Today (19 June) is World Sickle Cell day, an annual event to help raise awareness and increase public knowledge of the disease, which is the most common disorder caused by a single inherited genetic mutation in the UK.
To mark the day, we spoke with BSH member Dr Sonia Wolf about Sickle Cell Disease (SCD), and how haematology professionals can improve the care they give to patients with this disease.
Recently Sonia won the inaugural ‘Crucible Prize’ at the BSH Annual Scientific Meeting in Liverpool for her presentation 'Young, Black and Stigmatised' which focused on how haematologists do most harm to patients with SCD. Here Sonia elaborates on her presentation, why raising awareness of SCD is important, and what haematology professionals can do to improve the patient care they deliver.
Why is it important to raise awareness of SCD amongst the haematology community?
It is vitally important that we raise awareness of how to deal with and treat patients with SCD as haematology specialists. SCD is the most common disorder caused by a single inherited genetic mutation in the UK, and yet I don’t think we know how best to treat it yet.
In my view this comes down to how we are profiling and stigmatising SCD patients due to age and ethnicity – a study found that 33% of haematologists think over 10% of patients with SCD are addicts and 5% think it's over 90% (Shapiro et al, 1997). This institutionalised bias directly impacts on the hospital care patients will receive, and also has an impact on stifling advances in our understanding of the disease.
How can haematology professionals address this problem?
First, people must examine their own prejudices, and in doing so they can begin to tackle their biases, which in turn will improve the level of care you are able to deliver to patients. We cannot be effective advocates if we do not get our own house in order first.
We also need to examine the language we use, which in the case of SCD patients can often have negative connotations – if you witness a colleague using pejorative language about SCD patients, don’t be afraid to challenge it. That way more people can review their language, and importantly, their beliefs about SCD patients.
Lastly, we need to recognise how to see the patient, and not the disease. This is vitally important because we don’t want people to be defined by their disease. They are individuals, with hopes and dreams like everyone else, who happen to have a disease. Ask yourself, ‘do they need to be in hospital today?’. With a large proportion of SCD patients being in their teens and mid-twenties, we risk denying them their education and social lives that are so crucial amongst this age group.
Facts that haematology professionals may not know about SCD?
Our patients often live in poor socio-economic conditions – 60% live in the bottom quartile of deprived areas, rising to 80% in London (AlJubri et al, 2013). This often impacts on their physical and psychological health in ways we could not even predict, for example one colleague told me of patients who could not store medication that needed refrigeration because they could not afford to have a fridge. Unless we ask directly about these facts we may miss them. The best input we can give is multi-disciplinary, addressing their educational, social and psychological issues as well as their physical ones.
A lot of patients – and doctors – are afraid of hydroxyurea because they have heard negative stories about leukaemogenesis and fertility. The new BSH guidelines on the use of hydroxyurea are fantastic because they review the literature, which suggests that there is no evidence that hydroxyurea increases risk of leukaemia. When it comes to sperm analysis, a lot of patients with SCD already have abnormal sperm production secondary to the disease. Although hydroxyurea may cause some changes in sperm production, it seems that this recovers in most patients on stopping the drug, and in any case, there is no evidence that this correlates with an actual reduction in male fertility. In any case, the guidelines suggest considering semen analysis and cryopreservation before starting treatment, which seems like a sensible, pragmatic approach.
Common mistakes to avoid when treating SCD?
Patients withdrawing from opiates can often present with symptoms similar to that of a crisis, and in fact patients may not even be able to distinguish this themselves. Although both may get treated with opiates in the emergency department, a careful history is required to try and work out what is going on. Similarly, do not assume that a patient who presents frequently is not having a crisis – this often happens, and they can become quite unwell quite quickly. Remember to check their oxygen saturations off oxygen (providing they are not too low) as these patients are often young and can compensate well, particularly in the early stages of an acute chest syndrome, as well as other observations at least every 4 hours.
Sickle cell patients know their disease – and the best treatment for themselves often really well. Because SCD patients are often used to quite large doses of opiates, people (particularly non-haematologists) are often surprised at how much they are on, and many doctors sadly make negative assumptions about this. This leads to some patients being prescribed tiny doses of opiates which do not help their pain and lead to established crises with longer admissions. The best thing to do is check their protocol, and if they come from a different hospital, to ring that hospital and check it.
Sickle cell patients are often prescribed opiates for chronic pain, but opiates do not work especially well for this, and can lead to other problems such as dependency, especially as 1 in 3 SCD patients have pain almost every day. Consider disease modifying therapy such as hydroxyurea or a trial drug; non-opiateanalgesia such as neuropathic pain medication and psychological therapy, or pain management strategies in combination with our colleagues in psychology and pain medicine.
*The contents of this article do not reflect an official BSH position. We would value your thoughts on this article and hope it provokes discussion within the haematology community.