scholarly journals Gynaecological issues in women with bleeding disorders: CSL Behring Symposium

2019 ◽  
Vol 6 (2) ◽  
pp. 28-38
Author(s):  
Angelika Batorova ◽  
Karin van Galen ◽  
Sylvia von Mackensen ◽  
Ioannis Tsimpanakos ◽  
Evelyn Grimberg

Abstract The symposium focused on issues around surgery, ovulation bleeding, health-related quality of life (HRQoL) and pelvic pain in women with bleeding disorders. Surgery Young women with congenital bleeding disorders, especially those with severe forms, are more likely to experience gynaecological and obstetric disorders than unaffected women. Surgery may be required to manage heavy menstrual bleeding (HMB), ovulatory bleeding, endometriosis and delivery. Major surgery should be undertaken only in hospitals with a haemophilia centre and 24-hour laboratory capability. Correction of haemostasis, either by desmopressin, coagulation factor or platelet transfusion, is essential for a successful outcome of surgery. Management of pregnancy requires a multidisciplinary approach; the mode of delivery is based on the consensus of gynaecologist and haematologist, and with respect to the patient’s diagnosis. Ovulation bleeding Women with bleeding disorders are at risk for excessive gynaecological bleeding associated with menstruation, ovulation, pregnancy and delivery. Ovulation bleeding is associated with the rupture of ovarian cysts and causes abdominal pain; complications include haemoperitoneum, fertility problems and ovarian torsion. Management includes hormonal and haemostatic therapies, in combination if necessary, and surgery as a last resort. Current management is based on experience in a relatively small number of cases and more clinical data are needed. Health-related quality of life In addition to experiencing joint and tissue bleeds, women experience psychosocial and medical issues associated with menstruation, pregnancy, labour and delivery. HMB has the greatest impact, and is associated with impaired HRQoL in almost all and dissatisfaction with the burden of treatment. There is a need for focused psychosocial support and a specific tool for the assessment of HRQoL in women with bleeding disorders. Pelvic pain Gynaecological causes of pelvic pain in women with bleeding disorders include dysmenorrhoea, mid-cycle pain, bleeding into the corpus luteum and endometriosis. There is no correlation between bleeding tendency and endometriosis severity; however, screening for a bleeding disorder should be considered. Pharmacological management may be hormonal or non-hormonal. Gonadotrophin-releasing hormone agonists offer an alternative to surgery for women with severe bleeding disorders who have endometriosis. Paracetamol is the preferred early analgesic option. Endometrial ablation controls heavy bleeding and pelvic pain but is not recommended for women with large fibroids or a large endometrial cavity. Hysterectomy is an option of last resort. Education for health professionals should include raising awareness about the management of pain in women with bleeding disorders.

Author(s):  
Kristiina Pynnä ◽  
Pirjo Räsänen ◽  
Harri Sintonen ◽  
Risto P Roine ◽  
Piia Vuorela

Aim: To assess health-related quality of life (HRQoL) of patients with benign gynecological disorders. Materials & methods: Prospective 2-year follow-up with the 15D HRQoL-instrument of 311 women treated in Helsinki-area hospitals in 2012–2013. Results: The initially impaired HRQoL regarding excretion, discomfort and symptoms, and vitality and sexual activity improved after treatment. However, only sexual activity reached similar levels as in the general population. Treatment of endometriosis, fibroids and polyps resulted in best and that of unspecific pelvic pain and bleeding disorders in worst HRQoL scores. Results were independent of hospital size. Conclusion: The impaired HRQoL dimensions were improved by treatment but HRQoL still remained poorer than in the general female population. Treatment of unspecific pelvic pain and bleeding disorders needs further evaluation.


Blood ◽  
2021 ◽  
Author(s):  
Alessandro Casini ◽  
Sylvia von Mackensen ◽  
Cristina Santoro ◽  
Claudia Djambas Khayat ◽  
Meriem Belhani ◽  
...  

Due to its low prevalence, epidemiologic data on afibrinogenemia are limited and none are available on health-related quality of life (HRQoL). We conducted a cross-sectional international study to characterize the clinical features, the fibrinogen supplementation modalities and their impact on HRQoL in patients with afibrinogenemia. A total of 204 patients (119 adults and 85 children) from 25 countries were included. The bleeding phenotype was severe: 68 (33.3%) patients having at least one bleed per month and 48 (23%) a history of cerebral bleeding. About 35% (n=72) of patients were treated with fibrinogen concentrates or cryoprecipitates as prophylaxis, 18.1% (n=37) received more than one injection per week and 16.6% (n=34) were on home treatment. A thrombotic event was reported in venous and/or arterial territories by 37 (18.1%) patients. Thrombosis occurred even in young patients and recurrence was frequent (7.4%). The total HRQoL was lower in children than in adults. Discomfort linked to treatment and limitations to sports and leisure were the main concerns. Women and children were particularly affected in family relationships. In multivariate analyses, younger age, residence in Asia or Africa and a previous thrombotic event were statistically correlated with a worse HRQoL. In conclusion, our study underlines the severe bleeding and thrombotic phenotype and their impact on HRQoL in afibrinogenemia. The optimal strategy for fibrinogen supplementation needs to be determined.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4773-4773
Author(s):  
Neuner Bruno ◽  
Sylvia von Mackensen ◽  
Susan Halimeh ◽  
Susanne Holzhauer ◽  
Robert Klamroth ◽  
...  

Background Hereditary bleeding disorders (HBD) affect children from birth during their life course. Medical treatment and the overall prognosis of children with HBD have significantly progressed. But even in patients cared for in specialized treatment centers it remains unclear whether survival till adulthood goes along with health-related quality of life [Hr-QoL] comparable to other patients groups respectively comparable to population children. Therefore aim of the present study was to assess self- as well as parents/proxies reported Hr-QoL in children and adolescents with HBD and to compare the results with findings from children affected by another chronic medical condition respectively with healthy siblings and peers. Methods 91 patients with HBD (thrombosis / deep venous thrombosis, DVT, respectively Haemophilia A and B) aged 8 to 16 years from 6 Hemophilia study centers were investigated regarding Hr-QoL. Hr-QoL was assessed in patients and parents/proxies with the generic KINDL-R questionnaire exploring overall well-being and 6 sub-dimensions (physical well-being, psychological well-being, self-esteem, family-related well-being, friend-related well-being, and school-related well-being). Findings were compared with 70 children with stroke / TIA respectively with healthy controls (45 healthy siblings and 106 healthy peers). Results Overall well-being in children with thrombosis / DVT (77.6 ± 9.7 points) was comparable to healthy controls but in children with Haemophilia A or B (76.0 ± 9.6) lower compared with healthy peers (80.2 ± 9.7, p = 0.005). No differences occurred between healthy controls and children with stroke/TIA (74.2 ± 10.3). In children with HBD none of the KINDL-R sub-dimensions showed values below healthy siblings. Two KINDL-R sub-dimensions showed lower values compared with healthy peers. Parents/proxies rated the Hr-QoL of their children with HBD similar to their healthy children. The internal consistency of overall well-being in all study subgroups was acceptable (> 0.7) but showed poor results (< 0.5) in two KINDL-R sub-dimensions. Discussion The application of a generic Hr-QoL questionnaire in a sample of children and adults with HBD seems feasible. Their Hr-QoL seems comparable to their siblings but below their healthy peers. Disclosures: Halimeh: Octapharma AG: Investigator Other, Research Funding.


2017 ◽  
Vol 67 ◽  
pp. 96-101 ◽  
Author(s):  
Ulrike Nowak-Göttl ◽  
Hartmut Clausnizer ◽  
Dorothee Kowalski ◽  
Verena Limperger ◽  
Anne Krümpel ◽  
...  

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