Early diagnosis of hemophilia imperative
Debbie A. Lubis, The Jakarta Post, Jakarta
Nobody in the family had ever imagined that the small cut in Markus' mouth would cause his death. His parents panicked when they realized that the wound would not stop bleeding.
When the Papuan boy was admitted to Cipto Mangunkusumo General Hospital (RSCM) in Jakarta, doctors told his parents that Markus had hemophilia -- a blood defect that causes delayed clotting and prolonged bleeding.
Markus got the cut in the mouth after falling from a tree. Doctors in Papua were unable to stop the bleeding by way of stitching the open wound.
S. Moeslichan, a pediatric and oncologic professor at the Hemophilia Care Center of RSCM, says that a great many parents lack knowledge about hemophilia.
He estimates that about 20,000 males suffer hemophilia throughout Indonesia and that the disease affects one in every 5,000 male births worldwide.
He is surprised by a report by the Indonesian Hemophilia Foundation (YHI) that claims that the number of hemophiliacs throughout the country is only 530.
Moeslichan warns that late diagnosis of hemophilia delays proper treatment that may result in paralysis and eventual death.
"The public knows so little about this disease," he says. "Actually with proper treatment, a patient can lead a normal and productive life because the bleeding can be controlled," he said.
Hemophilia is an inherited bleeding disorder passed on by a mother through an X chromosome to male offspring. People with hemophilia lack Factor VIII or IX in their blood so they experience prolonged bleeding and even internal or spontaneous bleeding in joints, muscles and soft tissue.
Moeslichan says bleeding that involves muscles and joints causes painful swelling or lack of movement in an arm or leg because blood unable to clot is trapped under the skin.
"Parents usually learn about hemophilia when there is excessive bleeding from a minor cut or no obvious injury (spontaneous bleeding), surgery or a tooth extraction," he said.
Yudhistira Abidin, 22, lives a painful life he stumbled into a chair two years ago and surgeons had to remove a few liters of blood trapped in his calf. He has been confined to a wheel chair ever since.
"I first learned about my son's disease when he was circumcised 12 years ago. He bled and I rushed him to the hospi tal for a transfusion," Yudhistira's mother says.
Moeslichan says that there are two alternatives of lifelong treatment for hemophilia patients: transfusions or injections.
The simplest treatment is to give routine transfusions such as Cryoprecipitate -AHF for patients with hemophilia type A and fresh frozen plasma for patients with hemophilia type B, he says.
Hemophilia type A is the most common and is due to a deficiency of Factor VIII (FVIII) while hemophilia B is due to a deficiency of Factor IX (FIX).
Moeslichan says the amount of transfusion depends on a patient's height, weight and age. Generally, a hemophiliac needs 20 units for each kilogram of weight in one treatment. The treatment is usually given twice a week.
The treatment is very costly. P. Sianipar, a father of a 19- year-old hemophiliac, says that he spends Rp 1.2 million for 250 units of Cryoprecipitate.
Yayasan Hemophilia Indonesia, Jl. Pekapuran VIII No. 41 (d/h Jl. Betet), Kelurahan Tambora, Jakarta 11220, tel. (021) 6391044/6404507