Every parent wishes for their child to be healthy and happy. But, sometimes, unexpected medical conditions can emerge, presenting challenges that require awareness and understanding.
Haemophilia, a rare blood disorder affecting clotting, is one such condition that can manifest early in life.
It’s essential to understand haemophilia. Simply put, haemophilia is a condition where the blood doesn’t clot properly. If most of us get a cut, our blood quickly clots to stop the bleeding.
For someone with haemophilia, the clotting process doesn’t work as it should, leading to prolonged bleeding from even minor injuries.
Some of the common symptoms associated with haemophilia among infants and toddlers include excessive bruising that lasts for weeks, mouth bleeds and bleeding into joints, soft tissues and muscles.
As the global community observed World Haemophilia Day on April 17, under the theme “Equitable access for all: recognising all bleeding disorders”, the Gauteng Department of Health wishes to educate the public about the inherited blood disorder, its symptoms and treatment options.
There are two main types of haemophilia. The most common type, Haemophilia A, happens because of a lack of clotting factor VIII, while Haemophilia B is caused by a shortage of clotting factor IX.
Haemophilia A is rare, affecting one in 5 000 male newborns across the world. According to a 2022 report by the World Federation of Hemophilia, 2009 males have Haemophilia A and 395 Hemophilia B in South Africa.
Haemophilia B, colloquially known as Christmas disease, happens due to a lack of clotting factor IX. Haemophilia B occurs in around one in every 25 000 males born worldwide. Is a rare condition that makes it hard for blood to clot properly.
It’s a condition passed down through families, typically affecting men more than women.
Interestingly, about 30% of boys diagnosed with haemophilia don't have a known family history of the condition, but their mothers are likely to have a male relative on their side of the family who had it.
At the Red Cross War Memorial Children’s Hospital, Dr Helder de Quintal, a paediatric haematology and oncology sub-specialist, spoke about haemophilia awareness on World Haemophilia Day.
By increasing awareness, diagnoses will be improved and access to care more equitable and sustainable for developing countries where access to care is limited.
“I encourage patients, parents and health-care workers to raise awareness on this day through social media platforms in order to encourage continued partnerships, progress, policy and access for all,” said De Quintal.
Who is affected?
Haemophilia affects all races and ethnic groups but occurs almost exclusively in males due to the hereditary nature of the condition.
Although haemophilia is hereditary and can be tested for, there are some 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 joints.
- Blood in the urine or stool.
- Nosebleeds without a known cause.
- In infants, unexplained irritability can also be an indication
For someone with haemophilia, a simple injury can result in prolonged bleeding because their blood lacks the necessary components to form a strong clot.
While minor cuts might not pose a significant risk, bleeding into joints or internal bleeding can be serious and require medical attention.
“Early treatment of haemophilia will stop bleeding, minimise long-term complications and can save lives. The delay in treatment of a patient with a blood disorder may be life or limb- threatening.
“Life-threatening bleeds include, among others, in the head and neck, chest, abdomen, pelvis or spine, Iliopsoas muscle and hip, fractures or dislocations and any deep lacerations,” a Gauteng Department of Health statement read.
The Health Department has set up four special centres to help haemophiliacs. The centres are in big hospitals such as Charlotte Maxeke, Chris Hani Baragwanath, Steve Biko, and Dr George Mukhari Academic Hospitals.
To make sure haemophiliacs get the help they need right away, the Health Department has teamed up with the Hemophilia Foundation.
Together, they have trained nurses in clinics and smaller hospitals. The training helps nurses spot signs of haemophilia early, so they can send patients to the big hospitals for proper care.