Sickle Cell Disease

Care for People of All Ages

Resources The Quality Standard In Brief Advisory Committee About

Summary

This quality standard addresses care for children, young people, and adults with sickle cell disease. Where appropriate, it also addresses the needs of families and caregivers or other substitute decision-makers. It addresses screening for and the prevention of complications, the assessment and management of acute and chronic complications, and the use of disease-modifying therapies. It applies to all pediatric and adult health care settings (including hospitals, emergency departments, urgent care clinics, and primary care, specialist care, and home and community care settings).

Scope of This Quality Standard

This quality standard addresses care for children, young people, and adults with sickle cell disease. Where appropriate, it also addresses the needs of families and caregivers or other substitute decision-makers (for definitions of these roles, see Appendix 3, Glossary). It addresses screening for and the prevention of complications, the assessment and management of acute and chronic complications, and the use of disease-modifying therapies. It applies to all pediatric and adult health care settings (including hospitals, emergency departments, urgent care clinics, and primary care, specialist care, and home and community care settings).

Although many of the statements may apply to pregnant people, this quality standard does not directly address the management of sickle cell disease in pregnancy.

The Clinical Handbook for Sickle Cell Disease Vaso-occlusive Crisis by the Provincial Council for Maternal and Child Health and the Ontario Ministry of Health addresses care for sickle cell crisis (referred to as a “vaso-occlusive acute pain episode” in this quality standard) and applies to quality statement 3.

Why This Quality Standard Is Needed

Sickle cell disease is one of the most common inherited red blood cell disorders. The disease can lead to serious health conditions including infection, stroke, organ damage, and acute and chronic pain. It is most prevalent in regions of Africa, the Mediterranean, the Middle East, and India, although the sickle cell trait is found in all racial/ethnic groups. As a result of global migration, there has been an increase in the prevalence of sickle cell disease among people of African, Arab, and Indian racial/ethnic backgrounds in Canada.

About 3,500 people in Ontario and 6,500 people across Canada have sickle cell disease. These numbers are expected to increase with immigration from countries with high disease prevalence, with new births in Canada from parents who carry the sickle cell disease trait, and given improved care and treatment options, resulting in increased life expectancy. In Ontario and across Canada, sickle cell disease mostly affects racialized people, particularly those who identify as Black. These people often experience racism and anti-Black racism in their interactions with the health care system, which negatively impacts the quality of the health care provided to them. Owing to the systemic nature of anti-Black racism, Black people with sickle cell disease experience the intersections of ableism and racism in many areas, including education, housing, child welfare, employment, immigration, and justice systems, all of which negatively impact health and well-being. Further, sickle cell disease has historically been thought of as a “Black disease,” and the long societal and cultural association of sickle cell disease with people who identify as Black has led to structural marginalization, deprioritization, and neglect of care for impacted people in both high- and low-income societies around the world.

Living under stressful social and economic conditions can impact the overall health and well-being of people with sickle cell disease. Such stresses include social stigma, discrimination, and inadequate access to education, employment, income, and housing. Administrative data from Ontario show that while people with sickle cell disease live in all health regions, most live in areas around Toronto and Ottawa and that about 40% of patients live in the neighbourhoods with the lowest income. The communities in which most people with sickle cell disease live are also communities that are under-resourced with regard to access to social and health services and public transportation. More than half of caregivers surveyed reported that patient symptoms impacted various aspects of caregivers’ lives, such as their ability to attend work or school (56%), earning potential (54%), mental health (52%), and overall well-being (53%).

People living in rural, remote, and Northern communities may face additional challenges to accessing comprehensive care compared with their urban peers. These geographic barriers, which include the need to travel to large urban centres and hospitals and the time and financial resources required to do so, can further worsen continuity of care.

Acute pain episodes are the hallmark acute complication of sickle cell disease. Guidelines recommend rapid access to appropriate pain management, which for severe complications is often opioid analgesia. However, people with sickle cell disease often experience systemic racism and discrimination when they seek care in the emergency department for acute pain relief. Many report avoiding or delaying accessing care in the emergency department because of previous experiences of being stigmatized and falsely accused of “drug-seeking.” When people with sickle cell disease do seek care, they often face long waits, treatment delays, and a lack of respect and empathy, and they are often denied effective medication for adequate pain relief. In addition, when compared with the general population seeking emergency department care, people with sickle cell disease are more likely to be admitted to hospital, often have a longer hospital stay, and experience higher rates of repeat emergency department visits within 30 days of being discharged from the emergency department.

Opportunities for improvement in Ontario exist to ensure that high-quality, timely care is delivered in the emergency department and other hospital settings for people experiencing acute complications of sickle cell disease. A survey of people with sickle cell disease found the following:

54% felt that the quality of care received was below their expectations
For those presenting to the emergency department with pain, many waited more than 1 hour to see a nurse (31%) or physician (56%) and for the administration of pain medication (50%)
Only 60% reported that the first person they met at triage was respectful, empathetic, and caring
About 22% reported feeling stigmatized or afraid of dying during their hospital visit, and 44% reported feeling lonely or helpless
71% reported that their health care provider was not responsive or only somewhat responsive to their concerns
Although 60% of respondents reported that their health care providers were very or extremely knowledgeable, 40% reported that their providers were not at all or only somewhat knowledgeable

People with sickle cell disease have also identified a lack of awareness and education about sickle cell disease among some health care providers who work outside specialist centres. In Ontario, gaps in provider knowledge of sickle cell disease and negative attitudes toward people with sickle cell disease are key barriers to accessing and receiving high-quality care. Treatment advances have also led to people with sickle cell disease living longer; as a result, there is a crucial need to improve knowledge and capacity within the adult health care system to provide appropriate continuity of care for young people transitioning to adult care.

This quality standard includes eight quality statements that address areas identified by the Sickle Cell Disease Quality Standard Advisory Committee as having high potential to improve care for people with sickle cell disease in Ontario.

A quality standard on sickle cell disease strengthens Ontario Health’s commitment to Black health and, through collaborations with the Black Health Plan Working Group, furthers the goals of Ontario Health’s Equity, Inclusion, Diversity and Anti-Racism Framework.

Values and Guiding Principles

Values That Are the Foundation of This Quality Standard

This quality standard was created, and should be implemented, according to the Patient, Family and Caregiver Declaration of Values for Ontario. This declaration “is a vision that articulates a path toward patient partnership across the health care system in Ontario. It describes a set of foundational principles that are considered from the perspective of Ontario patients and serves as a guidance document for those involved in our health care system.”

These values are:

Respect and dignity
Empathy and compassion
Accountability
Transparency
Equity and engagement

A quality health system is one that provides good access, experience, and outcomes for all people in Ontario, no matter where they live, what condition they have, or who they are.

Guiding Principles
In addition to the above values, this quality standard is guided by the principles outlined below.

Acknowledging the Impact of Colonization and Racism
Health care providers should acknowledge and work toward addressing the historical and present-day impacts of colonization and racism in the context of the lives of Black people, Indigenous people, and racialized people throughout Canada. This work involves being sensitive to the impacts of intergenerational and present-day traumas and the physical, mental, emotional, and social harms experienced by Black people, Indigenous people, racialized people, families, and communities, as well as recognizing their strength and resilience. This quality standard uses existing clinical practice guideline sources that may not include culturally relevant care or acknowledge the traditional beliefs, practices, and models of care relevant to Black people, Indigenous people, and racialized people.

French Language Services
In Ontario, the French Language Services Act guarantees an individual's right to receive services in French from Government of Ontario ministries and agencies in 26 designated areas and at government head offices.

Integrated Care
People with sickle cell disease should receive care through an integrated approach that facilitates access to interprofessional services from multiple health care providers from different professional backgrounds and across health care settings to provide comprehensive services. Health care providers should work with patients, their families and caregivers, and communities to deliver the highest quality of care across settings. Interprofessional collaboration, shared decision-making, coordination of care, and continuity of care (including follow-up care) are hallmarks of this patient-centred approach.

Intersectionality
Intersectionality refers to the differences in experiences with discrimination and injustice that people have based on social categorizations such as race/ethnicity, class, age, and gender, and the interaction of these experiences with compounding power structures (e.g., media, education system). These interconnected categorizations create overlapping and interdependent systems of discrimination or disadvantage. For example, the stigma experienced by people with sickle cell disease can vary depending on clinical and demographic characteristics such as racial/ethnic background and age, as well as other characteristics such as language barriers or perceived socioeconomic status. Understanding how the various aspects of people’s identities intersect can provide insight into the complexities of the processes that cause health inequities and an understanding of how different people experience stigma and discrimination.

Racism and Anti-Black Racism
Racism and anti-Black racism refer to the systemic discrimination that harms racialized populations and groups living with health-related social needs and creates barriers to and disparities in accessing and receiving appropriate health care and community and social services. This type of racism often involves labelling, devaluation, judgment, and/or the social disqualification of a person based on their health condition, leading to negative health outcomes. Anti-Black racism is associated with policies and practices rooted in Canadian institutions such as the education, health care, social, and justice systems that mirror and reinforce beliefs, attitudes, prejudices, stereotyping, and discrimination toward people of African descent. Racism and anti-Black racism can have negative effects on a person’s health such as triggering stress-coping behaviours (e.g., smoking). The traumatic impact of racism and anti-Black racism can also lead to the development of additional health issues (e.g., depression, anxiety). Frequent experiences of discrimination have also been associated with obesity and lower self-rated health. Racism, particularly anti-Black racism, is the most significant source of stigma experienced by people with sickle cell disease.

Social Determinants of Health
Poverty and social isolation are two examples of economic and social conditions that influence people’s health, known as the social determinants of health. Other social determinants of health include employment status and working conditions, ethnicity, food security and nutrition, gender, housing, immigration status, social exclusion, and residing in a rural or urban area. Social determinants of health can have strong effects on individual and population health; they play an important role in understanding the root causes of poorer health. People with sickle cell disease often live under stressful social and economic conditions that may worsen their overall health and well-being, including social stigma, discrimination, and inadequate access to education, employment, income, and housing.

Strengths-Based Practice
A strengths-based practice actively involves the person and the care providers who support them in working together to achieve the person’s intended outcomes in a way that draws on the person’s strengths. The person is recognized and acknowledged as the expert of their own lived experience, while the clinician is recognized as an expert in their discipline and in facilitating a conversation that reinforces the person’s strengths and resources.

Trauma-Informed Care
Trauma-informed care is health care that reflects an understanding of trauma and the impact that traumatic experiences can have on human beings. This approach does not necessarily address the trauma directly; rather, the approach acknowledges that a person may have experienced a previous traumatic event that may contribute to their current health concerns and emphasizes understanding, respecting, and responding to the effects of trauma.

How to Use This Quality Standard

Quality standards inform patients, clinicians, and organizations about what high-quality care looks like for health conditions or processes deemed a priority for quality improvement in Ontario. They are based on the best evidence.

Guidance on how to use quality standards and their associated resources is included below.

For People With Sickle Cell Disease
This quality standard consists of quality statements. These describe what high-quality care looks like for people with sickle cell disease.

Within each quality statement, we’ve included information on what these statements mean for you, as a patient.

In addition, you may want to download this accompanying patient guide on sickle cell disease, to help you and your family and caregivers have informed conversations with your health care providers. Inside, you will find information and questions you may want to ask as you work together to make a plan for your care.

For Clinicians and Organizations
The quality statements within this quality standard describe what high-quality care looks like for people with sickle cell disease. They are based on the best evidence and designed to help you know what to do to reduce gaps and variations in care.

Many clinicians and organizations are already providing high-quality evidence-based care. However, there may be elements of your care that can be improved. This quality standard can serve as a resource to help you prioritize and measure improvement efforts.

Tools and resources to support you in your quality improvement efforts accompany each quality standard. These resources include indicators and their definitions (Appendix 2). Measurement is key to quality improvement. Collecting and using data when implementing a quality standard can help you assess the quality of care you are delivering and identify gaps in care and areas for improvement.

There are also a number of resources online to help you, including:

Our patient guide on sickle cell disease, which you can share with families and caregivers to help them have conversations with you and their other health care providers. Please make the patient guide available where you provide care
Our measurement resources, which include our measurement guide of technical specifications for the indicators in this quality standard, and our “case for improvement” slide deck to help you to share why this standard was created and the data behind it
Our placemat, which summarizes the quality standard and includes links to helpful resources and tools
Our Getting Started Guide, which includes links to templates and tools to help you put quality standards into practice. This guide shows you how to plan for, implement, and sustain changes in your practice
Quorum, an online community dedicated to improving the quality of care across Ontario. This is a place where health care providers can share information and support each other, and it includes tools and resources to help you implement the quality statements within each standard
The Health Equity Impact Assessment tool, which can help your organization consider how programs and policies impact population groups differently. This tool can help maximize positive impacts and reduce negative impacts, with an aim of reducing health inequities between population groups

What Does It Mean for Patients and Caregivers?

“A sickle cell disease quality standard would immediately improve the timing and quality of care for patients. For example, one day I showed up in the ED in a crisis, but instead of being fast-tracked to receive emergency care, I had to wait another hour before I was in a room and then had to wait longer to receive medication. I was then given a high dose of opioids while in a hallway unsupervised. In this case, my advocates had to ask the nurse for the hospital’s sickle cell disease protocol, which they had never heard about. Once they located it, I was immediately moved to observation and treated. I then was admitted because of the time it took to administer analgesia.”

“Having a quality standard for sickle cell disease will change patient care by acknowledging how anti-Black racism plays a part in delaying optimal care. Education, engagement, and implementing this quality standard will ensure positive outcomes for patients and their providers. Just knowing that there is now a quality standard for sickle cell disease is a sign of hope.”

- Serena Thompson, co-chair and lived experience advisor, Sickle Cell Disease Quality Standard Advisory Committee

What Does It Mean for Providers?

“As a practising pediatric hematologist in a large sickle cell centre in Toronto that cares for children and adolescents with the disease across the GTA, I’ve often struggled with advising my patients and families to seek acute care at their local hospital. My struggle stems from the wide variability in the quality of care patients receive across Toronto. Families are forced to travel longer distances to seek care in my centre, posing extra burdens on patients and their families.”

“I am looking forward to a future where I can confidently assure patients and families, regardless of where they live, of the convenience and advantages of seeking acute care near where they live because the quality of care is no different than it is in my centre in downtown Toronto. The development of this quality standard for sickle cell disease management in Ontario is crucial in helping achieve this goal.”

– Dr. Isaac Odame, co-chair, Sickle Cell Disease Quality Standard Advisory Committee

Timeline for This Quality Standard

This quality standard was completed in January 2023.

Evidence to Improve Care