Moving from Inpatient to Outpatient or Home Initiation of Non-Invasive Home Mechanical Ventilation.

Home mechanical ventilation (HMV) is an effective treatment for patients with chronic hypercapnic respiratory failure caused by restrictive or obstructive pulmonary disorders. Traditionally, HMV is initiated in the hospital, nowadays usually on a pulmonary ward. The success of HMV, and especially non-invasive home mechanical ventilation (NIV), has led to a steep and ongoing increase in the incidence and prevalence of HMV, in particular for patients with COPD or obesity hypoventilation syndrome. Consequently, the number of available hospital beds to accommodate these patients has become insufficient, and models of care that minimize the use of (acute) hospital beds need to be developed. At present, the practices for initiation of NIV vary widely, reflecting the limited research on which to base model-of-care decisions, local health system features, funding models, and historical practices. Hence, the opportunity to establish outpatient and home initiation may differ between countries, regions, and even HMV centres. In this narrative review, we will describe the evidence regarding the feasibility, effectiveness, safety, and cost savings of outpatient and home initiation of NIV. In addition, the benefits and challenges of both initiation strategies will be discussed. Finally, patient selection and execution of both approaches will be examined.

[Withdrawal of chronic mechanical ventilation at home: coordination, organisation and implementation]. / Beëindiging van chronische beademing in de thuissituatie.

In the Netherlands, an increasing number of patients are dependent on domiciliary ventilation. General practitioners and elderly care physicians caring for these patients are increasingly confronted with problems related to chronic ventilation. Most patients die due to progression of respiratory failure, however, patients may ask their physician to electively withdraw their assisted ventilation. According to the Dutch Medical Treatment Contracts Act, withdrawal of domiciliary ventilation at the request of a patient constitutes normal medical care and concerns neither the assessment of, nor the compliance with, a request for euthanasia. Currently, there is no Dutch guidance or guideline containing practical advice regarding the medical, ethical, organisational and supportive aspects of withdrawal of domiciliary ventilation. This paper addresses the planning necessary for the patient and between treating professionals, for the organisation and implementation of withdrawal of domiciliary ventilation at the patient's home, a nursing home or hospice.

The frontotemporal syndrome of ALS is associated with poor survival.

Thirty percent of ALS patients have a frontotemporal syndrome (FS), defined as behavioral changes or cognitive impairment. Despite previous studies, there are no firm conclusions on the effect of the FS on survival and the use of non-invasive ventilation (NIV) in ALS. We examined the effect of the FS on survival and the start and duration of NIV in ALS. Behavioral changes were defined as >22 points on the ALS-Frontotemporal-Dementia-Questionnaire or ≥3 points on ≥2 items of the Neuropsychiatric Inventory. Cognitive impairment was defined as below the fifth percentile on ≥2 tests of executive function, memory or language. Classic ALS was defined as ALS without the frontotemporal syndrome. We performed survival analyses from symptom onset and time from NIV initiation, respectively, to death. The impact of the explanatory variables on survival and NIV initiation were examined using Cox proportional hazards models. We included 110 ALS patients (76 men) with a mean age of 62 years. Median survival time was 4.3 years (95 % CI 3.53-5.13). Forty-seven patients (43 %) had an FS. Factors associated with shorter survival were FS, bulbar onset, older age at onset, short time to diagnosis and a C9orf72 repeat expansion. The adjusted hazard ratio (HR) for the FS was 2.29 (95 % CI 1.44-3.65, p < 0.001) in a multivariate model. Patients with an FS had a shorter survival after NIV initiation (adjusted HR 2.70, 95 % CI 1.04-4.67, p = 0.04). In conclusion, there is an association between the frontotemporal syndrome and poor survival in ALS, which remains present after initiation of NIV.

Hot Topics in Noninvasive Ventilation: Report of a Working Group at the International Symposium on Sleep-Disordered Breathing in Leuven, Belgium.

During the last few decades, attention has increasingly focused on noninvasive ventilation (NIV) in the treatment of chronic respiratory failure. The University of Leuven and the University Hospitals Leuven therefore chose this topic for a 2-day working group session during their International Symposium on Sleep-Disordered Breathing. Numerous European experts took part in this session and discussed (1) NIV in amyotrophic lateral sclerosis (when to start NIV, NIV and sleep, secretion management, and what to do when NIV fails), (2) recent insights in NIV and COPD (high-intensity NIV, NIV in addition to exercise training, and NIV during exercise training), (3) monitoring of NIV (monitoring devices, built-in ventilator software, leaks, and asynchronies) and identifying events during NIV; and (4) recent and future developments in NIV (target-volume NIV, electromyography-triggered NIV, and autoregulating algorithms).

Quality of life of adult men with Duchenne muscular dystrophy in the Netherlands: implications for care.

OBJECTIVE: To assess quality of life of adults with Duchenne muscular dystrophy in the Netherlands and to identify domains and major problems influencing quality of life. DESIGN: Cross-sectional. SUBJECTS: Seventy-nine men aged ≥ 20 years with Duchenne muscular dystrophy. METHODS: The Medical Outcome Study Short Form-36 (SF-36), World Health Organization Quality of Life - BREF (WHOQOL-BREF) and an interview were used to assess quality of life and problems. RESULTS: Compared with Dutch general population reference values, the SF-36 domains scores were lower on all domains except mental health and role limitations due to emotional problems. On the WHOQOL-BREF the social relationships domain score was lower. Main problems were intimate relationships, work, leisure, transport and meaningfulness of life. Seventy-three percent stated overall quality of life as "(very) good". The SF-36 domains mental health (rs 0.53, p < 0.001) and vitality (rs 0.49, p < 0.001) had the strongest associations with overall quality of life. CONCLUSION: Adult men with Duchenne muscular dystrophy assess their health status as low in the physical, but not in the mental, domains. Experienced problems are mainly in the area of participation. They are generally satisfied with their overall quality of life.

[Terminal care in patients with amyotrophic lateral sclerosis]. / De terminale fase bij amyotrofische laterale sclerose.

Patients with amyotrophic lateral sclerosis (ALS) often fear of dying from suffocation. This fear is also common in relatives and caregivers. Research has, however, shown that ALS patients seldom die from suffocation. More than 90% of all ALS patients die peacefully. Death is mostly preceded by a peracute decrease in consciousness due to hypercapnia caused by alveolar hypoventilation. Mechanical ventilation, especially at night, can reduce the symptoms caused by hypoventilation. However, little by little, the effectiveness of ventilation may decrease to such an extent that it is no longer useful or desirable. Termination of long-standing ventilation requires careful preparation. Intensive guidance of the relevant medical, practical and ethical aspects are necessary. Particularly in the pre-terminal and terminal phases, support given to an ALS patient requires a pro-active attitude on the part of the treating physician. To this end, physicians may seek advice from the Dutch ALS Center, a palliative care consultation team, a hospice physician or a center for home mechanical ventilation.

Pneumothorax as a complication of lung volume recruitment.

Lung volume recruitment involves deep inflation techniques to achieve maximum insufflation capacity in patients with respiratory muscle weakness, in order to increase peak cough flow, thus helping to maintain airway patency and improve ventilation. One of these techniques is air stacking, in which a manual resuscitator is used in order to inflate the lungs. Although intrathoracic pressures can rise considerably, there have been no reports of respiratory complications due to air stacking. However, reaching maximum insufflation capacity is not recommended in patients with known structural abnormalities of the lungs or chronic obstructive airway disease. We report the case of a 72-year-old woman who had poliomyelitis as a child, developed torsion scoliosis and post-polio syndrome, and had periodic but infrequent asthma attacks. After performing air stacking for 3 years, the patient suddenly developed a pneumothorax, indicating that this technique should be used with caution or not at all in patients with a known pulmonary pathology.

Pneumothorax as a complication of lung volume recruitment / Pneumotórax como complicação associada ao recrutamento do volume pulmonar

Lung volume recruitment involves deep inflation techniques to achieve maximum insufflation capacity in patients with respiratory muscle weakness, in order to increase peak cough flow, thus helping to maintain airway patency and improve ventilation. One of these techniques is air stacking, in which a manual resuscitator is used in order to inflate the lungs. Although intrathoracic pressures can rise considerably, there have been no reports of respiratory complications due to air stacking. However, reaching maximum insufflation capacity is not recommended in patients with known structural abnormalities of the lungs or chronic obstructive airway disease. We report the case of a 72-year-old woman who had poliomyelitis as a child, developed torsion scoliosis and post-polio syndrome, and had periodic but infrequent asthma attacks. After performing air stacking for 3 years, the patient suddenly developed a pneumothorax, indicating that this technique should be used with caution or not at all in patients with a known pulmonary pathology.

O recrutamento do volume pulmonar envolve técnicas de insuflações pulmonares profundas para se atingir a capacidade de insuflação máxima em pacientes com fraqueza da musculatura respiratória, a fim de aumentar o pico de fluxo da tosse e assim auxiliar a manutenção da patência de vias aéreas e melhorar a ventilação. Uma dessas técnicas é o empilhamento de ar, na qual se utiliza um ressuscitador manual para insuflar os pulmões. Embora as pressões intratorácicas possam aumentar consideravelmente, não há relatos de complicações por empilhamento de ar. Entretanto, atingir a capacidade de insuflação máxima não é recomendado em pacientes com anormalidades na estrutura pulmonar ou doença obstrutiva crônica das vias aéreas. Relatamos o caso de uma paciente de 72 anos que teve poliomielite quando criança, desenvolveu escoliose de torção e síndrome pós-pólio e tinha exacerbações de asma periódicas, mas infrequentes. Após realizar empilhamento de ar por 3 anos, a paciente subitamente desenvolveu pneumotórax, mostrando que essa técnica deve ser utilizada com cuidado ou não ser utilizada por pacientes com patologia pulmonar conhecida.

[Non-invasive home mechanical ventilation: qualification, initiation, and monitoring]. / Nieinwazyjna wentylacja w warunkach domowych--kwalifikacja, rozpoczecie i monitorowanie.

Following the introduction of non-invasive positive pressure ventilation (NPPV), the number of patients using home mechanical ventilation has increased substantially and continues to rise worldwide. This is primarily explained by both the effectiveness and comfort that are offered by NPPV in most patients, and particularly in patients with chest wall and neuromuscular diseases. For clinically stable patients the qualification for NPPV largely depends on the presence of complaints or signs of (nocturnal) hypoventilation with accompanying hypercapnia. For patients who are referred by an ICU there are additional prerequisites. In any case, the aims of NPPV should be met and NPPV should be effective. The initiation of NPPV, whether in the clinic or not, should always be tailored to the individual patient. Based on effectiveness, safety, and comfort, the best ventilator has to be chosen. Although with modern interfaces NPPV may be provided continuously, for continuing NPPV over the years, adding manual and/or mechanical cough augmentation techniques is usually mandatory. To control the ongoing effectiveness of NPPV regular monitoring of the patient is essential, and nowadays transcutaneous measurement of CO2 seems the most reliable and appropriate technique. For trend analysis, downloaded data of modern ventilators may be helpful as well. The ultimate goal of NPPV, to prevent tracheotomy, can only be reached if the patient has continuous access to a centre with expertise in cough augmentation techniques and both nocturnal and diurnal NPPV.

Thirty years of home mechanical ventilation in children: escalating need for pediatric intensive care beds.

PURPOSE: To describe trends in pediatric home mechanical ventilation (HMV) and their impact on the use of pediatric intensive care unit (PICU) beds. METHODS: Review of all children who had started HMV in a single center for HMV. RESULTS: Between 1979 and 2009, HMV was started in 197 patients [100 (51%) with invasive and 97 with noninvasive ventilation], with a median age of 14.7 (range 0.5-17.9) years. Most patients (77%) were males with a neuromuscular disorder (66%). The number of children receiving HMV increased from 8 in the 1979-1988 period to 122 in the 1999-2008 period. This increase occurred foremost in patients aged 0-5 years and was accompanied by a sharp rise in the use of PICU beds. In 150 patients (76%), HMV was initiated on an ICU with a total of 12,440 admission days, of which 10,385 days (83%) could be attributed to 67 patients who started non-electively with invasive HMV. Of the latter, 52 patients had been admitted to a PICU with a total of 9,335 admission days. At the end of the study, 134 patients (68%) were still being ventilated, 43 patients (22%) had died, 11 patients (6%) were weaned from HMV, 4 patients (2%) did not want to continue HMV and 5 patients (3%) were lost to follow-up. CONCLUSIONS: Over time, there was an impressive increase in the application of HMV in children. This increase was most obvious in the youngest age group with invasive HMV, and these children had very long stays in the PICU.

[Home mechanical ventilation in the Netherlands]. / Chronische beademing in Nederland.

Over the last 20 years the number of Dutch patients on home mechanical ventilation has increased from 200 to 2000. Home mechanical ventilation is a cost-effective treatment which significantly improves the quality of life of patients. In 2011 83% of patients on home mechanical ventilation in the Netherlands is living at home. In the future further growth can be expected in the number of patients with obesity hypoventilation syndrome and a potential new group of patients with COPD. Strict conditions are necessary to ensure safety in the complex care that home mechanical ventilation entails.

[Lung volume recruitment in impending respiratory failure]. / Longvolumerekrutering bij dreigend respiratoir falen.

Lung volume recruitment can improve peak cough flows and respiratory compliance in patients who either do or do not require mechanical ventilation. There are several lung volume recruitment techniques: air stacking, glossopharyngeal breathing and mechanical insufflation-exsufflation with cough assist devices. The principle of lung volume recruitment is based on the insufflation of air in the lungs after maximal inspiration. In air stacking, a manual resuscitation bag is used for insufflation. Glossopharyngeal breathing requires the use of oropharyngeal and laryngeal muscles by the patient. The mechanical cough assist device exsufflates the air after insufflation. These techniques may prevent pulmonary complications, hospital admission and tracheotomy in patients with a reduced ability to cough, a proclivity towards atelectasis and recurrent airway infections. The combination of long-term mechanical ventilation with lung volume recruitment has led to further improvement in the prognosis of chronic respiratory failure. More patients may potentially benefit from lung volume recruitment than only those being converted from short-term to long-term mechanical ventilation.

[Obesity hypoventilation syndrome--serious but reversible providing weight is lost]. / Obesitas-hypoventilatiesyndroom: ernstig, maar reversibel bij gewichtsreductie.

Obesity hypoventilation syndrome (OHS) is a condition in which obesity and chronic hypoventilation during waking hours are combined. Patients with OHS are more likely to be hospitalized and to require intensive-care monitoring compared with patients with similar degrees of obesity without hypoventilation. Treatment with chronic non-invasive positive pressure ventilation (NPPV) is associated with a lower morbidity and mortality. We present 2 cases of OHS; in the first case, a 56-year-old woman, we show that it is very important to diagnose and treat OHS at an early stage in order to avoid severe morbidity. In the second case, a 31-year-old man, we show that if a patient with OHS and chronic NPPV looses a significant amount of weight NPPV can be discontinued. Patients with OHS should be treated in a multidisciplinary team in order to achieve significant weight loss, so NPPV could be a temporary treatment or even be avoided.