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Wednesday, 7 November, 2001, 02:18 GMT
New hope for sickle cell patients
Cells in the condition take on a distinctive shape
Cells in the condition take on a distinctive shape
Scientists in America have found a way to reduce the severe pain suffered by sickle cell anaemia patients.

Using a new drug called Purified Poloxamer 188 (PP188) the researchers were able to reduce the duration of often-excruciatingly painful "crises".

The study, carried out in 20 hospitals across the US, found children suffering from the condition responded most effectively to the drug.

There was also evidence to suggest that the new treatment might give good results when combined with hydroxyurea - a standard treatment which reduces the number of poorly-formed red blood cells in the circulatory system.

Inherited condition

A total of 255 adults and young children took part in the study between March 1998 and October 1999.

Half of them were given PP188 and half were given an inactive salt solution.

The results showed patients taking PP188 were 50% more likely to be free of pain within a week than those taking the placebo.

But more marked improvements were seen in two types of patient.

Nearly 60% of children taking the new drug were free of pain within a week, compared to 27.8% taking only the salt solution.

Just under half of patients receiving hydroxyurea at the same time as PP188 were also better within a week, compared to just 14.3% just getting the conventional medication.

Dr Eugene P. Orringer of the University of North Carolina, who led the study, said: "While the effect wasn't profound, it clearly shortened crises.

"If we could reduce the frequency or severity of crises during childhood, we might be able to protect these patients from much of the organ damage that typically appears later in adult life.

"That possibility is particularly exciting."

Extremely painful

Sickle cell anaemia is an inherited blood disorder, which largely affects Afro-Caribbean and Asian patients.

It causes unpredictable and extremely painful episodes, known as 'crises'.

The condition is named after the distinctive "sickle" shape formed by red blood cells.

The cells' shape is affected because a protein within it - haemoglobin which carries oxygen from the lungs to the rest of the body - is different to normal.

This means sickled cells stick together and cannot get through small blood vessels as easily as healthy cells.

This can stop oxygen getting through, and cause damage to organs, or in some cases, strokes.

But PP188 appears to allow blood cells to slip around each other and more easily through blood vessels.

It works by reducing the thickness of the blood and its components, allowing even rigid, crescent-shaped sickle cells to move through narrow capillaries.

Dr Mark Layton, consultant haematologist at the Hammersmith hospital in London, said: "The findings of this study are very encouraging and suggest that there may be an important role for this new agent in the treatment of sickle cell crises, which is the most common cause of admission to hospital among those with the disease.

"One of the most intriguing conclusions is that there seems to be a greater benefit in children and in those patients who are already taking hydroxyurea.

"This suggests that there is a fundamental difference in the nature of painful crises in children and adults, and hints at the possibility that if treatment of this kind were started early enough in life it might actually have some beneficial effect later through preventing the repetitive cycle of tissue damage that follows sickle cell crises."

The results of the study appear in the Journal of the American Medical Association.

See also:

17 Jun 01 | Health
Sickle cell transplant hope
23 Nov 00 | Health
GP tackles sickle cell anaemia
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