Q&A: Understanding sickle cell

Nkechi Anyanwu, community matron and clinical lead for sickle cell disease, offers a primer on the condition for nurses

How did you get into sickle cell nursing?

I qualified as a nurse in 1985 in West Sussex and then as a midwife in 1988. I practised as a community midwife in Enfield, north London for about eight years.

In the 1980s there were few haemoglobinopathy counsellors but there were midwives with an interest in caring for women with sickle cell disease. At the time, Enfield didn’t have a large ethnic population so the maternity unit had no sickle cell counsellor. Occasionally, though, women came in from London who were carriers. In those days we could do a week’s refresher course as part of our learning and development and I wanted to do something with sickle cell, out of personal interest. So, I did the course at Middlesex University and then went into haemoglobinopathy nursing.

In 1998 I went to work at Whittington Hospital, which serves the London boroughs of Camden and Islington. I worked as an haemoglobinopathy counsellor for 12 years before going to Guy’s and St Thomas’ to become the community clinical nurse lead for sickle cell and thalassaemia for south-east London.

What does your role as community matron involve?

Sickle cell is a lifelong condition and the care we offer is a cradle-to-grave service. We start with genetic counselling and prenatal diagnosis, and provide ongoing care and support. We work in the boroughs of Lambeth and Lewisham and I’m based at the local centre. The job involves looking at the workforce to ensure there is cover for each clinic. We do home visits, inform parents of their baby’s results, conduct genetic counselling and screening, raise awareness in schools, and educate other healthcare professionals and the general public.

As a clinical nurse manager, my duties also include one-to-one staff supervision, staff support and development, appraisals and revalidation.

What care do you provide for those with sickle cell?

The first challenge is to support parents to give their child the best possible start, making sure they live in adequate accommodation. We also give advice on avoiding triggers for a sickle cell crisis, such as keeping the child hydrated and warm, and avoiding drafts. As the child gets older, we go into schools to support school nurses, special needs staff and health visitors, working closely with them to ensure better outcomes. We also support adult patients to live well with their condition.

We empower our patients and offer ongoing support to adults living with sickle cell. Some of them are on disease-modifying treatment, so we make sure they understand the importance of collecting their prescription, taking the medication properly, and attending appointments and follow-ups.

The illness has an economic impact and some of the patients are in employment where it’s hard for them to take time off for their appointments or if they have a crisis. We work with welfare support advisers who can help with housing and financial issues.

When is sickle cell typically diagnosed?

The NHS national antenatal and newborn screening programme¹ now tests babies at day five so we will know their haemoglobin status going forward. But there are obviously some adults who were not tested as children. There are conditions like sickle cell haemoglobin SC (HbSC), which can be slightly milder than haemoglobin SS, and patients with HbSC may be picked up during pregnancy or when they go for an operation. And some people come to the UK from other countries where newborn screening is not done or is not standard practice. When adults are diagnosed later in life, it can be quite difficult for them to come to terms with.

What are the symptoms of sickle cell crisis that nurses in general practice and primary care should be aware of?

It varies for children and adults. Children up to the age of five might have swelling in their hands and feet (dactylitis). They might feel unwell, have a raised temperature and be less responsive than usual. There can be splenic sequestration (enlarged spleen)², which is a serious complication, and the child can suddenly go quite pale and feel very unwell. There’s a risk of stroke for children with sickle cell so you should monitor changes in their behaviour. For example, is a child erratic or behaving differently, is their speech slurred, are they complaining of severe headache? It’s also important to be vigilant for small, silent strokes.

Generally, a sign of sickle cell crisis to look out for is pain. It can be spontaneous; it can be gradual from one side, and it can be fleeting as well. The patient might tell you the pain started in their arm or back and radiated to their leg. It can happen very quickly, and it can be triggered by dehydration, stress, infection or a sudden change in temperature. Observe changes in the patient’s environment, as above, and seek intervention quickly.

What other advice would you give to nurses in general practice with regard to patients with sickle cell disease?

Work closely with the families. Get to know the patient and where they get their care for sickle cell, who their consultant is and what their care plan is. A child who is in school should have a management care plan in place. It’s important to know in which hospital they receive their care, and where to seek medical help if the pain doesn’t subside or a crisis gets worse.

Are there any social or cultural considerations nurses should be aware of?

There can be stigma surrounding sickle cell and some people don’t talk about it much. Patients can keep it close within the family unit. Some people don’t want to discuss it because they don’t want to be labelled or limited. The word ’sickle’ or ‘sickler’ can cause people to think of a person as weak, but of course they’re not. So we try to move away from this terminology.

There’s also a culture around people feeling they’re not believed because there can be an attitude of, how can the person be laughing or listening to music one minute and the next minute they’re asking for pain relief? Some people don’t want to ask for help when they’re in crisis because they don’t think others will believe they’re in so much pain that they need strong pain relief. So nurses need to be mindful of their attitude towards patients.

Sometimes there are issues with patients getting repeat prescriptions from their GP. I’m not saying GPs don’t understand sickle cell, but some patients prefer to go to their hospital consultant rather than primary care.

Are there any common misconceptions?

There can be confusion surrounding sickle cell carriers and people who have sickle cell disease. In order for someone to have the disease, both parents need to be carriers of the sickle cell gene.

We used to say ‘sickle cell trait’ to refer to those who carry the gene but we’ve moved away from that now and the correct term is ‘sickle cell carrier’. If you do carry the gene, you’re likely to pass it on to your child but you will not develop sickle cell disease. The old term ‘trait’ caused confusion as some people mistakenly thought of it as a condition that would develop into ‘full blown’ disease.

Carriers don’t have any particular health issues but it could inform reproductive decisions in the future. If both partners are sickle cell carriers there’s a one-in-four chance their children will have sickle cell disease, so people need to know this. Carriers usually have no symptoms but may have problems with certain activities, such as mountain climbing at high altitude or deep-sea diving, where the lack of oxygen can cause difficulties.³

It’s important to educate patients and make sure they have the right information and support.

How much of a challenge do social and economic disparities pose in providing care and support?

There have been a number of sickle cell-related incidents over recent years. The death of a patient in hospital after what a coroner described as failures of care resulted in an inquiry led by the Sickle Cell Society. A report called No One’s Listening⁴ was subsequently published.

Patients can feel marginalised, and they’re already marginalised in society. They deserve a good standard of care wherever they are. People with diabetes can expect to be treated the same across the country and the same should apply to those with sickle cell.

The report made a number of recommendations. One was for sickle cell care to be brought into line with care for other long-term conditions, and for patients to feel equal with others in the way they are treated. The report is free to download and I recommend reading it.

Have there been any advances in treatment for sickle cell over recent years?

When I started it was mainly blood transfusion, or hydroxyurea/hydroxycarbamide as a disease-modifying treatment, but recently a new drug called crizanlizumab (Adakveo)⁵ has come onto the market to help sickle cell patients. There are other drug trials under way and gene therapy is being looked at closely. There have been other advances over the past 10 to 15 years – for example, when I started, bone marrow transplant was mainly for children, but now it’s also offered to adults.

What are your hopes for improving care for patients with sickle cell in the immediate future?

Knowledge needs to be addressed across the board. Ideally, we would include sickle cell disease as part of the nursing curriculum, at the start of foundation, so nurses take forward an understanding of the disease, its signs and symptoms, how crises can be identified and managed, when help needs to be sought, and the social and economic challenges patients encounter.

We need to start with the basics and empower people with appropriate knowledge so, for example, everyone in primary care understands the difference between being a carrier and having sickle cell disease.

We need to move care closer to where the patient lives, along with the information that goes with it. It’s about understanding and treating people with respect and dignity, believing what they say about their symptoms and empowering them to live well with their condition.

All primary care providers need to work with people living with sickle cell disease to ensure they get the best care and support that we can offer.

Nkechi Anyanwu, community matron, sickle cell & thalassaemia community services, South East London Sickle Cell & Thalassaemia Centre, and chair of Sickle Cell & Thalassaemia Association of Nurses, Midwives and Allied Professionals (STANMAP)

Interviewed by Jerome Smail

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