Carole LeBlanc

Lung volume recruitment slows pulmonary function decline in Duchenne muscular dystrophy.

OBJECTIVE To evaluate the long-term effect on measures of forced vital capacity (FVC) before and after the introduction of regular lung volume recruitment (LVR) maneuvers (breath-stacking) in individuals with Duchenne muscular dystrophy (DMD). DESIGN Retrospective cohort study of pulmonary function data, including FVC, cough peak flow (CPF), maximum inspiratory pressure (MIP), and maximum expiratory pressure (MEP). Data were collected for 33 months prior to and 45 months after LVR introduction. SETTING Ambulatory care in a tertiary level regional rehabilitation center in Canada. PARTICIPANTS All individuals (N=22) with DMD (mean age ± SD, 19.6±2.4y), who were prescribed LVR and reported adherence with therapy. INTERVENTIONS Introduction of regular LVR (breath-stacking); 3 to 5 maximal lung inflations (maximum insufflation capacity [MIC]) using a hand-held resuscitation bag and mouthpiece, twice daily. MAIN OUTCOME MEASURES Measures included the rate of decline of FVC in percent-predicted, before and after the introduction of regular LVR. Changes in maximum pressures (MIP, MEP), MIC, and cough peak flows were also measured. RESULTS At LVR initiation, FVC was 21.8±16.9 percent-predicted, and cough peak flows were <270L/min (144.8±106.9L/min). Annual decline of FVC was 4.7 percent-predicted a year before LVR and 0.5 percent-predicted a year after LVR initiation. The difference, 4.2 percent-predicted a year (95% confidence interval, 3.5-4.9; P<.000), represents an 89% improvement in the annual rate of FVC decline. CONCLUSIONS The rate of FVC decline in DMD patients improves dramatically with initiation of regular LVR.

Formal ventilation patient education for ALS predicts real-life choices

Abstract Our objective was to evaluate a single-session, hands-on education programme on mechanical ventilation for ALS patients and caregivers in terms of knowledge, change in affect and to determine whether ventilator decisions made after the education sessions predict those made later in life. Questionnaires were administered to 26 patients and 26 caregivers on four separate occasions. The questionnaires assessed knowledge of ventilatory support, feedback on the nature of the education programme, as well as self-reported emotional well-being. All patients were followed until their death or until initiation of invasive ventilation. Both groups demonstrated significant improvements in knowledge as a result of the education session which was retained after one month. There was no change in patient or caregiver reports’ self-reported emotional well-being. The choices of ventilatory support expressed at one month (T4) accurately predicted the real-life clinical choices made by 76% of patients. Any difference resulted from choosing palliative care. Hands-on patient and caregiver education results in improved knowledge, assists in decision-making with respect to ventilatory support, and is not associated with a worsening of affect. It also provides for an accurate prediction of real-life choices and avoids undesired life support interventions and critical care admissions.

Lung Volume Recruitment in Multiple Sclerosis

Introduction Pulmonary function abnormalities have been described in multiple sclerosis including reductions in forced vital capacity (FVC) and cough but the time course of this impairment is unknown. Peak cough flow (PCF) is an important parameter for patients with respiratory muscle weakness and a reduced PCF has a direct impact on airway clearance and may therefore increase the risk of respiratory tract infections. Lung volume recruitment is a technique that improves PCF by inflating the lungs to their maximal insufflation capacity. Objectives Our goals were to describe the rate of decline of pulmonary function and PCF in patients with multiple sclerosis and describe the use of lung volume recruitment in this population. Methods We reviewed all patients with multiple sclerosis referred to a respiratory neuromuscular rehabilitation clinic from February 1999 until December 2010. Lung volume recruitment was attempted in patients with FVC <80% predicted. Regular twice daily lung volume recruitment was prescribed if it resulted in a significant improvement in the laboratory. Results There were 79 patients included, 35 of whom were seen more than once. A baseline FVC <80% predicted was present in 82% of patients and 80% of patients had a PCF insufficient for airway clearance. There was a significant decline in FVC (122.6 mL/y, 95% CI 54.9–190.3) and PCF (192 mL/s/y, 95% 72–311) over a median follow-up time of 13.4 months. Lung volume recruitment was associated with a slower decline in FVC (p<0.0001) and PCF (p = 0.042). Conclusion Pulmonary function and cough decline significantly over time in selected patients with multiple sclerosis and lung volume recruitment is associated with a slower rate of decline in lung function and peak cough flow. Given design limitations, additional studies are needed to assess the role of lung volume recruitment in patients with multiple sclerosis.

Tracheostomy Decannulation and Cough Peak Flows in Patients with Neuromuscular Weakness

ObjectiveThe aim of this study was to examine the relationship between cough peak flows (CPFs) before and after tracheostomy tube removal (decannulation) in patients with neuromuscular respiratory muscle weakness. DesignFor 26 patients with occluded tracheostomies (capped or Passy-Muir valve), spontaneous CPF (CPFsp), CPF after lung volume recruitment (CPFLVR), and CPF after lung volume recruitment and a manually assisted cough (CPFLVR + MAC) were measured before and after decannulation. ResultsDecannulation resulted in a significant increase (P < 0.001) in CPF of 35.6, 34.5, and 42.6 l/min for CPFsp, CPFLVR, and CPFLVR + MAC, respectively. In addition, CPFLVR or CPFLVR + MAC with a capped tracheostomy in place were greater than spontaneous CPF with the tracheostomy tube removed. ConclusionsOur study suggests that assisted coughing with a capped tracheostomy tube in place can result in higher flows than removing the tube and relying on spontaneous cough alone. Postdecannulation CPF measured at the mouth can be predicted to be at least 34.5 l/min greater than predecannulation values, which may thereby lower the threshold of the CPF indicated for safe decannulation.

Data Downloads for Effective Noninvasive Ventilation in Patients With Neuromuscular Respiratory Failure

Patients with progressive neuromuscular diseases, chest wall abnormalities, and diaphragmatic dysfunction are at high risk for the development of respiratory failure, with resulting morbidity and mortality.[1][1] A large number of observational studies and a small number of randomized trials[2][2],[