Holding on to life amid time of tears
From afar, Ethan Suan looks like any other six-year-old boy, except that he wears a pair of gloves, even in the summer. This is because he suffers from Recessive Dystrophic Epidermolysis Bullosa (RDEB), a rare, life-threatening genetic disorder that causes skin to blister right from birth.
The condition affects one in a million people globally. RDEB is caused by a gene mutation. Due to the condition, Ethan's skin becomes very fragile and is easily damaged, so he needs to wear gloves to protect his hands.
Professor Andrew Burd, chief of plastic and reconstruction surgery at the Chinese University of Hong Kong, and Ethan's doctor at the Prince of Wales Hospital, in Sha Tin, says there is currently no cure for RDEB. All that can be done is to provide intensive wound care and nutritional support. Research has been done worldwide to replace abnormal genes through gene, protein and cell therapy.
Ethan's condition totally changed his family's life because of the many life-threatening complications associated with the disease. His throat may close up, leaving him unable to eat, and his wounds could stop healing. Cancers linked to the disease may also develop sooner than expected.
'The diagnosis felt like a death sentence,' said Ethan's father, Andrew Suan, a banker who gave up his job to take care of him full-time. 'He is destined to live a short and very painful life. [Our hope for him] to have a normal life ... just melted away,' Suan says.
Though constantly worried, Ethan's parents are trying not to dwell too much on the uncertainties or raise their hopes. Instead, they choose to live in the present and give him lots of love.
Suan feels great pride in what his son has accomplished and his will to thrive. 'I hope if we can stay disciplined, win the countless battles we face and get the right care, we can extend his life span,' says Suan.
Another patient suffering from RDEB, Shun Shun, aged two, came under Burd's care at the same hospital last year. He has been in hospital ever since. His condition being severe, Shun Shun requires dressings every day and several nurses to look after his needs.
Another patient, Simeon, endured a life of pain and suffering before he died, aged 23; he weighed only 25kg.
Simeon came to hospital in 2008 with a tumour on his right arm. The tumour was removed, but the cancer spread to his armpit and he needed further surgery and radiology.
His mother says her son's skin condition worsened as he grew older. His fingers and toes became fused together and it was painful for him to walk or even hold a pen.
Yet Simeon insisted on attending secondary school. 'Many people could not believe that he passed four subjects at the end of his Form Five education,' his mother says.
Simeon had dreamed that his disease could be cured one day. He had also wanted to be a scientist.
When he knew he was close to death, Simeon made a few special requests, including a meal of shrimp dumplings and roasted chicken. He died in October 2009.
In order to find a cure for the disease, Burd carried out a clinical trial into bone marrow transplants last year.
He also hopes to use human stem cells obtained from umbilical cords to treat the disease.
'This approach will overcome negative immune responses associated with cell therapy and complications associated with bone marrow transplants. There is much optimism that a cure will eventually be found,' says Burd.