Helping young people with sickle cell to have a sense of normality

Sickle cell disease is a lifelong, inherited disorder of the red blood cells. It affects haemoglobin, which is the oxygen-carrying protein within red blood cells, and causes the cells to change to a sickle shape and become fragile. When this happens, sufferers experience acute pain in different parts of their body and sometimes need to be admitted to hospital. For young people, growing up with sickle cell presents a whole set of extra challenges. That’s why Rachael Brown, clinical nurse specialist at St Mary’s Hospital, has set up support groups for adolescents and their parents living with the disease.

In my role as a clinical nurse specialist, I am responsible for providing holistic care to children and adolescents with sickle cell disease. My patients often experience what’s known as a ‘crisis’ – where there are low oxygen levels in their red blood cells in particular areas of their body like the back, legs, abdomen or chest. The haemoglobin in the cells becomes hard, thread-like and sticky, and the cells form a blockage. We treat these crises as an emergency and patients will come to A&E and are often admitted to the ward.

When a patient’s haemoglobin has dropped, they might need a blood transfusion or an automated red cell exchange – where we remove blood containing sickle cell haemoglobin and replace it with healthy blood from a donor. These patients are in hospital for a long time and it can have a huge impact on their lives. Attendance at school can be very low, creating delays in how they progress academically and patients may also experience behavioural issues and feelings of low self-esteem. The burden of sickle cell disease can leave a young person feeling isolated and different to their friends.

Learning to manage the disease

I started a support group in 2016 because I noticed how much adolescents with sickle cell needed support to take on more responsibility for managing their condition. In the group we try to equip our teenage patients with the knowledge to prevent serious crises happening and, as a result, unplanned stays in hospital. I encourage patients to talk about what’s worrying them. For example, teenagers increasingly want to go out socially with their friends so we’ll discuss as a group how alcohol affects their condition. I teach them what their limits are, how to stay safe: drinking causes dehydration, which triggers sickling of the cells, so it’s about advising them that even if they only have one alcoholic drink, they have to drink a lot of water to prevent a crisis.

I have started organising regular educational events for the group with the help of my colleagues in the adult sickle cell service, community nurses and other healthcare professionals. One concern many patients have is what will happen when they move from being cared for by children’s services to adult services. So we hold open days in the adult service at Hammersmith Hospital and young patients visit the ward and get to meet the team who will support them. At these open days, specialists give presentations on different aspects of sickle cell disease, like how it affects your mental wellbeing.

We help young patients to understand their own condition: things like what their normal haemoglobin level is and what can trigger a crisis for them personally. Not dressing warmly enough in cold weather can be a trigger for some people, for example. I encourage patients to keep a diary and keep track of what a low haemoglobin level is for them, so that if they are admitted to hospital while they are at university or away from home, they can explain their condition to the clinicians, and receive the treatment they need faster.

Building a supportive network

As well as being educational, the group is a source of social support. Young people with sickle cell can feel very isolated – they don’t always tell their friends at school they have it, so they may not have anyone their own age to talk to about how they feel. The group is an opportunity for them to meet people going through the same experiences– studying, socialising and planning for their futures while having sickle cell. We organise to go out socially sometimes too, to have some fun.

I’ve already seen the positive effect on patients. The support group has 16 members and is slowly increasing as more young people are identified in our clinics. Since they’ve come together, they’ve really bonded. It has empowered them to seek out support when they need it: after the presentation on the effects of sickle cell on mental health, a patient came forward and told us they felt low and asked to see a psychologist.

I’ve recently set up a support group for parents of sickle cell patients. It can be very challenging caring for a child with this condition, and by creating a forum to give informal advice and a supportive network of other parents, it has helped to alleviate some of the pressure.

As patients grow up, it’s crucial we meet their holistic needs – not only looking after their physical wellbeing, but helping them to grow into happy, independent adults. As child patients turn into teenagers, they crave the normality their friends without the condition have. By giving them the information they need to manage their condition more independently and avoid crises, and by connecting them with other young people with sickle cell, we can help them create their own sense of normality.

Interested in a career in children’s services? View our latest opportunities here.

Learn more about sickle cell disease from last year’s web chat with Dr Kirstin Lund.

Learn about our children’s haematology, blood and marrow transplantation service here.