By Peter Jones
, For the main half we. the haemophiliacs current at this Congress. have come from the good metropolitan centres with their complex scientific and social programmes for victims of haemophilia. We. the lucky from the haemophilia oasis. have a lot to benefit from one another. this is often vital. yet much more vital is the urgency to express your wisdom. your abilities. your event and your commitment to the haemophiliacs within the wilderness: 'We can purely start to comprehend the situation. the lifetime of a patient. through evaluating him to a soldier within the trenches of global struggle I. within the trenches the soldier seldom forgets that the subsequent second could carry dying or crippling. The haemophiliac is actually within the trenches. The soldier should be spared harm. yet discomfort awaits the haemophiliac. worry. in addition, is paramount to the ache. As within the trenches. the nervousness might be extra oppressive than the wound. ready to move excessive imposes a better pressure than the particular cost. For the warriors that survived global battle I within the trenches. four years appeared eternity; the haemophiliac by no means leaves the battlefield: beginning deal with, Frank Schnabel. international Federation of Hemophilia. Copenhagen. June twenty fifth. 1963. warfare can come to an oasis, peace can come to the trenches. With this booklet, Dr Peter Jones has joined the foreign fight. conscientiously, concisely and cogently. the textual content deals a grand process. With allies like Dr Jones we'll, sooner or later, in achieving victory. Frank Schnabel, Chairman.
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Extra resources for Living with Haemophilia
Once a mutation has occurred it is permanent-the misprinted gene is fixed to its chromosome and copied every time a cell divides. The misprint may appear in the sperm or egg to be passed to the next generation, and if its effect is to produce a disease we say the disease is inherited, or hereditary, and may find a • Readers who wish to know how this is done will find the reference in any large encyclopaedia, or they may like to read James Watson's own account of the discovery in his book The Double Helix.
The long sequence of factors provides the possibility of cut-off points at several stages. Clotting is full of cut-offs and fail-safe mechanisms. and it is because of them that a clot usually remains localized to the site of injury and does not extend into the circulation. The most important safety mechanism involved is that of fibrinolysis. This mechanism is responsible for the removal of unwanted fibrin. Within each clot are fibrinolytic substances which are activated in a way similar to the activation of the clotting factors.
With increasing knowledge of platelet function doctors are recognizing more conditions in which this stickiness is reduced. Some, like thrombasthenia (Glanzmann's Disease), are inherited and others are secondary to other diseases or drugs. The most important of the drug causes is aspirin, and this is one of the reasons why aspirin is so strongly condemned in the treatment of anyone with a bleeding disorder. Diseases that result in purpura because of a lack of platelets are acquired and temporary; people are not born with them and they need not concern us here.