As originally reported in CBC News, the problem wasn’t that Tom Salisbury had a cold. The problem was that it wouldn’t go away.
No rest and patience were enough and antibiotics couldn’t kick it. Soon his joints started to ache and he was coughing up blood. As time passed, his life that had always been healthy and active was becoming a memory. His family became concerned.
His family’s worries led him to the emergency room at St. Josephs Hospital in Hamilton. Doctors told him had he not come then, he was at certain risk for death. Luckily they identified the rare disease, a disorder affecting about 1 in 50,000 people each year. This does not make it ultra-rare, but it is ultra-challenging to deal with because it’s hard to diagnose, the onset is rapid, and the origin is unknown, as is a cure.
ANCA Vasculitis is an autoimmune disorder which makes the immune system turn from friend to foe, attacking the body it usually protects and causing the blood vessels to swell up in the affected part of the body. Symptoms can be displayed very differently from case to case depending on which organ is attacked. However, generally there may be a fever, inflammation of various parts of the body (including lungs, sinus, ears, and mouth), spots on skin, aches and pains, fatigue, weight loss, rashes, weakness, blood clots, and more. Tom’s lungs were hit by the disease, causing a cough, chest cold, and coughing up blood. The initial symptoms are compounded by the resultant propensity to develop other infections and organ failure. Treatment focuses on managing the frequent inflammation, as there is no cure.
The disease was once incredibly lethal and almost all patients fell victim to the terminal disease within a year of diagnosis. Today, medical advancements have significantly alleviated the risk, with 80% of patients surviving the first year.
Researchers have come a long way with ANCA vasculitis treatment and trials for experimental drugs create prospects for more. An experiment with collaboration between 95 centers have pioneered the next step for the community, running the most extensive trials ever for the disease that included 700 patients across the world. Salisbury was one of the participants.
They experimented on two treatments thought to be effective: plasma cell infusions and steroids. The half of the patients that received plasma came back with results ruling out the option, which actually seemed to make things worse. The remaining patients took varied levels of steroids and the physicians were able to give a lower dose with a more effective result in reducing rates of infections. The doctors clarify that saying one group took ‘steroids’ is not really specific enough, as the dosage, method of administration, and many other details have a great effect on results. What they discovered was not just that steroids are effective treatment, but found a better way to administer it to reduce death and produce less side effects.
Tom had the fortune to get access to this treatment and have the course of his disease turned around. He went into remission in 2015 and while it has come back since, this time they knew what to do to get the inflammation under control. ANCA vasculitis impacted the family enough that it inspired his daughter to return to school to study to be a pharmacy technician and she got hired by the same hospital that had treated her father.