Did It Work?

Did my Rituxan treatments work?

Here's what I told my kidney doctor on Sept. 29:

1. In general, I feel like I have a bit more energy.
2. The swelling in my legs, ankles and face is reduced.
3. I'm noticing less foam when I do my business.

All of these positive signs began about the second week of September -- so 10 weeks after treatment began.

My doctor then ordered blood work and a 24-hour urine test, and those results are back.

Positive news from blood work:
My serum albumin has increased. This is good! Albumin is important for tissue growth and healing. It was 3.3 before the treatments and is 3.7 now. (The target for adults with CKD is 4.0.)

Positive news from 24-hour urine:
The target range for protein in urine is 0.00 - 0.15 grams/24 hours.
My previous 24-hour urine, taken at Mayo Clinic last fall, found 3.93 grams/24 hours.
This week, I had 2.48 grams/24 hours.

Less protein spillage is good! Other than that, though, I can't draw many conclusions without talking to my doctor. I've stopped trying to figure out these tests results on my own (I was an English major for gosh sakes) -- and I don't have any psychic powers.

My rule of thumb is that if the UM Hospital # doesn't come up on my cell phone, then all is good and I should just keep rolling with things.

So I roll.   

Done!

1. 2. 3. 4. DONE.

Infusion #3 took 5.5 hours and overall went well. My IV backed up a few times, which didn't cause a problem, although it was unsettling to look at the line and see blood. I didn't have a reaction during the treatment, and I was awake enough toward the end that I watched a movie with my nephew. It was good to laugh! Post-infusion was much the same: a red, puffy face and a headache. This time I needed two days home afterward, as I was completely exhausted.

Infusion #4 took 5.5 hours and got off to a rocky start when the nurse couldn't get the IV line started. There were two failed and painful attempts before a different nurse intervened. Ouch. Once the line started there were no issues. I didn't have a headache this time (yeah!) but did get the red/flushed face.

Overall, I'm glad the treatments are done. I'm bruised and tired but completely relieved to have this step completed.

Two Down

So far, so good. I've had two Rituxan infusions and things are going well.

Infusion #1 took 7.5 hours. I had an allergic reaction about one hour into the treatment when my ears started itching like crazy. The nurses stopped the drip, gave me more benadryl, called my doc and waited about 30 minutes before restarting the treatment. Later that night I had a bad headache and my face was red; both lasted about 36 hours. Fatigue was a post-treatment issue. I stayed home the day after my treatment but then went back to work the following day. I was very tired but able to work, and that made me happy.

Infusion #2 took 6 hours and I didn't have a reaction during the treatment. Yes!  Treatment was done about 24 hours ago. I have a headache, my face is red and puffy, and I am exhausted. Benadryl is my friend. I feel much more tired this week than I was last week. But overall I'm doing well. The nurses at the UM Infusion Center are excellent and I get great care there.

A Chance

My insurance company has approved the treatment plan recommended by the Mayo Clinic and the University of Michigan. I will have infusions of Rituxan (Rituximab) on July 8, July 15, July 22 and July 29. Rituxan is typically used to treat lymphoma and leukemia, but recently it has shown some promise in treating other diseases. Getting this approved was no easy task, and I'm extremely grateful to my nephrology team at UM for working so hard on my behalf. Go Blue!

The first infusion on July 8 is supposed to take eight hours; the next three infusions are predicted to take five hours each. Treatments will be at the UM Infusion Center at East Medical Campus.

The above was easy to explain. More difficult is how I'm feeling about all of this. First was shock that treatment was actually approved. Then some excitement entered but was quickly tempered by how uneasy I'm feeling about pumping a drug into my body. Most of all, though, I have no clue if this will work. It's a chance -- but that's all.

Another study has just been published. Its authors call this "the largest case series to date using rituximab for fibrillary GN." There were 12 fibrillary GN patients treated with rituximab, and the treatment stopped progression of the disease in 4 cases. That may seem like a low success rate, but it has me feeling a bit like Lloyd Christmas in Dumb & Dumber ("So you're telling me there's a chance").

Thankfully, 4 out of 12 beats one in a million. Besides, if we were talking baseball here, those study results would have a .333 batting average and would be going to the Hall of Fame. I'll take my chances.

I'm ready for July. Batter up!

Lab Recap

I've been thinking about sports a lot lately. My apologies in advance. 

*****
Giffy turned in a career-high protein-creatinine urine ratio this week, scoring a 3.40. This impressive personal best topped her previous high of 2.91 in March 2013 and is huge jump from 1.68 in October 2013.

The normal range for this test is 0.01-0.18, so Giffy is losing the battle with proteinuria -- excess serum proteins found in the urine. The stat sheet tells the story:

Also making its first appearance: an elevated phosphorus level. 

Insurance hasn't ruled on the Rituximab treatment yet, so Giffy's kidneys continue to spill protein at an alarming rate. When interviewed after her test results came in, she said:

"You know, I'm just happy to be here and I hope I can help the ballclub. I just want to give it my best shot and good Lord willing, things'll work out. Gotta play 'em one day at a time."

Or something like that.

Diagnosis

I've finally learned how to spell fibrillary glomerulonephritis without referring to my medical paperwork. Now I need to learn how to pronounce it, because that is my diagnosis.

WHAT I KNOW:
1. Every online entry I've found about the disease includes this line: "Fibrillary glomerulonephritis is a rare disease with poor renal prognosis."

2. How rare? It only shows up on .05 - 1.0 percent of kidney biopsies.

3. There is no known treatment.

WHAT COMES NEXT:
UM and Mayo have recommended trying treatment with Rituximab, a lymphoma drug. UM has requested pre-authorization from my insurance company and we're now in wait-and-see mode.

HOW I FEEL ABOUT THIS DIAGNOSIS:
While I'm relieved that this is not amyloidosis, I'm also struggling with the reality of having a rare disease that I can't pronounce and no one understands. My health situation is exacerbating a deeper issue: feeling different. Remember the Sesame Street "one of these things is not like the other" skit? Yep. That's me. I'm the W on a chalkboard full of 2s. I've always felt out of place -- at jobs, on teams and even in my own family. And now I feel like I don't even fit in within the world of kidney disease patients. So far I've found one person to talk to -- a very nice 64-year-old woman in Florida with fibrillary glomerulonephritis who has been kind enough to answer my questions over email. For that, I am grateful. She has helped me realize that others have faced this disease and that I can, too.

I found another W.  

My Liver Biopsy Experience

As recently as two weeks ago, I didn't think I'd be writing this entry. But it turns out that I needed a liver biopsy on Jan. 23 in yet another step to definitively rule out amyloidosis.

The biopsy went well, and I attribute that to two things:

1. Drugs
2. Led Zeppelin

Here's what happened. We arrived at UM at 7:30 a.m. and they quickly took me back to prep. I had one goal: convince the doctor to give me some type of drug. Anything. Two nurses told me what I already knew -- that they don't like to give sedation for a liver biopsy because you have to be able to follow commands during the procedure and breathe in and out when asked. I was polite and listened but remained firm in my resolve. So they paged the doctor and asked, and he agreed to give me 2 mg of Midazolam through my IV. Yes!

When they rolled me into the procedure room, the doctor was in there listening to Led Zeppelin and preparing for the biopsy. It was awesome. I felt so happy about the vibe in the room. The doctor was smiling, the nurses were talking to me about classic rock and the albums and 8-track tapes that we had as kids, and I was in such a good mood that I almost forgot that someone was about to stick a needle into one of my major organs.

The doctor explained everything to me as he was preparing the site. He used ultrasound and also percussion (tapping) to find the right spot. He had me practice how he wanted me to breathe. But I really don't remember a lot about the procedure once the Midazolam was administered. I did feel the needle that numbed the biopsy site, and I did feel the needle that went into my liver to get the sample. It was a sharp pain but a 36 mm piece of tissue was quickly removed and then it was over. The official paperwork states that I was brought into the room at 8:21 a.m. and the procedure was complete at 8:46 a.m.

They had me lie on my right side for 30 minutes in the recovery room before letting me lie on my back for two hours. I was discharged at 11:30 a.m.

On the Giffy Suckage Scale, this liver biopsy wasn't bad at all. It's been three days and I'm still a bit sore, but I've had no complications.