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NHS patients with sickle cell disease will be able to benefit from a groundbreaking gene-editing treatment that offers the prospect of a cure for the condition. The one-off gene therapy, known as exagamglogene autotemcel (or ‘exa-cel’), has been approved for use on the NHS in England by the National Institute for Health and Care Excellence (NICE) for older children and adults with a severe form of sickle cell disease. Clinical trials suggest exa-cel can stop painful and unpredictable sickle cell crises – the most common symptom of sickle cell disease – where blood vessels become blocked causing severe pain, with experts saying the therapy offers patients a chance of disease-free life. Researchers concluded there was a ‘functional cure’ in 96.6% of exa-cel trial participants that received it. NHS chief executive, Amanda Pritchard, said: “More than a step, this is a leap in the right direction for people with sickle cell disease – which can be an extremely debilitating and painful condition. “This innovative, gene-editing therapy offers hope of a cure for people facing a severe form of the disease and could be absolutely transformative – it could enable patients to live free from the fear of sickle cell crises hanging over them. “It is just the latest in a series of revolutionary gene therapies NHS England has secured for patients, and we are funding this new treatment option straight away so patients can benefit from the enhanced quality of life it offers.” Read full story Source: NHS England, 31 January 2025 -
Content Article
Patient for Patient Safety India and National Thalassemia Welfare Society in collaboration with World Patients Alliance (WPA) and Global Action Network for Sickle Cell and Other Inherited Blood Disorders (GANSID) organised a webinar on Friday 20 Sep 2024 to mark World Patient Safety Day (WPSD). Since the theme of WPSD 2024 was focused on diagnostic errors, the webinar was on diagnostic errors in blood disorders thalassemia, sickle cell anaemia and haemophilia. Read the summary of the webinar attached.- Posted
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Health bosses are aiming to tackle “poor care” faced by many children and young people living with sickle cell disease when they move to adult healthcare providers. NHS Race and Health Observatory, an independent body which examines inequality in healthcare, will work with the Sickle Cell Society charity with the aim of improving the transition for patients into adult care. It is estimated around 300 babies are born with sickle cell disease each year in the UK and young people with the illness “often experience poor care in non-specialist settings” when moving to adult care, the body said. Professor Habib Naqvi, chief executive of NHS Race and Health Observatory, said: “By improving trust within the care pathway we can tackle gaps in care, learn from successful outcomes in transition from other disorders and initiatives, and ensure excellent patient-centred care is provided." Read full story Source: The Independent, 6 October 2024- Posted
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Sickle cell drug is withdrawn over safety concerns just months after rollout
Patient Safety Learning posted a news article in News
The sickle cell treatment voxelotor (Oxbryta) has been withdrawn from all markets by the manufacturer Pfizer after a “higher than anticipated” number of deaths were reported and clinical evidence showed that the drug’s benefits no longer outweighed the risks. In a recall notice issued on 30 September the UK Medicines and Healthcare Products Regulatory Agency (MHRA) told clinicians and patients that “emerging data” suggested an “unfavourable imbalance in vaso-occlusive crises (acute painful crises) and fatal events (deaths)” in patients treated with the drug. The agency shared a letter from Pfizer with UK healthcare professionals, which said that all patients currently having voxelotor prescribed must be contacted and advised to discontinue treatment and, where appropriate, discuss alternative treatment options. Patients should be instructed to return the product to the hospital pharmacy or homecare company that dispensed it. Read full story (paywalled) Source: BMJ, 1 October 2024- Posted
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Life will never be the same for Leila and Errol after the death of their 22-year-old son Darnell, who lived with disabilities including sickle cell disease. Darnell Smith died on 23 November 2022 after medics took his vital signs just once in 12 hours instead of the prescribed hourly checks. Earlier this year, a coroner called on health bosses at Royal Hallamshire Hospital in Sheffield to take action to prevent future deaths. Darnell, from Sheffield, received regular treatment for cerebral palsy and sickle cell disease, which is an inherited, long-term and potentially life-threatening condition, most common among Black people. It affects about 15,000 in England. As the UK observes Sickle Cell Awareness month in September, Leila and Errol say they do not feel the inquest’s ruling went far enough. “What we would like is more accountability from the NHS and the members of staff who were working that day,” Errol said. They say his case reflects the failures of care experienced by patients with sickle cell. “I feel like there’s still a lot of injustice still being done, not just because of what happened to Darnell but also to other sickle cell patients within hospital settings all over England. It’s not just in Sheffield,” Leila said. “There’s a lot of systematic racism, a lot of discrimination, whether it’s blatant, it’s there. We felt it, we saw it and I said that in the inquest.” A 2021 all-party parliamentary group (APPG) on sickle cell and thalassaemia inquiry into sickle cell care found “serious care failings” in acute services and evidence of attitudes underpinned by racism. Read full story Source: The Independent, 28 September 2024- Posted
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Sickle cell: NHS to investigate racial inequalities for first time
Patient Safety Learning posted a news article in News
Sickle cell patients’ experiences of barriers to treatment and racial inequalities will be investigated by an NHS body next month, The Independent has learned. The NHS Race and Health Observatory has collaborated with Public Digital, a consultancy group, to lead original research into the experiences of people with sickle cell, including listening to NHS patients’ and carers’ first-hand accounts of acute emergency hospital admissions and managing the condition at home. Research will focus on a series of interviews and ‘experience mapping’ workshops, the findings of which are anticipated to inform recommendations that will help improve emergency care and treatment pathways. “As a priority, we need to discover new measures and treatment plans that can help eradicate the often unacceptable, substandard care people with sickle cell have historically received whilst being unwell and in acute pain,” Dr Habib Naqvi, Director of the NHS Race and Health Observatory, said. This move comes after a parliamentary inquiry into avoidable sickle cell deaths called upon the Observatory to undertake work into sickle cell care in relation to race and ethnicity. The inquiry published a report, ‘No one’s listening’, in November 2021, which uncovered the bleak reality of patients grappling with racism in the NHS while attempting to access healthcare. Only half of healthcare professionals feel they have sufficient tools to manage the long-term damage that sickle cell disease brings, new research from Global Blood Therapeutics found, following extensive studies carried out across 10 countries including the UK, US and Canada. Read full story Source: The Independent, 22 September 2022- Posted
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Only half of healthcare professionals feel they have sufficient tools to manage the long-term damage that sickle cell disease brings, new research has revealed. The in-depth study by Global Blood Therapeutics - carried out across 10 countries including the UK, US and Canada - shows that patients living with the illness remain dramatically underserved by healthcare systems, while healthcare professionals don’t feel like they have the knowledge of the disease or their patients, to properly treat them. More than two in five (43%) doctors and nurses cited difficulties due to having different ethnic backgrounds from their patients, it was revealed, while almost three quarters (73%) stated patients of lower economic status can be more difficult to treat. Almost a third of healthcare professionals (31%) found it challenging to understand their patients’ needs. Sebastian Stachowiak, Head of Europe and GCC at Global Blood Therapeutics, told The Independent that the survey “confirms the lack of options for physicians” and expressed hope that, with recent advances in available treatment, patients can be better served in the future. The study also found that almost half (46%) of patients say that emergency room healthcare providers did not believe them about their symptoms, while 48% said that they have been treated like a drug seeker in the emergency room. Read full story Source: The Independent, 14 June 2022- Posted
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Sickle cell: NHS staff ‘failing to follow healthcare guidelines’
Patient Safety Learning posted a news article in News
NHS staff are failing to follow guidelines for providing care to sickle cell patients - and some of the advice has been branded as “unfit for purpose”. The NHS Race and Health Observatory commissioned research, undertaken by Public Digital, to explore the lived experience of people undergoing emergency hospital admissions for sickle cell and managing crisis episodes at home. The Sickle cell digital discovery report: Designing better acute painful sickle cell care, found that the existence of service-wide information tailored by the National Institute for Health and Care Excellence has “arguably not been designed for an ambulance, A&E and emergency setting”, and states it has been proven that this guideline is “not being used and adhered to consistently”. Moreover, healthcare professionals have warned that the National Haemoglobinopathy Register (NHR) - a database of patients with red cell disorders - is not being readily accessed, while patients reported being treated in a way that breached prescribed instructions. “We believe that sickle cell crisis guidelines could be improved in terms of their usability in a high-pressure emergency setting, and in terms of promoting access to them,” the report authors concluded, adding that current guidance should be adapted. Read full story Source: The Independent, 31 January 2023- Posted
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These standards for the clinical care of adults with sickle cell disease were produced by the Sickle Cell Society in collaboration with a broad multi-disciplinary group of healthcare providers, patients and support groups. The standards cover the following areas of care and treatment: Organisation of care Primary care Health and wellbeing Acute and chronic pain Neurological complications Cardiorespiratory complications Renal and urological complications Priapism Fever and sepsis Orthopaedic complications Gastroenterological and hepatobiliary complications Ophthalmological complications Anaemia Leg ulcerations Outpatient management Reproductive health Surgery Hydroxycarbamide Blood transfusion Iron chelation Haematopoietic stem cell transplantation Emerging therapies- Posted
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Imagine you have a disease that leaves you in severe pain, and frequently means you need emergency strong opioid pain relief. But your condition is rare, and A&E staff often have very limited knowledge of your condition. And on top of that you’re black, and staff assume you’re drug seeking, and this happens over and over again. This is just one of the ways people with sickle cell disorder face discrimination when trying to access health care. Is it any wonder you’d rather avoid the health and care service than have substandard care and/or face stigmatisation?- Posted
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Chronic pain is the most common complication affecting adults with sickle cell disease (SCD).Pain profoundly affects people’s quality of life, functional ability, and health care utilization. Clinicians are often unsuccessful at addressing chronic pain in SCD, especially among the large number of patients for whom nonopioid analgesics aren’t sufficient and those who have developed opioid tolerance. Why aren’t we doing better? In this perspective article in the New England Journal of Medicine, Childerhose et al. discuss how a biopsychosocial model can help capture people’s experience of chronic pain by affirming that biologic, neuropsychological, and socioenvironmental elements play a role in pain-related processes. -
News Article
Sickle cell patients are being put at risk because of a chronic shortage of specialist nurses to treat them, a damning new report has found. 'The Difference Between Life and Death', a new study by the Sickle Cell Society, found that there are not enough sickle cell workers to deliver a good standard of care. One patient called Abi Adeturinmo told researchers that previous traumatic experiences caused by delays in receiving pain relief medication and poor care meant she “tries not to go to the hospital when in sickle cell crisis unless it is life-threatening”. Another patient, Araba Mensah, whose daughter has sickle cell disorder, said there was a lack of “hands-on” nursing, and said patients who have difficulties feeding themselves or with personal hygiene were “left to suffer unattended”. John James, CEO of the Sickle Cell Society, said: “While there are undoubtedly workforce challenges across all parts of the health system, the evidence in this report suggests that sickle cell is disproportionately impacted as a result of the legacy of neglect of sickle cell care. “On behalf of everyone affected by sickle cell, we are urging NHS England to take action now to ensure all sickle cell patients have access to the specialist care they are entitled to.” Read full story Source: The Independent, 24 November 2023- Posted
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untilIn conjunction with the Patients Association with underserved communities living with cancer and blood disorders, this webinar will hear from patient Ram Paul, John James OBE Chief Executive at the Sickle Cell Society, and Alfie Bailey-Bearfield, Head of Health Improvement & Influencing at Pancreatic Cancer UK. The project, which is funded by BMS, explores how to reduce health disparities and improve access to quality care, and considers the impact of social determinants of health, such as income, education, and housing, on health outcomes. Head of Patient Partnership at the Patients Association, Sarah Tilsed will chair the session and guide the panel's debate, which will focus on the project's goal for reducing health disparities and improving access to care. Register- Posted
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Sickle cell patients have begun receiving the first new treatment for the blood disorder in over 20 years. The inherited condition can cause severe pain and organ failure, often requiring hospital admissions. Crizanlizumab is given as a monthly infusion and is thought to cut visits to A&E by 40%. Loury Mooruth, 62, received the treatment at Birmingham City Hospital, having suffered repeated periods of intense pain for decades. During a crisis, patients often need powerful opioid painkillers but Loury, like many others, has faced suspicion when at A&E. "You know the protocol when you go in, which needles and so on. They think straight away you are a drug addict - they don't believe you," she says. She has refused to go to hospital during a crisis for the past two years because of her negative experiences. A report from MPs last year found "serious failings" in sickle cell care with some evidence of discrimination against patients. Dr Shivan Pancham, a consultant haematologist at Birmingham City Hospital, told the BBC: "Our patients often find the experience in emergency departments challenging with a lack of understanding of the severity of pain. "It is hoped with these new therapies if we reduce the likelihood of attending emergency departments, ultimately this will be much better for the patients." Read full story Source: BBC News, 24 February 2022- Posted
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Landmark sickle cell disease inquiry finds evidence of racism in patient care
Patient Safety Learning posted a news article in News
A groundbreaking inquiry into sickle cell disease has found “serious care failings” in acute services and evidence of attitudes underpinned by racism. The report by the all-party parliamentary group (APPG) on Sickle Cell and Thalassaemia, led by Pat McFadden MP, found evidence of sub-standard care for sickle cell patients admitted to general wards or attending A&E departments. The inquiry also found widespread lack of adherence to national care standards, low awareness of sickle cell among healthcare professionals and clear examples of inadequate training and insufficient investment in sickle cell care. The report notes frequent disclosures of negative attitudes towards sickle cell patients, who are more likely to be people with an African or Caribbean background, and evidence to suggest that such attitudes are often underpinned by racism. The inquiry also found that these concerns have led to a fear and avoidance of hospitals for many people living with sickle cell. Care failings have led to patient deaths and “near misses” are not uncommon, leading to a cross-party call for urgent changes into care for sickle cell patients. Read full story Source: The Independent, 15 November 2021- Posted
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Changes to be made to blood donations to become more inclusive
Patient Safety Learning posted a news article in News
From the end of 2021, a question on sexual activity of partners in areas where HIV is widespread will be removed from the donor safety check form, in an effort to increase inclusivity among donors. The changes will particularly improve the ease to donate blood for Black African donors. Currently, prospective donors are asked if they have recently had sex with a partner who may ever have been sexually active in an area where HIV is endemic, which includes most of sub-Saharan Africa. If they have, the donor will then be deferred for three months after the last sexual contact with that partner. This can often mean Black African and other potential donors in long-term relationships have been unable to donate blood. Now, the UK Government has outlined plans to remove the question from those asked in the donor safety check, opening the door to a greater number of donations. Increasing blood donor inclusivity for those who are Black African, Black Caribbean, and of Black mixed ethnicity is particularly important because they are more likely to have the rare blood sub-group, such as Ro, that many Black sickle cell patients need. The change, making it easier for people from these groups to donate, will create greater opportunities to meet the ongoing need for rarer blood types and help improve and save lives in the UK. Read full story Source: National Health Executive, 11 October 2021- Posted
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A new multinational survey, on more than 1,300 patients, caregivers and healthcare professionals in 10 countries, shines a needed light on the misunderstood realities, unseen burden and care challenges of sickle cell disease. The Sickle Cell Health Awareness, Perspectives and Experiences (SHAPE) survey, one of the largest global burden of disease surveys conducted in sickle cell disease, identified long-term health complications of sickle cell disease as a key concern among 1,300 patients and healthcare professionals surveyed from 10 countries The survey also revealed that sickle cell disease patients' caregivers face profound physical, psychosocial, and economic burdens resulting from taking care of people living with the disease. The findings of the survey were presented during a poster presentation at the European Hematology Association (EHA) 2022 Hybrid Congress. “Sickle cell disease is a lifelong condition that causes damage in the body and has a profound impact on the quality of life of those who suffer from it and their caregivers. The SHAPE survey is important because it illustrates how vital it is that we understand our patients’ needs, and it suggests what we within the medical community can do to help change perspectives, increase education and awareness, and improve care,” said Dr. Baba Inusa, professor and consultant of paediatric haematology, Guy’s and St Thomas’ NHS Foundation Trust, London and chair of the National Haemoglobinopathy Panel in England. “These results are a wake-up call, and I believe that the actions that follow can enable us to help drive a better dialogue and improved conversations around the management and care of this long-neglected and devastating disease.”- Posted
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This report was triggered by the Coroner’s report into the death of Evan Nathan Smith in North Middlesex hospital. Evan was a young man with his whole life in front of him. The mistakes made in his treatment leading to his early and avoidable death brought into sharp focus the lack of understanding of sickle cell, the battles patients have to go through to get proper treatment and the terrible consequences which can come about as a result. Following the publication of the Coroner’s report, the All-Party Parliamentary Group (APPG) on Sickle Cell and Thalassaemia held three evidence sessions, hearing from patients, clinicians and politicians. This report is a result of that evidence. The findings in this report reveal a pattern of many years of sub-standard care, stigmatisation and lack of prioritisation which have resulted in sickle cell patients losing trust in the healthcare system that is there to help them, feeling scared to access hospitals, expecting poor treatment from some of those who are supposed to care for them and fearing that it is only a matter of time until they encounter serious care failings. Key findings from the inquiry include: evidence of sub-standard care for sickle cell patients admitted to general wards or attending A&E departments (including a widespread lack of adherence to national care standards) low awareness of sickle cell among healthcare professionals and clear examples of inadequate training and insufficient investment in sickle cell care frequent reports of negative attitudes towards sickle cell patients and a weight of the evidence suggests that such attitudes are often underpinned by racism. The inquiry also found that these concerns have led to a fear and avoidance of hospitals for many people living with sickle cell. While the inquiry did find that specialist haemoglobinopathy services are generally felt to be of a good standard, the report shows that this is far from the case on general wards or A&E departments. Care failings have led to patient deaths and ‘near misses’ are not uncommon. The inquiry heard that awareness of sickle cell among healthcare professionals is low, with sickle cell patients regularly having to educate healthcare professionals about the basics of their condition at times of significant pain and distress and that there is routine failure to comply with national care standards around pain relief when patients attend A&E.- Posted
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Sisters with sickle cell had devastating, and preventable, strokes
Patient Safety Learning posted a news article in News
It was 4am on a Sunday in San Antonio, US, when Dana Jones heard an ominous sound, barely audible over the whirring of box fans, like someone struggling to breathe. She ran down the hall and found her daughter Kyra, age 12, lying on her back, gasping for air. Terrified, she called 911. A police officer, the first to arrive, dashed into Kyra’s bedroom, threw the slender girl over his shoulder and laid her on a leather sofa in the living room. He asked her mother, an oral surgery technician, to give her CPR. Kyra’s lips were ice-cold. An ambulance whisked the girl to Methodist Children’s Hospital, where staff members swarmed her and put her into a medically induced coma. Kyra, who has sickle cell, had suffered a devastating stroke — her second — a common complication of this inherited disease, which afflicts 100,000 Americans, most of them Black. She most likely would never have had the strokes if she had been given an annual screening test and treatment proven more than two decades earlier to prevent 9 out of 10 strokes in children with the disease and recommended by the National Institutes of Health. But like countless other children with sickle cell, she was never screened. Read full story Source: New York Times, 23 May 2021- Posted
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Sickle cell patients are being ignored because they’re black, warn campaigners
Patient Safety Learning posted a news article in News
Public services are dismissing sickle cell patients because the illness disproportionately affects Black people, campaigners have warned. The blood disorder is prevalent among African and Caribbean communities and advocacy groups say this means it remains poorly understood within state institutions, often leading to the needless suffering and even death of those diagnosed. The issue has gained wider attention following the high-profile cases of two Black men, Richard Okorogheye and Evan Nathan Smith, who lived with the disease and died amid claims their vulnerabilities were overlooked by the NHS and police. Chris Abdullahi, co-founder of charitable initiative Sound of Sickle, told The Independent it is common for sickle cell patients’ painful symptoms to be ignored by healthcare practitioners. He said he has heard similar accounts “well over 100 times” from across the UK. “Just last week someone else mentioned that they were in hospital and had to battle their nurses for pain medication from opioids to something as simple as ibuprofen,” the 27-year-old, who also lives with sickle cell, said. These experiences serve to further entrench the “massive sense of distrust” in the healthcare system which is evidenced through lower vaccine uptake in Black communities, Mr Abdullahi explained. A lack of awareness about the disease has led sickle cell patients to often form informal support networks, through which information can be exchanged about the best hospitals at which to maximise the chances of their condition being taken seriously. Read full story Source: The Independent, 18 April 2021- Posted
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A young NHS patient suffering a sickle cell crisis called 999 from his hospital bed to request oxygen, an inquest into his death was told. Evan Nathan Smith, 21, died on 25 April 2019 at North Middlesex Hospital, in Edmonton, north London, after suffering from sepsis following a procedure to remove a gallbladder stent. The inquest heard Smith told his family he called the London Ambulance Service because he thought it was the only way to get the help he needed. Nursing staff told Smith he did not need oxygen when he requested it in the early hours of 23 April, despite a doctor telling the inquest he had “impressed” on the nurses he should have it. Smith’s sepsis is thought to have triggered the sickle cell crisis – a condition that causes acute pain as blood vessels to certain parts of the body become blocked. Barnet Coroner's Court heard Smith, from Walthamstow in east London, might have survived if he had been offered a blood transfusion sooner but the hospital’s haematology team were not told he had been admitted. Read full story Source: The Independent, 3 April 2021- Posted
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Medicine is a mirror for the racial injustice in our society; it is a field riddled with racial disparities in everything from research funding to patient care to life expectancy. There may be no population of patients whose healthcare and outcomes are more affected by racism than those with sickle cell disease (SCD). Patients with SCD are too often marginalised and dismissed while seeking medical care when their bodies hurt and they cannot breathe. As medical leaders around the United States issue statements denouncing racial injustice and calling for us to “dismantle racism at every level,” we must ensure that these pledges translate into durable improvements for patients with SCD. Alexandra Power-Hays and Patrick T. McGann propose a number of changes to reduce the impact of racism on patients with SCD in the US. -
Content Article
Following on from the care failures highlighted in the 2021 report, 'No one's listening', the Sickle Cell Society have published a new report taking a deeper look at sickle cell nursing care. The findings show the need for vastly more resources, training and support in this critical area of care. The report highlights that not only is no-one listening, but that lives are still being put at risk.- Posted
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Sickle cell disease is the name for a group of inherited red blood cell disorders that affect haemoglobin, which is a protein in red blood cells that carries oxygen through a person’s body. It mainly affects people from African or Caribbean backgrounds, though it can affect anyone. It affects approximately 15,000 people in the UK. In November 2021, the All-Party Parliamentary Group for Sickle Cell and Thalassaemia published a report detailing the issues that people with sickle cell disease experience in relation to their care. The report made 31 recommendations to organisations across the healthcare system to help address these issues. HSIB launched two investigations (see also: Management of sickle cell crisis) to find out what additional learning or knowledge could be added in this area and to provide further insights into the practical challenges that patients with sickle cell disease may face when receiving NHS care. This investigation set out to review the care of patients with sickle cell disease who need to have an invasive procedure. Invasive procedures involve accessing the inside of a patient’s body, either through an incision (cut) or one of the body’s orifices. Specifically, the investigation focused on: how haematology teams – the specialists who treat people with blood disorders – are involved and informed when a patient with sickle cell disease is treated in another area of healthcare how patients with sickle cell disease are prepared for invasive procedures how and where clinical information relevant to the patient is shared. The reference event was a patient, 45 years old at the time of the incident, who had a history of sickle cell disease that had led to her being admitted to hospital multiple times. For approximately 3 years she had been receiving regular exchange blood transfusions (transfusions that replaced her blood with donated blood) to help treat the symptoms of her sickle cell disease. Due to heavy, painful and frequent menstrual periods she was scheduled for a surgical procedure to help ease her symptoms. The haematology team were made aware of the procedure but were not told when it was occurring, the procedure was conducted 1 week before the patient was due to have her regular exchange transfusion. Her haemoglobin levels were low. The patient experienced a significant amount of pain during the procedure and was transferred to the haematology ward afterwards for treatment of a sickle cell crisis. Findings The treatment people with sickle cell disease receive varies depending on where they live and the number of patients that clinicians see. Guidance on the treatment of sickle cell disease is limited to specific circumstances, such as sickle cell crisis or blood transfusion, with limited wider guidance available on the through-life management of sickle cell disease. Compared to other conditions that affect similar numbers of people, there has been limited research into the lifelong management of sickle cell disease. This may indicate a health inequality. There is no national information system for storing and sharing clinical information for patients with sickle cell disease. Currently the main way in which haematology consultants are made aware that a patient with sickle cell disease is having treatment in another area of healthcare is when they are notified of this by the patient. It may be possible to set up healthcare IT systems so that they alert haematology consultants when one of their patients accesses another type of healthcare. However, consultants in areas with a high prevalence of sickle cell disease may then receive so many alerts that such a system would be ineffective. Recommendations HSIB recommends that NHS England identifies and implements a system for sharing clinical information about patients with sickle cell disease to improve access to this information for clinicians, and reduce the risk of a patient’s sickle cell disease not being treated consistently. HSIB recommends that the National Institute for Health and Care Research assesses the priority and feasibility of commissioning research into the management of patients with sickle cell disease, encompassing the requirements of patient-controlled-analgesia (pain relief) pumps. This will contribute towards building an evidence base for the generation of national clinical guidance and will reduce unnecessary variance in treatment for patients with sickle cell disease. Observations HSIB makes the following safety observations: It may be beneficial if trusts included a sickle cell disease notification within their electronic patient record systems, to ensure that clinicians treating patients with the disease consider it and seek advice from haematology teams about the impact of any planned treatments, such as invasive procedures. It may be beneficial if the healthcare sector explores, researches and fully understands the care provision to all patients with sickle cell disease, at all points in their life, encompassing day-to-day treatment through to sickle cell crisis.- Posted
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Sickle cell disease is the name for a group of inherited red blood cell disorders that affect haemoglobin, which is a protein in red blood cells that carries oxygen through a person’s body. It mainly affects people from African or Caribbean backgrounds, though it can affect anyone. It affects approximately 15,000 people in the UK. In November 2021, the All-Party Parliamentary Group for Sickle Cell and Thalassaemia published a report detailing the issues that people with sickle cell disease experience in relation to their care. The report made 31 recommendations to organisations across the healthcare system to help address these issues. The Healthcare Safety Investigation Branch (HSIB) launched two investigations (see also: Invasive procedures for people with sickle cell disease) to find out what additional learning or knowledge could be added in this area and to provide further insights into the practical challenges patients with sickle cell disease may face when receiving NHS care. HSIB used a real patient safety incident, referred to as ‘the reference event’, to explore how sickle cell crises are managed within hospital settings. In particular, the investigation considered: the knowledge nursing staff may have about the care of patients in sickle cell crisis how patient-controlled analgesia (PCA) – where a patient can use a device to give themself doses of pain relief medication – is considered holistically, such as monitoring the patient and staff workload. The reference event was Tyrone, 46 years old, who experienced a sickle cell crisis in spring 2021 during the COVID-19 pandemic. He called an ambulance because of the amount of pain he was experiencing. Tyrone was put onto a PCA pump, which was programmed to deliver morphine at a continuous dose (background rate) per hour, and also allowed him to press a button to deliver a dose at a regulated maximum rate. He was in pain and was restless throughout the night, but he was found unresponsive the following morning. A post-mortem report stated that Tyrone died from cardiorespiratory failure (his heart and breathing stopped) caused by acute sickle cell crisis and morphine toxicity. The record of inquest stated that ‘the nursing staff had insufficient training to manage and monitor [Tyrone] and his PCA pump’. Findings There is no minimum training requirement or nationally agreed content for Haemoglobinopathy Coordinating Centres to deliver to provide staff with knowledge about sickle cell disease or sickle cell crisis. Haemoglobinopathy Coordinating Centres are required to deliver training in line with their service specifications; however, they report finding it challenging to engage NHS trusts and NHS staff in this process in areas where the prevalence of sickle cell disease is lower. Trusts faced competing priorities during the COVID-19 pandemic and many trusts may have needed to alter the normal pathway of care for patients with sickle cell disease. Where reconfiguration of services requires patients in sickle cell crisis to be cared for in alternative wards/departments within the hospital or by new staff, there may not be the necessary equipment, knowledge or staff training required for care to be delivered safely. Staff workload, particularly during the COVID-19 pandemic and in emergency departments, impacted on the ability of staff to conduct increased monitoring requirements, such as the monitoring of patients on high-strength opiates delivered using a PCA pump. Recommendation HSIB recommends that NHS England reviews the existing training and competence requirements within sickle cell care provision and specifies the minimum training requirements and content for staff. The content can then be delivered by Haemoglobinopathy Coordinating Centres to increase knowledge about sickle cell disease and how to treat patients in sickle cell crisis.- Posted
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- Sickle cell
- Investigation
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