Health-related quality of life in children, adolescents and adults with hereditary and acquired bleeding disorders

2017 ◽  
Vol 67 ◽  
pp. 96-101 ◽  
Author(s):  
Ulrike Nowak-Göttl ◽  
Hartmut Clausnizer ◽  
Dorothee Kowalski ◽  
Verena Limperger ◽  
Anne Krümpel ◽  
...  
2011 ◽  
Vol 11 (1) ◽  
Author(s):  
Cédric Baumann ◽  
Marie-Line Erpelding ◽  
Christine Perret-Guillaume ◽  
Arnaud Gautier ◽  
Stéphanie Régat ◽  
...  

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.


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