Sickle cell disease (SCD) is an inherited condition that affects the shape of red blood cells, meaning they are sickle-shaped. Red blood cells carry oxygen around the body. Sickle-shaped red blood cells cannot carry oxygen around the body properly because the red blood cells stick together and cause blockages in the small blood vessels.
Over time, the blockages cause damage to the heart, lungs, kidneys and other body organs which can lead to episodes of severe pain and risks long-term diseases developing. SCD is a global problem; there are approximately 14,000 people living with SCD in the UK.
The treatment of SCD has improved and people with SCD are living longer. As people age, they accumulate more long-term medical conditions, and this is the case for people with SCD. Lots of research is exploring the effect of SCD on the lungs as adults age, but there is not a lot of research exploring the effect of SCD on the heart, specifically conditions caused by blocked blood vessels, including heart attacks (known as myocardial infarctions) and angina (known as coronary artery disease).
We would like to know whether adults with SCD are more likely to have coronary artery disease or myocardial infarctions than adults without SCD. If we find out that adults with SCD are more likely to have these heart conditions then we can start to unpick why this might be the case, which will then help us to prevent coronary artery disease and myocardial infarctions in adults with SCD.
Sickle cell disease (SCD), an inherited haemoglobinopathy, is associated with significant morbidity and mortality. Access to established management regimes mean that adults with SCD are living longer. The effects of increased exposure to the cumulative effects of SCD are generally well documented, for example retinopathy, renal disease, and pulmonary hypertension. However, the prevalence of coronary artery syndrome (including coronary artery disease, angina, and acute coronary syndrome) and myocardial infarction amongst adults with SCD is unreported despite potential common patho-physiological pathways. This knowledge gap will be addressed using a retrospective matched cohort study to describe the rate of coronary artery disease (angina and acute coronary syndrome) and myocardial infarction in a UK-based population of adults with sickle cell disease. The exposed population, defined as adults aged 18 years and older with a CPRD code pertaining to a diagnosis of SCD, will be matched for year of birth, sex, general practice and ethnicity to adults without a CPRD-coded diagnosis of SCD. Measured outcomes are event rates of coronary artery syndrome and myocardial infarction as determined through relevant codes in CPRD, Hospital Episode Statistics (HES) data and ONS Mortality data. Modelling rate of events through Poisson regression will be used to investigate the hypothesis that the rate of coronary artery syndrome and myocardial infarction is higher in adults with SCD compared to matched individuals without SCD. Prevalence and prevalence differences for each risk factor will be calculated to explore the hypothesis that traditional risk factors for coronary artery syndrome and myocardial infarction are more prevalent in adults with SCD than in matched individuals without SCD. The results will guide future research to ultimately develop cardiovascular preventative programmes for adults with SCD globally.
Rate (all events) of coronary artery disease; rate (all events) of angina; rate (all events) of acute coronary syndrome; rate (all events) of myocardial infarction.
Victoria Welsh - Chief Investigator - Keele University
Victoria Welsh - Corresponding Applicant - Keele University
Christian Mallen - Collaborator - Keele University
Chun Shing Kwok - Collaborator - Keele University
Ismail Yahaya - Collaborator - Keele University
Olalekan Uthman - Collaborator - University of Warwick
Ram Bajpai - Collaborator - Keele University
Sara Muller - Collaborator - Keele University
Toby Helliwell - Collaborator - Keele University
HES Admitted Patient Care;ONS Death Registration Data;Practice Level Index of Multiple Deprivation