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

Trial Summary

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 data supports the effectiveness of the treatment pneuRIP for respiratory monitoring in neuromuscular disease?

The research highlights the importance of careful respiratory monitoring and noninvasive ventilation in managing respiratory issues in neuromuscular diseases. While specific data on pneuRIP is not provided, similar noninvasive techniques have been shown to improve quality of life and reduce complications by aiding in early detection and management of respiratory problems. ^{1 2 3 4 5}

Is respiratory inductance plethysmography (RIP) safe for use in humans?

Respiratory inductance plethysmography (RIP) is a noninvasive method used to monitor breathing patterns and has been tested in various settings, including with children and patients with different lung conditions. The studies suggest it is generally safe for use in humans, as it provides reliable measurements without causing harm. ^{6 7 8 9 10}

How is the treatment pneuRIP, Respitrace system different from other treatments for neuromuscular disease?

The pneuRIP, Respitrace system is unique because it uses respiratory inductance plethysmography (RIP) to monitor breathing patterns noninvasively, which can help detect early signs of respiratory muscle fatigue in neuromuscular disease. This approach allows for continuous and convenient monitoring, potentially improving early intervention and management of respiratory complications. ^{1 2 4 11 12}

What is the purpose of this trial?

This study will clinically evaluate a newly developed respiratory monitor - pneuRIP. The pneuRIP uses Respiratory Inductance Plethysmography (RIP) bands to measure key breathing indices non-invasively. This study compares the pneuRIP to an existing Respitrace system (Carefusion, Yorba Linda CA). 10 normal children and 10 children with breathing difficulties will be monitored with both systems.

Research Team

Thomas Shaffer, PhD

Principal Investigator

Nemours

Eligibility Criteria

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

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

Treatment Details

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.

Participant Groups

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+

Findings from Research

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]

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]

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]

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]

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 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]

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]

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]

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]

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]

References

1. Netherlands pubmed.ncbi.nlm.nih.gov

Respiratory care in neuromuscular disorders. [2013]

2. United States pubmed.ncbi.nlm.nih.gov

Noninvasive determination of the tension-time index in Duchenne muscular dystrophy. [2016]

3. United States pubmed.ncbi.nlm.nih.gov

Prevention of extubation failure in high-risk patients with neuromuscular disease. [2018]

4. United States pubmed.ncbi.nlm.nih.gov

Respiratory considerations in patients with neuromuscular disorders. [2023]

5. Switzerland 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. [2022]

6. Switzerland pubmed.ncbi.nlm.nih.gov

Respiratory monitoring by inductive plethysmography in unrestrained subjects using position sensor-adjusted calibration. [2009]

7. United States pubmed.ncbi.nlm.nih.gov

pneuRIPTM: A Novel Respiratory Inductance Plethysmography Monitor. [2020]

8. United States pubmed.ncbi.nlm.nih.gov

Reference-Free Adjustment of Respiratory Inductance Plethysmography for Measurements during Physical Exercise. [2018]

9. United States 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. [2020]

10. United States pubmed.ncbi.nlm.nih.gov

Diagnostic differences in respiratory breathing patterns and work of breathing indices in children with Duchenne muscular dystrophy. [2020]

11. Spain pubmed.ncbi.nlm.nih.gov

[Physiopathology of respiratory insufficiency of neuromuscular origin]. [2009]

12. England pubmed.ncbi.nlm.nih.gov

Characteristics and feasibility of ambulatory respiratory assessment of paediatric neuromuscular disease: an observational retrospective study. [2023]