ALBERT Wirth always considered himself to be healthily robust. Up until three years ago the 58-year-old former delivery driver liked to unwind by lifting weights at the gym. Strong, fit and employed in a job he loved, little did he realise that his world was about to be turned upside down.
The only indication there was anything wrong was a need for him to have regular B12 injections to treat a vitamin deficiency. When his leg started to swell after a few months on the injections, he went to see his GP who took a blood test.
That same day, as he headed back to work, he received an urgent call from the surgery. Albert, of Abergele, said: “My doctor told me to go straight to the hospital for more blood tests.”
When he protested, the doctor more or less told him that if he didn’t it could be a matter of life or death. The blood test revealed that the platelet levels in his blood were at dangerously low levels. “My platelet count was 4,000. The average count is anywhere between 150,000-400,000,” he said.
Evans Syndrome is an auto immune disorder in which the body makes antibodies that destroy bloods cells – red, white and platelets.
In Albert’s case it was the platelets that were being attacked – a condition called Thrombocytopenia. This leaves him susceptible to internal and external bleeding, from the gums and nose, blood blisters in the mouth and down the back of his throat. The slightest knock could lead to an internal bleed with potentially fatal consequences.
Those six weeks in hospital proved to be the toughest in his life. Looking back at that time, he said: “It was very scary not knowing initially what was wrong and what was going on. I had endless blood tests – so many that one of my veins collapsed – and various types of treatment, including steroids and a couple of doses of chemotherapy which didn’t work.
“My gums and nose started to bleed, I was spitting out blood clots. I also had a platelet rash which are small red spots on the skin all over my body.”
One treatment which worked for a time was a platelet boosting drug called Romiplostim. “It helped in some ways in getting rid of the spots and made me feel a bit better, but they didn’t raise my platelets enough to carry them on which is a shame.”
In the end, while the worst of his symptoms eased, they could not find a cure. He was allowed home but had to go back to hospital every second day to monitor his condition.
Three years on and that has stabilised to fortnightly trips to Merseyside for blood tests and monthly visits to see his consultant, excluding emergency visits to Ysbyty Glan Clwyd, Bodelwyddan to stem his regular bleeds and a dizzying array of complications he has developed since his diagnosis. These include five mini-strokes, heart failure, osteoporosis and diabetes.
Life has got better but in a sense he feels he has only been give a stay of execution. “It is like having a death sentence hanging over my head,” he said.
That uncertainty about his future has had a corrosive effect at times on family life. Albert, or Bert as he is known to wife Amanda, has two children at home Callum, 15, and Sarah 18 . The couple also have four grown-up children between them.
The hardest part of living with Evans Syndrome is the impact it has had on his relationship with his two young children. “There are times when I get down and grumpy with the kids. Callum used to like to play fight and he gets upset that I can’t do that anymore. It’s the little things like not being able to take them out. The boy has a fishing rod but I can’t even go out and do that with him.”
There are times when this has left him in a dark place, but overall he has been determined to remain positive about his plight. “I like to have a laugh at life. While I know this has been a nightmare, not just for me but the family, I am trying really hard to stay positive.”
His outlook may be positive but he is also realistic about living with an incurable, potentially fatal disorder.
“The truth is no-one knows too much about what triggers the disease, and so far there is no cure. Some people go into remission but that is usually children who have developed the disease. At times my platelet levels have been close to zero and when my nose bleeds the blood just oozes out.
“I always have my notes with me in case I’m taken into hospital. I can’t do any exercise as such and I can’t even go swimming. If you use your muscles too much it can lead to internal bleeding. I have had five mini-strokes and a bleed on the brain. I get out on my mobility trolley and do what I can but that is not a lot.”
He says this with a laugh and the key to his survival over the last few years has been keeping his sense of humour. “I take every day as it comes but at the moment all I can hope for is that it will get better.”
His wife Amanda has been there at his side throughout the last three years offering her unstinting support. She said: “It’s a horrible disease and we want to raise more awareness of it so that more research can be done to find out what causes it and to find a cure.
“Bert has recently been to see a herbalist and that has helped ease some of his symptoms and given him and us a little bit of hope. But it has had a devastating effect on the family.
“It’s the uncertainty of it all. When someone is diagnosed with a disease like cancer, it’s horrible of course, but people can plan for some kind of future. Bert could have a major bleed and die today.”
That is why she is in the process of setting up a support group for other families in the UK affected by the syndrome. She has created a Facebook page called Evans Syndrome UK where people can get in touch. She is also organising a walk from Pensarn to Llandudno on March 31. To take part can call her on 07595702010.