Should I Play? Or Should I Stay?

I have to confess that I'm getting just a bit nervous as the date for my next rituximab infusion gets closer. Yep. On Tuesday I'll be rolling up my sleeve and asking to be poked with a large IV needle, if you can believe that.

I really do want to get this therapy started again, really I do. I just am hoping that my body cooperates and responds similarly to the way it did a year ago; I'd just rather not repeat the whole unpleasant low neutrophil blood count episode. So I'm thinking positive thoughts, exercising (kind of) regularly, eating (kind of) healthily by munching on all the fresh organic veggies and fruits that I can get my hands on and avoiding (kind of) wheat and sugar.

It always seems as though I'm more fatigued than usual for a week after the infusion; then two weeks later I lose another week after the second infusion before the sproing and spring in my step returns which lasts for the whole summer long. I sincerely hope. So yesterday I made a list of things that I wanted to do before the expected post-ritux tiredness sets in.

As I looked at the list, I realized that it was comprised of all very sensible items: cook some meals and then freeze them for my tired weeks, tidy the house, stock up on groceries, take down my Valentine's day stuff and drag out all of my Easter decorations.

I was sipping my morning coffee with Lulu on my lap. I don't think any of this stuff on my list looks like much fun, Lu, I sighed.

Lulu agreed. She told me she thought we should go to her favorite doggie park instead since it was a sunny day.

Well, if I thought we were going to play hooky from the to-do list today, girl, I'd.......well....... Ah. I'd grab Canon and head for the mountains. Wanna come? 
 
She agreed enthusiastically. So I grabbed my coat, Canon, and Lulu's leash and we headed out to the car. And yes, it happened just about that fast. Once I decide to avoid doing practical things, I waste no time in commencing the goofing off activities.

It's pretty easy to head for the mountains from my house. We live in the foothills of the Cascades so technically all I had to do was back my SUV out of the garage and I was there. But Lulu and I wanted to escape the city completely so we drove East along the Columbia river. It was a breezy day and we had some hazy sunshine. I looked West and could see that the next round of rain was moving our way right on schedule. We had no time to lose.

Let's take our pictures while the sun shines, right, my woogie?

After about a meandering hour-long drive, we were standing in one of my favorite spots of all in the Columbia River Gorge: where the river cuts a path through the Cascade mountains. Lulu and I could have arrived there much more quickly had we taken the freeway, but who wants to spend a beautiful day driving on a dumb stupid freeway? We take the back roads.

It was windy. Which is a gross understatement.

I stood face into the wind while Lulu sniffed excitedly around. Ahhhh. It was freezing cold, I could scarcely stand upright in the blast of wind, but golly. THIS was exactly what I wanted to do.

Yeeeaaahhhh. I snapped pictures until my ears and nose felt numb from the cold.

Lulu and I turned around and headed for home here at this beautiful marble rest stop built during the depression era WPA years.

The sun began to disappear behind the clouds as we started for home.

Lulu said that she was a bit peckish after her unexpected outing. I asked if she would like to split a small ice cream and she agreed. How fortuitous that we were passing an ice cream place just as we were having our discussion.

You eat from your side and I'll eat from mine. No slobbering.

Notice that I said earlier that I was KIND OF eating healthily.....

We pulled into our driveway just a couple hours after we left. Such a short trip but what a delicious day.

We'll be back. Probably as soon as I can concoct a reason to play hooky again.

Music To My Ears

File this one under Dang. Wish I Could Still Be Doing This.

I really enjoyed singing with this group during my pre-Sjogren's days. Here they are performing with the Dubrovnik (Croatia) Symphony Orchestra. Can you spot Terese? Lookin' good, girl. Sounding good too.

One To Watch

Ms. Smith's image found here. 

I would love an opportunity to see this documentary:

Award-Winning Filmmaker Reissues Groundbreaking Documentary to Benefit AARDA
Beauty Does Lie Tells the Stories of African American Women Triumphing Over Autoimmune Diseases

MARCH IS NATIONAL AUTOIMMUNE DISEASE AWARENESS MONTH 2014
Newswise — Award-winning documentary filmmaker, Courtney G. Smith, has teamed up with the American Autoimmune Related Diseases Association, Inc. (AARDA) to raise awareness of autoimmune disease among African American women in 2014.
The partnership will launch in March during National Autoimmune Disease Awareness Month with the reissuing of Smith’s groundbreaking documentary, Beauty Does Lie: The Untold Stories of Autoimmune Diseases in a digital format. The film traces the lives of seven African American women living with various forms of autoimmune diseases including lupus, multiple sclerosis, Sjogren’s syndrome, pernicious anemia and myasthenia gravis.

You can read more about it here.

Blame This on Aunt Katherine

I was tucked into bed last night all comfy and cozy and slightly drowsy. You know that feeling -- when you're teetering just on the very edge of falling asleep. Love it.

It's one of those times that my thoughts meander in the darndest directions. Last night my musings took a trip back in time to my high school days. I thought about how my Aunt Katherine, who was also my high school english teacher, had given our class an unthinkable assignment: to journal every single day for a semester. She gave us each an empty spiral notebook and asked us to write a paragraph or two on any subject of our choosing each day.

We all were indignant. Imagine that. To write something _every_single_day. What a mean teacher! How ruthless! And the worst thing of all? We even had to WRITE SOMETHING ON THE WEEKENDS. Saturday AND Sunday!

She told us that she would collect our notebooks every Friday and promised us that she wouldn't make any corrections on our grammar or sentence structure or even spelling.

"It's just an exercise in routinely recording your thoughts or things that happen to you every day that may otherwise seem unimportant. But when you look back and read your journals later in life, these notebooks will tell you a great deal about yourselves. You get credit for this assignment simply by completing it. Just write." she said.

Well. THAT's a dumb assignment, I thought. BICJ was alive and well even when I was sixteen.

But since I was a good student and more importantly, the teacher's niece and no dummy -- I knew that if I were disrespectful or goofed off she would let my parents know asap -- I dutifully scribbled away and filled page after page of the lined paper in my notebook. Every night before bed, I would sprawl belly down on the orange shag carpeting in my bedroom (Hey. Don't judge me here. It was the '70s.) and write. And every night I would grumble to myself about the unfairness of it all. Gee. I could be doing all sorts of other stuff instead of this dumb stupid assignment..

I wrote about the way night time sounds would drift in through my open bedroom window. I wrote about my mosquito bites. I described the sounds that our herd of cows made when they chewed their cud. I wrote about my dislike of that assignment. I described the floral pattern in the fabric of my favorite blouse. I critiqued my sisters' hair styles.

When the end of the semester arrived, I tossed my completed journal onto the stack on Aunt Katherine's desk and thought about how glad I was not to have to do anything like that ever again. Because writing about stupid stuff in my life every day was just....well, stupid.

So last night I found myself chuckling when I realized that I had learned two things from my late night musings inspired by Aunt Katherine:

• I'm a slow learner. It only took forty years or so for me to realize that writing something every single day is actually kind of fun. And...
• This blog is all Aunt Katherine's fault. 

Taste Troubles

image found here. 

Bleah.

I hate when my taste buds go missing. My sense of smell and taste has definitely been altered since Sjogren's syndrome made it's entrance into my life. Sometimes those senses are totally absent, other times they're completely out of whack; and then occasionally everything tastes and smells A-OK. I never know what my tastebuds will tell me until I lift the spoon to my lips.

Today I eagerly anticipated a home made chicken soup. I purchased a plump whole bird, cut up the whole thing and threw it in my favorite soup pot along with fresh garlic, chopped onions, and some salt, covered it all with water, started the burner and sat back to wait for the soupy goodness to begin.

Those are my very favorite seasonings for a really good chicken soup. Just garlic, onions, and salt. If the chicken is of good quality, it's yummy flavor doesn't need much embellishment.

Three hours later, as I tasted the broth, I was seriously disappointed.

Yuk! Hey John -- come and taste this soup. Something is wrong with it.

*sluuuurrrrrrpppp* "I think it tastes great, Babe."

Drat. Weirdo sense of taste strikes once again. John ate his fill and we put the abundant leftovers in the refrigerator. I'll try again tomorrow.

If you see my taste buds wandering around, tell them Julia wants them back. ASAP.

Dry Eye-wear

I saw a link to this company that supplies moisture-retaining eyewear. Has anyone experience with this company or used their products?

Ever since my cataract surgery, the dryness in my eyes has become a bit more significant. My eye surgeon warned me, and she was right...

I don't think I'm a the stage of needing moisture goggles yet, but having a pair of sunglasses for driving and those times spent outdoors in the wind may be just the ticket.

Here's the link to 7eye:

DRY EYE - SJÖGREN'S

No matter how mild or severe your dry eye symptoms are, you can benefit from the special protection provided by 7eye. Like old-fashioned moisture chamber goggles that have been prescribed for years for patients with extreme dry eye symptoms, glasses from 7eye help retain corneal moisture and block evaporation, but they do so much more.

7eye/ Your World. Your Vision. 

Let me know what you think.

A Prednisone Plan....

50mg prednisone tablet image found here. 

Now that Dr. Young Guy and I have made the decision to restart my rituximab infusions, I'm getting pretty excited to just get going with it all.

Come what may. I'm betting that my neutrophils will behave themselves this go-around. My first infusion will scheduled for the first week in March.

Two years ago after my first infusion, I thoroughly enjoyed the summer that followed. Ahhhh. How great it was to be doing stuff with my family. Not needing a nap every day. Able to wear blouses and tops that didn't come up to my chin since my Lupus rash had faded away into nothingness....

What I don't recall with any fondness whatsoever from my post ritux experiences are the side effects from all that prednisone which must accompany the infusions.

I didn't have issues with sleeping because I made certain to take it early in the day, and although I didn't particularly like the puffy face that followed it wasn't a long term problem since I tapered the dose eventually. No, the biggest issue for me with prednisone in any dose larger than 10mg/day is the drug creating an overwhelming urge in me to bury my face in a large chocolate cake.

Repeatedly.

I have to be truthful here, and anyone who's even remotely familiar with my eating habits knows: I'd be tempted to give myself a chocolate cake facial just about any day of the week. What changes with prednisone on board is that the temptation is almost completely irresistible.

On occasions there have been fairly cake-free periods in my life. Of varying durations.... But since Christmas, I've been trying to significantly reduce the amount of sugar and wheat in my diet.

(Hey, Terese. No fair telling about all those times that I was not successful, either. Like the cheesecake at the rib joint, and the pie at Shari's and...yikes.)

Notice that I said I was trying, and here's why: I'm hoping that if I have been eating a relatively low-sugar low-wheat diet before I need to bump up my current dose of prednisone from 5mg to 60mg/day, that perhaps the cravings for carbs will be a bit less severe.

What do y'all think? I really have nothing but my intuition guiding me here and no studies to back up my little idea, but I'm thinking that there's no harm in trying to reduce wheat and sugar regardless of what my medication regime is.

Comments? Ideas? Suggestions? I need all the help I can get.

Another Rituximab Discussion

Let's talk rituximab today, shall we?

The results of a study done in France on rituximab therapy in the treatment of Sjogren's syndrome were recently released; the abstract and study authors are published online in the Annals of Internal Medicine and can be found here. The study was titled Treatment of Primary Sjögren Syndrome With Rituximab: A Randomized Trial. The conclusion of the study:

"Rituximab did not alleviate symptoms or disease activity in patients with pSS at week 24, although it alleviated some symptoms at earlier time points....In conclusion, our data do not support the use of rituximab therapy in many patients with recent-onset or systemic [primary Sjogren's syndrome]," they stated."

Here's how the study was conducted:

The authors studied 120 patients with primary Sjögren syndrome between March 2008 and January 2011. To be eligible for inclusion, patients had to fulfil the European-American Consensus Group criteria for primary Sjögren syndrome and to have active disease, defined as a score of at least 50 mm on at least 2 visual analogue scales (VAS) for global disease, pain, fatigue, and dryness..... 

The primary study outcome was an improvement of 30 mm or more on at least 2 of the 4 VAS scores at 24 weeks.The patients were randomly assigned to receive a placebo or 1-g infusion of rituximab at weeks 0 and 2. Patients and investigators were blinded throughout the study. The researchers conducted follow-up examinations at weeks 6, 16, and 24....... 

At 6 weeks, 22.4% of patients in the rituximab group improved by 30 mm or more on at least 2 of 4 VAS scores compared with 9.1% of patients in the control group, a difference of 13.3 percentage points (95% confidence interval [CI], 0.8 - 25.8; P = .036). The most significant clinical improvement was seen in fatigue, with 34.7% of the rituximab group and 8.2% of the control group recording VAS improvements of 30 mm or more, a difference of 26.6 percentage points (95% CI, 15.7 - 37.5; P < .001). There was also a difference of 19.1 percentage points (95% CI, 4.4 - 33.7; P = .011) in physician-assessed disease activity. Significant improvements also were seen between the groups in levels of immunoglobulin A (IgA; P = .026) and IgM ( P = .004), but in no other physiological or immunological measures. By week 16, rituximab was still associated with a greater improvement in fatigue (27.2 percentage points vs 8.9 points; 95% CI, 4.1 - 32.6; P = .012), but no other significant difference in clinical improvement, and by week 24, even that difference was gone. However, improvements were seen in levels of IgG, IgM, C4 complement, and β2 microglobulin at 16 weeks, and in all of those components plus IgA at 24 weeks, with rituximab, despite the lack of significant clinical differences. [Bolding mine]

Immediately after the study release, several stories interpreting the results appeared, and the tone of these discussions were quite pessimistic, like this one: Rituxan Offers Little Help for Sjogren's, found on Medpage today. And this from Medscape Nurses: Rituximab Disappoints in Sjögren Syndrome.

Having only read the headlines, my initial reaction was one of disbelief. You may remember that two years ago, I began rituximab infusion cycles separated by six months, and until I had an unusual response (neutropenia) I was very happy with the results. I received one gram of rituximab IV along with some pre-meds; and two weeks later another gram IV. Initially after my infusions, I was pretty tired but within a month could see a distinct increase in my energy levels and I had a modest improvement in my saliva production. The positive effects lasted for about a total of four to five months; and at six months the cycle of treatment was repeated, and my increase in energy was repeated as well.

So in an attempt to understand this all a bit better, I went back to read the study reports and the articles discussing the study, and realized that my experience with the drug was exactly the same as the study participants.

Um. Wait a minute here....My experience with this drug was very similar to those described in the study. I consider my experience one that assisted me enormously in the day to day struggles with Sjogren's related fatigue. The researchers didn't see it that way: "our data do not support the use of rituximab therapy in many patients with recent-onset or systemic [primary Sjogren's syndrome]." We're both looking at the same data but drawing opposite conclusions.

Is it possible that once again, fatigue is not considered a debilitating element of our disease and was discounted as a significant symptom in this study?

Sigh. Let's go back and review:

• At six weeks......The most significant clinical improvement was seen in fatigue. Most definitely true in my case. 
• By week 16, rituximab was still associated with a greater improvement in fatigue. Let's see, that would be four months out. Yup. Sounds right.
• ....by week 24, even that difference (decrease in fatigue) was gone. At six months, I needed to have my infusions repeated. Which is exactly what Dr. Young Guy had told me would happen, which is what we did, and my energy rebounded right on schedule.

I'm confused. The study authors felt that these clinical improvements were significant but lasted less than six months. The patients were never given a second cycle of the drug. Were they expecting that one set of infusions would create clinical improvements that would last indefinitely?

Really?

The fact that the B cell depletion action of rituximab is considered only to last six to twelve months was surely not unknown to the researchers since the target antigen (CD20) is not found on hematopoietic stem cells, pro-B-cells, normal plasma cells or other normal tissues. (Translation: Although rituximab decreases peripheral CD20 B lymphocytes, they eventually grow back since the tissues that create them (hematopoietic cells) are not targeted by the drug).

Let's repeat that: The desired effect of rituximab is in it's depletion of mature B lymphocytes with a CD20 antigen. Since the drug does not deplete the tissues that produce these lymphocytes, and since it takes the body six to twelve months to replenish these cells, it would appear only logical that the effects of the drug would diminish once the B lymphocytes regained their pre-rituximab numbers.

Am I being too simplistic here?

Apparently, other folks share my skepticism of this study:

At least one outside expert was less surprised. "The length of this study was short, considering that Sjögren syndrome is a lifelong condition," said Solomon Forouzesh, MD, associate clinical professor of medicine and rheumatology, University of California, Los Angeles, Geffen School of Medicine and Cedars-Sinai Hospital. "And although it is a relatively rare condition, 120 patients is not a lot. The authors needed a larger sample size." Dr. Forouzesh also questioned the study design. "This is not a disease you can control with 2 infusions. The study should have been designed more like a study for rheumatoid arthritis, in which the infusions are ongoing. You must give this drug continuously over time in order to see improvements in lacrimal glands or salivary flow. A chronic disease like Sjögren syndrome requires ongoing suppression."

Hm. "A chronic disease like Sjogren syndrome requires ongoing suppression."

Well. French study notwithstanding, this sjoggie plans on re-starting rituximab therapy in March to provide that "ongoing suppression".

I took a year long hiatus from the drug since my last cycle produced a significant neutropenia, an unusual side effect of ritux. But after lengthy discussions with Dr. Young Guy and a new plan of increased monitoring of lab values, I have decided to give it another try in the hopes that the neutropenia will not reoccur. If it does -- dang. I don't get any more chances with rituximab. But I'm willing to try. Living for the past year with the return of truly bone crunching fatigue has convinced me of the value of B cell depletion therapy.

Go Joe!

Everybody watching the Olympics? I've always enjoyed following the games over the years, and usually my main interests have been in ice skating and ice dancing. I used to be enthralled by the skaters from the moment when their routine music would begin until the very last competitor left the ice and the professional commenters were rehashing the medal results.

I'm still enjoying skating this go-around, but I have found another area of interest:hockey.

Yeah. Very strange in that I've never been in the least bit interested in hockey before. But I was scrolling through the zillion or so channels that carry the events on TV when I paused on the USA/Russa men's hockey game last weekend. The only reason that I didn't continue flipping through the channels was because I heard the name of one of the US players: Joe Pavelski.

Hey, I thought. That's Mom's maiden name. And one of my favorite guys in the Pavelski clan was my great-uncle Joe. Joe Pavelski? Hmm. Are we related? Mom told me emphatically that we aren't, but I choose not to believe her. Because this guy is really, really good; at least as far as I can tell being pretty ignorant about the sport.  And because Joe has all of his teeth (which isn't always the case with hockey players from what I've observed) and he's pretty darned good looking.

We must be related.

See? Image found on Wikipedia. 

So I sat down and watched the game and it was a nail-biter. It went into some kind of overtime shoot out thingie which the US won. I was hooked.

My goal for the remainder of the Olympics is to learn more about hockey. Currently my level of understanding is pretty skimpy: I know that everyone has skates; the players wear short pants, they wave their hockey sticks around, the goalie has crazy amazing pads everywhere, and that there's three periods in a game. That's it.

Oh, and one other important thing: My relative Joe ROCKS hockey.

When Harry Met Candy

Candy didn't like Harry one little bit. 

I've grudgingly come to realize that there probably will be an ongoing need for a cane in my life. Not every day. Not all the time. But when my inflamed bursa in my hip acts up, or my inner ear gets cranky and makes my sense of balance all wonky, I just need to get over myself and use a cane.

I was at the community pool last week and while I was changing, a woman approximately my age came in and sat on the bench next to me. She also had a cane, so we exchanged smiles and names and diagnoses.

We cane people are friendly like that.

My new swim pal was sporting a nifty sleek black cane. I immediately coveted it.

That's a cool cane. Where did you get it?

"Oh, I ordered it online. I like it because the three points on the bottom make it more stable, and it folds up really easily so that it's much more compact when you want to take it on a bus, or in a church pew or," and here she gave it a quick snap, collapsed it into a third of it's original length, and tossed it into her locker, "when you're late to your swimming class and all of the big lockers are gone!"

I was at my computer within minutes of arriving home doing a search for Hurry Canes. Turns out you can buy them almost anywhere that sells medical supplies like canes, crutches, and walkers. I chose the Hurry Cane brand because I had a chance to see it in person, but I did notice that there were several other models from various manufacturers that looked very similar.

I love the Buy It Now button on Amazon.

So my new cane arrived yesterday and I gave it a thorough test drive. The first thing that I noticed was that this thing was definitely masculine. So I gave him a boy name: Harry the Hurry Cane. Harry was easily adjusted to the correct height, he's amazingly stable, and I really like the fact that he's an unobtrusive shade of black. With a twist of the handle, he can also stand up all by himself.

Spiff.

The three point base on this thing does increase my sense of stability when walking with it, but the base is smaller and less clunky than the traditional four-point canes.

Candy was miffed. "What's with the new kid on the block?" she snapped.

Candy, girl. I've to to tell you the unvarnished truth here. You're....shiny. And unwieldy.  You failed me big time when I was hanging on to you and we crashed face first onto the concrete garage floor. I just need a cane that doesn't scream orthopedic or geriatric when it's seen, and is more flexible and stable.

"I'm not cool enough for you. I see how it is."

Yeah. Candy will have to pout for awhile. I'll keep her as a back up, but for now she's retired. Hope she likes living in my closet. Sorry, girl.

Sjogren's Specific Genes

image found on Wikipedia

This amazing news can be found in the winter 2014 edition of the Sjogren's Syndrome Foundation's publication Sjogren's Quarterly:

First Genes Specific to Sjogren's Identified! 

"Scientists at the Oklahoma Medical Research Foundation have identified the first-ever Sjogren's-specific genes. Six genes in Sjogren's and additional genes already linked to autoimmune disease were discovered by the international consortium, Sjogren's Genetic Network (SGENE), led by Kathy L. Sivils, PhD.........Lead author on the publication, Christopher Lessard, PhD states, "Now that we've identified these genes, we can dig down and start to understand how these genetic variants alter normal functions of the immune system."..........A full article on the findings will be published in the spring issue of the Sjogren's Quarterly. The journal publication is: Lessard DJ et al. Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjogren's syndrome. Nat Genet. 202213 Nov;45(11):1284-92. dii: 101038/ng.2792. 

I can't wait to hear more about this extraordinary finding. The next time someone tells you that Sjogren's is "all in your head", tell them that actually it's in our genes. So there.

I Dream of Sweeping

I suppose I could practice with my real broom..

I had a great dream last night.

I know. Other people's dreams aren't very interesting, but what I wanted to share was one detail that shouldn't have been important but was.

So I was snoring away last night, and as per usual, my dreams were vivid and colorful and weird as heck. (I'm blaming the plaquenil.)

In my dream, I was still working at my last job which was at an Alzheimer's facility. We were having a party, all my favorite clients were there, and we were having a great time. The highlight of the entertainment was watching someone frost a cake with a hairbrush that turned the icing into pretty colors. So after the party was finished and all the residents had left, I was alone in this dream party room and started to clean up. The floor was covered with shiny pebbles for some bizarre reason, so I went to the janitor's closet, came back with a broom and vigorously swept away all the glossy brown and deep red pebbles.

When I awakened and thought about my dream, what struck me as odd wasn't the hairbrush that turned cake frosting into a vivid shade of pink, or the floor covered with colorful rocks.

No -- what I found amazing was that I grabbed a broom and swept a large room without breaking a sweat. I remember that even in my dream I was thinking, It feels so good to really use my muscles. I leaned into each draw of the broom as I swept my way over the large floor. It felt like a strange waltz of sorts.

Sjogren's has changed everything; even the things about which I dream.

Let's Try This Again

Oops.

Sometime during the last few weeks, a reader sent me an email. In it, she described several frustrations and problems that she was experiencing from her Sjogren's syndrome.

AND..

Sometime during the last few weeks, I must have inadvertently deleted it. Dang. I try very hard to answer all emails, so if you are this reader, would you consider re-sending your letter? I promise not to lose it again. Well...let me rephrase that. I promise to try not to lose it again. Cross my heart.

Facebook Funny

So John and I went out for the evening with Greg and Terese last night. It naturally follows that my brain isn't working this morning and I'm not moving very fast.

And we didn't even do anything wild and crazy! Ah. We're getting old, I guess.

My point here is that this will not be an informative post. Instead, it's something to laugh about, especially if you have a Facebook account.

See you tomorrow.

True Blue Valentine's Day

image found on free wallpaper site Fanpop

Happy Valentine's Day, everyone!

In the spirit of the day, I wanted to tell y'all that I love you guys. I really do. And before anyone gets the wrong idea, the kind of love I'm referring to here is what the Greeks identified as "philia": brotherly love. Or more accurately, true blue sjoggie friendship.

Group hug, people.

See y'all tomorrow.

Study This Before Reading the Study

No, I don't know what that big brown thing is on her blog page. 

I saw the link to this blog post on twitter the other day: How to read and understand a scientific paper: A guide for non-scientists. I thought the post, written by Jennifer Raff for her blog Violent Metaphors, was very good.

I wish I could remember who put up that tweet so I could give that person credit. Dang.

I thought it was particularly relevant since it seems that I have been posting about several studies recently. And we all know WHY Julia keeps putting up links to autoimmune research, don't we? All together now:

Because knowledge is power, people! *punches fist skyward*

Whew. Glad I got that out of my system.

Simply reading a study doesn't do any good if one is unable to make sense of the information, and becoming familiar with the usual format that these study results are published in goes a long way towards digesting the data contained within. When I read Jennifer Raff's piece, I appreciated her ability to clearly explain this process. I have included just a few of her steps here but head over there to read the whole thing:

1. Begin by reading the introduction, not the abstract. 

The abstract is that dense first paragraph at the very beginning of a paper. In fact, that’s often the only part of a paper that many non-scientists read when they’re trying to build a scientific argument. (This is a terrible practice—don’t do it.).  When I’m choosing papers to read, I decide what’s relevant to my interests based on a combination of the title and abstract. But when I’ve got a collection of papers assembled for deep reading, I always read the abstract last. I do this because abstracts contain a succinct summary of the entire paper, and I’m concerned about inadvertently becoming biased by the authors’ interpretation of the results. 

2. Identify the BIG QUESTION. 

Not “What is this paper about”, but “What problem is this entire field trying to solve?”
This helps you focus on why this research is being done.  Look closely for evidence of agenda-motivated research.

 Continue reading here.

Yes, that's right. This author suggests that you do not read a scientific paper or study from the beginning.  But you CAN read her post from beginning to end. So go do it.

Biotene Formulation Changes

Are you familiar with GlaxoSmithKline's Biotene dry mouth products? Along with moisturizing agents, at one time these products contained enzymes which were intended to help replace those decreased or absent in dry mouth patients. It's interesting that their webpage specifically mentions Sjogren's syndrome.

I don't have a great deal of experience with Biotene oral gel, toothpaste, or mouthwash. I use a fluoride prescription toothpaste from my dentist, and although I have occasionally used their mouth spray, overall I don't like the slimy sensation of artificial.....spit. Especially at room temperature.

Sorry. Gross but true.

However. It appears that Biotene manufacturers have changed their formulation by removing the three enzymes previously used in their products, among other changes. I have heard from several sjoggies that the change in this product is noticeable -- and not for the better.

A member of one of the Sjogren's Syndrome newsgroups that I follow recently alerted me to the change, and included ingredient lists for old and new products:

BIOTENE formulation changes 

BIOTENE ORAL GEL 

OLD FORMULATION =96 ORAL BALANCE
Water,  Hydrogenated Starch Hydrolysate, Glycerol, Xylitol, Butylene glycol, Sodium polyacrylate, Polyacrylic acid, Hydroxyethylcellulose, Sorbic acid, Benzoic acid, Lactoperoxidase, Lysozyme, Lactoferrin, Glucose oxidase, Disodium phosphate, Potassium Thiocyanate. Contains ingredients derived from milk and egg white.
Enzyme System: Lysozyme, Lactoferrin, Lactoperoxidase 

NEW FORMULATION 96 ORAL GEL
Water, Glycerin, Xylitol, Sorbitol, Carbomer, Hydroxyethylcellulose, Sodium hydroxide, Propylparaben 

BIOTENE TOOTHPASTE 

OLD FORMULATION
Active Ingredients: Sodium Monofluorophosphate (0.14% w/v Fluoride Ion). Purpose: Anticavity. Inactive Ingredients: Sorbitol, Glycerin, Calcium Pyrophosphate, Water, Hydrated Silica, Xylitol, Silica, Hydroxyethylcellulose, Isoceteth-20, Cellulose Gum, Flavor, Sodium Benzoate, Lactoperoxidase, Potassium Thiocyanate, Glucose Oxidase, Lysozyme, Lactoferrin, Calcium Lactate.
Enzyme System: Lysozyme, Lactoferrin, Lactoperoxidase 

NEW FORMULATION
Sodium fluoride, Water, Sorbitol, Hydrated silica, Glycerin, PEG-8, Cocamidopropyl betaine, Xantham Gum, Flavor, Sodium Saccharin, Sucralose, Titanium dioxide, Sodium hydroxide.
**Note the new formula does not contain Xylitol, but rather saccharin 85 

BIOTENE MOUTHWASH
OLD FORMULATION
Purified water, propylene glycol, xylitol, hydrogenated starch hydrolysate, poloxamer 407, hydroxyethylcellulose, sodium benzoate, flavor (peppermint oil), benzoic acid, disodium phosphate, zinc gluconate, lactoferrin, lysozyme, lactoperoxidase, potassium thiocyanate, aloe vera, calcium lactate, glucose oxidase.
Enzyme System: Lysozyme, Lactoferrin, Lactoperoxidase 

NEW FORMULATION
Water, Glycerin, Xylitol, Sorbitol, Propylene Glycol, Poloxamer 407, Sodium Benzoate, Hydroxyethylcellulose, Methyparaben, Propylparaben, Flavor, Sodium Phosphate, Disodium Phosphate

Do you use these products, and have you noticed the change?

Do you use other brands of dry mouth products?

True Cabin Fever

Yeah. I have it. It was so exciting to see the thermometer inch upward and hear the ice heading down. I sat out on our deck for awhile yesterday just to enjoy it all.

Watching ice thaw? Now that's REAL entertainment, people.



I think that my back yard sounded very much like Rice Krispies.



Thank goodness my back yard didn't look like Rice Krispies.

Is the physical burden of Sjogren's Syndrome underestimated?

Image source Shorpy. 

Last Wednesday I put up a post about a recent Tufts study in which a principal researcher summarized the study results in this quote from the Tufts press release:

“Our results suggest that people living with Sjögren’s still are able to maintain a reasonably high level of function, despite their perception of declining function over time. Sjögren’s can interfere with daily functioning and the burden of illness is very real. Nonetheless, it is apparent that even with these interferences people can compensate and function reasonably well,” said co-principal investigator and first author Lynn C. Epstein, M.D., a psychiatrist and clinical professor of psychiatry at Tufts University School of Medicine.

In my post, I expressed a few concerns regarding the study's treatment and examination of Sjogren's related fatigue:

Since fatigue is one of my most disabling symptoms of Sjs and I'm a bit touchy about labeling fatigue as an all in your head symptom, I found it interesting that elsewhere in the discussion section of this study, fatigue is included under the description of "neuropsychiatric problems". Continue reading here. 

 My post prompted several comments and responses which were very interesting, especially this from Dr. Sarah Schafer in which she references two important studies that seem to contradict the Tufts study conclusion: 

I’m sure some Sjogren’s patients live quite productive lives.  Some lupus patients do too. The variability in AI symptoms within a population is to be expected.  However, the conclusion by Epstein et al  that “ levels of function approach that of healthy controls” is disturbing and goes against much evidence and plain common sense.  This reinforces the old and incorrect bias that Sjogren’s patients are just not that sick.  A study like this can perpetuate the current lack of clinical concern/ obliviousness of medical professionals.   It can also reduce incentives to research this disease which has sent many strong willed,  previously highly functioning people out of the workforce.  

Dr. Schafer addresses functional disability caused by Sjogren's in this study:

 Primary Sjogren’s Syndrome: health experiences and predictors of health quality among patients in the United States -by Segal et al (Open Access article), 2009    Link:  www.hqlo.com/content/7/1/46 

She highlights these findings in the study:

p. 2  (second paragraph in article): “the degree of functional disability was as great in patients with primary SS as those with SLE” 

p. 5 under the  heading,  Predictors of Health-Related Quality of Life: “sicca severity and disease duration were not significant contributors to impaired quality of life in any of the full models (with age and disease duration taken into account). Somatic fatigue was the only unique predictor of general health… ” 

p. 6, under Discussion: “Our data demonstrates that the reduction in health-related quality of life in PSS is similar to that experienced by patients with RA and SLE…” etc (read on). 

The noted functional impairment in that article,  as well as the finding that 70 % of primary Sjogren’s have disabling fatigue (1), suggests to me that the physical burden of this illness is often simply overlooked.  Fatigue and pain are invisible, and therefore easy to disregard or minimize.   RA patients have fatigue and pain,  but they also have visible, measurable joint damage.  Lupus patients may develop end organ damage – sometimes fatal - that is not usually seen in Sjogren’s.  However, we have our own end organ issues, not just lacrimal and salivary glands. Neuropathies are common (not so easy to diagnose and measure). Vasculitis of skin is a problem for many but often overlooked.   Lymphoma, a real and potentially fatal Sjogren’s complication, could be considered a type of end organ damage, but this is fact is also often downplayed. 

The fatigue article:  

1. Ng, Wan-Fai. Primary Sjögren’s syndrome: Too dry and too tired. Rheumatology. 2010;49:844-853.

This is excellent information. 

In addition to Dr. Schafer's comments, I'd like to mention that the first study that Dr. Schafer quoted, Primary Sjögren's Syndrome: health experiences and predictors of health quality among patients in the United States found here, had a much larger sample size (277 primary Sjogren's syndrome patients were compared to 606 controls) than the Tufts study (37 primary Sjogren's syndrome patient participants and 37 control subjects). 

If you felt frustrated by reading the Tufts article, I strongly recommend that you take the time to read these two other studies. 

Missed That Photo

I've given up on taking pictures of the snow because it just keeps falling around here and the novelty is wearing off. It's very cold and getting icy and is cramping my running-off-in-Goldie style.

Oh, Mother Nature? You can make winter just go away anytime now, mmkay? Yeah...that would be great....

I wish that I would have had Canon in hand yesterday as I watched through the kitchen window at Lulu sniffing around in the snow. She made her way over to the crest of a hill, then.....just jumped off. And when she bellyflopped, made a perfect little snow-doggie print of herself in the drift. For anyone else, I would have called it a snow angel, but then, this is Lulu after all.

I laughed so hard that I think she heard me through the window and came chasing back into the house looking for a treat. She's used to being rewarded simply for responding to my voice by coming to find me. It's bribery, pure and simple, I know.

Worked great for my children too.

She was exhausted by her little extreme snow adventure. It's hard being a schnauzer.

Snowiness

Our whole neighborhood is enjoying the calm before the next snowstorm which is scheduled to arrive this evening. Schools have been cancelled and John has declared today a telecommuting day. I find myself wandering around the house with coffee cup and camera in hand, just enjoying the white and bright.

Awww. I love seeing the kids sled down the hill next to our house.

This is Lulu's designated "schnauzer thawing station" spot. 

Cane Gang

Whew. Yesterday we were almost blown off the top of our little inactive volcano here in the Portland area. I woke up to the wind blasting, snow flying, and a major winter storm alert scrolling across my iPhone.

Now, before anyone accuses me of being a whineybutt, I KNOW that just about everyone in the country has had weirdo weather. My friend Jenny, authoress of Understanding Invisible Illnesses, is in all probability sitting at home on the East coast under a thick layer of ice, and without power and heat.

I feel your pain, girl. Really, I do. We were chatting yesterday and she thought she would do well to hop a flight from Philadelphia over to Portland to escape the weather. Not a good idea, I told her. The weather's getting nasty over here, too. Wah wah wah....

I didn't want to get stuck up on the top of a thousand foot hill without those basic necessities to get us through a blizzard, so early yesterday I headed Goldie into the wind and down the hill to the nearest grocery store. As I pulled into the store parking lot I saw an elderly woman leaning into the wind as she deliberately and carefully made her way to the store. She had thrown her cane into a shopping cart and had draped the top third of herself over the cart to stabilize her as she inched her way along. I caught up to her just as the automatic doors whooshed open and we both stepped into the warmth.

"Ahhhhhh", she said. And laughed. I couldn't help but admire her spunk.

In my rush to beat the brunt of the storm, I had jumped into Goldie without Candy the cane, and by the time I had made my way around the store (while picking up and putting down cookies, ice cream, and chips; whew what a struggle) I was beginning to limp. The elderly lady and I arrived in the check out aisle just about the same time. As she was checking out, the store clerk automatically asked, "Need any help out?"

"NO!" She glared at the poor teenaged kid who only meant well. But it was evident that this was one _ stubborn_ woman. She continued her slow and deliberate journey out into the blustery weather. I followed her and nervously watched her struggle with the heavy car door.

Of course she drove an old enormous sedan. Don't all those feisty elderly ladies drive big old four-doors? I remember having one such gal in my caseload back when I did a brief stint in public health. She would toss her walker and huge oxygen cylinder into the back of her Cadillac and squeal the tires as she ripped out of the assisted living parking lot, her head higher than the top of her dashboard by only a half inch or so. If she thought no one was looking, she'd light up an unfiltered Camel cigarette as she turned her oxygen up a click or two.

Mercy.

But back to today's story.

As I approached the woman's car, I saw her attempting to brace the car door open while reaching for two very large boxes of wine. I couldn't stand it. All I could imagine was this poor thing lying flat on the snowy pavement with a broken hip. And cheap chablis puddling all around her.

Um. Ma'am?

She straightened up and looked defiantly at me.

Um. You know, sometimes I have to use a cane too. It's hard to shop with a cane. Can I put those groceries into your car for you?

Immediately her demeanor changed from one of defensiveness to relief.

"I guess so. Thank you... I try so hard to be independent, but..." she sighed, then appraised my cane-less person. "Well, I'm glad you don't have to use one today!"

Me too!

After I dumped my stuff into Goldie, which was NOT donuts and cookies and coffeecake, yay me, I watched her big boat of a car creep out of the parking spot. Then she gunned the thing and zoomed out onto the street. She waved and grinned as she passed me.

We cane people have to watch each other's back.

This Makes Me Angry

I am sad.

Yesterday, someone stole one of my precious cows from my little Holstein herd. And although two still remain.....it really hurts.

The girls and I go back a long way - May 13th, 2007, to be exact. They were a gift to me from John's sister and her husband. I was delighted to welcome this trio of bovines into our lives.

The girls have been really busy since then. They're rambunctious and completely out of control, which is why I love them. They rarely miss an opportunity to show off for large parties, all decked out in their finery. Holidays, birthdays, wedding showers, football games, you name it. Those three were there.

But now there's only two.

Since they were identical triplets, I'm not sure which one was taken, except that she was wearing a Seahawks outfit. Which means that I take back all the nice things that I have been saying about Seahawks fans since the Super Bowl.

Sniff.

Here's a collection of pictures of the girls, beginning with a photo from the first day they arrived. Of course I have a zillion more, but will post just a sampling.

Our schnauzer at the time was Bart. He didn't like the girls one bit.

The first time they ran away was to attend Greg's birthday party. They had such a good time that from then on, the girls were impossible to keep corralled.

This photo is the last one I have of them all together.

Good bye, my little bovine. I hope you are loved --- wherever you are.

Tufts Study: Despite Burden, Sjögren’s Syndrome May Not Impede Function

Yesterday, I received an email from the associate director of public relations at Tufts University, Boston Health Sciences Campus. (It was one of those moments in which I pointed incredulously at myself and asked, Me? This Important Person wants to share something with ME?) She thought that Tufts University's latest news release may be of interest to Reasonably Well blog followers, and she was right.

However, after reading the press release, the study abstract, and the actual study, I had several concerns as well as one major agreement. I realize that I am NOT QUALIFIED to evaluate or contradict this research. I'm a very retired RN whose only claim to expertise on Sjogren's syndrome is my personal journey with this disease. I have only respect for these researchers who have extensive education and experience in research, oral health, and neuropsychiatric disease.

So with those caveats in mind, what follows is simply a response to this study from a patient's perspective.

The study, Characterization of cognitive dysfunction in Sjögren’s syndrome patients, was conducted by  Lynn C. Epstein, Gina Masse, Jerold S. Harmatz, Tammy M. Scott, Athena S. Papas, and David J. Greenblatt. This was a small study with only 37 patient participants and 37 control subjects.

Let me begin by discussing this from the February 4th  press release from Tuft's University:

BOSTON (February 4, 2014) —People living with Sjögren’s syndrome, an autoimmune disorder characterized by dry eyes and dry mouth, appear to function at a level comparable to their healthier peers, according to a cross-sectional study published online in advance of print in Clinical Rheumatology. The study by clinicians and researchers at Tufts University reveals that people living with Sjögren’s perceive significant decline in cognitive, psychological and physical function. Nonetheless, despite the burdens of the disease, levels of function approach that of healthy controls. Continue reading here. 

This conclusion from the press release:

“Our results suggest that people living with Sjögren’s still are able to maintain a reasonably high level of function, despite their perception of declining function over time. Sjögren’s can interfere with daily functioning and the burden of illness is very real. Nonetheless, it is apparent that even with these interferences people can compensate and function reasonably well,” said co-principal investigator and first author Lynn C. Epstein, M.D., a psychiatrist and clinical professor of psychiatry at Tufts University School of Medicine.

I have to admit that I felt somewhat irritated at this section of the above quote: "....despite THEIR PERCEPTION of declining function over time." This was not a longitudinal study. Study authors would have no data to support their discussion of length of disease related issues.

The subjects of this study were tested extensively in psychological and cognitive health and function; however the physical testing appears to have been based on a large part from self-reported assessment: Fatigue Severity Scale; and The Short Form 36 (SF-36) which is a survey which measures health-related quality of life HRQOL based on 36 items and eight domain scales. I noticed that the assessment of fatigue -- or vitality as termed in this assessment tool -- was located not in the physical health section but rather in the Mental/Emotional locale. There were no physical evaluations of joint pain, pure neurological problems (as opposed to neuropsychiatric), pulmonary issues, renal problems, hepatic problems, gastrointestinal effects, POTS (postural orthopedic tachycardial syndrome) and other dysautonomia symptoms, to name just a few well-documented extra glandular manifestations of Sjogren's syndrome.

And then there's the issue of fatigue.

Since fatigue is one of my most disabling symptoms of Sjs and I'm a bit touchy about labeling fatigue as an all in your head symptom, I found it interesting that elsewhere in the discussion section of this study, fatigue is included under the description of "neuropsychiatric problems":

Neuropsychiatric problems described in association with Sjögren’s syndrome include a variety of psychiatric disorders, alterations in memory, sleep disturbances, cognitive dysfunction,
and excessive fatigue [10–16, 46–48]. The prevalence of these problems in Sjögren’s patients is variable among studies and case series available in the literature. An important clinical and mechanistic question remains regarding the extent to which these neuropsychiatric and cognitive sequelae of Sjögren’s represent actual components of the underlying autoimmune pathophysiology, as opposed to reactions to the stress, burden, and disability related to dry mouth and dry eyes. (Bolding mine.)

Is this evidence of researcher bias leaning toward the Sjogren's is just dry mouth and dry eyes train of thought? I couldn't say. In my personal experience, my disability from this disease is not related to dry mouth and dry eyes. My dry mouth and dry eyes are major inconveniences. I am truly disabled by the extent of my fatigue and joint pain.

However, I was intrigued by the exhaustive amount of testing on cognitive and psychomotor function which included CANTAB testing:

The CANTAB includes the following modules:
a) Psychomotor Coordination and Motor Speed: The Reaction Time Test measures subject's speed of response to a
visual target where the stimulus is either predictable (simple reaction time) or unpredictable (choice reaction
time). This test requires approximately 5 minutes.
b) Reasoning and Planning Abilities: The Stockings of Cambridge task assesses subject's ability to engage in spatial
problem solving. It makes substantial demands on executive function and is sensitive to frontal lobe deficits. It
requires 7-10 minutes.
c) Memory: There are three modules: (a) The Spatial Working Memory module requires that subjects find a blue
token in a series of displayed boxes and use these to fill up an empty column, while not returning to boxes where a blue token has been previously found. Some studies have shown this to be impaired in CFS patients (Joyce et al 1996). It requires 5-10 minutes. (b) The Pattern Recognition Memory test screens visual recognition memory in a 2-choice forced discrimination paradigm and is sensitive to temporal or hippocampal dysfunctions. It requires about 3 minutes. (c) The Spatial Recognition Memory test is a 2-choice forced discrimination paradigm that requires about 2-3 minutes.
d) Attention: There are two modules: The Auemioflal Shift; Intra/Extra Dimensional Shift task is a test of rule acquisition and reversal, featuring visual discrimination and attentional set shifting. It is sensitive to cognitive dysfunction in Parkinson disease and frontal -lobe deficits and requires approximately 5 minutes. The Sustained Attention: Rapid Visual Information Processing module is a visual continuous performance task (vigilance) with a small working memory component. It is impaired in patients with frontal lobe pathology. Its duration is 4 minutes. 

Digit–symbol substitution test (DSST) was also performed:

The Digit Symbol Substitution Task is a well established psychometric test paradigm which is used to measure general cognitive performance. Subjects must match symbols to digits and press the key which corresponds to the digit. The test is designed as a continous performance task in whcih the subject must accomplish as many as possible correct matches within a three minute test period.

The study's evaluation of CANTAB and DSST testing summarized:

Objective testing of information acquisition and recall using the CANTAB procedures indicated a small but statistically significant mean decrement among Sjögren’s patients compared to controls in the free recall score on verbal recognition memory. Otherwise the groups did not differ on other components of verbal recognition memory, or on scores from the paired associates learning or spatial working memory components of the CANTAB.

This is very good news for those of us who are distinctly uncomfortable with our perception of a change in our brains after Sjogren's syndrome enters our lives. But I find it interesting that my experiences with cognitive and memory issues seem to be directly related to my energy stores. If I were tested when feeling rested and have some modafinil onboard, I would guess that my results would vary a great deal from results obtained during a very low-energy period. As a matter of fact, after exhaustive personal clinical trials, I have developed an equation that accurately describes the relationship of my fatigue levels to cognitive ability:

Tired = Stupid

But I digress. Let's move on to other parts of this study that I find very encouraging. I would wholeheartedly agree with this quote from Dr. Lynn C. Epstein, co-principal investigator, especially sections that I have bolded:

“Our results suggest that people living with Sjögren’s still are able to maintain a reasonably high level of function, despite their perception of declining function over time. Sjögren’s can interfere with daily functioning and the burden of illness is very real. Nonetheless, it is apparent that even with these interferences people can compensate and function reasonably well,” said co-principal investigator and first author Lynn C. Epstein, M.D., a psychiatrist and clinical professor of psychiatry at Tufts University School of Medicine.

Here is my take-away from this piece of research: Patients dealing with Sjogren's syndrome are tough and resilient people with an impressive cache of coping tools. While some of us are severely disabled by this disease, the majority of sjoggies lead productive and active lives in spite of the challenges that this disease brings.

It's a very important message that is often overlooked.

I frequently focus on the disabling features of my disease. It's easy to do since my blog is my own personal soap box to blather away about the physical problems that I deal with daily. But what I often forget is the importance of focus and emphasis on my abilities, not disabilities.

Newly diagnosed Sjogren's patients need to understand that the vast majority of these patients lead lives that are changed by Sjs, yes, but those lives for the most part are ones that include a healthy functioning brain, fulfilling careers, supportive family relationships, and an active social life.

This study is a reminder to us all that life as a sjoggie can be challenging, but can also be fulfilling and productive.

And good.

Let's Not Get All Crazy Here, People

Even though I am and always will be a dyed-in-the-wool Green Bay Packers fan, still it's hard not to be happy for our Seattle Seahawks and their win in the Superbowl on Sunday. John and I attended a Superbowl party hosted by some serious Bronco fans. So did our rascally little herd of Holsteins.



I just couldn't stay up through the whole game so we left early. I watched the last quarter from the couch, and when the last second ticked off the game clock, we grinned as we heard celebratory fireworks going off in the neighborhood.

The next morning, the Seattle Times released this very funny (and on target) article that poked some good natured fun at the polite nature of the riots that broke out in Seattle after this their first Superbowl win:

.....Seattle’s general overall politeness (waiting for the crosswalk light to change while “rioting”?) has prompted the #HowSeattleRiots hashtag on Twitter....

My favorite:



You can read the Seattle Times article here; and read all of the #HowSeattleRiots tweets here. 

Yeah. We Pacific Northwesterners are awesome like that.

Walking Sideways

Good thing I wasn't holding my Candlemas candle at the time. 

I am still flummoxed by this vertigo thing. There I was, walking around at church, feeling good, feeling steady, confident in my decision to leave my cane at home, when.....the world suddenly tipped 90 degrees.

I stumbled and teetered but thankfully could grab a pew before I fell flat. Just as suddenly as it hit, the vertigo left.

How does that happen? How can you prepare for that?

Worth the Energy

Ahh. How much fun is a wedding dress shopping trip, I ask you?

Oodles.