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Improving the care of patients with sickle cell disease

People with sickle cell disease often experience shorter life expectancy and lower quality of life, but with a better understanding of the condition and improved multidisciplinary working they can expect to live fulfilling and happy lives, says sickle cell nurse specialist Sekayi Tangayi. 

People with sickle cell disease often experience shorter life expectancy and lower quality of life, but with a better understanding of the condition and improved multidisciplinary working they can expect to live fulfilling and happy lives, says sickle cell nurse specialist Sekayi Tangayi

Sickle Cell disease is one of the most common global genetic disorders. It is a highly debilitating condition and children with sickle cell disease are more likely to have a stroke, experience shorter life expectancy and lower quality of life.

But patients with sickle cell disease are often misunderstood. Many children and adults attending A&E with unpredictable, severe and sudden onset pain may not fit the normal pain model, and young adults with sickle cell disease can often be labelled 'drug seekers'.

The severity of sickle cell pain is recognised

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People with sickle cell disease often experience shorter life expectancy and lower quality of life, but with a better understanding of the condition and improved multidisciplinary working they can expect to live fulfilling and happy lives, says sickle cell nurse specialist Sekayi Tangayi 


Sickle cell nurse specialist Sekayi Tangayi (left) with a patient 

Sickle Cell disease is one of the most common global genetic disorders. It is a highly debilitating condition and children with sickle cell disease are more likely to have a stroke, experience shorter life expectancy and lower quality of life.

But patients with sickle cell disease are often misunderstood. Many children and adults attending A&E with unpredictable, severe and sudden onset pain may not fit the normal pain model, and young adults with sickle cell disease can often be labelled 'drug seekers'.

The severity of sickle cell pain is recognised in guidance from the National Institute for Health and Care Excellence, which recommends that patients presenting at A&E should receive treatment within 30 minutes. 

Sickle cell pain cannot be ignored, and it is vital that clinicians identify and deal with the causes of pain urgently; delays in treatment can lead to serious complications and, in some cases, loss of life. 

Hundreds of thousands of children are born with sickle cell disease every year. At East London NHS Foundation Trust, the multidisciplinary sickle cell and thalassemia service cares for 333 children in an area with high prevalence rates. 

Proactive and collaborative approach

Through collaboration and team working, we have developed an effective care model, run in the community by specialist nurses working in partnership with families, haematologists and paediatricians to prevent strokes and improve quality of life for children in the area. 

Since the launch of the community-based programme in 2008, we have scanned hundreds of children and identified those with high risk velocities. During this time, only three children have had a stroke event. 

Providing out-of-hours clinics in the local community has helped improve quality of life for families. School absence rates have gone down as children no longer have to attend hospital for appointments, and we have a dedicated space for parents and young people to share experiences and support one another.  

A peer support group has also co-developed an annual national conference for patients, families and professionals to discuss innovations in care.

Sickle cell disease is a global health issue which can have a devastating effect on people’s lives. But with medical breakthroughs, more research and a better understanding of the condition, people with sickle cell can expect to live fulfilling and happy lives.


About the author

Sekayi Tangayi is service manager and specialist nurse, haemoglobinopathies, at East London NHS Foundation Trust

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