thesearethefacesoflupus

Where Does It Hurt? Peripheral Neuropathy and SLE

Where Does It Hurt? Peripheral Neuropathy and SLE

By A. G. Moore 12/21/2013

Cutaneous nerves in the sole (foot)
From Gray’s Anatomy
Diagram drawn by Mysid
Gray’s Anatomy is in the public domain because of copyright expiration

Ask someone with active lupus the question, “Where does it hurt?” and the reply may be, “Just about everywhere”. Pain, with lupus and many other chronic conditions, becomes a backdrop to daily routine; only if pain spikes, or changes, is one likely to take note. In this setting, peripheral neuropathy (PN)–which often signals its presence by causing pain–can be easily misdiagnosed. An article published in the online journal, medpage today reports that though PN often occurs in conjunction with SLE, the two diseases have such similar symptoms that SLE may mask the presence of PN.   It is a patient who is best able to detect early signs of neuropathy; significant changes in pain or sensation should be reported to a healthcare provider.

Peripheral Neuropathy is a disease of the nervous system. The Neuropathy Association  describes PN as “…a disorder of the peripheral nerves—the motor, sensory and autonomic nerves that connect the spinal cord to muscles, skin and internal organs.” The symptoms include not only pain, but, among other things, tingling, burning, loss of sensation and muscle weakness. The disorder is often progressive and can be disabling; in some cases it leads to paralysis, loss of bowel function and even amputation. According to the Mayo Clinic, early treatment of PN offers the best chance for heading off more serious developments.

Dr. Franz Blaes, of the Grummesbach Hospital in Germany, describes the dilemma facing a physician treating someone who has both PN and an inflammatory rheumatological disorder, such as SLE or Sjogren’s Syndrome: “Patients can be classified neither as a rheumatic disease patient, nor as a patient with a neurological syndrome, but as both,” Dr. Blaes explains. The key to treatment, he suggests, is to get a clear picture of exactly what is going on. Is it PN, SLE, Sjogen’s, or a combination of conditions? Treatment is prescribed based on what this process reveals. In most cases where both the inflammatory rheumatological disease and PN exist, steroids are an effective intervention. However, regimens involving IVG therapy, plasmapherisis, cyclophosphamide and Rituximab may also be prescribed.

The most important thing SLE patients can do to help themselves, if there is a suspicion that PN is present, is to read the literature. Understand the tests that are given and what the results indicate; be aware of the kind of PN that is suspected; learn about treatment options, especially the one that is prescribed for your case. An article published in the September 2013 issue of Rheumatologist–Tips for Managing Peripheral Neuropathy in Rheumatic Disease by Timothy Collins–gives a good overview of peripheral neuropathy in SLE.

How does a doctor go about diagnosing PN, especially if someone also has an underlying inflammatory condition? According to Dr. Collins, this is not an easy task. The first step is to do a lab workup, including creatinine, complete blood count and liver enzymes. From these studies a doctor might eliminate some possible causes of the PN, such as diabetes. After getting the results from these tests, a doctor may turn to electrodiagnoistic studies. These help to determine the type of neuropathy and also may indicate the kind of underlying pathology that is causing the condition. A third kind of study that is sometimes suggested carries greater risk and often yields inconclusive results: nerve biopsy. According to Dr. Yvonne Lee of the Harvard Medical School, this “...shouldn’t be done before adequate clinical, laboratory, and electrophysiologic studies have been done.1 Results are often nonspecific, and complications, such as sensory loss and/or unpleasant abnormal sensations, are common.

Quality of life studies show that SLE patients with PN tend to score lower on quality of life metrics . This outcome is similar to other assessments of SLE patients who have central nervous system involvement.

Peripheral neuropathy is a serious condition; when it appears with SLE it may be overlooked or misdiagnosed. Sometimes PN may be the first noticeable symptom of SLE. Because the research on the relationship between PN and SLE is relatively recent, some doctors may not be alert to the issue.

The bottom line is this: if you have SLE and unexplained pain, PN is a possibility–one of many possibilities. In the past, rheumatologists believed this disorder was not likely to be seen in SLE patients; research is disproving this perception.

For most people with SLE, pain is simply a fact of life. But pain can be an important symptom and if there is a change in how it is experienced, then that should be noted. Perhaps the source of pain will not be discovered; perhaps the pain will not be alleviated. This may be a reality that a patient has to accept. However, one reality no one ever should accept is that pain, when reported, is disregarded. That course is irresponsible and even dangerous.

Barbara Enright

(Excerpt from These Are the Faces of Lupus )

Block 16 in Glitter Gulch, at the beginning of the twentieth century, was home to brothels and gambling parlors.  Local residents recall that prostitutes in scanty attire used to sit on porches and wait for their clients to call. Glitter Gulch, and Block 16, were both testaments to the fact that through much of Las Vegas’ history, the city owed its economic existence to the patronage of men – men who worked the gold and silver mines in one century and men who constructed Hoover Dam in the next.

By the time Benny Binion built the Horseshoe Casino on a plot that abutted Block 16, the seedy gaming parlors and brothels had been bulldozed and converted to parking lots.  Not that Benny would have objected to the brothels, or any other income-producing enterprise.  Legend has it that he had fled to Vegas from Dallas back in ’46 because the Chicago mob and local politicians muscled him out of town. The way some tell the story, Benny left behind in Dallas a legacy of multiple murders, bootlegging and organized gambling rackets.

When Benny Binion set up business in Glitter Gulch, Vegas was a pretty open town, but not open enough for Benny.  He didn’t like the $50 limits that were traditionally placed on wagers, so he raised the limit to $500, and then he removed the limit altogether, (on first bets). Binion’s Horseshoe Casino became the first establishment to offer a no limit game.  It was also the first casino to put down carpet (instead of sawdust) on its floors.

If Benny was anything, he was an original.  Thus, in 1970 he began a new tradition, one which today has grown to attract draws participants and spectators from around the globe.  It was Benny who hosted the first World Series of Poker tournament.  While the tournament’s million dollar pot was originally the main draw, now the prestige earned by winning the title and the tournament’s gold bracelets are at least as coveted as the cash prize.

Today, Benny Binion and the Horseshoe Casino no longer sponsort the World Series of Poker.  Benny died in 1989, and a few years later a corporate conglomerate (Harrah’s) bought the rights to the tournament.  Before Benny departed from the poker scene, however, he pulled off at least one more “first”: the Women’s Tournament in the World Series of Poker. In 1986, Barbara Enright became the first Women’s Tournament champion and the first female to claim the tournament’s coveted gold bracelet.  Nine years later, she won her second Women’s Tournament, and her second gold bracelet.  

Barbara did not rest on her laurels.  She continued to enter open (mixed gender) competitions, until, in 1996 she won the World Series Pot Limit Hold ‘em Tournament and, to the surprise of almost everyone (except herself) made it to the finals table in the Main Event – the only woman ever to do so.

In the shadow of block 16, where women were once sold for as little as a dollar and where they whiled their days awaiting the pleasure of men, Barbara Enright played with the best of them – and won. By the time she received her third trophy bracelet in 1996, Barbara had been diagnosed with lupus for twenty years.

Barbara Enright was born on August 19, 1949 in Los Angeles California.  In her youth, she was licensed as a cosmetologist and worked at a number of jobs: cocktail waitress, bartender, and stylist for Hollywood celebrities.   In the 1970’s she tried her hand at poker in the Gardenia California card rooms, where she did so well that she eventually quit her other jobs and dedicated herself to poker.

Barbara asserts that she has never played a “woman’s” game.  She comes at her opponents with decidedly “unfeminine” aggression.  Enright explains her success in the traditionally male-dominated poker room:  she simply states that she is aggressive and that if a woman is too “soft” in tournament play she cannot succeed.  But if she asserts herself and defies the expectations of the men sitting at the table with her, she can do “very well.”

On July 6, 2007, Barbara Enright was inducted into the Poker Hall of Fame, the first woman ever to be invited into that select academy.  As of 2007, it was estimated that her tournament winnings exceeded $1,200,000 , more than any other woman in tournament history (at that time).

Today she lives in Hollywood California with Max Shapiro, a poker player and a columnist for Card Player Magazine.

Barbara once said that her dream was for there to be world peace and free medical coverage for every person. Perhaps the content of this dream was influenced by her experience with lupus. While most of her bios refer to the fact that Barbara has lupus, her challenge with the disease has remained private. Some writers refer to the disease as almost a footnote in her life; many writers do not refer to it at all.  It seems that the kind of lupus Barbara lives with and the treatment she receives for it, are very different from the lupus that took Inday Ba’s life.* Since her diagnosis in 1976, Barbara has raised a child, managed a successful career in an exotic profession and earned a reputation for being smart, friendly and focused.

In the storied saloons of Glitter Gulch, where ladies were historically dispatched like grains of sand from the vast and unforgiving Mojave desert, Barbara Enright made her mark.  A combination of raw talent, hardscrabble determination and a dollop of luck helped her to secure a place in the history of Las Vegas and the world of professional poker.

* Actress who died or Lupus at the age of 32

What Is Lupus

What is Lupus?

By A. G. Moore

Autoimmune disease: auto, from the Greek, means self (as in automobile: a self-moving vehicle). An autoimmune reaction, in which the body essentially makes itself sick, can be as simple as a rash induced by contact with poison ivy, or a full-blown case of systemic lupus.

The analogy to a car is helpful here: a car (automobile) operates automatically only in the sense that someone turns it on, turns it off and directs its path. However, from time to time reports appear in the newspapers of cars that are out of control—cars that somehow accelerate on their own or fail to respond to instructions to stop, or turn. These automobiles, instead of being dutiful and useful servants, become dangerous agents. This is sort of what happens in an autoimmune disease.

The immune system exists to protect the body. A complex set of interactions go into operation when the body is under assault. The system includes the ability to surround and consume an alien entity (such as bacteria). The trigger—or ignition, if comparing it to a car—for the immune system to go into action is an external threat. In a healthy individual, when the threat is removed the immune system becomes quiet.

However, when autoimmune disease takes hold, the immune system goes haywire, like a car out of control. It starts attacking things that aren’t foreign because it sees healthy tissue as an enemy. In lupus, the attacking immune cells can go after just about anything—the lungs, heart, blood, brain, etc.

The mystery of why this happens is only vaguely understood. Some of the triggers that set off the autoimmune attack have been identified—Espstein-Barr and UV exposure, for example. But on the whole, the autoimmune syndrome that is at the heart of lupus remains a mystery. Once this mystery is solved, then a cure, or even a way of preventing the disease, may be found.

Right now, medicine can offer people who have lupus only one thing: possible control of their symptoms. In most cases this is achievable, though sometimes at great cost.

Lupus can be very resistant to treatment. Since it is still a mystery and since the remedies for the disease are diverse and variably effective, it makes sense that someone with lupus find the smartest, most skilled physician available to treat their disease. Only a doctor with insight, experience and top-notch training should be enlisted in the war against lupus, an obstinate and potentially fatal autoimmune illness.

Reference:

What is Lupus?http://www.health.ny.gov/diseases/conditions/lupus/fa

What is Lupus?http://www.lupusinternational.com/About-Lupus-1-1/What-is-Lupus-.aspx

What is Lupus?http://www.lupusalliance.org/content.asp?id=8

What is Lupus?http://www.lupus.org/webmodules/webarticlesnet/templates/new_learnunderstanding.aspx?articleid=2232&zoneid=523

Plaquenil

Plaquenil

By A. G. Moore

One of the first medications a lupus patient may be offered is Plaquenil. While every drug has side effects, Plaquenil is generally considered to be “safer” than many other medicines prescribed to treat lupus.

Researchers are finding out more about this drug all the time; the current state of science indicates that Plaquenil is effective against lupus, and some other inflammatory diseases, because it creates an inhospitable environment for some actors in the immune process. In this way Plaquenil inhibits the immune response and thus also inhibits inflammation.

Though Plaquenil is considered to be relatively “safe”, there are several populations in whom great care should be taken before the  medicine is prescribed. If you are over the age of 60, the chance for retinal damage increases. If you have psoriasis or porphyria, taking this drug may exacerbate your condition. Anyone with reduced liver or kidney function should also consider carefully if the risks of the drugs outweigh its benefits. Plaquenil can be extremely dangerous for children, especially children under the age of 6. People who have a metabolic disorder called G6PD run the risk of developing severe anemia on Plaquenil therapy.

The most commonly addressed side effect of Plaquenil therapy is retinal damage. Every responsible opinion I have read prescribes a visual screening before therapy begins and then a re-check several weeks later. While it is rare for eye damage to occur, especially if therapy lasts less than five years, it is important to keep in mind that once damage occurs, it is most likely irreversible. And the changes in the retina may not be noticed subjectively but can be detected by sophisticated testing in the ophthalmologist’s office. Many doctors recommend a yearly check-up; some recommend six months. If I were on Plaquenil I would go for six months because I’d like to detect damage before it goes very far.

With all of the warnings listed above (this is just a partial list of possible side effects) it would seem that Plaquenil is a dreadful drug. It really isn’t for most people. Lupus is a dreadful disease and sometimes you have to take out a big gun to control it. Plaquenil is actually one of the smaller guns in the arsenal against lupus.

Originally used as a treatment for malaria, Plaquenil is useful not only in treating SLE (though not severe SLE), but also other inflammatory diseases. The drug is supposed to be particularly effective at treating discoid lupus.

All of the sources I consulted implicate higher-dose and longer-term Plaquenil treatment in increased risk of eye damage. So if you are on Plaquenil for many years, certainly as many as 8, your doctor might begin a conversation with you about switching to an alternative treatment.

Several helpful sources I used in order to get information for this article were:

Plaquenil Data Sheet: http://www.medsafe.govt.nz/profs/datasheet/p/Plaqueniltab.pdf
eyeupdate.com: http://www.eyeupdate.com/plaquenil-updates.html
and
PDRhealth: http://www.pdrhealth.com/drugs/plaquenil

I think anybody contemplating taking this medication should read and understand each of these sources. In making this recommendation I am operating on the theory that, though we may not know everything the doctor knows, we always should be vigilant in supervising our own medical care.


Photosensitivity

Negotiating a planet in which light is the source of life is strategically challenging for someone to whom UV radiation is noxious. However, with information and planning, life away from light can be managed. Photosensitivity, for most, implies an adverse reaction to UV radiation. Artifical sources of light, indirect illumination and UV screens facilitate a life in the shade.

However, none of these accommodations addresses a major obstacle in negotiating a life away from light.

It is likely that I have been UV sensitive all of my life, although this phenomenon has only been quantified in the last twenty years or so. When I was a child my siblings used to taunt me with a chant: “fun in the sun”. Apparently I did not enjoy activities that were carried on for long periods in bright sun shine. As I got older, I sought shade as a natural place of comfort. Pictures of me with my children as infants reveal a woman squinting against the sun under a wide-brimmed hat.

The first time a physician suggested I was reacting to UV was when a dermatologist noted that a rash that had broken out followed like a chart the areas of my skin that had been exposed to the sun. Another dermatologist told me to wear sun screen year-round because of a pattern of discoloration that had become evident on exposed areas.

There came a time when I was diagnosed with systemic lupus. It was not surprising that photosensitivity was one of the manifestations of my disease. A lot of things became clear to me–why I would break into a sweat when I was in stores with bright overhead lighting. Not only would I sweat but my cheeks would redden and my discomfort would grow so that I had to flee from the store, sometimes leaving my as-yet-unpurchased items behind.

Of course, as people who are photosensitive know, reacting to UV is not simply a matter of feeling uncomfortable. It is not even a matter of getting a little rash. UV exposure makes us sick.And that’s the part where public perception comes in. That’s the part other people don’t get.

When I say, “I have to get away from these fluorescent lights,” I don’t mean I might get a little headache. I mean that a disease process will be activated.

Last year I wrote a book about lupus and one of the things that prompted me to write the book was when I read about Hannelore Kohl, the wife of the (ex) German Chancellore, Helmut Kohl. Hannelore Kohl committed suicide. Every news item I read described her death in the same way: she had an allergy to “bright lights” and had been living in seclusion because of this allergy. She was depressed by her situation and so she took her own life.
Photosensitivity is included in the ACR’s list of diagnostic criteria.  Estimates for the number of lupus patients who experience photosensitvity vary. Dr. Lenny Tuffanelli writes in Maryland Lupus Foundation newletter, “Thirty to forty percent of lupus patients are truly photosensitive” , though he cautions also that all people with lupus “may be harmed by excessive exposure”
Most of the estimates about occurrence are taken from the ACR, and implicit in these estimates  are issues of  reporting and interpretation.  As with the larger problem of lupus diagnosis and treatment, the physician is dependent upon a patient’s impression, and the physician adds to the mix a clinical filter. It should not surprise anyone that the results of such a process are unreliable.  For example – how precise  is an estimate which ranges from thirty percent (Dr. Tuffanelli) to seventy-five percent (upper range of ACR estimate)? How can a doctor, or patient make a decision based on such  vague numbers?
Dr. Victoria Werth, of the University of Pennsylvania Medical School, explains the process that causes a photosensitive reaction. Dr. Werth and her research team at Penn Medical School have “identified a variant of the human gene for tumor necrosis factor-alpha as a cause of photosensitivity in lupus.” Dr. Werth believes that identifying the gene variant that causes photosensitivity can help doctors to identify people who are likely to get lupus and can also contribute to an understanding of why the disease develops.
Dr. Werth discovered that in a those lupus patients who have one or two copies of the variant gene, exposure to sunlight stimulates the gene and subsequently the skin cells to undergo apoptosis (cell death).  The occurrence of cell death is then a trigger for immune system activation.
The photosensitivity of lupus patients ( and of others who have diseases the induce photosensitivity) has a pervasive effect on lifestyle.  Blithe pronouncements about the manageability of the condition with the use of sunscreens are ill advised.  For one thing, sunscreens can be treacherous.  My last experiment with one that I bought in Walgreens caused my face to swell so that I looked as though I was suffering from dropsy.  Not only did my face swell, but my whole system was put into overdrive and I was ill for about a week.
One well-intentioned site recommends patch testing a new product.  Patch test with extreme caution and maybe even medical supervision. I patch tested my new sunscreen twice, once on the inside of my arm and once on the outside.  I waited at least 12 hours between tests and another twenty four before putting the lotion (liberally) on my face.  Approximately eighteen hours after the last application I started to scratch.  First the chin, then the arm.  One side of the arm never reacted and the second only mildly.
Not only can sunscreens be harmful, but they are only partially effective.  Sunscreen, long sleeves and a hat – all together are helpful.  But this get up doesn’t mean its safe to wander around in full sun on a summer afternoon bathed in UV radiation.
I generally do not expect people to make exceptions for me or put themselves out, whether because of lupus or any other problem I have.  However, I think that so many people have an issue with UV radiation (not just those who have lupus) that simple structural modifications can be routinized.  Inexpensive filters can be placed over florescent lights, for example.  Certainly in a medical setting this should be mandatory.

Neutrophil Extracellular Trap

Neutrophil Extracellular Traps 

By A. G. Moore

This isn’t about basketball or hair management; this is about autoimmunity. It seems that one of the latest cuplrits to be discovered in the search for lupus antagonists is something called the neutrophil extracellular trap(see http://www.jimmunol.org/content/187/1/538.short).  Neutophils are the most plentiful disease-fighting cells in the immune system. The role of neutrophils is to attack and kill invading organisms. These highly motivated soldiers in the immune system’s army are willing to commit suicide in order to fulfill their mission. They do this by releasing chemicals between the cells. The chemicals from different neutrophils join together and form a trap, which serves as a kind of net to capture and destroy pathogens. In order to release enough of the deadly material to be effective, the neutrophil has to deplete its own supply and die. It does so willingly.

Unfortunately, this ally of the body, in waging war, sometimes does damage to the very organism it seeks to protect. When the NETs (neutrophil extracelluar traps) are floating around in the bloodstream they can cause inflammation.

The discovery of the role that NETs play in the development of inflammation is very important to understanding the mechanism which precipitates lupus flares. The more researchers understand this process, the closer they get to thwarting the process. Not only may it be possible to design better therapies so that many of the damaging effects of lupus can be avoided; it may also be possible, with an understanding of the role that NETs play in inflammation, to find a cure and maybe even prevent lupus.

Lupus and Vaccines


By A. G. Moore 9/2/2012

People with lupus have a greater susceptibility to infection (see: Lupus Foundation http://www.lupus.org/webmodules/webarticlesnet/templates/new_about.aspx?articleid=411&zoneid=2). The increased risk for infection arises in many cases from the immunosuppressive drugs that lupus patients take (such as methotrexate, prednisone and Azathioprine). Increased susceptibility can come also from the disease itself, although the degree to which this is true varies according the individual. In a 2005 study, published on the ACR website (http://onlinelibrary.wiley.com/doi/10.1002/art.1780170102/abstract) infection rates were compared for hospitalized patients who had rheumatoid arthritis, nephrotic syndrome, and systemic lupus. It turned out that those with systemic lupus had higher infection rates than other patients in the study—even those with SLE were not on immunosuppressive drugs. The ACR article’s conclusion is that the higher infection rate for lupus is not associated with compromised renal function or immunosuppressive drug therapy but to some other aspect of the disease.

So what does a person with lupus do about this increased susceptibility? For one thing, stay away, as much as possible from people who are evidently sick (with an infectious disease). Also, in cold and flu season, keep away from crowds. Simple things, like washing hands and not touching your face, have been proven to be effective infection controls.

Another recommendation, endorsed by the Lupus Foundation, is to keep your vaccinations up to date. However, according to the Foundation, someone with lupus should never receive a live vaccine and should avoid exposure to people who have recently received live vaccine. Also, the possibility exists that if the flu vaccine is given during a lupus flare, then a serious complication may ensue (see: http://www.lupus.org/webmodules/webarticlesnet/templates/new_about.aspx?a=411&z=2&page=3).

Personally, I approach flu vaccine season with ambivalence. While I was vaccinated most years without incidence, last year I experienced a pretty serious reaction very soon after being vaccinated. Not only did my arm blow up pretty quickly, but by the next morning I was ill and I stayed ill for about two weeks. Though I dread going through that again, I wonder how much sicker I would have been if I had caught the flu?

As for run of the mill upper respiratory infections, I take a few precautions. First of all, I use avoidance and barriers. I stay away, as much as possible, from people who are evidently sick (with an infectious disease). If I must be around someone who is ill—to nurse, let’s say, a family member— I wear double layer surgical masks (N95 rated) and disposable gloves. I wash the clothing that has been exposed as soon as I am out of the sick room.

If I seem to be coming down with a cold, I pop a Cold-EEZE lozenge into my mouth. The zinc in the lozenge is suppose to reduce the length and severity of the cold—if taken in the first 24 to 48 hours. The most recent study I found that supports the idea that these lozenges are therapeutic, was published in the Journal of Infectious Disease, in 2008 ( see: http://www.ncbi.nlm.nih.gov/pubmed/18279051). I usually don’t wait for a cold to develop to start this regimen. I keep Cold-EEZE in the house and as soon as my throat feels scratchy I suck on a lozenge and keep up the regimen till all signs of a cold have disappeared.

The final thing I do for myself is, if I am ill and things don’t seem to be going well, I call my doctor.

I don’t know if any of the measures I take will be helpful to someone else. I state again, as I always do: I am not a doctor. I read a lot and learn from what I read. I hope the information is helpful to others.