Sickle cell disease and cell-free haemoglobin
Research type
Research Study
Full title
Sickle cell disease and cell-free haemoglobin.
IRAS ID
207799
Contact name
Ian Crocker
Contact email
Sponsor organisation
University of Manchester
Duration of Study in the UK
0 years, 6 months, 0 days
Research summary
Sickle cell disease(SCD) is an inherited blood disorder characterised by defective haemoglobin(Hb). Red cells with SCD tend to block small arteries and capillaries causing pain and organ damage. It is particularly significant in children, impacting on their physical, neurological and psychological development, restricting oxygen to the brain causing strokes, whilst permanently damaging other organs.
Although the genetic causes are undisputed, there is still much to decipher. With vaso-occlusion and haemolytic anaemia the clinical hallmarks of the condition, care looks to manage these phenomena and associated pain crisis’s, mitigating long-term secondary effects.
In defining impending stroke, Doppler ultrasound measures of compromised cerebral blood flow are used specifically in children as the procedure cannot be performed on adults.
Within the Maternal and Fetal Health Research Centre, University of Manchester, we have examined vascular failure in the feto-placental circulation in utero, and defined a role for cell-free haemoglobin(fHb) in instigating prolonged and acute feto-placental vascular compromise. We believe that this pathway is ubiquitous, and offers explanation for a range of vascular conditions, including SCD.
To explore this, a single anti-coagulated blood sample(6ml plasma) is required from children with SCD attending the Royal Manchester Children’s Hospital (RMCH). This sample will be taken at the time of their transcranial Doppler(TCD) investigations for brain blood flow. We will measure fHb in these samples and further markers for its catabolism, i.e. removal/breakdown from the body. Once defined, we will associate these measures of fHb and its handling to clinical parameters of the children, specifically TCD, but also other measures of disease course.
We predict that increases in cerebral blood flow in children with SCD will be related to fHb in our collected blood, alongside disease severity and lower responsiveness to treatment.
This knowledge may hold implications for monitoring, assessing SCD, and opening avenues for new interventions.REC name
London - Central Research Ethics Committee
REC reference
17/LO/0654
Date of REC Opinion
16 May 2017
REC opinion
Further Information Favourable Opinion