Nearly half of people with sickle cell disease do not have their condition taken seriously, according to a landmark survey.
About 48% of patients who took part in research looking at the experiences of people living with the hereditary blood disorder said they were treated like drug addicts when they tried to get emergency pain relief.
More than 1,300 patients and healthcare professionals from 10 countries, including Brazil, Oman, France, Bahrain, the UK and Saudi Arabia, participated in the survey, published by biopharmaceutical company Global Blood Therapeutics (GBT).
More than a third (38%) of patients said they believed their ethnicity was a factor in receiving poor healthcare. Sickle cell disease is most common in people in, or with ancestors from, sub Saharan Africa.
More than half of those who took part in the sickle cell health awareness, perspectives and experiences (Shape) survey, one of the largest conducted into the disease, said they received poor care because medics lacked knowledge about the condition.
As well as severe pain, 84% of patients said fatigue had the biggest impact on their lives, but only 53% of medics said they felt they had the tools to treat it.
On average, patients said they missed more than seven days of school or work a month and more than half said the disease had affected their mental health.
The human toll of non-communicable diseases (NCDs) is huge and rising. These illnesses end the lives of approximately 41 million of the 56 million people who die every year – and three quarters of them are in the developing world.
NCDs are simply that; unlike, say, a virus, you can’t catch them. Instead, they are caused by a combination of genetic, physiological, environmental and behavioural factors. The main types are cancers, chronic respiratory illnesses, diabetes and cardiovascular disease – heart attacks and stroke. Approximately 80% are preventable, and all are on the rise, spreading inexorably around the world as ageing populations and lifestyles pushed by economic growth and urbanisation make being unhealthy a global phenomenon.
NCDs, once seen as illnesses of the wealthy, now have a grip on the poor. Disease, disability and death are perfectly designed to create and widen inequality – and being poor makes it less likely you will be diagnosed accurately or treated.
Investment in tackling these common and chronic conditions that kill 71% of us is incredibly low, while the cost to families, economies and communities is staggeringly high.
In low-income countries NCDs – typically slow and debilitating illnesses – are seeing a fraction of the money needed being invested or donated. Attention remains focused on the threats from communicable diseases, yet cancer death rates have long sped past the death toll from malaria, TB and HIV/Aids combined.
'A common condition' is a new Guardian series reporting on NCDs in the developing world: their prevalence, the solutions, the causes and consequences, telling the stories of people living with these illnesses.
Tracy McVeigh, editor
Sickle cell disease changes the shape of blood cells into crescents, hindering blood flow. Sufferers experience severe painful episodes, which can require hospital admission. The drug Hydroxyurea can reduce the number of episodes, but the only cure is a bone marrow transplant. Life expectancy can be 20 to 30 years shorter than the general population.
About 5% of the global population carry the gene and some 300,000 babies are born with the disease each year. The majority with the disease in low-income countries will die before they are five.
“In recent years, we’ve started to see some positive change, but it is still not enough,” said Dianaba Ba, who lives with sickle cell and is director of operations at the French patient advocacy group SOS Globi: The Federation of Sickle Cell and Thalassemic Patients.
“The challenges for patients seem endless and this survey highlights that, in spite of this, patients do not feel heard. They continue to face acute physical but also emotional damage, stigma and discrimination.”
Manjusha Chatterjee, from NCD Alliance, said sickle cell and other non-communicable diseases are more than a health issue.
“They are a major human rights and equity issue, as they disproportionately burden the poorest and most marginalised populations. In countries everywhere, this includes ethnic minorities. Urgent steps need to be taken so that health systems are inclusive and no one is left behind,” she said.
“Although millions of people around the world live with sickle cell disease, it is still considered a rare condition. Simply getting a correct diagnosis of a rare disease is difficult – on average, it takes seven years.”
Dr Baba Inusa, professor and consultant of paediatric haematology at Guy’s and St Thomas’ NHS foundation trust in London, called the survey results a wake-up call.
“The Shape survey is important because it illustrates how vital it is that we understand our patients’ needs, and it suggests what we in the medical community can do to help change perspectives, increase education and awareness, and improve care,” he said.
“I believe that the actions that follow can enable us to help drive a better dialogue and improved conversations around the management and care of this long-neglected and devastating disease.”
