What is the disease where you bleed does not stop? A Comprehensive Guide to Bleeding Disorders

According to the CDC, approximately 1 in 100 people in the U.S. have von Willebrand disease, the most common inherited bleeding disorder. So, what is the disease where you bleed does not stop? This phenomenon is a hallmark symptom of several medical conditions known as bleeding disorders.

Quick Summary

The disease where you bleed does not stop is a medical condition known as a bleeding disorder, such as hemophilia or von Willebrand disease, where the blood's ability to clot is impaired, leading to prolonged and potentially spontaneous bleeding episodes.

Key Points

• Bleeding Disorders: Conditions like hemophilia and von Willebrand disease are the primary diseases where you bleed does not stop due to blood clotting problems.

• Hemophilia: An inherited genetic disorder caused by a deficiency in specific clotting factors (VIII or IX), primarily affecting males.

• Von Willebrand Disease: The most common inherited bleeding disorder, affecting both genders, caused by a low level or defect of von Willebrand factor.

• Internal vs. External Bleeding: Severe forms of these disorders can cause spontaneous internal bleeding into joints and muscles, which is more dangerous than visible external bleeding.

• Diagnosis: Doctors diagnose bleeding disorders through blood tests that measure clotting times and the levels of specific clotting factors.

• Management: Living with a bleeding disorder involves medication (factor replacement), avoiding certain drugs like aspirin, and managing a safe, active lifestyle.

• Vitamin K Deficiency: While less common in the general population, a lack of vitamin K can cause serious bleeding issues, especially in newborns.

The Mechanism of Normal Blood Clotting

To understand bleeding disorders, one must first grasp the basic process of hemostasis, or blood clotting. When a blood vessel is injured, a complex chain reaction occurs. First, small blood cells called platelets adhere to the site of the injury, forming a temporary plug. Next, a series of proteins known as clotting factors work together to create a stable fibrin clot, which acts like a mesh to reinforce the platelet plug and stop the bleeding. In individuals with a bleeding disorder, one or more parts of this critical process are flawed.

Hemophilia: The Most Famous Bleeding Disorder

Hemophilia is perhaps the most well-known bleeding disorder, though it is one of the rarer types. It is an inherited genetic condition where the blood lacks sufficient clotting factors.

Types of Hemophilia

• Hemophilia A (Classic Hemophilia): The most common type, caused by a deficiency in clotting Factor VIII.
• Hemophilia B (Christmas Disease): The second most common type, caused by a deficiency in clotting Factor IX.
• Hemophilia C: A very rare form resulting from a Factor XI deficiency.

Symptoms of Hemophilia

Symptoms vary in severity depending on the level of clotting factors in the blood. Mild cases may only result in excessive bleeding after major surgery or trauma, while severe cases can cause spontaneous bleeding for no apparent reason.

• Unexplained and excessive bleeding from cuts, injuries, or dental work.
• Large or deep bruises that appear easily.
• Pain and swelling in the joints from internal bleeding.
• Blood in the urine or stool.
• Frequent or prolonged nosebleeds.

Diagnosis and Treatment

Diagnosis involves blood tests to check clotting times and measure specific factor levels. Hemophilia is typically treated by replacing the missing clotting factor, a therapy that has been greatly improved over the years.

Von Willebrand Disease: The Most Common Inherited Disorder

Though less famous than hemophilia, von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting both males and females equally. It is caused by low levels of the von Willebrand factor (VWF), a protein that helps platelets stick together and also carries Factor VIII.

Types of Von Willebrand Disease

VWD has three main types, with varying levels of severity.

• Type 1: The most common and mildest form, characterized by low levels of VWF.
• Type 2: The VWF is present but does not work properly. It is further broken down into several subtypes.
• Type 3: The rarest and most severe form, with very low or absent levels of VWF and Factor VIII.

Symptoms of VWD

Similar to hemophilia, symptoms of VWD can range from mild to severe, depending on the type and severity.

• Frequent and prolonged nosebleeds.
• Easy bruising.
• Prolonged bleeding from minor cuts.
• Heavy or long menstrual bleeding in women.
• Longer-than-normal bleeding after dental work or surgery.

Other Causes of Excessive Bleeding

While hemophilia and VWD are key inherited disorders, other conditions can also cause prolonged bleeding. These include:

• Platelet Disorders: Conditions where platelets are either too few (thrombocytopenia) or dysfunctional.
• Acquired Bleeding Disorders: These can develop later in life due to certain medical conditions, such as liver or kidney disease, or as a side effect of medications, such as blood thinners.
• Vitamin K Deficiency: Vitamin K is crucial for producing several clotting factors, and a severe deficiency can impair clotting. This is a particular risk for newborns, who are given a vitamin K shot at birth to prevent vitamin K deficiency bleeding (VKDB).

Diagnostic Testing for Bleeding Disorders

If a bleeding disorder is suspected, a hematologist will perform a series of diagnostic tests. The initial evaluation typically includes a complete medical history and physical exam.

1. Complete Blood Count (CBC): Measures red and white blood cells and platelets to check for abnormalities.
2. Partial Thromboplastin Time (PTT) and Prothrombin Time (PT): Measures how long it takes for blood to clot, indicating potential issues with clotting factors.
3. Factor Assays: Specific tests to measure the level and function of individual clotting factors.
4. Von Willebrand Factor (vWF) Tests: A panel of tests to measure the amount and function of vWF to diagnose VWD.

Hemophilia vs. Von Willebrand Disease

Feature	Hemophilia (A & B)	Von Willebrand Disease	Source
Cause	Deficiency in specific clotting factors (VIII or IX)	Low or dysfunctional von Willebrand factor (VWF)
Genetics	X-linked, primarily affects males	Autosomal, affects males and females equally
Inheritance	Almost always inherited	Most common inherited bleeding disorder
Severity	Can be mild, moderate, or severe	Most often mild, but can be severe
Common Symptoms	Deep internal bleeding (joints, muscles), easy bruising	Heavy menstrual bleeding, easy bruising, nosebleeds
Treatment	Clotting factor replacement therapy	DDAVP, factor replacement, antifibrinolytics

Managing Life with a Bleeding Disorder

Living with a bleeding disorder requires careful management and awareness to prevent complications. Individuals should work closely with a hematologist to develop a comprehensive care plan.

• Avoid certain medications: Nonsteroidal anti-inflammatory drugs (NSAIDs) like aspirin and ibuprofen can interfere with platelet function and should be avoided.
• Stay active but choose safe sports: Low-impact exercises like swimming, walking, and golf are generally recommended, while contact sports should be avoided.
• Maintain good dental hygiene: Regular dental care can prevent procedures that may cause bleeding.
• Wear medical identification: In case of emergency, this can alert first responders to your condition.
• Prepare for emergencies: Have a plan for how to handle a bleeding episode and know when to seek emergency medical care.

For more information on bleeding disorders and their management, visit Yale Medicine.

Conclusion

While a variety of conditions can cause prolonged bleeding, the most common are inherited bleeding disorders like hemophilia and von Willebrand disease. Both conditions affect the body's ability to form blood clots effectively, leading to symptoms such as easy bruising, spontaneous bleeding, and excessive bleeding after injury. With proper diagnosis and ongoing medical management, individuals with these conditions can effectively control their symptoms and lead active, full lives.

Frequently Asked Questions

Can a lack of vitamin K cause bleeding issues?

Yes, vitamin K is essential for producing certain clotting factors. A deficiency, particularly in newborns (VKDB), can lead to serious and potentially life-threatening bleeding problems.

Is hemophilia the only disease where you bleed does not stop?

No. While hemophilia is well-known, von Willebrand disease is actually the most common inherited bleeding disorder. Many other less common inherited or acquired disorders can also affect the blood's ability to clot.

How is hemophilia diagnosed?

A medical professional will take a full family history and perform blood tests. Key tests include Partial Thromboplastin Time (PTT) and Prothrombin Time (PT) to measure clotting speed, followed by specific factor assays to identify which clotting factor is low.

What are the main symptoms of a bleeding disorder?

Common symptoms include frequent or prolonged nosebleeds, easy bruising, bleeding from the gums, and heavy or unusually long menstrual periods in women. Severe cases may involve spontaneous internal bleeding into joints or muscles.

Can you develop a bleeding disorder later in life?

Yes. While many bleeding disorders are inherited, some can be acquired later in life. These are often caused by autoimmune conditions or other illnesses, or as a side effect of certain medications like blood thinners.

What should someone with a bleeding disorder do during a bleeding episode?

For minor cuts, apply pressure to the area. However, for severe or spontaneous bleeding, or bleeding into joints or internal organs, it is critical to seek immediate medical attention. Your hematologist will provide a specific action plan.

Are bleeding disorders more common in men or women?

Hemophilia is primarily an X-linked disorder, so it mostly affects males. In contrast, von Willebrand disease affects both men and women equally, though symptoms like heavy menstrual bleeding often lead to earlier diagnosis in females.