Knowledge of HIV/AIDS and emotional adjustment in a cohort of men with haemophilia and HIV infection: final report

This study presents the final report of a long-term psychological assessment of men with haemophilia and HIV infection. The knowledge, emotional impact regarding HIV infection and prospective changes over time and the need for psychological support were evaluated. The study group comprised 118 men with haemophilia, 66 HIV seropositive and 52 seronegative, from the Haemophilia Centres in Bari, Florence, Milan and Naples. All subjects performed psychological tests (STAI: state and trait anxiety inventory; SDS: self-rating depression scale) and completed questionnaires to ascertain their knowledge and the emotional impact of AIDS. After enrollment (1992-93) the assessment was repeated twice over a 2-year period. A high percentage of subjects in both groups answered the questionnaire on knowledge correctly and, more specifically, all (100%) admitted knowing that sexual intercourse was a risk factor for HIV infection, adding that sexual partners of haemophiliacs with HIV should be regularly tested. The percentage, however, decreased for seropositives who admitted to always using a condom during sexual intercourse (86%) and for those who declared that partners were periodically tested for HIV (60%). The most important feature of the study is that, contrary to predicted expectations, seropositive and seronegative subjects presented the same degree of emotional involvement: there are no statistically significant differences in average scores between groups either on the anxiety or depression scales. Moreover, for certain aspects, seronegatives revealed greater emotional involvement: at baseline evaluation, they felt more fear and unhappiness with statistically significant differences compared to asymptomatics. Furthermore, seronegatives more than seropositives continue to feel reluctance towards infusion and avoid blood products after learning of AIDS. These results emphasize the importance of paying due attention to the emotional status of seronegatives. Their reluctance towards the use of blood products (despite present safety) is a very important issue for the possible consequences of treatment with the risk of worsening the clinical condition. In conclusion, we believe that counselling on HIV infection/AIDS needs to address every person with haemophilia regardless of HIV status.     

Partners of men with HIV infection and haemophilia: controlled investigation of factors associated with psychological morbidity.

The study was concerned with determining the prevalence of psychosocial problems in partners of men with haemophilia and HIV infection, and with identifying factors associated with psychological morbidity. Partners of 17 HIV positive and partners of 19 HIV negative men with haemophilia were assessed in a cross-sectional controlled investigation. Outcome measures included current psychological status and psychiatric history; coping and health beliefs; and social and sexual functioning. Partners of HIV positive men with haemophilia, the majority of whom were asymptomatic, were not found to suffer more psychological difficulties than the partners of HIV negative men with haemophilia. However, partners of haemophiliacs regardless of HIV status suffer from higher levels of psychological distress than comparable women in the community, and those with past psychiatric history and current social difficulties are particularly at risk. The sexual relationships of couples with HIV positive men were more adversely affected than those of HIV negative couples.     

Comparison of the quality of life between HIV-positive haemophilia patients and HIV-negative haemophilia patients

Summary. In a prospective study, we tested the hypothesis that an already reduced quality of life in haemophilia patients is further diminished in those haemophilia patients who contracted the human immunodeficiency virus (HIV) as a result of transfusion of coagulation factor preparations. From an available pool of 92 males with haemophilia A or B, 18 patients seropositive for HIV infection and 11 seronegative patients were randomly selected for the study. We applied two instruments to measure the quality of life (QOL) in our patients. The first instrument was the quality of well-being (QWB) scale that unifies QOL into a single score based upon an assessment of the patient's symptoms and health-related reductions in mobility, physical activity and social activity. The second instrument was SF-36, the questionnaire from the Medical Outcome Study (MOS) that measures six dimensions of health status (physical functioning, role functioning, social functioning, pain, mental health and health perception). Measurements were obtained with both instruments at three interviews with each patient over a 1-year interval. As expected, HIV disease reduces QOL in haemophiliacs. The number of bleeding episodes within 2 months of interview was increased in the HIV-positive cohort but not within 6 days of interview, indicating that HIV disease independently affects QOL in haemophilia patients. In a typical 30-year-old patient, haemophilia itself has reduced quality of their lives by 9.3 years, and HIV disease additionally from 8.5 to 20 years. On the MOS scales, the two patient groups differed significantly only in the dimensions of health perception and pain magnitude. Although HIV disease led to a decrement in QOL of haemophilia patients, it also appears that haemophilia patients are able to develop coping skills to prevent more drastic effects of HIV disease on their QOL. Future studies will need to explore the nature and mechanisms of this ‘buffering’ effect.     

Sexual identity and HIV status influence the relationship between internalized stigma and psychological distress in black gay and bisexual men

Experiences of internalized homophobia and HIV stigma in young Black gay and bisexual men (GBM) may lead to psychological distress, but levels of distress may be dependent upon their sexual identity or HIV status. In this study, we set out to explore the associations between psychological distress, sexual identity, and HIV status in young Black GBM. Participants were 228 young Black GBM who reported on their psychological distress, their HIV status, and their sexual identity. Results indicated that internalized homophobia was significantly related to psychological distress for gay men, but not for bisexual men. HIV stigma was related to psychological stress for HIV-positive men, but not for HIV-negative men. Results indicate a need for more nuanced examinations of the role of identity in the health and well-being of men who have sex with men.     

Health care provider and self-assessment of health status and sexual behaviour in HIV-seropositive young men with haemophilia

We hypothesized that persons with HIV who perceive themselves as having waning health status may participate in fewer sexual behaviours than HIV-infected persons without HIV-related health problems. The object-ives of this study were to compare health care provider responses with participant responses for health status and for sexual activity and to examine the relationship of HIV-related health status to the sexual behaviour of HIV-seropositive adolescents and young men with haemophilia. A detailed questionnaire designed by researchers from 11 participating US haemophilia treatment sites and CDC personnel was administered to 306 HIV-seropositive young men with haemophilia aged 12–25 years. A health care provider from the haemophilia treatment centre also completed a questionnaire on each respondent. Self-assessment of HIV-related health status was similar to provider assessment of health status. Providers accurat-ely assessed participant sexual activity (overall Kappa = 0.62). Participation in vaginal intercourse and condom use was independent of health status. Following extensive educational efforts, most HIV-seropositive adolescents and young men with haemophilia are aware of the relationship between their HIV seropositivity and health status. Waning health status does not reduce participation in penetrative sexual behaviours or increase use of condoms. HIV prevention efforts should continue for this population.     

Age-related risk for HIV infection in men who have sex with men: examination of behavioral, relationship, and serostatus variables.

The study examined behavioral, relationship, and serostatus variables that potentially contribute to HIV infection risk in three age groups of men who have sex with men (MSM). MSM recruited in West Hollywood, California self-administered a questionnaire measuring unprotected insertive anal intercourse (UIAI) and unprotected receptive anal intercourse (URAI) with primary and nonprimary partners. The following relationship/serostatus variables were also assessed: recency of HIV testing, knowledge of own HIV serostatus, perception of partner's serostatus, seroconcordance (self and partner seronegative), and self-reported monogamy status. The prevalence of UIAI and URAI was higher with primary than nonprimary partners. These sexual risk behaviors with primary partners were substantially more prevalent among men younger than 25 years of age than among men aged 25 to 30 or over age 30. UIAI and URAI with nonprimary partners were uncommon in each age group, and there were no significant age differences on the serostatus and relationship variables. The findings suggest that young MSM may be at elevated risk for contracting HIV by virtue of their sexual risk behavior with primary partners. Targeted interventions for MSM need to address sexual risk in the context of primary relationships.     

A longitudinal study of family structure in Swedish persons with haemophilia

Haemophilia is an X‐linked inherited rare bleeding disorder affecting mainly men. The treatment consists of replacement therapy that has been associated with severe side effects, such as blood transmitted viral infections, but has markedly improved over the last decades. The aim of this study was to study family structure over time among Swedish persons with haemophilia (PWH), focusing on children, siblings and marital status. PWH A or B were identified from the haemophilia centres and the national Patient Registry. Each PWH was compared to five age‐ and gender‐matched controls. The national Multi‐Generation Registry was used to identify children and siblings. A total of 1365 children with a father suffering from haemophilia A or B and 1938 siblings of the PWH were identified. Having one or more children was significantly less common (P = 0.003) for PWH than for controls. Significantly lower rates of having a child were also found for the subgroups of persons suffering from severe haemophilia and those infected with HIV (P < 0.001). A higher proportion of PWH, with or without HIV and/or viral hepatitis had siblings compared to the controls (P < 0.001). However, the mean number of siblings was significantly lower for persons with severe haemophilia (P = 0.001). The number of marriages and divorces did not differ between PWH and controls. Our data indicate a negative impact of HIV and viral hepatitis on family structure for PWH despite the relatively good access to treatment in Sweden over the last few decades. This was particularly true for those with a severe form of haemophilia.     

Emotional adjustment of children and adolescents with haemophilia in relation to the HIV threat

A Dutch nation-wide study on young haemophilia patients, whose former treatment placed them at risk for HIV infection, was done to examine the effect of HIV testing, HIV status, disclosure of HIV status and the child's experiences with disease-related information on emotional adjustment. In the Netherlands HIV tests are not routinely performed for paediatric haemophilia patients, but the number of HIV-infected children is estimated to be low (8%). Seventy-two boys with haemophilia, five HIV positive, 51 HIV negative and 16 not tested, and their mothers were interviewed and administered standardized questionnaires. Multiple regression analyses showed no differences in anxiety and depression between HIV-positive, HIV-negative or untested boys, nor between children who were informed or not informed about their HIV status. Conversely, children who experienced more difficulties to obtain information about the their disease were significantly more anxious and tended to be more depressed. No significant differences in emotional distress were found between the patients and a standardization sample of healthy peers. The data provide evidence that children and adolescents with haemophilia cope effectively with their disease and the emotional impact of the HIV threat. There seems to be no reason to protect children from testing or knowing their HIV status. In the absence of compelling medical reasons, however, health care workers should not forcefully advocate testing or early disclosure. Distinct disease and treatment-related factors as well as social factors modify disclosure practices.     

HIV seroconversion in Swedish haemophiliacs: relation to type and dosage of factor concentrate

Human immunodeficiency virus (HIV) seroconversion among 40 Swedish haemophilia A patients has been investigated by retrospective sera testing. 22/40 patients had developed HIV antibodies before 1983, i.e., when heat-treatment of American factor concentrates was introduced. All patients had received American and Swedish factor concentrates, thus no case of seroconversion was seen among patients treated exclusively with non-heat-treated Swedish concentrates. Of 79 patients with severe or moderate haemophilia, all of whom had received both American and Swedish concentrates, the 36 seropositives were compared with the 43 seronegatives. The total number of units received did not differ between the two groups, though the seropositive group had received significantly more American concentrate. Two batches of concentrate were proved to have been infected. 29 seropositive and 13 seronegative patients had been treated with at least one of these batches. As expected, and unlike most of the seronegative patients, patients in the seropositive group generally had abnormal lymphocyte subsets.     

Human recombinant DNA-derived antihaemophilic factor (factor VIII) in the treatment of haemophilia A: conclusions of a 5-year study of home therapy

Fifty-eight previously treated haemophilic subjects were treated exclusively with the recombinant FVIII (rFVIII-KOGENATE®) produced by Bayer Corporation (Berkeley, CA) in an international multicentre prospective study of more than 5 years duration. Fifty-four of the 58 had severe haemophilia (< 2% FVIII) and four had moderate haemophilia (2–5% FVIII); 23/58 (40%) were seropositive for HIV, while 35/58 (60%) were HIV seronegative. Patients were monitored for safety and efficacy over a median period of 4.7 years (range 0.9–5.9 years) and received 17 922 infusions totalling 25.7 million units of rFVIII. Of 7107 bleeding episodes reported in home diaries, 5831 (82%) required only one treatment with rVIII. Twenty-five invasive surgical procedures in 17 patients, including eight joint replacements, were successfully accomplished and 13 serious bleeding episodes in eight patients were successfully treated. FVIII recovery performed on 885 occasions using 39 different lots of rFVIII showed mean incremental recovery of 2.48% IU^?1 kg^?1 (± 0.64). Adverse events were associated with 42 infusions (0.2%); none caused discontinuation of therapy. Immunological parameters remained stable in HIV-seronegative subjects treated with rFVIII; a small decrease in CD4 counts was noted in HIV-seropositive individuals (mean ? 37.2 cells mm–3 yr^?1). No de novo formation of inhibitors to FVIII was noted; and no clinical allergic reactions occurred to murine or hamster proteins. These conclusions from the longest monitored safety study ever performed for a haemophilia treatment product (with more than 5 years of observation) confirm previous interim study reports that rFVIII is well tolerated over the long-term, has biological activity comparable to that of plasma-derived FVIII, and is safe and efficacious for the treatment of haemophilia A.     

Fatherhood and HIV-positive heterosexual men

OBJECTIVES: The study of HIV and issues of reproduction is women-focused. HIV-positive men are overlooked and understudied. This study examined views on reproduction of heterosexual HIV-positive men. METHODS: Systematic questionnaire data were gathered from HIV-positive men (n=32) attending an HIV clinic in London. RESULTS: Heterosexual men were rarely given medical advice on reproduction (only 9.4%). Few felt fully informed (21.9%), while many felt uninformed (46.9%) or needed more information (28.1%). Over half would value fertility/fathering consultations, up-to-date information and quick referral to fertility clinics. Nearly half (43.8%) had considered having children and 37.5% had had a child prior to HIV diagnosis. HIV status affected views on fathering, and the advent of new treatments changed views in over half of the men. Almost half (41%) believed they would experience discrimination if they conceived a baby and a quarter would withhold their HIV status when attending antenatal clinics. The majority (81%) believed that a child gave meaning to life and something to live for - only 3.1% felt a child would be a burden. Most men overestimated potential vertical transmission and would value time to discuss fathering and fatherhood. CONCLUSIONS: There are gaps in provision. The majority of men felt that children gave meaning to life and a reason to live. Reproduction issues are not raised with HIV-positive men who are uninformed and unclear where to turn for information. Fatherhood should not be shunned as an issue for all HIV-positive men.     

The immunological and clinical outcome of HIV infection: 31 months of follow-up in a cohort of homosexual men

T-cell subsets, antibodies (Ab) against human immunodeficiency virus (HIV) and clinical status were evaluated during a 31 (24-35) month follow-up study of homosexual men. The study group included 50 homosexual men, with many sexual partners, who by 1982-83 were without symptoms and had a prevalence of HIV Ab of 38%. Among the men who were seropositive on the initial investigation a significant decrease occurred in the absolute number of CD4+ lymphocytes (p less than 0.01). 88% of these men experienced a decrease, and by follow-up 59% had CD4+ lymphocytes below the normal range. Also the men who seroconverted during the study had a significant decrease in CD4+ lymphocytes, while no changes were observed in the seronegative group. None of the subgroups had significant changes in CD8+ lymphocyte number. AIDS or AIDS related complex developed in 33% of the men seropositive at inclusion. None of these clinical syndromes developed in the seroconverting or the seronegative group. The men who eventually developed clinical symptoms did not differ significantly from the healthy HIV Ab positive persons, with respect to lifestyle parameters, presence of lymphadenopathy and isolation of cytomegalovirus. However, they had significantly lower CD4+ cells and CD4/CD8 ratio (p less than 0.01) at inclusion. It is concluded that in the majority of persons infected with HIV, phenotypic T-cell alterations will occur with a latency of years, but it remains to be seen if the alterations necessarily will result in clinical manifestations. Further, T-cell subset determination among healthy HIV Ab positive persons will provide prognostic information.     

Clinical manifestations of acute infection with human immunodeficiency virus in a cohort of gay men

During a prospective study of the natural history of human immunodeficiency virus (HIV) infection in a cohort of gay/bisexual men, information on self-reported symptoms lasting for 3 or more days during the previous 6 months was collected without knowledge of the subject's HIV serological status. Twenty-two people were retrospectively found to have seroconverted to HIV during the interval. Each seroconverter was matched to two seronegative and two seropositive controls. Matched case-control analyses using the seronegative controls determined that the following symptoms lasting for 3 or more days were associated with new HIV infection: fever greater than 37.7 degrees C, swollen lymph nodes, night sweats and headaches. Matched case-control analyses using the seropositive controls determined that the following symptoms lasting for 3 or more days were associated with new HIV infection: fatigue, fever greater than 37.7 degrees C, swollen lymph nodes, night sweats and headaches. It was notable that the majority of seroconversions were not associated with any symptoms lasting for 3 or more days. Due to their non-specificity, symptoms associated with seroconversion are not likely to have a high positive predictive value. In high risk populations, however, appearance of these symptoms may facilitate identification of early infection that may be important for studies of natural history or for optimal timing for initiating antiviral therapy.     

The impact of HIV antibody status on gay men's partner preferences: a community perspective.

As more gay men are tested for antibodies to human immunodeficiency virus (HIV), serostatus may influence the formation of primary partner bonds in this community. We compared seropositive (n = 157), seronegative men (n = 205), and those who had not been tested (n = 158) from our ongoing AIDS Behavioral Research Project (total response in 1988 = 540). Subjects responded to mailed surveys regarding sexual behavior, relationship status, HIV antibody testing and serostatus preference when forming relationships for romance and friendship. Sixty-eight percent of seropositive gay male respondents reported no serostatus preference in partners for romance, while 83% of seronegative respondents and 74% of untested respondents preferred seronegative partners for romance. In addition, 15% of seronegative respondents and 12% of untested respondents preferred seronegative individuals for friendship. Seropositive individuals were much less likely to be desired for romance or friendship by seronegatives and those who have not been tested--at a time when emotional support and companionship are obviously needed.     

Close relationships and safer sex among HIV-infected men with haemophilia

The present study sought to inform future behavioural intervention efforts by obtaining information from HIV-positive heterosexual men with haemophilia about their attitudes towards close relationships, attitudes towards risk reduction practices, and actual risk reduction practices. HIVinfected males with haemophilia ( n = 358) responded to a self-administered questionnaire. Men who reported being involved in a close relationship ( n = 237) were compared to men who said that they were not involved at the time of data collection ( n = 121). Involved men were more likely than uninvolved men to agree that close relationships provide benefits such as physical intimacy and communication, and that these benefits are important. Men who were not involved perceived more negative consequences of discussing HIV risk reduction with partners (including partner rejection and negative emotional reactions) than did involved men and were much more concerned about the potential negative consequences of risk reduction discussions. Involvement was associated with having disclosed HIV-seropositivity and having discussed HIV risk reduction. Risk reduction interventions for men with haemophilia who are not involved in close, sexual relationships should address positive and negative attitudes towards close relationships and towards discussing risk reduction. Interventions should emphasize communication skills and rehearsal of serostatus disclosure as well as of risk reduction discussions.     

The role of disease severity in influencing body mass index in people with haemophilia: a single‐institutional cross‐sectional study

The aim of this study was to evaluate the effect of haemophilia disease severity and potential intermediaries on body mass index (BMI) in patients with haemophilia. A secondary analysis of a cross‐sectional study of 88 adults with haemophilia was undertaken. On bivariate analysis, persons with severe haemophilia had 9.8% lower BMI (95% CI ?17.1, ?3.0) than persons with non‐severe haemophilia. The effect of haemophilia severity on BMI varied significantly by human immunodeficiency virus (HIV) status. Among HIV‐positive subjects, haemophilia severity was not associated with BMI (+5.0%, 95% CI ?22.4, 41.9). Among HIV‐negative subjects, severe haemophilia was associated with 15.1% lower BMI (95% CI, ?23.6, ?5.7). Older (>41 years) HIV‐negative subjects with severe haemophilia had a BMI that was 24.8% lower (95% CI ?39.1, ?7.0) than those with non‐severe haemophilia. No statistically significant association was detected between BMI and severe vs. non‐severe haemophilia for younger HIV‐negative subjects. Although joint disease, as measured by the World Federation of Hemophilia (WFH) joint score, did not influence the association between haemophilia disease severity and BMI, adjustment for the atrophy component of the WFH score reduced the association between haemophilia severity and BMI by 39.1–69.9%. This suggested that muscle atrophy mediated at least part of the relationship between haemophilia severity and BMI. Haemophilia disease severity is associated with BMI and appears to be mediated by muscle atrophy of surrounding joints. This association appears to be modified by HIV status and possibly age.     

Correlates of requesting home HIV self-testing kits on online social networks among African-American and Latino men who have sex with men

High levels of HIV stigma are one of the main difficulties in engaging African-American and Latino men who have sex with men (MSM) in HIV testing. The availability of home HIV test and the possibility of self-testing in private may improve uptake and counteract stigma. This paper sought to determine the correlates of requesting home HIV test kits among a sample of MSM social media users. The odds of participants requesting a test kit were significantly associated with using social networks to seek sexual partners (aOR: 2.47, 95% CI: 1.07–6.06) and thinking it is easier to use social networks for seeking sexual partners (1.87, 1.2–3.12), uncertain HIV status (4.29, 1.37–14.4), and having sex under the influence of alcohol (2.46, 1.06–5.77). Participants who had not been tested for more than 6 months were more likely to request a test kit than those who were tested in the past 6 months (2.53, 1.02–6.37). Participants who frequently talked to others about having sex with men online were less likely to request a test kit (0.73, 0.56–0.92). By reaching people over social media and offering them access to test kits, we were able to reach at-risk individuals who were uncertain about their HIV status and had not been regularly tested. The findings of the study will help to inform future HIV testing interventions.     

A longitudinal study of immunological status in Chinese haemophiliacs: importance of the heat viral inactivation of factor concentrates. I. Immunological associations with the consumption of factor concentrates

Summary . Twenty-four of 117 cases of haemophilia A (20.5%) and none of 18 cases of haemophilia B reported in this study had an antibody to the human immuno-deficiency virus (HIV). Both groups of patients showed similar immunological alterations. HIV-seropositive haemophilia A patients had an increased CD8 cell count and a similarly decreased CD4/CD8 ratio as compared to HIV-seronegative haemophilia A patients. Multiple regression analysis for the association of CD4/CD8 ratio with HIV infection status and dosage of plasma products in haemophilia A and B patients, respectively, revealed that there was a significant negative association of ln(CD4/CD8) with dosage of factor VlII concentrates (P = 0.0435) and factor IX concentrates (P = O.O028), respectively. N o association occurred between CD4/CD8 ratio and HIV infection as well as dosage of other plasma products. These data indicate that the immunological abnormalities of our haemophilia A and B patients in their early years were primarily caused by various viral infections and/or a suppressive effect of allogeneic protein through infusion of factor concentrates and not caused simply by HIV infection.     

Prevalence of human immunodeficiency virus type 1 DNA in hemophilic men and their sex partners. Hemophilia-AIDS Collaborative Study Group

Polymerase chain reaction (PCR) was used to detect human immunodeficiency virus (HIV)-1 DNA in peripheral blood mononuclear cells to assess in hemophilic men whether any were HIV-seropositive but uninfected or seronegative but infected and in seronegative sex partners of seropositive hemophilic men whether any were infected. Of 40 seropositive men, 38 (95%) were PCR-positive; one was PCR-indeterminate and one PCR-negative. None of 41 seronegative men who used only donor-screened, virus-inactivated coagulation factor products were PCR-positive. However, two of six who received noninactivated products were PCR-positive; one had low T-helper cell counts and died of unrelated causes and the other had seroconverted 11 mo later. PCR with a second primer pair also detected HIV-1 DNA in these two men. None of 25 seronegative female sex partners of seropositive men, including six men with AIDS and seven with AIDS-related symptoms, were PCR-positive. These data suggest that most seropositive hemophilic men are HIV-infected; whether some are infected with defective virus remains to be resolved as does the infection status of seropositive PCR-negative men. Identification of two seronegative PCR-positive men supports the possibility that HIV-1 DNA can be detected before seroconversion.     

Health-related quality of life assessment in adult haemophilia patients: a systematic review and evaluation of instruments

The objective of this study was to review and evaluate the performance of health-related quality of life (HRQL) and other health status measures used in studies of adult haemophilia and provide recommendations for future research. A systematic literature review was performed to identify HRQL and health status measures used in haemophilia. Literature was identified using medical databases, Internet and manual searches. The search was restricted to articles published in English since 1986. Ninety-six abstracts were located; 19 relevant articles were selected for detailed review. Three main types of HRQL measures were identified: generic psychometric-based HRQL (SF-36 and SF-12), utility-based HRQL [EQ-5D and Health Utilities Index (HUI)], and musculoskeletal-specific HRQL (Arthritis Impact Measurement Scale 2, AIMS 2) instruments. No patient-rated haemophilia-specific HRQL instrument was found. The SF-36, the EQ-5D, and the HUI were able to discriminate haemophilia patient subgroups with respect to disease severity and HIV comorbidity status. Sixteen additional scales were identified that were used to measure the different aspects of physical, psychological, and social functioning of patients. There were no clinical studies of haemophilia carried out that employed HRQL instruments, thus responsiveness of these instruments could not be evaluated. The variety of instruments used in haemophilia studies highlights the need for a tool that can capture the full impact of haemophilia and its treatment on patients' HRQL. Developing such a tool poses the unique challenge of accounting for common comorbidities, such as HIV and chronic hepatitis that may have a greater HRQL impact than the underlying disease.