Quality-of-Life Evaluation of Patients With Neuromuscular and Skeletal Diseases Treated With Noninvasive and Invasive Home Mechanical Ventilation

doi:10.1378/chest.122.5.1695

Chest

Volume 122, Issue 5, November 2002, Pages 1695-1700

https://doi.org/10.1378/chest.122.5.1695 Get rights and content

Background

Home mechanical ventilation (HMV) is known to be a successful therapy for chronic respiratory insufficiency, with regard to long-term survival. However, the quality of life (QoL) of patients receiving HMV has not previously been systematically investigated. The purpose of this study was to assess the QoL of patients with neuromuscular disorders and skeletal deformities (ie, restrictive lung disease) receiving HMV.

Methods

Patients receiving HMV treated by tracheostomy or noninvasive ventilation (NIV). Three different, standardized, and validated questionnaires were used: the Sickness Impact Profile (SIP), the Health Index (HI), and the Sense of Coherence (SOC) scale. Underlying diseases were postpolio dysfunction (37%), neuromuscular disorder (20%), scoliosis (15%), and other diseases (28%).

Results

The group treated with tracheostomy had higher HI scores than the group treated with NIV. For the three main diagnosis groups treated with tracheostomy or NIV, the patients with postpolio dysfunction treated with tracheostomy had lower SIP scores than the patients with postpolio dysfunction treated with NIV. This was in contrast to the patients with neuromuscular disorders treated with tracheostomy, who had higher scores in the SIP, compared with patients with postpolio dysfunction treated with tracheostomy and patients with neuromuscular disorders treated with NIV. A SIP score > 10% indicates a functional disability of clinical importance, and a high score on the HI and SOC scale indicates good perceived health. For the three main diagnosis groups treated with NIV, the patients with scoliosis had no dysfunction of clinical importance (4.6 ŷ 3.7) on the SIP score compared with patients with postpolio dysfunction (15.5 ŷ 7.6) and patients with neuromuscular disorders (13.2 ŷ 5.2) [mean ŷ SD]. The men showed more dysfunction in the SIP score than the women.

Conclusion

Patients receiving HMV reported a good perceived health, despite severe physical limitations. The patients with postpolio dysfunction and the patients with scoliosis treated with tracheostomy perceived the best health, compared with NIV for this diagnosis.

Section snippets

Materials and Methods

All the patients in this study were in need of respiratory support and were all treated in ambulatory care settings. They were all living in the county of Stockholm, Sweden, and were all included in the Swedish health insurance system. One hundred twenty consecutive patients were invited to participate; 91 patients (76%) answered three questionnaires and sent them by mail to our clinic. Sixty patients were treated with NIV, and 31 patients underwent tracheostomy.

There are several definitions of

Results

The patients included 51 women and 40 men (mean age, 58.8 ŷ 1.6 years; range, 17 to 85 years) [mean ŷ SD]. Thirty-four patients (37%) were ≥ 65 years old, with 65 years being the age for retirement in Sweden. Of the remaining 57 patients, 15 patients (26%) performed some sort of work. Thirty-three patients (37%) had postpolio dysfunction, 16 patients (20%) from a neuromuscular disorder, 13 patients (15%) from scoliosis, and 29 patients (28%) had various diagnoses.

Discussion

HMV for patients with chronic respiratory failure is known to prolong survival.¹⁶^,¹⁷ Studies comparing intermittent positive pressure ventilation with NIV with tracheostomy are often conducted in patients with amyotrophic lateral sclerosis.³^,¹⁸ This study focuses on HMV in patients with neuromuscular disorders and skeletal deformities (ie, restrictive lung disease).

The main findings were that the patients reported a high level of disability in most areas in the SIP, indicating severe functional

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Respiratory failure is a significant concern in neuromuscular diseases (NMDs). This CHEST guideline examines the literature on the respiratory management of patients with NMD to provide evidence-based recommendations.
An expert panel conducted a systematic review addressing the respiratory management of NMD and applied the Grading of Recommendations, Assessment, Development, and Evaluations approach for assessing the certainty of the evidence and formulating and grading recommendations. A modified Delphi technique was used to reach a consensus on the recommendations.
Based on 128 studies, the panel generated 15 graded recommendations, one good practice statement, and one consensus-based statement.
Evidence of best practices for respiratory management in NMD is limited and is based primarily on observational data in amyotrophic lateral sclerosis. The panel found that pulmonary function testing every 6 months may be beneficial and may be used to initiate noninvasive ventilation (NIV) when clinically indicated. An individualized approach to NIV settings may benefit patients with chronic respiratory failure and sleep-disordered breathing related to NMD. When resources allow, polysomnography or overnight oximetry can help to guide the initiation of NIV. The panel provided guidelines for mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management, and airway clearance therapies. The guideline panel emphasizes that NMD pathologic characteristics represent a diverse group of disorders with differing rates of decline in lung function. The clinician’s role is to add evaluation at the bedside to shared decision-making with patients and families, including respect for patient preferences and treatment goals, considerations of quality of life, and appropriate use of available resources in decision-making.
Mental health reported in adult invasive home mechanical ventilation through a tracheostomy: A scoping review
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Citation Excerpt :
An organised healthcare structure for users of home mechanical ventilation is critical to underpinning meaningfulness and good quality of life for even profoundly disabled people (Stuart and Weinrich, 2004), like those who are invasively ventilated through a tracheostomy at home. In a Swedish quality-of-life-study, people receiving home mechanical reported good perceived health, despite severe physical limitations (Markström et al., 2002). People receiving invasive home mechanical ventilation through a tracheostomy often have severe physical disabilities requiring respiratory treatment and extensive care.
Although people receiving invasive home mechanical ventilation through a tracheostomy are facing both physical and mental health challenges, healthcare services often focus mainly on physical symptoms. To ensure well-functioning treatment and care for people receiving tracheostomy ventilation in a home setting, their mental health needs to be promoted and seen as an integral part of their health in general.
This scoping review aimed to provide a summary of the current knowledge on the mental health of people receiving invasive home mechanical ventilation through a tracheostomy.
A scoping review of published and gray literature based on the framework developed by Arksey and O'Malley and refined by the JBI was performed. The Preferred Reporting Items for Systematic Reviews and Meta-analyses Extension for Scoping Reviews checklist was used for reporting the findings.
A literature search was conducted by two researchers independently in the PubMed, CINAHL and PsycINFO databases. Additional searches for gray literature were conducted in Google, Google Scholar, websites of selected organisations, and the reference lists of included studies. The software system Covidence was used in the study selection process. For critical appraisal, the Mixed Methods Appraisal Tool was used.
Thirteen studies were included in this review, of which six used qualitative, six quantitative, and one mixed methods. The majority of studies were authored in Europe (n = 10), followed by the Americas (n = 2) and the Western Pacific (n = 1). Mental health was investigated both directly and indirectly (61.5% vs. 38.5%). Categorizing the reported mental health outcomes, we found that emotional well-being was reported widely across the studies (n = 13), while psychological well-being (n = 5) and social well-being (n = 4) were less widely reported.
The mental health of people receiving home tracheostomy ventilation has received some scholarly attention. A heterogeneity of mental health outcomes was reported in the literature with emotional well-being being an important mental health area both in relation to the sub-components positive affect and quality of life appraisal. Mental health outcomes in relation to psychological well-being and social well-being were fragmented and only sparsely investigated.
Continuous noninvasive ventilatory support outcomes for patients with neuromuscular disease: a multicenter data collaboration
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Citation Excerpt :
Such outcomes suggest that commonly reported “NIV failure”46–48 can result from inadequate NIV settings, failure to use MI-E at adequate settings, and failure to use diurnal NVS5 and air stacking.49 This explains the “preference” for tracheostomy for patients not offered CNVS and MIE.50,51 Whereas special expertise is required for noninvasive management, since 19% of 157 successfully extubated unweanable patients in one study had critical care neuromyopathies and not NMD, the expertise should not be limited to isolated centers.
Typically, patients with progressive neuromuscular disorders (NMDs) develop acute respiratory failure (ARF), are intubated, and when failing spontaneous breathing trials (SBTs) undergo a tracheotomy and receive tracheostomy mechanical ventilation (TMV). However, increasing numbers of patients use nasal noninvasive ventilation (NIV), initially for sleep and this is extended to continuous dependence (CNVS). This can be used as a strategy to assist in successful extubation . We retrospectively reviewed 19 centers offering CNVS and mechanical insufflation-exsufflation (MI-E) as an alternative to TMV.
Centers with publications or presentations concerning CNVS outcomes data were pooled for amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and spinal muscular atrophy type 1 (SMA1). Progression to CNVS dependence without hospitalization, duration of dependence, and extubations and decannulations to CNVS were recorded. Prolongation of life was defined by duration of CNVS dependence without ventilator free breathing ability (VFBA).
There were 1623 part time (<23 h/day) NVS users with ALS, DMD, and SMA1 from 19 centers in 16 countries of whom 761 (47%) were CNVS dependent for 2218 patient-years. This included: 335 ALS patients for a mean 1.2 ± 1.0 (range to 8) years each; 385 DMD patients for 5.4 ± 1.6 (range to 29) years; and 41 SMA1 patients for 5.9 ± 1.8 (range to 20) years. Thirty-five DMD and ALS TMV users were decannulated to CNVS and MI-E. At data collection 494 (65%) patients were CNVS dependent but 110 (74 of whom with bulbar ALS), had undergone tracheotomies.
ALS, DMD, and SMA1 patients can become CNVS dependent without requiring hospitalization but CNVS cannot be used indefinitely for many patients with advanced upper motor neuron diseases.
Transforming normative, ableist, and biomedical orientations to living well and quality of life in nursing: Reimagining what a ventilated body can do
2023, Nursing Inquiry
Auβerklinische Beatmung: Lebensqualität und Lebensende
2023, Pneumologie
Chronic Neurological Disorders
2023, Noninvasive Mechanical Ventilation and Neuropsychiatric Disorders: Essential Practical Approaches

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Chest

Clinical Investigations: QuestionnairesQuality-of-Life Evaluation of Patients With Neuromuscular and Skeletal Diseases Treated With Noninvasive and Invasive Home Mechanical Ventilation

Background

Methods

Results

Conclusion

Section snippets

Materials and Methods

Results

Discussion

Respir Med

Chest

Soc Sci Med

J Neurol Sci

New problems in old polio patients: results of survey of 539 polio survivors

Orthopedics

Nocturnal respiratory failure as an indication of noninvasive ventilation in the patient with neuromuscular disease

Respiration

Long-term nasal ventilation in neuromuscular disorders: report of a consensus conference

Eur Respir J

Swedish population norms for the GHRI, HI and STAI-state

J Qual Life Res

Oral- and pharyngeal-cancer patients' perceived symptoms and health

Cancer Nurs

Psychosocial adjustment and general state of health in patients with ileal conduit urinary diversion

Scand J Urol Nephrol

Quality of life in predialytic uremic patients

J Qual Life Res

The Sickness Impact Profile: development and final revision of health status measure

Med Care

The Sickness Impact Profile (SIP): an instrument for overall health assessment; a basic evaluation

J Drug Ther Res

Clinical Investigations: Questionnaires
Quality-of-Life Evaluation of Patients With Neuromuscular and Skeletal Diseases Treated With Noninvasive and Invasive Home Mechanical Ventilation