Hong Kong boy with incurable disease undergoes risky surgery to help him breathe

A 10-year-old Hong Kong boy born with a rare and incurable genetic disorder has successfully undergone high-risk spinal surgery intended to slow the deterioration of his health.

Marcus Ng Pak-lam has spinal muscular atrophy (SMA), which causes his muscles to gradually weaken and waste away.

He completed an eight-hour procedure at Queen Mary Hospital in Pok Fu Lam on Monday (Sept 27) to correct scoliosis – a condition where the spine twists and curves resulting in a reduction in lung volume.

“The surgery was smooth and successful,” his mother Wenus Chan told the Post on Tuesday.

“The procedure was important as it can allow Marcus to breathe easier and stop his already weak lung function from worsening.”

SMA can leave sufferers unable to walk or even breathe. Marcus cannot move his body and needs round-the-clock care as he is either bedridden or in a wheelchair.

According to his mother, Marcus received tracheal intubation in an intensive care unit following the surgery but it was removed on Tuesday afternoon after the doctor was satisfied he could breathe properly on his own.

“Marcus was tired after the removal of the intubation, but he still managed to say to me that he was feeling OK,” she said.

“Finally I can heave a sigh of relief because at least he has overcome this difficult life challenge. He is really brave.”

Chan said her son’s lungs were so weak he still needed to be put on a ventilator.

“His lung function is only 10 per cent of that of a normal person,” she said.

“Without undergoing this surgery which prevents his lung function from deteriorating, his condition will be life-threatening.”

Doctors put Marcus on the drug Nusinersen more than two years ago when it became available in Hong Kong.

It is designed to increase production of a protein known as survival motor neuron, which is key to nerve control of muscles, and is injected into the spine. Nusinersen is the first drug of its kind for SMA patients.

In October 2017, the pleas of another SMA patient, Josy Chow Pui-shan, caught the attention of Chief Executive Carrie Lam Cheng Yuet-ngor, who pledged to make the drug available in the city.

But it costs about HK$6 million (S$1 million) per patient in the first year of treatment and HK$3 million annually after that.

Hong Kong has an estimated 85 SMA patients, according to 2017 figures, including 20 with the most severe form of the disease and who require frequent intensive care.

A passionate football fan, Marcus earlier this month received a letter from the manager of his favourite club, Manchester United.

Ole Gunnar Solskjaer passed on the well-wishes of the team and offered his own encouragement for Marcus to “stay strong through the challenging days”.