Bleeding Disorders, Clotting Process, Hemophilia

What are bleeding disorders?

In people with bleeding disorders, the clotting process doesn’t work properly. As a result, people with bleeding disorders can bleed for longer than normal, and some may experience spontaneous bleeding into joints, muscles, or other parts of their bodies.

Hemophilia is a bleeding disorder that affects approximately 1 in 10,000 people. People with hemophilia do not have enough clotting factor VIII or IX in their blood. As a result, they can bleed for longer than normal.

The most common bleeding disorder is von Willebrand disease (VWD). It is generally less severe than other bleeding disorders. Many people with VWD may not know that they have the disorder because their bleeding symptoms are very mild. 

Rare clotting factor deficiencies are disorders in which one of several clotting factors is missing or not working properly. Less is known about these disorders because they are diagnosed so rarely. In fact, many have only been discovered in the last 40 years.

Finally, inherited platelet disorders are conditions in which platelets don’t work the way they should, resulting in a tendency to bleed or bruise.

The clotting process

Clotting factors are proteins in the blood that control bleeding. When a blood vessel is injured, the walls of the blood vessel contract to limit the flow of blood to the damaged area. Then, small blood cells called platelets stick to the site of injury and spread along the surface of the blood vessel to stop the bleeding.

At the same time, chemical signals are released from small sacs inside the platelets that attract other cells to the area and make them clump together to form what is called a platelet plug.

On the surface of these activated platelets, many different clotting factors work together in a series of complex chemical reactions (known as the coagulation cascade) to form a fibrin clot. The clot acts like a mesh to stop the bleeding.

Coagulation factors circulate in the blood in an inactive form. When a blood vessel is injured, the coagulation cascade is initiated and each coagulation factor is activated in a specific order to lead to the formation of the blood clot. Coagulation factors are identified with Roman numerals (e.g. factor I or FI).

Drugs that can cause bleeding

People with bleeding disorders should avoid aspirin and other drugs that can cause bleeding. This list of drugs that can cause bleeding does not include drugs that have to be given as injections or intravenous infusions. The generic or “chemical” names are given since the brand names or marketing names differ between countries.

While every effort has been made to include as many drugs as possible, some may be missing. In addition, many non-prescription medications, such as cold remedies, contain aspirin. People with bleeding disorders should check with their hemophilia centre or physician, or consult the pharmaceutical company’s printed instructions before taking any new medication.

Aceclofenac 

Acenocoumarol 

Acetylsalicylic acid (Aspirin) 

Citalopram* 

Clopidogrel 

Dexibrufen 

Diclofenac 

Dicoumarol 

Escitalopram 

Fluoxetine* 

Fluvoxamine* 

Ibuprofen 

Indomethacin 

Ketoprofen Ketorolac  

Lornoxicam 

Meloxicam 

Nabumetone 

Naproxen 

Paroxetine* 

Phenprocoumon 

Piroxicam 

Sertraline* 

Sulindac 

Tenoxicam 

Ticlopidine 

Warfarin

*These antidepressants have a mild inhibiting effect on the platelet function, which may increase the bleeding tendency. However, since they are less harmful than most other antidepressants in other respects, it is possible to try the medication by starting at a low dose and carefully increase it. Many patients will tolerate these drugs well.

Herbal drugs (phytomedicines) that can cause bleeding

While every effort has been made to include as many herbal drugs as possible, some may be missing. Some herbal medicines have been reported in association with bleeding, but in these cases the patient also took regular drugs that could have caused the bleeding or that the documentation in other respects was weak. These have not been included in this list.

People with bleeding disorders should check with their hemophilia centre or physician, or consult the pharmaceutical company’s printed instructions before taking any new herbal drug.

Ginkgo biloba 

Garlic in large amounts 

Ginger (not dried ginger) 

Ginseng (Asian) 

Feverfew 

Saw Palmetto (Serenoa repens) 

Willow bark

Prepared by Sam Schulman, MD

What is prophylaxis?

Prophylaxis is the regular infusion of clotting factor concentrates in order to prevent bleeding.

The idea of prophylaxis came from the observation that people with moderate or mild hemophilia (who have clotting factor levels of 1% or more) rarely experience spontaneous bleeding. They also have less joint damage than people who have severe hemophilia.

Did you know?

Prophylaxis will not help repair joints that are already damaged.

However, it will decrease the frequency of bleeding,may slow progression of joint disease, and may improve quality of life.

Doctors believed that if they could keep minimum factor levels around 1% with regular infusions of clotting factor concentrates, they may reduce the risk of bleeding and prevent joint damage.

Since then, important studies have shown that children who receive prophylaxis do have fewer bleeds and healthier joints.

Prophylaxis is now the goal of treatment for people with severe hemophilia, allowing them to remain active and participate more fully in daily life.