Recommendations on multidisciplinary management of elective surgery in people with haemophilia.

Planning and undertaking elective surgery in people with haemophilia (PWH) is most effective with the involvement of a specialist and experienced multidisciplinary team (MDT) at a haemophilia treatment centre. However, despite extensive best practice guidelines for surgery in PWH, there may exist a gap between guidelines and practical application. For this consensus review, an expert multidisciplinary panel comprising surgeons, haematologists, nurses, physiotherapists and a dental expert was assembled to develop practical approaches to implement the principles of multidisciplinary management of elective surgery for PWH. Careful preoperative planning is paramount for successful elective surgery, including dental examinations, physical assessment and prehabilitation, laboratory testing and the development of haemostasis and pain management plans. A coordinator may be appointed from the MDT to ensure that critical tasks are performed and milestones met to enable surgery to proceed. At all stages, the patient and their parent/caregiver, where appropriate, should be consulted to ensure that their expectations and functional goals are realistic and can be achieved. The planning phase should ensure that surgery proceeds without incident, but the surgical team should be ready to handle unanticipated events. Similarly, the broader MDT must be made aware of events in surgery that may require postoperative plans to be changed. Postoperative rehabilitation should begin soon after surgery, with attention paid to management of haemostasis and pain. Surgery in patients with inhibitors requires even more careful preparation and should only be undertaken by an MDT experienced in this area, at a specialized haemophilia treatment centre with a comprehensive care model.

Home treatment of haemophilia patients with inhibitors.

Bleeding episodes in patients with inhibitors can be challenging to treat. Clinical guidelines recognize the importance of early treatment, ideally within 2 h of the onset of bleeding. On-demand haemophilia care at home has been shown to reduce the time between recognition of the symptoms of bleeding and initiation of treatment. Rapid resolution of bleeding is associated with longer-term benefits for the patient. Effective haemophilia care at home depends on patients and carers taking greater responsibility for treatment; however, many find this difficult. Education can help raise awareness of haemophilia treatment at home and provide helpful information for patients/carers. The haemophilia nurse has a key role in providing this support and education. This review discusses a number of recent guidelines and educational materials for haemophilia home care identified during a literature survey. The survey shows that most materials were not validated. In addition, the survey shows limited effectiveness data on techniques for training haemophilia patients about home care. Further education resources and research in the treatment of haemophilia at home are required.

Reproductive choices for couples with haemophilia.

: Summary. The impact of having a child with an inherited bleeding disorder such as haemophilia can have a far-reaching effect on the individual as well as other close family members. This situation is further complicated in the case of human immunodeficiency (HIV) serodiscordant couples, where the haemophilic man is affected with HIV through infected blood products whilst his partner is seronegative and wanting to have children. It is essential that information on the effects of haemophilia, its inheritance, the possibilities of antenatal diagnosis, the consideration of selective abortion and the new reproductive opportunities available to these couples are made accessible so that an informed decision about proceeding with having a family can be made. Couples may wish to have a family through nonreproductive methods such as fostering or adoption. Alternatively, they may wish to remain childless. In this paper, the terms 'having children' and 'having a family' will refer to conception through biological reproduction. Pre-implantation genetic diagnosis (PGD) offers families at risk of having a child with certain inherited genetic disorders the opportunity to give birth to an unaffected child. It may be considered as an option for couples who would not wish to have prenatal diagnosis leading to possible termination of a pregnancy. Assisted conception techniques, such as 'sperm washing' or the use of 'donor sperm', offer serodiscordant couples affected by HIV a risk-reduced or risk-free opportunity, respectively, to have a child without infecting the mother, who could in turn infect the fetus by vertical transmission. This article, in addition to outlining the inheritance of haemophilia and the more common prenatal screening and diagnostic tests, discusses in more detail the latest reproductive opportunities available for families affected by haemophilia and considering having a family.