Financial support needed to help hemophiliacs live a normal life

JAKARTA (JP): Widya was shocked to see her 11-month-old son Eric, who cut his lips after making several unsuccessful attempts to stand on his feet, hemorrhaging.

"He was bleeding profusely, like a running tap. I panicked and rushed him to hospital where he was given blood transfusion," she said. An official at the hospital referred her to Cipto Mangunkusumo General Hospital (RSCM) to do a blood test for her son.

"My heart sank when the doctor at RSCM said that Eric had hemophilia. A disease which requires expensive and lifelong treatment," she said.

This happened 11 years ago and today Eric is no different from other children.

Eric is among the thousands of hemophiliacs in the country. There is no accurate data on the number of sufferers of this fatal disease.

The Indonesian Hemophilia Foundation predicted that one in 10,000 males is a hemophiliac, meaning there are about 18,000 hemophiliacs in the country; some 1,000 of whom are in Jakarta.

"Many parents don't take their children to doctors for examination, making it difficult to monitor the number of hemophiliacs and fatalities," said Moeslichan, who is chairman of the RSCM's Hemophilia Comprehensive Care Team.

RSCM is currently treating 170 hemophiliacs.

He said with proper treatment, hemophiliacs could live like normal people and have the same life expectancy.

Hemophilia is an inherited blood disorder due to a deficiency in blood clotting factors. It affects males almost exclusively. The disease is characterized by the delayed clotting of the blood, making it difficult to even control bleeding due to a minor injury.

"Most parents only become aware of this health problem when their sons are circumcised," he said.

There are two types of hemophilia. The most common is hemophilia A, which is caused by a deficiency of Factor VIII, and hemophilia B, which is due to the absence of Factor IX. Both factors are required for normal blood clotting.

This sex-linked disease is caused by a defective gene on the X chromosome. So males, with XY chromosomes, who inherit an X chromosome with the defective gene will likely develop the disease, while females, with XX chromosomes, would become carriers and could transfer the gene to their offspring. Very rarely, a female with hemophilia is born, even if her mother is a carrier and her father a hemophiliac.

However, Moeslichan also pointed out that gene mutation due to viral infection, radiation and pollution could trigger hemophilia.

Symptoms of hemophilia include bruises, spontaneous bleeding, and bleeding into joints accompanied by pain and swelling. Untreated injuries can lead to bleeding to death or decaying of tissues around the wound, and bleeding into joints can cause paralysis.

Comprehensive health check

Moeslichan said Factor VIII and Factor IX could be supplied through intravenous injections of cryoprecipitate or Factor VIII, which is concentrated and extracted from blood donated to hospitals.

Doctors will administer the first few injections, but will later teach parents or the patients how to do it themselves at home.

As for prevention, Moeslichan stressed the importance of a comprehensive health check prior to marriage, and antenatal tests, to help detect irregularities, during the 12th week of pregnancy.

"If the fetus is diagnosed with hemophilia, the couple can decide whether to abort it," he said.

People with hemophilia are advised to cycle and swim to improve their stamina, and strengthen their muscles and joints. A patient is advised to wear a tag identifying him as a hemophiliac and his blood type to enable proper treatment during an emergency.

The doctor, who is also a professor at the University of Indonesia's School of Medicine, admitted that treatment for hemophilia was very expensive and sufferers would have to rely on it for the rest of their lives.

For routine treatment, adult hemophiliacs need some 24 bags of cryoprecipitate every week, each worth Rp 41,000. There is also an on-demand treatment for emergency cases, such as injuries with prolonged bleeding or for tooth extraction.

Concerns over funds was also aired by chairman of the Indonesian Hemophilia Foundation Erlan Taralan.

The foundation requires some 40,000 bags of cryoprecipitate annually for which it can only cover 15 percent of the total cost. Thirty percent is covered with funds derived from the Social Safety Net (JPS) and the remaining by RSCM and other institutions.

"We are turning to the public for funds because next year there will be no allocation from JPS," he said.

If volunteers donate their blood, why are patients burdened with the cost? A staff at RSCM's Blood Transfusion Division, Agi Herlina, said the cost of each bag was set to cover blood management, which includes tests, donation, processing, handling and distribution.

-- Maria Endah Hulupi