Scientists closer to reducing loss of life and risk of amputation caused by meningococcal disease

Scientists have discovered a trigger to a deadly chain of events that ends in amputations and death in patients with severe meningococcal disease. Their findings could now be used to help develop treatments to prevent such terrible outcomes for these patients.

Infection from meningococcal bacteria is the leading cause of meningitis and septicaemia in the UK and Ireland. Around one in ten people affected will die and a third of survivors will be left with after-effects, some as serious as amputations, brain damage, hearing loss and blindness. One deadly complication of meningococcal infection is purpura fulminans where blood clots develop in the bloodstream. These block small blood vessels and cause tissue to die. This is why patients with meningococcal septicaemia lose fingers, toes and entire limbs. Clotting can also damage vital organs.

In a study funded in part by charity Meningitis Research Foundation (MRF) and published in PLOS Pathogens, scientists from Institut Necker-Enfants-Malades (INSERM/CNRS unit at the University Paris Descartes), discovered that when the meningococcal bacteria bind to the lining of our blood vessels, a molecule called ADAM10 becomes active and prevents our body’s natural ability to stop uncontrollable blood clotting.

Xavier Nassif, from Institut Necker-Enfants-Malades, Paris, said, “We knew that purpura fulminans tends to be extreme in patients with meningococcal disease, which suggested that meningococcal bacteria intensify the clotting process in a unique way. Our research has identified a key step in the process which enables purpura fulminans to develop in patients with meningococcal disease. This new understanding of the role that ADAM10 has to play could help the development of new therapeutics.”

Linda Glennie, Director of Research at MRF said, “Meningococcal meningitis and septicaemia are deadly diseases that strike without warning. Rapid diagnosis and treatment in hospital with antibiotics provides the best chance of survival and reduces the chance of lifelong disability for patients. However, even with prompt diagnosis and treatment, some patients will develop uncontrolled blood clotting and lose limbs or even their lives as a consequence. This research is really exciting as it’s the first time that the link has been identified between meningococcal adhesion to cells lining the blood vessels, and widespread, disastrous blood-clotting. It brings us a step closer to finding a treatment that could stop purpura fulminans in its tracks and enable patients to survive meningococcal disease unscathed.”

Now, with MRF funding, these scientists are carrying out further studies investigating the ADAM10 enzyme to find out what can be done to prevent purpura fulminans in patients with meningococcal disease.

Brogan-Lei Partridge from Birmingham was seven years old when she was struck down with meningococcal meningitis and septicaemia in 2016. Her mum Aimee Partridge explains, “On the 27th June, Brogan-Lei did not seem herself. She’d had a sleepless night and she was vomiting. We took her to A&E but they discharged her saying it was a sickness bug. Just three hours later she developed a rash so we rushed her back to hospital. Doctors then confirmed our worst fears. We’re so lucky that she was saved but we were devastated when Brogan-Lei had to have her left foot amputated due to the septicaemia. The damage was done within just a few hours, but only time will tell how much the disease will affect her life. We’ve been told she will need to have her right foot amputated too and we are just awaiting a theatre slot. She is such a brave girl about it all. We wouldn’t wish this on anyone. It’s encouraging to hear that this new study could help find a way to stop this happening to other people in future.”