Cleveland Clinic trip

I posted this on Facebook, but I have a few friends and loved ones who don't hang out on FB, so I'm copying it here.



A quick (ha!) summary of my trip to CC, mostly so I don't have to type it out or text it over and over :)

First appointment was with Dr. Carol Langford, who is one of the leading docs in the nation dealing with Wegs patients. I was very impressed - so knowledgeable, very personable, sat and answered all our questions (mine were a little more emotional; Michael's were much more on the clinical/technical side as you might imagine.) Her initial impression is that the Wegs is still active and I need to shift to a different medication but she had several tests she wanted to run as well as other docs I should see while at CCF before making the final decisions.

Had blood drawn - kidneys are functioning well; sed rate is still high; CRP is high (C reactive protein - levels rise in response to inflammation), glucose was somewhat high. Pretty much confirmed that the disease is still active and that I am now diabetic (steroid induced.)

CT chest showed some small nodular opacities consistent with Wegs as well as ground-glass opacities. Again, consistent with Wegs, but hard to determine when all this formed. A CT back in Feb of 2010 was clear, so obviously sometime in between, but no way to tell from the CT if it happened early on or more recently.

Also, the CT showed I have a severely atrophic spleen - didn't get a chance to ask Dr. Langford about that but I haven't read anything that connects Wegs and spleen atrophy. I'll have to check into it further when she calls me this week.  Unfortunately, since I'm already immuno-compromised, lack of a well-functioning spleen means I am even more prone to bacterial infection, etc.

Met with the pulmonologist on Thursday and he went over the CT chest results with me. He thinks there is some bronchial obstruction and wanted to do a bronchoscopy. He was actually able to find a spot to squeeze me in...but then we ended up not being able to do it because Michael had already flown home. I needed a "responsible adult" to be with me since it is done under conscious sedation. I even promised to sit in the waiting room all day, but it was a no go. I seriously offered to "rent" a responsible adult if one was available! I'm working on getting that done through the pulmonologist here in Walla Walla. (update is that we are waiting for my films to come from the radiology library at CCF and they are currently stuck in all that snow - UPS is showing "adverse weather condition exception" with no estimated date of delivery.

Friday was ENT day. Got a nasal and tracheal endoscopy. Wegs tends to attack the subglottic portion of the trachea and they were checking to see if that was a cause for my shortness of breath and voice changes. Fortunately, there was only some slight narrowing (about 20%, which is not a lot by Wegs standards) and some inflammation, but nothing severe. The ENT also suspects bronchial obstruction since nothing in the trachea was a problem.

Nose is a different story - everything started in the sinuses back in Oct of 2009 and it is pretty messy. Deviated septum, lots of scarring, crusting...all kinds of gross stuff. He recommended a couple of scrips, one for an nasal cream formulated right there at CCF for Wegs patients and then another ointment you mix with saline for nasal irrigation. Much more aggressive than the neti pot ;). But, he said that the majority of his patients who follow these two recommendations are able to avoid future nasal surgery and saddle nose deformity (a very common Wegs defect.)

Pending the results of the bronchoscopy, it looks like I will by stopping the Cytoxan (chemo) and starting on Rituximab. CCF was just part of a big study with this drug and the results are promising. Could possibly become the "gold standard" for Wegs treatment and I read yesterday on a Wegs support group site that they are expecting FDA approval for Rituximab in treating Wegs patients. Currently, no drug (not even the Cytoxan, which has been used for 25-30 years) is FDA approved for Wegs.

It is administered by infusion, two treatments two weeks apart. Takes about 6 hours at the infusion center. But then it is good for about a year, at which time I would hopefully be in medical remission. It is super expensive because it is a targeted drug (targets B cells instead of the broad spectrum kill like Cytoxan) so I may have to fight a bit with our insurance, but Dr. Langford said she didn't feel it would be a problem since I've already had almost 10 months of chemo without the results we want. She is very willing to write letters, etc. to get it approved.

Prednisone -ugh! Unfortunately, it is a very unique drug and I will have to stay on it at a high dose for awhile, at least through the induction period for the Rituximab, but Dr. Langford was also anxious to begin tapering ASAP. Yay!

So that's about it. Very, very worth the trip. Just to be seen by people that see Wegs patients all the time was huge. It affects about 3 in 100,000, so I imagine I'm the only Weggie hanging out in Walla Walla :)