November 9, 2008

This Not-So-Royal Disease

by Andrea Trinidad-Echavez

This week, I am posting a wonderful article by colleague and friend Andrea, from the Philippines. I was privileged to travel with her for one week. We both met Jeffrey and his mother Rita.

Every year, Rita Rodriguez, 54, would religiously light a candle in the early mornings of All Saints Day in remembrance of her four sons– Noel, Ricky, Materno and Antonio — who died one after the other in a span of two decades.

Cursed, was how people from her hometown of Tanjay in Negros Oriental, thought of Manang Rita. Little did they know that a rare hereditary bleeding disorder called hemophilia was the culprit behind their deaths.

“I would have wanted to go home to Negros to personally visit their graves but life is hard,” said the 54-year-old mother of eight, who has been struggling to keep another son Jeffrey, from falling into the same fate as his older brothers. In between Jeffrey’s confinements, Manang Rita would make ends meet by offering services as masseuse or cleaning houses and doing laundries.

Jeffrey, 25, a psychology student in Adamson University, was diagnosed with hemophilia when he was barely three, following a supposedly minor injury on the head that caused profuse bleeding. By then, two of Jeffrey’s older siblings had already succumbed to strange internal bleeding episodes.

Manang Rita remembered her older brother, Luis Torres, would also profusely bleed from the smallest injuries. But they did not know that hemophilia runs in their family.

Manang Rita’s oldest child Noel died in the early ’60s from an unexplained ailment. He was only three years old. Noel bumped himself while playing. Shortly after, he developed a tumor-like lesion that wouldn’t go away. He died a few days later.

Another son, Ricky, died in the ’70s at the age of 5. He also died of strange bleeding after getting minor injuries from playing.

In both deaths, Manang Rita hardly understood what happened to her boys. She could only grieve over the loss and hoped well for her other children.

But her reprieve from grief ended when in 1989, Materno, then 25, died after days of suffering from severe headache.

“We gave him pain relievers and put ice on his head. But when it refused to go away, we decided to bring him to the hospital,” Manang Rita said in mixed Cebuano and Tagalog. By then, local doctors already knew the name of the strange disorder that plagued the boys in Manang Rita’s family.

“I was told the boys have hemophilia. It was only then that we became aware of the name of the bleeding disorder in our family,” Manang Rita shared, her voice quivering in between.

Doctors gave Materno a blood transfusion to replenish the blood lost in the internal bleeding. He was also given a dose of the expensive Factor 8 concentrates derived from human plasma that would instantaneously stop the bleeding.

But by that time, it was too late to avert Materno’s condition. He died in the hospital.

In 1997, another son, Antonio, complained of severe stomach pains. He urinated blood, causing her mother to be concerned. Unknown to Manang Rita, Antonio, then 26, fell off from a tricycle. Though he did not sustain any open wound, Antonio’s injury caused internal bleeding which lead to his death in less than one week.

“People thought she was cursed because of the deaths of her four sons,” said Rizalina Magalso, a cousin of Manang Rita and herself a mother of 13-year-old Dan Louie, also a hemophiliac.

In 1999, Manang Rita’s youngest son, Jeffrey sustained injuries from a fall and again suffered excessive bleeding. By then, with four sons dying of bleeding, Manang Rita was already on the verge of despair.

“I told the doctors I could no longer bear it if something bad happened to Jeffrey,” said Manang Rita.

The doctors sympathized with her and advised her to bring Jeffrey immediately to Manila and seek help at the Philippine General Hospital.

She sold all her family’s little landholdings if only to save her youngest son and admitted him at the PGH. After several bags of blood transfusion and over a month of stay at the hospital, Jeffrey became well enough.

But the deaths of her four sons led Manang Rita to decide to stay in Manila for good, where Jeffrey has better chances of surviving.

Called the “royal disease,” hemophilia is a hereditary bleeding disorder that affects the males of the family. Mothers are usually the carriers and they pass on the defective genes to their male offspring.

Several members of noble families in Europe were affected by it, thus the monicker “royal disease”. Written accounts identified Queen Victoria as the first royal carrier of the disorder, with her eighth child Leopold, born in 1853 as the first recorded royalty with the disease. Leopold died at the age of 31 from an intra-cerebral hemorrhage after a fall.

Two of Queen Victoria’s own daughters, Alice and Beatrice, were also carriers of hemophilia. The condition was transmitted through them to several royal families in Europe, including Spain and Russia. The most famous affected individual was the son of Tsar Nicholas II of Russia, Tsarevich Alexis, who was born in 1904. There were speculations that the illness led to severe strain within the royal family, and enabled Rasputin to gain influence over the family, ultimately leading to the downfall of the once powerful dynasty.

The WFH estimates that .01 percent of the population or about 1 in 13,000 are affected by hemophilia.

In the Philippines, the Hemophilia Association of the Philippines for Love & Service (HAPLOS) said about 6,000 to 8,000 could be affected by the disorder. However, only a little over 1,000 have been registered. Most of the sufferers come from the same families.

Thanks to modern technology, hemophiliacs like Jeffrey may soon lead normal lives with the availability of factor concentrates that can instantaneously stop bleeding. The medicines, however, are very expensive and are still unaffordable for people like Manang Rita, who barely manages to survive with her meager income as a masseuse.

“Even though life is hard here, we will endure if only for the sake of Jeffrey. I am thankful to God that despite everything, He has helped us survive this ordeal,” said Manang Rita.

Great Book I Just Read
Daughter of the Desert, by Georgina Howard

If I tried to describe Gertrude Bell to you, you might think I was making it up. But this is a true story about one of the world’s most amazing women. Born into a privileged English family in 1868, Gertrude Bell seemed to have been destined to become a daughter of the desert, with her sharp mind and energetic physique, she excelled at whatever she attempted. Her resume would include archaeologist, spy, Arabist, linguist, author, poet, photographer, mountaineer and nation builder. And despite her intrepid nomadic roaming, her warm and intimate letters to home revealed a sensitive, loving woman. She eventually would become perhaps the most famous woman of her time, exploring the deserts of Arabia on her own, learning Arabic, and meeting privately with the Bedouin. She learned more about Arab life and culture than any other westerner. Her skills and knowledge were only surpassed by her passion: she eventually grew to love the desert and the Arab people. The timing was perfect for England. During World War I, when the Ottoman Turks pushed into Arabia, the British needed someone to help bring the Arabs in as allies. Like her friend Lawrence of Arabia, Bell became the British government’s diplomat. She eventually helped win the Arabs to the British side, helped to build the nation of Iraq and was recognized by the Arabs for her dedication and achievements.

Anyone wanting an informed opinion about Iraq currently should read this book. It’s a sobering reality check when you read about the sacrifices made by the British 100 years ago and you think you are actually reading about what has happened in the last six years. It highlights the long history and complex nature of the Arab culture, beautiful and mysterious, and the obstacles in uniting a diverse group of people. Inspiring, informative. A whopping four stars. I can’t wait to read it again.

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