Exploring the relationship between condition severity and health-related quality of life in people with haemophilia A across Europe: a multivariable analysis of data from the CHESS II study.

BACKGROUND: Haemophilia A (HA; Factor VIII deficiency) is a congenital X-linked bleeding disorder characterized by trauma-related or spontaneous bleeding events, most notably arising within the intraarticular space and resulting in chronic inflammation and degeneration of affected joints. Endogenous clotting factor activity relative to normal levels determines the severity of HA symptoms, as mild (> 5-40%), moderate (1-5%), or severe (< 1%). Within the current environment of rapid evolution in HA management, we seek to understand the interplay of condition severity and health-related quality of life (HRQoL) to characterise and differentiate unmet needs among people with HA (PwHA). METHODS: A generalised linear regression model (GLM) was developed to explore the relationship between HA severity and EQ-5D-5 L index score from adult HA patients sampled in the "Cost of Haemophilia across Europe - a Socioeconomic Survey II" (CHESS II) cross-sectional, retrospective burden of illness study among adults with hereditary haemophilia A or B from eight European countries. HA patients of any severity with no active inhibitors during the 12 months prior to data capture and a completeEQ-5D-5 L response were included. A base GLM model was specified with covariates for demographic and clinical characteristics (age, body mass index, country, employment, HA severity, annual bleeding rate, problem joints, and chronic pain). RESULTS: Of 381 evaluable patients, 221 (58.0%) had severe HA, 96 (25.2%) had moderate HA, and 64 (16.8%) had mild HA. Among the covariates included in the GLM model and after controlling for haemophilia-related outcomes, a significant association was observed between mild HA and higher EQ-5D-5 L index score (average marginal effects, 0.084; p = 0.016) relative to severe HA. Patient country of residence and magnitude of HA-related chronic pain were also associated with significant differences in index scores, with the latter showing a negative relationship with HRQoL outcomes. CONCLUSIONS: Condition severity and chronic pain are significant predictors of HRQoL in PwHA. Durable bleeding protection and effective management of chronic pain have the potential to address unmet treatment needs in this population.

Humanistic burden of haemophilia A without inhibitors: A cross-sectional analysis of the HemoLIFE study.

AIM: To evaluate the impact of haemophilia A without inhibitors on humanistic outcomes in patients and caregivers. Herein, we report a cross-sectional analysis of the baseline data of persons with haemophilia (PWH) participating in the prospective study HEMOLIFE. METHODS: These data are part of a prospective, observational, and multicentre study currently being conducted in 20 hospitals in Spain by haematologists. We included subjects 12 years or older diagnosed with haemophilia. The evaluations included the Maladjustment Scale, Haemophilia-Specific Quality of Life Questionnaire for Adults (HaemoQol)/HaemoQol Short Form (Children), haemophilia-specific version of the Work Productivity and Impairment Questionnaire plus the Classroom Impairment Questionnaire (WPAI+CIQ:HS), Haemophilia Activity List (HAL)/Paediatric Haemophilia Activities List (pedHAL), visual analogue scale (VAS) for evaluating pain, Coping Pain Questionnaire-Reduced (CAD-R), and Hospital Anxiety and Depression Scale (HADS). RESULTS: A total of 81 PWH were recruited at 18 centres; 66 PWH were ≥18 years (i.e., adults), and PWH 15 were <18 years (i.e., paediatric patients). Out of the 79 evaluable subjects, 16 (20%) showed an impact of haemophilia on daily life, and the areas most affected were "leisure time" (58% showed maladjustment) and "work/studies" (47% showed maladjustment). Patients reported a higher impact of haemophilia on quality of life (mean [SD] of the transformed score) in the dimensions of "sport" (49.4 [28.6]), "physical health" (40.5 [25.8]) and "future" (37.7 [28.9]). In adults, according to HAL scores, greater impairment of function was observed in "lying/sitting/kneeling/standing," "function of legs" and "leisure activities and sports," with mean normalized scores of 64.7, 65.1 and 69.0, respectively. Productivity was mostly impacted by presenteeism. The pain was infrequent and moderate. According to the HADS scores, nine (11.5%) patients had clinical anxiety and depression. CONCLUSION: PWH without inhibitors exhibited impairments in adjustment, quality of life and functionality, especially related to leisure and sports activities, and exhibit relevant levels of anxiety and depression.

A qualitative study on the perioperative experiences and demands of haemophilic arthropathy patients undergoing total joint replacement.

INTRODUCTION: Total joint replacement is the optimal treatment option for patients with severe haemophilic arthritis. Current research emphasizes patient-reported outcomes as a vital measure for evaluating surgical outcomes and patient satisfaction. Nevertheless, very limited information about the subjective experience of perioperative haemophiliacs in the literature, highlighting the need for exploration in this area. AIM: To investigate the psychological experiences and health demands of haemophilic arthropathy patients during the perioperative period of total joint replacement. DESIGN: Qualitative descriptive research with semistructured individual interviews. METHODS: From June to September 2023, nine patients with severe haemophilic arthropathy who underwent total joint replacement at a Haemophilia Diagnosis and Treatment Centre in China were interviewed for average 37 min per person. Data were analysed using the traditional content analysis method and reported following the consolidated criteria for reporting qualitative research. The study is reported according to the COREQ checklist. RESULTS: Interviews described two main themes: (1) emotional decline which involves preoperative overoptimism, early postoperative anxiety and disease uncertainty during the early independent rehabilitation. (2) wellness aspiration which includes rehabilitation support and spiritual healing. CONCLUSION: This study reveals the patients' significant psychological changes and their well-being aspiration, particularly out-of-hospital rehabilitation needs. Strengthening communication between multidisciplinary teams and patients, enhancing the involvement of nurses, broadening the scope of functions at primary Haemophilia Treatment Centres, and developing telerehabilitation, these concerted efforts may improve the overall treatment experience for patients.

Chain-mediating effects of kinesiophobia and self-efficacy on pain catastrophizing and physical activity in haemophilia patients.

BACKGROUND: There is a lack of research on the relationship between pain catastrophizing, kinesiophobia, and physical activity (PA) in people with haemophilia (PWH), and the underlying mechanisms connecting these variables remain unclear. AIM: The study's aim was to clarify the roles of kinesiophobia and self-efficacy in the relationship between pain catastrophizing and PA in PWH. METHODS: This cross-sectional study included adult PWH at the Haemophilia Centre of a Tertiary hospital in Beijing, China. The following questionnaires were used to collect data: the general information, the International Physical Activity Short Questionnaire, the Pain Catastrophizing Scale, the Tampa Scale of Kinesiophobia Scale, and the Exercise Self-Efficacy Scale. RESULTS: The study included a total of 187 PWH, including 154 having haemophilia A and 33 having haemophilia B. The median interquartile range of PA was 594 (198, 1554) MET-min/wk. There were significant differences in PA of patients based on age stage, treatment modality, highest pain score within the last seven days, and presence of haemophilic arthropathy (p < .05). It was showed that pain catastrophizing could directly predict PA (p < .001), accounting for 38.13% of the total effect. Pain catastrophizing also had indirect effects on PA through the mediating factors of kinesiophobia or self-efficacy, and through the chain-mediating effect of kinesiophobia and self-efficacy, accounting for 38.40%, 17.07%, and 6.40%, respectively. CONCLUSION: The study discovered that PWH have limited PA due to pain catastrophizing. This not only directly affects their activity but also indirectly influences it through kinesiophobia and self-efficacy.

Validity and reliability test of the Indonesian version of the PedHALshort questionnaire.

INTRODUCTION: The PedHAL questionnaire is employed in measuring the activities of haemophilia children. The PedHALshort was developed in 2022 to determine the most relevant item for the assessment. Haemo-QoL questionnaire assesses the quality of life of haemophilia children. AIM: Determine the validity and reliability of the PedHALshort Indonesia language version compared to the Haemo-QoL in the Indonesian population. METHODS: A cross-sectional study was conducted in Jakarta, Indonesia. The subjects were children (4-16 years old) with moderate or severe haemophilia A or B with repeated joint bleeding. The daily activity was assessed using the PedHALshorts and Haemo-QoL. The validity and reliability were tested. The validity was determined with Pearson correlation test, construct validity was assessed using convergent and discriminant validity. The Spearman correlation was used to determine which domains of Haemo-QoL correlated with PedHALshort. The reliability was analyzed using test-retest reliability. Cronbach's α was used to determined internal consistency. RESULTS: Fifty children were included in the study (78% haemophilia A, 70% severe group). All domains were valid (r > .284). The convergent validity test showed PedHALshort had a moderate negative correlation with physical health and sports school domain of Haemo-QoL (r = -.479, p < .001). The discriminant validity test showed the PedHALshort demonstrated a moderate negative correlation with physical health (r = -.585), view (r = -.590), sport school domain (r = -.430) in severe haemophilia subjects. The internal consistency of the PedHALshort questionnaire was high (Cronbach's α of .85 (.74-.92)). CONCLUSION: The PedHALshort is valid and reliable, hence, it can be used to measure the functional physical activity of children with haemophilia.

Promoting pain coping skills in haemophilia: A remote intervention integrating exercise and pain education.

BACKGROUND: Chronic joint pain is a significant and widespread symptom in people with haemophilia (PWH). Despite medical advancements, effective pain management remains challenging. AIM: This study presents an innovative approach that integrates remote physical exercises, pain neuroscience education, and coping strategies to address chronic pain in PWH. METHODS: The remote intervention consisted of sixteen 5-min videos encompassing physical exercises for chronic pain management and pain education strategies. These videos formed an 8-week remote intervention program. Clinical and physical assessments were conducted before and after the intervention. RESULTS: A total of thirty-one PWHs, with a median age of 34 years (ranging from 16 to 59 years), completed the remote intervention. The study revealed significant improvements in pain intensity, disability, and physical performance among PWH with chronic pain. Enhanced functional capacity was evident in the Timed Up and Go and Single Leg Stance tests, accompanied by improved scores on the Functional Independence Score in Haemophilia (FISH). Although lacking a control group, our findings are consistent with other successful exercise and pain education programs. CONCLUSIONS: This innovative intervention holds promise for managing chronic pain in PWH, underscoring patient empowerment, education, and collaboration. Notably, our study stands out by uniquely combining pain education and coping strategies, bolstering evidence for effective pain management.

Anxiety and depression among adults with haemophilia A: Patient and physician reported symptoms from the real-world European CHESS II study.

INTRODUCTION: The physical pain and disability affecting many people with haemophilia A (PwHA) are known detractors from psychological wellbeing. While psychosocial support is considered a core tenet of the haemophilia comprehensive care structure, the extent to which mental health challenges are detected and monitored by the individuals treating haematologist remains relatively unexplored. AIM: To describe prevalence of anxiety and depression in a real-world cohort of adult PwHA and evaluate the congruence in reporting of anxiety or depression (A/D) between PwHA and their treating physicians. METHODS: Data for PwHA without inhibitors was drawn from the European 'Cost of Haemophilia: A Socioeconomic Survey II' (CHESS II) study. Haematologist-indicated comorbidities of anxiety and depression were unified into a single A/D indicator. The EQ-5D-5L health status measure was used to characterise self-reported A/D, with individuals stratified into two non-mutually exclusive subgroups based on level of A/D reported (Subgroup A: 'some' or above; Subgroup B: 'moderate' or above). RESULT: Of 381 PwHA with evaluable EQ-5D-5L responses, 54% (n = 206) self-reported at least some A/D (Subgroup A) and 17% (n = 66) reported at least moderate A/D (Subgroup B). Patient-physician congruence in A/D reporting was 53% and 76% for Subgroups A and B, respectively. Descriptive analysis suggested that individuals with physician- and/or self-reported A/D experienced worse clinical outcomes (bleeding events, joint disease, chronic pain). CONCLUSION: While adverse clinical outcomes appear to correlate with A/D, self-reports of moderate-severe symptoms occasionally lacked formal recognition from treating physicians. Cross-disciplinary surveillance of mental health issues could improve both psychological and clinical outcomes among PwHA.

Depression and anxiety in patients with hemophilia A and B.

OBJECTIVE: This study described the prevalence of and correlates of depression and anxiety in adult patients with hemophilia A and B. METHODS: In this cross-sectional study, we investigated patients with hemophilia who were being seen at an adult hemophilia center in Turkey. Participants were screened for depression and anxiety during their annual clinic visit, which included administration of the Beck Depression Inventory and State-Trait Anxiety Scale. RESULTS: Of the 90 patients, 22 (24.4%) met criteria for significant depressive symptoms and 67 (74.4%) met criteria for significant anxiety symptoms. There were no significant associations between depression and any psychosocial or clinical characteristics or adherence to hemophilia prophylaxis, except for educational status. No association was found between state and trait anxiety scores and psychosocial and clinical characteristics other than hemophilia type. After adjusting for confounding factors, multivariable analysis showed that high school education level was associated with depression (OR: 1.87, CI: 1.31-2.36, P = .010) and type B hemophilia was associated with anxiety (OR: 1.32, CI: 1.09-1.75, P = .042). CONCLUSION: Depression and anxiety are major psychiatric comorbidities in patients with hemophilia in Turkey. Routine evaluation for mood and anxiety disorders are important in the routine care of patients with haemophilia.

Prevalence and perceptions of pain in people with haemophilia: A UK study.

INTRODUCTION: Joint bleeds in haemophilia cause destruction of articular structures, impaired function and pain. Up to 70% of people with haemophilia (PWH) report chronic pain. Little is known about the pain experiences in PWH in the UK. AIM: To identify prevalence and perceptions of pain among PWH living in the UK. METHODS: A cross-sectional, non-interventional survey study conducted among PWH (all severities). The survey incorporated elements from validated tools (EQ-5D; EQ-VAS) and was distributed via participating treatment centres. RESULTS: Five hundred and ninety-nine PWH responded, 91% aged > 18. 81% used factor prophylactically or on demand. More pain was reported by those treated on demand versus prophylaxis particularly in those who reported daily pain. 65% reported 'problem joints' based on individual impact rather than medically defined 'target joints', 2/3 reported multiple joint issues. The ankle was most commonly affected. 59% reported frequent pain, with 56% aware of pain constantly or most of the time and were more likely to report less favourable EQ-5D or EQ-VAS scores (p < .001). Pain frequency/awareness was consistent across all severities. Most discussed pain with care teams, 31% only when asked; 25% did not discuss it. Pain discussions resulted in physiotherapy referral (63%) analgesia prescription (48%), and a minority specialist pain referral (9%). Most felt well supported with regard to their pain, but 70% reported learning to live with it. CONCLUSION: Pain affects PWH of all ages and severities even in a well-resourced country significantly impacting quality of life. Clinicians must be more aware of chronic pain in PWH. Biopsychosocial approaches to pain assessment and management are recommended.

The lived experience of mothers of children with haemophilia in Jordan: A phenomenological study.

BACKGROUND: Haemophilia is an inherited bleeding disorder. Mothers of children with haemophilia experience stress, anxiety and different types of burdens that affect their life negatively. OBJECTIVES: This study aimed to explore the lived experience of mothers of children with haemophilia. METHODS: A descriptive phenomenological design was utilized. The participants were selected purposively from the Jordanian Association for Thalassemia and Hemophilia. Data saturation was achieved by interviewing 20 mothers. RESULTS: Five themes emerged: (1) the challenges related to the diagnosis, the availability and administration of the clotting factors, and the occurrence of the bleeding emergency; (2) physical, social, psychological and financial burden; (3) fear of child death and disability; (4) stigmatization; and (5) lack of educational and medical support. CONCLUSION: Mothers of children with haemophilia suffer from physical, psychological and social consequences. Healthcare providers should carry out educational sessions regarding the importance of support for the family and throughout the life of the child.

HRQoL and psychosocial aspects of burden on caregivers to children with moderate or severe von Willebrand disease.

INTRODUCTION: Von Willebrand disease (VWD) is the most widespread congenital bleeding disorder. Caregivers are highly involved in its treatment, and from the time of the child's bleeding diagnosis, they face new demands such as recognition of bleeds and treatment options. AIM: The aim of this study was to assess Health related quality of life (HRQoL) in caregivers of children with moderate and severe VWD in Sweden, and to describe the impact of psychosocial aspects on the burden. METHODS: A multicentre, cross-sectional study. The Short Form 36 Health Survey (SF-36) was used to assess HRQoL. Caregiver burden was measured using The HEMOphilia associated Caregiver Burden scale (HEMOCAB). Children´s clinical data were collected from the Swedish national registry for bleeding disorders. RESULTS: Seventy caregivers of children with moderate or severe VWD were included. Caregivers of children with moderate VWD scored significantly lower in the mental health domains on SF-36, compared to matched normative data. Psychosocial aspects that significantly impacted the caregiver burden negatively measured by HEMOCAB total score were: if the caregiver reported that VWD affected their life in general (p = .001), if the child was absent from preschool/school ≥2 day/12 months due to VWD (p = .002) or that VWD had a financial impact on the family (p = .001). CONCLUSION: This study contributes to knowledge about caregivers' HRQoL and highlights the situation of caregivers of children with moderate VWD. Furthermore, the caregiver burden was negatively affected by psychosocial aspects. Clinical follow-ups should include assessment of psychosocial aspects to identify caregivers that are at risk of high burden.

Chronic pain in haemophilia: assessment and analgesic treatment.

People with haemophilia tend to experience pain from an early age because of venipuncture and hemarthrosis. If pain is not properly managed, it can become chronic and bedevil patients throughout their lives. Therapies are currently available that have been shown to effectively treat the different types of pain and their causes. Patients with haemophilia tend to experience either nociceptive or mixed pain. Identification of the cause of pain by means of imaging techniques, and understanding the characteristics, location and intensity of the pain, are essential for a more targeted therapeutic approach. Current data reveal that the most effective measures are administration of haematological and analgesic medication, intraarticular injections, and physical exercise. However, multimodal strategies such as lifestyle changes, physical modalities, psychological support and orthopaedic surgery can also prove of use. This article will look at the most effective analgesic measures used as a part of conservative clinical treatment. Collaborative studies are needed to better understand the nature of pain in the context of haemophilia.

The emotional experience of mothers of children with haemophilia: maternal guilt, effective coping strategies and resilience within the haemophilia community.

INTRODUCTION: Mothers of children with haemophilia (CWH) experience guilt related to this genetic condition. Several factors contributing to maternal guilt have been identified, but the scope and extent of guilt have not previously been quantified. AIM: This study provides insight into the experience of mothers of CWH and how they perceive and manage guilt. It then identifies the most common and helpful coping mechanisms. METHODS: Between May and October 2021, we distributed an anonymous electronic survey to mothers of CWH. The Parent Experience of Child Illness measured maternal guilt, the PROMIS Parent Proxy for Life Satisfaction measured perception of their child's life satisfaction and additional questions explored specific guilt factors and coping strategies. RESULTS: Eighty-seven mothers responded to the survey. Forty percent of mothers experienced increased guilt. The most common reasons for guilt included putting their child through pain during infusions and passing on the affected X chromosome. Perceived life satisfaction, increased age and genetic counselling were associated with less guilt. The most common coping strategies involved utilizing social support, self-education and connecting with other mothers in the community. CONCLUSION: Some mothers experienced increased feelings of guilt, illustrating the need for providers to tactfully provide anticipatory guidance and counselling. Tangible manifestations of haemophilia were more likely to trigger feelings of guilt than familial factors. Community immersion was beneficial, as other mothers in the community served as a source of social and educational support. Most mothers did not report guilt, illustrating the adaptability and resilience of the haemophilia community.

Psychological complications among patients with congenital bleeding disorders.

Congenital bleeding disorders (CBDs), including inherited platelet function disorders and rare and common bleeding disorders, are a heterogeneous group of bleeding disorders with a wide range of clinical presentations, including psychological complications. Due to the chronic nature of CBDs, psychological complications are relatively common in these patients, which can affect treatment adherence, quality of life, and even the frequency of bleeding episodes. Chronic pain causes many psychological problems in CBDs, disrupting their social interactions, affecting all aspects of their lives, including their emotional functioning and behavior, and eventually leading to social exclusion. About one-third of patients with severe hemophilia A suffer from anxiety, 64% from depression, and 60% from other minor psychological complications such as withdrawal/depression, anxiety/depression, attention problems, and emotional problems. Anxiety, depression, and uncontrolled pain interfere with treatment adherence. For this reason, psychological interventions are needed in people with CBDs. Psychological interventions increase quality of life and treatment adherence. Therefore, early recognition of psychological complications in CBD patients may increase treatment adherence, leading to a reduction in bleeding episodes and thus an improvement in quality of life.

Impacto da hemofilia na qualidade de vida em relação à saúde de pacientes acompanhados pelo hemocentro de Alagoas / Impact of hemophilia on health-related quality of life of patients accompanied by the blood center of Alagoas

A qualidade de vida é um aspecto importante a ser considerado no plano terapêutico de qualquer paciente, principalmente aqueles que sofrem de doenças crônicas, como a hemofilia. Diante disso, esse estudo teve como objetivo avaliar a qualidade de vida em pacientes portadores de hemofilia atendidos pelo Hemocentro de Alagoas e descrever o perfil demográfico e socioeconômico. Foram 50 pacientes envolvidos na pesquisa, maiores de dezoito anos e portadores de hemofilia acolhidos pela associação e pelo hemocentro de Alagoas. A coleta de dados foi realizada por entrevistas utilizando-se de dois questionários: um sobre características demográficas e socioeconômicas e outro sobre avaliação da qualidade de vida relacionada à saúde (Haem-A-QoL). Desse total, 100% eram do sexo masculino, em que a maioria tinha idade entre 20 e 40 anos (80%), solteiro (62%), residentes no interior de Alagoas (58%), aposentado (56%), beneficiários da aposentadoria por complicação da hemofilia (60%), com renda pessoal no último mês de até um salário mínimo (78%) e sem plano de saúde privado (86%). A média do escore total do Haem-A-QoL foi 40,08 (variação de 0­96,46) com pior desempenho nos domínios "esporte e lazer" (média igual a 55,52) e "saúde física" (média igual a 50,16) e melhor nos campos de "relacionamentos e sexualidade" (média igual a 17,48). De modo geral, os hemofílicos do centro estudado apresentaram boa QVRS. A versão brasileira do Haem-A-QoL, demostrou ser um instrumento confiável, com boa consistência interna, revelando alguns problemas enfrentados pelos hemofílicos e que impactam na QVRS (AU)

Quality of life is a critical aspect in the therapeutic plan of any patient, especially those who suffer from chronic diseases, such as hemophilia. Thus, this study aimed to assess the quality of life in patients with hemophilia treated by the Blood Center of Alagoas and describe the demographic and socioeconomic profile. 50 individuals were enrolled in the study, being over 18 years old and with hemophilia treated by the Association and the Blood Center of Alagoas. Data collection was carried out through interviews using two questionnaires: one on demographic and socioeconomic characteristics, and the other on health-related quality of life (Haem-A-QoL). Of this total, 100% were male, most aged between 20 and 40 years (80%), single (62%), living in the interior of Alagoas (58%), retired (56%), beneficiaries of retirement due to hemophilia complications (60%), with personal income in the last month of up to one minimum wage (78%) and without a private health plan (86%). The mean of the total Haem-A-QoL score was 40.08 (ranging from 0-96.46), with the worst performance in the domains "sports and leisure" (mean of 55.52) and "physical health" (mean of 50.16), and the best performance in the domain "partnership and sexuality" (mean of 17.48). In general, the hemophiliacs in the center studied reported good health-related quality of life. The Brazilian version of Haem-A-QoL proved to be a reliable instrument, with good internal consistency, revealing some problems faced by hemophiliacs that impact on HRQoL

Acquired haemophilia A in a patient who is a Jehovah's Witness.

We detail the case of a man in his 80s who was a Jehovah's Witness, presenting to hospital for the second time in 1 week with atraumatic, severe bruising affecting his right thigh and flank. He was subsequently diagnosed with idiopathic acquired haemophilia A (AHA) and was urgently treated with recombinant factor concentrate and immunosuppressive therapy. Management of his bleeding disorder and resultant severe anaemia was adapted in line with his religious beliefs. AHA is a rare bleeding disorder which should be considered in patients with an isolated prolonged activated partial thromboplastin time and a history of recent or acute bleeding. Prompt diagnosis and management are essential as delays may result in increased mortality. Given that this patient declined blood transfusion, management of his bleeding disorder presented a unique challenge.

Calidad de vida relacionada con la salud de personas con hemofilia en el noreste de Brasil / Quality of life related to the health of people with hemophilia in Northeast Brazil

Introducción: La hemofilia se caracteriza por la ocurrencia de hemorragias frecuentes y afecciones del aparato locomotor graves en los pacientes que causan daños físicos, sociales y psicológicos y afecta directamente su calidad de vida relacionada con la salud. Objetivo: Evaluar la calidad de vida de personas con hemofilia en el noreste de Brasil. Métodos: Estudio epidemiológico transversal cuantitativo realizado con 13 personas con hemofilia de un Banco de Sangre en el noreste de Brasil. La recolección de datos se realizó mediante la aplicación de un cuestionario sociodemográfico y clínico y de Haem-A-Qol, utilizado para evaluar la calidad de vida de las personas con hemofilia. Los valores brutos y medios se calcularon en general para todos los dominios y luego para cada dominio para que pudieran mostrarse. Resultados: Se obtuvo un promedio total de 30,94 en relación a los diez dominios evaluados. Los dominios con los promedios más altos fueron "salud física" con 49,23 y "afrontamiento" con 35,89 y con el promedio más bajo fue "planificación familiar". Conclusiones: Los participantes de la investigación tienen una calidad de vida insatisfactoria en relación con la salud física y el afrontamiento y, satisfactoria en relación con las relaciones y la sexualidad(AU)

Introduction: Hemophilia is characterized by the occurrence of frequent bleeding and serious musculoskeletal conditions in patients that cause physical, social and psychological damage and directly affect their health-related quality of life. Objective: To evaluate the quality of life of people with hemophilia in the northeast of Brazil. Methods: Quantitative cross-sectional epidemiological study conducted with 13 people with hemophilia from a Blood Bank in northeastern Brazil. Data collection was performed by applying a sociodemographic and clinical questionnaire and Haem-A-Qol, used to assess the quality of life of people with hemophilia. Raw and mean values were calculated overall for all domains and then for each domain so that they could be displayed. Results: A total average of 30,94 was obtained in relation to the ten domains evaluated. The domains with the highest averages were "physical health" with 49,23 and "coping" with 35,89 and with the lowest average was "family planning". Conclusions: The research participants have an unsatisfactory quality of life in relation to physical health and coping, and satisfactory in relation to relationships and sexuality(AU)