Haemophilia caregivers face limitations in their life leading to perceived burden. This single-centre study investigates the impact of burden on caregivers’ health-related quality of life (HRQoL).
Questionnaires for caregivers comprised demographic data, HRQoL (EQ-5D, SF-36) and caregiver burden (IOF: Impact on Family Scale). Children were also asked about their HRQoL (EQ-5D, Haemo-QoL Short Form).
20 consecutive parent/child dyads participated. 80% were mothers (mean age of 39.80±6.2 years) with 1-3 haemophilic children aged 8-17 years and 2.5±1.2 children <18 years living in the household. Mothers did most childcare (80%), 50% worked part-time, and 55% reported that haemophilia had an economic impact on their family. 80% of boys had haemophilia A; 60% were severely affected. Most received home treatment (85%) and prophylaxis (80%). Caregivers’ and boys’ HRQoL was similarly good (EQ-5Dparents M=0.90±0.1 vs. EQ-5Dchild M=0.81±0.3); by contrast, boys reported better values in the EQ-VAS (Mchild=90.25±10.0 vs. Mparents=82.16±14.8). Caregivers reported highest impairments in the dimensions ‘vitality’ (M=60.00±20.5) and ‘emotional role’ (M=70.37±42.6) of the SF-36. In the IOF, caregivers reported highest impairments in the dimension ‘negative impact’ (M=60.08±20.7). Caregivers reporting high burden had significantly worse HRQoL in the domains ‘bodily pain’ (p<.028) and ‘social functioning’ (p<0.024) of the SF-36. Caregivers who reported that haemophilia had an economic impact on their family and those with a chronic disease showed significant higher impairments in caregiver burden and their HRQoL.
The perceived burden of haemophilia has a direct impact on caregivers’ HRQoL. Further studies with haemophilia-specific instruments are needed to verify these findings.
Assessment of health-related quality of life (HRQoL) in haemophilia is important in order to provide information for clinical decision-making and to verify the impact of haemophilia on patients and their partners. A crosssectional single-centre study was performed to assess the health-related quality of life (HRQoL) and burden of the disease on partners of adult patients with haemophilia. Self-reported outcomes were completed by partners and patients (SF-36, VAS of Interference); partners also completed the Caregivers’ Burden Scale. A total of 108 of 150 eligible partners of adults with haemophilia (72%) participated. Mean age for partners was 44.7 years (range 20-79) and for patients 47.1 years (range 20-81). The majority of couples were married (65.7%). Couples reported being together a mean of 19.8 years and had, on average, 1.7 children. Partners of haemophilia patients across all severities reported lower HRQoL in the ‘emotional role’ domain of SF-36 (P=<0.041), with highest impairments observed among partners of moderately affected patients. Partners reported significantly less interference with daily life compared to patients (P<0.001). In general, partners reported low burden of haemophilia in the Caregivers’ Burden Scale; ‘emotional involvement’ was the greatest burden in the mild and moderate group; while in the severe group ‘general strain’ was the greatest burden. Partners of severe haemophilia patients on prophylaxis reported, in general, good HRQoL and low burden of the disease. Partners of moderate patients reported decreased HRQoL and higher burden. Our findings may be of importance for the care of the ageing person with haemophilia (PWH).
Prolonged menstrual bleeding interferes with daily life and causes marked blood loss, resulting in anaemia and fatigue. Treatment centres should address the issue of heavy menstrual bleeding (HMB) with pre-pubertal girls in advance of their first period, in order to best prepare them. It is common for a bleeding disorder to be overlooked in primary care and in gynaecology clinics, and women sometimes struggle to get a correct diagnosis. There are cultural taboos that inhibit open discussion of menstruation, and women tend to minimise the severity of their symptoms. Health professionals should work to destigmatise the issue and seek an accurate account of bleeding severity, with diagnosis and treatment provided in a joint clinic combining gynaecology and haematology expertise. Treatment should be individualised, taking into account personal, social and medical factors, with the aim of improving quality of life. Great care is needed with regard to choice of language when talking about treatment, and treatment centres should consider offering open access to women who need support in dealing with adverse effects. National member organisations have an important role to play in educating people with bleeding disorders, health professionals and the wider public about the burden of HMB associated with bleeding disorders.
The symposium focused on issues around surgery, ovulation bleeding, health-related quality of life (HRQoL) and pelvic pain in women with bleeding disorders.
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.
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.
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.