Being a carrier of haemophilia and having a haemophilic child was life changing. The women moved from a state of sad, guilty chaos to reconciling
themselves with the new situation. Our analysis revealed three acts in which phenomena appeared: the time after diagnosis, the turning point and AZD6244 reconciliation with a changing life. Emerging as crucial to the process of reconciliation with a changing life was a sense of being fully informed and supported. The Haemophilia Treatment Centre (HTC) should create an environment that encourages learning, and the team should invite and encourage the woman’s partner to be actively involved in the child’s care. Moreover, the results indicate that it would be beneficial to invite female carriers to receive patient education at the HTC before
they plan to start a family. During this visit, the woman may gain a greater understanding of her carriership to prepare her for future decisions concerning prenatal diagnosis, for example. “
“Summary. Haemophilia, if not properly managed, can lead to chronic disease and lifelong disabilities. The challenges and issues in infants/young children are different from those in older children and adults although episodes of bleeding still predominate as the diagnostic trigger. Awareness of clinical manifestations and treatment complications MG-132 order are crucial in instituting appropriate management and implementing preventive strategies. Currently, inhibitor development is a challenging complication of paediatric haemophilia and prophylaxis is emerging as the optimal preventive care strategy. In this section we will review some important aspects of haemophilia in children including early prophylaxis, current evidence relating to inhibitor development, including the aims of the SIPPET study
which is already ongoing and involves boys <6 years, and the potential of immune tolerance therapy for eradicating selleck compound the inhibitor and permitting a resumption of standard dosing schedules. In this section, dedicated to haemophilia during childhood, we will focus on two hot topics: prophylactic regimens and the development of inhibitors against FVIII. Primary prophylaxis, which has been used in Sweden for over 40 years, is the treatment of choice in severe haemophilia recommended by the World Health Organisation (WHO) and the World Federation of Haemophilia since 1994. The benefits of prophylaxis have been clearly demonstrated by numerous cohort studies and, more recently, by a randomized trial. Therefore, to prevent the development of haemophilic arthropathy, primary prophylaxis should be started after the first haemarthrosis during early childhood. However, the optimal regimen has not been fully established yet.