Quality Standard Details
Sickle Cell Disease: Care for People of All Ages
- Publication Date
-
2023-January-01
- Status
- Published
- Topic Area
- Pain
-
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. About 3,500 people in Ontario and 6,500 people across Canada have sickle cell disease.
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. Living under stressful social and economic conditions can impact the overall health and well-being of people with sickle cell disease.
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.”
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).
Quality Standard in Brief
-
Quality Statement 1: Racism and Anti-Black Racism
People with sickle cell disease (and their families and caregivers) experience care from health care providers within a health care system that is free from racism and anti-Black racism, discrimination, and stigma. Health care providers promote a culture that is compassionate, trauma informed, and respectful of people’s racial/ethnic and cultural backgrounds. They build trust with people with sickle cell disease (and their families and caregivers), work to remove barriers to accessing care, and provide care equitably.
Quality Statement 2: Comprehensive Health Assessment and Care Plan
People with sickle cell disease have a comprehensive health assessment at least annually from an interprofessional care team at a dedicated sickle cell disease centre to develop an individualized, person-centred care plan. The assessment and care plan are documented and shared with the person’s circle of care.
Quality Statement 3: Vaso-occlusive Acute Pain Episodes
People with sickle cell disease who present to an emergency department or hospital with a vaso-occlusive acute pain episode receive a timely pain assessment and clinical assessment. Their treatment begins within 30 minutes of triage or 60 minutes of presentation. Before discharge, they are involved in the development of a plan for continuing to manage their acute pain episode at home. This plan includes symptom management strategies and information on how to access follow-up care and support from health care providers, as needed.
Quality Statement 4: Life-Threatening Acute Complications
People who present to an emergency department or hospital with a potentially life-threatening acute complication of sickle cell disease have their condition and its severity identified through a prompt clinical assessment. Their condition is managed appropriately with an individualized treatment and monitoring plan.
Quality Statement 5: Chronic Complications
People with sickle cell disease are monitored by their local health care team for signs and symptoms of chronic complications of sickle cell disease. People with chronic complications are promptly referred to a dedicated sickle cell disease or other specialized centre for consultation and/or assessment and management of their condition, as needed.
Quality Statement 6: Referral to Health Care Professionals With Expertise in Chronic Pain
People whose quality of life is significantly impacted by chronic pain caused by sickle cell disease are referred to individual health care professionals or a chronic pain centre with expertise in chronic pain related to sickle cell disease and the ability to offer pharmacological and nonpharmacological interventions.
Quality Statement 7: Psychosocial Assessment, Information, and Support
People with sickle cell disease (and their families and caregivers, where appropriate) have regular psychosocial assessments to identify any psychosocial needs or barriers to accessing care. Those with unmet psychosocial needs are offered information and support to address these needs.
Quality Statement 8: Transition From Youth to Adult Health Care Services
Young people with sickle cell disease have a designated health care provider for the transition from youth to adult health care services. This provider works with the young person (and their parents and caregivers, where appropriate) to coordinate their care and provide support throughout the transition process. The provider continues to provide support until the young person (and their parents and caregivers, where appropriate) confirms that the transition is complete.
Supporting Documents
-
Patient guide for this quality standard
Know what to ask for in your care
Placemat for this quality standard
A quick-reference resource for clinicians that summarizes the quality standard and includes links to helpful resources and tools
Quality standard implementation toolkit
Quality improvement tools and resources for health care professionals, including an action plan template
Supplementary information to support the data collection and measurement process
The eReport is an online tool for hospitals. It allows you to access indicators that help enable quality improvement. The report is dynamic, and you can view data at the hospital and regional levels. You will need a ONE ID account to access the data.
Additional Resources
If you would like to receive these resources, please send us a message using our contact form:
- Case for improvement (slide deck)
Share why this standard was created and the data behind it, to get the support you need to put it into practice - Technical specifications
See the technical specifications for the indicators within the quality standard - Impact assessment and infographic
Learn how implementation strategies are improving outcomes and guiding quality improvement efforts - Summary of the public feedback we received
- Case for improvement (slide deck)
Last Updated: February 24, 2026
