ON April 9 the Philippines will observe the World Hemophilia Day. The organizer wants Filipinos all over the country to know something about this inherited bleeding disorder.
According to the World Hemophilia Federation, about 10,000 Filipinos have hemophilia but most don’t know they have it.
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 with von Willebrand’s Disease and other bleeding disorders.
All in all, only around 1,200 have been identified to have this lifelong condition that prevents blood from clotting properly. And many of those who had been diagnosed are suffering because they cannot afford the high cost of treatment.
• Very expensive disease
“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,” said Ric Felipe, the secretary of Hemophilia Advocates-Philippines, a national organization of Filipinos with hemophilia.
The disease is indeed very expensive. Take the case of Andea Echavez, the president of the Hemophilia Advocates. “I use 2,000 to 3,000 IUs (international units) per infusion, depending on severity of bleed. Infusion is every 12 hours until bleed stops,” she says. One vial of 260 IUs costs about P5,400. For a five-day treatment, she spent around P700,000.
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 percent of the blood’s volume. White blood cells (leukocytes) are fewer in number than red blood cells, with a ratio of about 1 white blood cells 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,” the Mayo Clinic says. “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.”
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.
• Signs and symptoms
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 at between 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. 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.
• A mother’s fight
Andrea Echavez, a former Philippine Daily Inquirer reporter, has such kind of bleeding disorder. “My mother died of excessive blood loss in 1988 during a biopsy operation,” she recalled in her blog. “Even though she insisted that she had bleeding tendencies, doctors refused to believe. Bleeding disorders only happen in boys and men, they said.
In 2006, Andrea’s daughter, Star, had been bleeding profusely in her nose daily for a month in a row. When she was born in 1998, the mother observed bruises all over her body. She was only three weeks old when the mother rushed her daughter to the emergency room after she had a nosebleed.
“That was the first,” Andrea wrote. “I suggested to her attending doctors that she could have inherited my mother’s bleeding problem. But they said it could not be possible. She’s a girl.” But her mother instinct told her there was something wrong. “So, when her nosebleeds became more frequent in 2006, we decided to go abroad to have her tested one more time. Because of my family history, the doctor tested me as well,” she wrote.
Three months later, the results came out: both the mother and daughter have von Willebrand’s Disease. “Suddenly, we were thrust into the unknown,” Andrea wrote. “Bleeding disorders are rare. But bleeding disorders in girls and women are even more rare. Back in the Philippines, there were hardly any girls and women diagnosed with it at that time.”
With a young daughter affected by a disorder that caused her mother’s death, Andrea is now determined to fight it. “I would not allow bleeding to consume us,” she said. “Never again. It deprived me and my siblings of a mother’s love in our teenage years. I would not allow it to deprive my daughter of living a normal life.”
• 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 all may help prevent injuries from falls and other accidents. Keep your home free of furniture with sharp corners.