What is a bleeding disorder?
A bleeding disorder is present when the blood does not clot properly. Blood clotting is a complex process that involves as many as 20 different plasma proteins. All clotting proteins and platelets need to work effectively and in the correct order to form a proper clot that is strong enough to stop bleeding. A bleeding disorder occurs when the body is unable to activate the entire clotting cascade correctly.
The body initiates the clotting cascade when an injury occurs. At a high level, coagulation works as follows:
Blood vessels constrict - When an injury occurs, the blood vessel at the site of injury contracts to limit the flow of blood to the damaged area.
Platelet plug forms - Within seconds of the injury occurring, small cells found in the blood, called platelets, spread on top of the injured vessel and release chemical signals to attract other cells circulating in the blood. Some of the cells attracted are clotting factors. The first factor to be activated during the clotting cascade is the von Willebrand factor. The vWf is a glue like protein that forms a plug that prevents blood from flowing to the site of injury.
Fibrin clot is created - Thirteen clotting factors (I-XIII) along with calcium, vitamin K and other proteins work together in a series of complex chemical reactions to form a fibrin clot. The fibrin clot acts as a net over the wound. Depending on the size of the injury, the fibrin clot strengthens over several days or weeks, and when the wound is healed, the fibrin clot dissolves.
If any of the steps in the clotting cascade don’t work properly or are deficient, the person bleeds longer and, therefore, has a bleeding disorder. The type and severity of the disorder is determined based on the protein that is missing from the blood and how much of it is missing.
What is Hemophilia?
Hemophilia is a rare bleeding disorder classified by abnormal blood clotting or the absence of blood clotting. It ranges from mild to severe, and if not properly treated can be very dangerous to those with the disease, as undetected internal bleeding and bleeding in the joints can damage organs and tissues.
Hemophilia is very rare. Only about 20,000 Americans have the disorder. It affects mostly males, as it is an X chromosome linked condition. However, under rare and specific circumstances it can occur in a female. Hemophilia affects 1 in 5,000 male births in the US, approximately 400 babies are born with hemophilia each year. All races and economic groups are affected equally. People who have hemophilia and access to factor replacement therapy have a normal life expectancy.
The Different Types of Hemophilia
Bleeding disorders are treated by determining what protein is missing in the blood. Hemophilia is the most common and is classified as such:
Hemophilia A – Occurs when factor VIII levels are deficient. Also known as “classic hemophilia.”
Hemophilia B – Occurs when factor IX levels are deficient. Also known as “Christmas disease.”
Hemophilia C – Occurs when factor XI levels are deficient.
Causes of Hemophilia?
Most hemophiliacs are born with the disorder. It is caused by a defect in one of the genes that determines how the body produces clotting factor. The gene that determines clotting factor production is located on the X chromosome, which is why it’s so rare for a female to be born with hemophilia. She would have to have the defective gene in both X chromosomes. If it is only on one, she is considered a hemophilia carrier. This means she may pass it on to her offspring, but may not develop symptoms herself.
Signs & Symptoms
A person with hemophilia can bleed internally or outside the body. People with hemophilia do not bleed more than people without hemophilia, they just bleed longer. The most common types of bleeds are into the joints and muscles.
Other symptoms include, but are not limited to:
At Cottrill’s we remain focused on providing outstanding care for patients with bleeding disorders, including the coordination of nursing services and supplies.
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