July is sickle cell awareness month, and Debbie Omodele, Children’s Haemoglobinopathy Nurse Specialist, explains the steps we’ve been taking to improve our care for children with sickle cell and thalassaemia disorder, both during Covid-19 and beyond.

Sickle cell disease is an inherited blood disorder where red blood cells become sickle or crescent shaped. Around 15,000 people in the UK have sickle cell disease and it’s particularly common in people with an African or Caribbean family background.

The main symptoms of sickle cell disease are anaemia and episodes of severe pain. 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 and are treated with strong painkillers.

We’re introducing ‘passports’ for children with sickle cell disease to help them get pain relief more quickly when attending our Emergency Departments with a sickle cell crisis.  

Debbie was one of the staff behind the launch of the passports and explains more:

“It’s recommended when children with sickle cell disease have a crisis they are given pain relief within 30 minutes of arrival to our Emergency Departments. The passport prioritises these children to be streamed quickly and to be seen by the triage nurse who will be able to administer pain relief, reducing their waiting time.”

“The feedback from parents of children with sickle cell disease has been really positive; no parent wants to queue while their child is in severe pain.”

People with sickle cell disease are in the extremely clinically vulnerable category during the Covid-19 pandemic.  Our Trust has adapted its services for patients with sickle cell disease so they can continue their treatment and reduce the risk of exposure to the virus.

Describing some of the changes that have taken place, Debbie said: “Patients who are having blood transfusions and specialist medications need to have regular blood tests. To help keep patients safe, my colleague Rachael Brown and I have been visiting patients at home to do their blood tests, to lower the risk of them being exposed to Covid-19.

“Hopefully, these patients having blood tests in the community is something which stays after Covid. Children who are having blood transfusions as part of their treatment miss out on school time to have their blood test in hospital. This is in addition to the next day spent in hospital having their transfusion.

“We’d like to eventually develop a nurse-led clinic in schools, so children with sickle cell disease and thalassaemia are missing only 20 minutes of their school day rather than hours.

“Covid has shown that some patient care can take place outside of hospital, which fits with the ambitions of the national NHS Long Term Plan.”

We’ve also adapted our services during the pandemic in other ways. Debbie said: “We are running virtual clinics, and parents with children with sickle cell are being kept up to date with information on a Whatsapp group as well as receiving letters, which is particularly important at the moment as shielding guidance has changed.”

“To help patients whose care is moving from the children’s team to the adult team, we’ve created a virtual tour of the new departments they’ll be visiting for their care, which can help them to feel more comfortable with the changes. We are also in the process of having our first virtual transition workshop as part of the transition programme.

“Where children do need to come into the hospital, we have a dedicated area within our children’s day unit department to lower the risk of transmission of the virus.”

For more information about sickle cell disease, visit the NHS website or Sickle Cell Society UK. For more information on thalassaemia, visit the NHS website or the UK Thalassaemia Society.