When Blood Doesn’t Clot Normally

by Admin-Phmp

By Henrylito D. Tacio

Every April 17th, World Hemophilia Day is being observed. The day, dedicated to people living with hemophilia, is observed to raise awareness and create a better for them. It is about bringing people with bleeding disorders globally together.

In the Philippines, then-President Gloria Macapagal Arroyo signed Proclamation No. 1478, not only observing April 17th as National Hemophilia Day but also declaring April as National Hemophilia Awareness Month.

In the Philippines, about 10,000 Filipino have hemophilia, according to the World Hemophilia Federation. Most of those having the disease don’t know they have it as they have never been diagnosed as such.

As it is a rare disorder, no one seems to pay attention to it. Unknowingly, another one million people in the country are possibly affected by von Willebrand’s Disease and other bleeding disorders. 

“The country still has a lot to do in order to address the harm of bleeding disorder,” said then-Senator Joseph Victor G. Ejercito in his explanatory note of Senate Bill No. 1389, “An Act Providing a Standard of Care for the Treatment of Persons with Bleeding Disorders, Establishing Treatment Centers and Appropriating Funds Therefor.”

“There is an absence or lack of awareness by both medical practitioners and patients regarding the disease, there is a high cost of treatment and the inadequate treatment provided to the patients,” Ejercito wrote.

Many of those who had been diagnosed are suffering because they cannot afford the high cost of treatment. Some reports said that a mild bleed in the joints can cost P30,000 to P50,000 per treatment. Severe bleeds like those in internal organs such as the gastrointestinal tract or the brain can cost hundreds of thousands, if not millions.

Blood connection

An adult person has more than a gallon of blood in his body. According to The Merck Manual of Medical Information, blood is a complex mixture of plasma (the liquid component), white blood cells, red blood cells, and platelets.

Plasma constitutes more than half of the blood’s volume and consists mostly of water containing dissolved salts (called electrolytes) and proteins. Red blood cells (also called erythrocytes) make up about 40% of the blood’s volume. White blood cells (leukocytes) are fewer in number than red blood cells, with a ratio of about one white blood cell to every 660 red blood cells.

Platelets (also known as thrombocytes) are cell-like particles smaller than red or white cells, with a ratio of about a platelet to every 20 red blood cells.

When a person bleeds, his body normally pools blood cells together to form a clot to stop the bleeding. The clotting process is encouraged by certain blood particles (platelets and plasma proteins). Hemophilia occurs when a person has a deficiency in one of these clotting factors.

“Small cuts usually aren’t much of a problem,” says the U.S.-based nonprofit organization Mayo Clinic. “The greater health concern is deep bleeding inside your body, especially in your knees, ankles and elbows. That internal bleeding can damage your organs and tissues, and may be life-threatening.”

Several types

Actually, there are several types of hemophilia, and they are classified according to which clotting factor is deficient. Hemophilia A, the most common type, is caused by insufficient clotting factor VIII. Hemophilia B, the next common type, is caused by insufficient clotting factor IX. Hemophilia C, in which signs and symptoms are often mild, is caused by insufficient clotting factor XI.

In like manner, signs and symptoms of hemophilia vary, depending on the level of clotting factors. If the clotting-factor level is mildly reduced, a person may bleed only after surgery or trauma. If the deficiency is severe, he may experience spontaneous bleeding.

The Mayo Clinic says the signs and symptoms of spontaneous bleeding include: unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work; many large or deep bruises; unusual bleeding after vaccinations; pain, swelling or tightness in your joints; blood in your urine or stool; and nosebleeds without a known cause. In infants, unexplained irritability is a sign.

On the other hand, emergency signs and symptoms of hemophilia include sudden pain, swelling, and warmth in large joints, such as knees, elbows, hips, and shoulders, and in your arm and leg muscles; bleeding from an injury, especially if you have a severe form of hemophilia; painful, prolonged headache; repeated vomiting; extreme fatigue; neck pain; and double vision.

Hemophilia may be diagnosed between the age of 9 months and two years old, sources claim. In some cases, a person may not be aware that he has mild hemophilia until he undergoes surgery or experiences an injury.

Sex-linked disorder

As hemophilia is a sex-linked disorder, it occurs more often in men than in women. But there are bleeding disorders that are not sex-linked but still can be inherited. The von Willebrand’s Disease is one of them. It is a hereditary deficiency or abnormality of the von Willebrand factor in the blood, a protein that affects platelet function.

Although hemophilia is hereditary by nature, it can also appear in people “even without a history of ailment in the family.” As Andrea Trinidad, president of Hemophilia Advocates-Philippines, told Philippine Daily Inquirer: “Mutation starts at birth. When the baby is delivered, he or she already has hemophilia even without family history.”

No cure yet

There’s no cure yet for hemophilia, the Mayo Clinic claims. “But with proper treatment and self-care, most people with hemophilia can maintain an active, productive lifestyle,” the Minnesota-based health organization states.

According to the US National Health, Lung and Blood Institute (NHLBI), the main treatment for hemophilia is called replacement therapy. Concentrates of clotting factor VIII (for hemophilia A) or clotting factor IX (for hemophilia B) are slowly dripped or injected into a vein. These infusions help replace the clotting factor that’s missing or low.

Replacement therapy, however, has some complications, which include: developing antibodies (proteins) that attack the clotting factor; developing viral infections from human clotting factors; and damage to joints, muscles, or other parts of the body resulting from delays in treatment.

Lifestyle and home remedies

Meanwhile, to avoid excessive bleeding and protect the joints of those with hemophilia, the Mayo Clinic suggests the following:

Exercise regularly. Activities such as swimming, bicycle riding, and walking can build up muscles while protecting joints. Contact sports – such as football or wrestling – are not safe for people with hemophilia.

Avoid certain pain medications. Drugs that can aggravate bleeding include aspirin and ibuprofen (Advil, Motrin, others). Instead, use acetaminophen (Tylenol, others), which is a safe alternative for mild pain relief.

Avoid blood-thinning medications. Medications that prevent blood from clotting include heparin, warfarin, clopidogrel, and prasugrel.

Practice good dental hygiene. The goal is to prevent tooth extraction, which can lead to excessive bleeding.

Protect your child from injuries that could cause bleeding. Kneepads, elbow pads, helmets, and safety belts may help prevent injuries from falls and other accidents. Keep your home free of furniture with sharp corners.

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