Sickle Cell Disease
What is Sickle Cell Disease
Sickle cell disease is a disorder of haemoglobin, the iron and oxygen carrying molecule in the blood. It is now the commonest inherited single gene disorder in the U.K. with over 300 new patients being born annually. There are estimated to be 12,500 people with sickle cell disease in England, with a large proportion of these living in London. Sickle cell disease may be caused by the co-inheritance of the sickle cell disease with another sickle cell gene i.e. HbSS; with another abnormal haemoglobin e.g. HbSC, HbSOArab, HbSD; or with a thalassaemia gene i.e. HbSbeta thalassaemia. Patients with sickle cell disease may suffer a variety of complications, some acute and some longer term. These conditions are complicated and difficult to manage and may lead to rapid clinical deterioration. Whilst in years gone by, patients were only expected to live into their third decade at best, now we have most our patient living into later adulthood and a large cohort now in older age. It is by a holistic preventative approach combined with access to specialists and specialist treatment e.g. red cell exchange and prompt and efficacious management of problems when they occur that patients can realistically hope to achieve the many things that a healthy life has to offer.
What can Dr Trompeter offer?
Dr Trompeter can:
- See patients as part of their annual review and general check ups
- Counsel regarding disease modifying therapy
- Supervise and arrange treatments e.g. red cell exchange and hydroxyurea
- Initial assessment of possibility of bone marrow transplant
- Prepare patients for operative procedures or during pregnancy
- Counsel carrier couples at risk of having a child with sickle cell disease and arrange for prenatal diagnosis or preimplantation genetic diagnosis, potentially creating bone marrow matches for already affected children.
- Admit patients for management of a sickle cell crisis onto the inpatient ward.