12 Oct 2023
Supporting Sickle Cell Disease patients
In this blog, Mary Aziz, Programme Development Lead for Health Innovation Manchester, explains how Spectra Optia, part of Accelerated Access Collaborative MedTech Funding Mandate policy, helps patients to have better care outcomes and quality of life.
Sickle Cell Disease (SCD) is a genetic, life-long condition, detected during pregnancy or soon after birth through the national screening programme.
The main symptoms experienced by people with SCD include painful episodes known as sickle cell crises, an increased risk of serious infections and anaemia.
Those with SCD also experience a significantly reduced life expectancy (NHS, 2019). Red cell exchange (RCX) transfusion is a key and often life-saving treatment for patients with sickle cell disease.
This is often required in an emergency setting for complications such as acute chest syndrome and stroke which are the most common causes of death in sickle cell disease.
However, up until October 2022, sickle cell patients received sub-optimal manual exchanges, when presenting as an emergency out of hours, due to lack of access to a 24/7 automated RCX service.
As manual exchanges are more time-consuming, carry more risk for the patient and require admission to critical care, there was a great need to standardise care, ensuring all have access to automated red cell exchange.
This was able to be achieved due to the MedTech Funding Mandate (MTFM) 2022/23.
The MedTech Funding Mandate aims to improve access to NICE approved technologies to standardise care and reduce healthcare inequalities.
The MTFM includes use of the Spectra Optia apheresis machine to enable automated red cell exchange for those with sickle cell disease who are eligible for exchanges.
The MTFM enabled wide system collaboration, which was instrumental in overcoming barriers to implementation due to the establishment of a national working group, consisting of:
- NHS England Specialised Commissioning Director of Finance
- Accelerated Access Collaborative Policy team
- NHS England National Programme of Care – Haemoglobinopathies
- A National Healthcare Inequalities Improvement Programme member
- The National Speciality Clinical Advisor for Haemoglobinopathy
- Terumo (Spectra Optia technology supplier)
- Co-opted clinicians from a variety of Trusts
- The Sickle Cell Society
Health Innovation Manchester was key to raising awareness of the need, in GM, nationally, allowing the Manchester case to be the first considered and approved by the National Working Group.
The North-West Haemoglobinopathy Co-ordinating Centre submitted a case to this working group, proposing for emergency automated red cell exchange provision to occur via NHS Blood and Transplant (NHSBT) across the region.
It was a great achievement for this case to be approved in July 2022 with immediate funding being provided via specialist commissioning.
As a result, as of October 2022, there is now improved equity of care for patients presenting out-of-hours across the North-West.
All those eligible can now receive high quality care equal to that provided to in-hours patients and out-of-hours patients in neighbouring areas.
Since implementation of the new service model, 13 people have benefitted, receiving an improved patient experience. A knock-on impact is the improvement in patient-flow due to the increase in critical care capacity allowing for other patients requiring admission to utilise these beds.
Without this provision, patients would have been admitted to critical care to receive manual emergency red cell exchanges resulting in an increase in length of stay and added pressure on workforce capacity.
A patient case study: Gloria Ademolu – the Automated Red Cell Exchange Service
Gloria was born with Sickle Cell Anaemia, a condition which remained undiagnosed until she was three years old when she suffered her first episode of extreme pain during crisis.
Gloria would often experience crisis monthly, where her pain resulted in her being hospitalised and requiring blood transfusion.
In 2018, Gloria was due to start a degree in Software Engineering at Manchester Metropolitan University, when she suffered two Sickle Cell crises in just three months.
These crisis episodes led to her consulting her GP who, after assessing her, advised that she was placed onto the Automated Red Cell Exchange service in Greater Manchester in November 2022 – helping her to manage her condition and allow her to study her degree.
She explained: “After consulting my doctor at the time, we decided to place me on Red Cell Exchanges (RCE) every six weeks, to keep my sickle down and allow me to complete my degree without a crisis.
“This worked, and although I was still in pain, I did not have a crisis. I hadn’t heard about the emergency Red Cell Exchange Service, rather it was an emergency and my doctor decided I needed one.”
After medical assessment at Manchester Royal Infirmary, Gloria was diagnosed with an infected Peripherally Inserted Central Catheter (PICC) line and a chest infection and was quickly moved to the Acute Medical Unit (AMU) ward for further observation.
She was then transferred to the High Dependency Unit (HDU) for urgent Red Cell Exchange treatment. Gloria spent 14-days at Manchester Royal Infirmary where haematology nurses carried out the Red Cell Exchange.
Since receiving the Automated Red Cell Exchange treatment, Gloria has seen vast improvements in her health and has expressed confidence in the service for other patients suffering from Sickle Cell disease.
“It is something I must live with forever and I want to use myself as an example to other sufferers that awareness is growing, and treatments are starting to get better, so we don’t need to suffer needlessly,” she said.
“As much as I do not pray to have another crisis, should I do, I would want to have the same treatment plan I had. If I had not gotten admitted, I don’t know what would have happened to me.”
Dr Tracey Vell MBE, Medical Director, Health Innovation Manchester, said: “It has been truly exciting to see a diversity of people and organisations link together to ensure the highest standard of healthcare is delivered to an often-over-looked population. The benefits experienced exemplify the positive impact which can result from a unified approach across the system. I look forward to seeing the continued positive impact this work will have on people with Sickle Cell Disease in Greater Manchester.”
Dr Joseph Sharif, Consultant Haematologist & Northwest England HCC Deputy Clinical Lead in Sickle Cell, said: “The new pathway will ensure that patients with sickle cell will have access to emergency automated red cell exchange regardless of which hospital in the region they are being treated at.”