A better quality of life (APS)

Increasing someone’s quality of life is just one of the many ways in which blood and plasma donors assist the local community.

Joe Guarnieri knows this first-hand––he benefits from the generosity of more than 80 donors every day.

Joe is this year’s Australian Public Service (APS) Defence Blood Challenge ambassador. He was diagnosed with the inherited bleeding condition haemophilia B soon after birth.

“It doesn’t always get diagnosed straight away but usually gets noticed during teething or when a child starts to crawl,” Joe says.

“That’s when it came up for me. Mum first knew something was up when we went to Italy and I was teething on the plane and I just didn’t stop bleeding.”

Haemophilia A and B affect about 1 in 6000-10,000 males worldwide with varying levels of severity.

Joe has a severe form of haemophilia B which means his body produces less than one per cent of clotting factor (Factor IX) found naturally in blood that helps to control bleeding. He requires daily injections of Factor IX concentrate to replace the clotting factor he is missing, but this was not always the case.

“There were stages in my life where I couldn’t have any blood products,” Joe says.

“I had antibodies to the product, which meant my body defended against it and would reject it.

“Antibodies are fickle things and developing an antibody is probably one of the biggest complications for people with haemophilia. The health-related problems that I have now are as a result of not having any product.”

Joe says these complications are not always what people expect.

“A lot of people think that I’ll cut myself and bleed to death, but it’s not like that,” he says.

“I always think of it like a slowing down of the clotting mechanisms in the blood and the issue is really more about internal bleeding and bleeding into the joints rather than cuts.

“If people don’t have access to blood products or can’t have them…then they can go for months with multiple joint bleeds. There were long periods in my life where I was out of action for months with bleeds, which are quite painful. Not only that but they destroy the joint. It only takes three internal bleeds for the joint to be permanently damaged. So the importance of factor concentrates is really critical to a person with haemophilia.”

At the time, treatment was not available in Australia so Joe sought treatment in Sweden to get rid of the antibodies.

“When I could have blood products for the first time it was nothing short of a miracle. I wouldn’t be here if it wasn’t for that.”

Unfortunately, five years ago Joe’s antibodies started to develop again.

“Luckily it hasn’t come back as strong as when I was a kid and the blood products still work, but they don’t work as well as they did,” he says.

Factor IX concentrate is made by pooling blood and plasma donations. Joe takes a minimum dose of 3000 units each day. It takes 186.6 blood donations or 83.4 plasma donations to make 3000 units of Factor IX concentrate.

“Factor concentrates are so important for people with haemophilia,” Joe says.

“It’s really important for people to realise we need blood products to ensure our lives are healthy. Without products I wouldn’t call it much of a life; you get by but the struggles are on a totally different level.

“While at the moment it’s a bit of background pain and tiredness, without products it’s severe pain, monthly disruptions with every bleed and deterioration of joints to the point that you’ll eventually be in a wheelchair.

“One donation can help so many people and there are so many products that come out of plasma donations that help people with various conditions.”

Joe has been working for Defence for 20 years and has had a variety of roles from project management to software development. He says it means a lot to him to be able to give something back.

“Defence has been really good to me so I want to show my appreciation in ways that I can,” he says.

“The services have always been really good at donating blood, but I would like to see more of a civilian uptake. To get more civilians donating within Defence, and also within Canberra, is what I’d like to try to help achieve this year.

• “If giving blood is something you have always thought of doing, take the opportunity to start during the Defence Challenge.”