In addition to celebrating Heart Awareness, February is also Black history month. I can remember growing up and being inspired watching the Williams sisters rise to the top of their tennis games. I loved being able to watch these girls/women who I could identify with display such amazing athletic ability.
I still watch them both in amazement every time they step onto the court! The whole world has watched Venus Williams win so many major tournaments in her career so it came as a surprise when she announced her withdrawal from the 2011 US Open due to her diagnosis of an autoimmune disease.
Venus has Sjorgren’sSyndrome which is a disorder of the immune system that can affect the gland’s ability to produce saliva and tears. During her interview with Good Morning America in 2011 after she withdrew from the championship, she described how she struggled with symptoms for several years prior to her diagnosis. She had noted extreme fatigue, stating that she didn’t have the stamina to maintain her daily training. She experienced dry eyes, dry mouth in addition to joint pain and swelling in her hands.
She reported having trouble lifting her arms with the racket over her head and felt heaviness in her arms. She was also worried about losing feeling in her hands and having numbness. She started to have shortness of breath but was previously diagnosed with exercise-induced asthma that wasn’t improved with medications.
Right before the 2011 US Open, she finally received a diagnosis of Sjogren’s Syndrome and had some challenges dealing with and accepting the diagnosis initially. This condition can affect someone at any age, however usually females in their forties and older are more commonly affected.
HALLMARK SYMPTOMS
Venus describes having Primary Sjogren’s Syndrome since there is no other rheumatic disease present. Autoimmune thyroid disease or Hashimoto Thyroiditis can be seen with Primary SS. Secondary Sjogren’s Syndrome can be seen with Lupus and Rheumatoid Arthritis. Most individuals living with this autoimmune disease (where the body’s cells attack its own organs ) express having dry eyes and dry mouth ( sicca symptoms or xeropthalmia and xerostomia) as the hallmark symptoms.
This dryness occurs because the mucous-producing glands have decreased the production of tears and saliva. Individuals can complain of a “gritty or irritating/painful” sensation in the eye and a “cotton ball” feeling in the mouth. Often many people with this condition report feeling thirsty despite continuous hydration.
Debilitating fatigue and a feeling of weakness or generalized pain are commonly present.
OTHER SYMPTOMS
| a) Low-grade fevers may occur b) Parotid swelling – These glands lie behind the jaw and in front of the ear. c) Joint pain, stiffness d) Muscle aches e) Skin dryness, skin lesions or rashes associated with inflammation of small blood vessels (vasculitis) f) Nasal dryness) Hoarseness and chronic dry cough from throat and trachea dryness g) Raynaud phenomenon- color changes and constriction of blood vessels in extremities from cold exposure i) Vaginal dryness j) Reflux and difficulty swallowing |
DIAGNOSIS
Like Venus, sometimes this condition is difficult to diagnose initially because symptoms may mimic other diseases and individuals may present in different ways. A good clinical history and physical examination by a physician is necessary.
The rheumatologist will check for inflammation markers in the blood, a urinalysis, white blood cell and platelet levels as well as specific proteins of the immune system.
These antibodies may include:– Anti- nuclear antibodies (ANA)- Anti- SSA/SSB- Rheumatoid Factor- Immunoglobulins ( High levels or hypergammaglobulinemia can be seen)
An eye examination is often conducted. An ophthalmologist can measure the amount of dryness or tear production in the eye with a Schirmer tear test. The cornea should also be examined. Other tests can analyze the function of salivary glands. A Sialogram/ Salivary Scintigraphy will look at the saliva flow in the mouth and salivary glands. A physician may also recommend a lip/minor salivary gland biopsy to look at the types of inflammatory cells in the glands.
COMPLICATIONS
Excessive dry eyes from decreased tear production can lead to photosensitivity as well as damage to the cornea. Sometimes infection of the eye and eyelid can occur (conjunctivitis, blepharitis). These individuals are prone to develop dental caries/cavities or oral yeast infections (thrush) because of low saliva production. Please have frequent dental visits!!!
Inflammation in various organs can be seen.-Lungs (bronchitis, pneumonia)-Heart (pericarditis)-Liver (hepatitis, cirrhosis)
Inflammation of the kidneys, thyroid, joints, skin can also occur. Neurological involvement or peripheral neuropathy may be detected.
It is important to know that lymphoma, cancer of the lymph nodes, can develop in a small percentage of patients so it is imperative to follow up with a physician and have blood tests checked and frequent visits!
TREATMENT
There is no cure but most therapies do target specific symptom relief.
1. Over the counter artificial tears, eye drops and lubricants can be used initially. If there is no relief, then medications to decrease eye inflammation will be prescribed. Restasis ( cyclosporine eye drops) is used for moderate to severe dry eyes.
2. Salagen (pilocarpine) and Evoxac (cevimelene) can increase saliva flow andproduction. Beware of side effects such as stomach pain, flushing and sweating. Sugarless gum can be used as well.
3. Nonsteroidal Anti-inflammatory agents like naproxen or ibuprofen can be used forjoint pain.
4. Antifungal medications treat oral yeast infections.
5. Immunosuppressants such as Hydroxychloroquine and Methotrexatetreat
systemic symptoms like fatigue and joint pain.
6. Surgery involving inserting plugs into tear ducts may be recommended by specialist.
7. Vaginal lubricants and moisturizers may help for dryness.
8. Nasal saline spray is helpful.
9. Rituximab, an immunosuppressant that targets specific cells can be used to treat lymphoma associated with this disease.
LIFESTYLE TIPS
Venus Williams has been outspoken about changing her eating regimen to a
What else can you do?
- Stop smoking!!!
- Increase water intake.
- Decrease alcohol and caffeine consumption.
- Increase humidity in your environment.
- Engage in frequent teeth brushing and use of antibacterial mouthwash.
- Limit candies.
- Get some sleep!!
- Make sure to follow up with your internist, rheumatologist, dentist, And ophthalmologist!
People living with Sjorgren’s Syndrome can lead healthy and productive lives. Venus went on to win the 2012 Wimbledon Doubles Championship and played in the 2017 Australian Open final against her sister!
She has been an inspiring patient advocate during the last several years and has shown courage, determination and grace while living with this autoimmune condition. Join me in celebrating her achievements during Black History Month and all those who live with Sjorgren’s Syndrome!
Please check out The Sjorgren’s Syndrome Foundation for more information sjogrens.org
References:Current Diagnosis and a Treatment in Rheumatology. 2nd editionAmerican College of Rheumatology
Disclaimer: This blog contains my personal opinion based on personal and clinical experience, tips from trainers, health coaches and lastly research. This blog does not endorse specific treatments, procedures, products. You should always consult with a doctor, nutritionist, or other healthcare professional to discuss your own health and lifestyle goals and regimen based on your medical history. Thank you for reading!