Poor knowledge cost hemophiliacs lives

Poor knowledge cost hemophiliacs lives

JAKARTA (JP): Thousands of hemophiliacs in Indonesia remain
untreated and eventually die because knowledge of the disease
among Indonesian people remains very poor, specialists in Cipto
Mangunkusumo General Hospital (RSCM) revealed on Friday.

Hemophilia is an inherited bleeding disorder which stems from
a defect in blood clotting, resulting in much longer bleeding in
its sufferers.

The disease inflicts one out of 10,000 people in the world and
it affects males almost exclusively as women with the disease
pass it on through their X chromosome.

Unfortunately, statistics show that 80 percent of hemophiliacs
around the world do not have enough knowledge of how to treat the
disease.

That trend is also found in this country.

Statistically, Indonesia is estimated to have around 210,000
hemophiliacs out of its 210 million population. But in 1995,
there were only 288 recorded cases all over the country.

"In Jakarta for example, with 10 million population, from an
epidemiological point of view there should be about 1,000
hemophiliacs. But in RSCM, there are only 170 patients
registered," the hospital's clinical pathologist, Rahajuningsih
Setiabudy, said on the sidelines of a seminar on hemophilia.

This, she said, is probably due to lack of information and
knowledge about the disease.

"Some may be afflicted with severe hemophilia and die in early
childhood, while others are mild hemophiliacs who are not easily
diagnosed," Rahajuningsih added.

Hemophilia is divided into Hemophilia A and B with each class
also divided into mild, moderate and severe categories.

Hemophilia A is the most common form and is due to a
deficiency of liver glucoprotein clotting factor called Factor
VIII (FVIII).

The latter is caused by a deficiency of Factor IX (FIX), a
clotting factor which is also produced in the liver. Man needs
Vitamin K to produce FIX.

The normal range of both factors is 50 percent to 100 percent.

The range for hemophiliacs is 6 percent to 30 percent for the
mild one, between 1 percent to 5 percent for the moderate and
less than 1 percent for the severe case.

According to Rahajuningsih, bleeding in mild level hemophilia
is usually associated with major injuries, surgical operations or
tooth extractions.

"Diagnosis may not be made until adulthood when unexplained
bleeding may follow one of the above events. That's why the
disease is undetected," she said.

Bleeding occurs in moderate hemophilia after minor injuries as
well as operations and tooth extraction.

While in severe hemophiliacs, bleeding into joints, muscles
and other tissues occurs with minimal or no obvious injury
(spontaneous hemorrhage) as well as with surgery and tooth
extraction.

"The symptom can be detected when an infant starts to crawl.
But many parents are clueless about this. They wonder why their
child has many bruises," Rahajuningsih said.

Many parents end up sending their children to a masseur, she
added, instead of a doctor.

Unfortunately, cure remains unavailable. Medicine is used to
treat factor deficiency but it is relatively expensive, she said.

Injected medicine Koate High Purity (Koate HP), which consists
of FVIII, for example, costs between Rp 817,500 (US$91) to Rp 1.8
million ($200) per vial.

"It can cost a patient several hundred thousands rupiahs for
an injection. While he or she should get it at least once a
week," Rahajuningsih said.

Pediatrician Djajadiman Gatot said that a hemophiliac can live
a normal life with treatment.

"Too bad, private health insurance has yet to cover insurance
for patients with inherited disease. While it can really ease the
patients' financial burden," he said. (hdn)