Is there any speculation as to what’s driving this big upswing in the diagnosis of men and children with Sjögren’s?
My gut feeling is that it has to do with the improvements in awareness and medical and dental education in recent years. The Sjögren’s Syndrome Foundation (SSF) has spent years trying to train the physicians and nurse practitioners about how prevalent and serious the disease is. We finally have a celebrity who unfortunately was diagnosed with Sjögren’s and although nobody likes to see somebody become ill, it has done a lot to help the entire public realize how serious it is, particularly the idea that people look a lot better than they feel and that it may take years to diagnose it unless you take the symptoms seriously.
I can tell you at the University of Pennsylvania, where I work, the oldest medical school in the United States, we only started giving our first Sjögren’s lecture to the first year medical students about four years ago. And that was only after years of me fighting with the curriculum committee to get it included in the rheumatology course for the first year students. So, we’ve made a lot of progress and I think that’s an example of the benefits of all this work.
-Frederick B. Vivino, MD, MS, FACR
This "Question & Answers" article was first printed in the The Moisture Seeker, SSF's patient newsletter for members.
I am honored that on July 1, I was introduced as the new Chairman of the Sjögren’s Syndrome Foundation Board of Directors. My wife, Anne, and I became passionate volunteers for the SSF when Anne was diagnosed with Sjögren’s, and I have seen first-hand what it’s like to live with this devastating chronic disease.
The SSF is an inspiring organization and I am personally excited, as well as honored, to be leading us over the next two years! I hope you will join me, and also consider making a donation, as the SSF embarks on a two-year journey to intensely focus on changing the way Sjögren’s is treated, managed and monitored. We can't do this without you.
The past few years have seen amazing progress in awareness and research for Sjögren's. At the last American College of Rheumatology (ACR) Annual Meeting, the premier scientific and clinical meeting for rheumatologists in the United States, more abstracts and presentations specific to Sjögren's were on the program than ever before. The SSF annual educational luncheon, held for rheumatologists during ACR, was standing room only and we had more rheumatologists visit our exhibit booth requesting complimentary patient brochures for their offices than at any previous ACR meeting.
Additionally, during ACR, late breaking news was announced that six genes specific for Sjögren’s were identified by SSF-supported research at the Oklahoma Medical Research Foundation. This identification is the first time that genes have been connected solely to Sjögren's and marks the beginning of unraveling the genetic basis for Sjögren’s. This research was a huge breakthrough and could lead to determining a patient’s individual risk of certain complications and developing future treatment.
That is why today I am asking for your support. The SSF needs your help to expand our efforts to educate healthcare professionals, increase clinical trials for new therapies and to develop better diagnostics. Just imagine what these accomplishments would do for the quality of life of a Sjögren’s patient - a matter very close to my heart.
As a proud husband of a Sjögren’s patient, I see the struggles that my wife experiences each and every day and I want nothing more than for her disease to be nonexistent. The critical work of the SSF brings us HOPE for the future as they lead the charge to improve the lives of all patients.
Thank you in advance for your gift as we strive to keep our momentum alive and impact the future of Sjögren’s.
Chairman, SSF Board of Directors
Plaquenil, hydroxychloroquine (HCQ), is an anti-malarial medication that has been proven to be useful in the treatment of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and other inflammatory and autoimmune diseases. In Sjögren’s, Plaquenil is used to treat many symptoms of Sjögren’s including fatigue, joint symptoms of arthritis and arthralgias (joint pain), dry mouth and dry eyes. Similar to its use in systemic lupus erythematosus, many clinicians feel that it is useful in reducing general Sjögren’s “disease activity.”
One of the reasons that physicians feel comfortable in prescribing Plaquenil is its low risk to benefit ratio. This means that the side effects of Plaquenil are mild and infrequent compared with its potential benefits. As with any medication, allergic reactions including skin rashes and non-allergic reactions can occur. The side effect that is of greatest concern is retinal toxicity.
Retinal toxicity of Plaquenil may manifest itself with subtle disturbances of the retinal pigment epithelium which may eventually lead to complete destruction of the macula in the form of bull’s-eye maculopathy.
Several risk factors may increase the likelihood of retinal toxicity from Plaquenil such as, age of greater than 60 years, daily dose more than 6.5 mg/kg; use of the drug more than 5 years, obesity, preexisting retinal disease and, renal or liver failure. Early detection of the maculopathy is of critical importance to discontinue Plaquenil in order to stop or slow retinal damage. Unfortunately, clinically evident early structural changes can be subtle and usually preceded by abnormalities in functional tests such as visual field examination, multifocal electroretinography (mfERG), fundus autofluorescence (FA) imaging, and optical coherence tomography.
Recent findings suggest that Plaquenil toxicity can develop among patients that are taking the drug at a daily dose lower than the suggested “safe” dose and/or have been on Plaquenil for shorter than five years. Unfortunately, cessation of Plaquenil intake may not be a remedy since not infrequently, patients will develop objective evidence of progression despite discontinuation of the drug. Thus, the possibility of toxicity should not be disregarded and close monitoring of the ocular findings is required.
As a precaution, patients treated with Plaquenil are advised to get a baseline eye exam prior to starting the drug and then annually thereafter
This information provided by Neil I. Stahl, MD & Tongalp H. Tezel, MD was first printed in the The Moisture Seeker, SSF's patient newsletter for members.
Sicca is a word derived from the Latin siccus, meaning “dry.” Dryness of the exocrine glands, particularly the eyes and mouth, is referred to as “sicca syndrome” or “sicca complex” when there is no evidence of autoimmune disease present. While sicca symptoms occur in the vast majority of Sjögren’s patients, not everyone with these symptoms has Sjögren’s. Because of this, it is important to establish an autoimmune cause for the dryness. Sometimes other causes may be found, such as radiation therapy to the head, certain medications, or Hepatitis C or HIV infections. If no cause is found, the patient should be followed carefully for possible Sjögren’s because it sometimes takes years for the diagnosis to become clear.
Dryness from Sjögren’s may affect any organ in the body that secretes moisture. In addition to changing the quantity and quality of saliva and tears, dryness may manifest in the airways, nasal passages, sinuses, throat, skin, and in women, the vagina. Some Sjögren’s patients initially present with recurrent sinus infections, severe vaginal dryness, chronic dry cough, and so on. All types of specialists, not just eye doctors and dentists, need to keep Sjögren’s in mind as a diagnostic possibility, especially when dryness is severe, persistent, or accompanied by systemic symptoms such as fatigue and widespread muscle and joint pain. Dryness can be quite serious, causing dental disease, eye pain and even visual impairment. However, these issues should not detract from the often missed point that Sjögren’s is much more than sicca syndrome. Sjögren’s is a serious systemic autoimmune disease that can affect almost any organ in the body.
-Sarah Schafer, MD
This information was first printed in the April issue of The Moisture Seeker, SSF's patient newsletter for members.
As anyone with Sjögren’s knows, many things can exacerbate the discomfort of dryness, while there are other factors that can either soothe the dryness or advance a condition of moisture that can prevent it.
Here are things you can do on a day-to-day basis that can alleviate your symptoms and help you feel and look better.
- Do Exercise
Regular exercise unquestionably does all sorts of good things for us. The main medical benefit is perhaps the power to decrease inflammation, which it does through the release of endorphins. For that reason, exercise contributes to the health of the ocular surface. Regular exercise- at least 20 minutes of exercise that increases your heart rate 5x a week- is highly recommended for dry eye sufferers.
- Do Take Showers
A hot bath can be a relaxing indulgence, but the steam tends to rise away from you. It's much better to be upright in a shower, with the steam coming at you constantly. Moreover, whether you intend it or not, water from the shower head or bouncing off your body, splatters into your eyes and literally cleans them out.
- Do Catch some Zzzzzzs
I cannot emphasize enough how important getting as much sleep as possible is to mitigating the discomfort of dry eye. A deep sleep, replenishes the tear film and soothes the ocular surface.
- Do Drink Water
You should drink 6-8 glasses of water a day. That's water- plain and simple- not sodas, sugary juices or artificially flavored drinks. Water is needed by all of the body's organs- by the skin, the kidneys, the liver, the heart and the eyes as well.
- Do Keep up with Friends & Family
There is increasing evidence that social interaction is as good for us as exercise, a good night's sleep or eating natural food. It is also a fact that the smile you wear while you're happy with friends can actually reduce the exposure of the ocular surface.
- Don't get Stressed
Stress can affect many other factors that have a direct impact on dry eye: sleep, your blink rate, and even what you eat. All of that leads to the kind of inflammation that can exacerbate a range of ailments, including a dry eye disorder. There are many different kinds of stress and there are many ways to manage it. Find the way that works for you, and learn as best you can to keep stress at a minimum.
- Don't work your eyes too long
Perhaps the most important thing to avoid if you suffer from dry eye is a long stretch of consecutive visual tasking. Whether it's working at a computer, watching television or reading- break up the time you spend doing it.
- Don't Smoke, Drink Alcohol or Caffeine
Smoke, alcohol and caffeine all dehydrate the body, including the eyes. Be aware of what these activities are doing to your dry eye, and try to reduce the frequency or eliminate all three if you can.
This information is provided by Robert Latkany, MD
Author of "The Dry Eye Remedy" and Founder & Director of the Dry Eye Clinic at the New York Eye & Ear Infirmary
Thank you to our Dry Eye Awareness Month Partner:
July is Dry Eye Awareness Month! The Sjögren’s Syndrome Foundation partners with various companies during July to help educate the public about dry eye symptoms, treatment options, and the possible cause being Sjögren’s. We hope you enjoy our July blogs aimed to promote dry eye awareness and education.
Q) Many eye drops claim to have disappearing preservatives. Are these the equal of preservative- free drops, or should they still be used like eye drops with standard preservatives?
A) The development of “disappearing preservatives” has allowed eye drops to be formulated in multi-use dropper bottles for convenience without the risk of surface damage that can occur with the more potent and persistent preservatives. The mechanism by which such new preservatives “disappear” is usually due to chemical changes in the preservative that occur upon exposure to air or the tear film. The most common such chemical reaction is oxidation of the preservative, turning it into an inactive molecule. It must be remembered, nevertheless, that the inactive molecule can be something to which sensitive patients may react. It is worthwhile, therefore, that the patient be alert to any intolerance of such medication which can occur as irritation, discomfort or red eyes. The “disappearing preservative” eye drops can be used up to four times a day in most cases without difficulty and some patients can use them even more frequently than drops with regular preservatives. It should be remembered that other eye drops, particularly those used to treat glaucoma, can contain preservatives as well and, therefore, it is important for patients to keep track of how many drops are being instilled in the eye during the day.
Truly preservative-free eye drops contain no such preservative chemicals but, therefore, require special packaging that limits the amount of the solution in the dropper to usually only one or two drops. The challenges of the smaller packaging can be a nuisance, but if the patient is sensitive to even the “disappearing preservative” this nuisance can be worth the better tolerance to the lubricant.
-Gary N. Foulks, MD
Thank you to our Dry Eye Awareness Month Partner:
While the exact reasons are unknown, many patients with Sjögren’s suffer from gastroesophageal reﬂux disease (GERD). This can cause a wide variety of symptoms that can be mistaken for other conditions. Symptoms may include persistent heartburn and/or regurgitation of acid, stomach pain, hoarseness or voice change, throat pain, sore throat, difﬁculty swallowing, sensation of having a lump in the throat, frequent throat clearing and chronic cough (especially at night time or upon awakening).
Tips for combating gastroesophageal reﬂux in the throat:
1. Avoid lying ﬂat during sleep. Elevate the head of your bed using blocks or by placing a styrofoam wedge under the mattress. Do not rely on pillows as these may only raise the head but not the esophagus.
2. Don’t gorge yourself at mealtime. Eat smaller more frequent meals and one large meal.
3. Avoid bedtime snacks and eat meals at least three-four hours before lying down.
4. Lose any excess weight.
5. Avoid spicy, acidic or fatty foods including citrus fruits or juices, tomato-based products, peppermint, chocolate, and alcohol.
6. Limit your intake of caffeine including coffee, tea and colas.
7. Stop smoking.
8. Don’t exercise within one-two hours after eating.
9. Promote saliva ﬂow by chewing gum, sucking on lozenges or taking prescription medications
such as pilocarpine (Salagen®) and cevimeline (Evoxac®). This can help neutralize stomach acid and reduce symptoms. Check the SSF's Product Directory (free of charge to all SSF members) to see the products available.
10. Consult your doctor if you have heartburn or take antacids more than three times per week. A variety of OTC and prescription medications can help but should only be taken with medical supervision.
The SSF thanks Soo Kim Abboud, MD for authoring this Reﬂux and Your Throat Patient Education Sheet. Dr. Abboud is an Assistant Professor with the Department of Otolaryngology, Head and Neck Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
Joint and muscle pain in Sjogren’s syndrome may result from a variety of causes including inflammation, fibromyalgia, age-related osteoarthritis, vitamin D deficiency, hypothyroidism etc.
Work with your rheumatologist to identify the specific cause(s) of your pain and find the best treatment regimen for you. Maintain a positive attitude and be an active partner in the management of your pain.
The tips below will also help:
- Become knowledgeable about your medications
- Get a good night’s sleep
- Maintain a regular sleep schedule.
- Set aside an hour before bedtime for relaxation. Listen to soothing music.
- Consider taking a warm bath before going to bed
- Make your bedroom as quiet and comfortable as possible.
- Avoid caffeine and alcohol late in the day.
- Avoid long naps during the day.
- Exercise regularly with the goals of improving your overall fitness and keeping your joints moving, the muscles around your joints strong and your bones strong and healthy
- A physical therapist, occupational therapist, or your health-care provider can prescribe an exercise regimen appropriate for your joint or muscle problem.
- Start with a few exercises and slowly add more.
- Make your exercise program enjoyable. Do it with your spouse or a friend. Include recreational activities, such as dancing, walkingand miniature golf.
- Try different forms of exercise, such as Tai chi, yoga and water aerobics.
- Balance rest and activity
- Pace yourself during the day, alternating heavy and light activities and taking short breaks to rest.
- Control your weight
- Protect your joints and muscles
- Use proper methods for bending, lifting, and reaching.
- Use assisting devices, such as jar openers, reach extenders and kitchen and garden tools with large rubber grips that put less stress on affected joints.
- Use various therapeutic modalities that can relieve joint and muscle pain
- Use heat (heating pads, warm shower or bath, paraffin wax) to relax your muscles and relieve joint stiffness.
- Use cold packs to numb sore joints and muscles and reduce inflammation and swelling of a joint
- Consider massage therapy.
- Practice relaxation techniques, such as guided imagery, prayer and self-hypnosis.
Thank you Alan Baer, MD for these tips. Dr. Baer is an Associate Professor of Medicine, Director, Jerome L. Green Sjogren’s Center, Johns Hopkins University School of Medicine
This is a revival of an essay I wrote ten years ago, originally entitled 11 Types of Fatigue. I’ve been asked by the Sjögren’s Foundation to give it new life. I thank them for the opportunity and hope that in the ten years that have passed since the original essay, there is increased acknowledgment of fatigue as a major factor in Sjögren’s. I’ve decided to add two new types of fatigue to the list, which is by no means meant to be exhaustive.
A quick update: The friend mentioned below was my first friend with Sjögren’s and as such, has always been very special to me. We still speak in a kind of shorthand, as I do with most if not all of my Sjögren’s friends. The friends I have made over the years are the only good outcome of this disease. As a group, we understand what it means to say that we are ‘fine’. We know that when we say we are “tired”, it means really tired, i.e. that something is going on, something out of the ordinary, beyond the everyday feelings of fatigue. ‘How are you?’ is not a question in our culture. It is a greeting. As such, it deserves a real answer only in those circumstances where there is an understanding that the person asking truly wants to know.
For those of you who haven’t seen it, here is most of the original article with a few additions and revisions:
"How are you?" I asked a friend who has Sjögren’s. "Tired," she said, "how are you?" "Tired," I replied, knowing we understood each other. We were talking about a special brand of fatigue. Later that day, a friend who did not have Sjögren’s asked me the same question. "How are you?" she said. "Fine" I responded, thinking it was the simpler way to answer a basically rhetorical question.
Not everyone with Sjögren’s suffers from fatigue, but many of us do. According to a 2012 survey done by the SSF, fatigue was the third most prevalent and disabling symptom of Sjögren’s. Fatigue has been a problem more disabling than dry eyes or dry mouth for me. I long for normal energy and the ability to sustain activity, any activity. I long for the kind of fatigue that gets better with a good night's sleep. I want to be able to do things spontaneously. I wish I did not have to pace myself or plan rest stops. I wish that I could just get up and go, but, reluctantly and somewhat resentfully, I know that fatigue is a permanent part of my life. Having decided that if you must live in a particular landscape, you should learn the subtleties of the territory. I've come up with the following subtypes. Your experience may vary:
1. This is the inherent fatigue that I attribute to the inflammatory, autoimmune nature of Sjögren’s. It's with me all the time. It differs from normal fatigue in that you don't have to do anything to deserve it. It can vary from day to day but is always there. For me, there appears to be a correlation between this kind of fatigue and sed rate (ESR). When one goes up, so does the other. I don’t know how often this phenomenon occurs. I could also call this my baseline fatigue, which fluctuates and gets better or worse. All of the following are superimposed on this basic fatigue.
2. If I push myself too far and ignore the cues my body is sending me to stop and rest, my body will fight back. When I do more than I should, the result is an immobilizing fatigue. It comes on after the fact, i.e., do too much one day and feel it the next. If I push myself today, I very likely will have to cancel everything tomorrow. An extended period of doing more than I should will almost certainly cause a flare.
3. This 'crumple and fold' phenomenon makes me resemble a piece of laundry. It comes on suddenly, and I have to stop whatever I'm doing and just sit down (as soon as I can). It can happen anywhere, at any time. It is the kind of fatigue that makes me shut off the computer in mid-sentence. It is visible to those who are observant and know what to look for, even though I make gargantuan efforts to disguise the fact that it is happening.
Weather related fatigue
4. Not everyone has this particular talent, but I can tell that the barometric pressure is dropping while the sky remains blue and cloudless. I feel a sweeping wave, a malaise, that sometimes lifts just after the rain or snow has started. Likewise, I know when a weather front is moving away, even while torrents of water are falling from the sky. I feel a lightening in my body and begin to have more energy. This kind of fatigue is accompanied by an increase in muscle aches and joint pain.
Molten lead phenomenon
5. This fatigue is present when I open my eyes in the morning and know that it is going to be a particularly bad day. It feels as if someone has poured molten lead in my head and on all my limbs while I slept. My muscles and joints hurt, and doing anything is like walking with heavy weights. It is often associated with increased symptoms of fibromyalgia and sometimes helped by heat and massage.
6.Tired-wired is a feeling that comes from certain medications, such as prednisone, too much caffeine, or too much excitement or perhaps it is just a function of Sjögren’s. My body is tired but my mind wants to keep going and won’t let my body rest.
7. Flare-related fatigue is an unpredictable state of increased fatigue that can last for days or weeks. It may be caused by an increase in disease activity or an undetected infection. If the latter, it either resolves on its own, or eventually presents other signs and symptoms that can be diagnosed. Additional rest is essential to deal with this kind of fatigue, but rest alone will not necessarily improve it or make it go away. Once a flare begins, it is impossible to predict where it will go or how long it will last.
Fatigue induced by other physical conditions
8. Fatigue related to other physical causes, such as thyroid problems or anemia superimposed on Sjögren’s. This kind of fatigue makes you feel that you are climbing a steep hill when you are really walking on level ground. It resolves once the underlying organic condition is diagnosed and treated. Thyroid problems and anemia are both common among Sjögren’s patients, but many other kinds of fatigue may be superimposed.
Fatigue that impairs concentration
9. Fatigue that impairs concentration precludes thought, makes me too tired to talk, think or read. Fatigue robs me of memory and encloses me in a fog of cotton wool so thick I can't find my way out until the fog miraculously lifts. For me, brain fog goes hand-in-hand with other kinds of pernicious fatigue.
Stress, distress, anxiety or depression
10. Stress, distress, anxiety or depression all can create a leaden kind of emotional fatigue that can be as exhausting as one due to physical causes. Although some people do not associate their increased fatigue with emotional states, many are aware of the effects of increased anxiety and depression, even if they cannot control what they feel. Intense emotion is very draining. Stress, anxiety and depression all are known to disrupt sleep.
Fatigue that comes from not sleeping well
11. Some people with Sjögren’s have trouble both getting to sleep and staying asleep. Some wake up in the morning feeling as if they had never slept at all. Many aspects of Sjögren’s affect sleep: being too dry, in too much pain or malaise; multiple trips to the bathroom, the need for water or to put in eye ointment all deter a good sleep. Lack of restorative sleep increases fatigue.
And two new ones:
Fatigue that comes with normal aging
12. I’m old enough for Medicare now and my friends are more tired too, although they seem to be able to do two or three or even four times what I can do on any given day. In fact, the gap between what they can do and what I can do just seems to be growing, despite my best efforts. It’s been a long time since I tried to keep up, but it still hurts that I can’t.
Chronic Illness Fatigue
13. Fatigue that comes from a chronic illness that just won’t quit. We’ve all heard the expression “sick and tired of being sick and tired” and that phrase truly captures what many of us feel. I would take it one step further. There’s a fatigue that comes with the uncertainty of a chronic disease. It’s a debilitating fatigue born of never knowing what will come next. The chronicity of Sjögren’s can wear me down and I have to make special attempts not to let it. When these attempts don’t work, I wait a while and try to find something else that distracts me from my illness.
It's difficult to explain the unnatural quality and intensity of this fatigue to someone whose only experience has been with what is normal. We're not talking about the same stuff. It’s apples and artichokes. Sjögren’s fatigue is pervasive. It assaults everything I do. There isn’t a part of my life that hasn’t been touched by it. It is there even on my happiest days.
Because people don’t understand, it’s often misinterpreted. "Is it depression?" a health care professional who didn't know much about Sjögren’s asked me. I tensed. Was he saying it was all in my head? I began to get angry but then gave him the benefit of the doubt. I put my first reaction aside and decided he was trying to understand. I was relating something outside his frame of reference, and he was attempting to find a point with which he could identify.
When you describe Sjögren’s fatigue to someone who has never experienced it, you are asking him or her to think outside the box. You want them to understand an experience that is common to those who have Sjögren’s and many other autoimmune diseases but rare otherwise. Perhaps their first reaction will be negation or denial. While it’s always difficult to encounter expressions of disbelief, it is not uncommon. I tried to see it as an opportunity to educate.
"No," I said to the doctor who asked about depression, "think of it as a never-ending flu that varies in intensity but never goes away." He grimaced.
This article was first published in the November/December Issue of The Moisture Seekers. Suggested reading:The Sjögren’s Syndrome Survival Guide, by Terri Rumpf, PhD, author of 13 Types of Fatigue article, and Katherine Moreland Hammitt, SSF Vice President of Research, this SSF best seller is often referred to as a “support group in a book."
You are the voice of the Sjögren’s Syndrome Foundation and the reason for its existence. April is Sjögren’s Awareness Month and we want to help by giving you 30 ways to increase awareness through a different Sjögren’s fact or tip every day on our Facebook and Twitter pages.
Awareness comes in many forms and we encourage you to follow us for the next 30 days and look for opportunities to talk about Sjögren’s and the SSF in your life. You can help make Sjögren’s a household name!
#TipTuesday: Talking about your disease can help reduce the anxiety associated with a chronic illness, but the SSF knows how difficult that can be when friends, family and some physicians can’t even pronounce Sjögren’s (SHOWgrins). With an estimated 4 million Americans living with Sjögren’s, do the people in your life know how to pronounce it?
Please share this with your family and friends to help raise awareness of Sjögren’s this April!