Ethnicity and Health Unit & Sickle Cell
NIHR ARC NWL's predecessor NIHR CLAHRC NWL has been working with the Sickle Cell Society, Picker, the London School of Hygiene & Tropical Medicine and public partners, for several years to support Sickle Cell research with aims to improve quality of life and quality of services for those living with Sickle Cell Disease.
Ganesh Sathyamoorthy, Assistant Director of the Ethnicity and Health Unit, has been a trustee for the Sickle Cell Society for the last 6 years. We continue to work closely with the Sickle Cell Society on ongoing projects and research.
Through the Ethnicity and Health Unit, NIHR ARC NWL plan on building on our historic progress.
What is the health problem?
Sickle Cell Disease is a chronic multisystem disorder resulting in debility, organ damage, frequent hospitalisations and reduction in lifespan. It affects over 12,500 people in England. SCD is the nation’s most common genetic disorder with approximately 240,000 carriers. Despite this, there is minimal awareness of this disorder and there was little or no evidence available in the UK as to what matters most to those living with SCD, nor any tools available to appropriately and reliably capture their experiences of care.
No One's Listening
A groundbreaking inquiry report published by the All-Party Parliamentary Group (APPG) on Sickle Cell and Thalassemia, on the 15th November 2021, highlights shocking failures as cross-party MPs call for major changes into care for sickle cell patients. The inquiry, led by Rt Hon Pat McFadden MP, Chair of the APPG on Sickle Cell and Thalassaemia, has found “serious care failings” in acute services and evidence of attitudes underpinned by racism.
The ‘No One’s Listening’ report, which is based on the inquiry’s findings, is jointly published by the APPG on Sickle Cell and Thalassaemia and the Sickle Cell Society.
Key findings from the inquiry include:
evidence of sub-standard care for sickle cell patients admitted to general wards or attending Accident & Emergency (A&E) departments (including a widespread lack of adherence to national care standards)
low awareness of sickle cell among healthcare professionals and clear examples of inadequate training and insufficient investment in sickle cell care
frequent reports of negative attitudes towards sickle cell patients and a weight of the evidence suggests that such attitudes are often underpinned by racism
The inquiry also found that these concerns have led to a fear and avoidance of hospitals for many people living with sickle cell.
What did we do about it?
Launched Nationwide Survey
NIHR CLAHRC Northwest London, in partnership with the Sickle Cell Society, commissioned the first nationwide survey of experiences of SCD care in the UK. A Patient Reported Experience Measure (PREM) was designed by Picker. The PREM was identified as a key requirement for evaluation of services. With three audience-tailored surveys (children, parents and adults), the PREM collected over 700 responses nationwide.
The survey was informed by focus groups to ensure it covered areas of care that are most important to patients and their families, as well as capturing key elements of person-centred care. The survey concentrated on access to care services, information and support for their condition, as well as capturing views on healthcare across inpatient, outpatient and emergency care settings.
Examined Transition to Adulthood
This Sickle Cell Life is a collaborative research project at London School of Hygiene & Tropical Medicine (LSHTM), funded by the National Institute for Health and Care Research (NIHR) Health Services and Delivery Research Programme. The experiences of young people with sickle cell was examined as they transitioned to adulthood and move from using child to adult services. The project aimed to facilitate dialogue about how transitions into adult healthcare can be improved, including how to improve health services for people with sickle cell.
What happens when children become adults can affect their future lives. If you have a long-term condition you must learn to manage it yourself more as you get older. The healthcare services you use change as you transition to adult services. Those transitions do not always progress smoothly, which can have major effects on young people. This Sickle Cell Life has been exploring how young people with sickle cell disease move from using child to adult healthcare services.
What have we found?
PREM Findings
UK's First National Sickle Cell Disease Patient Reported Experience Measure (PREM)
Over 1 in 3 (38%) did not have enough information about coping with pain.
1 in 5 (21%) did not have enough information about different treatment options but would like this.
Only 1 in 3 adults reported that emergency staff helped to ease their pain quickly enough.
2 in 3 children said that the ward they stayed on was ‘definitely’ suitable for their age – compared to only 2 in 5 adults.
Nearly two thirds (62%) of respondents said that healthcare staff do not give enough information to others (eg their school, college or workplace) about their condition and how it affects them.
The majority of respondents (86%) said that their friends do not know enough about their condition.
Londoners responded more positively than those living outside of London on:
Perceived staff knowledge of SCD (planned and urgent care settings)
Having enough information about treatment options for SCD
Not having to repeat their story to different staff.
We Collaborated with NIHR CLAHRC East Midlands who promoted the use of the Sickle Cell PREM, including at their ‘BIG HEALTH EVENT’ on 16 October 2017. CLAHRC East Midlands designed a youth friendly comic version of our infographic to highlight our findings.
This Sickle Cell Life Findings
Barriers to practising patient expertise
We found that a key challenge for young people with SCD during transition relates to barriers to having a voice about their condition and practising patient expertise - knowledge of their body and of how the condition affects them – in interactions with others. On the one hand healthcare providers and parents/carers demand that the young person must learn more about their condition and body, and act responsibly to stay healthy. On the other, when the young person attempts to practise these new behaviours and use their patient expertise for instance by making requests about the care they receive, they are often disregarded, questioned or made to feel invisible. This feeling of not being listened to happened most within non-specialist healthcare settings during unplanned visits including Emergency care and on general hospital wards, as well as in social contexts such as school.
Mistrust in non-specialist care
The main areas of concern for young people with regards to obstacles to receiving quality unplanned hospital care included pain relief needs not always being managed in a timely way; being ignored when asking for help with basic bodily care; and non-specialist staff not knowing enough about SCD. These healthcare experiences during the period of transition can negatively affect young people’s healthcare-related behaviour. Our findings suggest transitioning can involve young people with SCD developing mistrust in non-specialist hospital care. This can lead to avoidance tactics to stay out of hospital as much as possible. Going to hospital for unplanned care was viewed as a last resort, and participants pushed themselves to manage painful episodes at home despite knowing this could pose serious health risks. They also worried about the impact of their condition on their parents/carers. They learned how to protect parents/carers by concealing pain.
Struggle to develop adult identities
Our study also found that young people with sickle cell disease struggled to develop adult identities in healthcare transitions; that is, new ideas about how they should behave as adult patients. Young people saw themselves as in constant need of improvement to reconcile plural demands on the self; to produce bodily efficacy and stay healthy while working hard to become competent in other areas of their life, such as education, in order to become the type of productive adult they are hoped to be. Fulfilling educational demands is difficult for young people with SCD, who have to spend time in hospital, or who are in pain, or have to rest regularly to avoid painful episodes. At the same time, healthcare staff and parents push young people to take individual responsibility and become competent at self-management. The difficulties in trying to develop self-actualising adult identities whilst trying to be a disciplined adult patient can be played out in stigmatising identities including perceptions of 'laziness'.
‘This Sickle Cell Life’ Participatory stakeholder event
We ran a participatory event for ‘This Sickle Cell Life’ in April 2019, which provided us with an opportunity to engage directly with stakeholders, including young people with sickle cell disease and other long term and/or chronic conditions, parents and carers, sickle cell charity staff, policymakers, clinicians and researchers.
Working with the RCPCH &Us involvement team at the Royal College of Paediatrics & Child Health (RCPCH), we asked attendees to reflect on the research findings and draw on their different backgrounds and experiences to work together to co-produce ideas for resources to improve transitions that can be targeted to different stakeholders involved in the care of young people with SCD.
The participatory workshop fostered an extended dialogue between young people, carers and specialists with key stakeholders who we specifically wanted to influence such as A&E doctors and non-specialist healthcare providers and educational staff. They collaboratively reflected about the implications of our qualitative research findings for practice and co-produced ideas to improve transitions for young people with SCD at a personal, community and strategic level.
Attendees made their own pledges to support the future of ‘This Sickle Cell Life’. The event allowed us to reflect on the implications of our findings for practice, and in some cases turn our research findings into action to improve experiences of young people with sickle cell disease. The event was positively evaluated across the range of attendees. Evaluation feedback from non-specialist NHS clinicians was also positive: one respondent pledged to improve hospital emergency department training; another wrote of their intention to amplify the voices of young people at their emergency centre when designing care approaches.
How can you help?
We welcome collaboration and partnership opportunities from patient, carers, public partners, charities, industry, academic and healthcare trusts and professionals.
Useful Resources
FINAL_Guide-To-Sickle-Cell-and-Employment-Version_1_2019.pdf