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Key Clinical Summary: Strengthening the Sickle Cell Disease Triad

This is a micro-learning module summary of Prof Smith's Education session which you can find here.

Before participating please read our CME and disclosure information which can be found here. This program was supported by an independent medical education grant from Novo Nordisk. This content is intended for US Healthcare Professionals only.

Introduction:

This educational session provides an overview of the "Sickle Cell Disease Triad"—a model emphasizing the crucial collaboration between patients, the healthcare system, and community-based organizations (CBOs) to improve outcomes for individuals with sickle cell disease (SCD). SCD is a severe genetic hemoglobinopathy initiated by a single point mutation in the β-globin gene, leading to the polymerization of hemoglobin S, red blood cell sickling, vaso-occlusion, and subsequent multi-organ damage, pain, and reduced life expectancy. The management of SCD is complicated by significant cultural and communication gaps between the biomedical community, patients, and society at large. This summary outlines the core challenges within the SCD care landscape and strategies to strengthen the triad for better, more equitable patient care.

The Fractured State of SCD Care:

A major barrier to effective SCD management is the fragmented and under-resourced state of the adult care system. This leads to a perilous transition from pediatric to adult services, with many young adults becoming "medically homeless".

  • Provider Shortage and Lack of Confidence: There is a significant shortage of adult healthcare providers with expertise in SCD. Many primary care physicians feel uncomfortable managing these complex patients. This lack of confidence extends to establishing sustainable, financially viable SCD programs, unlike in other chronic diseases like cancer.
  • Underestimation of Disease Burden: Because most SCD symptoms, including severe pain, are managed by patients at home, the healthcare system only sees the "tip of the iceberg". This leads to a vast underestimation of the disease's true quantity and severity.
  • Poor Care Coordination and Outcomes: The lack of providers and system support results in insufficient care coordination, poor handoffs from pediatric care, and a failure to use essential therapies. For instance, it is estimated that only 28% of adult SCD patients who access specialty care receive hydroxyurea, a life-saving medication. This systemic failure contributes to premature death, often in patients' 20s and 30s.

The Role of Community and Cultural Humility:

Strengthening the SCD triad requires empowering CBOs and fostering a culture of humility within the healthcare system.

  • Underfunded and Disconnected CBOs: Many SCD CBOs are small, underfunded, and disconnected from one another, operating with budgets often less than $50,000. This is in stark contrast to patient advocacy groups for other diseases. For example, while cystic fibrosis affects about 30,000 people in the U.S. and receives roughly $5,074 per person in private philanthropic support, SCD affects approximately 100,000 people but receives only $6 per person.
  • The Power of Community Health Workers (CHWs): A key strategy to bridge this gap is the integration of CHWs. These are trusted individuals from the patient's own community who can help navigate the complex healthcare system, improve communication, and connect patients with resources.
  • Cultural Humility and Trust: Historical trauma, racism, and discrimination have created a legacy of mistrust in the healthcare system. Clinicians must practice "cultural humility"—approaching each patient with respect, recognizing inherent biases, and valuing the patient's lived experience and resilience. This is critical for building the psychological safety needed for shared decision-making. Awareness of SCD is also critically low; only about one-third of African Americans know that the disease disproportionately affects people of African descent.

A Path Forward: The ST3P-UP Model:

Inspired by patient advocates like the late Dr. Trevor Thompson, the Sickle Cell Trevor Thompson Transition Project (ST3P-UP) offers a model for enhancing collaboration within the triad. The goal is to create a system where the patient, the healthcare system, and the community work as trusted partners.

  • Building Trust and Communication: The foundation of this model is improving communication and building mutual trust among the three partners, who are often unaccustomed to working together. This involves developing a common language, learning empathy, and starting with small, trust-building collaborations.
  • Shared Power and Decision-Making: The ultimate aim is to move beyond the traditional power imbalance, where patients often feel powerless, towards a model of shared decision-making and shared power.
  • National Mandate for Change: The 2020 National Academies of Sciences, Engineering, and Medicine report on SCD reinforces this need, calling for comprehensive team-based care, new payment models, and directly addressing institutional racism to improve health outcomes.

Conclusion:

Improving outcomes in sickle cell disease requires a fundamental shift from a fractured, provider-centric system to an integrated, collaborative triad of care. This involves empowering patients, adequately funding and organizing community-based organizations, and fostering a healthcare environment built on cultural humility, empathy, and mutual trust. By addressing the deep-seated inequities and systemic barriers, the healthcare community can begin to move towards a future of justice and improved health for all individuals living with SCD.