–33.
- Campell-Taylor. Dysphagia 1998;2:220–1.
P32 THE SAFETY AND EFFICACY OF PEG TUBE INSERTION IN ALS/MND PATIENTS IN A TERTIARY INSTITUTION
Erjavec S, Brenninger V, Tarrant ME, McCoy S, Reynolds T, Hannan E, Walters S, Joffe D, Hibbert M, Barckley K, Zeman B, Rowe DB
Departments of Neurology, Allied Health, Thoracic Medicine and Rehabilitation, Royal North Shore Hospital, Sydney, Australia
E‐mail address for correspondence: [email protected]
Background: Percutaneous endoscopic gastrostomy (PEG) in patients with ALS/MND has been shown to improve quality of life and stabilizes or increases weight among ALS patients. Furthermore, life expectancy is prolonged by provision of adequate hydration and nutrition in patients with progressive malnutrition secondary to dysphagia. PEG insertion can be risky, particularly in the patient with borderline respiratory function. This retrospective study reviews tube placement outcomes in 39 MND/ALS outpatients seen by a multi‐disciplinary team during a six‐year period from 1999 to early 2006 at a tertiary hospital in Sydney, Australia.
Objectives: To determine rate of complications associated with early insertion of PEG tubes by reviewing outcomes based on measures of respiratory function, nutritional status and a decline in functional speech and swallowing. This review will enable us to inform patients with bulbar onset ALS regarding benefits of PEG tube placement and possible complications. Furthermore, it would allow us to review our current practice of early intervention, particularly in bulbar onset of ALS/MND.
Methods: A retrospective review of all PEG tube placements in an ALS/MND outpatients' clinic between September 1999 and March 2006 was completed. Data were collected from medical records, describing patients' nutritional status, respiratory function as well as speech and swallowing function at the time of tube placement. These data were related to trends seen at the initial assessment of nutritional, respiratory and bulbar function as seen in the outpatients' ALS clinic. All tubes were placed in the tertiary hospital with support of non‐invasive ventilation. Full intensive care support was used if required.
Results: There were two serious complications associated with tube placement in 39 consecutive patients, with peritonitis secondary to leakage of stomach contents at the time of insertion. Both patients survived with early laparotomy, repair of the site of leakage, and replacement of gastrostomy tube at the time of laparotomy. Five patients required the replacement of PEG tubes due to mechanical failure of the tubes. There were no deaths related to tube insertion. Rationale for early tube placement was influenced by the rate of weight loss, reduced food intake, inadequate fluid intake and excessive meal times.
Conclusions: Our data indicate that early PEG tube placement in MND/ALS patients is safe and effective. These data support early tube placement with a focus on using PEG tube for survival and eating for pleasure. PEG insertion is a safe procedure, particularly when supported with non‐invasive ventilation.
P33 CHARACTERIZATION AND FOLLOW‐UP OF PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS BY SWALLOWING SCINTIGRAPHY
Grosso M1, Bongioanni P2, Fattori B3, Mattone V1, Rizza R1, Cingolani C1, Nacci A1, Ursino F3, Rossi B2, Mariani G1
1Regional Centre of Nuclear Medicine, 2Department of Neuroscience, Neurorehabilitation Unit, 3Department of Neuroscience, ENT Unit, University of Pisa, Pisa, Italy
E‐mail address for correspondence: [email protected]
Background: Deglutition deficit is one of the most frequent and invalidating symptoms reported by amyotrophic lateral sclerosis (ALS) patients. Quantitative assessment methods for severity of oropharyngeal dysphagia and response to therapy are lacking. Oro‐pharyngo‐oesophageal scintigraphy (OPES) allows a functional and semi‐quantitative study of different swallowing phases.
Objectives: The aim of our study was to assess swallowing in a population of ALS patients by means of OPES at baseline and during follow‐up.
Methods: We evaluated 38 patients (18 women and 20 men; mean age ± SD, 64.5±10.4 years) diagnosed as having definite or probable ALS according to the revised El Escorial criteria. Patients, clinically assessed through the ALS Functional Rating Scale (ALSFRS), underwent OPES with 99mTc‐nanocolloid by using a liquid and a semi‐solid bolus at time of diagnosis, three and six months thereafter. We analysed the following parameters: oral transit time (OTT), pharyngeal transit time (PTT), oesophageal transit time (ETT), oro‐pharyngeal retention index (OPRI), oesophageal retention index (ERI), and oesophageal emptying rate (EER).
Results: OPES performed with semi‐solid bolus identified a higher proportion of abnormal values for swallowing parameters than that with liquid bolus. By grouping ALS patients into classes according to their bulbar ALSFRS scores, we found significantly increased OTT (p<0.004), PTT (p<0.02) and OPRI (p<0.0003) values in patients with more severe bulbar involvement. In the follow‐up a high correlation was observed between increased OPRI values with semi‐solid bolus and disease progression (p<0.0003). OPES was useful in determining the exact bolus percentage inhaled into tracheo‐bronchial branch.
Discussion: Our findings show that disease worsening leads to enhanced OTT, PTT and OPRI values, whereas ETT, ERI and EER values do not change significantly. Such data further support the neurogenic component of dysphagia in ALS patients who present typical derangement of swallowing oro‐pharyngeal stage rather than that of the oesophageal stage.
In summary, OPES with 99mTc‐nanocolloid is a simple, inexpensive, non‐invasive and reliable technique that allows better clinical definiton of deglutition deficits and accurate follow‐up of ALS patients, by supplying semi‐quantitative data on swallowing performances and percentage of bolus inhaled.
P34 LARYNGOLOGICAL DETECTION OF PRECLINICAL BULBAR SIGNS IN PATIENTS WITH LIMB ONSET AMYOTROPHIC LATERAL SCLEROSIS
Tomik J1, Tomik B2, Wiatr M1, Partyka D2, Ostrowska M2, Skladzien J1, Szczudlik A2
1ENT Department, Medical College Jagiellonian University, Krakow, Poland, 2Department of Neurology, Medical College Jagiellonian University, Krakow, Poland
E‐mail address for correspondence: [email protected]
Introduction: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder of unknown aetiology. While multidisciplinary medical teams may be involved in the diagnostic process, the participation of otolaryngologists in evaluation of ALS patients has been seldom reported. We have previously stressed that hyper‐/hyponasality, articulation defects, voice harshness and breathiness as well as swallowing difficulties should all be viewed as possible early signs of ALS and may allow the otolaryngologist to be the primary diagnostician.
Although the first clinical presentation of ALS is usually either limb or bulbar level only, some data suggest that degeneration of motor neurons of motor cortex, brainstem, and spinal cord may occur simultaneously in ALS individuals.We hypothesize that careful laryngological examination can reveal signs of early dysfunction of the vocal cords in ALS individuals with limb onset only, and thus may indicate the early presentation of bulbar dysfunction.
Objective: To test the hypothesis that preclinical signs of bulbar dysfunction occur in limb onset ALS individuals, via assessing vocal cord changes using fibreoscopic and/or videostroboscopic examination.
Material and methods: Thirty‐four selective limb onset ALS individuals, diagnosed according to El Escorial criteria in the Krakow MND Centre of Jagiellonian University, in 2005–2006, were enrolled in the study. None of them presented with any bulbar symptoms or signs in the neurological examination. To detect vocal cord abnormalities, the standard laryngological examination, as well as the fibreoscopic and/or videostroboscopic examination, was performed in the Department of Otolaryngology, Head and Neck Surgery of Jagiellonian University in Krakow.
Results: There were no abnormalities on the standard laryngological inspection.The fibreoscopic examination revealed some signs of the early dysfunction of vagus nerve/s in 25 out of 34 patients: slowness of one/both vocal folds, lack of complete closure during phonation, unilateral decrease of vocal fold tension with limited abduction. The videostroboscopic examination confirmed the fibreoscopic findings in limb onset ALS cases and has in addition revealed: vocal fold bowing, decreased abduction of the true vocal fold and mucus pooling in some cases. In both examinations, incomplete vocal fold closure was the most frequent observation.
Conclusions: We report dysfunction of vagus nerve/s in most limb onset ALS cases studied demonstrating a clinically ‘silent’ bulbar dysfunction in these patients. Our results support the hypothesis that degeneration of all the motor neurons in ALS may occur simultaneously, with unknown factors determining the different first clinical presentation/type of disease onset in each patient.
Acknowledgement: This work was supported by grant Jagiellonian 501/NKL/194/L.