Vasculitis: On the Road to Recovery

AT AGE 67, Andrew Green is more physically fit and active than many people his age. The retired entrepreneur practices tai chi five days a week and embarks on daily bike rides — that is, when he’s not globe-trotting with his wife, Judy. The couple’s travels have taken them to such exotic locales as Japan, Mexico and, most recently, Barcelona. A glance at his day planner would never even hint that Green lives with a rare and debilitating chronic illness that just a few years ago nearly killed him.

Green was diagnosed with central nervous system vasculitis (CNSV) in 2001 at age 58. An avid runner and cross-country skier, Green first sensed something was amiss when he developed sudden-onset fatigue and body aches that shortened his runs and eventually made it difficult for him to simply walk around the block. His health continued to deteriorate until one afternoon he became disoriented during a conversation with his wife and put his head down on the kitchen counter to try to sleep. Alarmed, his wife rushed him to the emergency room at the Cleveland Clinic, where his symptoms prompted doctors to run dozens of invasive tests, but ultimately offered few clues as to what was actually wrong with him.

“I was fortunate that there was a doctor from the rheumatology department working that evening,” recalls Green. “After I was admitted to the hospital, I underwent eight spinal taps, a bone marrow biopsy, brain biopsy, a biopsy on a nerve in my foot, echocardiograms of blood vessels and several MRIs of my head. Unfortunately, there was no conclusive answer about my diagnosis.”

It was the beginning of a long and frustrating journey for Green, who endured misdiagnoses for nearly six months, even going to the Mayo Clinic at one point for a second opinion. In the end, a team of specialists finally made the diagnosis of CNSV. During the diagnosis process, it was discovered that Green had several additional autoimmune diseases too, making his treatment plan somewhat complex.Fortunately, his proximity to the renowned Cleveland Clinic afforded him the best possible care. After undergoing various treatment options with limited improvement, Green’s doctors prescribed off-label use of intravenous immune globulin (IVIG) to boost his immune system; within a week, he finally began to feel better. Green started with twice-monthly infusions and now infuses every six weeks. He says the treatment has given him back a quality of life he thought was gone forever.

“My life seems normal now,” he says. “I’m active; I exercise; I enjoy woodworking. The only thing I’ve had to give up is cross-country skiing — my battery doesn’t stay charged long enough for that!”

Understanding Vasculitis

Vasculitis is an inflammation of the blood vessels that causes thickening, weakening, narrowing and scarring. There are many different types of vasculitis, and symptoms often mimic those of other diseases or disorders, which is why it can be so difficult to diagnose.

Vasculitis can be acute or chronic, and so severe that the tissues and organs supplied by the affected vessels don’t get enough blood. The shortage of blood can result in organ and tissue damage, and even death. The signs and symptoms of vasculitis vary, depending on which blood vessels and organ systems are affected. However, general signs and symptoms that most people with vasculitis experience include fever, fatigue, weight loss, muscle and joint pain, loss of appetite and nerve problems, such as numbness or weakness. The main goal of vasculitis treatment is to stop the inflammation. And, steroids and other medicines to stop inflammation are often prescribed.

Advocating Awareness

Never one to sit around and feel sorry for himself, Green keeps busy as an active member of the Education Awareness Council representing CNSV, where he dedicates his time to helping patients get a quicker, more accurate diagnosis for vasculitic diseases. He also finds it rewarding to encourage those who have already been diagnosed. “It’s not uncommon to hear you’ll be dead in six months once you get this diagnosis,” he explains. “It is so rare and you feel so alone — even doctors know very little about it. It’s important to provide a forum and a platform for patients to connect with one another for advice and encouragement. It helps me to know I am a shoulder to lean on for someone who is just learning about vasculitis.”

Green says if he’s learned anything during this journey, it’s that getting one doctor on your team to act as your “quarterback” can significantly help weed through the complex procedure and treatment options. He also advises patients to educate themselves about their disease state. “When it comes to rare diseases, knowledge really is power,” he says.