Patients with hemophilia differ in the cognitive and behavioral adaptive coping mechanisms used to manage the disease depending on personal characteristics and disease severity, according to the results of a multicenter cross-sectional study published in Haemophilia.

Investigators for the Adherence to Treatment in Hemophilia study (ADHERENCE; Identifier: NCT02191436) aimed to distinguish psychosocial attributes that could affect the approach and evolution of hemophilia in adolescent, young, and adult patients.

A total of 63 patients (mean age, 37 years) with hemophilia A (81%) or B (19%) with varying degrees of severity (51% severe, 17% moderate, and 32% mild severity) were included in the analysis; 37% were receiving prophylactic treatment, 63% were receiving on-demand treatment, and 52% of all patients reported coinfection with HIV, hepatitis C, or both.

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The questionnaires used included 8 primary scales derived from a Likert scale: problem-solving, cognitive restructuring, social support, emotional expression, avoidance of problems, wishful thinking, social withdrawal, and self-criticism.

Significant differences in problem-avoidance strategies were found across varying disease severities, with this strategy most frequently used by patients with moderate disease severity. Patients who received prophylactic treatment used maladaptive strategies such as self-criticism, social withdrawal, and wishful thinking more frequently than patients who received on-demand treatment. In addition, younger patients used more negative coping strategies compared with middle-age patients, who used more positive coping strategies.

By evaluating illness behavior and perception of its manageability and correlating these findings with coping strategies, the investigators “found that patients with better perception of their disease adopt more positive coping strategies (social support, problem-solving, and cognitive reconstructing). “On the other hand,” they noted, “it is observed that patients with more irritable and hypochondriac attitudes show negative coping (social withdrawal), and patients with good illness behavior use better coping (emotional expression and social support).”

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“We believe that the lack of understanding of the psychological variables (which influence the patient’s response to the disease), and no joint decision-making between professional and patient, complicate the management of the disease and non-adherence of treatments despite [of] their improvements in the last [latter],” concluded the investigators.


Torres-Ortuño A, Cuesta-Barriuso R, Nieto-Munuera J, Galindo-Piñana P, López-Pina JA for the Adherence to Treatment in Hemophilia (ADHERENCE) study. Coping strategies in young and adult haemophilia patients: a tool for the adaptation to the disease [published online April 17, 2019]. Haemophilia. doi:10.1111/hae.13743