ARCHIVES study v.1.0
Research type
Research Study
Full title
Assessment of the Relationships between Carboxyhemoglobin, Hemolysis, Infection and Vaso-occlusive Events in Sickle cell disease (ARCHIVES study)
IRAS ID
203162
Contact name
Aubrey Cunnington
Contact email
Sponsor organisation
Imperial College London
Duration of Study in the UK
2 years, 5 months, 28 days
Research summary
Sickle cell disease (SCD) is an inherited blood disorder which affects more than 300,000 children born each year. It causes rigid “sickle” shape red blood cells, which block blood vessels and release toxic contents causing painful episodes and damage to vital organs. It also causes susceptibility to severe bacterial infections, partly because of damage to the spleen which is a major organ for removing bacteria from blood. In our preceding study, we found that children with SCD also have an abnormality in the way that white blood cells work to kill invading bacteria. This defect was due to activation of heme oxygenase-1, a molecule that is crucial for protection against the non-infectious complications of SCD. This implies a "trade-off" between avoiding one type of complication and being susceptible to another. The severity of the neutrophil abnormality varied between individuals with SCD, but could be predicted based on two measurements: number of neutrophils in blood, and carboxyhemoglobin saturation (which can now be measured by a simple probe placed on the finger). We would like to examine whether these simple markers can be used to identify patients at highest risk of infection or other types of complication. We propose to conduct a prospective study in our paediatric SCD clinic patients (approximately 150 individuals), following subjects for a period of 18 months. We will record carboxyhemoglobin saturation, and routine blood test data (including neutrophil count) at each clinic attendance. We will test whether there is a statistical association between carboxyhemoglobin concentration, neutrophil count, and risk adverse events overall, and of infectious and non-infectious events separately. The findings may help us to personalise treatment for SCD patients at an early stage in life, before complications occur.
REC name
London - South East Research Ethics Committee
REC reference
16/LO/0849
Date of REC Opinion
3 May 2016
REC opinion
Favourable Opinion