UK sickle cell specialists are calling for a database of patients with sickle cell disease and thalassaemia to be set up after the first national survey of deaths caused by these conditions showed that many patients did not receive care based on best practice.
Cause of death also needs to be better evaluated, as the results showed an unexpectedly high number of cases where the precise cause of death was unknown.
The enquiry, coordinated by the National Confidential Enquiry into Patient Outcome and Death (NCEPOD), asked all hospitals and primary care trusts in the United Kingdom to provide information, including case notes, for patients who had died from haemoglobinopathies over a two year period from 1 January 2005 to 31 December 2006.
Expert reviewers assessed the ongoing care of 55 patients and the final episode of care for 41 patients for whom full notes were provided, from the total of 81 deaths reported. These patients ranged in age from one year to 77 years, with six patients being children under 16. More than two thirds of the deaths occurred in hospital.
The causes of death for the 40 adults with sickle cell disease were typical for the condition—seven deaths were caused by stroke and three by multiorgan failure. The cause of death, however, was unknown for 11 patients.
David Mason, consultant anaesthetist at the John Radcliffe Hospital, Oxford, a clinical coordinator with NCEPOD and coauthor of the enquiry report, said, “The results highlighted that haemoglobinopathies are complex and complicated diseases. We found a surprisingly high number of cases where we did not know the actual cause of death.”
To improve information on the conditions, the expert panel has recommended setting up a national database of patients with sickle cell disease and thalassaemia. This would provide a framework for systematic collection of clinical and outcome data, to allow audit of patient outcomes and treatments.
“Haemoglobinopathies are now the most common inherited diseases in the UK, and the prevalence of sickle cell disease is increasing with the growing number of people of African and Caribbean origin,” Dr Mason noted. “Sickle cell disease affects every organ of the body, so we need much better information on these patients and their management in order to improve their care.”
The reviewers considered that care during the final clinical episode before death followed good practice in fewer than half of the patients (17/41). They identified room for improvement in the clinical care provided to just over a third of patients (14/41) and considered that several aspects of clinical or organisational care were unsatisfactory in a sixth of cases (7/41).
One of the major problems was that patients with sickle cell anaemia who became acutely ill and were admitted to hospital were often not cared for by sufficiently experienced medical staff.
As a result, the report recommended that patients with sickle cell disease or beta thalassaemia major should be managed by, or have access to, clinicians with experience of haemoglobinopathy management. This could be achieved by setting up networks to ensure that all patients have access to expert care.
The inquiry highlighted a particular need to improve the monitoring of patients with sickle cell disease who are given powerful analgesics, such as opioids, to avoid overdose and respiratory failure. Acute episodes of severe pain are common in sickle cell disease. Nine of the 19 patients with sickle cell disease who had pain on admission and who then died had been given excessive doses of opioids. Five of these patients died of complications caused by overdose.
In response, the report recommended that all patients admitted to hospital with sickle cell disease should be regularly assessed for acute pain, sedation, and respiratory rate. The frequency of these observations should reflect the degree of pain and dose of opioids given to allow opioid overdose to be recognised.
A Sickle Crisis? is available at www.ncepod.org.uk