Respiratory Monitoring Techniques for Neuromuscular Disease

Overseen By Tariq Rahman, PhD

Age: < 18

Sex: Any

Trial Phase: Academic

Sponsor: Nemours Children's Clinic

Disqualifiers: Severe breathing difficulties

No Placebo Group All trial participants will receive the active study treatment (no placebo)

Compensation: Varies

Number of Visits: 1

20 Participants Needed

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial tests a new device called pneuRIP (Respiratory Inductance Plethysmography monitor), which monitors breathing without invasive procedures. It compares pneuRIP to the existing Respitrace system (Carefusion) to evaluate its effectiveness in measuring breathing in children, both with and without breathing difficulties. The trial includes healthy children and those with mild breathing issues from neuromuscular diseases, provided they can follow simple instructions. As an unphased trial, it offers participants the chance to contribute to innovative research that could enhance non-invasive breathing monitoring for children.

Do I need to stop my current medications for the trial?

The trial information does not specify whether you need to stop taking your current medications.

What prior data suggests that this respiratory monitoring technique is safe for children?

Research shows that the pneuRIP monitor is easy to use. The technology behind pneuRIP, known as Respiratory Inductance Plethysmography (RIP), has been safely used for over 30 years. This technology monitors breathing without requiring anything to be placed inside the body. Studies have found that RIP causes little discomfort to patients.

The pneuRIP is being compared to another system called Respitrace. Both systems are designed to measure breathing safely. Current studies provide no direct evidence of negative effects from using the pneuRIP. This suggests it is likely safe, especially since it doesn't involve any drugs or invasive procedures. ^{1 2 3 4 5}

Why are researchers excited about this trial?

Researchers are excited about the trial on respiratory monitoring techniques for neuromuscular disease because it explores new ways to understand breathing patterns in patients. Unlike traditional monitoring methods, this trial examines two different techniques: one using the Respitrace system for breathing without resistance, and another using pneuRIP for breathing with resistance. These approaches aim to provide deeper insights into how neuromuscular disease affects respiration, potentially leading to more tailored and effective interventions. By investigating these distinct breathing scenarios, the trial hopes to uncover valuable data that could transform respiratory care for individuals with neuromuscular conditions.

What evidence suggests that these respiratory monitoring techniques are effective for neuromuscular disease?

Studies have shown that the pneuRIP monitor, one of the devices tested in this trial, effectively measures breathing patterns in children, particularly those with muscle-related conditions like Duchenne muscular dystrophy (DMD). It uses special bands to track breathing without invasive procedures. Research indicates that pneuRIP can identify various breathing issues in children, offering promise for improved diagnosis. Previous studies suggested that this technology can be as reliable as traditional methods like spirometry, while being more comfortable for patients. Although data is limited, early findings suggest that pneuRIP could be a valuable tool for understanding and monitoring breathing problems. Another device tested in this trial is the Respitrace system (Carefusion), which monitors breathing without resistance. ^{3 4 6 7 8}

Who Is on the Research Team?

Thomas Shaffer, PhD

Principal Investigator

Nemours

Are You a Good Fit for This Trial?

This trial is for children who are either healthy or have mild breathing difficulties due to neuromuscular disease. Participants must be able to understand and follow spoken instructions. Children with severe breathing problems cannot take part.

Inclusion Criteria

I have a neuromuscular disease and mild breathing problems.

I can understand and follow spoken directions.

Healthy volunteers

Exclusion Criteria

I have severe trouble breathing.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

1-2 weeks

Testing

Participants are fitted with respiratory monitors and undergo breathing tests with and without resistance

1 day

1 visit (in-person)

Follow-up

Participants are monitored for safety and effectiveness after testing

1 week

What Are the Treatments Tested in This Trial?

Interventions

• pneuRIP
• Respitrace system (Carefusion)

Trial Overview The study is testing a new respiratory monitor called pneuRIP, which uses bands around the chest to measure breathing without being invasive. It's being compared with an existing system, Respitrace, in both healthy children and those with mild neuromuscular-related breathing issues.

How Is the Trial Designed?

Group I: pneuRIP(breathing with resistance) Experimental Treatment 2 Interventions

Testing the subjects breathing with resistance

Group II: Respitrace system (Carefusion) (breathing without resistance) Active Control 1 Intervention

Testing subjects breathing without resistance

Who Is Running the Clinical Trial?

Nemours Children's Clinic

Lead Sponsor

Trials

128

Recruited

18,000+

Published Research Related to This Trial

Respiratory failure in patients with neuromuscular disorders arises from multiple mechanisms, including respiratory muscle weakness, muscle fatigue, altered ventilation control, impaired gas exchange during sleep, and upper airway dysfunction.

Nocturnal ventilatory assistance has been shown to effectively address respiratory failure by targeting these various mechanisms, improving respiratory function during the day for these patients.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

[Physiopathology of respiratory insufficiency of neuromuscular origin]. Díaz-Lobato, S., Ruiz-Cobos, A., García Río, FJ., et al.[2009]

The study developed a new technique for adjusting the calibration of portable respiratory inductive plethysmography (RIP) using position sensor feedback, allowing for accurate monitoring of breathing patterns in unrestrained subjects, including healthy individuals and patients with lung disease.

With this new calibration method, the RIP showed a minor mean difference in tidal volume measurements compared to traditional methods, indicating high accuracy and improved precision during various body positions, making it a valuable tool for studying respiratory physiology in everyday activities.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Respiratory monitoring by inductive plethysmography in unrestrained subjects using position sensor-adjusted calibration. Brüllmann, G., Fritsch, K., Thurnheer, R., et al.[2009]

The pneuRIPTM system is a noninvasive, wireless tool that can quickly assess respiratory function in children, showing 100% compliance in a clinical setting with tests completed in under 10 minutes.

Children with Duchenne Muscular Dystrophy (DMD) exhibited significantly abnormal respiratory patterns compared to healthy peers, indicated by elevated thoracoabdominal asynchrony and work of breathing indices, highlighting the potential of pneuRIPTM for early detection of respiratory issues.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Diagnostic differences in respiratory breathing patterns and work of breathing indices in children with Duchenne muscular dystrophy. Ryan, L., Rahman, T., Strang, A., et al.[2020]

In a study of 83 children with neuromuscular disease (NMD), it was found that the apnea-hypopnea index (AHI) and oxygen desaturation were significant indicators of respiratory issues, with AHI being positively correlated with the oxygen desaturation index (ODI).

The study suggests that measuring forced vital capacity (FVC) can help predict significant sleep-disordered breathing or hypoxia, and that using nocturnal oximetry along with daytime peak flow measurements could be an effective and convenient method for monitoring respiratory health in these children.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Characteristics and feasibility of ambulatory respiratory assessment of paediatric neuromuscular disease: an observational retrospective study. Zhang, C., Wei, CJ., Jin, Z., et al.[2023]

The study developed a reference-free adjustment method for respiratory inductance plethysmography (RIP) that significantly improves the accuracy of measuring breathing characteristics, achieving 84.69% of breaths within 20% limits of equivalence compared to only 43.63% with existing methods requiring simultaneous flowmeter data.

Using only the rib cage band for RIP measurements yielded better results than using both bands, indicating that a simpler setup can still provide reliable tidal volume measurements, which enhances the potential for RIP use in various ambulatory settings.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Reference-Free Adjustment of Respiratory Inductance Plethysmography for Measurements during Physical Exercise. Leutheuser, H., Heyde, C., Roecker, K., et al.[2018]

Respiratory inductive plethysmography (RIP) using the Respitrace Plus monitor showed that while it can measure tidal volumes (VT) in patients, it was not consistently accurate, especially in patients with COPD, where deviations were significantly higher compared to those with normal pulmonary function or acute lung injury (ALI).

The accuracy of RIP for measuring changes in end-expiratory lung volume (deltaEELV) was also limited, with only 25.8% and 46.2% of measurements being precise within +/-10% and +/-20%, respectively, indicating that RIP may not be reliable for quantitative evaluations in patients with respiratory conditions.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Evaluation of respiratory inductive plethysmography in controlled ventilation: measurement of tidal volume and PEEP-induced changes of end-expiratory lung volume. Neumann, P., Zinserling, J., Haase, C., et al.[2020]

Respiratory muscle involvement is common in childhood neuromuscular disorders and can lead to serious complications, including morbidity and mortality, making early detection crucial.

A preventive approach is essential for managing respiratory issues in these disorders, which includes regular monitoring of symptoms, pulmonary function assessments, and proactive treatment of respiratory infections.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Respiratory care in neuromuscular disorders. Estournet, B.[2013]

The tension-time index of the inspiratory muscles (TT MUS) is significantly higher in patients with Duchenne muscular dystrophy compared to healthy individuals, indicating a predisposition to respiratory muscle fatigue, especially as patients age.

TT MUS correlates with the extent of ventilator use, with higher values observed in patients requiring more hours of ventilation per day, suggesting that monitoring TT MUS can help assess the need for mechanical ventilation in these patients.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Noninvasive determination of the tension-time index in Duchenne muscular dystrophy. Hahn, A., Duisberg, B., Neubauer, BA., et al.[2016]

The new noninvasive pulmonary function instrument, pneuRIPTM, effectively measures respiratory work in children and shows promising accuracy compared to the existing Respitrace system, particularly in counting breaths under both normal and loaded breathing conditions.

In tests with ten normal children, pneuRIPTM demonstrated significantly better accuracy in measuring breathing rate, achieving 13.2% accuracy for normal breathing and 16.9% for loaded breathing, compared to Respitrace's 36.4% and 60.7%, respectively.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

pneuRIPTM: A Novel Respiratory Inductance Plethysmography Monitor. Rahman, T., Page, R., Page, C., et al.[2020]

In a study of 20 patients with neuromuscular disease (10 receiving noninvasive ventilation (NIV) and assisted coughing after extubation and 10 receiving standard medical therapy), those treated with NIV had significantly lower rates of reintubation (3 vs 10) and tracheostomy (3 vs 9).

Patients who received NIV and assisted coughing also had a shorter stay in the respiratory intensive care unit (RICU), averaging 7.8 days compared to 23.8 days for those on standard therapy, highlighting the efficacy of this intervention in preventing complications.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Prevention of extubation failure in high-risk patients with neuromuscular disease. Vianello, A., Arcaro, G., Braccioni, F., et al.[2018]

In a study of 55 adult patients with neuromuscular diseases, nocturnal hypercapnia (high CO2 levels) measured by transcutaneous capnometry was found to be significantly associated with negative health outcomes, including mortality and respiratory events requiring ICU admission, over a follow-up period of up to 5 years.

In contrast, nocturnal hypoxemia (low oxygen levels) measured by oximetry did not predict these outcomes, suggesting that capnometry should be included in the assessment of home mechanical ventilation efficiency for patients with neuromuscular diseases.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Prognostic Value of Initial Assessment of Residual Hypoventilation Using Nocturnal Capnography in Mechanically Ventilated Neuromuscular Patients: A 5-Year Follow-up Study. Ogna, A., Nardi, J., Prigent, H., et al.[2022]

Patients with neuromuscular disorders experience respiratory impairment due to muscle weakness, leading to complications like reduced lung volume and increased risk of lung infections, which can be evaluated through vital capacity and respiratory muscle strength measurements.

Noninvasive ventilation is preferred by most patients for maintaining mobility and independence, while technological advancements in respiratory support have significantly improved their quality of life and longevity, despite challenges in home care for caregivers.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Respiratory considerations in patients with neuromuscular disorders. Patel, N., Howard, IM., Baydur, A.[2023]

Citations

1. pubmed.ncbi.nlm.nih.gov pubmed.ncbi.nlm.nih.gov/29999598/

Measures of respiratory inductance plethysmography (RIP) in ... Results: Patients' means showed an increase of 119.8% for Ф, 15.7% for LBI, and 19.9% for HR compared with healthy subjects, when adjusted for ...

2. researchgate.net researchgate.net/publication/311852658_pneuRIPTM_A_Novel_Respiratory_Inductance_Plethysmography_Monitor

(PDF) pneuRIP(TM): A Novel Respiratory Inductance ... Patients with history of chronic lung disease and chronic neuromuscular disease with baseline habnormal breathing patterns were excluded.

3. withpower.com withpower.com/trial/phase-neuromuscular-diseases-7-2014-957d6

Respiratory Monitoring Techniques for Neuromuscular ... The pneuRIP uses Respiratory Inductance Plethysmography (RIP) bands to measure key breathing indices non-invasively. This study compares the pneuRIP to an ...

4. journals.plos.org journals.plos.org/plosone/article?id=10.1371/journal.pone.0226980

Diagnostic differences in respiratory breathing patterns and ... In this study, we show that pneuRIP TM can be used in a hospital clinic setting to differentiate WOB indices and breathing patterns in children with DMD.

5. mdpi.com mdpi.com/1999-4893/17/6/261

Artificial Intelligence-Based Algorithms and Healthcare ... RIP has been studied as an alternative to spirometry and shown promising results. Moreover, RIP data can be analyzed through machine learning ( ...

6. clinicaltrials.gov clinicaltrials.gov/study/NCT02851043

NCT02851043 | Non-Invasive Respiratory Monitor This study will clinically evaluate a newly developed respiratory monitor - pneuRIP. The pneuRIP uses Respiratory Inductance Plethysmography (RIP) bands to ...

7. sciencedirect.com sciencedirect.com/science/article/abs/pii/S1526054218300769

Review Age-related ranges of respiratory inductance ... Respiratory inductance plethysmography (RIP) has been used for more than three decades and enables noninvasive respiratory monitoring with minimal patient ...

8. pmc.ncbi.nlm.nih.gov pmc.ncbi.nlm.nih.gov/articles/PMC5318692/

A Novel Respiratory Inductance Plethysmography Monitor For BPM the pneuRIPTM is shown to be more accurate than the respitrace when compared to manually counting the breaths: 13.2% versus 36.4% accuracy for normal ...