Global Study Identifies Burden of SCD on Provider, Patient, Caregiver

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The survey identified long-term health complications of sickle cell disease as a primary concern among patients and providers and overall understanding of the disease needs more educational support.

New results from one of the largest studies examining the global burden of sickle cell disease were published just prior to World Sickle Cell Day, June 19th.

The multinational survey identified long-term health complications of the rare inherited blood disease as a primary concern among patients and healthcare providers (HCPs). Additionally, the physical, psychosocial, and economic burdens experienced by patient caregivers is extreme.

“Sickle cell disease is a lifelong condition that causes damage in the body and has a profound impact on the quality of life of those who suffer from it and their caregivers," Baba Inusa, MD, Professor and Consultant of Paediatric Haematology, Guy’s and St Thomas’ NHS Foundation Trust, Chair of the National Haemoglobinopathy Panel, said in a statement.

The SHAPE Survey

“Our aim for this survey is to add to the body of knowledge around better understanding and characterizing the misunderstood, under-reported experiences and unmet needs of people living with SCD, as well as those caring for and treating people with the condition,” Kim Smith-Whitley, MD, Executive Vice President, Head of Research and Development, GBT said.

The Sickle Cell Health Awareness, Perspectives and Experiences (SHAPE) survey was performed in 10 countries including Bahrain, Brazil, Canada, France, Germany, Oman, Saudi Arabia, United Arab Emirates, United Kingdom, and the US. Results were reported by more than 1300 patients, caregivers, and healthcare professionals. Fieldwork took place from September 2021-February 2022.

"The SHAPE survey is important because it illustrates how vital it is that we understand our patients’ needs, and it suggests what we within the medical community can do to help change perspectives, increase education and awareness, and improve care," Inusa stated.

The survey was commissioned by Global Blood Therapeutics (GBT) and conducted by Ipsos Healthcare, "a global health research agency dedicated to understanding the motivations, behaviors and influences of the multiple actors in the healthcare sector."

The Quantitative surveys assessed various aspects related to the burden of the disease, caring for the disease, and unmet needs. Surveys were completed by 919 patients, 207 caregivers and 219 HCPs. Eligible patients were at least 12 years old, caregivers were at least 18 and HCPs were eligible if they cared for a minimum of 10 patients with SCD.

This month, the results of the HCP portion of the survey were shared during a poster presentation at the European Hematology Association (EHA)2022 Hybrid Congress.

Findings Provided by HCP Surveys

According to a statement, the SHAPE survey was able to highlight the complex environment of treating people with sickle cell disease. The results indicated the long-term impact of the disease needs to be further addressed with more supportive education and tools for physicians.

“These results are a wake-up call," Inusa said, "and I believe that the actions that follow can enable us to help drive a better dialogue and improved conversations around the management and care of this long-neglected and devastating disease.”

Ultimately, findings displayed the need for improved overall awareness and understanding of sickle cell disease among HCPs.

  • Despite fatigue/tiredness being identified as having the biggest impact on patients’ lives (84%), only half (53%) of HCPs felt they have effective tools to treat this symptom.
  • Most HCPs (84%) believed that treating hemolytic anemia (low hemoglobin due to red blood cell destruction) can improve most aspects of SCD, including reducing organ damage.
  • Most HCPs (78%) felt confident in their knowledge of the long-term effects of SCD, but 83% wanted more support in educating their patients on this topic.
  • Nearly one third (31%) of HCPs found it challenging to understand patients’ concerns, with 43% citing difficulties due to having different ethnic background from their patients.
  • Two-thirds (66%) of HCPs felt that patients do not always understand the long-term effects of SCD, and more than one third (37%) were reluctant to raise potential future challenges or complications with a patient or their family.
  • Most HCPs (82%) agreed that SCD causes financial difficulties for patients, which may consequently affect their access to care.

Understanding Patients and Caregivers

The results from the patient and caregiver portions of the survey, were featured today in an oral presentation at the 4th Global Congress on Sickle Cell Disease.

Among the findings were significant effects of SCD on the everyday life of patients living with it. Not only do these individuals have to bear intense pain, fatigue and mental health issues, but all of these experiences interfere with their ability to be present and successful at important events, work or school, and as a result reduces their earning potential.

“SCD is the reality of so many people around the world, and yet patients often experience poor care, especially by non-specialist healthcare providers who may lack the training to provide good comprehensive care, Dianaba Ba, Director of Operations, SOS GLOBI: The Federation of Sickle Cell and Thalassemic Patients, and an individual who lives with SCD, said in a statement. "In recent years, we’ve started to see some positive change, but it is still not enough."

Patients and caregivers experience impact from sickle cell disease that extend beyond the immense physical pain experienced by patients. Both groups deal with broad, lifelong series of physical, emotional and social barriers.

  • Most patients (93%) agreed that reducing their risk of long-term health issues due to SCD, such as organ damage, was important to them.
  • Fatigue/tiredness was a symptom experienced by most patients (84%) in the past year, which also had the greatest negative impact on their ability to attend and be successful at school or work, and, thereby, on their earning potential.
    • Patients reported that due to SCD they missed an average of 7.5 days of school/work in the past month.
  • Mental health symptoms affected the majority of patients with SCD in the past year, including 62% who reported feeling depressed, low mood or down, and 57% who indicated they felt worry or nervousness.
  • Patients reported more positive experiences with specialist HCPs as compared with emergency departments and general HCPs.
    • More than half (54%) of SCD patients reported they believe they had experienced poor care in the past from emergency medicine professionals due to such providers’ lack of knowledge about SCD.
  • Over half of caregivers reported that patient symptoms negatively impact their own lives, such as their career and education (56%), long-term health prospects (55%) and overall well-being (53%).

"The challenges for patients seem endless and this survey highlights that, in spite of this, patients do not feel heard," Ba said. "They continue to face acute physical but also emotional damage, stigma and discrimination.”