Haemophilia is a blood disorder where the blood does not clot properly. It can lead to a person bleeding severely from even a minor injury.
World Haemophilia Day, on Sunday April 17, was marked by Red Cross War Memorial Children’s Hospital.
The condition is caused by a mutation, or change, in one of the genes that provide instructions for making the clotting factor proteins needed to form a blood clot. This essential blood-clotting protein is called factor VIII (8), also known as anti-haemophilic factor.
Dr Helder de Quintal, a paediatric haematology and oncology sub-specialist at the children’s hospital, says more awareness of haemophilia is needed.
“Increasing awareness will improve diagnoses and will help provide equitable and sustainable access to care specifically for those in developing countries with limited access,” he says.
Cayden Solomons, 5, was diagnosed with severe haemophilia A, shortly after birth. His uncle also has the hereditary condition.
Severe haemophilia A, also called factor VIII deficiency or classic haemophilia, occurs in individuals with less than 1% of normal factor VIII clotting activity.
It means that Cayden and others with haemophilia A bleed longer than other people. Bleeds can occur internally, into joints and muscles, or externally, from minor cuts, dental procedures, or injuries.
Cayden’s mother, Jamie Solomons, says raising a child with haemophilia can be very stressful. “Cayden is very active and loves to explore. I always feel like I need to wrap him up in bubble wrap because I’m so afraid of him injuring himself.”
Although no cure exists for haemophilia, doctors can successfully treat the condition. Treatment focuses on replacing the missing protein and preventing complications and involves giving or replacing the clotting factors that are too low or missing.
Ms Solomons says it is hard watching her son undergoing treatment because he has very difficult veins so he gets pricked more than once. “Seeing my child cry in pain always breaks my heart, but I know it’s something he needs.”
She is grateful for the support given by her family and the children’s hospital. “There are days you will feel so helpless seeing your child injured, or admitted into hospital, so it’s always good to have that family support.”
Ms Solomons has some tips for parents of children with haemophilia. “Getting treatment as soon as possible is important to help reduce the risk of damage to joints, muscles, and other body parts. If you suspect your child is injured, take them to hospital. I’ve personally been there many times and 30% of the time it wasn’t an injury, but I always say ‘rather safe than sorry’.”
Head of the blood and cancer service at the children’s hospital Professor Alan Davidson strongly agrees with Ms Solomons. “There are two main types of haemophilia: haemophilia A is four times more common than haemophilia B. Of those, more than half of people with haemophilia A have the severe form,” he says.
Professor Davidson says haemophilia affects all races and ethnic groups but occurs almost exclusively in males due to the hereditary nature of the condition.
There are several symptoms to look out for:
- Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work.
- Many large or deep bruises.
- Unusual bleeding after vaccinations.
- Pain, swelling or tightness in your joints.
- Blood in the urine or stool.
- Nosebleeds without a known cause.
- Unexplained irritability in infants.
If you are concerned, you should visit your nearest primary health-care facility for medical advice.