When Valerie Porter from Bangor was 19, doctors gave her 20 years to live. 38 years later, and still defying their prognosis, Valerie has shared with us what it is like to live with cardiomyopathy, a hereditary heart condition that has caused her to develop Atrial Fibrillation and a stroke.
Valerie was brought up in Bessbrook, one of five children. When she was around 7 years old, she told her mum she thought there was something wrong with her heart. Her mum took her to the doctor but was told it was just a heart murmur, most of which require no treatment, and nothing to worry about.
However as she got older, it became more noticeable. She would get breathless when other children didn’t. On a Sunday, she used to take her uncle’s dinner to him, a ¼ mile cycle uphill, but she couldn’t cycle it and had to walk.
Eventually, at the age of 19, she was sent to see heart specialists who diagnosed her with hypertrophic cardiomyopathy. Doctors said the best prognosis they could give her was 20 years. It was a shock, but 20 years still seemed a long time to a 19 year old, so it didn’t upset her unduly.
In hypertrophic cardiomyopathy, the walls of the heart chambers thicken which means the chambers are reduced in size and can’t hold as much blood. The walls of the heart chambers stiffen which makes pumping the blood around the body more difficult. Valerie is what clinicians describe as very symptomatic, which is rare. Unlike most people with the condition, Valerie has always been very aware of her symptoms.
Following the diagnosis, Valerie was put on beta blockers and fluid tablets, had appointments at the City Hospital every three months and life went on. She got married shortly after the diagnosis but was advised against having children because they would be unlikely to survive past infanthood.
For many years, the cardiomyopathy did not cause Valerie many problems. She always noticed that she would be more out of breath than anyone else doing the same thing. She took up hockey and had to hide how breathless she was from her friends. She taught in a college and could feel palpitations when she was taking the stairs to the top floor where her classroom was situated. There was no lift and she had to stop frequently to get her breath on the way up.
As Valerie’s cardiomyopathy worsened, she went into AF. Atrial Fibrillation (AF) is the clinical term for a type of abnormal heart rhythm. When someone has cardiomyopathy and the main heart chambers become stiff, this can lead to back pressure on the smaller collecting chambers. In turn, this can lead to AF.
The first time Valerie went into AF, she was given a cardioversion, a treatment that returns your heart to the correct rhythm by passing electricity through it. The first cardioversion lasted 1½ years and during that time her heart was stable. However, the second treatment only lasted a week. In total Valerie had eleven cardioversions, one more than the recommended maximum of ten. Sometimes she was back in AF before she left the hospital.
The AF is much more difficult to live with than the cardiomyopathy. Valerie can feel when the AF is coming on. When she can feel when her heart is going out of its normal rhythm, she sits down and relaxes until it passes. Sometimes it is not so easy. One day when she was walking her dogs in Crawfordsburn, her heart went funny and she knew she was going to black out. She sat on the bank and lost consciousness. The dogs sat with her until she came round again.
In her mid-30s, around the same time as the AF developed, she was offered a myectomy, an operation that removed part of the heart muscle wall, to make it thinner. Valerie was not keen and sought a second opinion through the Cardiomyopathy Association, a London based organisation. She was seen by Professor McKenna, one of the world’s leading experts on cardiomyopathy and president of the Cardiomyopathy Association. His view was that the operation was not necessary. However, he advised her to give up her work.
Valerie loved her job. She’d trained as a hairdresser but while she had been on a part-time course on advanced hairdressing, she was offered a full time job. Teaching was her dream so she left the salon and took the job. So giving up work was very difficult but it was the only way to avoid open heart surgery.
Soon afterwards, Valerie had a stroke.
She’d just returned from a weekend in Italy. She’d been out of breath a lot, carrying her case around Milan and sightseeing, but this was not unusual. A few days after her return, Valerie was visiting a friend when suddenly she couldn’t talk. Next her arms went limp. Valerie had no headache or pain but she was totally disorientated. Her friend realised what was happening and called 999. She knew it was a stroke. She was able to walk out to the ambulance but then lost consciousness. When she came round, she thought she was talking coherently but it was just gobbledygook. She was also paralysed down one side.
She spent 4 months in hospital, 2 months in the Ulster and 2 months in Musgrave. Her speech soon returned but she still has limited mobility on her right hand side. She has to wear a splint on her right leg and has special shoes which she dislikes. She got used to writing with her left hand but she uses the iPad and computer a lot.
When she was in hospital, Maureen Boyle from Northern Ireland Chest Heart and Stroke came to see her. Valerie was feeling very low. She talked to Maureen, and though her depression didn’t lift, she agreed to go to the support groups the charity runs. She’s been going to the Young Stroke Group for ten years now. She says it gets her out of the house and is fun.
Nowadays she also has carers to help her get out more, for example to the gym. She is also able to drive herself places. Her visits to the gym, to build muscles for balance and strength, have paid off. She goes for spasticity injections at Musgrave every four months to counteract the contractions in her leg and arm muscles. However, on the last visit, the nurse said she didn’t need the injection in her leg due to some progress made!
As well as making some physical progress in recent years, Valerie feels like her brain is now understanding things more easily. She can work the TV better and explain things more clearly.
Despite the physical effects of the stroke that Valerie has to live with every day, she says that the cardiomyopathy is worse than the stroke. It is the bigger risk to her health and life. She does not like the stroke defining her. It is cardiomyopathy that has been her life, not the severe stroke.
Valerie has some secondary stroke related problems such as arthritis in her right leg. She needs a hip and knee replacement but due to her severe cardiomyopathy, doctors are unfortunately able to perform these procedures.
Find out more about the cardiac support services that Northern Ireland Chest Heart and Stroke offer.