Rita Baron-Faust, MPH, CHES

Advancing Wellness on Many Fronts


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THE AUTOIMMUNE CONNECTION BLOG

What We can All Learn from Venus Williams

July 11, 2016

Tags: Sjögren’s syndrome, Venus Williams, fatigue, new clinical guidelines, Sjögren’s Syndrome Foundation, AARDA, hydroxychloroquine, biologic drugs, musculoskeletal pain

OK. I admit it: I’m a major Venus Williams fan. Actually, I root for Venus and Serena.

Watching Venus power through moments of fatigue, both mental and physical, on the tennis court is always inspiring for me. Not just because of her consummate skill, elegant play, and powerful serve (not to mention her campaign on behalf of equal pay for women in tennis), but also because she’s living with an autoimmune disease that takes a toll on body and spirit.

When Venus revealed in 2011 that she had Sjögren’s syndrome, many people expected her to retire from tennis. The fatigue and joint pain that accompanies Sjögren’s and many other autoimmune diseases can be debilitating for anyone -- even for a star athlete.

“It’s been a journey, but it’s something that I had to do. And it’s made me stronger,” Venus remarked after defeating Yaroslava Shvedova in straight sets July 4th in the Wimbledon quarter-finals. “The most difficult part of the journey is just not being in control because when you’re an athlete, you’re used to being in control, being able to work for anything. Not being able to do that is a challenge,” she said during a post-match press conference.1

Primary Sjögren’s syndrome, the result of an autoimmune attack on moisture-producing tissues, is 10 times more common in women than in men, possibly affecting as many as 3.1 million Americans. That prevalence doubles in secondary Sjögren’s, coupled with other autoimmune and connective tissue diseases.2

An online survey of nearly 8,000 patients by the American Autoimmune Related Diseases Association (AARDA) in 2015 found that 98% experience fatigue that many describe as “profound,” preventing them from doing even the simplest of everyday tasks.3 At least 60% to 70% of women with Sjögren’s are beset by fatigue.4

On July 7, the Sjögren’s Syndrome Foundation (SSF) issued the first-ever U.S. clinical guidelines for managing the disease, including fatigue.2 In those guidelines, the SSF continues to recommend aerobic exercise for fatigue (details below).

A Difficult Diagnosis

Sjögren’s can be difficult to diagnose -- especially in cases like Venus Williams', when fatigue, painful swollen joints and shortness of breath are the most noticeable symptoms -- rather than the hallmark dry eyes.

Venus was initially told she had exercise-induced asthma. “I’d go to doctors, but I never got any answers, so there was nothing I could do but keep going,” she told the New York Times in 2011.5 “It was frustrating, always being in the dark and not having anything to help me but my own will.”

That’s an experience shared by millions of women with autoimmune diseases.

Data from AARDA show that among autoimmune diseases, Sjögren’s syndrome takes the longest time to diagnose – 6.5 years of multiple doctor visits. And almost 60% of Sjögren’s patients are told their disease was “imagined” or they were just being “overly concerned.”6

When a diagnosis finally comes, it’s a relief, patients often remark. At least you know what you’re up against and that you’re not crazy.

What Treatments Help?

Problem is, all FDA-approved treatments for Sjögren’s target dry eye, dry mouth and other signature “sicca” symptoms, but none are aimed at alleviating fatigue or joint pain.

A recent review from the division of rheumatology and immunology at Duke University notes that new drug development has focused on ways to modulate the immune system and dampen chronic inflammation. But “other mechanisms may be at play, such as neuroendocrine abnormalities, that affect some of the disease manifestations, such as fatigue,” and could be potential targets for therapy.7 At this stage, the Duke reviewers note “no disease-modifying drugs have been shown in randomized, placebo-controlled trials to be effective for the treatment of primary Sjögren’s syndrome.”7

While the anti-malarial hydroxychloroquine (Plaquenil) has been used to reduce fatigue and joint pain in primary Sjögren’s, a randomized controlled trial reported in 2014 that the oral drug “failed to “failed to significantly reduce symptoms of dryness, pain, and fatigue over placebo.”7

More recently, biological drugs -- the tumor necrosis factor (TNF) inhibitors infliximab (Remicade and etanercept (Enbrel) -- have been investigated for their efficacy and safety in patients with primary Sjögren’s syndrome. But neither was found to be effective for improving disease outcomes.8, 9

And the new clinical treatment guidelines from the SSF, published online in Arthritis Care & Research strongly recommend against the use of biologics for any aspect of Sjögren’s.2

However, the SSF says hydroxychloroquine, may be considered as an option for treating fatigue – a recommendation largely based on patient experience (in one study 1/3 of patients found it helpful).2 Even though the evidence for its use against fatigue is weak, the SSF notes that hydroxychloroquine is a safe medication.4

The guidelines do recommend hydroxychloroquine as the first-line treatment for musculoskeletal pain in Sjögren’s and, if that’s not effective, methotrexate may be added.

Short-term low-dose corticosteroids may also be tried if that approach isn’t helpful, with longer-term higher doses as an option.2

Other conventional disease modifying anti-rheumatic drugs (DMARDs), such as leflunomide (Arava) and sulfasalazine can be considered. (Venus is taking medications for her Sjögren’s.)

Fighting Fatigue

So what can you do about fatigue?

The SSF guidelines, drafted by a panel of experts with input from Sjögren’s patients, recommend daily exercise as an effective way to fight fatigue, citing a number of clinical trials (considered the gold standard of medical research) that found exercise an effective fatigue fighter.2

The SSF generally recommends women gradually work up to 25 minutes a day of aerobic exercise (and, no, you needn’t play tennis!).9

Other ways to fight fatigue from the SSF: pacing yourself and not taking on too much, giving yourself a 20-minute "time-out" every few hours, and getting enough sleep.9

Some women have found adopting an “anti-inflammatory” diet helps reduce joint pain and fatigue. Venus says she juices twice daily, and eats raw, unprocessed, vegan foods.

Another approach is to imitate the diet purportedly eaten by our prehistoric ancestors – meat, fish, vegetables and fruit, but no dairy, grain, or processed foods – often dubbed the “Paleo” diet. But there's no real evidence that it works. (We’ll talk about diet and anti-inflammatory foods in a future post.)

Other advice to combat fatigue from the SSF: pacing yourself and not taking on too much, giving yourself a 20-minute “time out” every few hours, and getting enough sleep.9

But sometimes the fatigue can be too much.

In “The Autoimmune Connection,” we quoted Sjögren’s patients who said that when fatigue hit “the body can stiffen up, become painful, and feel as if it had ‘run out of energy,’ ‘given up,’ or ‘had flat batteries,’ forcing them to put their life on hold.”10

Indeed, Venus briefly curtailed play after dropping out of the U.S. Open in 2011 because of fatigue and back pain. “The fatigue is hard to explain unless you have it,” Williams remarked at the time.5 “Some mornings I feel really sick, like when you don’t get a lot of sleep or you have a flu or cold. I always have some level of tiredness. And the more I tried to push through it, the tougher it got,” she told the New York Times.

But her passion for the game and her determination to win ultimately propelled Venus back into the game. Both qualities were on ample display at Wimbledon, both in her victory over Shvedova as well as in her defeat by Angelique Kerber in the semis July 8.

The very next day, she and sister Serena went on to win their 14th Grand Slam doubles match -- just a couple of hours after Serena captured her 22nd Grand Slam singles title against Kerber.

“It’s easy to be afraid. You have to let fear go,” Venus told the press at Wimbledon earlier in the tournament. “You’ve got to believe in yourself. No matter how things are stacked against you, you just have to every time.”1

Venus Williams defeated on the court? Occasionally. Defeated in life. Never.

That’s a lesson we can all learn -- even if we’re not tennis champs.

References:


1 Venus Williams: Quarter Final. The Championships, Wimbledon 2016. Wednesday, 6 July 2016. http://www.wimbledon.com/en_GB/news/articles/2016-07-06-/venus_williams_quarterfinal.html

2 Carsons SE, Vivino, FB, Parke A, et al., Treatment Guidelines for Rheumatologic Manifestations of Sjögren’s : Use of Biologics, Management of Fatigue and Inflammatory Musculoskeletal Pain. Arthritis Care and Research. 2016; Jul 7. doi: 10.1002/acr.22974. [Epub ahead of print]. http://onlinelibrary.wiley.com/doi/10.1002/acr.22968/abstract. Accessed July 8, 2016.

3 American Autoimmune Related Diseases Association, Autoimmune Patient Survey on Fatigue, Released March 23, 2015. http://www.aarda.org/wp-content/uploads/2015/03/EMBARGOED+PressReleaseFatigueSurvey.pdf
4 Mengshoel AM, Norheim KB, Omdal R. Primary Sjögren’s syndrome: fatigue is an ever present,
fluctuating, and uncontrollable lack of energy. Arthritis Care & Research. 2014;66(8):1227–1232. doi:10.1002/acr.22263.

5 Crouse, K. Williams Says She Struggled With Fatigue for Years. New York Times, September 1, 2011.
http://www.nytimes.com/2011/09/02/sports/tennis/2011-us-open-venus-williams-describes-fights-with-fatigue.html

6 American Autoimmune Related Diseases Association/National Coalition of Autoimmune Patients Survey Data, 2014.

7 Holdgate N and St. Clair WE. Recent advances in primary Sjögren’s syndrome. Version 1. F1000Res. 2016; 5: F1000 Faculty Rev-1412. Published online 2016 Jun 17. doi: 10.12688/f1000research.8352.1 PMCID: PMC4916986. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4916986/ Accessed July 4, 2016.

8 Gottenberg JE, Ravaud P, Puéchal X, et al. , Effects of hydroxychloroquine on symptomatic improvement in primary Sjögren syndrome: the JOQUER randomized clinical trial. JAMA. 2014;312(3):249–58. 10.1001/jama.2014.7682.

9 “Fatigue Fighters in Sjögren’s,” January 16, 2014. The Sjögren’s Syndrome Foundation, http://info.sjogrens.org/conquering-sjogrens/bid/331597/Fatigue-Fighters-in-Sj-gren-s

10 Baron-Faust R, Buyon JP. “The Autoimmune Connection, 2nd Edition,” 2016, McGraw-Hill, NY. Page 177.