Advice for a novice please?

[Jules]

Still a relative newcomer to this, so am hoping you guys can offer me some advice.

Two weeks ago my nephrologist reduced my dosage of cyclophoshamide as he was concerned my lymphocyte count was falling too low. A week later (having taken the reduced dose for a week) it had fallen further. As a result I was taken off it for last week. Had appointment today and he has reintroduced an even lower dose, and will review on results of bloods that were also taken today.

My query is though, during that week off, I had a huge increase in crusting, most of it blood stained and had a nosebleed today. The consultant said the cyclophoshamide would still be kicking around in my system and that it should have it under control - but the sinus stuff was one of my main presenting symptoms.

Is blood stained crusting and nosebleed par for the course with WEGS that will just come and go intermitently, or should I be concerned? Is it possible for these symptoms to present so quickly after a week off the ctx? How do I know when to be concerned? Aaargh. . .I'm confused!!!!:unsure:

It has also been suggested that I may need to have injections to give my bone marrow a bit of a kick up the backside - anyone got any experience of this?

Such basic questions I know to all you gurus out there! Just so glad I found this site to be able to ask the basics. Jules

[coffeelover]

Blood stained crusting and nosebleeds ARE par for my course. Sinus rinses and humdifiers help considerably.
Besides a nephrologist (whatever that is?) ARe you seeing a Wegeners specialist? Or at least a Rheumatologist to start?
I am no expert, but if your doctor is not versed In WEGs then you may need to find another doctor or use both of them. I, Personally keep 4 doctors in my back pocket, one being a Wegeners specialist/ENT and one being a rheumatoligist with WEG experience.
Best of luck shopping for what you need.
LIsa (coffeelover)

[Jules]

The nephrologist is the consultant dealing with the kidney complications (I had to look it up when I first came across it. . .oh how WEGS broadens our knowledge in so many ways!!!). However, as I have Goodpastures as well, the hospital I was referred to in London for the renal stuff, want to deal with the whole package as its "so interesting and unusual" to them. My Rheumatologist is based at a different hospital, where the diagnosis was originally made. However, the two seem to have different ideas . . . are your doctors at the same hospital and singing from the same song sheet, or do they have conflicts of opinion?

[NicShaf]

Hi Jules,
I am also new to this, so I can only offer a little of what I have experienced. I am also on cyclophosphamide, started as of the first of the year, and since I started, my crusting and bleeding has slowly subsided. It is still there, but every week it is a little less. It did take about 3-4 weeks to start making a difference though. From what I have seen, in myself and from others talking about it, crusting and a little blood is par for the course with Wegs, especially if you Wegs is active and you are just starting your treatment. But if it were me, I would be a little concerned that it came back so quickly and so strong. What did your doctor say about it?

[Jules]

Thanks Nicole. I started my ctx on 13th Jan and, like you, had noticed a marked improvement almost to the point of it not being a problem, which is why I was surprised at its quick return during last week. The Doc just seemed to think that the ctx in my system would have it under control!?

[marta]

Hey Jules,

I had a similar problem after my last flare in Oct/Nov. They were taking me off the cytoxin because of my liver function tests being off the charts. I could feel within a week of being off the drugs the disease start to show it's ugly signs inside my head (I too have some serious sinus issues) - then I talked them into putting me back on, but they only kept me on for just over a week.... within that week (in about a week actually) I started to feel things get better, then they took me off again. At the end I ended up putting myself back on the drugs two days before my doctor's appoitment because, again, I felt things take a slide downhill. I appreciated them taking care that my liver doesn't blow up, but my gut feeling was that my liver acts up when my disease is active, and they were experimenting with stuff that could cause me permanent damage. In the end, it turned out I was right. We actually upped my cytoxin in January and my liver function tests were within normal range two blood tests ago. My doc actually told me "you were right about your liver hypothesis". My understanding is that it takes a week for the cytox to take effect after you start taking it and about a week for the effects of it to stop after you stop taking it - that's how it felt inside my body too. Sangye said it's a month, and it's quite possible that the full effect of the drug takes that long, but I definitely felt a difference at the one week marker, in either direction.

When I first got diagnosed, I felt really out of control. Uncertainty was killing me. Before I got sick, I was very self aware, and now I'm going back to that sence of trusting my gut feeling and what my body is telling me. If you're feeling like there's something wrong with the decision, go back and question it. Do a little research and bring them stuff. Especially if your docs are on different pages. I use to be a very passive patient before Wegener's, you should see me now. I question everything. I want to know the reason for every decision, and how it will affect me. I need to be alive for a long time, so I'm all over it. Hope you get this sorted out soon.

[drz]

The nephrologist is the consultant dealing with the kidney complications (I had to look it up when I first came across it. . .oh how WEGS broadens our knowledge in so many ways!!!). However, as I have Goodpastures as well, the hospital I was referred to in London for the renal stuff, want to deal with the whole package as its "so interesting and unusual" to them. My Rheumatologist is based at a different hospital, where the diagnosis was originally made. However, the two seem to have different ideas . . . are your doctors at the same hospital and singing from the same song sheet, or do they have conflicts of opinion?

Since you are an especially rare case having both Goodpastures and Wegener's, our experience may not be pertinent to your situation. In my case the symptoms of Wegener's ebb and flow, even when I was on Cytoxan. I did have to give up on Cytoxan cause my WBC and HGB kept dropping from it even when at the lowest dosage. At the lowest dosage it stopped helping control the Wegener's so they switched to azathioprin (Imuran) which seems to be working better for me. I was also set up to get epogen shots to help reduced the anemia but once it was set up I never hit single digits again with HGB so i never got one.

What do you lab results indicate for inflammation markers? Symptoms are most valid indicators of how you are doing but lab work can be very helpful in confirming what is going on in your body?

[Sangye]

Jules, you really need someone who specializes in Wegs. I have a hard time believing the increase in crusting was just a coincidence. I was not able to tolerate ctx-- it caused bone marrow toxicity in me, even at a sub-therapeutic dose. So I wasn't able to take the recommended dose of 150mg and maintain bone marrow. That meant I became quite sick and the Wegs was not at all controlled.

That may be what is happening to you.

[Jules]

Thanks Sangye, being tried on just 50mg of ctx this week to see if I can tolerate that and if things improve. . .decision on bone marrow jab being made on Thursday..... fingers crossed!

[Daggar]

Hey Jules, sorry to hear about the wbc drop.... they were thinking of dropping Holly's dose of Cyclophosphamide due to the rise in liver enzymes and drop in lymphcytes but I don't think they would of cut it out completely without changing to another med?? Luckily things came back to nearly normal without changing her schedule.

Your case with the WG/GP combo makes the "kidneys" the priority and if your Nephrologist is experienced with either of these autoimmune disorders I'd be more apt to listen to them but it really depends on their knowledge of the diseases.

Hope the wbc recovers!!