It is essential, for those who suffer from it, to always have Factor VIII available. Today, there is a wide range of formulations.
Some things take little, but that little must not be missing. This is the case of coagulation Factor VIII, an essential protein to regulate the complex cascade of biochemical reactions that allows the blood to remain fluid enough to flow through veins and arteries, but to coagulate quickly in case of internal microbleeding or small and medium-sized wounds (a cut to a finger, a peeling to the knee or a common dental intervention, etc.).
When Factor VIII is scarce, hemophilia type A (the most common form) sets in, a hereditary genetic disease that affects only male individuals and determines a marked propensity to develop bleeding and hematomas, even serious.
1 percent is the level of Factor VIII that marks the dividing line between normal (or almost) and impaired clotting. Above this threshold, there are no problems. Below, hemophilia appears.
The Key Factor
Until a few decades ago, suffering from haemophilia could lead to significant limitations in daily life as well as the high risk of long-term complications, especially at the joint level (hemophilic arthropathy).
Although even in hemophiliacs, small cuts and superficial scratches sometimes healed spontaneously, subjects with severe untreated forms had to try to avoid as much as possible activities at risk of falling, injuries and trauma.
They also had to do everything not to subject muscles and joints to excessive stress, or with sudden or repeated movements, or overloading them with excessive weights.
In fact, the microlesions that commonly occur in these circumstances at the level of the small blood vessels that supply muscles and joints (harmless to healthy people), in subjects with a level of Factor VIII less than 1 percent can continue to bleed for a long time, causing the formation of hematomas of considerable size and “hemarthrosis”, ie accumulations of blood inside the joint sheaths that cause swelling, intense pain and difficulty moving.
While superficial hematomas generally heal without leaving sequelae, the recurrence of hemarthroses affecting the same joint can determine the development of permanent deformities and functional disability.
Therapy on demand
To prevent these complications it is essential to intervene quickly by administering an adequate amount of Factor VIII, through intravenous injections every time a hemarthrosis occurs.
The same type of treatment is necessary in the presence of bleeding caused by wounds of a certain importance or during surgery.
It is what is called demand therapy, because it is implemented after the damage to the blood vessels has already occurred, and is indicated in hemophiliac subjects who have few bleeding episodes per year and in those who have levels of Factor VIII not too low (2-5%).
Prophylaxis
For those who tend to develop haemarthrosis, hematomas or bleeding more frequently (from once a month to 2-3 times a week) the possibility of undertaking a prophylaxis, based on periodic long-term intravenous administration of Factor VIII (usually, three times a week, every other day, with a break) should be evaluated together with the doctor. regardless of the occurrence of trauma or acute bleeding.
This strategy represents a real cure of hemophilia because, by bringing the level of Factor VIII above 1 percent, it effectively eliminates the disease.
A small residual risk of bleeding persists during activities involving a high probability of trauma or bleeding (activities characterized by a high probability of trauma, surgery, etc.): in these cases, the administration of Factor VIII can be planned on a daily basis.
When the child is small, Factor VIII is usually administered by the parents, but the ideal is that he is accustomed little by little to become familiar with intravenous injections and begins (generally around 10-12 years) to administer the therapy alone.
In this way, he will soon become independent and will also feel more secure.
To each his dosage
The dosage of Factor VIII is decided by the doctor of the haemophilia center depending on the case and according to a precise treatment plan.
The patient is asked to keep track of infusions made via an infusion diary.
Today there are several formulations on the market that cover the entire range of possible dosage needs, from a minimum of 250 IU up to a maximum of 3,000 IU to be reconstituted in a reduced volume.
Some products also have an advanced reconstitution system without exposure of needles or sharp parts, potentially dangerous for those suffering from hemophilia.
In addition, some formulations of recombinant Factor VIII are also able to withstand outside the refrigerator, as long as the temperature does not rise above 25 ° C.
This is an undoubted advantage because it allows you to always carry the drug with you, without worrying about its storage.
Joycelyn Elders is the author and creator of EmpowerEssence, a health and wellness blog. Elders is a respected public health advocate and pediatrician dedicated to promoting general health and well-being.
The blog covers a wide range of topics related to health and wellness, with articles organized into several categories.