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Delivering the best care for people living with sickle cell disease
Consultant Haemotologist, Dr Kate Ryan, explains why she’s celebrating World Sickle Cell Day, and how NHS England is currently looking at the best way of delivering specialised services for people living with this serious long term condition.
Sickle cell disease is the name for a group of inherited conditions that affect the red blood cells. The most serious type is called sickle cell anaemia.
Sickle cell disease mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. Here it’s particularly common in people with an African or Caribbean family background, with around 13,000 people in the UK suffering from the condition.
People with sickle cell disease produce unusually shaped red blood cells that can cause problems because they don’t live as long as healthy blood cells and they can become stuck in blood vessels. This causes acute painful episodes as well as acute and chronic complications that can affect any part of the body.
We should use world sickle cell day to celebrate the progress we have made. A disease that once meant many children didn’t grow to adulthood, is now something that increasingly people can manage and go on to live long, busy, healthy lives. Some of this improvement is due to the availability of safe and effective blood transfusion and dedicated teams of doctors and nurses and other health professionals working, together with our patients, to a common set of care standards.
Our job in the NHS is to make sure we’re providing the right treatment and support so that everyone living with sickle cell can get on with those busy lives. And whilst recent developments are cause for celebration, we can’t forget for those living with the condition, sickle cell remains a very serious and painful condition that can only be cured in a very small minority of patients. We have hopes for the future with potential major advances in treatment including new medications, wider availability of stem cell transplantation and gene therapy. But for now enabling people to manage their long term condition while enjoying the fullest life possible, is my priority.
As well as being a Consultant Haematologist at Manchester Royal Infirmary, I am also Chair of the Clinical Reference Group (CRG) for haemoglobinopathies at NHS England. This covers sickle cell, thalassaemia and other rarer forms of anaemia. We’re currently looking carefully at the way we provide specialised services across the country for people living with these conditions to see how they could be improved. We know there are issues to address, and some challenges to getting things right. We’ve made some great progress in increasing patient access to automated exchange transfusions by using our contracts to encourage more haemoglobinopathy services to offer these.
Specialised services are generally those that are limited to a relatively small number of people at a small number of hospitals, and that in itself is a challenge – how do we make sure that people have access to the right care, at the right time and in the right place when expert advice may be in short supply?
Patients have told us that their experiences of health services can be variable in quality and access, depending on where they live, and that’s just not good enough.
Those of us who are specialists in this area are a fairly small group, and with a number of colleagues close to retirement, we need to look at developing new expertise and ways of working while making best use of the resources we have. We must remember to keep the patient at the centre of any systems we put in place, and we’ll be continuing to talk to patients and carers over the next few months as we begin to think through new ways to help people manage their ongoing care, while enabling speedy access to urgent care during painful crises, effectively.
If you are living with sickle cell, thalassaemia or a related condition, or if you have an interest in this area you can sign up as registered stakeholder for the Haemoglobinopathies CRG to receive regular updates and find out how to get involved.
Hi my daughter has a very rare anaemia Dba and I would love to be engaged with the crg .i have done some work designing a care pathway for my daughter we have been dealing with this condition for 30 years and I have a lot a knowledge on it
Thanks for your comment Angela.
Please do register as a stakeholder for the specialised haemoglobinopathies services clinical reference group (by clicking the link at the end of the blog) as we will be keeping people informed about ways to get involved and share their expertise.