Treatment of sickle cell anaemia is compromised by health workers' lack of knowledge, a report warns.
The first national survey of the disease found seriously ill patients were not offered support from sufficiently experienced staff.
Patients' use of painkillers was not effectively monitored, in some cases leading to in fatal overdoses, it said.
The inherited genetic disease affects the ability of the red blood cells to carry oxygen around the body.
It can cause severe pain and damage to the organs.
Not enough support
The charity, the National Confidential Enquiry into Patient Outcome and Death, carried out the research into the deaths of 55 patients with sickle disease or the related condition thalassaemia.
Of 19 patients in the study who had complained of pain and who subsequently died in hospital, nine had been given excessive doses of painkillers and five of those patients died because of complications due to overdose.
The report "A Sickle Crisis?" also found the cause of death of some patients was unclear.
"Doctors and nurses need to be more familiar with what needs to be done if patients' vitals signs become abnormal "
Professor Sebastian Lucas, one of the study authors, said: "We were surprised that our review found such a high number of cases where we did not know the actual cause of death.
"This is a wake-up call to the clinical community. Sickle cell disease is as common as cystic fibrosis, yet less is known about the severe complications that can lead to death in sickle cell disease patients."
The report also found the take-up of vaccinations by sickle cell disease patients at their GP practice was low.
Dr David Mason, co-author of the study, said: "Doctors and nurses need to be more familiar with what needs to be done if patients' vitals signs become abnormal.
"We need a multidisciplinary approach to acute pain management and regular reviews of therapy to control pain adequately."
People it affects
Around 12,000 people in the UK have sickle cell disease and it is one of the most common reasons for hospital admissions.
Sickle Cell mainly affects people of Afro-Caribbean, African, Eastern Mediterranean, Middle East and Asian origins. Thalassaemia mainly affects people from Asia, the Mediterranean and the Middle-East.
The demographics of where patients live have changed. At one time sickle cell disease was mostly found in London and the West Midlands, but it is now across the UK.
A Painful Condition
Dr Mason said "We need a supporting health network from GP practice to hospitals to expert centres, to ensure that their care is managed well regardless of where they are in the UK."
Professor Lucas said it is now vital to set up the national database of sickle and thalassaemia patients proposed by the Department of Health because of the scale of the problem.
"Only when we have this framework for the uniform collection of clinical and outcome data, can we then audit patient outcomes and treatments.
"Then we will understand the disease better and so improve the management and quality of acute and chronic care."
Dr Asaah Nkohkwo, chief executive of the Sickle Cell Society helped with the NCEPOD study.
He said: "I am appalled by the findings. They show how much of a gap exists in the delivery of the care services for sickle cell patients including the lack of knowledge of health professionals and the consequences that families affected have had to face as revealed by the autopsy reports."
Dr Nkohkwo said the research has been used in the development of the NHS standards of care document for adults with sickle cell disease which is being launched at the House of Commons next month.
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