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Living with Haemophilia,
  1. PAUL GIANGRANDE, Consultant haematologist

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    Living with Haemophilia, 4th ed. By Jones P. (Pp 412; paperback £14.99; hardback £26.95.) Oxford: Oxford University Press, 1995. ISBN 0 192 62961 1 paperback; 0 192 63030 X hardback.

    The diagnosis of haemophilia in a newborn son often leaves parents feeling completely overwhelmed. Many mistakenly believe that a simple scratch in a boy with this congenital bleeding disorder can lead to death through exsanguination, or that their son will end up in a wheelchair because of early arthritis. In fact, the prognosis for young people with haemophilia is excellent, and boys in developed countries can look forward to an essentially normal life. The introduction of recombinant coagulation factor concentrates has eliminated the risk of transmission of viral infections such as HIV or hepatitis, and it is now usual practice to give factor VIII on a regular, prophylactic basis to prevent most spontaneous bleeds, and thus long term disability. Haemophilia actually confers protection against coronary artery thrombosis, which is the principal cause of death in middle aged men.

    Haemophilia is a relatively rare disorder, affecting approximately 6000 boys and men in the UK. Few doctors and nurses will have experience of dealing with a condition that affects only 1 in 10 000, and parents may be frustrated by difficulty in finding answers to their many questions. This book distils the personal experience of a paediatrician, which extends over 30 years, in the treatment of this rare disorder. The book is primarily aimed at the parents of boys with haemophilia. However, it will undoubtedly also be an invaluable source of reference for all health care professionals or others (such as teachers) who come across children with haemophilia. The value of this book is reflected in the fact that the book is now given as a gift to all parents registering for the first time with the Haemophilia Society, the national patient charity. The basic medical facts about blood clotting and haemophilia are clearly set out, and excellent illustrations help to get the basic messages over. There is comprehensive advice on matters about management of all bleeding problems, with specific advice on exact dosage of coagulation factor concentrate. There is also general advice on issues such as employment and travel. There is also a section aimed at female carriers of haemophilia, with up to date information on the latest methods used for genetic identification of carriers as well as antenatal diagnosis of haemophilia. It may come as a surprise to some to learn that boys with haemophilia are now positively encouraged to participate in sports at school, and this book gives very sensible advice on which sports are to be encouraged (such as swimming and tennis) and those that are best avoided (such as hockey and rugby). A programme of physical exercise helps to maintain muscle strength and prevent joint bleeds. People with haemophilia should not take aspirin, which exacerbates the bleeding tendency, and I am sure that patients will find the list of almost 600 over the counter products that contain aspirin very useful.

    This 5th edition was first published in 1995 but is now available in paperback for the first time. This is a rapidly moving field and some significant recent developments are not covered in the book. Recombinant factor IX is now available for all young patients with haemophilia B, and recombinant factor VIIa is now licensed and has proved to be invaluable in the treatment of the minority of patients with inhibitory antibodies. However, this is an excellent, comprehensive, and well illustrated book that will be invaluable to all who come into contact with boys with this rare condition.

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