Irish doctors have made a major breakthrough in the treatment of the deadly disease, meningococcal septicaemia. Dr Owen Smith, a consultant paediatric haemotologist, and his team at the National Children's Hospital, Harcourt Street, and St James's Hospital, Dublin, have discovered that Protein C, a natural anticoagulant, can save patients who, in the past, would almost certainly have died.
Dr Smith and his team used Protein C on 12 pre-morbid patients aged three months to 27 years. Each was on the brink of death with septic shock, widespread skin necrosis (a blackening of the skin due to blood clotting) and devastating organ failure, yet all 12 survived.
"We believe that the addition of protein C therapy in the most malignant disease in paediatrics is a truly significant therapeutic advance," Dr Smith told The Irish Times.
Meningococcal septicaemia is caused by the meningococcal bacterium, the bacterium which causes meningitis. Meningitis affects the lining of the brain and more than 90 per cent of cases are cured with antibiotics when treated early.
Meningococcal septicaemia, however, is so difficult to treat that the death rate from the disease can reach 80 per cent, even with aggressive therapy. Hundreds of Irish people are infected annually with meningococcal infection and up to 30 have the severe form - meningococcal septicaemia. In the end stages of the disease, patients develop a distinctive red rash and bruising causing by severe blood clotting beneath the skin. All other major organs become inflamed and go into failure.
Patients who survive may suffer significant long-term damage such as limb amputations, skin grafting and kidney failure requiring life-long dialysis. The understanding of how the meningococcal bug actually causes so much damage has, until recently, been very limited.
"We now know that the endotoxin, the coat of the bacterium - the meningococcal bug - causes lethal abnormalities in many different organs of the body, including the kidney, liver, lung and cardiovascular system," Dr Smith said. The central mechanism for these potentially fatal complications is the reaction of the immune system, which goes into overdrive in its attempt to eliminate the endotoxin.
Since his return from Britain, Dr Smith, along with his research fellow, Dr Barry White, has been offering Protein C to patients in extremis. Protein C is not a miracle drug used in isolation and must be given in conjunction with other anticoagulants and with life-support equipment such as ventilation and blood filtering.
Nearly 20 lives have been saved since January 1996.