Sports participation and physical activity in patients with von Willebrand disease.

INTRODUCTION: Patients with bleeding disorders may experience limitations in sports participation and physical activity. Several studies on sports participation have been performed in haemophilia patients, but studies in patients with von Willebrand disease (VWD) are lacking. AIM: We assessed the sports participation and physical activity of a large cohort of VWD patients. METHODS: Patients were included from the "WiN study." All patients completed a questionnaire on sports participation, physical activity, quality of life and bleeding symptoms (Tosetto bleeding score). RESULTS: From the 798 included patients, 474 had type 1, 301 type 2 and 23 type 3 VWD. The mean age was 39 ± 20 (standard deviation) years. Five hundred and fifty-two patients (69.3%) participated in various types of sports. Type 3 VWD patients more often did not participate in sports due to fear of bleeding and physical impairment, respectively, OR = 13.24 (95% CI: 2.45-71.53) and OR = 5.90 (95% CI: 1.77-19.72). Patients who did not participate in sports due to physical impairment had a higher bleeding score item for joint bleeds 1.0 (±1.6) vs 0.5 (± 1.1) (P = 0.036). Patients with type 3 VWD and patients with a higher bleeding score frequently had severe limitations during daily activities, respectively, OR = 9.84 (95% CI: 2.83-34.24) and OR = 1.08 (95% CI: 1.04-1.12). CONCLUSION: The majority of VWD patients participated in sports. Patients with type 3 VWD, a history of joint bleeds and a more severe bleeding phenotype frequently experienced limitations in sports participation and physical activities during daily life.

Patient, caregiver, and provider perceptions of pain and pain management in adolescents and young adults with bleeding disorders.

INTRODUCTION: Recurrent bleeding and associated pain are critical components in the management of bleeding disorders, yet scant data describe perceptions of pain in this patient population. OBJECTIVE: This study assessed perceptions of pain and pain management in adolescents and young adults (AYAs) with haemophilia or von Willebrand disease (VWD) to determine agreement/disagreement between patients, caregivers and health care providers. METHODS: Using an online questionnaire, AYA patients (N=89), their caregivers (N=77), and providers (N=54) reported on pain perception, pain treatment and pain control. Acute and chronic pain was measured in patients via the Faces Pain Scale-Revised (FPS-R). Questionnaires queried about pharmacologic and non-pharmacologic pain management methods and how well providers and caregivers helped to manage pain. RESULTS: Poor agreement existed between patients and caregivers across all pain levels, perception of pain control and effectiveness of pain management. Specifically for chronic pain, poor agreement was noted between patients and caregivers (kappa=0.04; 29% agreement) and patients and providers (kappa=-0.07; 21.4% agreement). Among patients reporting acute or chronic pain, only 67% and 43%, respectively, utilized medication for their specific pain. Patients used more opioid medications than expected by their providers. On average, AYAs reported initial use of pain medications for chronic pain at 11.5 years. CONCLUSIONS: Ongoing research is needed in haemophilia and VWD pain management, and on the differences in pain perception between patients, caregivers and providers. As chronic pain often begins at an early age, optimal pain management should include acknowledging patient complaints, exploring pharmacologic and non-pharmacologic options, and optimizing prophylaxis.

Achieving self-management of prophylactic treatment in adolescents: The case of haemophilia.

OBJECTIVE: Adolescents with a chronic disorder, such as haemophilia, need to attain responsibility for their disease. The aim was to gain insight into how adolescents achieve self-management of prophylactic treatment. METHODS: In three Dutch Haemophilia Treatment Centres, adolescents (10-25 years) received structured questions on treatment responsibility and self-management (pre-specified definitions) during routine nursing consultation. RESULTS: In total, 155 interviews were performed in 100 patients (median age 14.4 years). Self-infusion was initiated at a median age of 12.3 years (IQR 11.5-13.0) and self-management was achieved 9.6 years later, at a median age of 22.6 years. This process included three phases coinciding with known stages of adolescence. In early adolescence, patients acquired the technique of self-infusion (12.3 years) leading to independent self-infusion in middle adolescence (17.2 years). In late adolescence, patients demonstrated an increase in more complex skills, such as bleeding management and communication with the haemophilia physician (19.9-22.6 years). CONCLUSION: Although, the first steps in self-management with regard to self-infusion are taken in early adolescence, complete self-management was achieved in late adolescence after almost 10 years. PRACTICE IMPLICATIONS: Insight in this transitional process helps to provide individualized support and emphasizes the need for continued education with regard to self-management skills.

The impact of social factors on outcomes in patients with bleeding disorders.

INTRODUCTION: In haemophilia, clinical outcomes are mainly determined by the severity of clotting factor deficiency, treatment regimen, availability of clotting factor concentrate and age. Information about the relevance of patient-related factors such as education, social status or impact of the disease on the patient's life is scarce. AIM: To assess the impact of social status and disease-related impairment of certain aspects of the patient's life on clinical and psychosocial outcomes in patients with inherited bleeding disorders (PWBD). METHODS: Consecutive patients of a single centre were assessed by questionnaires on social status and quality of life (SF-36). Social status was defined by school and professional education, employment and financial income of patients as well as school education of their parents. RESULTS: Fifty-seven PWBD (mean age, 38 ± 16 years) were enrolled, 60% were treated on-demand; PWBD had a median number of 2.5 (0-34) annual bleeds and a median orthopaedic joint score of 6 (0-38). No significant differences were found for clinical and psychosocial outcomes across social status groups. More than half of the patients reported that haemophilia had an impact on their school education, childhood and leisure activities. Patients with a high impact of haemophilia on their lives were less satisfied with their lives (P < 0.002), reported worse quality of life in all domains of the SF-36, had a worse joint score (P < 0.024) and reported more pain (P < 0.013). CONCLUSION: The perceived impact of haemophilia on patients' lives seems to have a stronger impact on clinical and psychosocial outcomes than patients' actual social status.

Bleeding disorders, menorrhagia and iron deficiency: impacts on health-related quality of life.

von Willebrand disease (VWD) is a bleeding disorder that occurs in up to 1% of the general population. The great majority of females with VWD experience menorrhagia. The morbidity burden in females with VWD may relate to iron deficiency resulting from menorrhagia. To explore relationships between bleeding disorders, menorrhagia, iron deficiency and the outcomes of health-related quality of life (HRQL) and educational attainment. All subjects with VWD, and females with other bleeding disorders, in the Canadian national registry who were more than 12 years of age were eligible for survey. Survey measures included the HEALTH UTILITIES INDEX(®); abridged Clinical History Assessment Tool; socio-demographic questions and serum ferritin. Statistical analyses included testing differences among groups of means using analysis of variance and of proportions using chi-squared test. Significant size differences in mean HRQL scores were detected between VWD females and both females with other bleeding disorders [diff = (-0.08); P = 0.017] and VWD males [diff = (-0.07); P = 0.039]. Mean HRQL scores differed between females with and without menorrhagia (P < 0.001). Mean HRQL scores were not significantly different between females with and without iron deficiency. Educational attainment was not associated with disease group, menorrhagia status or iron status. Females with VWD have a greater morbidity burden than females in the general population, females with other bleeding disorders and males with VWD. Menorrhagia is associated with low HRQL scores in females with bleeding disorders, including VWD. Further investigation should assess how menorrhagia impacts HRQL in females with bleeding disorders.

Quality of life in women with bleeding disorders.

The assessment of health-related quality of life (HRQOL) has been increasingly used over the last years, is regarded one of the most relevant health outcome measures and is included as secondary and primary endpoint in clinical and observational trials. Bleeding disorders and their treatment impact on patients' HRQOL, especially in women with bleeding disorders and can affect the everyday life of patients and their families. In women with inherited bleeding disorders, menorrhagia is the most common symptom, manifest by significant bleeding and pain leading to limitation in conducting daily activities and changes in social functioning with an adverse effect on women's HRQOL. Only few studies used validated questionnaires for the assessment of HRQOL in women with bleeding disorders, mainly generic instruments. Few disease-specific instruments are available for the adequate assessment of the impact of menorrhagia on HRQOL, namely the Ruta Menorrhagia Severity Scale, the menorrhagia multi-attribute utility scale and the Menorrhagia Impact Questionnaire. The von Willebrand disease (VWD)-QOL questionnaire, a disease-specific questionnaire for patients with VWD contains a specific dimension 'menstruation' for women. These studies revealed that menorrhagia has a larger impact on HRQOL in women with inherited bleeding disorders compared with women with normal haemostasis. Moreover, age, type of VWD and gender have an influence on the HRQOL of patients with VWD. The need of disease-specific instruments for an adequate assessment of HRQOL in women with bleeding disorders could be demonstrated in these studies.

Impact of von Willebrand disease on health-related quality of life in a pediatric population.

BACKGROUND: Von Willebrand disease (VWD) is the most frequent inherited bleeding disorder. Whether VWD is associated with health-related quality of life (HR-QoL) in children is unknown. OBJECTIVES: This nationwide cross-sectional study measured HR-QoL in children with moderate or severe VWD. Our primary aim was to compare HR-QoL of VWD patients with that of reference populations. Additionally, we studied the impact of bleeding phenotype and VWD type on HR-QoL. METHODS: HR-QoL was assessed with the Infant/Toddler QoL Questionnaire (0-5 years) and Child Health Questionnaire (6-15 years), and compared with reference population scores. Multivariate analysis was used to evaluate the influence of type of VWD and bleeding phenotype on HR-QoL scores. RESULTS: Preschool children (0-5 years, n = 46) with VWD had lower HR-QoL scores for general health perceptions and parental time than reference populations. School children (6-15 years, n = 87) with VWD had lower scores for physical functioning, role functioning - emotional/behavioral, general health perceptions, and physical summary. Type of VWD was associated with HR-QoL in school children for bodily pain, general health perceptions, parental emotion, family activities, and physical summary. Scores of children with type 3 VWD were, on average, 15 points lower than those of the reference population on the above-mentioned scales. A more severe bleeding phenotype was associated with a lower score on 11/15 physical, emotional and social scales. CONCLUSION: HR-QoL is lower in VWD children than in reference populations, in particular in school children. The negative impact of VWD is sensitive to type of VWD and bleeding phenotype; as well as physical scales, emotional and social scales are affected.

Health-related quality of life among adult patients with moderate and severe von Willebrand disease.

SUMMARY BACKGROUND: von Willebrand Disease (VWD) is the most frequent inherited bleeding disorder. It is unknown how this disorder affects quality of life. OBJECTIVES: This nationwide multicenter cross-sectional study determined health-related quality of life (HR-QoL) in adult patients with moderate or severe VWD, and assessed whether bleeding severity and type of VWD are associated with HR-QoL. METHODS: HR-QoL was assessed using the Short Form (SF)-36, and bleeding severity was measured using the Bleeding Score (BS). RESULTS: Five hundred and nine patients participated; 192 males and 317 females, median age and range 45 (16-87) and 47 (16-84) years, respectively. Compared with the general population, HR-QoL in VWD patients was lower in the vitality domain (61 vs. 66 P < 0.001 for females, 67 vs. 72 P < 0.001 for males). Patients with the most severe bleeding phenotype (highest quartile BS, BS > 17) had a lower HR-QoL in eight domains than patients with a less severe bleeding type (lowest quartile BS, BS < 7) in the univariate analysis. After adjustment for age, gender, co-morbidity and employment/educational status, a more severe bleeding phenotype was associated with lower scores on the domains of physical functioning, role limitations due to physical functioning, bodily pain, general health, social functioning and physical component summary. CONCLUSIONS: HR-QoL is lower in VWD patients compared with the general population. HR-QoL is strongly associated with bleeding phenotype.

Living with haemophilia and von Willebrand's: a descriptive qualitative study.

OBJECTIVE: The aim of this qualitative descriptive study was to describe the experience of living with bleeding disorders and to identify the associated salient issues from the perspectives of people living with haemophilia or von Willebrand's Disease (vWD). METHODS: Nine members of The Haemophilia Society took part in a semi-structured interview. The interviews were tape recorded and transcribed and the results subjected to thematic content analysis. RESULTS: Participants described the physical aspects of their condition, such as bleeding into joints, the implications of bleeds and development of co-morbid conditions (e.g. arthritis). Many felt that their bleeding disorder had impacted on their education, work, social activities and family life. Anxiety and depression were associated with daily management of symptoms, the unpredictable nature of bleeding disorders and concerns about the future. All participants had encountered a degree of stigmatisation related to their condition and felt that there was a public misconception about bleeding disorders. Many had experienced discrimination in educational and work settings. Some participants expressed a preference for seeking treatment from specialist services. CONCLUSION: Living with haemophilia and vWD poses a number of daily and longer-term challenges not only for individuals, but also for their families. Further investigation of ways to support such individuals is warranted. PRACTICE IMPLICATIONS: Greater awareness and understanding of the issues faced by people living with haemophilia and vWD is needed for the general public, employers, and healthcare staff, particularly those working in Accident and Emergency Departments.

Health status and health-related quality of life associated with von Willebrand disease.

Von Willebrand disease (VWD) is the commonest inherited disorder of hemostasis and the majority of women with this disorder experience excessive uterine bleeding. Yet very little information is available on the health-related quality of life (HRQL) in individuals with VWD. To test the a priori hypotheses that these individuals will have poorer HRQL than members of the general population, and that this burden of morbidity will correlate with the severity of VWD, a cross-sectional study was undertaken of a population-based cohort in a regional hemophilia program in Ontario, Canada. A survey was made of individuals over 13 years of age with VWD who self-reported their health status using a standard 15 item questionnaire. The responses were converted to levels in the Health Utilities Index Mark 2 (HUI2) and Mark 3 (HUI3) health status classification systems to form multi-element vectors from which single attribute morbidity and overall HRQL utility scores were determined. As a group, individuals with VWD were shown to have poorer HRQL than members of the general population and those with Type 2 disease carried a greater burden of overall morbidity than those with Type 1 disorder. Morbidity was evident mainly in the attributes of emotion, cognition with pain. A striking difference was observed between males and females, with the latter having overall HRQL utility scores similar to those reported previously for HIV positive, severe hemophiliacs. It is possible that this remarkable burden of morbidity reflects chronic iron deficiency associated with menorrhagia. A national study has been proposed to address this likelihood as it offers an opportunity for effective therapeutic intervention (iron supplementation) with a concomitant gain in health status and HRQL.

Medical, reproductive and psychosocial experiences of women diagnosed with von Willebrand's disease receiving care in haemophilia treatment centres: a case-control study.

OBJECTIVE: To assess the medical, gynaecological and reproductive experiences of women with von Willebrand's disease (VWD) and to evaluate the impact of VWD on mental health and life activities. METHODS: A total of 102 women with VWD who were registered in haemophilia treatment Centres (HTCs) in the United States and 88 controls were interviewed regarding medical, gynaecological and reproductive history, life activities and symptoms of depression. Symptoms of depression were measured using the Center for Epidemiological Studies Depression Scale (CES-D). RESULTS: Excessive bleeding symptoms were reported in 74% of VWD cases compared with 6% of controls. Women with VWD had a higher prevalence of menorrhagia, excessive postpartum bleeding, other gynaecological conditions, arthritis and migraine headaches than did controls. More VWD cases than controls reported that menstruation had a negative impact on overall life activities. No difference in the prevalence of depression was found between cases and controls. DISCUSSION: Women with VWD experience menorrhagia and other gynaecological conditions at a higher frequency than women without bleeding disorders. Menstruation in women with VWD has a negative impact on life activities. The prevalence of depression was not elevated in this group of women whose VWD is being managed in an HTC.

Percepçäo subjetiva de enfermidade na infecçäo por hiv e aids: um estudo comparativo entre pacientes HIV+ com coagulopatias congênitas e sem elas / Subjective perception of illness in the HIV infection and AIDS: a comparative study of HIV+ patients with congenital coagulopathy and without congenital coagulopathy

A AIDS é uma doença que ameaça a sobrevivência das pessoas infectadas pelo HIV. O modo como uma pessoa HIV+ percebe a gravidade da infecçäo e a forma de adaptaçäo ou enfrentamento que utiliza, pode determinar o bem-estar e a qualidade de vida percebida. A partir do conceito de percepçäo subjetiva de enfermidade (PSE), realiza-se um estudo comparativo-descritivo entre dois grupos de pacientes HIV+, um com coagulopatias congênitas (hemofilia ou Von Willebrand) e outro sem ela. Säo utilizadas medidas de PSE, nível de ansiedade e variáveis clínicas e imunológicas. Os resultados indicam que os grupos apresentam percepçöes e níveis de ansiedade diferentes. Foram encontradas correlaçöes entre PSE, ansiedade e estados clínico e imunológico. Sugere-se complementar o tratamento farmacológico destes pacientes com programas psicoeducativos dirigidos a modificar sua percepçäo subjetiva da doença, a fim de melhorar o bem-estar e a qualidade de vida dos mesmos

Coping strategies, pain, and disability in patients with hemophilia and related disorders.

OBJECTIVE: To analyze the use of various coping strategies in homogeneous groups of patients with hemophilia and von Willebrand's disease and to investigate the relationship between the state of the disease, the use of coping strategies, and management of the disease. METHODS: The coping strategies measured by the Coping Strategies Questionnaire were analyzed in 3 homogeneous groups of 224 patients. Psychosocial well-being (PWB) measured by the Rand 36-item Health Survey 1.0 was used as an indicator of management of the disease. The pain factor consisted of the following variables: pain intensity, use of analgesics, Functional Disability Index, and physical activity level. RESULTS: The groups of patients differed significantly only in the use of the catastrophizing strategy (CAT). In all pain groups, distraction was the most commonly used coping strategy. A significant interaction effect of pain factor and age on PWB (P = 0.04) was found. The mediating function of the CAT strategy was confirmed by the series of regression analyses. CONCLUSION: The coping strategy profile in hemophilia was found to be similar to those in other chronic pain states. The use of the strategies does not depend on the severity of the disease. We confirmed the role of age and the use of the CAT strategy as, respectively, moderator and mediator in the pattern of relationships between the clinical state of the disease and psychosocial well-being.

Clinical severity of disease, functional disability and health-related quality of life. Three-year follow-up study of 150 Finnish patients with coagulation disorders.

A questionnaire-based follow-up study of 150 Finnish patients with coagulation disorders was conducted to evaluate the changes in clinical manifestation of the disease, pain intensity, functional disability and patient's health-related quality of life, occurring during a 3-year period. Health-related quality of life was assessed using the MOS-36 health survey. The clinical severity of the disease manifestation was estimated using the frequency of bleeding episodes into joints and soft tissue during the previous 12 months. Severity of musculoskeletal morbidity, which reflects simultaneous presence of pain and disability, was estimated using intensity of pain and level of disability. Results showed that clinical severity of the disease remained unchanged during the 3-year period for the majority of patients. Nonetheless, the annual number of bleeds in patients with severe or moderately severe haemophilia was still disconcertingly high and the number of bleeding episodes into soft tissue had increased. The clinical severity of the disease manifestation, rather than the severity of the disease based on molecular classification, was a factor that negatively influenced the intensity of pain (both acute and chronic), as well as level of disability. Severity of musculoskeletal morbidity at time 1 and changes in severity of musculoskeletal morbidity over time were found to be significant predictors of physical role, bodily pain, vitality and social functioning. In conclusion, the disease can be relatively well controlled by the availability of coagulation products, thus stabilizing the clinical severity of the disease manifestation during the 3-year period studied. The findings stress the importance of preventing haemorrhages to avoid resulting permanent joint impairment and disability.

Should hemophiliac patients be circumcised?

Social and cultural integration of hemophilic boys into society is one of the most important cornerstones of modern hemophilia therapy. Circumcision, a traditional procedure, is an important ritual for Muslims and Jews and an important social problem for the hemophiliac patient and his family. The aim of this study was to evaluate the psychosocial dimension of circumcision and the opinions of parents and children. A total of 105 hemophiliac patients and parents were interviewed and surveyed. Of these, 94% of the parents of uncircumcised patients wanted circumcision for their children. Most parents saw circumcision as a mandatory procedure. Hemophilic boys (60%) and their parents (82%) have an inferiority complex because the boys are unable to be circumcised. Bleeding risk is the primary reason of anxiety (70%). The parents of all the circumcised patients were happy after circumcision. In conclusion, circumcision is an important social problem of hemophilic patients that needs to be solved.

Quality of life during menstruation in patients with inherited bleeding disorders.

Menorrhagia is a common and major problem for patients with inherited bleeding disorders, especially vWD. Quality of life during menstruation was assessed in 99 patients with inherited bleeding disorders including vWD (n = 57), carriers of haemophilia A (n = 17), carriers of haemophilia B (n = 7) and FXI deficiency (n = 18), and comparison was performed with an age-matched control group (n = 69). A questionnaire was used that included four main sections: (i) general health, (ii) health and daily activities, (iii) dysmenorrhea and (iv) quality of life during the menstrual period. Although patients with inherited bleeding disorders felt that their health (in general) was very good, they had significantly poorer quality of life on all the scales used than controls. Thirty-nine per cent reported having cut down on the amount of time spent on work and other activities as a result of their menstruation; 47% felt that they accomplished less than they would like during this period, 38% felt that they were limited in the kind of work and other activities that they could do, and 40% found that it took extra effort to perform their work. Fifty-one per cent experienced moderate, severe or very severe dysmenorrhoea. Quality of life was statistically poorer in patients with vWD, menstrual scores > 100 according to the pictorial blood assessment chart (PBAC), those who had periods > or = 8 days and those who experienced flooding or passage of clots. In conclusion, menstruation has a negative effect on the quality of life in patients with inherited bleeding disorders especially in those with objectively confirmed menorrhagia.

A controlled study of anxiety and morbid cognitions at initial screening for human immunodeficiency virus (HIV) in a cohort of people with haemophilia.

AIM: This study examines the relationship between anxiety, psychological state and Human Immunodeficiency Virus (HIV) stages as defined by the Centers for Disease Control at the time of initial screening for HIV in a cohort of people with haemophilia who were at risk of prior exposure to HIV transmission from blood products. METHOD: Psychological scores, immunological measures, and clinical data from case notes for 116 potentially HIV exposed people with haemophilia attending initial screening for HIV infection in 1984-1985, were used to examine the relationship between psychological variables, clinical state and their clinical classification under the Centres for Disease Control categorization. Psychometric test results were obtained for 63 HIV seronegative patients and 53 HIV seropositive patients. Planned comparisons, multiple and logistic regressions, were used to explain observed differences between seronegative and seropositive subjects. The potential confounders of sex, age, severity of haemophilia, haemophilia type and blood product usage were controlled. RESULTS: The major finding of this study was that higher levels of State Anxiety at the time of initial screening for HIV, were observed in those patients who lacked recognized symptoms of HIV infection and were seropositive, compared with seronegative subjects. The State Anxiety scores were predicted by HIV infection or alternatively CD4+ T-cell levels. CONCLUSION: The findings of this study suggest that HIV infection can produce psychological effects prior to any physical symptoms of infection being apparent.