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Treatment

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. Some children self infuse. Bleeding can be internal or external.

Treatment for bleeding into the joints and muscles:

  • Coagulation factor concentrate (IV),
  • R.I.C.E. – (Rest, Ice, Compression & Elevation)

Prophylaxis

It is recommended that all children with severe haemophilia who are over two years of age or who 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 and factor IX deficiency, this involves an injection of coagulation factor concentrate into the veins on a regular basis, usually one to two times per week. In Ireland, the factor concentrates currently used are extended half life factor concentrates which are infused less regularly then regular factor concentrates and offer more protection against bleeding episodes. In small children and children with poor veins repeated intravenous injections can be very difficult and a port-a-cath or ‘port’ may be needed until the veins have developed. By the time your child is ready to start prophylaxis, you will have discussed intravenous access and ports with your haemophilia team.

Subcutaneous Therapy

From late 2019, a new generation of treatment for people with severe factor VIII deficiency is available as an option in Ireland. This treatment, called Hemlibra, is a monoclonal antibody which mimics the effect of factor VIII. It can be infused weekly or every 2 weeks subcutaneously (under the skin) thereby avoiding the requirement for regular intravenous injections. This treatment offers a high degree of protection against bleeding but can not be used to treat bleeding episodes. In the event of a bleeding episode, intravenous infusion with FVIII concentrate will continue to be required. This subcutaneous therapy can not be used for factor IX deficiency so, in this case , the routine treatment will continue to be extended half life factor IX concentrate.

Identifying a bleeding episode during infancy can be very challenging for parents. During the first six months of life, bleeding episodes are minimal. Superficial bruising can be commonly seen on the chest or back from picking up the infant. These bruises are usually minor and do not require treatment. They can be minimized by cradling the infant when picking him up.

General guidelines for administering immunizations include using the smallest gauge needle possible. An ice pack can be applied for five to ten minutes after the injection to help decrease the risk of bleeding. Also, place a barrier such as a gauze or a washcloth between the ice pack and the injection site so the ice will not be in direct contact with the skin. Immunizations commonly cause redness, warmth and minor swelling at the site of injection. Infants can also become irritable following immunizations. Children with haemophilia should be observed carefully for signs that could indicate a bleed.

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.

You should:

  • Apply an ice pack to the site
  • Call your clinic if symptoms persist

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