Many clinical studies are centered on finding a path toward a better or longer-lasting therapeutic approach to a particular disease. The tenacity and fortitude of medical researchers who painstakingly source data, build on the work of their predecessors, and plow forward with innovative tests and proposals are indeed admirable.
Other clinical studies that equally deserve praise and attention are those that focus on the impact of a disease on the quality of life of a patient. In the busyness of clinical practice, it can sometimes be easy to forget that patients with chronic diseases are experiencing challenges on a daily basis. This, in turn, affects the quality of life of their parents, guardians, and carers.
Put simply, disease is suffering. In between optimistic exchanges during long-term follow-ups are the very real challenges that patients face when living with a rare and incurable disease.
Results of the Global SWAY Study
A global team of researchers recently conducted an international survey to glean insights into how sickle cell disease affects patients’ quality of life. The International Sickle Cell World Assessment Survey, dubbed “SWAY,” was an ambitious project, covering a number of countries across 6 geographical regions. The purpose of the survey was to assess the impact of sickle cell disease on patients of all ages—from the perspective of both healthcare professionals and patients.
The survey was intended to examine the impact of living with sickle cell disease on patients’ daily activities, education, relationships, employment, and emotional life. The survey also asked participants about the frequency of vaso-occlusive crises and their satisfaction with their treatment regimes.
Read more about sickle cell disease etiology
A total of 2145 patients with sickle cell disease were recruited in 2019, and 1461 completed the survey. The questions were carefully crafted with input from various stakeholders. The findings of the survey were extensive. Below are some statements that more than 50% of the respondents reported:
- The avoidance of intense physical activity
- Feeling worried about painful episodes while working out
- Feeling worried about feeling exhausted while working out
- The belief that sickle cell disease has impacted their emotional well-being
- Feeling frustrated by having to live with symptoms of the disease
- Feeling worried about their sickle cell disease getting worse
- Feeling worried about family/friends who need to take care of them
- Feeling that sickle cell disease has limited their career options
- Experiencing fatigue
- Experiencing body aches.
The list of items on the survey that resonated with participants provides for a heartbreaking read; healthcare professionals who are deeply engaged with caring for sickle cell patients will have had many of these same complaints expressed to them.
Similar Findings in the Middle East and North Africa
Another team of researchers conducted a systematic review of the quality of life of pediatric sickle cell patients in the Middle East and North Africa region and reported similar findings to the SWAY study. In their systematic review, Pandarakutty and colleagues reported that:
- Studies suggest that the quality of life of a patient is adversely affected by pain, among various parameters.
- Sickle cell disease resulted in social and psychological problems, such as enuresis, depression, school absenteeism, and deteriorating academic performance.
- Studies indicate that both quality of life and adherence to hydroxyurea therapy were low, suggesting that patients were uncomfortable with its effectiveness and side effects.
- Patients with sickle cell disease scored low on physical, emotional, and academic functioning domains.
It is therefore clear from both studies that the impact of sickle cell disease goes beyond just the number of sick days and the cost of health care. Both studies demonstrate the devastating effect that sickle cell disease can have on a patient’s emotional, mental, physical, and social well-being.
The cross-border nature of both studies also suggests that the disease burden suffered by patients with sickle cell disease is universal. Whether the patient is in the Middle East or in Europe, reports of the negative impact of sickle cell disease on quality of life are nearly identical.
Improving Support for Patients With Sickle Cell Disease
What can be done to provide better support to patients with sickle cell disease? Pandarakutty wrote, “The authors recommend the implementation of mandatory awareness and premarital screening programs, thereby increasing understanding, reducing stigma and minimizing the incidence of hereditary diseases such as sickle cell disease.”
In addition, they also recommended play therapy among pediatric patients to encourage socialization and reduce psychological stress.
Read more about sickle cell disease treatment
Upon reflection of the findings of the SWAY survey, the international research team wrote, “Healthcare professionals should work closely with their patients to understand how sickle cell disease is affecting them personally and, in turn, tailor management strategies according to the individual patientʼs experience.”
Osunkwo I, Andemariam B, Minniti CP, et al. Impact of sickle cell disease on patients’ daily lives, symptoms reported, and disease management strategies: results from the international Sickle Cell World Assessment Survey (SWAY). Am J Hematol. 2021;96(4):404-417. doi:10.1002/ajh.26063
Pandarakutty S, Murali K, Arulappan J, Al Sabei SD. Health-related quality of life of children and adolescents with sickle cell disease in the Middle East and North Africa Region: a systematic review. Sultan Qaboos Univ Med J. 2020;20(4):e280-e289. doi:10.18295/squmj.2020.20.04.002