A child with haemophilia does not bleed any faster than usual. However, it will take longer for the bleeding to stop. The condition can be mild, moderate or severe and the severity of the condition is directly related to the severity of the deficiency. Factor VIII (A), factor IX (B) and von Willebrand’s factor are the clotting factors most commonly affected. Children with mild and moderate bleeding disorders will have few problems. Their blood clots less efficiently than normal. However, this only causes trouble during surgery or after major trauma. Children with severe bleeding disorders have inefficient blood clotting and this can cause bleeding even when the person is apparently not injured.
With appropriate treatment, haemophilia can be managed effectively. The most common treatment is an injection of the missing clotting factor into the child’s veins. Early treatment is the key to quick rehabilitation. Some parents have been trained to give treatment to their children. Other children will need to be taken to the hospital for treatment. Bleeding can be internal or external.
The most common types of internal bleeding are into the joints or muscles and may occur without any obvious cause. Repeated or untreated bleeding into joints and muscles can cause permanent damage such as arthritis, chronic pain and joint damage requiring surgery. Older children should tell you if they have a bleed. Younger children may seem upset, or may protect a limb by limping or not using it.
Signs of bleeding into the joints or muscles are:
. The child will be reluctant to move the affected limb/joint.
. The child will complain of pain in the affected joint/limb.
. There may be swelling and heat in the affected joint or limb if the bleed is not treated promptly.
Treatment for bleeding into the joints and muscles is:
Coagulation factor concentrate (IV),
Rest, Ice, Compression and Elevation
Joint/muscle bleeds should be treated as soon as possible. If a child complains of any of the above signs please contact the child’s parents and/or seek medical advice, elevate the limb and apply ice (if available) and do not allow the child to use the limb until his parent/guardian arrives. Do not apply pressure until the child has had coagulation factor concentrate as constricting swelling can be dangerous. Children can sometimes return to school when recovering from a bleed. However, they should be allowed to elevate the affected limb and be excused from activities such as PE.
It is recommended that all children with severe haemophilia who are over two years of age or have had two joint bleeds receive regular doses of coagulation factor concentrate to prevent spontaneous bleeding and reduce the risk of long-term side effects. This is called prophylaxis. For children with severe factor VIII deficiency this involves injection of coagulation factor concentrate into the veins three times per week, for children with severe factor IX deficiency twice per week and for children with severe factor X deficiency once to twice per week. In small children and children with poor veins repeated intravenous injections can be very difficult and a port-a-cath or “port” is needed until the veins have developed. By the time your child is ready to start prophylaxis, you will have discussed intravenous access and ports many times with your haemophilia team.