Wounds and injuries are an inevitable part of life. The good news is, our body is perfectly equipped with an amazing blood clotting system that can change blood from a liquid state to a solid state. This allows our wounds to heal easily if we are physically hurt.
The protein in our blood that performs this “magic trick” is called platelets. When a wound occurs, a blood vessel is essentially injured. Platelets will gather and stick at the site of the injury before spreading along the affected area in order to stop the bleeding.
At the same time, a chemical signal is also sent to other cells to lend a helping hand in the clotting process. This is called coagulation.
However, there is a relatively rare blood disorder which affects the blood clotting process called haemophilia. According to The World Federation of Haemophilia, as of 2012, an estimated one in 10 000 people are diagnosed with this condition.
What is Haemophilia?
What exactly is haemophilia and what are its effects? This blood disorder, which is usually genetically inherited, contributes to an extremely long clotting process. This means a wounded person with haemophilia will bleed for far longer than someone who is not afflicted with this condition.
Haemophilia can be inherited from either parent but a new mutation can sometimes occur — affecting a child whose parents are not carriers of the haemophilia gene.
There are several types of haemophilia but generally the spotlight falls on two of the most common forms namely Haemophilia A and Haemophilia B.
Haemophilia A is the most widespread version and is found in 80 percent of all those who are diagnosed. Haemophilia A is also known as classic haemophilia. A person diagnosed with Haemophilia A is someone who does not have enough clotting factor 8 in their genes to help with the coagulation process.
Haemophilia B is known as Christmas disease (named after Stephen Christmas who is the first patient diagnosed with this disease). Haemophilia B is diagnosed in a person who does not have enough clotting factor 9 to contribute to a healthy blood coagulation process.
There are also different levels of severity when it comes to this blood disorder. A person who does not experience any obvious symptoms until a wound appears (such as from a dental procedure or an injury) is considered to have mild haemophilia. On the other hand, moderate to severe haemophilia presents symptoms such as irritation in the joints, blood in the faeces or in the urine and unexplained bleeding of the gums.
Symptoms of Haemophilia
If you have a family history of haemophilia, you should be extra vigilant and look out for signs and symptoms related to this condition.
Be aware that these may not occur immediately. Some people experience symptoms from infancy, while others experience them only much later in life. Generally, symptoms include:
- Prolonged bleeding from cuts, injuries or after a surgery
- Multiple, large bruises
- Blood in the urine or stools
- Internal bleeding which causes joint pain and swelling
- Frequent nosebleeds with unknown causes
There are also some emergency signs and symptoms to look out for which may need swift medical attention. If you have been diagnosed see a doctor immediately if you:
- Have an injury or wound
- Experience extreme fatigue
- Endure painful, lasting headaches
- Have recurrent double vision
Babies and Haemophilia
If haemophilia is inherited, a person can experience its signs and symptoms at any stage of life — from infancy, right through to the golden years.
The good news is, if either parent has this blood disorder, there are various tests that can reveal if the foetus or infant has haemophilia. These include a blood test or a blood sample from the umbilical cord.
If there is no family history of haemophilia the infant is likely to remain untested as the chances of having this disorder are significantly lower. However, do keep in mind that this disease can form new mutations so look for signs and symptoms that regularly occur in children such as:
- Easy bruising
- Prolonged bleeding following circumcision among baby boys
- Excessive or unusual bleeding during teething
- Swollen joints or bruises when learning to walk
Fortunately, most babies have limited mobility during their infant years, which decreases the chances of getting injured. If you spot any of the symptoms, be sure to get in touch with a medical expert for the next course of action.
Treatment for Haemophilia
Thanks to advances in medicine and technology, there are now several types of safe, efficient treatments that can help those with haemophilia.
Firstly, it is important to take note that treatment options depend on the type of haemophilia that has been diagnosed as well as its severity. Each treatment needs to be individually tailored to ensure that symptoms are managed well.
Here are some of the common treatment options:
Clotting factor – Given intravenously (through a needle in your vein), clotting factors include blood plasma which has been donated or synthetic plasma which has been DNA-derived.
Medication – Medication is available for those diagnosed with a mild form of haemophilia. This medication helps by increasing the concentration of a specific, insufficient factor in the blood such as factor 8 for patients diagnosed with Haemophilia A.
Other forms of treatment that help to manage health problems associated with haemophilia — such as bleeding joints — include making lifestyle adjustments like careful monitoring of daily activities to prevent injury or internal bleeding.
Complications Related to Haemophilia
If left unmanaged, haemophilia can lead to excessive and frequent blood loss which can cause a series of health complications and even death. Some of the possible complications that may be presented along with haemophilia include:
- Severe anaemia from blood loss
- Blood in the urine which can cause severe pain
- Bleeding in the digestive system
- Internal bleeding in the head
Although haemophilia is a relatively rare disorder, it is still important to take note of this disease and be on the look out for symptoms. With the right treatment and lifestyle modifications, haemophilia can be managed and life-threatening health problems can be avoided.