When Martin McGuinness announced in January that he was standing down from politics because of his health, few people were aware of the reason. He looked drawn and sounded slightly breathless when speaking but otherwise it was not obviously apparent that he was in fact a dying man, and dying rapidly. He said at the time that his condition was serious but gave few other details.
We now know the reason why, and in fact his fate may have been sealed from the day he was born. Without wishing to draw on too many political analogies, he was a walking health timebomb and his own heart would be the casualty. He was suffering from the rare condition amyloidosis, which in his case was probably linked to his mother who had been born in Donegal, as up to one per cent of people from that area are said to carry the gene for the condition.
Amyloidosis is a group of conditions caused by deposits of abnormal protein, called amyloid, in body organs and tissue. In healthy people, proteins originate as strips of amino acids that fold into 3D structures but in amyloid this intricate folding goes wrong, leading to clumps of thick protein forming. These slowly build up in the body, typically causing organ failure.
In the case of McGuinness this triggered his heart to slowly enlarge and fail as it struggled to pump blood effectively around his body. This in turn caused breathlessness, fatigue, fluid retention, arrhythmia and ultimately death.
About 600 cases of amyloidosis are diagnosed each year in Britain, usually in older people, with the most common type being AL amyloidosis. Kidney failure is perhaps the most common presentation I see in my surgery but any organ can be affected and it can be a tricky condition to diagnose, especially in its early stages. However, AL amyloidosis cannot be inherited so it is likely that McGuinness had inherited another type of the disease. (A build-up of amyloid protein is also implicated in the development of Alzheimer’s disease, with the pharmaceutical industry currently spending billions in trying to develop the first effective treatment to prevent this.)
The fact remains, however, that there is currently no treatment that can remove amyloid deposits from the body, so therapies are directed at attempting to prevent the production of abnormal protein chains and slow any organ damage that is occurring. Kidney transplants can be effective if it is that organ which is primarily affected, and chemotherapy can also be used to protect bone marrow function and inhibit amyloid production. Survival rates depend on the severity of the amyloid damage, the age of the patient and their general health, and how well they respond to any treatments that are given.
McGuinness will have known that there was no cure for him, and that when he went into Derry’s Altnagelvin hospital he was unlikely to come back out. Amyloidosis was simply a bullet he was never going to be able to dodge.