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. 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. As of July 09 Ben must also
watch his potassium intake.)
My hero list is short. Two strong
woman who love the Lord made tremendous impressions on me. One
lived with devastating rheumatoid arthritis. The other was an incredible
caregiver to her ailing husband.
Ben now on my hero list because of his courage and humor.
Treatment:
Doctors are attempting to turn off the production of the anca antibody with an
immune suppressant drug. Major
doses of steroids are used to reduce inflammation.
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.
Prognosis:
Based
on the improvement of Ben's blood levels it appears that dialysis will be a
fading memory (at least for now). That would allow life to
return to 'normal.' Normal now includes avoiding colds and flu, careful monitoring
of blood pressure, temperature and weight, daily
medication and a treatment described above.
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.
"Feed your faith and your
doubts will die of starvation."
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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
