The relationship of self-compassion and hope with quality of life for individuals with bleeding disorders.

INTRODUCTION: Minimal research has been conducted examining the relationship of positive psychology variables with quality of life (QOL) for individuals with bleeding disorders. While many individuals manage their bleeding disorders well, some are at higher risk of developing psychosocial complications due to the daily stressors of managing illness-related symptoms. AIM: The purpose of this study is to better understand the relationships between two positive psychology variables, self-compassion and hope and QOL (overall, psychosocial and physical) among individuals diagnosed with bleeding disorders. METHODS: Participants completed a survey identifying demographic information as well as rating scales of self-compassion, hope and quality of life. We conducted Pearson correlational and standard multiple regression analyses to explore the bivariate and linear relationships between the aforementioned variables in a sample of 86 patients with bleeding disorders between the ages of 15 and 65. RESULTS: Self-compassion and hope were significantly related to QOL. Together, self-compassion and hope were predictive of overall QOL, psychosocial QOL and physical QOL. However, hope was the only individual predictor of all three QOL dimensions. CONCLUSION: Due to the significant relationships found between self-compassion, hope and QOL in this sample, it may be beneficial to incorporate positive psychology factors into the treatment of those diagnosed with bleeding disorders, especially those at higher risk for decreased QOL.

Barriers and challenges faced by women with congenital bleeding disorders in Europe: Results of a patient survey conducted by the European Haemophilia Consortium.

INTRODUCTION: Historically, issues faced by women with bleeding disorders (WBD) have been underestimated. While advances in genetic testing have resulted in improvements, significant challenges remain in the initial recognition of abnormal bleeding and referral of WBD. METHODS: The European Haemophilia Consortium (EHC) developed a questionnaire for WBD to provide insights into the barriers and challenges faced by WBD in Europe. RESULTS: In total, 709 WBD responded to the survey from 32 countries, predominantly from western European countries (94%). A delay in ascertaining the diagnosis of a congenital bleeding disorders (CBD) remains, with a median age at diagnosis of 16 years. The presence of family history is strongly associated with a lower median age at diagnosis of 6 years. WBD reported significant disease impact on their day-to-day life, most evident for the rarer CBD. The bleeding symptom of biggest impact on daily life is heavy menstrual bleeding (HMB), reported by 55% of women. Importantly, 25% of WBD reports that their condition severely impacted their decision to have or has prevented them from having children. Respondents registered with Haemophilia Treatment Centres (HTC) are 2.2 times more likely to receive treatment compared to WBD in other hospital services. CONCLUSION: Improved education for both patients and healthcare providers is essential to improve time to diagnosis, access to treatment and psychosocial supports for WBD in Europe.

Parents Empowering Parents (PEP) Program: understanding its impact on the bleeding disorders community.

The Parents Empowering Parents (PEP) Program gives parents tools to improve the lives of children with bleeding disorders. The aim of this study was to evaluate the efficacy of PEP. Eleven haemophilia treatment centres (HTC) participated in the study and 301 participants completed the survey. Parents who did not attend PEP were divided into three groups based on their reasons for not attending: (Not Offered, Bad Time and Don't Need). Those who attended (Attended) PEP reported less use of yelling, spanking, slapping and giving-in after attending PEP. The Not Offered group used Praising (P = 0.016), Natural Consequences (P = 0.002), Being Consistent (P = 0.016), Ignoring (P = 0.006), Distracting (P = 0.002), Setting Limits (P=0.009), Giving Choices (P = 0.049), Being Consistent (P = 0.014) and Distracting (P = 0.019) less than all other groups. The Bad Time group used Time-Out (P = 0.037) and Ignoring (P = 0.019) more than the other groups that did not attend PEP. The Don't Need group used Spanking (P = 0.008) and Time-Out (P = 0.003) and Yelling (P = 0.014) less than all other groups. Attending PEP seems to decrease the use of negative parenting techniques. Those who reported PEP was not offered to them used positive parenting techniques less than all other participants.

Levonorgestrel-releasing intrauterine system for the management of heavy menstrual bleeding in women with inherited bleeding disorders: long-term follow-up.

BACKGROUND: There are currently limited data on the use of the levonorgestrel-releasing intrauterine system (LNG-IUS) for the management of heavy menstrual bleeding (HMB) in women with inherited bleeding disorders (IBDs) particularly on its long-term (>12 months) efficacy. STUDY DESIGN: This study involves a case series of women with IBDs who received the LNG-IUS as treatment for HMB. Menstrual blood loss before its insertion and at the time of follow-up was assessed by the pictorial blood-loss assessment chart (PBAC) and hemoglobin (Hb) concentrations. A questionnaire was used to evaluate quality of life (QOL) during menstruation before and after insertion of the LNG-IUS. RESULTS: Twenty-six women were included. The median duration of LNG-IUS use at follow-up was 33 months (range, 14-103). The median PBAC score decreased from 255 (range, 134-683) to 35 (range, 0-89) with LNG-IUS use. The median Hb concentrations (11.2 to 13.2 g/dL) and QOL scores (median, 26 to 52) improved significantly with LNG-IUS use (p<.01). CONCLUSION: The LNG-IUS appears to be an effective long-term treatment for HMB in women with IBDs.

Quality of life in children and families with bleeding disorders.

Quality of life (QoL) in children and adolescents with bleeding disorders and their families is a relatively new topic. It is important to understand more about QoL in this patient population to evaluate and if necessary to improve the care patients receive. To achieve this aim, a questionnaire to assess patients' QoL in hemophilia was developed and psychometrically tested. Three hundred twenty hemophiliac children and adolescents from six European countries and their families were asked to fill out a questionnaire regarding different aspects of their well-being and functioning, as well as their views on hemophilia care. Generic QoL questionnaires showed that children with hemophilia have a higher QoL than other patients with chronic disease, such as asthma/atopic dermatitis and obesity. Several determinants affected patients' QoL (e.g., number of bleeds, social support). Parents' and children's assessments differed with regard to social and emotional aspects of QoL. The study showed that variations in QoL can be explained by clinical and psychosocial factors and suggested that QoL can be assessed and enhanced both by medical and non-medical (e.g., psychological) interventions.

[Factitious disease caused by secret administration of the oral anticoagulant phenprocoumon: study of 16 personal cases]. / Die artifiziell manipulierte Krankheit durch heimliche Einnahme des oralen Antikoagulans Phenoprocoumon: Untersuchung an 16 eigenen Fällen.

BACKGROUND: It is frequently difficult to disclose covert ingestion of cumarins being the underlying cause of a bleeding disorder. PATIENTS AND METHODS: We report on 16 cases (15 patients) of phenprocoumon (Marcumar) abuse. All patients (9 females, 6 males) were admitted to the hospital because of bleeding and/or prolonged thromboplastin times. Analysis of phenprocoumon in the blood was performed by HPLC. RESULTS AND CONCLUSIONS: The clinical presentation of the bleeding was highly variable. Thromboplastin times ranged from < 5 to 58%. With the exception of two patients with hepatic dysfunctions factor V activity was normal. All but one patient denied the ingestion of Marcumar. One patient died from hemorrhage. Two types of patients emerge from our series: Type I is the young nurse (8 cases), type II the usually male patient formerly treated with phenprocoumon (5 cases). Type I patients had long medical histories and showed autoaggressive behavior including the artificial manipulation of disorders other than hemorrhage. Thromboplastin times however were within or close to the therapeutic range (10 to 25%). Type II patients did not simulate other diseases in addition to hemorrhage. Thromboplastin times were longer than in type I patients. In all instances discrimination between non suicidal and suicidal behaviour was difficult. Psychiatric consultance is indicated.

[Psychodiagnosis and psychotherapy in medico-social adaptation of patients with hemorrhagic diseases]. / Psikhodiagnostika i psikhoterapiia v mediko-sotsial'noi adaptatsii bol'nykh gemorragicheskimi bolezniami.

The investigations were conducted in 264 patients with hemorrhagic diseases. Based on the clinical and psychological analysis most of the patients were found to have borderline psychic disorders. Psychotherapy has promoted diminution of psychic disorders, and in patients with hemorrhagic thrombocytopathy it has resulted in decreased hemorrhage and improved platelet function.