Saturday, September 14, 2013

Transitions, White Button Mushrooms, and Clams




I am a fan of the transitional seasons.  This cool air somehow actually is correctly called "crisp." It's hard not to think of the savage weather and nature's backbite elsewhere -- still, the sky here is perfectly blue and the pines sway.  And the sound that has the felines jumping is the same sound I struggle to decipher each year at this time:  the first acorns falling.

Would that we could transition so flawlessly.

Don't worry, I'm not in a moralistic essay mood.  You lucked out today.

However, I have the fidgets.  Dancing legs, jerky hands, twitchy eyes.  It's one of those days requiring a decision:  whether wearing myself out, most likely in the kitchen, for the pleasure of enticing aromas and edible accomplishments will be worth the debilitating pain that will hit around 4 pm and stay a few days.

I'm worried about Fred's ears.  This does Fred, nor Fred's ears, any good at all, and I surely don't need additional fret material.  Still, this is ridiculous.  Way back in March, he lost hearing in his right ear suddenly and was badgered into a visit to the medics.  They saw serious infection in both ears, and prescribed an ineffective antibiotic and some drops.  He spent about six to eight weeks in the "eh?" mode, then got sufficiently better such that he could, in an act of pure machismo, ignore the problem again.  The medics still saw infection but were going with that cutting edge "watch and wait" strategy.  So last week, Fred is suddenly pretty exhausted again, and Lo!  He announces, "I can't hear out of my right ear at all... and I don't feel very well." And you know that's true, because he made an immediate appointment with the doc and did not go to his ukulele band practice.

None of that is why I am worried about Sweet Fred's ears.  It's what the doc said she saw.  "Raw, bloody eardrums." *That* is what worries me.  She's prescribed the same treatment because, I suppose, it was so freaking successful the first time.  I did highly athletic and artistic cheerleading maneuvers when Fred protested the antibiotic and she duly called in a different one.  That's my adorable health care consumer, advocating for himself!  Now, if I could just get His ADHD-Ness to go pick the new antibiotic up from the pharmacy, we'd have it made in the shade, we'd be cooking with gas, we'd be doing the hokey-pokey.  Or something.

Having lived with raging infections and almost constant high levels of inflammation for... well, at least since the inception of this everlovin' blog, I can attest to the fact that persistent infection/inflammation tires a soul out. So I've a case of the Guilts.  Fred has been into calling himself an "old man," daily staring death in the face, etc.  I, of course, have ignored this aberrant and manipulative behavior.  And now must see to it that the poor guy gets the best napping spots, curative foods, miraculous spices, the most angelic cat of the hour, the softest of throws (I am the Queen of Throws), and constant encouragement carefully mixed with adequate commiseration.

Fred is King of Pasta, if I am Queen of Throws.  He makes Scott Conant look like an amateur.  However, part of this Ear Thing has been a propensity for prepared foods, frozen meals, and such -- a propensity helped along by my lack of activity in the kitchen.  If you've read much of my blog, you know that I start most days by loudly whining:  "O dear Lord, I don't feel good." See?  I even give up distinguishing between correct adverbs and incorrect adjectives.  We are talking serious pain, fever, and general... O dear Lord... MALAISE.

In the last two weeks, however, I've made myself prosecute a war against Fred's frozen foods and pre-fab concoctions that contain enough sodium and unpronounceable chemical agents and preservatives to make anyone feel like an "old man." And last night, my Dear Readers, I peaked.

Even when Fred is ill, I make him do the grocery shopping.

Not true.  I can't do the grocery shopping.  So, if Fred would like to eat, Fred must do the market runs. As mentioned, my guy has a full-fledged case of adult ADHD, so these trips can be exacting and frustrating, both for the shopper and the one waiting at home, praying that he adheres to the list.

The Grocery List.  Source of goodness, spawn of evil. I know I've written a few posts about shopping, and since I wrote them, and I am writing this, it's all from my point of view.  Oh, right, I just remembered -- one of my best efforts involved the purchase of a laxative.  That figures. But I've an imagination, and ears (both functional and not in the least bloody), so I know that the invigorating atmosphere of our local mega-store can induce splattered concentration patterns within mere minutes for Fred.  Or, he will concentrate so very well that he enters the twilight zone of hyperconcentration, hyperfocus.  That's when, after leaving with a list of fifteen items, he will return with fifteen versions of one of those items, each with its own peculiar story. Fifteen different kinds of cumin, from seed to ground, from organic to fragrant with pesticide.  The other fourteen items?  Forgotten.

So the art of The List matters.  I try to stick to restocking, which cuts down on the need to choose between brands, sizes, colors, etc.  But there are times when a body wants something different -- lately, for me, it has been root vegetables.  I want beets.  Turnips.  Rutabagas. Celeriac. On that Pyramid quiz show, the correct response would be: "Vegetables you can buy in the produce section of most any grocery store."

But the answer I get is always, "I looked.  They don't have any."

This answer applies to a new brand of shampoo, as well as to tomato paste.  It's the same response when I put down sherry or bay leaves.

Then there is the ENORMOUS problem of Fred's innate sweetness.  I will usually give up on my desired list item after two or three trips to the store, and two or three "I looked and they don't have any." I no longer ask him whether he asked an employee for assistance -- that would be the same as suggesting he ask someone for directions were we ever lost in this massively ill-designed metro area.  So I stop writing it on the list, give up whatever recipe or food hankering was in play, and go back to the basic restocking format.

But then, aha!  Fred will be shopping and suddenly SEE the former list item in question.  In his sweetness, he will then usually purchase every bit of the item that is on display.  It might be three months since I listed it, and while both need and desire for whatever it is has long disappeared, Fred's sweetness raises the importance of the item like poor Lazarus trying to take an unperturbed dirt nap.

I went through a mushroom phase recently.  I was practicing making sweet and savory rustic tarts, and a caramelized mushroom and onion tart is a heavenly thing.  But in the interest of not consuming so many pounds of butter (using Julia Child's pastry recipe) and preserving our lives, my tart-making was put on hiatus.  With certain items, given the economic realities of life here in Tête de Hergé (also rationing, oy!), I often make a parenthetic entry on the grocery list: "buy only if on sale." So it goes with mushrooms.

Okay, enough back-story.

Under the influence, but unbeknownst to him, of bloody eardrums and a raging infection, tired from chauffeuring some sick old woman around to her medical appointments, Fred made a run to the store.  The old woman had given the old man a short, restock sort of a list, because she was cognizant of his fatigue, and does love him.

But the Evil Produce Manager decided to put all of his freaking mushrooms on "Manager's Special." Not just "regular" special, "Manager's Special." Super-duper special.  And so it came to pass that our refrigerator was filled to the brim with mushrooms, all, as they say, "on the verge..."

It's a wonderful food,  the mushroom.  I just consulted a nutrition site for info about raw white button mushrooms (the exotic type with which we insulated the fridge) and read:

This food is low in Saturated Fat and Sodium, and very low in Cholesterol. It is also a good source of Dietary Fiber, Protein, Vitamin C, Folate, Iron, Zinc and Manganese, and a very good source of Vitamin D, Thiamin, Riboflavin, Niacin, Vitamin B6, Pantothenic Acid, Phosphorus, Potassium, Copper and Selenium.
Now, really, isn't that grand?

Oh, hell, there's more to the back-story.  This is commonplace, I believe.  It's like the war about how to properly hang toilet paper.  In this instance, of course, I mean, do you wash your mushrooms, clean them in some other fashion, or just use the suckers as they come, removing the obviously gross, such as slime and gobs of dirt?  I despise a wet mushroom and opt for the paper towel method of vaguely wiping at them. Fred, dear heart, is phobic.  Wash them, scrub them, and don't you dare use those nasty stems!  It's an argument I cannot win, especially if he watches me cook.  (I've gotten used to gorging on mounds of amputated stems while he makes sickening gagging noises.)

Yesterday was one of those days.  Our beloved neighbor, Richard, still high from adding a male grandchild to his collection of six girl grandbabies, came banging on our door in search of help starting his car.  Richard, like everyone we know, cannot fathom our sleeping habits, mostly because we have none.  It was about 8 am and Fred had gone to bed at about 6:45.  I was bebopping around, denying a 100+ fever at that time of day and converting temperature points into domestic energy. So after warning Richard that Fred might be a bit... dim, I woke the poor guy up and off they went to do guy stuff.

The thing about Fred, and me, too, and perhaps it's true of you -- once wakened?  We're up. So with his hour's worth of sleep, he made coffee and I indulged in my second SuperMug-sized Italian roast.  We did doff our hats to sleep, out of respect, by drinking the coffee in bed, while having a stimulating discussion of "What We Can Do With Mushrooms Today."

The wise among you are probably shouting about how nothing makes a better "new boy grandchild" gift than thirty pounds of white button mushrooms, on the verge.  Well, we're just not as sharp as you are: Harrumph.

Fred finally fell asleep, fell being the operative word, as I witnessed the weaving pattern he made just before collapse.  Competely wired, I began to make the world's largest batch of spaghetti sauce.

It turned out to be a thing of beauty.  We had no tomato paste, of course, that being one of those items that are no longer carried in the modern grocery... so I worked with some of the wondrous dehydrated tomatoes that Brother-Unit Tumbleweed supplies by mail, as well as several varieties of canned 'maters.  Through years of List Training, I have a great selection of either no-sodium or low sodium unseasoned diced tomatoes.  That way, if there is any screwing up of the tomatoes, it's on me.  Don't get me started on the amount of sodium in canned goods, and the pre-seasoning, in general, that has run so rampant as to ruin the fabric of this country...

I decided to opt for doing more of a traditional "gravy," slow cooked, layer upon layer of flavor.  Nutty garlic, sweet onions, so on and so forth.  And a ton of chunky-chopped shrooms.  I showed the mushrooms the water, grabbed a paper towel for looks, and pretty much just rough chopped them as they came.  No slime, minimal dirt.  I even minced the stems so nicely that they'd impart great flavor but not be visible to the naked eyes of this old woman and her old man.

Things may have gotten a bit out of hand.  I know that simple is best.  I know that less is more.  But I was febrile and trying to balance a very unbalanced sauce.  It was, you see, a bit heavy on the mushroom side of the flavor profile.  Out came the sherry and some exotic vinegars.  A bit of shredded this, a touch of sugar.
And then... in the back of what I consider the Canned Tomato Cabinet, I found a huge can of clams.

I hate clams.  But Fred loves them!  And considers their liquor divine.  So once I had balanced earthy, sweet, tart, and various kinds of spices, and the "gravy" had married it all together well... I turned off the blessèd flame and added the can of clams, mixed it all well, a big smile on my prednisone moon face, and covered it to let the clams do their thing unperturbed.

Just as I washed the last dirty dish and pot, Fred got up (again).  That's an amazing phenomenon, one worthy of scientific inquiry.  I finish vacuuming, and Fred says, "Is there anything I can do to help?"  I finish laundry, and Fred says, "Can I swap that load from the washer to the dryer for ya?"  Funny, though, that the tendency will lapse after instances where my brain explodes all over the ceiling.

But I was happy, so we made more coffee.  The First Lady would be proud of our fluid consumption, and I know my cardiologist would have adored my heart rate.

As Fred is Pasta King, a few hours later he made the pasta.  By which I mean he boiled the dry pasta until it was perfectly al dente and sufficiently salted to destroy my sodium-sparing efforts.  We each had two heaping bowls and declared it a good thing.

The troubles began about an hour later.  Not for me, just for Fred.

And, of course, it had to be the unwashed mushroom, and those minced stems.  I have, evidently, built up an immunity to mushroom detritus over the many years I've been munching on that earthy delight.

Fred never blamed me, or the mushrooms.  He was too weak.

But I think it could have been the clams.

On the up side of things, Fred didn't mention his ears for the rest of the night.  And when he went to bed, he stayed there, zonked.  He is still there, in fact, and it is now 1 pm.

There are leftovers, lots of leftovers.  And I have a feeling they are mine, all mine.  Fred is likely to insist on prepackaged, frozen foods, and may even eat alone in his office.

What am I going to do with the rest of these mushrooms?

Enjoy the beautiful day, as I hope you are beneath the same delicate china sky, surrounded by the same crisp air.



© 2013 L. Ryan

Friday, September 13, 2013

ORANGES and SARDINES

Sardines, Mike Goldberg, 1955




found this on the always inspiring american idyll -- for which i'd be a pimpless blogwhore, even if its organizing genius were not kin.

as the marlinspike hall household is now clearly divided into the visual artistry and music of the fredster, and my own overwrought wordsmithing, this very much appealed to me as being... accurate:

I am not a painter, I am a poet.
Why? I think I would rather be
a painter, but I am not. Well,

for instance, Mike Goldberg
is starting a painting. I drop in.
"Sit down and have a drink" he
says. I drink; we drink. I look
up. "You have SARDINES in it."
"Yes, it needed something there."
"Oh." I go and the days go by
and I drop in again. The painting
is going on, and I go, and the days
go by. I drop in. The painting is
finished. "Where's SARDINES?"
All that's left is just
letters, "It was too much," Mike says.

But me? One day I am thinking of
a color: orange. I write a line
about orange. Pretty soon it is a
whole page of words, not lines.
Then another page. There should be
so much more, not of orange, of
words, of how terrible orange is
and life. Days go by. It is even in
prose, I am a real poet. My poem
is finished and I haven't mentioned
orange yet. It's twelve poems, I call
it ORANGES. And one day in a gallery
I see Mike's painting, called SARDINES.

--Frank O'Hara
Why I Am Not A Painter

Frank O'Hara by Alice Neel, 1960
The Paris Review
**********************
**********************

and i permit myself the luxury of adding one of my favorites in the ut pictura poesis game.  i am to be forgiven, as it was the basis for my thesis, though i was then confined to master diderot strolling from one gilt frame to another in the salons (1759 through 1771, 1779 and 1781).

The Painter
BY JOHN ASHBERY

Sitting between the sea and the buildings
He enjoyed painting the sea’s portrait.
But just as children imagine a prayer
Is merely silence, he expected his subject
To rush up the sand, and, seizing a brush,
Plaster its own portrait on the canvas.

So there was never any paint on his canvas
Until the people who lived in the buildings
Put him to work: “Try using the brush
As a means to an end. Select, for a portrait,
Something less angry and large, and more subject
To a painter’s moods, or, perhaps, to a prayer.”

How could he explain to them his prayer
That nature, not art, might usurp the canvas?
He chose his wife for a new subject,
Making her vast, like ruined buildings,
As if, forgetting itself, the portrait
Had expressed itself without a brush.

Slightly encouraged, he dipped his brush
In the sea, murmuring a heartfelt prayer:
“My soul, when I paint this next portrait
Let it be you who wrecks the canvas.”
The news spread like wildfire through the buildings:
He had gone back to the sea for his subject.

Imagine a painter crucified by his subject!
Too exhausted even to lift his brush,
He provoked some artists leaning from the buildings
To malicious mirth: “We haven’t a prayer
Now, of putting ourselves on canvas,
Or getting the sea to sit for a portrait!”

Others declared it a self-portrait.
Finally all indications of a subject
Began to fade, leaving the canvas
Perfectly white. He put down the brush.
At once a howl, that was also a prayer,
Arose from the overcrowded buildings.

They tossed him, the portrait, from the tallest of the buildings;
And the sea devoured the canvas and the brush

As though his subject had decided to remain a prayer.

Thursday, September 12, 2013

Dear Phan Thi Kim Phúc: Your Thoughts?

Nasty people provoke a nastiness in me.

I'm not proud of it, and have spent much of my life both regretting it and trying to change.  Although aging and fatigue are not valid reasons to cease trying to become an extraordinarily forgiving and non-snarky person, that's the only petition I can offer the court.

Adam Sorkin tweeted something today that ought to be the theme song of many lives, and which makes me laugh, at least, in the midst of my leftist, materialistic, smartass grinchiness:

–I will not be the subject of your mockery. 
–Oh, I think you shall.

I've been thinking, even against my will, about Syria.  My active will has been doing as much reading about Syria and the "situation" there as my weepy, waxing-and-waning-in-visual-acuity eyes will allow.  And then, of course, there are dreams.

An excellent beginning article comes from scholar Juan Cole's attempt at an independent-minded news analysis blog, Informed Comment:  "Top Ten things Americans need to Know about Syria if they’re going to Threaten to Bomb It."  I'd never heard the quote he leads with, and appreciated the addition of more Ambrose Bierce in to my muddled Devil's Dictionary of a mind -- “War is God’s way of teaching Americans geography.”

Of course, Juan Cole most likely doesn't have the refrain "Nothing matters" as the running backbeat to that bit of Bierce irony.

I'm a hawk on this one.  A nasty hawk.

Perhaps it's that I know and trust in USAmerican air power and ability to surgically strike.  Obama was right when he said the "US doesn't do pinpricks."  It's the understanding of how disabling an air strike will be that has al-Assad issuing cocky demands today, via Lakhdar Brahimi, the U.N.-Arab League envoy for Syria, and Russian Foreign Minister Sergey Lavrov.  The proposal that Russia has fabricated is going to be, my omniscient self foresees, woefully inadequate.  It certainly ought not be the basis for a war criminal's demands.

Ouch.  

That's right, I shock myself whenever I toss around that label, knowing that the USA is signatory to the League of War Criminals. I could also argue that any war, any military action, is criminal.  But reductio?
That's arguing for wimps.

Or that's arguing for the angelic.

I don't fall into either category, though I've wimped out often enough.  Still, neither wimpiness nor halos are basic to my character.

I dream of napalm and agent orange.  I dream about 9-year-old Phan Thi Kim Phúc and Nick Ut's iconic photo.  

She lived, and now lives in Canada, where she has the gall to say such things as: 
Forgiveness made me free from hatred. I still have many scars on my body and severe pain most days but my heart is cleansed. Napalm is very powerful, but faith, forgiveness, and love are much more powerful. We would not have war at all if everyone could learn how to live with true love, hope, and forgiveness. If that little girl in the picture can do it, ask yourself: Can you?
-- Kim Phúc, NPR in 2008

No, Kim Phúc, I can't.

Because the iconic photos keep rolling in.  Because you survived, in large part, not by love, hope, and forgiveness, but by the actions of Nick Ut in getting you to a hospital.  Because someone (guided by human decency, yes) DID something.

At least I can definitively give Bierce's guiding "nothing matters" a resounding "ef-off" at this point in life. Sadly, though, and in high contrast to most of my dearest friends and those relatives of mine who actually think about things beyond what they will inherit, I also give the Grand Whiff-Off to platitudes that impart worldly usefulness to notions of personal improvement.

Of course, I've no idea what Kim Phúc thinks of the gassed dead in Syria, particularly the children.  She may be a stern master, an avenging well-armed angel.  I don't know.  


22 August 2013, Duma neighbourhood of Damascus, REUTERS

South Vietnam, 9 June 1972 | Nick Ut






Wednesday, September 11, 2013

CRPS-ers! What would you do, if you could do anything?

From National Pain Report, 9/9/2013Originally published on Casey Mullins' blog Moosh in Indy, 8/29/2013

Sheila and Dave
September 9th, 2013 
I had never heard of Complex Regional Pain Syndrome (also known as Reflex Sympathetic Dystrophy or Causalgia) before my wife Sheila was diagnosed with it earlier this year.
In April of 2013 her big toe went numb for no apparent reason. The numbness, accompanied by weakness, quickly spread up her leg until her entire right leg below the knee was rendered useless. Within about 10 days the pain set in.
She was diagnosed with CRPS in May and she passed away on July 29th, 116 days after her toe first went numb.
CRPS is a rare nerve condition that often goes undiagnosed for months or even years. Symptoms include abnormal sweating, changes in skin temperature and color, softening and thinning of bones, muscle twitching and tremors, wobbliness, falling, visionary disturbances and severe pain. Sheila suffered from all of these.
We’ve all experienced pain. It is part of being human and it serves a very specific purpose most of the time. CRPS inflicts a level of pain that most of us can’t comprehend and will never experience.
The McGill Pain Index ranks CRPS as the highest level of pain possible for any chronic condition. CRPS ranks significantly higher than cancer, fractures, unmedicated child birth and even amputation of a digit. The pain from CRPS never goes away and pain medications are almost useless.
The pain my wife experienced was so severe it prevented her from wearing shoes, socks or pants. She couldn’t submerge her leg in water and even a light breeze could bring her to tears.
Initially my wife would try to explain to people how bad her pain was. People were sympathetic, but they really didn’t understand. There was no effective way for her to accurately explain the amount of pain she was in. Her leg would appear red and swollen at times, but usually looked to be relatively healthy.
A former roommate of ours even accused Sheila of faking her condition in order to get workers compensation, even though compensation of any kind was nothing that she ever pursued.
Sheila eventually stopped trying to get people to understand. She just put a smile on her face and tried to hide her pain from everyone.
It is estimated there are between 1 and 3 million people in the United States who suffer from CRPS, 75% of them women. The majority of victims develop the disease in their 30′s and 40′s, but it can hit anyone at any age.
CRPS is usually preceded by an injury or some kind or surgery. There is no cure for CRPS and treatment results vary greatly from patient to patient. Sheila’s form of CRPS was one that did not come with a favorable prognosis. We tried a number of treatments and nothing seemed to bring her relief.
My wife’s doctors, and there were many of them, didn’t know what to do. Some of them had heard of CRPS, but most had no idea how to treat it.
We had read of promising results using the drug ketamine. We specifically asked for it, but when we finally found a doctor that agreed to treat her with it, our insurance company refused to help with the expense. Ketamine is used as a recreational drug and it’s very difficult to find doctors that will use it and insurance companies that will pay for it, despite reports of positive results.
They instead handed out narcotic painkillers like they were candy. Our insurance company had no problem paying for these.
On Monday, July 29th I came home from work and found my wife unresponsive on our couch. She had passed away in her sleep from an accidental prescription drug overdose. She was 32 years old.
Sheila and I were very active before she got sick. We completed many half-marathons together, mountain biked, snowboarded and were at each other’s side on a variety of other adventures. Sheila was a young, vibrant, healthy, beautiful wife and mother of two daughters. She was in the prime of her life.
When asked what she would do, if she could do anything, Sheila said…
“I would run! I would run as far as I could, to the middle of nowhere, and then run home. Or, I would ride my mountain bike in Moab, all day, everywhere possible. Or I would swim. I’d swim laps until my muscles were so weak I couldn’t stand.”

Open Access CRPS Research: BMC Neurology - "Partial CRPS of the hand..."

Diagnosis of partial complex regional pain syndrome type 1 of the hand: retrospective study of 16 cases and literature review

Michel Konzelmann1*Olivier Deriaz2 and François Luthi1

1Department for musculoskeletal rehabilitation, Clinique romande de réadaptation suvacare, 90 avenue du grand champsec, Sion 1951, Switzerland
2Institut de Recherche en Réadaptation (IRR), Clinique romande de réadaptation suvacare, 90 avenue du grand champsec, Sion 1951, Switzerland
For all author emails, please log on.

BMC Neurology 2013, 13:28 doi:10.1186/1471-2377-13-28

The electronic version of this article is the complete one and can be found online at:http://www.biomedcentral.com/1471-2377/13/28

Received:2 July 2012
Accepted:11 March 2013
Published:18 March 2013
© 2013 konzelmann et al.; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background

The partial form of the complex regional pain syndrome of the hand type 1 (CRPS 1), involving only 1 to 3 fingers, is a rare condition first described in 1972. The aim of the study is to define more precisely the diagnosis workup and the prognosis of this clinical entity.

Methods

Retrospective study of CRPS1 partial form observed during five years in a rehabilitation ward. Application of The Budapest criteria, evaluation of radiological exams, therapeutic results and vocational outcomes. Comparison with cases from literature review.

Results

132 patients were hospitalized with the diagnosis of CRPS type 1 of the hand. 16 partial forms were isolated: 11 men, 5 women with a mean age of 43 years. Among these patients, 14 (88%) met The Budapest criteria and the two remaining cases were diagnosed by using the three phase bone scintigraphy. Only moderate improvement was obtained in the majority of the patients. At the maximal time of follow-up (4 to 9 years), 50% of the patients hadn’t returned to work. From the literature review, 19 cases were eligible for clinical comparisons. The main differences between our series and the literature were: more men involved, later diagnosis and worst prognosis in term of return to work.

Conclusions

This is the largest series of consecutive partial form of CRPS. The Budapest criteria are sufficient for the diagnosis in 88% of cases. As in complete form of CRPS1 of the hand, three phase bone scintigraphy should only be used in doubtful cases in the first six months of the illness. Partial form of CRPS1 of the hand is rare and its prevalence remains unknown. Long term prognosis (4 to 9 years) is poor in our series, 50% of patients didn’t returned to work.
Keywords: 
CRPS1; Partial form; Hand-Budapest criteria

Background

Complex regional pain syndrome type 1 (CRPS1) most commonly involves the extremities and the hand in particular [1,2]. The clinical form that involves the whole hand is the most familiar. Diagnosis is generally straightforward. Partial forms involving one to three fingers have been described. Dammann [3] was the first to report three cases in 1972 which he called “fingers isolated Sudeck’s syndrome”. In 1977 and 1979, Lequesne et al. [4,5] described a form of partial algodystrophy termed “radial”, which follows a metameric topography on one or two rays of the hand or foot. Since then, a dozen articles have been devoted to the subject [6-16]. However, various diagnostic criteria were used in the literature and no clear diagnosis process for partial CRPS of the hand was given. The aims of our study are to present this clinical form in a patient population with CRPS1 of the hand, to assess whether the criteria developed by Harden et al.[17,18], the so called Budapest criteria, can be applied to this particular form and to define whether the various radiological examinations are still useful in order to propose an accurate diagnosis process for this rare entity. The results of this series are also compared to the data obtained from a literature review.

Methods

Population and diagnostic criteria

This is a retrospective single-centre study of patients admitted to a tertiary rehabilitation centre between January 2004 and December 2009. This study was approved by the regional medical ethics committee (commission cantonale valaisanne d’éthique médicale) with the reference number 043/07. All the patients signed an informed consent form for their participation and publication of images. The majority of patients were admitted to our hospital at the request of the leading Swiss accident insurer. Most patients were men employed in industry and the building trade with persistent deficiencies as a result of an industrial, road traffic or sporting accident. Patients with a partial form involving a maximum of 3 rays [15,16] were selected from those with complete CRPS1 of the hand. All our patients satisfied the French criteria for CRPS1 (algodystrophy) [1]. These criteria were in used in our hospital before Budapest’s criteria. The other inclusion criteria were: age between 18–65 years, no invalidity pension, no central nervous system lesion or significant lesion of the peripheral nervous system (CRPS type 2). The criteria developed in Budapest [17] for CRPS, validated in 2010 [18], were used and applied retrospectively to our series through the computer medical record which prospectively collects clinical data since 1999. The socioeconomic and other clinical data were also collected from this system. CRPS was treated according to current guidelines [19,20].

Radiological data

The radiological data were gathered from available examination reports and images. For standard radiography, an anteroposterior view of both hands on the same film was used as the reference image [21]. The radiographic features of CRPS have been described in detail by Doury [1]: diffuse or peri /juxta-articular demineralisation, mottled demineralisation, predominantly subchondral bone resorption, endosteal resorption of the intracortical bone which may occur in combination according to the stage of CRPS. These signs and their localisation were noted for each file. For three-phase bone scintigraphy (TBPS), the scintigraphic classification of CRPS into 3 stages [22-29] was used: stage 1, early increased vascular and tissue perfusion and early and delayed bone hyperfixation; stage 2, absence of early hyperperfusion and delayed hyperfixation and stage 3, early tissue hypoperfusion, normalisation of bone fixation. The criteria described by Schürmann et al. [30] were used for magnetic resonance imaging (MRI): bone marrow oedema in a spot pattern of the carpal bones, subcutaneous oedema, subcutaneous and/or synovial contrast uptake during Gadolinium injection and joint effusion.

Evaluation of the results of treatment and of course

During hospital stay, pain severity was assessed on admission and discharge using a visual analogical scale (VAS) from 0 to 100 mm (0 = no pain; 100 = the worst possible pain) [31,32]. A beneficial treatment effect on pain was considered to be present if the baseline value was reduced by at least 30% between admission and discharge [31]. Hand function was assessed using the Disabilities Arm Shoulder and Hand (DASH) questionnaire at the start and end of the hospital stay[33]. A reduction of at least 12.75 points on DASH between the start and end of the hospital stay defines the minimum detectable change of the DASH, that is to say the change observed which cannot be attributed to measurement error with a 95% confidence interval [33]. The perceived overall efficacy of treatment was also assessed on the basis of an external criterion [31]. The patient completed a VAS of overall treatment efficacy at discharge (0 mm = no effect; 100 mm = the greatest possible beneficial effect). The patient was considered to be improved if an efficacy of more than 30 mm on the VAS was detected, which corresponds to the smallest clinically significant change [31]. The results of these measures (VAS, DASH, overall treatment efficacy) are presented as the mean and percentage of patients considered being responders. The outcome at the maximal follow-up (4 to 9 years after hospitalization) was evaluated with the insurance records.

Literature review

The articles in English, French and German published between 1970 and 2012 that dealt with partial CRPS 1 were searched with Pubmed and the reference lists of the articles identified. The following key words were used: algodystrophy, reflex sympathetic dystrophy, Sudeck’s atrophy, transient osteoporosis, CRPS, partial, segmental, hand. Exclusion criteria were as follows: review articles, articles in a language other than French, English and German, articles without a useful description of the cases and articles on CRPS type 2.
The following data were compared with the cases reported in the literature: sex, age, trigger factors, diagnostic delay, number of fingers affected, symptoms, clinical signs, standard radiography, TBPS and evolution.

Results

Population and clinical criteria

Tables 1 and 2 summarizes the data. In the study period, 132 patients were admitted with the diagnosis of CRPS of the hand. Of these 132 patients, 16 cases of partial forms of CRPS involving three rays at the maximum were selected. All fulfilled the French criteria [1]. The cases involved 11 men and 5 women with a mean age of 43 years, admitted for 30 days on average. Of these 16 cases, 14 fulfilled all the Budapest clinical diagnostic criteria [17,18]. With regard to the criteria (see Table 2), 4 symptoms were present in more than 50% of our patients: continuous pain, reduced mobility, hyperaesthesia/allodynia and oedema. Six clinical signs were also present in more than half: reduced mobility, change/asymmetry in colour, trophic disorders, change/asymmetry in sweating, hyperaesthesia/allodynia and oedema. Motor dysfunction was observed in 4 patients: exclusion of the thumb from function with thumb-in-palm (2 cases), thumb in permanent extension (1 case) and permanent reducible passive flexion in 4th and 5th fingers (1 case) (see Figure 1). During the hospitalization, two motor dysfunctions improved and the two others didn’t.
Table 1. Description of the two populations: demographic characteristics, triggering factors, initial injuries and outcome
Table 2. Clinical and radiological characteristics of the two populations
thumbnailFigure 1. Clinical aspect:23-year old female patient, contusion of the hand, development of pain and attitude of contracture that was partly reduced in the 4th and 5th fingers of the left hand.

Radiological examinations

The data are summarised in Table 2. Standard radiography of both hands in the anteroposterior view was performed on the same film in 14 patients (88%). Radiography showed localised demineralisation of the affected ray(s) in 6 patients (43%). The demineralisation appeared mottled in 2 cases only. TBPS was performed on the 16 patients, but was available for 15 only. It was performed 220 days (median 155 days) on average after the initial accident. TBPS was performed during hospital stay in 87% of cases by the same specialist. In 12 cases (80%), TBPS supported the presence of CRPS. When it was performed before 6 months (53% of cases), six cases of stage 1 and two of stage 2 were found. When TBPS was performed (7 cases) more than 6 months after the trauma, the images did not support CRPS (3 cases) or were consistent with stage 2/3 or 3 (2 cases). In only 2 cases there was a suggestion of stage 1 or 2. In the two patients who did not meet the Budapest criteria for CRPS, TBPS was clearly in favour of CRPS stage 1. The MRI of the hand, performed 4 times, provided no evidence to support CRPS in 3 cases out of 4. In the last case, carpal oedema was associated with synovial thickening causing contrast uptake, 1 month after the accident, compatible with incipient CRPS.

Evaluation of treatment’s results and evolution

The pain on a VAS at admission and discharge was available for 14 of the 16 patients (88%). Mean pain on the VAS on admission was 55 mm and on discharge was 43 mm. Seven patients (47%) showed at least 30% improvement compared to the admission VAS (from 59 mm to 29 mm on average). The DASH questionnaire on admission and discharge was available for 12 patients with a mean score of 55/100 on admission and 51/100 on discharge giving a mean reduction of −4 points. Only 3 patients (25%) had an admission/discharge difference greater than 12.75 points. The VAS “patient beneficial treatment effect” was available for 14 patients (mean of 59 mm). In 11 cases (79%) it was greater than 30 mm (from 37 to 100 mm). With regard to professional activity, 4 to 9 years after the hospitalization, 50% of patients hadn’t returned to work, but we didn’t know the exact reason. 44% returned to the same job or an adapted job. One patient had an invalidity pension because of another injury (severe cranial trauma). The mean compensation duration was 202 days before and 463 days after hospitalization.

Literature review

Fourteen articles were identified [3-16]. Half of these were excluded [4,5,7-9,14,15]. Two articles presented case series but without details of the clinical findings and thus could not be used in this review [8,15]; four articles were about CRPS type 2 [4,5,7,14] and the case in the last article was not convincing [9]. Seven articles were finally included [3,6,10-13,16] involving 19 cases. The comparative sample consists of these articles. The diagnostic criteria used, when given, varied (Doury, Amadio, Veldman) and no study applied the Budapest criteria. The diagnosis of CRPS was assessed on the basis of the clinical and radiological descriptions in the articles. The literature data are summarised in Tables 1 and 2. Compared to our series, there were more women and 75% of cases were post-traumatic in origin. Mean diagnostic delay was much shorter (2.7 months versus 7.5 months). The main symptoms were pain, hyperaesthesia/allodynia and oedema. The clinical signs present in more than 50% of cases were reduced joint mobility, hyperaesthesia/allodynia and oedema. Standard radiography was performed in 58% of the cases and demonstrated demineralisation of the affected ray(s) in 72% of cases. TBPS was performed in 52% of cases. It was always considered to support the diagnosis of CRPS, but only data from the delayed phase were described. No data on MRI were available.
With regard to evolution, 8 patients (42%) were cured and 6 (31%) improved with an extension of 6 to 12 months. Two patients were in permanent disability at 1.5 and 9 years.

Discussion

This series is the largest well-documented consecutive series of partial CRPS 1 of the hand. It is also the first to use the recently validated diagnostic criteria, the so called Budapest criteria[17,18], in this context. Among our 16 patients, 14 (88%) fulfilled these criteria and in the two remaining cases, where just one symptom was missing, a TBPS enabled the diagnosis to be confirmed. Validated criteria accepted by the majority of the medical community make the comparison of studies and grouping of data from small series possible, which facilitates the advancement of knowledge in rare diseases. It should be kept in mind that there is still no consensus in the literature on the number of rays that define the partial form of CRPS. Doury and Lequesne et al. [1,4,5] proposed that one or two fingers at most must be affected. However, Soucacos et al. [15] and Bianchi et al. [16] proposed three fingers. Based on our experience, we propose to apply the diagnosis of partial CRPS when one to three rays are affected and when the disease does not spread to the whole hand later as part of overall CRPS progression. Some authors discussed a neurological origin of partial forms of CRPS [6,12,13,16] but they gave no evidence to support this hypothesis.
In these partial forms of CRPS of the hand, special importance should be given to differential diagnosis which meets the Budapest criterion n° 4 [17,18] i.e. that no other diagnosis may better explains the signs and symptoms. For the hand, many other diagnoses should be excluded[1,4,5,10]. Radiological examinations may therefore remain useful especially for doubtful or borderline cases. It would be appropriate then to specify their importance when partial involvement is suspected.
Based on the available data, standard radiography and MRI have limited value. They should not be performed for confirming partial CRPS of the hand, but only for differential diagnosis [21,29,30].
TBPS has been used since the early eighties in CRPS for the upper limb [22-27], but still not for the partial forms of CRPS. A review article published in 1995 [28] concluded that TBPS was the most useful for positive diagnosis of CRPS when performed within the first 6 months of the disease. In another hand, in Dutch guidelines published in 2006 [34], TBPS was considered to have no additional diagnostic value. Since the publication of these guidelines, two meta-analysis were recently published [29,35] with contradictory results. Moreover, 3 prospective studies have been also published [26,27,30] confirming the usefulness of TBPS as additional tool in doubtful cases[30] and when the duration of the pathology is shorter than 3 [26] or 5 months [27]. Two other retrospective studies [36,37] using the Budapest criteria were also recently published. Moon et al.[36], found a low utility of TBPS for diagnosis of CRPS and AlSharif et al. [37] found a positive scintigraphy in patients with vasomotor symptoms, motor and/or trophic changes, with a duration of less than 3 months. In our study, TBPS was also particularly helpful when it was performed in the first 6 months. Figure 2 shows the TBPS images in a partial form. Taking all these results together, the role of TBPS in the diagnosis of CRPS is still uncertain. It is certainly not a screening tool for diagnosis of CRPS in which clinical findings remain the gold standard. Nevertheless, we believe that TBPS should be only recommended in the first months of the disease progression for unclear situations which do not fully meet the Budapest criteria [29,30]. Based on the present knowledge, we have proposed a diagnosis flow chart (Figure 3) on the application of the Budapest criteria and of radiology in cases of partial CRPS.
thumbnailFigure 2. Three-phase bone scintigraphy: early phase (a) and delayed phase (b) Same patient as Figure 1. Staged early and delayed hyperfixation on 4th and 5th fingers suggesting CRPS stage 1.
thumbnailFigure 3. Partial CRPS type 1 of the hand: proposed diagnostic flow chart.
The disease course, reported briefly in the literature, is described as favourable in the majority of cases (see Table 1), but return to work is addressed in only 2 patients (10%). In relation to our patients, progress during their hospital stay was modest. Nearly half of our patients (47%) reported improvement of at least 30% in pain, which is considered the smallest clinically significant change that could be detected [31]. The patients’ perception of global improvement was more than 50 mm and can be defined as clinically significant change (i.e. more than 30 mm) in 80% of patients. This more global parameter probably reflects patient satisfaction with the whole interdisciplinary rehabilitation process. Regarding return to work, in our series, 50% of patients didn’t return to work with a follow up of 4 to 9 years. We cannot explain this long lasting sick leave but none of our patients had an invalidity pension related to CRPS. The literature is not very precise, especially concerning return to work, and to date our study is the first to use detailed insurance data. Hence, it could happen that old studies may have been more optimistic than those performed more recently [1,38]. But it is also possible that a too late diagnosis, as in our cases, may have had a negative impact on healing, especially on chronic pain. Finally, it could not be excluded that cases with favourable outcomes were not sent to our centre.
Compared to the results of literature, our patients are all post traumatic with a higher prevalence of males. This is due to the selection of our patients, i.e. most of them were blue collars (industry and building workers) insured to the leading Swiss insurance company which owns our clinic (selection bias). Then the diagnosis of CRPS was made more belatedly and the prognosis seems worse in our series. This probably due to the fact that this syndrome is rare, ignored by the majority of medical practitioners and discovered in specialised wards [3-16]. In our medical environment, traumatic patients are only sent to tertiary centres in case of unfavourable outcome after months what probably explains this late diagnosis (202 days on average).
Different diagnostic sets were used in the literature. This may also affect comparisons with our series [39,40]. Nevertheless, with the exception of motor dysfunction, only recently introduced in CRPS criteria, all the other symptoms and signs were somehow already mentioned in former classifications and used in hand rehabilitation facilities for many decades [1,2]. Moreover, we have only kept studies with precise clinical description. For these reasons, we assume that all these cases are partial form of CRPS. Computer medical records are very helpful to trace these criteria. In our hospital, for instance, clinical data are prospectively recorded and were used as database for all CRPS cases since 2002 for the lower limb [41] and 2004 for the upper limb with only few missing data.
The first limitation of our study is its retrospective nature. With this design, we can’t exclude a placebo effect to explain a part of therapeutics results [42]. Because symptoms can also fluctuate over time [40], it is also possible that retrospective design with only one assessment may present a low precision of the measurement. The third limitation is related to the selection bias i.e. mostly blue-collar male patients with unfavourable outcome are hospitalized in our clinic (see above). For these reasons, our results cannot be generalised to all cases of partial CRPS. Finally, the literature review remains limited by the diversity of diagnosis criteria, but the articles were carefully selected with precise clinical description and were assumed to be partial forms of CRPS.

Conclusions

In conclusion, partial CRPS type 1 of the hand is a rare clinical form. For clinical practice we recommend the use of the Budapest criteria validated in 2010 [18], with a maximum of 3 rays involved without subsequent spread. Only, in case of doubt or borderline form (10 to 20% of cases), TBPS should be performed in the first six months of the disease course after trauma, other radiological examinations being devoted to differential diagnosis above all. In our series, the prognosis is poor, 50% of patients didn’t return to work 4 to 9 years after hospitalization.

Abbreviations

CRPS: Complex regional pain syndrome; TBPS: Three bone phase scintigraphy or scan; MRI: Magnetic resonance imaging; VAS: Visual analogical scale; DASH: Disabilities arm shoulder and hand; IASP: International association for study of pain.

Competing interest

The authors declare that they have no competing interest.

Authors’ contribution

MK extracts the patient’s data, analyzes it, wrote the article, and made the tables and figures. OD participated in the redaction of some sections of the revise manuscript in English. FL participated in the redaction, correction and review of this article. All authors read and approved the final manuscript.

Acknowledgments

To Mrs Viriginie Crittin for her help with the tables
To Mr Pierre-Dominique Varone for the long term insurance’s data.

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