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Dr Kilali Ominu-Evbota is Co-lead of the Mid and South Essex Paediatric Sickle Cell Service. Kilali explains what sickle cell disorder is and why it’s so important that people know the signs of a sickle cell crisis.
Sickle cell disorder affects the haemoglobin in the red blood cells which makes the cells take up a sickle or – what I like to call – a banana shape.
Normal red cells are shaped like donuts. These banana shaped cells get stuck as they make their way around the body and cause a variety of problems. The commonest symptom is severe pain, and when people with sickle cell experience this pain it is known as a ‘sickle cell crisis’.
The Oxford dictionary definition describes crisis as ‘a time of intense difficulty or danger’, which is exactly what it is for people living with sickle cell. A sickle cell crisis is an emergency – just like a heart attack or a stroke, it should be treated immediately as a crisis can be fatal.
Anyone can be born with Sickle Cell disorder, but it is most common amongst people from a black Caribbean or black African background. A recent ‘No one’s listening’ report highlighted failings in care of people living with sickle cell including substandard care on general wards and in A&E, a lack of joined up sickle cell care, low awareness of sickle cell among healthcare professionals and inadequate training, negative attitudes towards sickle cell patients and inadequate investment in sickle cell care.
NHS England have recently launched a brand new ‘Can You tell It’s Sickle Cell?’ campaign to raise awareness of the signs of a sickle cell crisis, with resources available for Trusts. There is also a new free e-learning module for healthcare professionals which provides training on sickle cell and the health inequalities related to the condition. This new activity comes after the NHS struck a deal last October to roll out the first sickle cell treatment in 20 years – a drug called crizanlizumab.
In Mid and South Essex, we have been working hard to improve the treatment and care of sickle cell patients over the last few years. Patients now have 24/7 access to standardised patient-centred care.
We have created a paediatric sickle cell nurse specialist post and increased the number of nurses in the community. Patients can now email or phone a specialist nurse 12 hours a day for advice on everything from a painful crisis to care plans for school.
We have designed an integrated care pathway that is now used in all three hospitals in the trust. Patients have direct access to the paediatric assessment unit and are seen by a paediatrician at each attendance.
77% of our patients in 2021 received pain-relief within 30 minutes of arrival to the department, this is above the national average and means pain is treated promptly in our service.
A dedicated daycare unit was opened in July 2021, where children who receive monthly blood transfusions now have their treatment, rather than on an open ward as before.
We are working to maintain this current good practice by continuing to listen to service users and their families and providing care closest to home, and we are also working towards specialist haemoglobinopathy team status.
The new NHS England campaign is important because it highlights the issues and signposts people to information. I believe everyone who looks after this vulnerable group of people; clinicians, teachers and employers etc, should be aware of the signs of a crisis. If someone has symptoms of a crisis, they should seek urgent medical attention – this may be via A&E, 999 or their local haematology unit – and should be treated immediately. The signs are:
- Signs of infection including fever
- One-sided paralysis or weakness in the face, arms or legs
- Difficulty walking or talking
- Sudden visual changes
- Unexplained numbness
- Severe headache
- Breathlessness, chest pain or low oxygen levels
If a person with sickle cell says they are in pain, we must respect that and treat it promptly and appropriately.
So, it is time to ask yourself; Can You Tell It’s Sickle Cell?