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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.

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

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?  

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. 

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.”

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.

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.

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.

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.

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.