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The person with Haemophilia may experience bleeding problems in any part of the body at one time or another. The areas in which bleeding occurs and the frequency vary considerably from one individual to another. Bleeds for those with severe haemophilia may be due to some trauma, but many appear to be spontaneous. Some parts of the body are more susceptible to injury than others, such as the knee, ankle and elbow joints, and hence bleeding into these areas are more common.
The person with moderate haemophilia generally suffers less frequent bleeds and these bleeds are rarely spontaneous. Bleeding into the joints can still be a problem.
The person with mild Haemophilia usually only has a bleeding problem after major trauma, tooth extraction or surgery.
First priority - TREAT
As soon as a bleed is suspected treatment should be given. Unless the bleeding is from a small superficial cut this treatment will be in the form of infusing the missing clotting factor. (Replacement therapy)
Dealing with different types of bleeds
External cuts and abrasions
Superficial cuts usually pose no problem Small cuts often stop bleeding by themselves, but in most of these cases a band aid or small bandage will stop the bleeding.
Mucous membrane bleeding
Small cuts of the mouth, tongue, tooth extractions and nose bleeds, may ooze for days. Replacement therapy must be sought. Tranexamic acid (Cyklokapron) will also be prescribed. This drug helps to maintain any clot that is formed and limits bleeding. Cold soft foods are recommended until after bleeding has stopped. Rebleeding may occur 7-8 days after replacement therapy, sometimes requiring a further dose.
Soft tissue bleeds
Superficial bruising is common and not dangerous. They can vary in size and often appear as a raised lump with blue purple colouring. Replacement therapy is rarely necessary and the application of a icepack, wrapped in a towel to prevent direct contact with the skin, will limit the extent of the bruising. The skin of the forehead and scalp has a very rich blood supply and bruises in this area may enlarge rapidly and require replacement therapy. If the size of the bruise continues to increase seek medical advice
Some superficial bruising may occasionally be the presenting feature of a deep muscle bleed. This should be considered potentially dangerous and is very painful. These commonly occur in the muscles of the forearm, thigh and lower stomach/groin (psoas bleed). If left untreated they can lead to nerve damage and possible muscle paralysis and wasting. Replacement therapy, immobilisation e.g. splints, slings or rest and physiotherapy to maintain muscle strength, flexion and extension once the pain has ceased and the swelling down are essential.
Joint bleeds (haemathrosis)
Joint bleeds are the single most crippling complication of severe haemophilia and can result in chronic disabling arthritis. While not life threatening they should never be left untreated, no matter how mild they appear. Signs and symptoms of a joint bleed are, pain, a reluctance to move the affected joint, swelling and warmth. If untreated the membrane surrounding the joint (synovium) will become distended causing severe pain. Prompt adequate replacement therapy and immobilization is essential. Failure to treat the joint will ultimately lead to gradual destruction of the smooth joint surfaces (cartilage) resulting in arthritis. Physiotherapy to maintain good range of movement and muscle tone must follow as soon as the pain and swelling has subsided.
Blood in the urine (haematuria)
This complication occurs infrequently and spontaneously in severe haemophilia. A blow to the lower back or side may result in bleeding from one or both kidneys. Rest and increased fluid intake are recommended initially. Replacement therapy will only be given under supervision when the blood in the urine is not so dark.
Internal Bleeding
Bleeding from the stomach or intestinal tract, in the lungs or other organs is an uncommon feature of haemophilia If it occurs replacement therapy must be given immediately and medical advice sought.
Head Injuries
A bad knock on the head to a person with haemophilia should be regarded as potentially serious and the person should be taken to hospital immediately.
Replacement therapy is indicated immediately. Look out for symptoms of headache, vomiting, dizziness, drowsiness, irritability, or confusion. These indicate the need for immediate attention.
Eye Injuries
If trauma to the eye occurs, give replacement therapy immediately
Neck and Throat bleeds
These are potentially dangerous as the swelling can obstruct the air passages. Bleeding in these areas may be mistaken for mumps, so care must be taken in the diagnosis. Tonsillitis, sore throat, or severe coughing associated with bronchitis or whooping cough may precede a bleed.
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