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A global Pfizer-sponsored survey found that some HCPs feel they don’t have the adequate support to educate patients1Common causes of mortality in patients with SCD27 A Pfizer-sponsored 2024 global survey assessed the perspectives of the burden of sickle cell disease (SCD) on patients and their caregivers and captured the viewpoints of HCPs1 Data from a 2024 global survey included 919 patients, 207 caregivers, and 219 HCPs (hematologists, hematologist-oncologists, pediatric hematologists, pediatricians, internists, and general/family practitioners). 295 patients, 30 caregivers, and 50 HCPs were based in the United States. HCPs who participated in the survey were from the United States, Canada, UK, Germany, France, UAE, Saudi Arabia, and Brazil.1,2

Information provided from this survey should not be considered conclusive.

Limitations:
The Sickle Cell Health Awareness, Perspectives, and Experiences (SHAPE) survey is not a validated questionnaire, and only includes 10 countries (many of which were high income). SHAPE was not validated or optimized for use across various countries. This survey was conducted by Ipsos Healthcare (London,​ UK) and sponsored by Pfizer.1
Help your patients understand their symptoms
Select symptoms in key organs should prompt additional evaluation3

Cardiopulmonary and renal complications due to SCD can be the most challenging to treat. In otherwise asymptomatic patients, the following day-to-day signs or symptoms may warrant a consultation per the most recent American Society of Hematology (ASH) guidelines3:

  • Dyspnea at rest
  • Hypoxemia at rest
  • Chest pain at rest
  • Increase in exercise limitation
  • Sleep-disordered breathing
  • Wheezing or increased cough at rest
The American Society of Hematology (ASH) guideline panel addressed questions related to the management of cardiopulmonary and renal complications in SCD. The areas of focus were: pulmonary hypertension, pulmonary arterial hypertension, chronic lung disease, sleep-disordered breathing, hypertension, proteinuria, chronic kidney disease, and venous thromboembolism.

Limitations3:
These guidelines are limited by the low certainty of evidence in many of the questions. In the absence of evidence from SCD populations, the ASH guideline panel relied on indirect evidence from the general population for recommendations. The questions prioritized by the panel and guidelines may not include a full list of questions deemed clinically important to other HCPs in the cardiopulmonary and renal space of SCD.

For more information regarding the ASH guidelines, visit hematology.org

References:de Montalembert M, Anderson A, Costa FF, et al. Sickle Cell Health Awareness, Perspectives, and Experiences (SHAPE) survey: perspectives of adolescent and adult patients, caregivers, and healthcare professionals on the burden of sickle cell disease. Eur J Haematol. 2024;1‐11.de Montalembert M, Anderson A, Costa FF, et al. Sickle Cell Health Awareness, Perspectives, and Experiences (SHAPE) survey: perspectives of adolescent and adult patients, caregivers, and healthcare professionals on the burden of sickle cell disease. Eur J Haematol. 2024;1‐11(suppl.):1-32.Liem RI, Lanzkron S, Coates TD, et al. American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease [published correction appears in Blood Adv. 2023; 7(14):3530]. Blood Adv. 2019;3(23):3867-3897. 
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