scholarly journals Long-term non-invasive ventilation in muscular dystrophy

2016 ◽  
Vol 14 (1) ◽  
pp. 33-36 ◽  
Author(s):  
W Kinnear ◽  
J Colt ◽  
L Watson ◽  
P Smith ◽  
L Johnson ◽  
...  

Long-term non-invasive ventilation (NIV) was introduced in the 1980s, initially mainly for patients with poliomyelitis, muscular dystrophy (MD) or scoliosis. The obesity-hypoventilation syndrome has since become the commonest reason for referral to most centres providing home-NIV. Patients with MD are numerically a much smaller part of the workload, but as their disease progresses the need for ventilatory support changes and they require regular comprehensive assessment of their condition. We have examined the trend in MD use of home-NIV in our unit over the last 25 years. The number of new referrals appears to be stabilizing at around 20–25 over a 5-year period, equivalent to approximately 0.5 per 100,000 of population per year. The mean age at commencement of home-NIV is now 37.5 years, with 5-year survival rates of 70–75%. Ten-year survival rates are just over 40%. The distance of usual place of residence from our unit is fairly stable, currently at a mean of 27 km. Excellent survival rates mean that patients with MD, while numerically small, are likely to remain an important part of the workload of centres providing home-NIV. Our data should prove useful in the planning of future services for this group of patients.

2007 ◽  
Vol 101 (6) ◽  
pp. 1191-1195 ◽  
Author(s):  
Stefania Redolfi ◽  
Luciano Corda ◽  
Giuseppe La Piana ◽  
Sara Spandrio ◽  
Paola Prometti ◽  
...  

Respirology ◽  
2012 ◽  
Vol 17 (8) ◽  
pp. 1269-1274 ◽  
Author(s):  
OLALLA CASTRO-AÑÓN ◽  
RAFAEL GOLPE ◽  
LUIS A. PÉREZ-DE-LLANO ◽  
MARÍA JESÚS LÓPEZ GONZÁLEZ ◽  
EDGAR J. ESCALONA VELASQUEZ ◽  
...  

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
César Maquilón ◽  
Mónica Antolini ◽  
Nicolás Valdés ◽  
Marianela Andrade ◽  
Krishnna Canales ◽  
...  

Abstract Background Home mechanical ventilation (HMV) is a viable and effective strategy for patients with chronic respiratory failure (CRF). The Chilean Ministry of Health started a program for adults in 2008. Methods This study examined the following data from a prospective cohort of patients with CRF admitted to the national HMV program: characteristics, mode of admission, quality of life, time in the program and survival. Results A total of 1105 patients were included. The median age was 59 years (44–58). Women accounted for 58.1% of the sample. The average body mass index (BMI) was 34.9 (26–46) kg/m2. A total of 76.2% of patients started HMV in the stable chronic mode, while 23.8% initiated HMV in the acute mode. A total of 99 patients were transferred from the children's program. There were 1047 patients on non-invasive ventilation and 58 patients on invasive ventilation. The median baseline PaCO2 level was 58.2 (52–65) mmHg. The device usage time was 7.3 h/d (5.8–8.8), and the time in HMV was 21.6 (12.2–49.5) months. The diagnoses were COPD (35%), obesity hypoventilation syndrome (OHS; 23.9%), neuromuscular disease (NMD; 16.3%), non-cystic fibrosis bronchiectasis or tuberculosis (non-CF BC or TBC; 8.3%), scoliosis (5.9%) and amyotrophic lateral sclerosis (ALS; 5.24%). The baseline score on the Severe Respiratory Insufficiency questionnaire (SRI) was 47 (± 17.9) points and significantly improved over time. The lowest 1- and 3-year survival rates were observed in the ALS group, and the lowest 9-year survival rate was observed in the non-CF BC or TB and COPD groups. The best survival rates at 9 years were OHS, scoliosis and NMD. In 2017, there were 701 patients in the children's program and 722 in the adult´s program, with a prevalence of 10.4 per 100,000 inhabitants. Conclusion The most common diagnoses were COPD and OHS. The best survival was observed in patients with OHS, scoliosis and NMD. The SRI score improved significantly in the follow-up of patients with HMV. The prevalence of HMV was 10.4 per 100,000 inhabitants. Trial registration This study was approved by and registered at the ethics committee of North Metropolitan Health Service of Santiago, Chile (N° 018/2021).


2017 ◽  
Vol 11 (1) ◽  
pp. 31-40 ◽  
Author(s):  
Thomas Blankenburg ◽  
Christin Benthin ◽  
Stefanie Pohl ◽  
Anett Bramer ◽  
Frank Kalbitz ◽  
...  

Background:Home ventilation is an effective treatment option for obesity hypoventilation syndrome (OHS). This therapy is still controversial for stable chronic obstructive pulmonary disease (COPD). A recent study showed reduced mortality for COPD patients receiving home ventilation with high inflation pressures and back-up respiratory rates [so called High Intensity non-invasive ventilation (NIV)].Objective:The purpose of this study is whether High Intensity NIV applied in the routine care of COPD and OHS patients can lead to CO2reduction and survival data comparable to data from controlled studies.Method:In this prospective non interventional study fifty-one patients with COPD (FEV1 0.95l, corr. 32.8%) and 34 patients with OHS (VC 1.74l, corr. 50.7%) with chronic hypercapnic respiratory failure, who were treated with NIV were followed up for four years.Results:Elevated CO2values before NIV in COPD patients (8.6kPa), and in OHS patients (8.3kPa), could be lowered significantly to the upper normal range (COPD: 5.9kPa; OHS: 5.85kPa). The one-, two-, and three-year survival rates for COPD patients were 83%, 73%, and 55%, respectively. The one-, two-, and three-year survival rates for OHS patients were 85%, 72%, and 68%, respectively.Conclusion:High intensity NIV within routine care is effective in reducing blood CO2levels in COPD- and in OHS- related chronic respiratory insufficiency. The survival rates obtained here are comparable to data from controlled clinical trials on COPD.


2018 ◽  
Vol 54 (9) ◽  
pp. 455-459 ◽  
Author(s):  
Ramón Fernández Álvarez ◽  
Gemma Rubinos Cuadrado ◽  
Ines Ruiz Alvarez ◽  
Tamara Hermida Valverde ◽  
Marta Iscar Urrutia ◽  
...  

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