Surviving CNS Vasculitis

By Jim and Becci Bornac and Cindy Webber

October 2007

His head throbbed with excruciating pain as if someone was continually bashing it with a brick-every hour of the day and night. Jim's pain was beyond belief. Although he had experienced headaches for many years, these headaches never let up. He was no longer able to sleep or to function well at his job of 13 years as a movie theater manager. Then began the long journey looking for a cause for the extreme headaches and bizarre symptoms he endured. Thus, he searched for a doctor that could help him.

The first doctor suggested something for depression or nerve pills. The pain continued. The second doctor thought the root of Jim's pain was allergies.This treatment again failed and soon Jim was back in his office with extreme disorientation, speech defects and an unnatural balance. This allergy doctor called for an MRI which proclaimed a slight abnormality in the brain. They titled it "unremarkable". Stroke-like symptoms continued along with the severe headaches. Jim found a neurologist in his own home town. Again, the doctor could find no problems. Then one day, Jim came home disoriented and could find no communicable speech at all. He was glassy-eyed and was slobbering down his tie.

Jim's wife Becci kept an accurate report of events to try and help the doctors figure out what was wrong with Jim. Becci continues to be Jim's advocate to this day. She called the neurologist and explained to him the symptoms and he said, "I want to see you both in my office tomorrow. Jim had told me he was fine."

After running every test imaginable, including: spinal taps, angiograms and blood tests, Jim's ailment was still unnamed. Eventually the neurologist convinced a neurological surgeon to do a biopsy. The surgeon was unconvinced to do a brain biopsy as it was very risky in the area that he would have to invade. He performed a muscle biopsy instead. It proved nothing in the way of a diagnosis. A decision was made by both doctors that a brain biopsy was in order for treatment at best! The day before Thanksgiving in 1993, Jim underwent the brain biopsy, a risky and invasive process.

The neuro-surgeon met with Becci in the waiting room and described his amazement to the particulars of the surgery. A diagnosis was finally found-- a rare form of Cerebral Vasculitis. It was called Granulomatous Angiitis of the Central Nervous System. (GACNS). Since it is so very rare, we refer to it more commonly as CNSV. Jim was immediately treated with prednisone and Imuran. And a barrage of other medications, including Dilantin and meds to thwart the side effects of the medications.

Things were looking better until the doctors tried to reduce the prednisone too drastically. Jim suffered the worst headaches in his life! While the doctors could not agree whose responsibility it was to handle the drastic call, Becci called 911 to get the help Jim needed.He suffered three brain hemorrhages and was hospitalized for many days. His neurologist called him a 'miracle'.

Jim has been off of prednisone for a year now and is now at half dose of Imuran. His next task of medicine reduction will be Methotrexate. He will never be off all medications with this disease but at least he may be able to eliminate the more dangerous ones.

Jim is a "miracle" in that he never gives up and has never lost his sense of humor. He never fails to help others with vasculitis and continues to research the disease.

For those newly diagnosed, he suggests that people keep positive and take life one day at a time- do not think too far ahead. This advice has served Jim well. He is now focusing his energies into supporting others with vasculitis find accurate information and support.

Profile of Andrew Green, Member of the VF Education and Awareness Council

By Kathy Savickas, February 2007

Andrew Green is a member of the Education Awareness Council and represents Central Nervous System vasculitis, which he was diagnosed with in 2001 at the age of 58. During his working years, Andrew was president and co-owner of a small plastic molding company in Cleveland, Ohio. He is now retired and lives in Shaker Heights, a suburb near Cleveland.
Andrew had been an avid runner and cross-country skier with 5 and 10k races under his belt and always spent a week every year cross-country skiing with friends. During the 1999 trip he lost his hearing in the left ear and was diagnosed with an autoimmune hearing loss.

During the summer of 1999, his runs became shorter and shorter and he started to have trouble completing even short training runs. He tried a long bike ride with a friend and the following day he was sore from head to toe. What troubled him most was that his workouts were not out of the ordinary but his reactions to them were.

As summer progressed, things became worse and he was eventually unable to walk around the block. Then walking around the house became difficult and he often started to fall asleep during the day. One afternoon in mid December, while conversing with his wife, he had trouble constructing an answer to a question and kept putting his head down on the kitchen counter to sleep. She became extremely concerned and took him to the emergency room.

There were a few visits to the emergency room at the Cleveland Clinic on one visit fortunately, Dr. Sudhakar Sridharan from the Rheumatology Department was working the night Andrew arrived at the Cleveland Clinic Emergency Room. He was admitted to the hospital and had several tests: 8 lumbar punctures (spinal taps), a bone marrow biopsy, brain biopsy, a biopsy on a nerve in his foot, echocardiograms of blood vessels, several MRI's of his head and other tests.

At first, the doctors thought he had Wegener's granulomatosis. However, the more tests they ran, the less he fit into the WG mold. Andrew asked that Dr. Sridharan "head up" his team of doctors that would help make the decisions on what tests and procedures would be performed. He feels lucky to have had Dr. Sridharan as his doctor and is indebted to him for all the help and guidance he provided. Andrew was in and out of the Cleveland Clinic from December to May and even went to the Mayo Clinic for a second opinion. In the end, it was Dr. Sridharan and Dr. Leonard H. Calabrese, also at the Cleveland Clinic, who made the diagnosis of Central Nervous System vasculitis. Finally, the doctors administered Gamaglobin IV and within a week Andrew finally began to feel better. He continues to receive Gamaglobin IV every six weeks.

Andrew is especially grateful to his wife, Judy for her love and support that helped him through this entire ordeal. Even when things were at their lowest, she remained by his side to help made some difficult decisions about his medical care while working with the doctors at the Cleveland Clinic.

Prior to his diagnosis of CNS vasculitis, Andrew was diagnosed with Hashimoto's disease, also an autoimmune condition where the thyroid becomes inflamed and often leads to an underactive thyroid. He uses a drug called Synthroid to try and control the Hashimoto's disease and has just recently started to inject the medication intramuscularly three times a week.

Andy does not run or cross-country ski any more. He says his battery just doesn't stay charged long enough these days. Now he uses Tai Chi, which is not only great exercise but also helps him work on his balance. He feels it is important to keep things flowing.

One of Andy's passions these days is his woodcarving. He has made beautiful bowls and artwork from what he describes as distressed wood - wood that has an interesting grain, is rotting or has insect holes. Andy and his wife also enjoy traveling. They visited Japan last spring and recently got back from San Miguel de Allende in Mexico. This June they will be visiting Barcelona.

Andy's advice to anyone with vasculitis is to first get one doctor to be the "quarterback". This doctor should coordinate all tests, medications and decisions with regards to your case. Andy asked Dr. Sridharan to do this for him. Secondly, make sure you always understand what tests are being run and what they hope to find out from the results. Become familiar with the drugs you are taking and their side effects just in case you have a reaction.

Judy, Andy's wife, suggests that you become as educated as possible and do not be afraid to ask questions or for clarification. Take responsibility for example, by learning the vocabulary and do not ever assume anything. And finally, don't be afraid to ask for help.

Although Andy has multiple autoimmune disorders, none of his doctors have suggested that they are in some way related. He also states that to his knowledge no autoimmunity runs in his family.

During his time with the Education Awareness Council, Andrew would like to work on helping patients get quicker, more accurate diagnosis when faced with a vasculitic disease. It saddens him to think of how many patients have lost their lives because of an improper diagnosis.