PURPOSE: Impaired cough secondary to weakness from neuromuscular disease (NMD) can cause serious respiratory complications, including atelectasis, pneumonia, small airway obstruction, and acidosis. Measurement tools are needed to assess at a distance, the effects of interventions to improve muscular strength, preserve cardiovascular function, sleep disturbances, anxiety, decrease chest rigidity, improve cough, measure cough mechanics and frequency, and measure pulmonary mechanics including the use of abdominal muscles. With gradual deterioration compliance with devices is a clinical problem. Access to sleep centers is difficult, particularly for rural atients. Objective: To determine the portability, safety, acceptability, and effectiveness of the LifeShirt,Nonin Wrist Oximeter,Nonin Bluetooth recorder, and MicroMedical hand held spirometer in a neuromuscular disease population.
METHODS: The use of the mechanic in-exsufflator (MI-E),high frequency chest wall oscillator (HFCWO), and nocturnal non-invasive ventilator (NNIV)support were assessed. Data recording utilized a light weight respiratory inductive plethysmography device in the home with separate day and sleep algorithm, wrist and wireless oximeter with recording capability, and hand held spirometer. Participants: Five male patients with NMD followed in Muscle Disorders and Pediatric Pulmonary Center were recruited for this pilot study. They were between 8 and 22 years of age and introduced to the use of the HFCWO, MI-E device, and NNIV within the last 18 months. Patients and caretakers reported productive cough, sleep disorders, and anxiety during clinic and home visits. No patient has a tracheostomy or gastrostomy tube.
RESULTS: Unsuspected nocturnal hypoxemia,detection of gradual pulmonary deterioration, tolerance of mechanical interventions, Konno-Mead plots and their relation to position,ventilatory strategy and activity over a continuous 24 hour period were obtained.
CONCLUSION: Recommendation for sleep assessment under the ATS recommendations can be met with home monitoring. Frequent home spirometry can be utilized as a tool for detection of pulmonary deterioration.Screening with home oximetry is better tolerated than respiratory inductive plethysmography but with less information.Use of home monitoring provided improving compliance.
CLINICAL IMPLICATIONS: Currently available technology may be utilized by patients to provide insight into their care in a naturalistic environment.
DISCLOSURE: Chris Landon, Consultant fee, speaker bureau, advisory committee, etc. Scientific Advisory Board Vivometrics Scientific Advisory Board Hill-Rom.