Researchers who assessed new parameters of health-related quality of life (HRQoL) in patients with sickle cell disease (SCD) discovered that coherency and self-efficacy can help healthcare professionals better plan clinical interventions.

The study was published in Annals of Hematology. 

SCD is perhaps most well-known for the long list of complications that can occur in patients; among them are blindness, stroke, acute chest syndrome, and renal failure. Even if patients with SCD suffer from relatively few complications, they are still at a high risk of experiencing recurrent and painful vaso-occlusive crises that require hospitalization. 

SCD is a condition that carries a relatively high mortality rate. Nevertheless, advances in SCD therapeutics mean that individuals are living longer. The challenge for physicians, of course, is to support adolescent/adult patients with SCD as they grapple with school/work, relationships, and finance. 

Read more about SCD etiology 

Patients with SCD struggle with both the physical and more psychological aspects of their disease. For example, researchers have explored the patient mindset with regard to their level of confidence in carrying out certain tasks. In addition, investigators are determining how patients’ view on rewards and gratification influence their motivation levels. All these parameters are part and parcel of perceived quality of life. 

“Sense of coherence” is a term used to describe the ability of an individual to adapt to crises and challenges in life. Studies have reported that self-efficacy and a sense of coherence are positively correlated. Because the medical literature on these issues are rare, the authors of the study set out to study HRQoL as expressed in these terms in a cohort of patients with SCD. The data collected was then used to explore predictors of overall HRQoL. 

The research team carried out their study at the Shahid Beheshti University of Medical Sciences and Ahvaz Jundishapur University of Medical Sciences. Eighty-three patients with SCD were recruited. The recruited participants were asked to fill in a number of surveys, including the RAND 36-item survey, the Sickle Cell Self-Efficacy Scale, and the Sense of Coherence Scale. 

Read more about SCD comorbidities

Overall, the results of the study indicate that patients with SCD do not feel sufficiently supported, either physically or mentally. In the surveys sent out, most participants reported their physical and mental HRQoL as “poor” and “average.” This is especially true in the cohort of patients who received blood transfusion therapy and hydroxyurea. 

“Also, our results show that sense of coherence was the predictor of the mental HRQoL, but self-efficiency was the predictor of the physical HRQoL,” the authors wrote. “These results can assist clinicians and nurses to plan holistic clinical interventions by focusing on the sense of coherence and self-efficiency to improve SCD patients’ HRQoL.” 


Ahmadi M, Beiranvand S, Poormansouri S, Matbouei M, Rohani C. Sense of coherence or self-efficacy as predictors of health-related quality of life in sickle cell disease patientsAnn Hematol. 2022;10.1007/s00277-022-05007-2. doi:10.1007/s00277-022-05007-2