Evaluating Thromboelastography (TEG) and ETP in Sickle Adults
Evaluating Thromboelastography (TEG) and ETP in Sickle Adults
The primary aim of this study is to investigate the reported enhanced coagulation status
 (prothrombotic status) in patients with sickle cell disease using 2 laboratory tests;
 thromboelastography (TEG) and Endogenous Thrombin Potential (ETP), and comparing the results
 to healthy race matched controls to ascertain if there is a significant difference. Race
 matching of the control participants is being carried out due to the well reported racial
 differences in coagulation parameters that exist in healthy individuals. The investigators
 are aiming to study the clotting state in sickle patients on regular transfusion therapy and
 those on hydroxycarbamide, both treatments offered to sickle patients to ameliorate the
 condition. The study will assess the reported prothrombotic state using TEG and ETP.
Sickel cell disease (SCD) is the most common inherited red cell disorder worldwide, the
 genetic mutation in SCD results in the production of abnormal haemoglobin (HbS). This leads
 to anaemia and unpredictable painful episodes referred to as a sickle cell crisis. Sickle
 cell crises result in significant chronic health problems including stroke, kidney failure,
 breathing problems, leg ulcers and chronic and ultimately leads to a decrease in life
 expectancy.

 The method by which sickling results in the above issues is recognised as very complex and it
 is thought these patients may have enhanced blood clotting which may play a role in the
 complications they get. Sickle cell patients are reported to have higher risk of blood clots
 such as deep vein thrombosis.

 The primary aim in this study is to investigate the reported enhanced coagulation status in
 sickle cell adult sickle cell patients using 2 laboratory tests, thromboelastography (TEG)
 and Endogenous Thrombin Potential (ETP) , the investigators will compare the results in
 sickle participants to healthy race matched control participants to show if there is a
 significant difference. The investigators also aim to study the coagulation status in sickle
 participants on a variety of treatments such as transfusion and hydroxycarbamide, both
 treatments offered to sickle patients. Many trials have shown the benefits of both
 transfusions and hydroxycarbamide in SCD, they reduce the risk of stroke and frequency with
 which patients get crises.

 To the best of teh investigators knowledge this will be the first study evaluating the
 clinical utility of TEG and ETP in adult patients with SCD

 The trial will be conducted in compliance with the principles of the Declaration of Helsinki
 and the principles of Good Clinical Practice. It has been submitted for approval to the
 London NHS Research Ethics Committee.

 Trial Design & Flowchart This is a cross sectional case controlled clinical study in patients
 with sickle cell disease. Control participants will be race and age matched participants
 without sickle cell disease. Written informed consent will be obtained from all participants.
 The samples will be taken as part of the usual clinic or inpatient procedure with 10 extra
 millilitres of blood venesected at a phlebotomist or nurse scheduled to venesect participants
 as part of their usual medical care. Healthy controls will be asked to provide a 10ml blood
 sample which will be taken by a qualified member of staff. The investigators are aiming to
 collect samples from 100 patients in total and 20 healthy controls for comparison
Inclusion Criteria: Patient participants must be over 16 years of age with sickle disease
 HbSS or HbSb0, SCD genotype which must have been previously confirmed by high performance
 liquid chromatography with results on GSTT trust electronic patient record (EPR), all
 participants must be able to give written consent.

 Exclusion Criteria:

 currently pregnant have a known diagnosis of an inherited bleeding disorder such as e.g.
 von willebrand disease on anticoagulant therapy, have severe liver disease with liver
 transaminases greater than 5x upper limit of normal.

 Healthy control paticipants will be race and age matched to the patient cohort and must
 also not be currently pregnant, have a known diagnosis of an inherited bleeding disorder
 such as e.g. von willebrand disease. The investigators will also exclude healthy
 participants on anticoagulant therapy as well as those with severe liver disease and liver
 transaminases greater than 5x upper limit of normal.