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Young people with sickle cell disease may experience poor care in non-specialist settings when they transition from paediatric to adult health services. New research from This Sickle Cell Life project studied their experiences on general hospital wards and during unplanned visits to A&E departments.
The research found that young people would have better experiences of care if they were recognised as experts in their own condition. Training in sickle cell disease for non-specialist healthcare staff would be helpful. Wider awareness of the condition in and outside of health care settings, including in schools, could help them achieve life goals.
Researchers interviewed young people with sickle cell disease who were moving (or had moved) into adult services. The young people felt their knowledge of how the condition affects them, and their needs, were ignored or not understood in non-specialist hospital settings, at school, and by their peers. Many stopped trusting A&E departments or general hospital wards after a bad experience. They avoided seeking medical help and instead self-managed at home.
Beyond healthcare, many were reluctant to talk about their condition in social settings. They would not mention sickle cell disease to explain their fatigue and pain, because they expected others would not understand, or would worry about them. The young people struggled to meet competing demands. They wanted to do well at school. But working hard can risk their health and they are also trying to stay healthy and take responsibility for their condition.
What’s the issue?
Sickle cell disease affects around 15,000 people in the UK; it is more common among Black people. It is an inherited disorder and means that red blood cells are less able to carry oxygen around the body.
People with the disorder experience anaemia, jaundice and extreme tiredness. Episodes of excruciating pain become more frequent as they get older and can lead to severe infections, stroke or other serious conditions. Around one in three report having pain nearly every day.
Children with sickle cell disease have to learn to manage their own health as they get older and start using adult health services. This brings challenges especially when they need urgent attention. The transition to adult care does not always go well, partly because of the change in setting, and also because of other changes at this stage of life. Children usually receive care from specialists at local centres. Adults in severe pain tend to be seen by non-specialists in A&E departments. Many of these staff have little or no experience of the disorder.
Young people (age 16-20) with sickle cell disorder report poorer experiences of care than those in other age groups, particularly during emergency attendances. And those aged 20-29 are hospitalised more often than people in other age groups. This suggests young people reaching adulthood need extra support.
The study included 48 young people with sickle cell disease. Researchers interviewed them several times throughout the transition to capture the evolving experience of living with the condition. They also interviewed 10 healthcare specialists in sickle cell disorder.
The young people transitioning to adult services described challenges which eroded their trust and led them to avoid unplanned hospital care:
- Non-specialist health-care staff do not know enough about sickle cell disease: “I was so confused… this is a hospital, they should know exactly what it [sickle cell disorder] is. But, er, it turns out a lot of people don’t know what it is.”
- Non-specialist health-care staff do not recognise people’s expertise in their own condition and needs; young people felt unheard and left out of decisions about their care: “They [staff]’re always patronising. And they think I don’t know what’s best for me . . . they have to know how I feel, about what I’m feeling.”
- Requests for pain relief are ignored or provided after delays. Several young people had sometimes felt that staff suspected them of drug-seeking: “So they [ward nurses]’ll be like saying it [that you are a drug addict] … you can hear them whispering to each other, you know, maybe his pain’s not that bad, he just wants some morphine.”
- They are ignored when they ask for help with basic bodily care: “The doctors and nurses, they kind of kept nagging at me to try and walk to the, um, the bathroom, instead of having to use a commode. Um, and I kept telling them that I couldn’t do it ’cause it was too painful and I, I had to use a wheelchair. But, um, they didn’t really listen at all.”
- Bad experiences led to them avoiding hospitals: “I try my best to hold it [pain] at home, even though I know it, it’s, it’s the worst option, I shouldn’t be doing this, I should go straight in. . . . There might not be any chairs in A&E, you might not even get like a, a bed to be on until they call you out”
- Friends, peers and adults at school/work undermine their reports of acute pain or else become over-protective. This made them reluctant to talk about their condition: “I don’t think they [teachers] were really that concerned. . . . Like if you would try to explain to them like the situation they wouldn’t really wanna hear it, they would just think it will just be like an excuse.”
Why is this important?
This research suggests that non-specialist healthcare services are failing young people with sickle cell disorder. This is a critical period in their development when they are learning to live as adults. It is difficult to manage conflicting demands, such as wanting to work hard at school but needing to rest to avoid pain. Experiences at this stage of life will impact their future lives.
If young people are treated by staff who don’t listen to their needs or have inadequate knowledge of their condition, they lose trust in the services, avoid going to hospital and potentially compromise their health. That negative stereotypes about drug use prevail in healthcare settings is particularly concerning because young people with sickle cell disease may face discrimination and be denied opioids for pain relief when they need them.
To combat these problems, the researchers say sickle cell care should be more person-centred and healthcare staff should recognise that patients are experts in their own condition and listen to their needs. The researchers suggest healthcare professionals could also work with schools to create supportive services that can help young people with sickle cell disease achieve their life goals. Young people need to be heard for more youth-friendly services to be developed.
The researchers conclude that more work is needed to raise awareness of sickle cell disease, educate the public and address the stigma associated with the condition.
The researchers presented their findings at an event at the Royal College of Paediatrics and Child Health (RCPCH). Young people with sickle cell disease, their families and healthcare professionals worked together to come up with a ‘universal sickle plan’ - recommendations for healthcare providers and the government for making transitions easier for young people with sickle cell disease.
The findings have helped shape the Self Over Sickle (SOS) programme, devised by the Sickle Cell Society to support transitions for young people with sickle cell disease and their families. The programme promotes improved physical, psychological, social and emotional well-being for young people aged 16–25 years, with resources available online, such as podcasts and events.
Further research could examine perceptions of sickle cell disease – and young patients with the condition – held by non-specialist healthcare professionals including A&E staff. This would provide insight into specific issues that need to be addressed in training. An intervention for teachers and students to aid understanding of sickle cell disorder in the community would also be helpful.
You may be interested to read
The full paper: Renedo A, and others. Understanding the health-care experiences of people with sickle cell disorder transitioning from paediatric to adult services: This Sickle Cell Life, a longitudinal qualitative study. Health Services and Delivery Research 2020;8:44
A paper on the project co-produced by academics and people with lived experience: Miles S, and others. Obstacles to use of patient expertise to improve care: a co-produced longitudinal study of the experiences of young people with sickle cell disease in non-specialist hospital settings. Critical Public Health 2020;30:5
Funding: This project was funded by the NIHR Health Services and Delivery Research programme.
Conflicts of Interest: Several of the authors have received fees from pharmaceutical companies.
Disclaimer: NIHR Alerts are not a substitute for professional medical advice. They provide information about research which is funded or supported by the NIHR. Please note that views expressed in NIHR Alerts are those of the author(s) and reviewer(s) and not necessarily those of the NHS, the NIHR or the Department of Health and Social Care.