Sickle Cell Anaemia
Conditions
Brief summary
The hypothesis is that in sickle cell anaemia, nocturnal oxyhaemoglobin desaturation, is associated with low processing speed index, and this morbidity can be reduced with overnight auto Continuous Positive Airways Pressure and/or oxygen supplementation.
Detailed description
Intervention: Overnight auto Continuous Positive Airways Pressure (CPAP) with oxygen supplementation if mean overnight oxyhaemoglobin saturation is not \>94% after 2 weeks of autoCPAP
Interventions
Sponsors
Study design
Eligibility
Inclusion criteria
1. Age \>4 years. 2. Informed consent with assent in accordance with UK ethical committee(COREC) system must be signed by the patient's parent or legally authorized 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.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change in processing speed index | — |
Secondary
| Measure | Time frame |
|---|---|
| Adverse events e.g. headache, anorexia, weight loss, nausea, vomiting, reduction in steady state red or white cell count | — |
| Change in Blood pressure | — |
| Number of omissions on Conners Continuous Performance Test | — |
| Frequency of pain measured via SMS and pain diary | — |
| Change in Behaviour Rating Inventory of Executive Function (BRIEF) | — |
| Change in number of abnormalities (Adams' criteria) on TCD | — |
| Change in Chervin sleep Questionnaire | — |
Countries
United Kingdom