What Is Hemophilia?

Hemophilia is a bleeding disorder caused by a genetic inability for the blood to clot properly. This can result in serious complications as well as bleeding, following an injury or surgery. Clotting factors are proteins found in the blood that can assist stop bleeding. Factor VIII (8) or factor IX (8) levels are low in people with hemophilia (9).
The amount of factors in a person’s blood determines the severity of their hemophilia. The lower the level of the factor, the more probable bleeding may occur, potentially causing major health consequences. An individual can develop hemophilia later in life in rare situations. The majority of victims are middle-aged or elderly adults or young women who have recently given birth or are near the end of their pregnancy. With the right treatment, this issue can usually be resolved.

Types of Hemophilia

Hemophilia is divided into two types: type A and type B.

Hemophilia A

This is characterized by a deficiency in clotting factor VIII. This accounts for approximately 80% of all hemophilia cases. The severe type of hemophilia A affects about 70% of those who have it.

Hemophilia B

Someone with hemophilia B, also known as “Christmas illness,” lacks the clotting factor IX. Hemophilia affects about one out of every 20,000 males born around the world.
Depending on the amount of clotting factor in the blood, A and B can be mild, moderate, or severe. Mild clotting factor levels range from 5 to 40%, moderate levels range from 1 to 5%, and severe levels range from less than 1%.

What Causes Hemophilia?

Hemophilia is caused by a mutation or change in one of the genes that give instructions on how to make the clotting factor proteins that help blood clot. This alteration or mutation can cause the clotting protein to stop operating or go missing entirely. The X chromosome contains these genes.
Females have two X chromosomes, while males have one X and one Y chromosome (XY) (XX). The X chromosome is passed down from mothers to sons, and the Y chromosome is passed down from fathers to sons. Each parent gives a female one X chromosome. Many genes found on the X chromosome are not found on the Y chromosome. This means that males have only one copy of most X chromosome genes, whereas females have two copies.
As a result, if a male inherits an affected X chromosome with a mutation in either the factor VIII or factor IX gene, he can get hemophilia. Hemophilia can also affect women, but it is far less common. Both X chromosomes are afflicted in these circumstances, or one is diseased but the other is absent or inactive. The bleeding symptoms in these women may be comparable to those in men with hemophilia.
Acquired hemophilia is another rare form of the disease in which a person’s immune system targets blood clotting factors. It has a connection to:

• pregnancy
• autoimmune diseases
• cancer
• multiple sclerosis

Despite the fact that hemophilia runs in families, some families have no history of hemophilia. By chance, there may be carrier females in the family, but no affected boys.

Symptoms of Hemophilia

Excessive bleeding and easy bruising are two indications of hemophilia. The intensity of symptoms is determined by the blood’s clotting factor levels. Common signs of hemophilia include:

• Bleeding in the joints, which commonly affects the knees, elbows, and ankles, causing swelling, pain, and tightness in the joints
• Bleeding into the skin (bruising) or muscle and soft tissue, resulting in a blood build-up in the area (called a hematoma)
• Bleeding in the mouth and gums, as well as bleeding that is difficult to stop after a tooth is lost
• Bleeding following vaccines or other shots
• An infant’s head is bleeding after a tough delivery
• Blood in the feces or urine
• Nosebleeds that are difficult to stop
• Bleeding in the head and, in certain cases, the brain, which can lead to long-term issues like seizures and paralysis
• If the bleeding cannot be stopped or if it occurs in a vital organ such as the brain, death may result

Complications of Hemophilia

Severe Internal Bleeding

Your limbs may enlarge if you have deep muscle bleeding. Numbness or pain may result from the swelling pressing on nerves.

Infection

Hemophiliacs are more likely to need blood transfusions, which increases their risk of acquiring tainted blood products.

Joint Degeneration

Internal bleeding can cause considerable discomfort by putting strain on your joints. Frequent internal bleeding, if left untreated, can lead to arthritis and joint degeneration.

Likely Adverse Effects with Treatment

The immune system reacts negatively to the clotting agents used to treat bleeding in some persons with severe hemophilia. When this happens, the immune system produces inhibitors, which inactivate the clotting factors, reducing the effectiveness of treatment.

Preventive Lifestyle Remedies for Hemophilia

Regular Exercise

Swimming, cycling, and walking are all good ways to strengthen muscles while also protecting joints. People with hemophilia should avoid contact sports such as football, hockey, and wrestling.

Proper Dental Hygiene

The goal is to avoid having to pull a tooth, which can cause a lot of bleeding. Make sure your child is safe from any injuries that could result in bleeding. Kneepads, elbow pads, helmets, and safety belts can all help protect you from injuries caused by falls and other mishaps. Ensure to keep sharp-cornered furniture out of your home.

Avoid some Pain Relievers

Aspirin and ibuprofen (Advil, Motrin IB, others) are two drugs that can make bleeding worse. Instead, for modest pain treatment, try acetaminophen (Tylenol, others), which is a safer choice.

Avoid Blood-Thinning Drugs

Heparin, warfarin (Coumadin, Jantoven), clopidogrel (Plavix), prasugrel (Effient), ticagrelor (Brilinta), rivaroxaban (Xarelto), apixaban (Eliquis), edoxaban (Savaysa), and dabigatran (Pradaxa). are all blood thinners.

How to Treat Hemophilia

The basic treatment for severe hemophilia is to get a replacement clotting factor through a tube inserted into a vein. This replacement therapy can be used to stop a bleeding episode from getting worse. It can also be given on a regular basis at home to help reduce bleeding. Some persons receive replacement therapy on a long-term basis. Other treatments include:

Desmopressin

This hormone can cause your body to release more clotting factors in some cases of mild hemophilia. It can be given as a nasal spray or injected slowly into a vein. To enhance clotting and healing, these drugs can be administered directly to wound sites. Fibrin sealants are particularly beneficial in dental treatment.

Medications that Keep Clots from Forming

These drugs work by preventing blood clots from forming. For minor cuts, use first aid:

The bleeding can usually be stopped by applying pressure and a bandage. Use an ice pack to treat minor areas of skin bleeding. Minor bleeding in the mouth can also be treated with ice pops.

Vaccinations

Despite the fact that blood products are checked, illnesses can still be contracted by those who use them. Consider being immunized against hepatitis A and B if you suffer from hemophilia.

Physical Therapy

This helps to alleviate the symptoms of internal bleeding to the joints.

Now that you Know…

Depending on the amount of clotting factor VIII in a person’s blood, hemophilia A can be mild, moderate, or severe. The severe type of hemophilia A affects about 50-60% of patients with the disease. People with hemophilia A can live healthy and active lives with the right education and treatment, however, response to treatment and the existence of underlying health issues may affect life expectancy.