The Effects of Hemophilia on Socialization

BACKGROUND: Hemophilia, a congenital bleeding disorder resulting from a deficiency in clotting factors, typically VIII or IX, results in a propensity for severe, sometimes life-threatening or disabling, bleeds. Severity is determined by the baseline level of factor in plasma. Patients often require regular intravenous infusions of factor to prevent or treat bleeding episodes. Joint bleeds can lead to disabling arthropathy and past contamination of blood products has resulted in an epidemic of HIV and hepatitis in this community. We hypothesize that these issues may result in declines in quality of life, psychosocial well-being, and socialization (integration into society) and that socialization correlates with health-related quality of life (HR-QoL).

METHODS: We developed and conducted a socialization survey and interview of patients age >20 with hemophilia A (n=14) or B (n=4) and their spouses/significant others (SSOs) (n=9). The interviews were analyzed using PROMIS (patient-reported outcomes measurement information system-29) domains. Patients completed surveys in health-related quality of life measures including both A36 Hemofilia-QoL and WHOQOL-BREF. Patients were also scored according to the Colorado Joint Assessment Scale and Karnofsky Performance Scale. With the exception of the Colorado Joint Assessment Scale, higher scores in all surveys reflect better health status. IRB approval and informed consent were obtained.

RESULTS: 19 patients were enrolled, but one withdrew. Ages ranged from 24-78. Nine patients had severe hemophilia, 5 had moderate disease, and 4 had mild disease. Four patients did not have SSOs (22%); these included two severe and two moderate patients, one with HIV, and three with hepatitis C. Five SSOs declined participation. For the WHOQOL-BREF, patients reported overall quality of life in the physical domain an average score of 60 (13-94), standardized 0-100, 66 (31-100) in the psychological domain, 66 (31-100) in the social relationship domain, and 81 (44-100) in the environmental domain. For the A36 Hemofilia-QOL, the median total score was 94 (43-132) out of a possible 144 adding the following domains: Physical health, daily activities, joint damage, pain, treatment satisfaction, treatment difficulties, emotional functioning, mental health, and relationships and social activities. Colorado Joint Assessment Scale-QOL provided average scores of 6.1 out of 19 for ankles without gait and 8.3 out of 21 with gait, 4.2 for knees without gait, 6.3 with gait, and 3.6 for elbows. Preliminary analysis of interviews reflects social support as a common domain in both patients and SSOs and high reports of anxiety amongst SSOs compared to patients.

CONCLUSION: This study characterizes quality of life by different measures in a hemophilia population and may affect their ability to integrate socially. The study employed established instruments as well as novel questionnaires and interview structures, although the latter have not been validated. Analysis of interviews is ongoing, but preliminary results point towards higher levels of anxiety in SSOs compared to patients. Both health-related-QoL and disease severity appear to be associated with domains of socialization. Patients with more severe disease may be less likely to have SSOs.