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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
Sickle cell care needs to be brought into line with care for other long-term conditions Nkechi Anyanwu
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 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 Listening 4 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 .
• Written by Jerome Smail