London

A lack of medical knowledge of sickle cell disease in London's hospitals has cost the lives of several patients.

Sickle cell is an inherited genetic disorder primarily affecting people of Asian, Afro-Caribbean or Mediterranean descent.

The condition distorts the normal round shape of red blood cells to sickle form.

It means the cells cannot move through the body easily and this can result in life-threatening symptoms like enlarged spleen, leg ulcers, pneumonia and, above all, excruciating pain.

Lack of knowledge

David Rees, a world-renowned consultant haematologist at King's College Hospital in South London says the disease isn't prioritised in medical school:

There’s a limited awareness in hospitals and at GP level – and that could mean the difference between life and death.

Medical training is very general – rarer conditions like sickle cell are not really taught at all.

So a lot more needs to be done at undergraduate level to increase knowledge of the disease amongst new doctors and nurses, to ensure there are no more preventable deaths.

These concerns are backed up by a government-funded report, which looked at the case notes for patients, who had died from sickle cell over a two-year period (1 January 2005-31 December 2006).

It concluded that that "many patients did not receive care based on best practice".

The report of the National Confidential Enquiry into Patient Outcome and Death (2008) was the first national survey of deaths caused by sickle cell disease and covered all hospitals and primary care trusts in the UK.

One of the causes of death was the mismanagement – the overuse or underuse - of opiods given to ease pain.

The report concluded that "many patients, for example, nine out of 19 patients, who had pain on admission and who then died had been given excessive doses of opiods".

It also said that the frequency of observations was inadequate, and for seven of the patients there was lack of knowledge regarding acute pain management.

In all of these cases, junior trainee medical staff were responsible for the prescribing of analgesia.

No apparent attempt was made to seek advice from consultants in haemoglobinopathies or acute pain management.

'Cinderella disease'

Dr Ade Olujohungbe knows about the 'hit and miss' nature of sickle cell treatment, as a consultant haematologist specialising in sickle, as well as having the condition himself:

"I’ve been in UK hospitals screaming in pain, but have been treated badly by medical staff and told I’m making up the pain.

"But, then when I’ve told them I’m a consultant, they’ve suddenly started treating me well.

"It’s a Cinderella disease. Patients can get a bad deal, depending on the medic you meet.

"Patients are often stigmatised as no hopers. It’s appalling.

"Two main things are going wrong. A) Patients are undertreated – like I’ve experienced or B) patients can be over-treated – for example, given an overdose of opiods.

"Basic knowledge could have prevented lives being lost - checking respiratory suppression, temperature, treating infection and treating pain."

Loss of life

Inside Out London has phoned round all of the capital's 31 A&E departments and found that seven of them have no protocol at all on how to treat sickle cell.

"King's College Hospital has a special protocol," explains Dr Rees.

"But, if some A&E departments have no protocol, who is to say serious mistakes won’t be made by junior doctors or nurses, who don’t know enough about the condition," says Dr Ade.

A mother from Romford knows only too well about how poor sickle cell care can result in a preventable death.

Cecilia Shoetan lost her 24-year-old daughter to sickle complications eight years ago.

"If she had received appropriate treatment and had died that would have been fine.

"But she had walked in and been neglected. It will live with me until I die."

Too much pain

Cecilia now runs the Barking, Dagenham and Havering Sickle Cell Support Group, one of 30 support groups around the country, in Romford, Essex.

She say, "Lorraine’s sudden death has had an awful impact on our family’s life.

"We try to move on in life but sometimes the pain is too much to bear. I pray that no family goes through what we have been through.

"Unfortunately, these mistakes keep occurring day after day."