Living with emphysema: 'When you cannot breathe it's very frightening, you are scared to go anywhere in case you have an attack'
When Marion Kelly was invited to take part in a clinical trial for a particular drug, she jumped, albeit breathlessly, at the chance. She tells our reporter that if she hadn't been given that opportunity, she might not be here today
When Marion Kelly (52) took part in a clinical trial, she didn't know if she'd been given a placebo, or the drug in question. But within months, she felt so much better, she had a sneaking suspicion she'd been given the actual medication being tested. Now, 10 years down the line, she is immensely grateful that she did get the opportunity to take part in that trial.
Marion has suffered from breathing problems ever since she opened a hairdressing salon in Co Tipperary, about two decades ago. She'd learned her trade in Dublin, having been apprenticed there for four years. But as soon as she could, she hightailed it back to her native county, and there she stayed, in spite of the fact that she had experienced some terrible tragedies on the home front. The first happened when she was just eight years old, when her beloved mother died following an operation for a pancreatic condition.
The second was the result of an incident in which Eddie, her 20-year-old brother, swallowed an excessive amount of water in a swimming pool in London. "He was able to go home afterward," explains Marion, "but then he smothered in his sleep." She is immensely comforted by the fact that two close friends happened to pop in to visit her brother shortly before his unexpected demise. "It helps knowing Eddie wasn't completely alone that night," she says. Marion was 21 at that time; her daughter Aideen was just 17 days old.
After Marion's marriage failed, she got on with the business of being a working mother. At first, she was employed by a salon in Puckane, the village where she was reared. Then she opened The Hair Studio in Nenagh. "I absolutely loved having my own business," she enthuses. "I met many wonderful people. You'd hear such stories. Some were hilarious, some were sad, and some were just too much information. One of my favourite regulars was Terry. She could say anything to me and I could say anything to her - and I mean anything. I have such fond memories of her and of my other clients."
But there were difficult times too, when Marion had to attend to customers who were having problems at home or at work, or were experiencing illness. On two occasions, she did the hair of clients who had died. "It was sad, but I was glad to do it anyway," she says. Soon after she opened the salon, Marion began to experience breathing problems. Her GP diagnosed asthma. Products such as hairspray and ammonia, in common use in a hair salon, were making her worse. She was given a nebuliser, which converts asthma medication into a mist, which can then be breathed through a mask or mouthpiece. Nonetheless, as time went on, Marion's condition worsened. She would get breathless doing mundane tasks, such as carrying towels around the salon. Then one night she woke, unable to breathe, and got a terrible fright. Eventually, she returned to the GP, who referred her to Limerick Regional Hospital. "The consultant did some blood and breathing tests," Marion recalls. "But when he came to the breathing tests, I was so bad, he thought the machine was broken." Three weeks later, she was diagosed with alpha-1 antitrypsin deficiency (alpha-1). According to the Alpha One Foundation, alpha-1 antitrypsin is a vital protein produced by the liver to protect the lungs from infections and inhaled irritants. It's the most common genetic lung disorder in Ireland, affecting up to 250,000 people. Consequently, a national alpha-1 screening programme has been established, to reduce the under-diagnosis and misdiagnosis of the condition.
And while genetics plays an important role in alpha-1, smoking is particularly harmful for patients. Marion had been using tobacco products since she was a teenager. "The consultant told me I already had emphysema, and that he would 'kill me', if he ever caught me smoking again," she says. So Marion stopped there and then. She was also given oxygen to use at home.
In time, Marion's condition worsened even further, so she was referred to Professor Gerry McElvaney at Beaumont Hospital. "I knew it was serious if they were sending me to Beaumont," she says. Following tests, she learned that both her parents had been carriers of the defective gene. Marion also discovered that she had the "worst form".
At that time, a decade ago, Prof McElvaney was involved in a clinical trial of a new drug, specifically for patients with alpha-1. Marion readily agreed to take part in the trial. "A nurse gave me the 'drug' in an intravenous drip at home," she explains. "You didn't know if you were getting the drug or a placebo. But after six or seven months, I knew I was getting the real medication, because I was so much better. My breathing was easier and I had a lot more energy."
This trend continued. Now, instead of being hospitalised on average four times a year, it's down to once every three years. But even more importantly, the improvements in Marion's health have helped restore some of the confidence she lost when she was more acutely ill. "When you cannot breathe, it's very frightening," she explains. "You are scared to go anywhere in case you have an attack. I fell once, and my foot got jammed. Then I started to panic and that made my breathing even worse. I tell you, I thought the end was nigh, on more than one occasion."
She says one of the other remarkable effects of this particular drug is that it appears to be slowing down the progression of her disease. "My breathing has remained steady, and that's amazing, because before the trial, it was going downhill fast. I believe that if I hadn't had access to this drug, I'd have died a relatively young woman."
Marion still needs at-home oxygen 16 hours a day, but otherwise she leads a pretty normal life. She likes to potter about in her pretty flower garden, and to write quirky or meaningful sayings in calligraphy, in her leather-bound notebook, or on the white bathroom walls.
She is also very close to her extended family and has a wonderful relationship with her daughter, Aideen, who is a beautician and lives nearby. Jodie, her granddaughter, is also the apple of her eye. "Even though she's only 12, she knows all about my oxygen, all about my meds, and she's really good at calming me down when I panic," says Marion, glowing with love and deep gratitude.
For more information, contact the Alpha One Foundation, tel: (01) 809-3871 or see alpha1.ie
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