2.9.08

For those who don't like to read: here's the short of it...

• John's blood in the urine and new sinus type symptoms shows that he is doing worse, although the (way too high) doses of steroids have his pain and swelling down.
• The doc in Cleveland and here do not agree on diagnosis. Either way, they agree on new tests and treatment.
• Tests = Cat scan of upper body (came back clean mostly) and kidney biopsy (to be scheduled for early next week). Both approved by insurance.
• Treatment = continued steroids (eventually in lesser doses) and a chemotherapy-like med that can wipe out the vasculitis, ...but also wipes out his entire immune system and can cause bladder cancer or Leukemia over the next two years. He starts that med on Wed regardless of what the biopsy shows, for many different reasons.

For those who want the nitty-gritty, read on...

We are back from Cleveland. People ask me if the trip was worthwhile, ...hum. I believe yes, but we are still in such a limbo place that it is discouraging. Here's the details...

Left after school on Tuesday (after voting of course...rock the vote) and traveled with three fevered kids and John's nephew, Bobby, who was a huge help on the trip, and of course, one Max the dog. Fun times. Arrived at John's sisters that evening and mapped the trip for the next day.

Wednesday morning we went to Cleveland Clinic's Vasculitis Center and saw a specialist there. We were treated well and the facility is wonderful. The doctor took her time and answered questions with us. She ordered one blood test, one cat scan, and recommedned a kidney biopsy. the insurance wouldn't approve the cat scan (pre-approval required) w/o further info so Cleveland said it could possibly take days and since the test is relatively simple and can be done anywhere (as can the biopsy) we should go home. The doc in Cleveland agreed to work in consultation with our local doc here so she will still be in the loop. We waited until Thursday morning in hopes that the cat scan would be approved sooner rather than later, but it was not. John had one more consulation with her via phone Thursday morning and also a call to his doc here to schedule an appt for Friday. We headed home. We were back in town Thursday night and had a doc appt the next day. They will call us on Monday to tell us when the biopsy is and John starts meds (more on that later) when we go in for the next appt on Wed.

So, that's all the easy part to report. Time lines are easy. I've been putting off writing this because it's the medical info that is hardest to speak about. Partly b/c I'm worried, partly because I'm so confused about it all. So, here's that part...

Both docs agree that John has vasculitis, we need a cat scan of the upper body and a biopsy of the kidneys, and that we need to change the course of treatment. That's pretty much where the agreement ends.

The doc in Elmira feels it is Henoch Schlein Purpura (HS), (as I reported previously), just that it's "an exceptionally bad, stubborn case". If you have to have vasculitis, HS is the one ('best of the worst') you'd want. He makes his case by saying that John has most of the symptoms of HS, and the blood tests for the other types (HS, unfortunatley, has no specific diagnostic test but most of the others do) have come back negative. He admits that to get HS in adulthood w/o having had it in childhood is rare, but "not unheard of if you've had kidney disease since childhood" (which John has). The treatment for HS is steroids (often leading to diabetes/insulin) and pain meds to manage the pain. That is what we've been doing for four weeks. John is on 80 mg of steroids, which is exceedingly high, but lower doses do not produce any results. On that point, the doc is stumped and agrees that we need to change treatment plans. He cannot stay on such a high dose indefinitely.

The doc in Cleveland feels that John does not have HS. She stated that it is "exceptionally rare" for an adult to have HS w/o having it in childhood so for her to diagnose it as HS John would have to have all of the other symptoms, which he does not. She said she is the specialist that all of the 'exceptionally rare' cases get sent to so she would know what to look for. She also had a son with HS when he was 3 years old so she knows what to look for intimately, not just professionally. She goes on to argue that HS responds wonderfully to steroid treatment and for John to be at this high a does, for so long, w/o remission, confirms that it is not HS. She reported that if this is NOT HS and we keep him on a steroid-only treatment, he will stay prepetually ill for the rest of his life. She believes that it is Wegener's Granulomatosis (WG) (ironically, one of the two worst kinds of vasculitis. Leave it to John to go from the best to the worst in one jump. He has been an extremist as long as I've known him. ...haha. insert nervous laugh. work with me. i'm trying to keep my head above panicy-water here).

John has been tested for WG three times, twice in town and once in Cleveland. All three tests were negative (says the local doc). And WG has a track record (1/3 of all patients) of having false negatives disgnostically (says the Cleveland doc). A biopsy of the kidney MAY disgnosis the WG, however, both docs stated that the fact that a) John has had kidney disease since childhood and b) he's been on steroids (prednisone) for a month, may alter the validity of the biopsy. It may not, but it may.

We've all agreed that since it may proove helpful to accurate diagnosis, and since correct treatment is directly tied to accurate diagnosis, we will give it a shot and hope for the best. The local kidney specalist (whom we've not met yet) will call on Mon to schedule the biopsy for early next week. Since it is an outpatient procedure, the insurance does not need pre-approval. (phew). And the cat scan that was in limbo on Thurs was approved later that same day so we had that completed in Elmira on Friday. It was a cat scan of the lungs and sinuses. It was mostly clear (a little swelling and fluid in one sinus but we have three sick kids so that was to be expected). We got those results back at our appt yesterday.

Here is a reader-friendly link with specific info:
http://www.vasculitisfoundation.org/wegenersgranulomatosis

WG is not contagious or hereditary. It is rare. There is no known cause or cure. Only treatment and (hopefully) remission, with constant monitoring lifelong. It impacts the upper (sinuses and nose) and lower (lungs) respitorty systems, but is also systematic (which means it impacts the entore body as a whole) including ears, eyes, throat, skin and other organs. It can cause blindness, the bridge of the nose to explode and need cosmetic surgery to repair, the throat swelling shut and needing esophigical surgery to repair, tumors in the lungs (which is why the clear lung and sinus cat scan was good news), gangrene in extremities, and kidney failure to name a few. Not to mention the chronic pain and swelling and debilitation. If diagnosed early and treated correctly, it can be put into remission and prevent organ failure. Remission can last for 5-20 years. Relapses happen in 50% of all cases.

The reason the Cleveland doc is so convinced of WG is b/c of all of John's sinus and upper symptoms (ear aches, bloody noses, sores on the roof of his mouth, splitting of the tongue). I'm not sure if I've reported on all those to you all. Things have been progressing fast.

In short, it's terrible. John's immune system is haywire. It's identifying healthy tissues of his body (blood vessels, kidneys) as foreign and attacking them (leading to the ruptured vessels/spots and the protein and blood...blood is new...in the urine). We need to in essence kill off the old immune system and try to bring back a new.

So, ...treatment:
It is continued steroids (eventually in decreased doses) but the addition of a chemotheraphy-like drug. It is taken orally and is called Cyclophosphamide (common brand name: Cytoxan). He will start taking it Wed regardless of what the biopsy shows (which will take a week to get results back anyway) and he can anticipate not seing any improvement from the drugs for two weeks at least. Both docs agree that he should start taking it b/c no matter what type of vasculitis he has, this drug would knock all of them out and, even if is 'just' HS, the steroids weren't working anyway. So both docs are agreeing to this drug. We can't remember how long exactly the doc said he'll be on it, we think 4-6 weeks.

During that time, the drugs will be wiping out his immune system almost completely. This means that he will be very vulnerable to secondary illness and infection. He's on an anti-biotic now to help with that but it really doesn't help much. It's like a band-aid on a bullet hole. Honestly, he could die if he gets sick while on this med. For that reason, we are putting the house under a sort of semi-quarantine. No visitors for four weeks. We ask that people please honor this request. I will still go to work and the kids to school; we still need to live. But we are being as careful as we can. After 4-6 weeks his meds will be changed to a less severe drug for an extended period of time (I believe). Until remission. Then we monitor from there.

Here's the very scary part, this med is like making a deal with the devil. Yes, it will take care of the vasculitis (which, if we don't do, could lead to a very painful death, and there is really no other option, so we feel compelled to do it) but, the med (cytoxan) is cancer causing.
The doc s-t-r-u-g-g-l-e-d to come to this decision. He said that he does not use this drug ever unless he truly feels there is no other option. He stated that he has had a few dozen patients over the years who have been treated successfully with it and not gotten cancer as a result. He also confirmed that he has had "some" (whatever that means, I didn't ask the odds) patients that got cancer from it. He truly seemed distressed over prescribing this med. There are various types of cancer that this drug can cause but primarily it is cancer of the bladder and/or leukemia. After successful treatment and he is off the med, he will need to be monitored for two full years for the cancer. The whole thing is nerve-wracking.

As for 'how is John doing'...he is ok. The increased steroid seems to help for now, though there are of course side effects from that (the diabetes, the fragile skin, the always thirsty, antsy and unable to sleep, etc) but in return he has decreased swelling and pain so he feels it is worth it. He is back to walking like he is only 60ish years old and not 90ish, like last week. And he actually almost slept through one whole night without getting up with pain, which is unheard of lately. So all that is good. He is in good spirits for the most part, though he is worried; especially more so now that the docs disagree on diagnosis, he needs a biopsy, and this med is carcenogenic. He's really worried about catching the kids illness too. I don't blame him. Also, the pain meds are taking a toll mentally; confusion, sometimes stuttering, inverting words in a sentence. Also (as part of the WG probably) his hearing is somewhat impaired. But overall, he is ok.

Thanks everyone for your care and concern. Please keep the prayers coming. I think that doing this by blog will allow me, now that you are all caught up, to keep the updates shorter. I can hop on easily and throw out a few sentences instead of waiting a whole week or so and having to write an essay. Things really do change so frequently and fast. I think this blog will just work out better for me time-wise and for you all with information. Please feel free to pass the blog address on. I'll keep you all posted. Thank you for caring. It's easy as life goes on to forget about someone's pain, especailly when you don't see that person often. Thank you for not forgetting about John. For those of you who pray specifically, we're asking for prayers for an uncomplicated biopsy with clear results early next week, and for no cancer overall. Also and always, for decreased pain and increased healing for John, strength for me, and comfort for the kids.
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