Certain people or injuries bleed more than others. For those with hemophilia, bleeding is not a trivial episode or a simple incident.
A few words about hemophilia
Normally, when someone is injured, clotting helps form a blood clot to avoid excessive bleeding. However, for people who have hemophilia, this process fails.
Coagulation results in the activation chain of several factors called coagulation factors that ultimately form an effective blood clot. In hemophiliacs, one of these factors is missing or is present, but in a smaller amount. This prevents the coagulation chain from continuing to the end of the process, and the clot does not form. Therefore, the blood remains thinner and the wound bleeds for a longer period.
There are two main types of hemophilia: A and B. A different clotting factor is defective depending on the type, but the result is the same. This disorder is not common, 1 out of 5000 newborn boys is affected by it. Other rarer types also exist.
What role does genetics play in hemophilia?
Genetics is directly linked to hemophilia. The ability to complete the clotting process is in our genes, and it is our parents who pass on intact or mutated genes to us.
This disorder is passed on almost exclusively to boys. However, girls can be carriers without showing any signs of the disease. Therefore, mothers can pass it on to their sons. In very rare cases, and in specific situations, a girl can have hemophilia.
It should be noted that, in one third of cases where hemophilia is diagnosed, a genetic component is not found. This means that the defective gene mutated at the time of conception.
What are the risks associated with this disorder?
Because the blood of hemophiliacs is thinner, the disorder generally manifests itself by more significant bleeding as well as by other symptoms:
- bleeding in the joints
- painful, warm and red joints
- muscle pain
- bleeding in the mouth during dental work
- blood in the urine
- prolonged nose bleeds
- prolonged bleeding during a surgical procedure, and
- intracranial hemorrhage
Bleeding caused by an accident can be dangerous for hemophiliacs, because they don’t have what is needed to stop the bleeding quickly. However, a minor cut is often without consequence, aside from the fact that they will bleed for a longer period. It is an injury related to internal bleeding, such as bleeding in the joints, muscles or soft tissue that can have the most serious consequences. When bleeding occurs in a vital organ, such as the brain, this can be life-threatening.
The severity of the disorder varies according to the concentration of the defective coagulation factor in the blood. When the concentration is very low, the risk of bleeding is higher. When it is moderate, the disorder isn’t as serious.
Can hemophilia be treated?
There is no cure for hemophilia. The first-line treatment consists of replacing the defective coagulation factor intravenously. This can be done preventively or when bleeding occurs. Various medications can be taken to prevent or stop minor bleeding. Speak to a healthcare professional such as your doctor or pharmacist for additional information about the treatment of hemophilia.
Do you have hemophilia? You may find the follow tips to be useful or they could also provide information if a family member is affected by it.
- Avoid activities presenting a risk of injury.
- Apply ice to all wounds promptly.
- Tell the people around you about your health condition, including school or childcare staff. Inform them of the risks of bleeding and the measures that should be taken if this occurs.
- Get vaccinated against hepatitis A and B.
- Avoid using medications that can thin the blood, such as anti-inflammatory drugs, without your doctor’s approval.
- Avoid receiving intramuscular injections whenever possible.
- Always wear a MedicAlert® bracelet. This will be very useful for medical staff in the event of an accident.
- Speak to a specialist from a clinic specialized in hemophilia or clotting disorders for additional tips or information.
- Consult a doctor or go to emergency room after any potentially serious injury.
Hemophilia requires great vigilance because it presents a serious bleeding risk.
Don’t hesitate to speak to your pharmacist if you have any questions about medications or health.