Sickle cell anaemia Haematological Disorders Sickle-cell disorders
Conditions
Interventions
Overnight auto Continuous Positive Airways Pressure (CPAP) with oxygen supplementation if mean overnight oxyhaemoglobin saturation is not more than 94% after two weeks of autoCPAP versus no treatment.
Sponsors
Institute of Child Health (UK)
Eligibility
Inclusion criteria
Inclusion criteria: 1. Age more than four years old 2. Informed consent with assent in accordance with UK ethical committee (Central Office for Research Ethics Committees [COREC]) system must be signed by the patient's parent or legally authorised guardian acknowledging written consent to join the study. When suitable, patients will be requested to give their assent to join the study 3. Haemoglobin SS (homozygous sickle cell anaemia) diagnosed by standard techniques. Participating institutions must submit documentation of the diagnostic haemoglobin analysis
Exclusion criteria
Exclusion criteria: 1. Existing respiratory failure 2. Decompensated cardiac failure 3. History of severe epistaxis 4. Trans-sphenoidal surgery, or trauma that could have left a cranio-nasopharyngeal fistula 5. Perforated ear drum 6. Bullous lung disease 7. Bypassed upper airway 8. Pneumothorax 9. Pathologically low blood pressure 10. Cerebral Spinal Fluid (CSF) leaks, abnormalities of the cribriform plate, prior history of head trauma, and/or pneumocephalus 11. Patients on chronic regular blood transfusion 12. Patient who received treatment with anti-sickling drugs or hydroxyurea within three months 13. Patient with other neurological problems, such as neurofibromatosis, lead poisoning, or tuberous sclerosis 14. Pregnancy 15. Sinus or middle ear infection (temporary)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change in processing speed index. | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Frequency of pain measured via SMS and pain diary 2. Adverse events e.g. headache, anorexia, weight loss, nausea, vomiting, reduction in steady state red or white cell count 3. Change in blood pressure 4. Number of omissions on Conners Continuous Performance Test 5. Change in Chervin sleep questionnaire 6. Change in Behaviour Rating Inventory of Executive Function (BRIEF) 7. Change in number of abnormalities (Adam's criteria) on Trans Cranial Doppler (TCD) | — |
Countries
United Kingdom
Outcome results
None listed