Sickle cell: ‘The revolutionary gene-editing treatment that gave me new life’

Jimi Olaghere thought he would have to wait decades to be freed from his sickle cell disease - but now scientists have engineered his blood to overcome the disease which left him in constant pain.

"It's like being born again," says Jimi, one of the first seven sickle cell patients to have benefited from a revolutionary new gene-editing treatment being trialled in the US. He says it has changed his life.

"When I look back, it's like, 'Wow, I can't believe I lived with that.'"

Jimi, 36, has lived with sickle cell since childhood. "You always have to be in a war mindset, knowing that your days are going to be filled with challenges."

The disease runs in families. It is caused by a genetic mutation that leads to the body making abnormal haemoglobin. This is the protein that is packed into red blood cells and carries oxygen around the body. Red blood cells are normally round and squishy, but mutated haemoglobin can make them rigid and take on their characteristic sickle shape.

gene edit 2

These sickled cells struggle to navigate the body's blood vessels and get stuck, leading to blockages that stop flow of blood. The risk of heart attack, stroke and organ damage are all higher in people with sickle cell disease. Jimi may need a hip replacement because part of his bone tissue has died after being starved of blood - an irreversible condition called avascular necrosis.

Pain has been a lifelong companion for Jimi. It is like "shards of glass flowing through your veins or someone taking a hammer to your joints", he says. "You wake up in the morning with pain and you go to bed with pain."

But the hallmark of sickle cell disease are the severe pain episodes called crises, which need hospital care and morphine to take the pain away. For years, Jimi was in and out of hospital almost every month. Winters were the worst - as cold weather narrows the blood vessels near the skin and increases the risk of blockages. It is why Jimi moved his family across the US - down from New Jersey to the warmer climes of Atlanta, Georgia.

gene edit 3

The disease has affected every aspect of his life. He is a technology entrepreneur because he couldn't imagine any employer being sympathetic to his frequent hospital visits. To escape from the constant pain, Jimi found "pockets of happiness" in video games and watching Liverpool play football.

His family urged Jimi to take part in other clinical trials or have a bone marrow transplant - which is an option for some people with sickle cell. But he thought they would involve too much time in hospital for the improvement in his quality of life. Instead, he pinned his hopes on a cure that was yet to be invented. He told his family: "One day in the future, probably 20 to 50 years from now, I'm going to get my DNA edited, and it's going to cure my sickle cell."

The future arrived a lot sooner than he imagined.

In late 2019, Jimi read an article about a new clinical trial using gene editing and he immediately emailed the medical team. A month later Jimi and his wife Amanda, who was eight months pregnant, travelled to the Sarah Cannon Research Institute in Nashville to see if he would qualify. When news came back that he had been accepted into the trial, Jimi said it was "the best Christmas present ever". And then he was lucky. The pandemic threatened to disrupt the trial as growing numbers of internal flights were cancelled due to low passenger numbers but he was able to commit to making a four-hour journey by car for each and every session. Continue...