Respiratory Disease and SCD

Participants:

  • Host
    • Dr. Carolyn Rowley – Executive Director & Founder, Cayenne Wellness Center
  • Moderator
    • Dr. Keith Quirolo
  • Speaker
    • Dr. Elizabeth “Liz” Klings
      • Associate Professor, Department of Medicine, Boston University School of Medicine
      • Director, Center for Excellence in Sickle Cell Disease, Boston Medical Center
      • Director, Pulmonary Hypertension Clinic, Boston Medical Center

Course Description
This CME/CE mini-seminar features pulmonologist and sickle cell expert Dr. Elizabeth (Liz) Klings in conversation with Dr. Carolyn Rowley and Dr. Keith Quirolo as they unpack the complex relationship between pulmonary hypertension (PH), venous thromboembolism (VTE), and sickle cell disease (SCD). Using real patient cases, Dr. Klings explains why pulmonary vascular complications are a leading cause of morbidity and mortality in adults with SCD, and how to recognize, diagnose, and manage them in clinical practice.

Participants will learn how to interpret key echocardiogram findings (including TR jet velocity), understand when and why right heart catheterization is required, and differentiate between pre-capillary and post-capillary pulmonary hypertension. The session also explores the impact of chronic anemia, thrombosis, sleep apnea, central venous catheters, pregnancy, and comorbid conditions on pulmonary outcomes. Practical guidance is provided on treatment strategies such as optimizing sickle cell–specific therapy (e.g., hydroxyurea, transfusions), use of PAH-targeted medications, anticoagulation decisions, and when to refer to pulmonology or cardiology.

By the end of this course, clinicians will be better equipped to identify high-risk patients, initiate appropriate workups, collaborate with specialists, and counsel individuals with SCD and suspected or confirmed pulmonary hypertension.

Learning Objectives
 After completing this course, participants will be able to:

  • Describe the epidemiology and incidence of pulmonary hypertension among adults living with sickle cell disease.
  • Interpret key echocardiographic markers (e.g., TR jet velocity) and explain why right heart catheterization is required to confirm a diagnosis of pulmonary hypertension.
  • Differentiate pre-capillary from post-capillary pulmonary hypertension in SCD and discuss how this distinction guides management.
  • Identify clinical risk factors and red-flag symptoms (e.g., progressive exertional dyspnea, VTE history, leg ulcers, proteinuria, sleep apnea) that should prompt screening and referral.
  • Outline evidence-based approaches to treatment, including optimization of SCD therapies, anticoagulation, oxygen and diuretic use, and appropriate use of PAH-targeted drugs.
  • Discuss special considerations for pediatric and adolescent patients, pregnancy, and patients with recurrent VTE or chronic thromboembolic disease.

Target Audience

  • Hematologists and oncologists
  • Pulmonologists and cardiologists
  • Primary care providers and hospitalists
  • Advanced practice providers (NPs, PAs)
  • Nurses, social workers, and allied health professionals caring for individuals with sickle cell disease

Course Content