MedPath

Nebulised Hypertonic Saline in Children and Young People With Neuromuscular Disease and Cerebral Palsy

Completed
Conditions
Neuromuscular Diseases
Interventions
Drug: Nebulised hypertonic saline
Registration Number
NCT03623698
Lead Sponsor
Imperial College London
Brief Summary

Pneumonia, respiratory exacerbations, and chronic pulmonary infection are important causes of emergency admissions, hospitalisations and death in children with Neuromuscular disorders and Cerebral Palsy. Hence, there is a need for research on how to therapeutically aid airway clearance and decrease respiratory exacerbations. Studies have shown that nebulised Hypertonic Saline is well tolerated, reduces pulmonary exacerbations and improves lung function and Lung Clearance Index in patients with Cystic Fibrosis, and enhances mucociliary clearance in asthmatic patients. Nevertheless, to the investigators' knowledge, there is no available data concerning the use of nebulised Hypertonic Saline in the management of children with Neuromuscular disorders and Cerebral Palsy. This study aims to assess the effectiveness of nebulised Hypertonic Saline to decrease hospitalisations and courses of antibiotics in children with Neuromuscular disorders and Cerebral Palsy.

Detailed Description

Chart review of children and young people with Neuromuscular disease or Cerebral Palsy who are cared for in the Royal Brompton Hospital and that have been treated with nebulised hypertonic saline for at least 12 months.

To further complement data from hospital records, two questionnaires will be applied. Parents of children who meet criteria will be asked to complete the following questionnaires:

1. The National Health and Nutrition Examination Survey (NHANES) for Hospitalisation and access to are - HUQ.010; and

2. Questionnaire on Hypertonic Saline treatment.

Children from 10 - 18 years will be asked to complete the Questionnaire on Hypertonic Saline treatment.

AIMS

1. Explore whether treatment with nebulised Hypertonic Saline in children with Neuromuscular disease or Cerebral Palsy decreases respiratory-related complications.

2. Evaluate whether the treatment with nebulised hypertonic saline in children with neuromuscular disease or cerebral palsy improves the ease of airway clearance.

3. Explore how parents of children with Neuromuscular disease and children with Cerebral Palsy perceive the treatment with nebulised hypertonic saline compared with previous management.

Sample Size:

The investigators aim to recruit 40 participants for each group, including children and young people and their parents or legal guardians, as this is a pilot study.

STATISTICAL ANALYSIS PLAN

* Univariate X2 analysis for categorical variables to investigate Courses of antibiotic treatment.

* Univariate X2 analysis for categorical variables to investigate Number of hospitalisations.

* Student t testing will be used to analyse nocturnal oxygenation and ventilation outcomes comparing one year before and after starting treatment with Hypertonic Saline.

* Independent t testing and Mann-Whitney U test to analyse Rate of decline in pulmonary function.

* Cox proportional hazard model to test differences in primary endpoints for different baseline FVC.

* Univarate analysis to analyse Ease of airway clearance.

* Univariate analysis on perception of treatment.

Data and all appropriate documentation will be stored for a minimum of 10 years after the completion of the study, including the follow-up period.

ETHICS APPROVAL The Study Coordination Centre has obtained approval from the Yorkshire \& The Humber - Leeds West Research Ethics Committee (REC) and Health Regulatory Authority (HRA). The study also received confirmation of capacity and capability from each participating NHS Trust before accepting participants into the study or any research activity was carried out. The study will be conducted in accordance with the recommendations for physicians involved in research on human subjects adopted by the 18th World Medical Assembly, Helsinki 1964 and later revisions.

CONSENT Consent to enter the study must be sought from each participant only after a full explanation has been given, an information leaflet offered and time allowed for consideration. Signed participant consent should be obtained. The right of the participant to refuse to participate without giving reasons must be respected. After the participant has entered the study the clinician remains free to give alternative treatment to that specified in the protocol at any stage if he/she feels it is in the participant's best interest, but the reasons for doing so should be recorded. In these cases the participants remain within the study for the purposes of follow-up and data analysis. All participants are free to withdraw at any time from the protocol treatment without giving reasons and without prejudicing further treatment.

CONFIDENTIALITY The Chief Investigator will preserve the confidentiality of participants taking part in the study and is registered under the Data Protection Act.

PUBLICATION POLICY Data ownership rights will lie with the institution. Findings of this study will be presented as a Dissertation and will be available through Open Access. The investigators aim to publish findings in peer-review journals.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
24
Inclusion Criteria
  • Children with Neuromuscular disease or Cerebral Palsy who have been on treatment with nebulised Hypertonic Saline for at least 12 months.
Read More
Exclusion Criteria
  • Children also diagnosed with cystic fibrosis.
Read More

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
After treatmentNebulised hypertonic salineChildren and young people with neuromuscular disease during the 12 months after being prescribed treatment with nebulised saline (0.9% - 7%)
Primary Outcome Measures
NameTimeMethod
Number of Hospitalsations Due to Respiratory ExacerbationsChange from baseline (before treatment) and 12 months after treatment

Number of respiratory exacerbations that required not planned hospitalisation

Change in Antibiotic CoursesChange from baseline (before treatment) and 12 months after treatment

Treatments due to respiratory exacerbations

Secondary Outcome Measures
NameTimeMethod
Apnea Index (AI)Change from baseline (before treatment) and 12 months after treatment

The number of apneas recorded during the study per hour of sleep

%SpO2Change from baseline (before treatment) and 12 months after treatment

Nocturnal oxygen saturation

Peak Expiratory Flow (PEF)Change from baseline peak expiratory flow at 12 months after starting treatment with hypertonic saline

Peak expiratory flow percentage of predicted

FVC% Predicted Rate of DeclineChange from baseline (before treatment) and 12 months after treatment

Rate of decline of Forced Vital Capacity (FVC) percentage of predicted. Rate of decline is a measure of slope of FVC%.

Baseline slope: \[(FVC% at baseline / FVC% 12 months before treatment) - 1\] \* 100 After treatment slope: \[(FVC% 12 months after treatment / FVC% at baseline) - 1\] \* 100

Participant's Perception of TreatmentAt 12 months after starting treatment with hypertonic saline

Questionnaire: "Hypertonic saline treatment questionnaire". Perception of overall usefulness of nebulised hypertonic saline: "Useful", "Not useful", "I don't know".

Parent's or Legal Guardian's Perception of TreatmentAt 12 months after starting treatment with hypertonic saline

"Hypertonic saline treatment questionnaire for legal guardian". Measures the perception of overall usefulness of nebulised hypertonic saline through a likert scale: Very useful, useful, neither useful or not useful, not useful, not at all useful.

Score on the Ease of Airway Clearance Pictorial Analogue Scale From Children and Young Adults as ParticipantsChange from baseline (before treatment) and 12 months after treatment

Pictorial visual scale "Facial Rating of perceived exertion Scale". Measures ease of airway clearance. Values range starting in 0 (Extremely easy) to 10 (Extremely hard), including 0, 1, 2, 3, 4, 5, 6, 7, 8, 9, 10. Numbers are also associated with facial expressions.

Score on the Ease of Airway Clearance From Parents or Legal GuardiansChange from baseline (before treatment) and 12 months after treatment

Measures ease of airway clearance through a 1-5 likert scale: 1) Very easy, 2) Easy, 3) Neither easy nor difficult, 4) Not easy, 5) Not at all easy.

AHIChange from baseline (before treatment) and 12 months after treatment

Nocturnal Apnoea Hipopnea index: total number of apnea events plus hypopnea events divided by the total number of minutes of actual sleep time and then multiplied by 60.

FEV1% Predicted Rate of DeclineChange from the baseline (before treatment) and 12 months after treatment

Rate of decline of Forced Expiratory Volume in first second (FEV1) percentage of predicted.

Rate of decline is a measure of slope of FEV1 percentage predicted.

Baseline slope: \[(FEV1% at baseline / FEV1% 12 months before treatment) - 1\] \* 100 After treatment slope: \[(FEV1% 12 months after treatment / FEV1% at baseline) - 1\] \* 100

TcPCO2Change from baseline (before treatment) and 12 months after treatment

Nocturnal Transcutaneous Carbon Dioxide in kPa

FEV1/FVC %Predicted Rate of DeclineChange from baseline (before treatment) and 12 months after treatment

Rate of decline per year of Tiffenau index

Nocturnal ODIChange from baseline (before treatment) and 12 months after treatment

Oxygen desaturation index: Number of desaturations per hour of sleep

Trial Locations

Locations (1)

Royal Brompton Hospital

🇬🇧

London, United Kingdom

© Copyright 2025. All Rights Reserved by MedPath