Dedicated to helping sickle cell patients
About sickle cell disease
SCD describes a group of inherited red blood cell disorders. People with SCD have abnormal haemoglobin, a protein in red blood cells that carries oxygen throughout the body.
Sickle-shaped cells can stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen cannot reach tissues close by.
There are different types of SCD and different levels of severity. Sickle cell crises — painful events that occur in the hands or feet, belly, back, or chest — are the most common symptoms of sickle cell disease. This pain may last from hours to days. Most people with sickle cell disease also get anaemia.
SCD is genetic and is not contagious.
The drug Hydroxyurea and regular blood transfusions are typical treatments for SCD.
Edward “Donnell” Ivy’s hips started failing him when he was 9.
By the time he reached university, he would often suffer terrible pain that would put him in hospital for weeks.
Then the crisis hit. The then 23-year-old was on duty as a resident paediatrician, and could not breathe.
A haematologist diagnosed acute chest syndrome. The potentially life-threating lung-related complication is linked to sickle cell disease and can lower the levels of oxygen in the blood.
“I had to be intubated,” Dr Ivy said. “They called my parents because they weren’t sure if I was going to make it.”
The haematologist prescribed a daily dose of a drug called Hydroxyurea. It stopped his severe pain and changed the doctor’s career path.
“I realised there were already effective drugs and treatment options out there for patients with sickle cell,” he said. “The problem was patients, their families and their doctors often didn’t have access to information about treatment options and resources.”
Since then, he has made it his life’s work to ensure that patients have access to the resources they need. More recently he has achieved this through the National Institutes of Health in Washington DC. “I’ve worked on sickle cell clinical practice guidelines,” the 44-year-old said. “I’ve also worked on projects involving cardiovascular disease and diabetes. We have a lot of knowledge about these diseases, but how do we help people live a healthier lifestyle?”
The North Carolina native visited Bermuda this week to help observe Sickle Cell Awareness month here. He spoke with members of the Bermuda Sickle Cell Association and talked about treatment options with the medical community.
His advice to people with sickle cell is to not let the disease get in their way.
In his early life, that advice was hard to follow.
SCD exploded his childhood dream of becoming a fighter pilot.
“My stepfather was in the military and I spent part of my childhood on an air force base,” he said.
“I dreamed of flying jets, and I was determined, even though I knew the military didn’t admit people with SCD.”
The US Air Force does not accept people with sickle cell disease as they have severe pain crises in times of high stress.
Dr Ivy got as far as a home interview with an air force recruiter. Then his mother walked in on them and dropped the bomb.
“She said, ‘Donnell, you know you can’t join the air force, you have sickle cell’. After that, I knew it was all over.”
He resolved to go into research and then he found his career path.
“There are limitations to what you can do,” he said. “Obviously, you can’t join the air force, but there are lots of other things you can do. It is important to learn about your body and what you can take in terms of physical exertion. You can do a lot.”
• Visit Bermuda Sickle Cell Association on Facebook or e-mail email@example.com.
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