Monday 19 June 2023 marks World Sickle Cell Awareness Day – a great opportunity to increase awareness of this disease, and the challenges facing patients, their families and carers.
Sickle cell disease is the name for a group of inherited health conditions that affect the haemoglobin in red blood cells. People with sickle cell disorder are born with the condition, it is not contagious. It can only be inherited from both parents each having passed on the gene for sickle cell.
The main symptoms of sickle cell disorder are anaemia and episodes of severe pain. The pain occurs when the cells change shape after oxygen has been released. The red blood cells then stick together, causing blockages in the small blood vessels.
These painful episodes are referred to as sickle cell crisis. They are treated with strong painkillers such as morphine to control the pain. It’s a serious, long-term condition particularly common in people of African or Caribbean background. About 1 in 79 babies born in the UK carry the trait and about 15,000 people in the UK live with sickle cell disorder. Read more about Sickle Cell here and here.
To support local groups focussing their work on Core20PLUS5 priorities, the East Midlands AHSN provided part of their Patient and Public Involvement Fund to the Haemoglobinopathy Coordinating Centre for Sickle Cell Disorder in the East Midlands, who do great work supporting teams delivering care for patients.
In addition, the EMAHSN is also working with local NHS systems and the Coordinating Centre to implement innovative medical technology to support better care for Sickle Cell patients.
In 2021 the All – Party Parliamentary Group on Sickle Cell and Thalassaemia published the report ‘No one's Listening’, following an enquiry into avoidable deaths and failures of care for those affected by sickle cell.
To explore the topic further we invited Jodie Port, the Haemoglobinopathy Co-ordinating Centre Network Manager for the East Midlands to collaborate with us on a guest blog. This blog highlights why it’s important to maintain a spotlight on sickle cell and the need to do more to support patients and families affected by this disorder.
What is your role and the work of your organisation?
I am the Haemoglobinopathy Coordinating Centre Network Manager for the East Midlands, based at University Hospitals of Leicester (UHL). We hold the contract as the Haemoglobinopathy Coordinating Centre for Sickle Cell Disorder in the East Midlands. Across the East Midlands, we have two specialised Haemoglobinopathy teams based in Leicester and Nottingham that support local hospitals to manage the care of people living with Sickle Cell Disorder.
The overall aim of the service is to reduce levels of morbidity and mortality and improve the experience of all haemoglobinopathy patients by reducing inequities and improving timely access to high quality expert care.
My role is to support the Haemoglobinopathy teams across the East Midlands in the development of services and pathways for patients, working with clinical colleagues to create equity in services for Haemoglobinopathy patients. We are constantly looking for innovative ways in which we can support local people living with Sickle Cell. I am very fortunate to work with an amazing team across the East Midlands who work extremely hard to support patients with Haemoglobinopathy disorders.
Why is this work important?
There are around 15,000 known patients with sickle cell disorder in the UK with around 720 of those patients living within the East Midlands. These patients live with a chronic disorder that has a significant impact on their ability to work, daily living and quality of life. The work of the Haemoglobinopathy Teams within the East Midlands is to support patients in managing their health to live independent and fulfilling lives.
- The No Ones Listening report highlighted a number of key findings from the inquiry including:
- Low awareness of sickle cell among healthcare professionals and clear examples of inadequate training and insufficient investment in sickle cell care.
- Evidence of sub-standard care for sickle cell patients admitted to general wards or attending Accident & Emergency (A&E) departments.Frequent reports of negative attitudes towards sickle cell patients and a weight of the evidence suggests that such attitudes are often underpinned by racism.
What is the impact on the NHS and what are some of the health disparities and inequalities faced by people with Sickle Cell
Education and awareness of sickle cell amongst healthcare professionals outside of the specialist Haemoglobinopathy teams is limited which leads to poor patient experiences and poor outcomes. People with sickle cell are at risk of complications stroke, acute chest syndrome, blindness, bone damage and priapism (a persistent, painful erection of the penis).
Over time people with sickle cell can experience damage to organs such as the liver, kidney, lungs, heart and spleen. Death can also result from complications of the disorder. Treatment of sickle cell mostly focuses on preventing and treating complications.
New treatment options mean that patients are living longer and we are able to better support them to live well whilst living with Sickle cell.
What made you apply for the EMAHSN PPI fund and what work has it supported?
Last year, we worked with Citizens Advice LeicesterShire to develop a role that would support sickle cell patients across the East Midlands - a dedicated resource to support patients and their families with a range of welfare matters. The fund has enabled us to put on roadshow events across the East Midlands to showcase this role to patients and ensure communities are aware of the support that the service can provide. It is especially pertinent that we have entered into a cost of living crisis since we established this role, so support is needed more than ever.
Can you provide any service user quotes from the event you did with the PPI funding? Or any other service user quotes that support the work you do
The fund is a step in the right direction to raising public awareness. But it’s highlighted the need to continue both public awareness of services, and staff awareness and education to deliver better quality care for sickle cell patients.
This quote from a parent sums up the call for increased understanding and delivery of holistic care across both adult and children’s services in our region and across sectors. “I have had mixed experiences of the service with my son and have been shunted up and down the country over the years. The inpatient experience I feel is second to none. The experience for us when he was transitioning from children’s to adult services could have been better. And then he wasn’t looked after properly as an adult. There was no awareness of his condition and we weren’t advised that there was support available for us in Leicester so there were times that we had to travel to London. Quite honestly the Leicester team have saved my son’s life. Paediatric education needs to improve and general education of the condition is not very good overall outside of the specialist teams. A focus needs to be on increasing school visits and giving better awareness to teachers.”
Do you have any recommendations for the steps people / healthcare system can take to improve the way Sickle Cell is dealt with?
Improvement in staff training and awareness for healthcare colleagues working outside of haemoglobinopathy teams is key. Better awareness and education for healthcare professionals will drastically improve the experience and outcome of sickle cell patients.
From the perspective of patients and their families accessing services, continue to advocate for your own health and that of your relatives. Engagement with services is key and we encourage patients and families to lend us their voices to develop our services in a way that is meaningful and leads to the best outcomes for patients.