Haemophilia

Haemophilia is an inherited bleeding disorder that prevents the blood from clotting properly. The affected gene can be passed on from a man with haemophilia or a woman who carries the gene.

Signs and symptoms?

• Bruises
• Cuts and grazes
• Nose bleeds
• Mouth or tongue bleeds
• Joints and muscles
  • Pain or a ‘funny feeling’
  • Swelling of the affected joint or muscle
  • Heat or warmth felt over the joint or muscle
  • Loss of movement
  • Protecting or not using the affected limb.

How is the condition diagnosed?

A diagnosis of haemophilia happens when someone has excessive bleeding or bruising and/or a family history of a bleeding disorder. Clotting factors are proteins in the bloodstream that allow blood to clot. Factor replacement is the medication (protein taken from blood or made synthetically) that replaces a missing clotting factor.

The diagnosis of haemophilia A (low factor VIII (8) level) or haemophilia B (low factor IX (9) level) is confirmed by sending blood to the laboratory to measure the clotting factor levels.

Treatment

Use first aid (RICE):

• Rest – rest the limb.
• Ice – apply ice to affected area to reduce swelling and pain for 10 – 15 minutes at a time.
• Compression – wrap the injured area with an elastic bandage to help slow the bleeding. Make sure the bandage is not uncomfortably tight.
• Elevation – place the injured limb at a level higher than the heart to help slow down bleeding.

Bruises

Bruising is a part of childhood and the majority of superficial bruising rarely needs treatment. The changing colours of the bruise represent the healing process. Bruises may only be a problem if they are quite painful or becoming more raised (swelling up and away from the skin).

Cuts and grazes

Superficial cuts and grazes will not need factor (the chemical that helps your blood clot) replacement. Clean the cut, apply pressure and cover with a Band-Aid. Deep cuts or those that might need stitches will need a visit to the emergency department and possible treatment with factor replacement concentrate.

Nose bleeds

These should normally stop by applying firm pressure to the bleeding nostril for 10 – 15 minutes. An ice pack can also be put on the bridge of the nose for five minutes whilst sitting the child upright.

If bleeding continues after 20 minutes of continuous firm pressure, then please go to your nearest emergency department.

Mouth or tongue bleeds

Bleeding in the mouth is harder to manage as any clots that form to stop the bleeding are dislodged by the tongue, food, or washed away by the saliva. Sucking an ice cube may help, but bleeding in the mouth will need treatment at the emergency department and advice from the haemophilia team.

Joints and muscles

Young babies or children may be miserable and/or crying for no obvious reason, they may protect a limb by limping or not using it. Older children should be able to tell you if they are having a bleed. The most common sites for a joint bleed are: shoulder, elbow, wrist, hip, knee and ankles.

Key points to remember

Early treatment with factor is important but it is also important to properly rest the affected limb or joint. The haemophilia physiotherapist can assist you with this.

Do not give aspirin or aspirin-containing products, Nurofen, Ibuprofen or other non-steroidal anti-inflammatory drugs to a child with Haemophilia. Give only paracetamol (Panadol).

Contact the Haemophilia Centre for advice on treatment for any of the above signs of a bleed.

When to seek help

See your GP if your child has any common symptoms.

In an emergency, call Triple Zero (000) and ask for an ambulance.

If you're not sure whether to go to an emergency department, call 13 HEALTH (13 43 25 84) and speak to a registered nurse.

References

• Haemophilia Foundation Australia (HFA) (2013) Haemophilia Booklet (PDF)
• Haemophilia Foundation Australia (HFA) (2012) Mild Haemophilia Fact Sheet (PDF)

Developed by the Haematology Department, Queensland Children’s Hospital. We acknowledge the input of consumers and carers.

Resource ID: FS343. Reviewed: April 2019.

Disclaimer: This information has been produced by healthcare professionals as a guideline only and is intended to support, not replace, discussion with your child’s doctor or healthcare professionals. Information is updated regularly, so please check you are referring to the most recent version. Seek medical advice, as appropriate, for concerns regarding your child’s health.