Our newest book, Hong Kong Noir: Fifteen true tales from the dark side of the city, has been on the South China Morning Post‘s top five bestseller list since Christmas. Author Feng Chi-shun has been interviewed by RTHK Radio 3 and HK Magazine, and the book has been reviewed by Susan Blumberg-Kason, the SCMP and Asia Times, which said:
Who can resist a story told by a pathologist about a hemophiliac, Ah Fai, who chooses to join the notorious 14K triad at the tender age of 15 and enjoys nothing more than the bloodletting of a full-on, violent street fight?
As you might guess, Ah Fai spends a lot of time in the intensive care unit of hospitals, where his striking good looks and unusual charm make him something of a celebrity to the doctors and nurses who treat him. Their affection for the reckless gangster spurs them to work especially hard to save him every time he shows up awash in his own blood at an emergency ward. In the end, however, it turns out there is only so much anyone can do for a hemophiliac who has made such a poor career choice.
We print this story below.
Hemophilia: A hereditary disease characterized by a defect in the clotting of blood.
Hemophiliac: A person who suffers from hemophilia.
Every story about a hemophiliac is worth telling.
This is the story of a young man, Hong Kong born and bred, who suffered from hemophilia from birth and died of its complications at the age of 25.
Hemophilia is a disease which teaches medical students more about genetics than all others. The pathogenesis of the disease is the deficiency of a clotting agent in the blood (a “Factor” in medical vernacular) known as Factor VIII.
Although other “Factors” in our blood are known to be deficient, some congenital, some acquired, hemophilia remains the most famous and fascinating.
Hemophilia is a disease like no other. It is one of the first diseases discovered to be connected to the sex chromosomes (a sex-linked disease, in medical terms). The gene responsible for producing Factor VIII lies in chromosome X. If the gene is defective in a female, chances are she won’t be affected because she has another X chromosome as a back-up (except in consanguineous marriages, when both X chromosomes may be affected.) There is no back-up in a male, because his other sex chromosome is Y.
The fact that it affects males almost exclusively makes one think it is nature’s way of bringing equity to both sexes by compensating women for having exclusive female conditions such as menstruation and childbirth. But then again it is not, because the misfortune of a son causes despair to his mother more than anyone else.
A Jewish mother in ancient times watched her newborn sons die of post-circumcision bleeding one after another. It was only after the death of her fifth son in succession that her Rabbi would finally relent and grant her exemption from the religious rite of circumcision.
A hemophiliac tends to bleed non-stop from the slightest cut and the mildest bruising. Blood transfusion has been known to be effective in stopping the bleeding since time immemorial – hence ‘love of blood’ has become the disease’s nickname.
The life of a hemophiliac is never easy. The mainstay of treatment is the replacement of Factor VIII. In ancient times, it was achieved by whole-blood transfusion, and more recently by other blood components such as plasma and cryoprecipitate derived from frozen plasma. Blood transfusion is time-consuming and cumbersome, laden with inherent risks, and disruptive of the lives of the hemophiliac and his family. The narration of the trials and trepidations from parents taking care of a hemophiliac child is material worthy of a Pulitzer Prize.
In later years, scientists found a way to produce Factor VIII concentrates en masse, first from a large pool of plasma from thousands of donors, then more recently by genetically engineered methodologies. Such products improve a hemophiliac’s life tremendously because of their ease of administration, as most cases no longer require hospitalization.
Older blood products carried the risk of having been contaminated with viral diseases, such as hepatitis or HIV AIDS. Thousands of hemophiliacs have died of these infectious diseases over the years. The disease carries other complications as well, involving bleeding into joints and body cavities.
One relatively rare but deadly complication is the development of antibodies to Factor VIII, a result of repeated injection of Factor VIII replacement therapies. Having this complication means that no matter how much Factor VIII is poured into the patient’s circulatory system, it is ‘neutralized’ or ‘inhibited’ by the antibodies whose levels rise further when challenged by the injection of high doses of Factor VIII.
In recent years, the outlook has become rosier for these patients