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.

My Path to a Diagnosis

I've had 127 tests at UM in the past 15 months. I didn't realize this until today, when I was reviewing some test results in my health portal and finally did the math. One hundred twenty seven. And this is just the count since Sept. 24, 2012 -- when UM started using this new portal system. Who knows how many tests I've had since this all started.

And when did this all start, you ask? I trace it back to 2005, when my primary physician found blood in my urine during a routine visit.

Now, as 2013 is coming to a close, I thought it would be a good idea to provide a little recap of my health journey. Here is a clear and straightforward [*cough cough sarcasm alert*] graph that shows my path to a diagnosis -- which, by the way, I still do not have. Enjoy!

114 Days

It has been 114 days since I first heard the word "amyloidosis." I don't remember much about the phone call from my nephrologist on July 31 -- other than she sounded like someone just died. And she said that she was sorry. I had her spell amyloidosis for me, and I jotted it down, but for the most part I was extremely confused.

August and September were somewhat of a blur. I was referred to hematology. Had a bone marrow biopsy. Heart tests. A fat pad biopsy. Blood and urine tests. I went to Mayo. Old tissue samples were stained. It has been 114 days of worrying and waiting that has all led to this:

Today my hematology team broke up with me. And they didn't even pull a George Costanza ("it's not you; it's me"). Nope. Instead they told me it was because of ME -- or more specifically -- because they can't find evidence of amyloidosis in me. I received an email that read, in part, that "we are very confident in our assessment that you do not have amyloidosis." They do not feel that I need any follow up in the hematology clinic. They did, however, recommend that one of my other doctors check for abnormal proteins once a year.

As far as hematology is concerned, amyloidosis is off the table. While this is a great relief, amyloidosis will always be my Boogie Man. It will be the shadowy figure lurking in the dark. And I'm not sure I will ever completely let my guard down -- especially knowing that my proteins will continue to be monitored.

So where do we go from here? I see the liver doctor on Jan. 3 so that he can weigh in on my liver issues, and then I see my nephrologist on Jan. 13. I'm guessing that the diagnosis will be fibrillary glomerulonephritis and that we will finally discuss a treatment plan (Rituximab infusions) in greater detail.  



  

The Suckage Scale

I had a liver MRI last week, and here's what I learned: they should never shove an overweight woman with anxiety into a tube. I found the test uncomfortable. There is no physical pain -- you just lie on your back in a tube -- but it's loud and confining and I fought claustrophobia the entire time. Thirty minutes seemed like three hours.

Afterward, I needed to vent. I am so sick of these tests and just needed to complain a bit before regaining my composure. I texted my sister Danielle, who is A.) hilarious and B.) colorful in her use of language. Most of what we text can't be repeated here, but trust me....it's good. She's always able to make me laugh. She's also empathetic but doesn't pity me. I appreciate that. Anyway, in one recent conversation she was asking me about a test and wanted to know how it compared to the other tests I've had. More importantly, she wanted to know if it was "complete suckage." 

Complete suckage? Lol. Never heard that one. That's when I knew I needed to create a "suckage scale." So, for you Danielle, here's my take on some of the tests I've had the last few months -- and how much they sucked. 

 (Thanks to my friend Jason for the artwork!)