Bleeding disorders, a collection of conditions that prevent blood from clotting correctly, can be life-threatening. They’re caused by issues such as aberrant platelets, abnormal clotting proteins, or problematic blood vessels. Understanding how blood clots normally can help elucidate why these disorders are so serious.
In a standard clotting process, platelets, a type of blood cell, act as first responders to an injury site. These platelets work in conjunction with clotting factors to form a fibrin clot, a sort of gel-like plug that keeps platelets in place. This clot serves two main purposes: it promotes healing and prevents blood loss. While over-clotting can cause strokes or heart attacks, under-clotting can lead to excessive, potentially life-threatening bleeding. Common bleeding disorders are haemophilia and the Von Willebrand Disease.
Haemophilia: A Deep Dive
Haemophilia is a prime example of a bleeding disorder, with over 815,000 estimated global cases and 1 in 5,000 male births affected in the U.S. alone. Those with haemophilia have insufficient clotting factors, making them prone to dangerous internal bleeding.
Depending on which clotting factor is lacking, haemophilia can be classified as:
- Haemophilia A – where there is a lack of clotting factor VIII.
- Haemophilia B – stemming from a lack of clotting factor IX.
It’s important to note that haemophilia affects more males due to its genetic nature related to the X chromosome. Early diagnosis is pivotal, as is genetic testing during pregnancy, which can help parents prepare for a child affected by this disease.
While there is no cure for haemophilia, with proper lifelong care those affected can lead normal lives.
Von Willebrand Disease (VWD)
VWD is another crucial bleeding disorder, where patients either lack sufficient von Willebrand protein or have a non-functional variant. Although incurable, most cases are mild, and with appropriate care and an early diagnosis, those affected can lead active lives. VWD is notably prevalent among women, especially as noticeable symptoms such heavy menstrual bleeding make the disease easier to diagnose.
The most efficient treatment for bleeding disorders is factor replacement therapy. This involves injecting patients with clotting factor concentrates, available in two primary types:
- Plasma-derived Factor Concentrates: Sourced from human plasma.
- Recombinant Factor Concentrates: Genetically engineered.
The clotting factors are found in plasma, obtained from the blood and further processed to obtain the clotting proteins. Most of the clotting factors used for the treatment are obtained from human plasma however, in 1992 the U.S. Food and Drug Administration first time approved a genetically engineered recombinant factor VIII.
Safety is paramount when handling these concentrates, ensuring they don’t carry bloodborne viruses.
The Importance of Blood Management Solutions
For those with blood disorders, safe blood products are non-negotiable and companies like B Medical Systems, which specialize in Blood Management Solutions, continue to innovate to ensure that modern therapies are as effective as possible.
Blood Bank Refrigerators guarantee optimal storage temperatures for blood bags while plasma bags are stored safely in either Plasma Storage Freezers or even Ultra-Low Freezers. Moreover, right after the separation of plasma from the blood, it’s essential to freeze it effectively and rapidly to a core temperature of -30°C, a feat achieved using B Medical Systems’ Contact Shock Freezers. Such measures ensure that the clotting factors remain intact, both in quality and recovery rate. The meticulous extraction and storage of fresh frozen human plasma are crucial, serving not just for transfusion but also as raw material for coagulation factor concentrates.
While bleeding disorders can be challenging, advances in medical technology, awareness campaigns, and blood management solutions are making a significant difference in patients’ lives. Proper plasma preservation, in particular, plays a pivotal role in this life-improving mission.