Impact of an early respiratory care programme with non-invasive ventilation adaptation in patients with amyotrophic lateral sclerosis.

BACKGROUND AND PURPOSE: Forced vital capacity (FVC) <80% is one of the key indications for starting non-invasive ventilation (NIV) in amyotrophic lateral sclerosis (ALS). It was hypothesized that a very early start of NIV could lengthen the free interval before death compared to later-start NIV; as a secondary outcome, the survival rate of patients on NIV without tracheotomy was also evaluated. METHODS: This retrospective study was conducted on 194 ALS patients, divided into a later group (LG) with FVC <80% at NIV prescription (n = 129) and a very early group (VEG) with FVC ≥80% at NIV prescription (n = 65). Clinical and respiratory functional data and time free to death between groups over a 3-year follow-up were compared. RESULT: At 36 months from diagnosis, mortality was 35% for the VEG versus 52.7% for the LG (P = 0.022). Kaplan-Meier survival curves adjusted for tracheotomy showed a lower probability of death (P = 0.001) for the VEG as a whole (P = 0.001) and for the non-bulbar (NB) subgroup (P = 0.007). Very early NIV was protective of survival for all patients [hazard ratio (HR) 0.45; 95% confidence interval (CI) 0.28-0.74; P = 0.001] and for the NB subgroup (HR 0.43; 95% CI 0.23-0.79; P = 0.007), whilst a tracheotomy was protective for all patients (HR 0.27; 95% CI 0.15-0.50; P = 0.000) and both NB (HR 0.26; 95% CI 0.12-0.56; P = 0.001) and bulbar subgroups (HR 0.29; 95% CI 0.11-0.77; P = 0.013). Survival in VEG patients on NIV without tracheotomy was three times that for the LG (43.1% vs. 14.7%). CONCLUSION: Very early NIV prescription prolongs the free time from diagnosis to death in NB ALS patients whilst tracheotomy reduces the mortality risk in all patients.

Inhaler technique knowledge and skills before and after an educational program in obstructive respiratory disease patients: A real-life pilot study.

INTRODUCTION AND OBJECTIVE: Patients present poor knowledge and skills about their respiratory disease and inhaler device. We aimed to: (1) evaluate COPD and asthmatic patients... ability to manage inhaled drugs (2) identify differences among devices and (3) correlate clinical data with patient ability. MATERIAL AND METHODS: Patients (n=134) admitted for pulmonary rehabilitation (PR) were given an ad-hoc questionnaire covering 0% as the worst and 100% the best value of global ability (indicating the sum of knowledge and skills in managing inhaled drugs) at baseline (T0) and discharge (T1). Educational program was provided during PR. Setting of rehabilitation, age, sex, diagnosis, spirometry, CIRS score, level of autonomy to use medications, if na..ve about PR, educational level, and number/type of prescribed inhaled drugs were recorded. RESULTS: Most patients used 1 drug while 37% used 2 drugs. DPIs were the main device prescribed. At baseline, patients... mean level of knowledge and skills were 73% and 58%, respectively. There was a significant difference in level of skills (p=0.046) among device families, DPIs resulting worst and pMDIs best. Global ability, skills and knowledge improved after educational support (p<0.001) but did not reach the optimal level, 88%, 87% and 89%, respectively. Baseline global ability was positively correlated to female gender, younger age, previous PR access, outpatient status, higher education level and GOLD D class. CONCLUSIONS: At hospital admission, global ability was not optimal. Education may improve this, irrespective of the type of device used, in particular in male, elderly, na..ve to PR, low educational level patients.

Clusters of individuals recovering from an exacerbation of chronic obstructive pulmonary disease and response to in-hospital pulmonary rehabilitation.

INTRODUCTION AND OBJECTIVES: Due to the present low availability of pulmonary rehabilitation (PR) for individuals recovering from a COPD exacerbation (ECOPD), we need admission priority criteria. We tested the hypothesis that these individuals might be clustered according to baseline characteristics to identify subpopulations with different responses to PR. METHODS: Multicentric retrospective analysis of individuals undergone in-hospital PR. Baseline characteristics and outcome measures (six-minute walking test - 6MWT, Medical Research Council scale for dyspnoea -MRC, COPD assessment test -CAT) were used for clustering analysis. RESULTS: Data analysis of 1159 individuals showed that after program, the proportion of individuals reaching the minimal clinically important difference (MCID) was 85.0%, 86.3%, and 65.6% for CAT, MRC, and 6MWT respectively. Three clusters were found (C1-severe: 10.9%; C2-intermediate: 74.4%; C3-mild: 14.7% of cases respectively). Cluster C1-severe showed the worst conditions with the largest post PR improvements in outcome measures; C3-mild showed the least severe baseline conditions, but the smallest improvements. The proportion of participants reaching the MCID in ALL three outcome measures was significantly different among clusters, with C1-severe having the highest proportion of full success (69.0%) as compared to C2-intermediate (48.3%) and C3-mild (37.4%). Participants in C2-intermediate and C1-severe had 1.7- and 4.6-fold increases in the probability to reach the MCID in all three outcomes as compared to those in C3-mild (OR = 1.72, 95% confidence interval [95% CI] = 1.2 - 2.49, p = 0.0035 and OR = 4.57, 95% CI = 2.68 - 7.91, p < 0.0001 respectively). CONCLUSIONS: Clustering analysis can identify subpopulations of individuals recovering from ECOPD associated with different responses to PR. Our results may help in defining priority criteria based on the probability of success of PR.

Prevalence of exercise-induced oxygen desaturation after recovery from SARS-CoV-2 pneumonia and use of lung ultrasound to predict need for pulmonary rehabilitation.

BACKGROUND: Persistence of breathlessness after recovery from SARS-CoV-2 pneumonia is frequent. Recovery from acute respiratory failure (ARF) is usually determined by normalized arterial blood gases (ABGs), but the prevalence of persistent exercise-induced desaturation (EID) and dyspnea is still unknown. METHODS: We investigated the prevalence of EID in 70 patients with normal arterial oxygen at rest after recovery from ARF due to COVID-19 pneumonia. Patients underwent a 6-min walking test (6MWT) before discharge from hospital. We recorded dyspnea score and heart rate during 6MWT. We also investigated the possible role of lung ultrasound (LU) in predicting EID. Patients underwent a LU scan and scores for each explored area were summed to give a total LU score. RESULTS: In 30 patients (43%), oxygen desaturation was >4% during 6MWT. These patients had significantly higher dyspnea and heart rate compared to non-desaturators. LU score >8.5 was significantly able to discriminate patients with EID. CONCLUSION: In SARS-CoV-2 pneumonia, ABGs at discharge cannot predict the persistence of EID, which is frequent. LU may be useful to identify patients at risk who could benefit from a rehabilitation program.

Clinical standards for diagnosis, treatment and prevention of post-COVID-19 lung disease.

BACKGROUND: The aim of these clinical standards is to provide guidance on 'best practice' care for the diagnosis, treatment and prevention of post-COVID-19 lung disease.METHODS: A panel of international experts representing scientific societies, associations and groups active in post-COVID-19 lung disease was identified; 45 completed a Delphi process. A 5-point Likert scale indicated level of agreement with the draft standards. The final version was approved by consensus (with 100% agreement).RESULTS: Four clinical standards were agreed for patients with a previous history of COVID-19: Standard 1, Patients with sequelae not explained by an alternative diagnosis should be evaluated for possible post-COVID-19 lung disease; Standard 2, Patients with lung function impairment, reduced exercise tolerance, reduced quality of life (QoL) or other relevant signs or ongoing symptoms ≥4 weeks after the onset of first symptoms should be evaluated for treatment and pulmonary rehabilitation (PR); Standard 3, The PR programme should be based on feasibility, effectiveness and cost-effectiveness criteria, organised according to local health services and tailored to an individual patient's needs; and Standard 4, Each patient undergoing and completing PR should be evaluated to determine its effectiveness and have access to a counselling/health education session.CONCLUSION: This is the first consensus-based set of clinical standards for the diagnosis, treatment and prevention of post-COVID-19 lung disease. Our aim is to improve patient care and QoL by guiding clinicians, programme managers and public health officers in planning and implementing a PR programme to manage post-COVID-19 lung disease.

Advanced COPD patients under home mechanical ventilation and/or long term oxygen therapy: Italian healthcare costs.

BACKGROUND AND AIM: Little information is available on healthcare costs for patients with very severe chronic obstructive pulmonary disease. The aim of the current work was to evaluate Italian healthcare costs in these patients. METHODS: Prospective 1-year analysis was assessed in three subgroups of patients; non-invasively ventilated (n=30); invasively-ventilated (n=12) and on long-term oxygen therapy (n=41). Acute costs for care were a sum of fees for doctor's consultations, admissions to hospital (ward and intensive care units) and emergency drugs. Chronic costs were the sum of costs for pharmacotherapy and home ventilation and/or oxygen care. RESULTS: Mean cost/day/patient was 96 +/- 112 Euro (range 9-526 Euro), with acute costs accounting for 72% and chronic costs for 28% of the total cost burden, with no significant differences in costs associated with the three subgroups. Acute costs had a non-normal distribution (range 0 to 510 Euro) being cost for hospitalisation the highest cost burden with more than 30% of acute care costs attributed to only a small segment of patients. Chronic care costs were also unevenly distributed among the various groups (ANOVA p = 0.006), being home oxygen supply the highest cost burden. CONCLUSIONS: The current Health Care System is in urgent need for a reassessment of the high cost burden associated with hospitalisations and home oxygen supply.

Last 3 months of life in home-ventilated patients: the family perception.

We studied the family's perception of care in patients under home mechanical ventilation during the last 3 months of life. In 11 respiratory units, we submitted a 35-item questionnaire to relatives of 168 deceased patients exploring six domains: symptoms, awareness of disease, family burden, dying, medical and technical problems. Response rate was 98.8%. The majority of patients complained respiratory symptoms and were aware of the severity and prognosis of the disease. Family burden was high especially in relation to money need. During hospitalisation, 74.4% of patients were admitted to the intensive care unit (ICU). 78 patients died at home, 70 patients in a medical ward and 20 in ICU. 27% of patients received resuscitation manoeuvres. Hospitalisations and family economical burden were unrelated to diagnosis and mechanical ventilation. Families of the patients did not report major technical problems on the use of ventilators. In comparison with mechanical invasively ventilated patients, noninvasively ventilated patients were more aware of prognosis, used more respiratory drugs, changed ventilation time more frequently and died less frequently when under mechanical ventilation. We have presented good points and bad points regarding end-of-life care in home mechanically ventilated patients. Noninvasive ventilation use and diagnosis have impact on this burden.

Does timing of initiation influence acceptance and adherence to NIV in patients with ALS?

BACKGROUND: The role of nocturnal non invasive ventilation (NIV) to prolong tracheostomy-free survival, is still controversial in amyotrophic lateral sclerosis (ALS) patients and the best timing to initiate NIV is unclear. OBJECTIVE: As NIV acceptance and adherence can be influenced by many factors, we aimed to compare immediate acceptance and short-term NIV adherence between NIV initiated very early and NIV initiated later. METHODS: This is a post hoc analysis of our previous cohort retrospective study of 88 ALS patients: 53 under later NIV (late group - LG) (forced vital capacity [FVC]<80% pred. at NIV prescription) and 35 under very early NIV (very early group - VEG) (FVC>80%). We compared hours of NIV use as immediate acceptance of NIV (use ≥4h/night) and dherence at 4 months post-initiation (defined as use ≥4h/night or 120h/month). RESULTS: No differences were found between VEG and LG in use of NIV (>5h/night in both groups), immediate acceptance (85.7% vs. 85.0%, p=0.927) and short-term adherence (81.3% vs. 87.2%, p=0.469); 39.7% of patients increased their NIV use (35% by >60min/night). A decline in adherence was observed in 12.5% of patients irrespective of group affiliation. CONCLUSIONS: In ALS patients, initiation of very early NIV does not reduce its immediate acceptance or the short-term adherence. However, at least 1 in 10 patients may be at risk of reducing their adherence irrespective of early or late NIV prescription. As still under debate and not conclusive, further literature on early NIV benefit is welcomed.

Tele-assistance in chronic respiratory failure patients: a randomised clinical trial.

Chronic respiratory patients requiring oxygen or home mechanical ventilation experience frequent exacerbations and hospitalisations with related costs. Strict monitoring and care have been recommended. The aim of the present study was to primarily evaluate reduction in hospitalisations and, secondly, exacerbations, general practitioner (GP) calls and related cost-effectiveness of tele-assistance (TA) for these patients. A total of 240 patients (101 with chronic obstructive pulmonary disease (COPD)) were randomised to two groups: an intervention group entered a 1-yr TA programme while controls received traditional care. No anthropometric and clinical differences were found between groups both in baseline and in mortality (18% for TA, 23% for controls). Compared with controls, the TA group experienced significantly fewer hospitalisations (-36%), urgent GP calls (-65%) and acute exacerbations (-71%). Only COPD patients, as a separate group, had fewer hospitalisations, emergency room admissions, urgent GP calls or exacerbations. Each patient referred to staff a mean+/-sd 36+/-25 times. After deduction of TA costs, the average overall cost for each patient was 33% less than that for usual care. In chronic respiratory failure patients on oxygen or home mechanical ventilation, a nurse-centred tele-assistance prevents hospitalisations while it is cost-effective. The chronic obstructive pulmonary disease group seems to have a greater advantage from tele-assistance.

Episodic medical home interventions in severe bedridden chronic respiratory failure patients: a 4 year retrospective study.

BACKGROUND AND AIM: Home care for respiratory patients includes a complex array of services delivered in an uncontrolled setting. The role of a respiratory specialist inside the home healthcare team has been scarcely studied up to now. Our aims were to analyse the number and quality of episodic home visits performed by respiratory physicians to severe bedridden Chronic Respiratory Failure (CRF) patients, and also to evaluate the safety of tracheotomy tube substitutions at home. METHODS: 231 home interventions (59.8/year) in 123 CRF patients (59 males; age 63 +/- 17 y, 24 on oxygen therapy, 35 under non invasive mechanical ventilation, 46 under invasive ventilation, 74 with tracheostomy) located 35 +/- 16 km far from referred hospital, were revised in a period of 4 years (2005-2008). RESULTS: Chronic Obstructive Pulmonary Disease (COPD) (31%) and amyotrophic lateral sclerosis (ALS) (28%) were the more frequent diagnoses. Interventions were: tracheotomy tube substitution (64%) presenting 22% of minor adverse events and 1.4% of major adverse events; change or new oxygen prescription (37%); nocturnal pulsed saturimetric trend prescription (24%); change in mechanical ventilation (MV) setting (4%); new MV adaptation (7%). After medical intervention, new home medical equipment devices (oxygen and MV) were prescribed in 36% of the cases while rehabilitative hospital admission and home respiratory physiotherapy prescription was proposed in 9% and 6% of the cases respectively. Patient/caregiver's satisfaction was reported on average 8.48 +/- 0.79 (1 = the worst; 10 = the higher). The local health care system (HCS) reimbursed 70 euros for each home intervention. Families saved 42 +/- 20 euros per visit for ambulance transportation. CONCLUSIONS: Home visits performed by a respiratory physician to bedridden patients with chronic respiratory failure: 1. include predominantly patients affected by COPD and ALS; 2. determine a very good satisfaction to patients/caregivers; 3. allow money saving to caregivers; 4. are predominantly made up to change tracheotomy tube without severe adverse events.

Effectiveness of manual therapy in COPD: A systematic review of randomised controlled trials.

PURPOSE: Manual therapy (MT) has been proposed in pulmonary rehabilitation programmes for patients with chronic obstructive pulmonary disease (COPD), but an updated systematic review of the evidence is lacking. We aimed to systematically review the effectiveness of MT interventions, alone or added to exercise, on lung function, exercise capacity and quality of life in COPD patients, compared to other therapies (e.g. exercise alone) or no treatment. MATERIALS AND METHODS: We searched MEDLINE, EMBASE, Physiotherapy Evidence Database, and Cochrane Central Register of Controlled Trials databases, using the terms: COPD, manual therapy, manipulation, joint mobilisation, osteopathic manipulation. Only randomised controlled trials (RCT) were considered. RESULTS: Out of 555 articles screened, 6 fulfilled the inclusion criteria. The study designs were heterogeneous (with different intervention schedules) and there was a high risk of bias. No effect on lung function was found, while results on exercise capacity were contrasting. MT had no effect on quality of life, although valid measures were available only in one study. Only mild adverse events were reported. CONCLUSIONS: Few RCTs of poor methodological quality are available on the effects of MT in COPD. More and better quality RCTs are needed before this technique can be included in rehabilitation programmes for these patients.

Efficacy of pulmonary rehabilitation in chronic respiratory failure (CRF) due to chronic obstructive pulmonary disease (COPD): The Maugeri Study.

UNLABELLED: While the effectiveness of pulmonary rehabilitation (PR) in chronic obstructive pulmonary disease (COPD) is well established, its effectiveness in the most severe category of COPD, i.e. patients with chronic respiratory failure (CRF), is less well known. OBJECTIVE: To verify the effects of PR in patients with CRF, and compare the level of improvement with PR in these patients to that of COPDs not affected by CRF. METHODS: A multi-centre study was carried out on COPD patients with versus without CRF. The PR program included educational support, exercise training, and nutritional and psychological counselling. Lung function, arterial gases, walk test (6MWT), dyspnoea (MRC; BDI/TDI), and quality of life (MRF(28); SGRQ) were evaluated. RESULTS: Thousand forty seven consecutive COPD inpatients (327 with CRF) were evaluated. In patients with CRF all parameters improved after PR (0.001). Mean changes: FEV(1), 112 ml; PaO(2), 3.0 mmHg; PaCO(2), 3.3 mmHg; 6MWT, 48 m; MRC, 0.85 units; MRF(28) total score, 11.5 units. These changes were similar to those observed in patients without CRF. CONCLUSIONS: This study, featuring the largest cohort so far reported in the literature, shows that PR is equally effective in the more severe COPD patients, i.e. those with CRF, and supports the prescription of PR also in these patients.

Home mechanical ventilation patients: a retrospective survey to identify level of burden in real life.

BACKGROUND AND AIM: Home care for patients under home mechanical ventilation (HMV) may cause dramatic physical and economic burden in addition to the burden of time on family/caregivers and health care service (HCS) with difficult resource allocation decision-making. Our aims were: 1. To identify conditions causing major care burden in managing HMV patients according to family and payer's perspectives related to characteristics of the disease, dependency and accessibility; and 2. To find, if any, differences among diseases. METHODS: A questionnaire was sent to eight pulmonary centres to identify factors connected with the greater care burden. Retrospective data of 792 patients still alive and in HMV was reviewed. RESULTS: Compared to neuromuscular disorders (NM) and chest wall deformities, the COPD group have presented a statistically greater number of hospitalisations/yr (1.37 +/- 0.77), greater length of stay (13 +/- 10 days), higher number of outpatient visits/yr (2.55 +/- 1.73) or emergency room accesses/yr (0.74 +/- 1.08). Patients with NM diseases need more home care. The prevalence of one, two and three among five selected burden criteria (needs of MV > 12 hrs/day, tracheotomy, high dependency, distance from hospital, frequent hospitalisations) was respectively 19%, 30% and 33% of the cases; the NM was the group most represented. CONCLUSIONS: In HMV patients: 1. underlying disease, level of their dependency, hours spent under MV, presence of tracheotomy, home distance from hospital, hospital accesses are the causes of major care burden; and 2. as a novelty we have demonstrated that more than fifty percent of them present two or three contemporaneous criteria selected as care burden, being NM and COPD patients the most representative group necessitating of family's and HCS's care respectively.

A pilot study of nurse-led, home monitoring for patients with chronic respiratory failure and with mechanical ventilation assistance.

We assessed the feasibility of telemedicine for home monitoring of 45 patients with chronic respiratory failure (CRF) discharged from hospital. The patients transmitted pulsed arterial saturation (pSat) data via a telephone modem to a receiving station where a nurse was available for a teleconsultation. A respiratory physician was also available. Scheduled and ad hoc appointments were conducted. Thirty-five patients were on home mechanical ventilation, 13 with invasive and 22 with non-invasive devices. The main diagnosis was chronic obstructive pulmonary disease (COPD). The follow-up period was 176 days (SD 69). In all, 376 calls for scheduled consultations were received and 83 ad hoc consultations were requested by the patients. The actions taken were: 55 therapy modifications, 19 hospitalizations in a respiratory department for decompensated CRF, three hospitalizations in an intensive care unit (ICU), 22 requests for further investigations, 25 contacts with the general practitioner (GP), 66 demands for respiratory consultations and 10 calls for the emergency department. The mean time recorded for the 459 calls was 16 min/patient/week. In 82% of calls, a pSat recording was received successfully. The nurse time required to train the users in the operation of the pSat instrument was high (mean time 30 min). However, the results showed that home monitoring was feasible, and useful for titration of oxygen, mechanical ventilation setting and stabilization of relapses.