Sickle cell: A victim of bigotry?

Sickle cell: A victim of bigotry?

When Hellen Adom, who has sickle cell anaemia, was just 16-years-old doctors told her she might only live another 10 years.

She confounded the pessimists, and now, aged 43, she is still well.

However, she is also very aware that despite medical advances, the average life expectancy for a person with sickle cell is still only 47.

Sickle cell is an inherited genetic condition in which there is an abnormality in haemoglobin, the oxygen-carrying protein found in red blood cells.

Some experts fear research into the condition is being underfunded because it mainly affects ethnic minorities.

More information needed

"I can't really remember knowing that I had sickle cell before I was about 16," said Hellen.

"But I knew that I had something. I was always a frail child and used to have crises, where I would go to bed in pain.

"My mum would keep me off school and keep me warm and well hydrated.

To have terminated because they had sickle cell would have been like terminating myself before I had time to live

Hellen Adom

"Then when I was 16 they told me that the average life of a sickler (person with sickle cell) was 28.

"I was devastated. I was only 16, I had just started living."

Hellen, who had five brothers and sisters, was the only one with the condition in her family and when she was growing up there was little information around for her.

This became a huge problem when she settled down, and began to start a family.

"Nobody told me that my husband and I were at risk of having children with sickle cell until I was well into my first pregnancy."

Her first child Ben, who died aged 11, from meningitis, was negative, but her second child Misha, now 18, has the condition, as does one of her twins, 15-year-old Simeon. The other twin Sam is clear.

Doctors found that not only did Hellen, from Blackheath, London, have sickle cell, but that her husband had sickle cell traits - meaning that three out of the four relevant genes inherited by their children would be affected.

"I had the tests but I could never have terminated the pregnancy," she said.

"I had sickle cell and yet had enjoyed my life."

Ethnic funding bias claim

Today more information is available about sickle cell, but experts believe it is still a condition which remains largely overlooked, both in terms of research and support services.

Dr Phil Darbyshire, consultant paediatric haematologist at Birmingham Children's Hospital, is one of those who believes ethnicity was an issue in funding provisions.

"These minority communities do not have a high degree of awareness of these conditions and do not have a strong political voice to lobby for improvements in services," he said


"There is at present a great deal of practical work going on to assess what is needed within the haemoglobin disorders sickle cell and thalassaemia, to improve services, to lobby those commissioning services and to set up standards and appraisal systems similar to those developed several years ago in the cancer arena.

"However if these were disorders that mainly affected the Caucasian population, such as leukaemia, I think there would have been more funding available over the years."

More needs to be done

Lorna Bennett, Chairwoman, Sickle Cell Society agreed: "Around 12,500 people in England currently live with sickle cell, compared to the 7,500 people affected by cystic fibrosis.

"Both are genetic diseases yet, sickle cell, which affects predominantly black and minority communities, has been comparatively under-funded.

"This is no longer acceptable. More needs to be done to help those living with sickle cell and thalassaemia receive the care and support, both in the community and in hospitals, to ensure the best quality of life possible."

Dr Allison Streetly, of the NHS Sickle Cell and Thalassaemia Screening Programme said more is being done


"We have come such a long way since the programme was established in 2001. Every child born in England is now offered screening for sickle cell disease.

"Sadly, however, investment in care for these genetic blood diseases has historically not been adequate for the needs of the most affected populations.

"I hope that in the future co-ordinated and managed care and support will be as developed for these conditions, as they are for other serious children's conditions, such as children's cancers".

Hellen, who won the Floella Benjamin Award from the Sickle Cell Society, said she was already educating her children and others about the disease.

She has also written a book about families living with it and did her MA on the subject.

She is determined that nobody should be as ignorant as she was.