A paper cut, or a simple scrape on the knee, is easily dismissed by most of us; but for people living with the rare blood disorder haemophilia, either could be life-threatening.
Ray Greenidge, 26, who was diagnosed as a haemophiliac at three, has refused to be limited by his condition; but he takes nothing for granted.
“In primary school, I had an injury to my ankle . . . . I was running and hit my knee. It was a small injury to most, but because there was internal bleeding to the joint, the joint was severely damaged. I was on crutches for seven years.”
Erica Worrell’s ten-year-old son Jaylen is also living with the disorder, and the mum readily admits it has been scary at times.
“For example, simple things that you take for granted –– like the change from baby teeth to adult teeth –– are some of the challenges I have with my son.
“When your tooth comes out, you bleed a little; and you rinse out your mouth. He would bleed for days off and on; he would have to be infused with the factor to stop that bleeding.”
Ray and Erica are executive members of the newly formed Barbados Haemophilia Association, which seeks to support haemophiliacs and raise awareness about the disease. Haemophilia is an inherited condition that affects the blood’s ability to clot.
Normally, when you get a bruise, substances in the blood, known as clotting factors, combine with blood cells called platelets to make the blood sticky. This makes the bleeding stop eventually.
However, in haemophilia, there aren’t as many clotting factors as there should be in the blood. This means someone with the condition bleeds for longer than usual.
Erica, president of the Barbados Haemophilia Association, told Health TODAY, more than 50 people in Barbados had admitted living with the condition, and since the group had been established yet more were coming forward.
She discovered her son had haemophilia when he had an unusual incident at just three months.
“My son just had an ear bleed and I tried to put a little pressure on it. I realize that while I was doing that the blood was flowing down my hand. I tried to apply a little ice because it was a hot day, but then as soon as it would clot, it would start flowing again.
“I spoke to my grandmother and she said probably it was the heat, and then she said even if it was the heat, he shouldn’t be bleeding so long.
“So I took him to the doctor the next day and the doctor said that sounds a bit strange, and she took me to the hospital to get tests done. When the results came back, I found out that he had haemophilia.”
Haemophilia is a life-long disorder, but it can be managed with treatment.
For Ray, who is still actively involved in sports, cycling, swimming and go-karting, it’s a case of knowing one’s body and taking care of it while getting on with life.
His wife says Ray is an inspiration. She says even in challenging circumstances he refuses to complain.
Erica no longer panics when an incident occurs. She is learning to take it one day at a time. With a laugh, she said she had adopted a new policy: “when in doubt call the QEH [Queen Elizabeth Hospital]”.
Added the Barbados Haemophilia Association president: “Jaylen acts like the average boy, but he knows his limitations. He knows no contact sports. He is allowed to play softball cricket; he bats well. He loves the beach, so he is allowed to swim. Jaylen has settled down and he is loving life.”
Haemophilia is not contagious, and both Ray and Erica want Barbadians to be aware of this. The disorder is a gene passed down from parent to a child.
The genes for haemophilia A and B are on the X chromosome.
Women who have the haemophilia gene are called carriers. They sometimes show signs of haemophilia, and they can pass it on to their children. For each child, there is a 50 per cent chance a son will have haemophilia and a 50 per cent chance a daughter will carry the gene.
In rare cases, a person can develop haemophilia later in life. The majority of cases involve middle-aged or elderly people, or young women who have recently given birth or are in the later stages of pregnancy. Experts say this condition is often resolved with appropriate treatment.
Common symptoms include big bruises, bleeding in muscles and joints, spontaneous bleeding (sudden bleeding inside the body for no clear reason; prolonged bleeding after getting a cut, removing a tooth, or having surgery; bleeding for a long time after an accident, especially after an injury to the head. Haemophiliacs also experience swelling, pain, stiffness and difficulty using a joint or muscle.
People with haemophilia usually receive treatment known as “factor replacement therapy” to help their blood clot. However, in some people, the immune system starts to believe the treatment is harmful. The immune system will then start to produce antibodies to block the effects of the treatment. These antibodies are known as inhibitors. Erica recounts her son’s experience with inhibitors. She says it’s a horrible experience.
“My son went through a year or so of having inhibitors. It is very painful. A bleed with an inhibitor is extremely painful. My son came near death’s door many times.”
Despite the challenges, Ray and Erica say haemophilia is not a death sentence.
They are hoping more people living with the disease will no longer hide it. At the same time, they are urging Barbadians who don’t suffer from the haemophilia to join the group.
Says Erica: “I wanted my son to know that you are not alone . . . . When I am having
a problem, I can call one of the parents. If they are having a problem, they can call me. We are here for one another.”