Written by Mary Isokariari on 28 September 2016
The number of people with sickle cell has more than doubled in the capital within the past five years. Despite the rise, there is widespread concern that healthcare professionals, including primary care, lack the sufficient training and awareness of Sickle Cell Disease (SCD) and thalassemia.
SCD is a genetic blood disorder affecting approximately 15,000 people in England with 10,000 of those affected based in London. The condition is more common in people of black African or African-Caribbean, Mediterranean and Asian origin.
People with sickle cell disease produce unusually shaped red blood cells that can cause problems because they do not live as long as healthy blood cells and they can become stuck in blood vessels.
It can cause episodes of severe pain known as ‘crises’ which can require hospital treatment and can even cause death without the right care.
The Sickle Cell Society founded in 1979 by a group of patients, parents and NHS staff, said the lack of understanding of sickle cell disorders and the inadequacies of treatment “contributed to patients’ bad experiences of healthcare.”
Zoe Williams, Communications and Social Media Officer, said: “We are working with Brent CCG to provide a more intensive advice and support service for Brent residents, and are hoping to secure funding to extend these services.”
The Royal College of Nursing (RCN) called for more training of healthcare staff following the results of a survey carried out by the charity Picker Institute Europe in December 2015.
Fewer than half (45 per cent) of people with SCD said that staff in emergency care settings had a good knowledge and understanding of the condition. Many avoided going to hospital for treatment because of stigma and a lack of awareness of the condition.
An NHS England spokesperson said: “Sickle cell disease can be an extremely debilitating and distressing condition which is why NHS England is committed to providing patients with the highest standards of treatment, care and support. Whilst specialist service are central to this, ensuring that local services can respond to the needs of people with sickle cell disease is also vitally important and we welcome all efforts at awareness raising amongst patients and families and all the relevant services that care for them.”
Carey Johnson, a Sickle Cell and Thalassaemia Adult Specialist Nurse, who admitted the item to the RCN Congress in June said: “Poor care for Sickle Cell Disease doesn’t just impact quality of life; it can put the patient’s life at risk.
“There are some really good specialist services in pockets around the country, but too many people have to rely on general hospitals and A&E where there is a very poor level of awareness.
“Two things would make a huge difference to the thousands of people with Sickle Cell Disease. First, provide more specialist services and secondly provide health care staff with better training about the condition.
“Health care staff always want to do the very best for their patients, but without the right training and guidance they are unable to do this. That’s why nurses are raising this important issue. Better care for people with SCD starts with better awareness and we’re determined to make that happen.”
Simon Dyson, a Professor of Applied Sociology and Director of the Unit for the Social Study of Thalassemia and Sickle Cell at De Montefort University, argues there needs to be greater priority and appropriate resources.
He said: “Legitimate questions about the lack of priority accorded to them remain. Why are clinical networks of care only just being formalised for SCD, when a network of specialised centres for haemophilia has been long established?
“Why does cystic fibrosis (which in England affects 10,000 people, compared with 15,000 with sickle cell disorders) garner more than 30 times as much financial support as sickle cell disorders?
Dr Lola Oni, a Senior Specialist Nurse Consultant at Brent Sickle Cell & Thalassaemia Centre, said the lack of awareness was due to people associating the disease as a “minority issue.”
“It’s genetic so people shouldn’t always assume sickle cell only affects black people because there are now a lot of interracial relationships and marriages. You can’t go by colour.”
Reports of negligence among non-specialist staff are highlighted in the cases of three year old Obed-Edom Bans, sent home from an NHS walk-in centre, and 21-year-old university student Sarah Mulenga, after two trainee paramedics refused to take her to hospital, both whom subsequently died.
Dr Oni said: “If we can improve education and improve attitudes this could go a long way to help people with these conditions get better care.”
“Most of the curriculums [of medical, nursing and allied professionals] in a lot of hospitals do not include sickle cell. Even in areas where hospitals have a large number of ethnic minorities, it is still not part of the curriculum, unlike diabetes and heart disease, which I think is very terrible.”
The National Institute for Health and Care Excellence (NICE) has set pathway guidelines on pain management. It states that when a person is admitted to hospital suffering from an acute painful sickle cell crisis episode, they should be treated as an acute medical emergency and the patient should receive treatment within 30 minutes of assessment.
Kenny Salami (pictured below), 35, a Youth Worker, from South London, was recently admitted at a local hospital in crisis, suffering from acute chest syndrome and joint pain.
He said: “I was ignored for 90 mins before seen by anyone in A&E and as a result my condition deteriorated. I did not receive regular medication to help with the pain because the medical staff had no access to my treatment plan. At this point, I thought he was going to die.”
After three days of inadequate care, Salami discharged himself from hospital.
A spokesperson for King’s College Hospital NHS Foundation Trust said: “We are sorry to hear Mr Salami is unhappy with the care he received. He has contacted the Trust about his hospital stay and we are looking into his concerns. We are unable to comment further until we have the outcome of the investigation.”
Salami recalled experiences of ambulance paramedics lacking the knowledge in knowing how treat to him, “They are ill-equipped on pain management and I had to tell them what to do. It’s worrying as this could potentially cost lives.”
However, a spokesperson from Association of Ambulance Chief Executives (AACE) said they had specific guidelines on how to deal with sickle patients:
“Paramedics are given extensive training to assess, treat and manage a wide variety of patient presentations including illness, injury and diseases.
While it is encouraged for nurses and healthcare staff to be more aware of SCD, the same could be applied for schools, colleges and workplaces to prevent discrimination.
Dr Oni who is working on creating medical passports for patients to carry at all times believes care plans for every child with the disease should be rolled out in schools across the UK.
“The problem we have is that the government hasn’t recognised that sickle cell is not in pockets anymore it is now very widespread. As a result, the government need to extend education to areas they originally didn’t think was a problem.
“The care plans in the schools in Brent have been successful as teachers now understand they need to keep this child warm when it’s cold, cool when it’s hot and give them opportunity to drink plenty of water and go toilet a lot more and it’s not the case they are skiving. That’s why we need to educate and change the attitudes of the teachers.”
A national survey of 569 students with sickle cell, conducted by Professor Simon Dyson, a sociologist from De Montfort University, and a team of researchers, found many of the respondents reported having negative experiences at school.
A Department for Education spokesperson said: “Schools have a duty to make arrangements for supporting pupils at their school with medical conditions, including sickle cell disease. This includes ensuring that children are able to enjoy the same opportunities at school as any other child.
“As part of complying with this duty, schools should work with the families of any affected children to agree whether it would be appropriate to develop an individual healthcare plan. This is designed to provide clarity about what needs to be done to support the child, when it needs to happen and who is responsible. Governing bodies should also ensure that suitable training is provided for any member of staff who is supporting a pupil with medical needs.”
Dyson says children are stigmatised and seen as ‘lazy’ if falling asleep in school or miss school for appointments.
“The lack of public knowledge and awareness about sickle cell must be very frustrating for people living with sickle cell in the UK. Sickle cell disorder (SCD) is the most common single-gene condition in England, with 1 in every 1850 newborn children having sickle cell disorder, and 1 in every 70 being a carrier of a gene relevant to sickle cell disorders. And yet we barely see any press or media coverage of sickle cell.
“No one is covering the fact that people with SCD have been forced out of their homes through the bedroom tax and placed in accommodation unsuitable for someone with a chronic illness, leaving them to become progressively more ill.
“No-one is covering the manner in which employers are able to discriminate against people with SCD if they have a period of illness. And no one is covering the successes and achievements of people living with SCD: they can become doctors, nurses, university lecturers, business-people, actors, journalists and much more besides.”
Salami, who was diagnosed with sickle cell in twenties, started SickleKan, a community charity group, to raise awareness about the hereditary blood disorder and provide a community platform for others to engage with and share advice and their own personal experiences of the disease.
“Sickle cell disease is underreported and has next to no funding when it comes to medical treatment and media exposure. Sickle cell has affected my education, work life and to some form of extent social life.
“I have suffered from chronic back and hip pain, reduction in vision of my left eye to 40 per cent due to crisis episodes. I also has seen a decline in physical activity and increase in fatigue. I’ve had two near death experiences mainly due to chest pain (acute chest syndrome) associated with having sickle cell disease and have had disruptions in mobility.”
“I consider myself lucky considering how many people die yearly of having the condition mainly in silence more need to be done about awareness and providing a support network for those that are dying of the condition.”