Novel Methods of Ambulatory Physiologic Monitoring in Patients With Neuromuscular Disease

Abstract

This is a summary of the presentation on novel methods of ambulatory physiologic monitoring in patients with neuromuscular disease, presented as part of the program on pulmonary management of pediatric patients with neuromuscular disorders at the 30th annual Carrell-Krusen Neuromuscular Symposium on February 20, 2008. Pediatrics 2009123:S250-S252 R ECENTLY, CONSENSUS GUIDELINES were published for the respiratory care of patients with Duchenne muscular dystrophy and spinal muscular atrophy. These were practice-based guidelines, because the ability to generate evidence-based guidelines is limited because of the relatively rare nature of these diseases. The respiratory care guidelines provide precise recommendations for the timing and extent of respiratory examinations and care, from initial diagnosis through end-oflife directives. The process that produced these guidelines and the recent anesthesia and sedation guidelines, reviewed by Birnkrant in this conference, serves as a model for developing consensus practice parameters thataddress the multisystem involvement seen for many of the muscular dystrophies. [1][2][3][4][5] In the context of continuous quality improvement, they provided an AIM statement and a clear guide to muscle disorders clinics of the role of pediatric pulmonary evaluation and management (Table 1 www.pediatrics.org/content/vol123/ Supplement_4).The welcome shift from hospital ventilation to home ventilation, the emergence of technologic and biomedical advancements, and maximizing the benefits of therapies through appropriate timing have brought about a search for pulmonary outcome measures. Respiratory disease accounts for ϳ80% of the mortalities of patients with Duchenne muscular dystrophy. Our current measures consist of spirometry (forced vital capacity, forced expiratory volume at 1 second, oxygen saturation awake and asleep, and peak inspiratory and peak expiratory pressure) and rates of pneumonia, hospitalization, and respiratory failure. [6][7][8] The routine evaluation of sleep has been hindered by the expense associated with technician-monitored studies geared at screening for the justification of expensive home therapies for adults with obstructive sleep apnea syndrome, lack of pediatric sleep laboratories (with the insufficiency made more difficult by the increased recommendations for evaluation of primary snoring with inadequate infrastructure in place), variability in interpretation, and inadequately developed standards of "normal." 9 Home sleep monitoring has been hindered by the frequent need for restudy, which has resulted in the denial of development of a payment structure to foster a business case for innovation. 10 The reliance by private payers on Center for Medicare and Medicaid Services approval led to a reevaluation being released in March 2008, spurred by the deluge of obesity-related obstructive sleep apnea in adults with inadequate infrastructure for evaluation before initiation of home continuous positive airway pressure intervention.In the face of these d...

Measures of respiratory inductance plethysmography (RIP) in children with neuromuscular disease

“…We acknowledge that pneu RIP is a research tool and is not readily available to all clinicians; however, with further approvals (FDA), this instrument in combination with other non‐invasive pulmonary testing methods (ie, FOT) may be used to guide clinical care decisions such as respiratory support intervention and/or sleep studies. As better methods for assessment of pulmonary function are pursued, the pneu RIP could provide a more efficient, real‐time method component for patient evaluations in NM clinics as well as at home. It is noteworthy that patients with NM disease with ongoing mild‐to‐moderate respiratory muscle insufficiency appear to be using variable breathing pattern strategies to balance energy expenditure and gas exchange.…”

“…Methods used to assess breathing in NM diseases can be expensive, time consuming, and exhausting for the patients. Better methods for assessment, including in‐home evaluations, are sought . The pneu RIP could provide a more efficient method for patient pulmonary evaluation.…”

Quantitative Analysis of Thoracoabdominal Asynchrony in Pediatric Polysomnography

“…One evaluated breathing at home in ambulatory patients with Duchenne muscular dystrophy. 28 Another examined RIP as a possible clinical decision tool to predict admission of patients with asthma exacerbation from the emergency room. 29 Though a couple of studies have investigated quantitative use of RIP in infant sleep, only one looked at sleep in older children.…”

“…26 We did not change our standard PSG practice to include QDC; however, this may be worth considering for future studies. The advantage of our decision not to use QDC is that we were able to determine the viability of quantitative measures of TAA for PSG as previously reported 23,28,29 without change in practice or additional cost or effort.…”

Artificial Intelligence-Based Algorithms and Healthcare Applications of Respiratory Inductance Plethysmography: A Systematic Review

“…RIP was implemented by integrating RIP belts in wearable garments in a 2007 study to assess the effect of adherence of RIP belts to the human body [72]. RIP has demonstrated its ability to be used for long-term monitoring purposes, i.e., in patients with neuromuscular disease [73].…”