2023-04-17 16:32:55
World Hemophilia Day is commemorated every April 17. This disease unknown to many affects more than a million people in the world.
World Hemophilia Day is a day to raise awareness about this still incurable disease that affects 1 in 5,000 male babies born, according to data from the Centers for Disease Control and Prevention (CDC). The World Federation of Hemophilia was the institution that promoted this dissemination and prevention action.
Since 1989, April 17 is the day chosen for being the birth of Frank Schnabel, founder of the Federation. This man from Canada was born with hemophilia type A and founded a non-profit organization in 1963 to improve the treatment of the disease.
Sadly, Schnabel passed away in 1987.. The reason was the HIV infection that she contracted by receiving blood transfusions that were contaminated with the AIDS virus. This highlighted the problem faced by hemophilia patients around the world, who were receiving biologics that were not properly processed.
What is hemophilia?
Hemophilia is an inherited disease. This means that it is passed from parent to child through genetics.
In this particular case, the transmitted disorder affects blood coagulation. Patients often have deficiencies in some coagulation factors. These are proteins that circulate in the blood and contribute to the formation of clots.
World Hemophilia Day reminds us of the stories of those who had the disorder and died from minor injuries that led to profuse bleeding. Before the understanding of the clotting mechanism, it was a mystery to science why certain men could not stop their bleeding naturally.
The genetic mutation behind the disease is located on the X sex chromosome. Since women have two copies of this chromosome and men only one, they are usually carriers and they are the ones who manifest the symptoms.
Female carriers may have mild symptoms of hemophilia. In addition, one third of male patients have no family history.
There are two types of hemophilia:
- Type A: It is the one that affects coagulation factor VIII. Most of the diagnosed cases belong to this category.
- Type B: the affected one is the factor IX of the coagulation. Also Known As christmas sickness.
Apart from the classification by types, each patient is framed in a level of severity of the affectation. This depends on the concentration of the affected clotting factor in the blood:
- Light: These are people who have up to 50% normal concentration of the factor in question.
- Moderate: these patients only achieve a 5% normal clotting factor in their blood.
- Grave: the most severe form of clinical presentation occurs because there is less than 1% normal factor.
What are your symptoms?
While World Hemophilia Day raises awareness about the disorder and its symptoms, It is still a disease unknown to the majority of the world’s population.. Those who know something about it know that these are people at high risk of bleeding. However, they are not always visible.
Patients with hemophilia suffer internal bleeding that can be dangerous, since it is not detected in time. Joints can be affected, with blood pooling inside the knees or elbows, for example.
Blood can also accumulate within the muscles. This situation constitutes a challenge for doctors, as they comment in the publication of Seminars in thrombosis and hemostasis. Intramuscular bleeding can go unnoticed until it generates excessive pressure, which can affect the nerves that pass through the area with the hemorrhage.
Preventing bleeding is the key to living with hemophilia
According to the National Hemophilia Foundation in the United States, there are about 30,000 people living with the disorder in the United States. Recent estimates made by teams of researchers place the worldwide prevalence at more than 1,100,000 men. This is more than double the estimate that was previously made, which put the total cases on the planet at 400,000.
This large population group you should follow certain recommendations to reduce the risk of life-threatening bleeding. Thus, World Hemophilia Day is also an opportunity to remember these life-saving practices.
next to the doctor
Hemophilia patients they have to learn how to administer clotting factor correctly, which is the main treatment, as stated in the WFH clinical guidelines. It is also essential to receive training on how to stop external bleeding, should it occur.
In any case, in the event of bleeding, patients must attend a health center. First aid is effective, but the professional approach cannot be delayed, since we are dealing with abundant bleeding.
On the other hand, patients with the disorder cannot take any medication for common ailments, such as a headache. Non-steroidal anti-inflammatory drugs (ibuprofen, for example) are able to affect coagulation and worsen ongoing bleeding.
The alternative is usually paracetamol.
With the dentist it is necessary to review the correct method of tooth brushing and flossing. The gums are susceptible to bleeding due to a poor oral hygiene technique. This can lead to the person reducing the frequency of their dental care routine. In the long run, beyond bleeding, poor hygiene will lead to cavities, gingivitis and periodontitis.
Finally, together with the treating physician, the benefits of carrying out a family genetic study must be evaluated. In this way, it is possible to know the risk of transmitting the mutation to the children. If you do not have children, it will be important information to make a responsible decision about paternity.
in daily habits
During World Hemophilia Day, health organizations remind patients that there are positive habits to prevent bleeding. Diet, exercise and precautions when doing leisure activities are the key.
Bleeding causes loss of iron and, with it, increases the risk of iron deficiency anemia. It is optimal to have dishes that provide the micronutrient in usable amounts for the body, prioritizing fat-free red meat and green vegetables.
Physical activity must be carried out, but always avoiding contact and trauma situations. Hemophilia patients will have to opt for low-impact exercises that protect the joints. Swimming seems the right choice. Cycling, other than mountain biking and in controlled environments, can be practiced, as long as protection elements are used.
When traveling, for work or pleasure, a few prior considerations will reduce risks and complications. It is useful to carry an identification in the luggage and in the wallet, with the description of the disease and the medication that is usually taken.
Before leaving, locate the health centers that will be at your destination.
World Hemophilia Day: hope for the future
A recent study published in The New England Journal of Medicine demonstrated favorable results in the treatment of type B hemophilia. Gene therapy seems to open the doors to possibilities unthinkable a century ago.
The hope of a better approach and even of a cure through DNA manipulation exist as palpable realities. Nothing has been said, but we are not at the beginning of the path either. There is hope for the not so distant future.
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