Improvement of pain and quality of life in patients with sickle cell disease with auto-adjusting continuous positive airways pressure therapy

Not Applicable

Completed

• Conditions: Sickle cell disease; Haematological Disorders; Sickle-cell disorders

• Registration Number: ISRCTN46012373
• Lead Sponsor: niversity Hospital Southampton (UK)

Brief Summary

2018 protocol in https://pubmed.ncbi.nlm.nih.gov/29357947/ (added 17/12/2020)

Detailed Description

Not available

Study Timeline

• Results First Posted: 1900-01-01
• Study Start: 2015-07-02
• Study Completion: 2018-04-30
• Last Update Posted: 5 January 2021

Recruitment & Eligibility

• Status: Completed
• Sex: All
• Target Recruitment: 60

Inclusion Criteria

1. Age >=8 years
2. Informed consent with assent in accordance with the institutional policies (UK ethical committee) and European guidelines must be signed by the patient or patient's parent or legally authorised guardian acknowledging written consent to join the study. Patients < 16 years will be requested to give their assent to join the study
3. HbSS diagnosed by standard techniques (HPLC, IEF and MS). Participating institutions must submit documentation of the diagnostic haemoglobin analysis
4. Able to speak and understand English
5. Patient or parent/guardian able to use smartphone app
6. Overnight oximetry showing minimum overnight oxygen saturation of <94%

Exclusion Criteria

1. Patient already on overnight respiratory support, or has used it in the past
2. Hospital admission for acute sickle complication within the past 1 month
3. Patient with >6 admissions for acute sickle complications within the past 12 months
4. Existing respiratory failure
5. Overnight oximetry showing mean overnight saturation of <90% for >30% of total measured time
6. Severe sleep apnoea (OSA) defined by 4% ODI > 15/hr; Epworth sleepiness score >10
7. Exclusions to APAP therapy:
7.1. Decompensated cardiac failure
7.2. History of severe epistaxis
7.3. Trans-sphenoidal surgery, or trauma that could have left a cranio-nasopharyngeal fistula
7.4. Perforated ear drum
8. Bullous lung disease
9. Bypassed upper airway
10. Pneumothorax
11. Patient at increased risk of aspiration
12 Pneumocephalus has been reported in a patient using nasal Continuous Positive Airway Pressure. Caution should be used when prescribing APAP for susceptible patients such as those with: cerebral spinal fluid (CSF) leaks, abnormalities of the cribriform plate, prior history of head trauma, and/or pneumocephalus
13. Pregnancy
14. Patients on chronic blood transfusion regimes, or has had blood transfusion within past 3 months
15. Any acute or chronic condition which would limit the patient’s ability to complete the study

Study & Design

• Study Type: Interventional
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<br> 1. Pain is the most common symptoms experienced by patients with SCD and will be measured before starting treatment and in the last month of treatment using an electronic pain diary. This was used effectively in the first part of the study<br> 2. Brain function will be measured before and after six months of treatment by a psychologist who will use several different tests. The trial will also look at MRI brain scans before and after treatment and at heart function<br><br> Added 01/08/2017: As described in the study protocol (24/12/2015), this trial was powered on change in cancellation from the Wechsler scales comparing results before and after the intervention.<br>