|
The appalling death of 21-year-old Evan Nathan Smith in 2019 brought into stark relief the impact of racism on the care of individuals with sickle cell disease (SCD) in the UK. Smith was forced to call emergency services from his hospital bed when healthcare workers failed to recognize the risk of an impending sickle cell crisis and administer the appropriate treatment. Despite the fact that SCD is the most common genetic blood disorder globally, and well over a century has passed since its first description in Western medical literature, the subsequent inquest identified a “lack of understanding of sickle cell disease in the medical and nursing staff” as one of the main reasons for his death (https://www.bbc.com/news/uk-england-london-56647361). Clearly, our improved understanding of the causes and consequences of SCD have not translated into better medical care for affected individuals.
The trait for SCD is thought to have arisen as the result of a single mutation conferring increased malarial protection upon a child in Saharan Africa 7300 years ago. Today, SCD is particularly prevalent in areas of sub-Saharan Africa, eastern Saudi Arabia, and central India. In the UK, an estimated 15,000 individuals have SCD, predominantly those with African or Caribbean ancestry (https://www.sicklecellsociety.org/about-sickle-cell/). These are individuals who have inherited two mutated beta-globin genes, one from each parent, resulting in changes to the structure of the hemoglobin protein found in red blood cells. While several genetic variants of SCD are known, the most common and severe variant, also known as sickle cell anemia, is caused by homozygosity for the sickle mutation. Individuals with sickle cell anemia are at risk of a number of complications, including extremely painful vaso-occlusive crises (VOCs) that can cause cumulative organ, bone, and tissue damage; infections; cerebral vasculopathy; avascular necrosis; blindness; and others, all of which are variable, unpredictable, and associated with significant morbidity and poor quality of life. Treatment options are also limited; in the last two decades, the only treatment that has received National Health Service (NHS) approval for sickle cell is crizanlizumab, which can reduce the incidence of VOCs and was made available to the UK patient population in September 2021 under a managed access agreement, providing time-limited access to therapy while further patient data is collected (https://www.england.nhs.uk/2021/10/nhs-announces-deal-for-life-changing-sickle-cell-treatment/). In total, the UK has only five approved treatments for SCD, in contrast to other genetic conditions like cystic fibrosis, where 10,500 patients have 511 approved treatments. The dual impact of severe complications and limited treatment options on the patients’ health and quality of life has unfortunately been significantly exacerbated by deeply entrenched racist beliefs that are weaponized to deny appropriate care. Black SCD patients seeking emergency care experience significantly longer wait times on average, and those seeking pain relief during VOCs are often accused of being drug addicts, with physicians and nurses significantly underestimating the degree of pain experienced by Black patients. The global opioid crisis has also had a devastating impact on SCD patients, with opioid painkillers routinely being denied for use in treating VOCs even though they are highly effective in managing VOC-associated pain and the rate of opioid addiction in this population is comparable to that seen in most chronic pain sufferers (https://www.nhlbi.nih.gov/news/2017/opioid-crisis-adds-pain-sickle-cell-patients). It is not surprising that results from the International Sickle Cell World Assessment Survey (SWAY) found that 39% of patients experiencing a VOC sought to avoid medical care due to reported poor experiences in the emergency room or hospital, with another 26% believing that healthcare professionals do not understand SCD. Spurred by several instances of substandard care delivered to SCD patients in the UK, an All-Party Parliamentary Group on Sickle Cell and Thalassemia was tasked with conducting an inquiry in collaboration with the Sickle Cell Society, of which I am Chair. The APGG’s findings were detailed in a final report entitled “No One’s Listening”; key messages included clear evidence of sub-standard care, low awareness of the disease and inadequate training among healthcare professionals, and widespread negative attitudes toward SCD patients underpinned by racism (https://www.sicklecellsociety.org/no-ones-listening/). The report also outlines a number of recommendations that can lead to sustainable improvements in patient care and outcomes. These include investigating the possibility of setting up dedicated sickle cell wards at specialist centers, ensuring compliance with existing clinical guidelines around the delivery of pain relief for sickle cell patients within 30 min, developing comprehensive training programs on sickle cell, implementing diversity and racial bias awareness training as part of healthcare curricula, establishing patient advisory groups to provide oversight of NHS sickle cell services, and providing increasing funding for sickle cell services to correct a pattern of consistent underfunding. The APPG inquiry report has generated a Westminster Hall Parliamentary debate and prompted calls for the Health Secretary and NHS England to prioritize taking urgent action, deliver the report recommendations, and improve the healthcare system for people living with sickle cell to prevent any further avoidable deaths. If we are serious about “leveling up,” we must acknowledge the misdeeds of the past and change policies and practices to create a fairer health service and ensure no one gets left behind.AcknowledgmentsK.G. is the Chair of the Sickle Cell Society, UK, and would like to acknowledge the Sickle Cell Society and the Sickle Cell and Thalassemia All Party Parliamentary Group.
0 Comments
 A report has exposed how the NHS is failing those with the condition
This week’s “No One’s Listening” report, by the all-party parliamentary group on sickle cell and thalassaemia, comes hot on the heels of recent high-profile inquests into avoidable deaths from the condition, which found two patients had died because of poor basic care and/or neglect while in hospital. MPs are now calling, in response, for urgent changes to be made to the healthcare provided for sickle cell. Sickle cell disorder (SCD) is classified as a “medical emergency”, putting it in the same category as heart attacks and strokes. SCD is the world’s most common genetic blood condition, characterised by deoxygenated cells becoming rigid, sickle-shaped – like crescent moons – and sticky. These cells block capillaries, causing excruciating pain likened to being repeatedly hit with a hammer. It can occur in any part of the body, often in multiple places at once, leaving the sufferer unable to move, other than to writhe in agony. The pain levels are so high, I have often heard women with sickle cell describe these episodes as worse than childbirth. Without appropriate care the blockages can escalate, leading to death. The inquiry, chaired by Pat McFadden MP, included the late David Amess as one of the parliamentary group’s elected members. The findings make for very uncomfortable and frustrating reading, and reinforce the coroner’s findings. However, the broad range of people who provided evidence from across the country were unified in their assertion that issues of race and inequality underpinned the failures at every level of service, policy and performance delivery. I felt very proud to participate in the report – alongside fellow “sick cell warriors”, bereaved parents and medical figures – and to give evidence to the inquiry panel. The chance to have a voice, and hopefully help other patients, families and the NHS has been a long time coming. I offered several recommendations, most notably “listen to the patient”. Patients are often experts in the condition and their needs. Many have additional safeguards such as care plans and explanatory letters from health and social care professionals that are ignored on arrival at A&E in many cases. Additionally, healthcare attitudes stigmatise the patient, while the NHS-commissioned standards of care that are meant to be provided to patients are often not delivered. This chain of events frequently only leads to one place: preventable harm. Though some white people have SCD, it predominantly affects people of African and Caribbean heritage. What truly shocked the inquiry is patients – ill and in desperate need – reporting open racism: being called the N-word, or worse. Patients were also accused of being drug addicts, without foundation, and were commonly denied treatment and pain relief. The report calls these issues out in the hope that urgent actions will be taken. Today, there are more than 15,000 people living with SCD in the UK, and only five approved treatments. A comparator genetic condition is cystic fibrosis, which has 10,500 patients and 511 approved treatments. This is not about pulling anyone down, it’s about saying, “OK, what about us?” The inquiry calls for sustainable improvements in training, attitudes, treatments and accountability, among other things, in order to prevent future deaths like those of Evan Nathan Smith and Tyrone Airey, which are far from isolated incidents. In both cases, inquests found their lives could have been saved with better healthcare. Racism within an organisation is a product of its wider culture, practice and character. To that extent should chairs and chief executives take the lead in holding their employees to account? Attitudes must change, much as they must in the world of cricket. Indeed, a parliamentary select committee on SCD deaths might be a sensible next step forward. The NHS may now respond to stem concerns over racism and poor care by pledging to clean up the service and apologise for hurt caused. I believe a healthy society treats people fairly, and so the organisations that serve within it must do the same. Excellence undoubtedly exists within the NHS – I do not deny that – and the MPs’ report doesn’t call for all healthcare staff to become experts. But understanding how sickle cell affects people can change a practitioner’s perspective. Government studies since the 1980 Black report talk of disparity and poor health care delivered to the black community, and the need to level up. The Acheson report in the 1990s said the same. The Marmot report of 2010, and the follow-up review in 2020, noted that the gap between the healthcare services received by white people and those received by black people continued to grow. If we are serious about “levelling up”, we must acknowledge the misdeeds of the past and change policies and practices to create a fairer health service and ensure no one gets left behind. The director general of the World Health Organization, Tedros Adhanom Ghebreyesus, said last month: “The WHO stands in solidarity with all marginalised patients in communities not consulted, not engaged, or empowered in their own care.” The parliamentary group inquiry’s report calls on the health secretary and NHS England to prioritise taking urgent action, deliver the report recommendations, and improve the healthcare system for people living with sickle cell to prevent any further avoidable deaths. Kye Gbangbola is a sickle cell patient and chair of the Sickle Cell Society  Can the UK regard itself as a Life Sciences Superpower without addressing the inequalities present in the healthcare system? Public Policy Project’s (PPP) Grace Girling continued discussions held at the Global Genomics Conference with Kye Gbangbola, reflecting on the problems faced by those living with Sickle Cell Disorder and the crucial reasons why diversity must be engrained in genomics research.
Global Genomics Conference in May 2022 hosted a variety of discussions encompassing the policy and ethical considerations of furthering genomics’ widespread benefits to ensure they reach all pockets of global society. Kye Gbangbola, former Chair of the Sickle Cell Society and central member of the Sickle Cell and Thalassaemia All Party Parliamentary Group, articulated the barriers to greater diversity in genomics in the first session of the in-person conference. Kye works tirelessly to transform the current healthcare system into one which is equitable and accounts for everyone’s needs. His upcoming book “The Sickle Cell Guide” is a comprehensive source of extensive information on sickle cell disorder (SCD), to help organisations and individuals on their journey of understanding and better healthcare. The treatment and care of Sickle Cell Disorder (SCD), an inherited blood condition which affects mainly people of African and Caribbean heritage, is a microcosm of existing health inequalities. Currently, although it is the world’s most common genetic blood condition, there are just five approved treatments in the UK for a patient community of 15,000, compared to the 511 treatments for Cystic Fibrosis which has a patient community of 10,500. Kye immediately addresses that, “this is never about pulling anyone down, it’s about saying, what about us?” Genomics offers the opportunity to ‘level up’; the primary concept of generating a fairer society. Hence, if the genomics ecosystem aligns correctly, this will have a profound positive impact on the Sickle Cell community through the development of effective treatments. Following the conference and engaging in further dialogue with Kye, it is evident that advancing genomics carries the risk of continuing the trajectory of a healthcare system which sustains systemic inequalities. However, recognising and addressing such problems is achievable with the efforts of stakeholders from across the health and genomics ecosystem. The meaning of genomics beyond science The UK’s Life Sciences sector is at the forefront of the genomics revolution. Although it is clear that leading the advancement of genomics will solidify the UK’s position as a ‘Life Sciences Superpower’, understanding what this means for society, particularly marginalised communities, should not be lost through a myopic vision of this title. For those living with SCD, genomics represents the opportunity of a life free from the unmet medical needs and daily burdens, such as ongoing physical and mental suffering, which result as the consequence of living with this inherited genetic condition. In addition to this, SCD patients face increasing multi-dimensional poverty, with over 66 per cent of people living with SCD in the UK living in the most deprived communities. Undeniably, genomics being applied to gene therapies will engender a much-improved quality of life for patients and their families. If the human aspect is not convincing enough, genomics also has a huge economic case. As SCD results in regular hospitalisation of patients requiring resources from a myriad of disciplines, an effective, or even preventative, treatment would diminish the financial cost this necessitates – both on the patient and the wider health service. As such, the case has been made by Jonathan Salcedo, Jennifer Bulovic and Colin Young to show that a hypothetical cell or gene therapy for SCD is likely to be cost effective, with the large upfront costs being offset by significant downstream economic and human health gains. However, diversity in genomics across the datasets and the wider ecosystem is crucial to the roll out of new treatments. Parallels can be drawn with Covid-19 with respect to health inequalities, felt both by certain ethnic groups and marginalised communities in and between nations as a result of underrepresentation in vaccine trials. As reiterated by Kye, “Diversity should be a key trait of being a life science superpower.” To achieve this, systemic health inequalities and poor healthcare for marginalised groups must be addressed. Health inequalities and equity in genomics Currently, 40 per cent of people living with SCD fear going to hospital due to widespread poor care standards for the condition which carries a high risk of death. Following an inquiry into the preventable deaths of those living with SCD by the All Party Parliamentary Group on sickle cell and thalassemia, it was highlighted that race and inequality are at the root of these issues of poor care. Raising awareness of this neglect is crucial in provoking systematic change in the healthcare system. The Sickle Cell Society’s report ‘No one’s Listening’ published recommendations based on this inquiry, which included high-level policy shifts to provide sickle cell patients with the standard of care and respect deserved. As Kye emphasises, “Cell and gene therapies are here, delivering remarkable outcomes, people with sickle cell are being cured. These cures offer the opportunity to prevent and mitigate the harm that health inequalities cause. Genomics can act on communities as a key element of building and editing back better”. If the purpose of the NHS Race and Health Observatory to remove health disparity could be embedded in health policy, we could avoid perpetuating the injustices of the past and move towards equitable healthcare for all in the future. Design for equity and equality: Health is not just medical Not only should equity be a key goal of the advancement of genomics, but the mitigation and elimination of existing health inequalities should be at the heart of the decisions taken by both the public and private sector stakeholders. Institutions should be continuously reflecting on how they can incorporate factors which will generate trust and alleviate public concern over accountability. SCD is the world’s fastest growing, and most common genetic blood condition. People living with sickle cell are highly correlated with a lower socioeconomic status, this impacts health literacy, which limits the ability of this community of people to demand quality healthcare. This is a vicious cycle which can be tackled with initiatives to increase health literacy amongst disadvantaged groups, in turn this will also increase participation in clinical trials which will manifest into treatments with higher efficacy. Presently, participation in clinical trials and research for treatments, including gene therapies, is hindered by the forms of employment marginalised populations are often engaged in which can be restrictive in terms of paid absence for participation. Specifically, individuals earning below 50,000 USD are 27 per cent less likely to participate in clinical trials. These issues must not be side-lined, they must be at the centre of research design to provide adequate representation of marginalised groups. For example, positive action has been taken in the EU and in the USA to provide compensation as a component where work conflicts may act as a barrier to participation. This is particularly important for conditions such as SCD where diversity is imperative to the success of the research, resulting in an effective treatment. Outlook for a fairer future Genomics is the future of healthcare; however the present system is still not functional for all groups in society. The Sickle Cell community is just one example of a group which has faced decades of substandard care and lack of attention. As detailed, treatment for SCD would be beneficial in a multitude of ways. In order to secure the UK’s position as a Life Sciences superpower, it is essential that health inequalities are recognised and not perpetuated by the cell and gene therapies that result from genomics research. As stated by Kye “if we are serious about ‘‘levelling up,’’ we must acknowledge the misdeeds of the past and change policies and practices to create a fairer health service and ensure no one gets left behind.” Article by Grace Girling >> |
RSS Feed