Howard Fok had no chance of living, said the doctors. Fortunately his parents thought otherwise, writes Margaret Cheng
Seven and a half years ago, Fok Mei-ling gave birth to the child she had longed for - a round faced, strong healthy little boy.
For the first couple of months he did what most babies do - kept his parents busy meeting his needs, grinned when he saw them, cried when he needed them. But then Howard began to weaken; his body began to feel floppy. Soon he was unwilling to eat, and his mother, a lawyer, and his father, a doctor, were certain something was very wrong.
Soon their worst fears were confirmed. Howard had a genetic condition, Spinal Muscular Atrophy (SMA), which was causing paralysis of virtually all his muscles. It is a condition so uncommon Howard's father had never seen a patient with it. He had only read about it in medical school.
SMA comes in three forms in children. The type Howard developed, type one, is so severe the textbooks say victims die before the age of two. By the time he was diagnosed, Howard's muscles were so weak he needed a ventilator to help him breathe. Doctors told his parents to "think about having another baby because the chances of survival are zero."
But today Howard is still alive and very much with us. Although he sits in a wheelchair, needs constant care and a portable ventilator, he enjoys all the things boys his age like. He watches videos, reads books with his mother and has learned to use a computer with his big toe, the one part of his body still able to move.
He has even developed his own system of communication. Although he lacks the muscles to speak, at three he learned to change the tone of his ventilator-assisted breathing so he could use a different tone for each letter of the English alphabet. Now he uses the tones to spell out to his mother his thoughts, feelings, and needs.
"He is the oldest survivor of severe type one SMA in Asia," said Ms Fok, "and probably the only baby in Hong Kong to go home on ventilation."
With doctors telling her Howard should stay in hospital until he died, it was very difficult for Ms Fok to find the things Howard needed to come home. The first ventilator was huge and sounded like a washing machine; its noise and size terrified Howard.
Eventually her husband located better equipment - he now has the same model used by the actor Christopher Reeve, who was totally paralysed after a fall from a horse. And each time he entered a new stage of development or had new problems Ms Fok embarked on a new odyssey to find help or answers.
During those seven hard years she has learned that while the condition Howard has is rare, Spinal Muscular Atrophy is not something society should ignore.
It is a genetic condition, and one in 40 people carry the genetic information that could lead to SMA in their child. It is cased by absence of one of several genes crucial to the functioning of nerve cells, known as the anterior horns cells, which are found in the spinal cord. These cells are vital to the messages sent by nerves to control muscles. If they do not work, the muscles do not work.
But SMA is a recessive condition, meaning both parents have to contribute a defective gene before a child with SMA will be born. That is why it is not common. The other reason is that in the past, most children, especially those with type one SMA, died. It is one of the main causes of death from genetic disease in early childhood.
"We don't know how many have died. I'm sure many die quite quickly if not helped," said Ms Fok. When she wrote to the Hospital Authority to ask how many SMA patients they knew of in Hong Kong she got back a few lines saying "I regret that we cannot offer information on the subject of your enquiry."
Now, thanks to medical advances, those children are living, but getting very little help to do much beyond that. But every day they survive they get closer to a chance of a better life, because researchers have already located the gene responsible for the condition and are much closer to understanding exactly what goes wrong. Once they understand the process, a cure should be possible.
Because she struggled to find answers every time a new problem arose, and because there was little knowledge or understanding of the condition in Hong Kong, Ms Fok thought about setting up a support group or foundation to help people with SMA.
Then, last Christmas, she was contacted by someone who knew a family being pressured to give up on their little boy and put him away in hospital, even thought he had a much milder form of the condition. She was asked if the mother of that child could talk to her.
Ms Fok invited the family to her home and met the other boy, whose main problem was weakness in his legs. He could not stand, much less run about, but was a bright, energetic child frustrated by his limitations, He looked at her Christmas tree in wonder. But his mother saved her own wonder for Howard, sitting there in his chair, living as normal a life as possible, despite his severe limitations.
That family went away determined not to let the professionals give up on their child. And the experience made Ms Fok determined to do more for other families.
"They went away completely changed. They love their child, and they really take good care of the child. I thought if I could do so little and help so much in one family there must be more I could do. I could at least bring to everyone's attention this is not a disease that you can just dismiss." On May 1, Ms Fok established the Families of SMA Charitable Trust. It aims to raise funds to help people with SMA get the medical and social support they need to live as normal a life as possible. Already, said Ms Fok, she has met people who have survived to adulthood with different forms of SMA and though severely paralysed, are contributing more than most to society.
One man, Poon Ka-wing, 30, is running a tour bus company, controlling his computer and telephone system with the only part of his body he can move - his left foot.
Mr Poon recently took on the Families of SMA hotline service as well, saying to Ms Fok that since he answered phones all day, he may as well answer their hotline, too. However there are other children who miss the chance to survive or develop into someone as impressive as Mr Poon. Despite research suggesting most of these children have superior intellects, so many are put away in institutions their brilliant minds have little chance to develop.
One such child is a two-year-old boy now living in large public hospital intensive care unit. His father, a truck driver and his mother, who lives on the mainland, have nothing to do with him. But they have not signed him off, so people like Ms Fok, who would like to see much more being done for him, have no legal right to interfere.
Ms Fok does not want to force his parents to take care of him because she knows few families have the resources she has. But she wants to at least develop a centre where children like him can live in a home-like environment providing education all the other rehabilitation services they need to enjoy life.
"Despite the odds I've been successful in helping Howard but I'm not advocating is that victims and their families should be given choices, options and information. "I didn't have that. I don't wish for any parent to go through what I've gone through. For the past seven years I've just groped in the dark. But I do see light at the end of this tunnel."
- South China Morning Post (Wednesday October 21, 1998)