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Nebulised Hypertonic Saline to Decrease Respiratory Exacerbations in Neuromuscular Disease or Neurodisability

Not Applicable
Not yet recruiting
Conditions
Neuromuscular Diseases
Neurodevelopmental Disorders
Cerebral Palsy
Interventions
Device: saline
Registration Number
NCT06134401
Lead Sponsor
Imperial College London
Brief Summary

Research Aim: This study investigates whether a 12-month treatment with hypertonic saline (salty water) can reduce antibiotic use in individuals with neuromuscular disease or cerebral palsy who frequently experience chest infections due to difficulty clearing mucus from their airways.

Methodology: Participants will be randomly assigned to receive nebulised hypertonic saline (7% salt in water) or normal saline (0.9% salt in water). The study is open-label as both participants and researchers are aware of the treatment, necessary due to the differing tastes of the solutions. Two centers, Royal Brompton Hospital in London and Queens Medical Centre in Nottingham, will conduct the research.

Before starting the treatment, participants will undergo various assessments, including questionnaires to measure quality of life and treatment satisfaction, sputum/throat swab collection, lung clearance index, forced oscillation technique, electrical impedance tomography, and lung ultrasound. Once these assessments are completed, participants will take the assigned treatment at home, administered twice daily for 12 months, with monthly follow-ups regarding difficulties and chest infections. After 12 months, the treatment will cease, and participants will repeat the assessments.

Significance: This research will provide valuable insights into the efficacy of nebulised hypertonic saline for individuals with neuromuscular disease or cerebral palsy, potentially aiding both patients and doctors in making informed treatment decisions.

Dissemination: The study's findings will be shared through publication in scientific journals and presentation at conferences.

Detailed Description

Not available

Recruitment & Eligibility

Status
NOT_YET_RECRUITING
Sex
All
Target Recruitment
40
Inclusion Criteria
  • Diagnosis of NMD or neurodisablity by a physician independent of the study, on standard criteria.
  • Age 5 years and above, including adults.
  • Must be able to tolerate nebulised 6% hypertonic saline.
  • Must have a history of at least one respiratory exacerbation requiring antibiotic treatment with or without the need for hospitalisation in the 12 months prior to recruitment.
Exclusion Criteria
  • Patients with additional diagnosis, for example, CF, but those with aspiration and/or bronchiectasis secondary to respiratory complications of NMD will be included.
  • Patients who are already prescribed daily HS in any concentration (i.e, 3%, 5%, 6%, 7%) will be excluded, but those who are on daily NS or have HS prescribed as part of their escalation plan (i.e., PRN) will be included.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
TreatmentsalineNebulised 6% Hypertonic saline
ControlsalineNebulised 0.9% normal saline
Primary Outcome Measures
NameTimeMethod
Course of antibiotics for respiratory infectionsfrom baseline to week 52

Full courses of antibiotics as prescribed for respiratory infections, both oral and intravenous, (excluding prophylactic antibiotic prescriptions). 1 course of antibiotic would be the full treatment for one event of respiratory infection, irrespective of the number of days that the course was prescribed for.

Secondary Outcome Measures
NameTimeMethod
Lung clearance indexat baseline before and within 2 hours after drug response assessment, and at week 52.

measured by multiple breath washout

Ease of airway clearanceOnce monthly for 52 weeks.

0-10 Visual analogue scale, where 0 is most easy, and 10 is most difficult.

Health economicsbaseline, week 26 and week 51.

Quality-adjusted life years

Airway inflammationbaseline and at week 52

Levels of IL-1b in sputum or throat swab.

Patient and main carer treatment satisfactionweeks 12, 26, 39 and 51

Treatment Satisfaction Questionnaire for Medication (TSQM Version 1.4). Scores range from 0 to 100, with higher scores indicating higher satisfaction.

Family impactBaseline and at week 51.

PedsQL™ Family Impact Module. The scale has five Likert response options, 'never', 'almost never', 'sometimes', 'often' and 'almost always' (corresponding to scores of 100, 75, 50, 25 and 0). Regarding the interpretation of the scale, higher scores indicate better functioning (less negative impact).

Electrical Impedance Tomographyat baseline before and within 2 hours after drug response assessment, and at week 52.

Electrical impedance tomography (EIT)-based global inhomogeneity index (quantification of homogeneity of the tidal volume distribution). The image matrix in EIT consists of 32 × 32 pixels. Global inhomogeneity (GI) is calculated as the sum of the absolute differences between the median value of tidal variation and every single pixel value, divided by the sum of all impedance values, to normalise the calculated values.The smaller the GI, the more homogeneous the tidal volume is distributed within the ventilated area. A GI of zero represents a perfectly homogeneous distribution of ventilation.

Health-related quality of lifeAt baseline and at week 51.

Pediatric Quality of Life Inventory (PedsQL)™. 0-100 scale, where higher scores indicate better HRQOL (Health-Related Quality of Life).

Forced oscillation techniqueat baseline before and within 2 hours after drug response assessment, and at week 52.

Respiratory reactance (Xrs).

Lung ultrasoundat baseline before and within 2 hours after drug response assessment, and at week 52.

Global Lung ultrasound score. The global lung ultrasound score (LUS) quantifies lung aeration by translating lung ultrasound patterns into a numerical score across 12 lung regions (six areas on each side of the chest: two ventral regions, two lateral regions, and two posterolateral regions) and summing the results. The aeration pattern observed in each region is scored from 0 to 3 as follows: 0 = A pattern with ≤2 B lines; 1 = \>2 separated B lines that cover ≤50% of the pleural line; 2 = B lines that cover \>50% of the pleural line; or 3 = lung consolidation. In theory, the global LUS score can range from 0 (normal aeration in all regions) to 36 (severe abnormal aeration in all regions).

Bacterial diversitybaseline and at week 52

Bray-Curtis dissimilarity index. Examines the abundances of microbes that are shared between two samples, and the number of microbes found in each.

Trial Locations

Locations (2)

Nottingham University Hospitals

🇬🇧

Nottingham, United Kingdom

Royal Brompton Hospital

🇬🇧

London, United Kingdom

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