Professional Patient

World Sjogren's Day

On July 23rd, the Sjögren's Syndrome Foundation will join with 16 other Sjögren's groups around the world as we all celebrate the 1st Annual World Sjögren's Day.

SSF chose July 23rd as World Sjögren's Day because it was Dr. Henrik Sjögren's birthday. In honor of this special day, SSF is  asking you to consider joining with patients and advocates worldwide to spread information about Sjögren's through your family, friends and communities; display a poster, handout brochures or just tell someone about Sjögren's.

A New Sjogren's Low

I reached a new Sjogren's low while on vacation last week. I was chewing Orbit sugar-free gum, and my mouth was so dry that the gum stuck to the roof of my mouth! I had to scrape it off with a fingernail. Eww. 

You Might Be a Sjogie If...

You might be a Sjogie if, every time you pass a mirror, you don't check your hair, or your makeup, or straighten your shirt. That would be too normal! You might be a Sjogie if every time you pass a mirror, you check your smile for food stuck in your teeth, white saliva crust on your lips, and gook in the corners of your eyes.

It's a sexy life, but somebody has to do it!

Alternate-Day Prednisone

I have been on prednisone, 7 mg per day, for more than a year and a half. Prednisone is great - I have better energy, clearer thinking, more stamina, and my anemia and leukopenia are gone. But prednisone is trouble over the long term... it can cause an array of terrible side effects like diabetes, cataracts, broken bones (caused by osteoporosis), vicious mood swings, hunger, extremely thin skin, weight gain, insomnia, tremor, and inability to fight off infection. Also on the list: muscle weakness, especially in the shoulders and thighs. 

My muscles started feeling weak over 6 months ago. And I have been on prednisone for far too long. So I think it's time to wean off. Maybe my muscles will feel better after I am prednisone-free; maybe not. But I'm ready to find out. 

Prednisone works in place of hormones that your adrenal gland would normally produce. When someone takes prednisone every single day for a long time (more than a month), the adrenal gland slacks off and stops doing its work. So your body stops producing (or produces much less of) its own adrenal hormones:

"Prednisone mimics the function of the natural hormones produced by the adrenal glands. When patients take prednisone for long periods of time, the production of natural adrenal hormones decreases because of prednisone's effect on the pituitary, the master gland that controls the adrenals. This typically happens when the dose is higher than 5 - 7 mg per day and used for periods longer than one month." Source: Weaning from Prednisone, from Sarcinfo.com) 

Your adrenal glands also shrink from this lack of activity: "By this time [four weeks after you start taking prednisone], some shrinking of the adrenal glands will occur, as their burden of producing cortisone has been relieved." (Source: Systemic steroids, from Dermnet New Zealand)

The normal approach to taking someone off prednisone (someone who has taken prednisone for a long time) is to gradually taper the dose - I might take 6 mg per day every day for a month, then 5 mg per day every day for a month, then 4 mg per day every day for a month (and so on)... this gives your adrenal gland time to wake up. I have tried this in the past (I tapered from 10 mg to 7 mg a while back), and man, does it make me tired. I don't think my adrenal gland is in any mood to wake up and get back to work. 

My rheumatologist has suggested that we try something a little different: alternate-day dosing. One day I take approximately double the normal dose of prednisone (10 mg), and the next I take 0. This is intended to allow people to stay on prednisone for an even longer period of time while minimizing some of the side effects. The rationale is that the alternate-day schedule forces the adrenal gland to wake up and start working again on those days when no prednisone comes in. 

Rather than using alternate-day dosing as a way to stay on prednisone for even more time, I want to use it to get ready to quit prednisone. By spending some time on an alternate-day schedule, my adrenal gland can start to warm up and get back to work before I begin to taper off the medication completely. 

First, I have to taper into the alternate-day plan. I will go 10 mg on even days and 5 mg on odd days for a month, then 10 mg/2.5 mg for a month (I'm starting this phase right now), then 10 mg/0 mg. 

I plan to stay at that level for a few weeks, and then will likely taper off the med altogether. I can envision going 9 mg/0 mg for a month, then 8 mg/0 mg for a month, then 7 mg/0 mg for a month... until I'm at 0/0. This article on systemic steroid use suggests that the adrenal gland may not fully recover for up to one year after steroid therapy. Maybe by the time I get through my elaborate, alternate-day tapering plan, my adrenal gland will be fully energized and ready to go. 

Side effects of going to an alternate-day dosing schedule so far have included increased appetite, periods of improved sleep (longer sleep) mixed in with periods of insomnia, and extreme mental fatigue during the first one to two weeks after stepping down my prednisone dose (I may need to try something like provigil to help get my mental focus back - the quality of my work is really suffering). My energy level within any given day is really unpredictable - I can be OK one minute and ready to pass out from fatigue the next. My muscles feel better, although not all the time - they hurt, but they are less tired. The biggest surprise for me so far is my depression - prednisone had made it nearly disappear, and now it's back (even though I am at the 10 mg/2.5 mg level rather than 10 mg/0 mg). 

Learn more:

Prednisolone tablet information from consultantlive.com. Scroll down to the "Dosage" section and look for the Alternate-Day Therapy heading. 

Systemic steroids, from Dermnet New Zealand. 

Steroids (oral cortisone), from the American Osteopathic College of Dermatology.

Weaning from Prednisone, from Sarcinfo.com. 

Pesky Parotid Lump: Aspiration, Take Two

Someone left a comment asking about how my pesky parotid lump situation worked out.

When I last posted, I was heading to the big university hospital in town for a second fine needle aspiration. The first aspiration had not generated enough material to complete the flow cytometry that my doctor had requested.

My second needle aspiration was interesting and surprising. A pathologist (rather than an ENT) did the aspiration herself. She did NOT use any kind of local anesthetic, because she said that the anesthetic is typically drawn back out through the needle, washing out or hiding the real fluid/material from your parotid. The doctor also did the FNA in stages... she poked me enough to get a decent amount of fluid, then put that fluid on slides and analyzed the slides under a microscope. Then, based on what she saw, she poked me again, put the fluid on slides, looked at them under a microscope, and so on. By doing the FNA in stages, the pathologist was able to make sure she had enough fluid/material to do a proper analysis under the microscope and to complete the flow cytometry.

I think we finished after three rounds. The pathologist drew out as much fluid as she could to make my "lump" shrink down to almost normal size. It was great while it lasted... but within 48 hours, my lump was back to its old size!

I can say with conviction that having a needle poked into your face repeatedly, without any kind of injected anesthethic, requires some machismo. It was not the kind of pain that would make you black out or scream, but it certainly was the kind that would make you jump and say "OW!" Next time, I might ask if a topical anesthetic would help - it at least would not sink into the face. 

Another benefit of having the pathologist do my FNA and analyze it as she worked - she was able to tell me immediately whether she saw anything concerning on the slides. I went home knowing that the pathologist was pretty certain that my cyst was just that... a harmless cyst.

A few days later, the final results came in: not cancerous. Totally benign! Woohoo!

New Lupus/Sjogren's Blog

I just stumbled across Wicked Antibodies, a blog written by "Crabcakes,"  a medical technologist, with a recent diagnosis of lupus and Sjogren's, who has a daughter with MS. 

Good stuff! I often have docs tell me I have "lupus-y" Sjogren's... so it's helpful to read about someone who definitely has both.

E-mail ListServs (Discussion Groups) for Sjogies

There are two long-running e-mail listservs (discussion groups conducted through e-mail) that support patients with Sjogren's syndrome.

The granddaddy of them all is the SS-L listserv. This list is devoted to discussions of medical information - symptoms, treatments, how to get a diagnosis, etc.

The TalkSjo list is for "support and sociability" among Sjogies. See http://www.dry.org/talksjo.html for more information.) This is a place to chat with people who have been through it all, too. 

You can also find online support at Sjogren's World (including a Sjogren's World discussion forum and live chats) and Sjo Girls at Cafe Chronique (including a Sjo Girls discussion forum). 

Below are instructions on how to join the SS-L and Talk Sjo lists. 

To join the SS-List:
Send an email message to LISTSERV@LISTSERV.ILLINOIS.EDU, containing these lines, written just like this:
SUBSCRIBE SS-L ANONYMOUS
END

You will receive a confirmation request from the "listserv", usually within an hour or so of your Subscribe message. The confirmation request requires an "OK" response from you. To make your OK response, use your email program's Reply button, and edit your reply to contain just these two lines:
OK
END

Once you have OK'd the confirmation request, you will receive (within an hour or so) two messages from Listserv with background information about using SS-L. You will also begin receiving email messages as they are sent by list members. If you see no messages for a day or two, then something has gone wrong, and you should try again from the beginning.

Additional SS-L links:

• Change the email address that receives your SS-L messages: http://www.dry.org/address.html 
• Take a vacation from SS-L (temporarily stop receiving SS-L messages): http://www.dry.org/nomailssl.html
• Resume delivery of SS-L: http://www.dry.org/mailssl.html
• Receive a daily digest of SS-L (this is incredibly helpful to limit how many e-mails you receive from the list): http://www.dry.org/digestssl.html
• Unsubscribe from the SS-L list: http://www.dry.org/unsubssl.html 
• Search the SS-L archives: http://www.dry.org/archivessl.html [User name:  archives   Password:  searchme]

To join the TalkSjo list:

To join the TalkSjo group, send the subscribe message first.  The post messages, use the "post message" address.   TalkSjo is a private list, so it doesn't show up in the Sjogren's lists on Yahoo groups.  If you have a yahoo account, you can add it to your yahoo groups once you are already a member. You can unsubscribe any time, or set up a Yahoo account for free that will allow you to change your setting at the yahoogroups.com website.  That's a little trickier though, so start with these addresses.

Post message: TalkSjo@yahoogroups.com 

Subscribe:
TalkSjo-subscribe@yahoogroups.com 

Unsubscribe:
TalkSjo-unsubscribe@yahoogroups.com

Dry Mouth Resolutions for 2010

A few new or different things to help your dry mouth stay healthy in 2010.

• Once a week, brush your teeth using only water. Toothpaste gives your mouth a smooth feel… which makes it hard to locate the rough patches of plaque in your mouth. Brushing without toothpaste gives you a chance to find the corners of your mouth that may need a little extra help.
• Use a straw when drinking anything sweet. The straw keeps the drink from contacting the front of your teeth.
• After brushing, wipe your tongue with a towel or washcloth to remove toothpaste residue. This can reduce irritation and can help keep toothpaste-tinged saliva from accumulating on your lips.
• Ditch the tartar-control toothpaste. Studies are increasingly showing that tartar control ingredients are more irritating and can contribute to contact stomatitis around the lips. 
• Remove food from between your teeth after meals. This reduces the risk of decay and keeps breath fresher. Use a Proxabrush, stim-u-dent, toothpick, or similar tool.
• Do more by doing less. My periodontist tells me that if something hurts in my mouth, I need to treat it very gently, or skip that spot altogether for a day or two. Which seems backwards to me – I always want to give more attention to sore spots.
• Try a soak before brushing. Some dentists recommend that you use your mouthwash 15 to 30 minutes before brushing. This lets the mouthwash start to break up the plaque so that brushing can remove it more easily. My periodontist also recommends that if you use a proxabrush or similar tool to clean between your teeth, you should dip it in baking soda or Biotene mouthwash first, which will help deliver it to the surfaces between teeth.
• Ban the brushing motion. If you use a power toothbrush, just say no to the standard brushing approach. Place your toothbrush on one tooth, let it sit for a few seconds, then lift it and place it on the next tooth. And so on. If you’re anything like me, you may find that this helps you brush every tooth – I was actually missing a few. 
• Try Recaldent/MI paste. This is the new wonder-tool that periodontists and dentists use to recalcify sensitive, cavity-prone teeth. Read more in this article on minimally invasive dentistry from the Academy of General Dentistry website. 
• See a periodontist before you have any gum problems. Make a least one appointment to learn the proper techniques for cleaning troubled teeth and gums. It may save you a periodontist visit later on.
• Raise the moisture level of your mouth. Try a humdifier, use a drug like evoxac, or try a fish or flaxseed oil supplement. You will be more comfortable.
• Trade your floss for a waterpik. Floss can cut delicate gums. Waterpiks work just as well.

Purple Toe After Snow? Could Be A Chilblain (aka Perniosis)

It's winter again, its cold and wet outside again, and my toes have little purple blistery things on them... again. My podiatrist tells me they are chilblains (folk name), aka "pernoisis" in medical speak. 

Chilblains are small areas of the skin (usually the on toes, but also sometimes on fingers, nose, and cheeks) that have suffered a cold injury. The most common way someone can get chilblains is when they are suddenly exposed to cold/wet weather and don't properly protect themselves from the cold and wet. Like when there's a sudden snowstorm, and you find yourself outside shoveling snow in your uninsulated cowboy boots because you can't find your warm, fuzzy, waterproof snow boots.

The cold injury happens as follows. First, the area of skin gets very cold, so the tiny blood vessels constrict. Then the area warms up, perhaps very rapidly (think of bringing your cold, wet feet inside and immediately holding your toes up to a heater). The constricted blood vessels can't open up fast enough in response to the sudden heat. And viola! You get small, red or purple swollen spots that may itch intensely and then hurt. 

Chilblains are more common in people with poor circulation or Raynaud's phenomenon. I suspect mine are related to my Sjogren's Syndrome and the Raynaud's that goes with it. Chilblains can also be associated with a special kind of lupus called chilblains lupus. Once you get your first chilblain, you may get them every winter, no matter how carefully you care for your feet. 

What can you do once you have a chilblain? See a foot doctor or your family doctor. He/she will probably tell you to simply soak the affected body part in warm (not hot) water for 15 minutes once or twice a day, then dry thoroughly, and repeat until the swelling goes down some and the color fades. You may also be able to use topical steroids or vasodilators to treat the chilblain. And if your chilblain opens and gets infected, you will of course need antibiotics for that. 

The best way to prevent chilblains is to keep your hands and feet warm and dry. Buy insulated, waterproof boots and gloves. And wear them... not just when it snows, but when it's just biting cold outside. Once you have been outside and gotten cold or wet, be sure to dry off and gently warm up your hands and feet (don't jump in a hot tub or sit next to a fireplace; try tepid water or simply sit in your warm house and wait). 

Learn more:

Chilblains, from ePodiatry.com. 

Chilblain lupus, from Dermis.net

Chilblains (pernio), from DermNet New Zealand. 

Testing for Peripheral Neuropathy

I may have developed peripheral neuropathy, a nerve problem that can occur in chronic illnesses such as Sjogren's Syndrome. Peripheral neuropathy is most often described as causing numbness and tingling sensations in one or both hands and/or one or both feet. For me, it feels a lot scarier than that - burning sensations in my arms, legs, and sometimes my torso and chest. Itching/prickling sensations. Numbness. Occasional stabbing pain. Cramping in my toes and forearms. Cramping in my thighs and calves. Muscle twitching. Poor fine motor skills - fingers that won't do what I want them to. Feeling like I am vibrating or shaking all over... even though I am not. Feeling like my legs won't hold me up, or like I can't move an arm or a knee. Rapid muscle fatigue and pain with exercise. And extreme, life-flattening exhaustion. 

Neuropathy occurs when something injures the nerves and causes them to lose function and/or send false messages to your brain. In autoimmune disease, this phenomenon is related to the immune overactivity that is damaging other parts of your body as well. Sjogren's World has a nice page that reprints a lecture/article by Elaine Alexander on Peripheral Neuropathy and Sjogren's. It explains the various flavors of neuropathy and gives some (slightly outdated) information about treatments. MayoClinic.com has a more updated set of information about peripheral neuropathy treatments and drugs. 

If you think you might have neuropathy, you need to go to a neurologist for a thorough exam. When I saw my neurologist, he did things like test my strength in arms, leg, shoulders, hips; check my deep tendon reflexes in arms, legs; and use a safety pin tip to test where my skin registered sharp sensations. Then he recommended a slew of tests to check the function of my nerves.

Testing for neuropathy is not a fun experience. I had it done once before, and all of my results came back negative. This was in early 2008. Now that my symptoms have really ramped up and are a daily problem, they want to put me through all of the tests again. 

Step 1 is a nerve conduction study and electromyelogram (EMG). WebMD's Electromyelogram and Nerve Conduction Studies web page explains that an EMG tests for neuromuscular issues (damage to muscles, nerves, and junctions between them), while a nerve conduction study focuses specifically on how signals move through the entire chain of nerves in your body. Both involve getting little zaps of electricity sent through your affected area (they will probably test a leg and/or arm for me). It doesn't hurt, but it's not pleasant, either. 

Step 2 is a skin punch biopsy. True peripheral neuropathy is sometimes caused by damage to the small-fiber nerve cells, which are grouped farthest away from the spinal cord, near the surface of your skin. EMG and nerve conduction testing can't detect this kind of neuropathy - your test results will be normal. To check for small-fiber neuropathy, a technician will take a small section of skin from an affected part of your body. They use a small-diameter circular "punch" tool (a tiny open-ended cylinder) to remove the skin. For me, they plan to take one "punch" of skin from my upper thigh, lower thigh/upper knee, and ankle on each leg - for a total of six segments. This skin is then prepped into slides and reviewed by a pathologist. The pathologist is trained to identify damage, swelling, and abnormal nerve cells that may be visible on the slides. Johns Hopkins University's web site includes a detailed explanation of the skin punch biopsy procedure, with pictures. 

I found my first skin punch biopsy to be unpleasant at best. I had a hard time lying still long enough for the skin samples to be removed. And then, a few hours later, the biopsy sites really started to hurt. And kept hurting for at least 36 hours. Also, my sites didn't heal up as quickly or prettily as the take-home instructions said they would. So plan to have advil handy and be careful about washing and drying your biopsy sites until they are fully healed! Also, if you take blood thinning medications, be sure to stop taking them two weeks before the procedure, so you don't bleed excessively.