April is Sjögren’s Awareness Month
This April, Real World Health Care is honored to shine a spotlight on a rare autoimmune disease that most people have probably never heard about. Sjögren’s (SHOW-grins) is a systemic autoimmune disease that affects the entire body. Along with symptoms of extensive dryness of the eyes and mouth, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies and lymphomas.
An estimated one to four million people in the United States currently live with Sjögren’s, including tennis star Venus Williams. According to the Sjögren’s Foundation, nine out of ten Sjögren’s patients are women. The average age of diagnosis is late 40s, although it can occur in all age groups, including children, and in both sexes.
“Sjögren’s is an incredibly complex disease, and it can be difficult to understand,” said Janet Church, president and CEO, Sjögren’s Foundation. “That is one of the reasons Sjögren’s Foundation encourages patients to share their stories about what it’s like to live with the disease.”
Coming Together to Conquer Sjögren’s
Sjögren’s Awareness Month was first established in 1998 when late New York Congresswoman, Louise Slaughter, read it into the Congressional Record. Today, the Sjögren’s Foundation works to keep the spirit of national awareness alive every April. This year, the Awareness Month theme is Coming Together to Conquer Sjögren’s.
Throughout the month of April – and beyond – the Foundation will be highlighting the many Faces of Sjögren’s and expanding its #ThisIs Sjögrens online campaign on Facebook, Twitter, and LinkedIn. Every day in April, the Foundation will highlight a different patient and share a glimpse into their life living with the disease. The goal is to not only raise awareness of Sjögrens, but also to share how patients are effectively coping with the disease and to offer advice for others.
“While each daily post will only give a small insight into Sjögrens, by the end of the month, we hope these 30 posts will showcase the complexity of the disease and the strength of our community,” said Church.
Supporting the Sjögrens Community Through COVID
People living with Sjögrens have needed extra support throughout the COVID pandemic, said Church. Because many people with the disease take immunosuppressants to help manage their symptoms, they are more susceptible to catching colds, flus and the novel coronavirus than the average public.
“Staying at home and socially isolating are regular facts of life when you are taking immunosuppressants for an immune-deficiency disease like Sjögrens,” Church said. “However, during the COVID pandemic, people with Sjögrens haven’t been able to physically connect with their normal support systems and loved ones. That lack of in-person support leads to fear and stress, which can compound physical symptoms.”
While some with Sjögrens may have considered suspending their use of immunosuppressants to keep their immune systems strong during the pandemic, the Sjögrens Foundation strongly recommends patients not stop the use of any drugs, including immunosuppressants, without first talking with their physicians, as some drugs need explicit steps to be taken when decreasing or stopping medication.
Another COVID-related factor driving fear and stress among Sjögrens patients is the lung complications that can co-occur with the disease, which can occur in up to two-thirds or more of patients. According to Church, patients and health care providers may not be attuned to which pulmonary symptoms are related to their Sjögrens and which may be attributed to COVID.
To help the Sjögrens patient community throughout the COVID pandemic, the Sjögrens Foundation has offered support and guidance through an online FAQ and vaccine position statement, as well as through virtual support groups, online Town Halls, and a virtual National Patient Conference.
“Shifting to online formats for support groups, our national conference and other events has opened up accessibility and allowed us to reach more people,” Church said.
The Foundation also has been active in addressing the shortage of Hydroxychloroquine (HCQ), a medication taken by about 40 percent of Sjögrens patients, and which was anecdotally discussed as being effective for the treatment of COVID-19. While HCQ is not an immunosuppressant, it does improve the way Sjögrens patients’ immune systems work and thereby improves symptoms. It works similarly in other immune diseases like rheumatoid arthritis and lupus.
“Shortages of HCQ early in the pandemic created havoc among patients, who were worried that they may not be able to access their needed medications,” Church said. “We have been working with government groups, insurance commissioners, industry associations and boards of pharmacy to enlighten them about the shortage issue and work toward increasing supply of this critical drug for autoimmune patients.”
Clinical Trials and Patient Outcomes
Throughout the COVID pandemic, the Sjögrens Foundation has not lost sight of its commitment to the development of new therapeutics for the disease.
The Foundation encourages patients to participate in clinical trials and acts as a conduit to the pharmaceutical industry to get companies interested and engaged in developing treatments for the entire disease, not just one symptom.
“There is an unmet medical need among Sjögrens patients,” Church said. “They are clamoring for treatments to improve their quality of life. We need to make sure their voices are heard when companies are evaluating development priorities and researchers are designing trials.”
To that end, the Foundation has connected researchers with Foundation staff and Sjögrens patients to offer insights on trial design, participate in focus groups and interviews, and review marketing materials. These input sessions have addressed issues including clinical trial eligibility, timing and requirements around stopping or modifying current treatments to participate.
“We’re also helping researchers develop better outcome measures for their trials,” Church added, noting that the standard EULAR Sjögrens Syndrome Disease Activity Index (ESSDAI) used to measure disease activity doesn’t address quality-of-life concerns such as dryness and fatigue. “We’re pushing for more patient reported outcome measures to reflect how symptoms affect quality of daily life. What does a specific ESSDAI score mean in terms of how the patient feels, whether they could go to the store that week or put in a productive day at work?”
The Foundation also supports research to uncover biomarkers for Sjögrens and has joined the Foundation for the National Institutes of Health (FNIH) biomarkers consortium, a public-private biomedical research partnership that endeavors to discover, develop and seek regulatory approval for biomarkers to support new drug development, preventative medicine and medical diagnostics.
“Diagnosing Sjögrens typically requires a number of objective and subjective tests,” Church concluded. “Wouldn’t it be great if instead, physicians could administer a simple blood, saliva or tear test that would effectively diagnose the disease as well as its severity and projected course? These questions are the type of innovative, out-of-the-box, and meritorious research projects we want to fund to make real impacts on patient quality of life.”