Ben was diagnosed with this disease at age 12.
These pages were created to share difficult information
without constantly reliving it.
Wegener's
Granulomatosis
Vasculitis Foundation Cleveland Clinic Bruce's webpage
THE MOST IMPORTANT THING FOR YOU TO KNOW....Ben's immune system has to be suppressed to shut off the attack on his kidneys and prevent other small blood vessel involvement. Please help us protect him from common colds and flu as they can turn into something much more serious for him. Be aware: we are asking visitors to wear a mask around Ben if they have been exposed to an illness or have recently been ill.
Ben's Diagnosis (July 29, 2003): Ben has c. anca vasculitis, specifically Wegener's Granulomatosis. It is a rare, serious auto immune disease that attacks the small blood vessels in the kidneys, lungs, sinuses, eyes, ears and joints. Preliminary diagnosis of elevated anca antibody blood levels was 16 hours after arrival at Children's. Nine days later the kidney biopsy confirmed Wegener's.
To back up for a moment, Ben had been throwing up for five days. I took him to our local pediatrician on Thursday, July 24. It looked like the flu that was going around. I was told to bring him back Monday or Tuesday if he was still having a problem. Blood was drawn Monday afternoon, July 28. At 10:30 p.m. our local Emergency Room doctor called with the lab results of Ben's BUN and creatinine numbers which at the time meant nothing to me. What I did understand were the words 'kidney failure' and 'do not wait until morning' to go to the hospital.
The biopsy showed some permanent scarring. The remaining kidney tissue was severely inflamed. The kidney filters were 'jam packed full of toxins' so the kidneys were not able to filter blood. In order to keep his blood filtered, Ben needed an artificial kidney to do the job for 27 days. Click to see details of dialysis. As for return of kidney function, we wait and see and then, I suspect, wait and see some more. Ben appears to be stable off of dialysis. It is important for him to continue being very careful with his diet.
Ben is on the 'renal diet.' The diet is very complicated: the hardest part for me is the restrictions on the staples...potatoes, beans, whole wheat, tomatoes and cheese. We look at food in a new way; ice, jello and sherbet count as fluids. He is very dedicated to staying within his dietary limits and is honing his cooking skills to do so. (As of Dec. 03, Ben's remaining kidney function allows him to eat or drink most foods as long as he restricts sodium.)
My hero list only has a few people on it; Ben is one of them. He is full of courage and humor.
Phase 1: during the initial hospital stay: Three days of IV steroids and one IV treatment of Cytoxan (a
chemotherapy drug)
Phase 2: Daily oral steroids plus six months of overnight stays at Children's for the Cytoxan
IV. Meanwhile Ben takes a pile of pills to fight some of the side effects
of the aggressive treatment and loss of kidney function. Blood
levels are monitored weekly. There is always a specialist on call at
Children's to discuss our
concerns. (After
findings in Ben's lungs in Sept. 03, he was placed on oral daily Cytoxan
pills for two months. He was unable to tolerate that and switched
back to IV treatments which concluded in Jan. 04.)
Phase 3: If things continue to improve next spring (2004), steroids will be tapered down and the immune suppressant drug might change to a daily oral dose of Methotrexate. (Steroids started at 100mg per day in July. As of Dec. 03 Ben takes 5mg per day.)
Potential side effects of medications: vast and scary. Since the alternative of not seeking aggressive treatment is unacceptable, we have no choice but to settle for life with side effects.
Ben's cheeks before and
after prednisone. (taken July 03 & Sept 03)
Picture on right also shows fluid retention from low kidney
function.
My understanding is that Ben will be heavily medicated and frequently monitored over about the next two years (with or without dialysis). Assuming no complications, he would be considered in remission at that point. The anca blood level would continue to be monitored at prescribed intervals watching for indication of a relapse. A relapse could occur in any of the small blood vessel groups (kidneys, lungs, sinus, ears, eyes, joints). Dialysis will resume if his creatinine reaches 3.5 (five times his normal level).
Questions:
Do we know what
caused Wegener's? Is it related to the 39 days of vomiting last
fall? No and no. Yes and yes. There is no known cause
for Wegener's but there are higher instances of it in the Northwest,
especially in Montana indicating environmental causes. This disease
is not currently reported to the Center for Disease Control. Last
fall's vomiting episode may have been unrelated, but there is no doubt it
was beginning of the spiral that weakened Ben's immune system...possibly
enough to let the overactive anca antibody take hold.
What was it like at Children's? Todd and I both set personal records and made it through 48 hours on one hour of sleep. The staff was amazing. They started out by explaining that they needed to determine if this was a pre-renal, renal and post renal problem and then walked us through every step of ruling things out. They were understanding of our questions and gave us everything we wanted. There are social workers and dieticians specifically for nephrology. For our long stay we were very fortunate to be in a private room where there was a fold out couch for a parent to sleep. Other rooms are very cramped with four patient beds plus four parent sleeping chairs. (Children's opened their new wing Feb. 04 and I understand most rooms are private now.) The nurses were gentle, especially kind, and unbelievably considerate of us in our fragile mental state. We didn't get to choose who was the nephrologist on call the night we checked Ben in. If we could have, we would have chosen the one assigned to Ben. He is the best: quick thinking, clear speaking, and most of all knowledgeable and kind. He managed to 'sing and dance' and make things happen quickly. He has our highest praise and thanks.
For quite a while we couldn't figure out why Rheumatology was coming in and manipulating Ben's joints since they were not affected. It took us longer than it should have to realize Rheumatology manages treatment for auto-immune diseases. Again we were blessed. We can't say enough good things about Ben's primary Rheumatologist. News doesn't seem quiet as bad when it is delivered in an Australian accent!
Children's is a teaching hospital meaning that the attending physicians travel with students. It is like each doctor has at least four body guards. The first several days each person wanted to hear the story and see the symptoms for themselves. Since Ben needed surgery and Wegener's has the potential to involve the kidneys, sinus, lungs, ears, eyes, and joints, Ben saw the Renal team, ENT team, Audiology team, Ophthalmology team, Rheumatology team, and surgery team. We told the same story over.....and over.....and over.
On our first stay Ben was the first patient for a third year medical student who gave our family lots of special attention. When Ben started feeling better he enjoyed the Teen Zone with computers, video games, movies and fooz ball. In his room there was a VCR and cable TV. A Great Dane came to visit, clowns came in the room, two Mariners came and signed balls, the Blue Angel pilots came (we missed them), there was a bird nest with a baby in it outside of his window.....
The nurses at the Children's dialysis unit are excellent. They are obviously empathetic, cautious dealing with sore spots, friendly and informative. They know their stuff and patiently explained it to us. We are truly blessed to have these people involved in Ben's care.
What is the difference between a resident and a fellow? A person graduates from high school, then college, then medical school. After that they have a residency where they focus on a particular area of medicine for a few years. They then enter a fellowship program for another few years. I am not sure if the required years differ depending on the specialty.
Will Ben be able to go hunting this fall ('03)? One way or another, Ben will go 'hunting' even if it isn't the usual style of hunting and camping.
"Feed your faith and your doubts will die of starvation."
Disclaimer:
This web site is provided for informational purposes only.
Reliance upon the information contained on or accessed through this web
site is entirely at your own risk.
Updated Feb. 2004
Questions I didn't answer or
something not clear? Email me