Not long for this world

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A family in the Mekong Delta has been cursed with a genetic condition that leaves their limbs long and their lives short

 
Phan Van Quan (L) and his nephew, 19- year-old Phan Tri Thuc, both suffer from Marfan syndrome a genetic disorder that can lead to early death.

Phan Van Quan liked being big.

His hands and legs stretch down past his hips, making him an excellent laborer. The 37-year-old resident of Vinh Long Province says he used to be able to do twice the work of his peers.

But that all ended with a heart attack in June, 2008.

Quan became weak and couldn't do manual labor anymore. In fact, he could barely walk.

In a way, he expected all that to happen. His mother once told him, "Our family has lived with a strange disease and those who are taller than 1.80 meters high won't live to see 45."

Indeed, his grandfather (who stood 1.89 meters) died at 40. His 1.90-meter tall father died at 49 and his older brother died at 32. The condition has also afflicted his aunt and cousin.

They all died following a sudden pain in chest, Quan said.

As his condition worsened, Quan headed for the hospital. His doctor told him he suffered from a heart disease and needed an urgent operation or he would certainly die.

Because his family couldn't afford the costly treatment, Quan sent out numerous letters to charities asking for help. No one responded, until he got approval from the Ho Chi Minh City Sponsoring Association for Poor Patients.

Last December, Quan was hospitalized at the University of Medicine and Pharmacy's Hospital in HCMC, where he was diagnosed as suffering from a rare genetic disorder called Marfan syndrome.

The syndrome is characterized by unusual height, long limbs and weak hearts.

Quan went under the knife that month. Doctors say they saved him from certain death, but warned him not to perform any demanding work or ride a motorbike.

Strenuous activity could kill him at any time, they warned.

Today, Quan worries about his younger sister, Phan Thi Xuyen, 27, and his nephew Phan Tri Thuc, 19 - both of whom stand 1.8 meters high. One of his siblings, Phan Thi Bich Phuong, 34, meanwhile, is just 1.6 meters high, but her limbs are abnormally long.

Quan said that although his family members have yet to exhibit any health problems, the risks are still high. Doctors told him that there's a 50-percent chance that they inherited the congenital syndrome.

Now he's trying to solicit charitable support to get his family members medical attention.

If performed early enough, doctors say that surgeries could help them live to be more than 70 years old.

Last year, the University of Medicine and Pharmacy's Hospital treated four patients with the syndrome, which can affect the skeleton, eyes and blood vessels.

Symptoms usually don't appear until patients hit puberty, according to Dr. Nguyen Hoang Dinh, chief of the hospital's cardiovascular operation department.

The greatest risk is that the disease will enlarge the patient's aorta, causing sudden death, he said.

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