“I’ve had sickle cell anaemia since I was born, but my earliest memory of it was when the head teacher in primary school picked on me because I wore socks over my tights to keep my ankles warm,” says Pamela. Keeping warm can help prevent a sickle cell crisis, or bout of pain.
“One of the reasons I want to raise awareness is so that people can understand how the disease affects others, and how they can be sensitive towards sicklers, especially in schools.”
Talking about sickle cell
Pamela got involved in publicising sickle cell disorder after letting her consultant and her sickle cell advisor know that she wanted to raise awareness. She has since given interviews in the national media, talking honestly and openly about her life with sickle cell.
“I want people living with sickle cell to have more confidence in themselves and to realise they aren’t the only ones, and that we can still achieve,” she says. “It gets a bit stifling working hard for something, like exams, and not being able to do well due to bad health.”
Sickle cell disorder (SCD) is a group of inherited blood disorders that evolved thousands of years ago in areas of the world where malaria occurs. The most common is sickle cell anaemia. Most people affected by sickle cell anaemia are of African or Caribbean origin.
SCD affects the blood’s ability to carry oxygen around the body. A protein called haemoglobin doesn’t work properly, and turns red blood cells into sickle (crescent) shapes. These can get stuck in blood vessels and block the flow of blood, causing terrible pain, known as a sickle cell crisis.
People with sickle cell anaemia can also feel very tired. Resting, drinking water and keeping warm can help stop a crisis coming on.
How a crisis affects your life
“Crises come on in different ways,” says Pamela. “Sometimes I feel pain in my arm, deep in the tissue. Sometimes the pain is in my leg or waist, which is worse. It feels a bit like electric shocks. I take painkillers and drink lots of water to try to stop it coming on. There are times when it’s too painful to move, and I just have to rest in bed.”
Her most serious crisis happened when she was 16. Severe pain in her shinbones meant Pamela couldn’t bear any weight on her legs, and she was taken to hospital in an ambulance on Christmas Eve. “They gave me morphine and I was allowed home after five days,” she says.
Pamela had to take months off school, and was unable to walk without a stick or someone to lean on.
She says her biggest difficulty has been the effect on her education. “My GCSE predictions were A stars, As and Bs, but I got eight Bs,” she says. “I missed some work deadlines as I was in hospital. If I could just have at least 80% attendance, I know I would achieve the grades my brain is capable of.”
Pamela repeated the year and is now studying for A levels in maths, biology and chemistry. She hopes to become a doctor.
Family history
Pamela’s family is supportive of her work to publicise sickle cell anaemia. Her parents, originally from Ghana, both have sickle cell trait. This means they have no symptoms but they have the gene associated with the disorder, and can pass it on if they have a baby. They weren't aware of this until they had Pamela. Their three younger daughters don’t have SCD.
There's now a screening programme to detect sickle cell trait in pregnant women and sickle cell disorder in newborn babies. Men and women can get screened before starting a family.
Since April 2007, Pamela has had blood transfusions every month to help stop her body producing sickle cells. As a result of the transfusions, she is also having treatment to prevent the build-up of iron in her blood, something that people with sickle cell cannot cope with.
“Sometimes I feel sickle cell spoils my dreams, but I have to stay positive and think of other ways of doing what I want,” says Pamela. “I want to get people talking about sickle cell disease, so there’s less stigma."
