Patient gets her second wind
Elaine Wong, 50, is no stranger to challenges. She was born with a congenital bone condition called osteogenesis imperfecta that made her bones fragile and twisted her spine. But thanks to a loving and supportive family and her strong character, Wong did not let the disease get in the way of her life. She completed school and went on to pursue a career.
She knew her health was not robust, so she shrugged off her daily morning headaches, breathlessness and general fatigue. But she didn't realise that the twisting of her spine was growing more severe as she aged, thereby compressing her upper body, including her lungs. This prevented her diaphragm and chest wall from pumping air efficiently into her lungs.
Her lungs were unable to take in enough oxygen from the air she breathed and expel carbon dioxide. Her body was simultaneously starved of oxygen and poisoned by high levels of carbon dioxide, which resulted in, among other things, her morning headaches and fatigue. Wong was suffering from chronic respiratory failure and didn't know it.
Things came to a head in 1999 when Wong was in her late 30s. Her weakened respiratory muscles were unable to help her cough up phlegm and sputum in her windpipe, and she eventually came down with pneumonia and had to be admitted to hospital for more than a month. After recovering, she would fall ill again.
In 2000, Wong decided to seek care at the United Christian Hospital, where she met Dr Chu Chung-ming, a respiration specialist. At that time, Wong was in great discomfort - very short of breath even with minimal activity, and her heart was constantly racing.
Wong's blood test showed she had respiratory failure. While healthy people have between 96 and 100 per cent saturation of oxygen in their blood, Wong had levels as low as a life-threatening 40 per cent.
Chu noted that Wong's lungs could perform the gas-exchange function normally, but it was the compression of her thoracic space by the crooked spine that caused her body's pump function to fail. So, she needed an external or artificial pump to help bring enough air into her lungs. Because her condition was irreversible, Wong would need long-term care or help to breathe.
Conventionally, invasive ventilation is used. A tube is inserted into the patient's mouth or neck to pump pressurised air into the lungs. This requires a prolonged stay in hospital, involves much pain and discomfort, and increases the risk of contracting pneumonia.
Chained to the hospital bed, the patient is not able to speak, eat, sleep or function normally. If a patient wants to be cared for at home, the total cost is well over HK$100,000 a month, excluding trained help.
But technological advances enable non-invasive ventilation (NIV), whereby the patient only wears a special mask. The mask is attached to machines that pump pressurised or oxygen-enriched air.
Although this option has been available in Hong Kong hospitals for about 20 years, improvements in technology and training mean that patients can now undergo the treatment at home.
Chu suggested Wong consider buying or renting an NIV machine for use at home during sleep. While sleeping, respiratory muscles tend to rest, says Chu. Sleep then becomes a highly vulnerable time as the pump function weakens further.
The machine would cost between HK$10,000 and HK$60,000 to buy, or from as little as HK$1,000 a month to rent. With a little training, the machine is easy to use as Wong needs only to put on the mask, turn on the machine and go to sleep.
The normalisation of the oxygen and carbon dioxide levels that take place during sleep will help support her body during the daytime as she goes about her life.
But Wong was not keen. She says she did not like the idea of being hooked up to a machine because it made her feel disabled. She was also afraid that it might take away her ability to work and that it would cause a great deal of inconvenience.
Chu advised her that without the NIV treatment, Wong would grow progressively weaker and become unable to care for herself. Her life was also at risk. Conversely, studies in Europe showed that patients with similar conditions who used the home NIV treatment enjoyed the same life expectancy as others.
But he knew that patients needed time to digest the idea of lifelong dependency on a machine. He asked Wong to think about it. A month later, Wong agreed.
Initially, Wong had to get used to sleeping with the mask and the machine. The machine was noisy, and it was uncomfortable having a mask strapped to the head. The first night, she hardly slept.
But she grew accustomed to it within a week. She noticed improvements to her health. The morning headaches vanished, and her energy levels picked up. She could concentrate better and no longer felt as fatigued or breathless. Wong can also walk further distances and prepare her own meals.
Although she needed to add oxygenated air to her treatment in 2007, Wong has not had to be admitted to hospital since she started using the machine.
Wong says: 'This disease has brought me limitations, but my life is now good. I can even go out for an hour or two unless the air outside is bad.'