Please Watch This Video

NephCure Kidney International, in partnership with the National Kidney Foundation, has just released a new video -- and it resonated with me. I'm sharing the link here in hopes that others will watch the video and learn more about the Nephrotic Syndrome journey.

As NephCure CEO Mark Stone said in his email release of the video, Nephrotic Syndrome can be difficult for patients and caregivers to explain, and difficult for friends and family to understand.

I know that I struggle to explain how I'm feeling about this disease; perhaps the video will do a better job than I do.

Thanks for watching. 

  

Shingles > Fibrillary Glomerulonephritis

So a funny thing happened when I had shingles a few weeks ago.

People understood. Everyone had heard of shingles. Most people I told had either had shingles themselves, or had a close family member who once did so. That's not surprising, as one in three people will have shingles at some point in their lives. (Get ready. And yes, it sucks.)

I went to urgent care within 48 hours of the outbreak, so I was given a seven-day course of an anti-viral medication called valacyclovir, and the drug worked well for me.

What struck me during this episode was that people understood how I was feeling, and that never happens with my kidney disease or liver disease. Those are invisible and a bit harder for people to understand. "But you look OK," people will say. "I always forget," others tell me.

With shingles, people looked at me like someone died. "Oh I'm so sorry."

There was a small patch of shingles on my back, and the pain was a combination of burning fire and needle poking. But people heard, and would talk to me about it, and share their own personal shingles story. Fibrillary glomerulonephritis makes me feel isolated and alone. Shingles made me feel like I got invited to a party where all of the cool kids were. I was in the club.

It may sound silly, but having shingles wasn't that bad. There was a treatment available. My case was mild and didn't spread. And once the nerves in my back calmed down and the blisters crusted over, well then the healing began.

There was a beginning, and there was an end.

It's true.

Shingles > Fibrillary Glomerulonephritis.

And Then There Were Two

I'm now down to just two meds. I met with my rheumatologist yesterday, and together we decided that I should stop taking the plaquenil and see how it goes. Our thought process was:

• Neither of us was convinced it was making a difference
• The rheumatologist feels that the Rituxan treatments I've received will help me more than the plaquenil
• My kidneys are my main concern, so we should focus on meds that directly help my kidneys

That means I now take:

1. Lisinopril 40 mg (blood pressure)
2. Spironolactone 25 mg (diuretic)

I recently met with my liver doctor and everything is stable there, and I'm going through the vaccination process for hepatitis A and hepatitis B. I'm two shots into the three-shot, six-month process. I'm also still waiting on dermatology to do the procedure on the two basal cell carcinoma spots -- although I'm in no rush for that to happen. The biopsy spots have healed and it's nice not having open wounds.

I'm pretty excited to be at this point, as I continue to feel the best I've felt in a long time. I'm getting my life back!

I feel like an Olympian.

 With my Mom at Whistler, British Columbia

Best. Rash. Ever.

My latest medical adventure has nothing to do with my kidneys. 

On May 30, I started noticing small bumps on my left wrist. They were hard to ignore, since they itched like crazy. Soon I had more bumps on my wrist, then more on my tricep, knee and ankle -- all on the left side. I tried to self treat at home for six days until I couldn't take the itching anymore and went to UM acute care.

The doctor said that this was a rash that I probably got from gardening--even though I told her that I haven't been doing any gardening. She prescribed a steroid cream, referred me to a dermatologist and sent me on my way.

On June 11, I was able to see the dermatologist. By this time, the rash had spread to my right side, including a very painful patch on my right hip. The itching was intense.

I was waiting to hear the dermatologist's rash diagnosis when she said:

"I'm so glad you came here today. You have a lot going on."

Huh?

Soon she was using liquid nitrogen on my face to treat some actinic keratosis (pre-cancerous) on my left cheek. Then she did shave biopsies on two places she said that she was concerned about: one on my right hand and one on my left upper arm, just above my elbow. Neither of these spots were near my rash or had anything to do with the rash, yet they had her full attention.  

On Friday -- 20 days after this dumb rash sent me searching for relief -- the dermatologist confirmed that the two spots were basal cell carcinoma skin cancer. We are now waiting for my biopsy wounds to heal before treatment. The doctor is recommending a basic surgical procedure called curettage and electrodesiccation. It all sounds pretty straightforward and I'm not too worried. I'm not exactly excited about the doctor digging into my hand and arm again, since I'm guessing these will be deeper than the shave biopsies, but I'll deal with it when the time comes. 

I now think my "dumb" rash is a pretty cool rash, since it led to the discovery of my skin cancer. 

The official rash diagnosis, by the way, was contact dermatitis. Ha. I basically got exposed to something that my body didn't like, hence the rash. The doctor looked at the steroid cream the acute care doctor had prescribed, shook her head and with wide eyes said, "That's not gonna be enough" -- delivered in the same tone and timing as the famous Jaws line -- when Martin Brody (Roy Scheider) said, "You're gonna need a bigger boat." It cracked me up. 

Above -- what the first doctor prescribed. 

This is my loot from the dermatologist. A bigger boat indeed!  The rash is now nearly gone, thankfully.

The Cost of One Rituxan Treatment

What's posted above is a cut/paste from a recent billing statement. This is the cost breakdown from one of my Rituxan infusions. And I've had EIGHT so far -- four in July 2014 and four in February 2015. 

My treatments were pre-approved by my insurance company, so my own portion of this bill is not nearly as obnoxious as the sticker price. 

But seriously -- who can afford this stuff? Why is this drug so expensive? I just don't understand our health system.

Fibrils

I was talking to a friend about fibrils the other night, because hey, who doesn't love a good fibril conversation? I was trying to explain why I'm still sometimes uneasy about my fibrillary GN diagnosis, and of course I struggled to verbally make sense of things.

That's when I turned to my good friend Google and found this image:

Fibrillary GN fibrils are typically 15-30 nm.
Amyloid fibrils are typically 9-12 nm.

My fibrils are 9-10 nm.

This means nothing, of course. I don't think Mayo and UM are wrong. My sample didn't stain congo red.

I just think it's one piece of information that is unique to me and -- in the words of C + C Music Factory -- is a thing that makes me go hmmmm.

[Obscure '90s music reference free of charge.]

My Kidneys are Tweeting

My kidneys may not filter so well, but they can Tweet.

Follow me on Twitter - @giffyskidneys

Marcia Brady, for the win!

Here's a quick update. The text question was from my sister Danielle.





I am staying on the low phosphorus and low sodium diet. The UM nutritionist was awesome, though, and extremely helpful. I think we have a great plan and I have the clarity I need to make good choices. "Onward and upward," as my pops used to say.

Hey Jan Brady - My Liver Knows How You Feel

Lost in my medical story is that I also have liver disease. This has been confirmed by test results, scans and a liver biopsy. It's serious, yet I rarely think about my liver.

The only change I've made due to my liver disease is that I gave up alcohol. I had one drink in 2014 -- a Stella Artois on my birthday. 

Other than that, everything is about my kidneys and trying to stop the progression of fibrillary glomerulonephritis. The frequent tests and office visits. The expensive and experimental chemotherapy treatments. The special diets. It's kidneys, kidneys, kidneys all of the time.

My liver? I don't really give it much thought.

That changed a few weeks ago, when I saw the liver specialist at UM. During her warning that I could be headed toward cirrhosis, she gave me a pep talk about my diet.

"This is really simple," she said. "Only eat whole grains, and things like whole wheat pasta and brown rice instead of white rice."

I laughed and shook my head, knowing that my kidney doctor has me on a low phosphorus diet that has me avoiding all whole grains, wheat pasta and brown rice. The diet is completely counter-intuitive, but it's pretty standard for where I'm at with my kidney disease.

That's when I said that I wasn't going to deviate from what my kidney doctor has ordered.

And that's when I thought of The Brady Bunch - and how my kidneys are Marcia Brady.   

Jan Brady once lost her mind about "Marcia, Marcia, Marcia!"



If my liver could talk, I know it would whine, "Kidneys, kidneys, kidneys. I'm tired of being in the kidneys' shadow all of the time."

 


So what's next? I've been referred to a UM dietitian who will try to create a diet plan that will work for my kidneys and my liver. I go on March 4 and I'm actually pretty excited about the appointment.

[Big thanks to Rachel for the awesome drawing featured in this post!]

"No Pain. No Pain. No Pain." - Rocky Balboa

 
I've noticed something about myself lately. I'm terrible at drawing analogies. Yet despite my known deficiency in this area, I keep trying to make things analogous. I will blurt out "that's just like this situation blah-blah-blah" and before I can finish my sentence even I know that I'm not making sense.

This may be one of those times.

I had a kidney appointment this week, and I learned that the four Rituxan treatments from July are, in fact, making a difference. In particular, my serum albumin has improved and my protein spillage has decreased. My kidney doctor feels very positive about my progress.

I walked out of my appointment and immediately thought: I am Rocky Balboa, and I just cut Ivan Drago.

I'm still in the ring and still fighting the powerful bully. And the effort is working. (Anyone who is not a child of the 80s and doesn't know Rocky IV by heart, check out the video link above.)

I just heard the bell for Round Two. I have four more infusions scheduled, beginning in late January. These are weekly infusions that will kick my butt but will hopefully help me ultimately defeat fibrillary glomerulonephritis (that's Ivan Drago for those of you unable to follow my spot-on analogy!).

As I mentally prepare for another round of Rituxan, I keep thinking to myself: "No pain. No pain. No pain."

Let's do this!

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.

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!)