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 bleeds 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 extractions 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). This treatment will either be given by the person with haemophilia or parent or by experienced healthcare professionals.
Until such time as the person with haemophilia and his family have gained experience in distinguishing potentially serious bleeds from relatively minor ones, expert medical advice should be sought. Parents may need to be assertive at times with inexperienced hospital staff, to ensure prompt treatment is given to their child.
Dealing with different types of bleeds
External cuts and abrasions
Superficial cuts usually pose no problems. Small cuts often stop bleeding by themselves, but in most cases a sticking plaster or small bandage will stop the bleeding and assist healing. Healing is also helped by keeping the wound dry. Bleeding from abrasions and larger cuts can be arrested by the application of gauze or cotton type stypwool under a firm bandage.
Cuts and abrasions to moist surfaces
Small cuts to the mouth or tongue may ooze for days, and replacement therapy should be sought. Tranexamic acid (Cyklokapron) tablets may be prescribed by your haematologist. This drug helps maintain any clot that is formed and limits bleeding. Initially, sucking a smooth ice block may help. Cold, soft foods are recommended until after bleeding has ceased. Re-bleeding may occur 7-8 days after replacement therapy, sometimes requiring a further dose of treatment.
Soft tissue haemorrhage
Superficial bruising is common in haemophilia and is usually neither dangerous nor painful. Superficial bruises can vary considerably in size and often appear as a raised lump with blue-purple colouring. Replacement therapy is rarely necessary and the application of an ice pack, wrapped in a towel to prevent direct contact with the skin, will limit the extent of 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, medical advice should be sought.
Some superficial bruising may occasionally be the presenting feature of deep muscle haemorrhages, which should be considered potentially dangerous and can be very painful. These commonly occur in the muscles of the lower stomach/groin (psoas bleeds) and the muscles of the forearm, the thigh (quadriceps) and calf. If left untreated, they can lead to nerve damage and possible muscle paralysis and atrophy (wasting).
Replacement therapy and immobilisation, e.g. splints, slings or rest, are essential, and physiotherapy, when the pain has ceased, is recommended to maintain muscle strength.
If a bleed is suspected in the groin or inner wrist, there is the possibility of nerve or vessel compression if it is not treated promptly. Any suspected hip bleeds must also be treated at the hospital.
Joint haemorrhage (haemarthrosis)
Haemarthroses 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 minor they may appear. Haemarthroses are often indicated by pain, with a reluctance to move the affected joint and, if severe, by warmth and swelling. If left untreated, the membrane surrounding the joint (synovium) will become distended causing severe pain.
Prompt, adequate replacement therapy and resting of the limb are essential and will very quickly reduce the swelling and pain. Failure to treat immediately will ultimately lead to a gradual destruction of the smooth joint surfaces (cartilage) resulting in arthritis. A programme of physiotherapy, as soon as the pain and swelling have subsided, is most important to maintain good joint movement and muscle tone, and will hasten the return to normal activities.
Blood in the urine (haematuria)
This complication occurs infrequently, often spontaneously, in severe haemophilia. A blow to the lower back or side may result in bleeding from one or both kidneys. Haematuria itself is painless but can be very persistent. It can be painful when clots begin to form and perhaps block the passage between the kidney and the bladder (ureter) resulting in very severe pain (clot colic).
Rest and increased fluid intake are recommended initially. Oral steroids are sometimes used to stop haematuria. Replacement therapy may not stop the bleeding and should only be carried out under close surveillance.
Bleeding from the stomach or intestinal tract, in the lungs or other organs, is an uncommon feature of haemophilia. If it occurs, it is usually as a result of some other problem and medical advice should be sought promptly.
A bad knock on the head to a person with haemophilia should be regarded as potentially serious and the person should be taken to the hospital immediately. The haematologist should be contacted. If untreated, a haemorrhage inside the skull may occur which could result in brain damage or even death. Careful observation in hospital for symptoms of drowsiness, dizziness, irritability, nausea and/or vomiting, dilated or unequal pupils, headache or mental confusion associated with any head injury is important. This is an indication that immediate attention is essential.
If trauma to the eye occurs, treatment must be sought immediately.
Neck and throat haemorrhages
If treatment is not sought immediately, these are potentially dangerous as the swelling can obstruct the air passages. Tonsillitis, sore throat or severe coughing associated with bronchitis or whooping cough may precede a throat bleed. However, bleeding in these areas may be mistaken for mumps, so care in diagnosis is needed.
Nose bleeds (epistaxis)
Nose bleeds are sometimes spontaneous and are often associated with a ‘cold in the head’, hay fever, nasal polyps or just blowing the nose too hard. Firm pressure applied to the soft part of the nostril and, if desired, an ice pack to the bridge of the nose for 5 minutes by the clock, may be sufficient to stop the bleed. The person must sit forward, if able, while holding the nose, so that any blood going down the back of the throat will be spat out and not swallowed. If bleeding persists, however, professional packing, cautery or replacement therapy must be sought.
Last Reviewed: 24 May 2007