The 2020 HLTH VRTL conference focused on how the COVID-19 crisis is accelerating trends that are transforming healthcare. In this article, I will describe the picture painted by the speakers of what the soon-to-be post-COVID health care world will look like.
I viewed the conference through my multifocal lens as a financial analyst, consultant, and patient and in keeping with my mission at Your Autoimmunity Connection, I kept one question in mind:
How could these accelerating post-COVID trends help patients with the subset of chronic inflammatory diseases with which the organization is most concerned: autoimmune and autoinflammatory disorders?
In Part 1 of this story, I described how COVID-19 has accelerated the use of telemedicine and remote patient monitoring. At the same time, it has speeded the migration from FFS by showing that a VBC reimbursement model can be a more reliable revenue stream for practices in disruptive times. These disruptor-driven trends have also spurred new primary care delivery models and consumer competition for the digital front door as described below. Companies highlighted in Part 1 were Amwell, Oscar Health, Massachusetts General Physicians Organization, and Aledade.
Novel, experimental primary care delivery models are emerging from inside and outside the current system. Not only big players (payers, providers, practice systems, and innovative employers), but also DTC online and walk-in acute care, “doc-in-a-box” online providers, drugstore, retail, and tech giants are all competing to be healthcare digital front doors.
It has been widely recognized for delivering high-quality healthcare at a low cost through an integrated delivery system model.
Jaewon Ryu, the President and CEO of Geisinger, said,
“Primary Care is the front door and it is how we drive affordability. We have been on a journey during the last three years on which we have been redesigning primary care. We believe there is an opportunity to introduce different flavors for those with multiple chronic conditions.”
As a large integrated delivery system, Geisinger has already developed products and services that could potentially help autoimmune patients, such as:
These are obvious targets for geriatric populations, but what about other chronic conditions that could benefit from home visits? That includes more severe autoimmune and autoinflammatory conditions, including chronic fatigue and post-viral (led by post-COVID) syndromes.
One Medical is a membership-based, technology-powered primary care model-of-care. It offers seamless digital health and inviting in-office care. The offices are convenient to where people work, shop, live, and go online. Their vision is to delight millions of members with better health and better care while reducing costs. Its mission is to transform health care for all through a human-centered, technology-powered model.
Their suite of services goes beyond the traditional primary care to include
These types of services give patients more convenient access.
Amir Dan Rubin, CEO of One Medical, explained,
“We are trying to transform healthcare through our modernized primary care approach and simultaneously address the needs of key stakeholders.”
“We have created a staff model, HMO-like approach hidden in concierge sheep’s clothing. We have all salaried primary care providers. We have built our own software using machine learning to route the messages to virtual team members. In addition, we get reimbursed at an organizational level through conventional insurance. But we have also built the underlying incentives to deliver value-based, coordinated care.”
Improvements in care collaboration and coordination, along with a more preventive mindset related to value-based care (VBC), could benefit autoimmune patients. However, care delivery models must to choose to focus on them.
One Medical’s chronic disease list, as usual, omits mention of autoimmune, autoinflammatory, and immune-related chronic inflammatory conditions. Until such conditions are more than an afterthought, these primary care innovations are unlikely to improve autoimmune/autoinflammatory care. Even though improved care for these types of patients could be a competitive edge for new companies if they seize the opportunity.
Their service offerings include same-day appointments, video visits, quick prescription refills, and more time with providers.
Fay Rotenberg, CEO of Firefly Health sees primary care as “bonding, steering, and health care fiduciary.” She says,
“We are transforming from a reactive, transactional, doctor-centered model, towards a proactive, personalized, digitally-enabled approach that supports healthy behaviors to drive better outcomes and lower costs. Our mission is to provide half-priced healthcare that is twice as good clinically and emotionally.”
Firefly Health was designed with the consumer in mind so it is easy to use. First, download the app, then connect with your care team who can help you in person, virtually, and via chat.
Their tech-enabled digital care platform, Lucian™, is built to track users’ care over time. For diagnostic testing, Firefly has an extensive, curated network of partners, some of which offer in-home testing. In addition, their teams can manage chronic diseases such as diabetes and high blood pressure. For more serious conditions, they partner with specialists.
The Firefly Health tech-enabled digital care platform and coordinated care teams offer elements that could be directed to help autoimmune and other chronic immune disorder patients better manage their daily health issues.
I can imagine how Firefly Health’s proactive personalized app could expand its functionality to help autoimmune patients with lifestyle modifications that could lower stress levels and reduce flares. But there’s no mention of chronic fatigue or autoimmune/inflammatory on their website. Instead, it’s just the usual mention of anxiety, diabetes, and high blood pressure as examples of chronic conditions.
They already have behavioral health specialists on care teams, and as we know, mental health issues, especially anxiety, loom large in autoimmune patients. Maybe mental health is the wedge to get Firefly Health to focus on immuno-inflammatory disease patients, too.
Will community pharmacies be the next neighborhood healthcare destination, an increasingly digital front door to more than prescription and OTC products?
Walgreens is part of the Retail Pharmacy USA Division of Walgreens Boots Alliance, Inc., a global leader in retail and wholesale pharmacy. They operate more than 9,000 retail locations across America, Puerto Rico, and the U.S. Virgin Islands. Walgreens is considered a neighborhood health destination serving approximately 8 million customers each day.
Walgreens pharmacists provide a wide range of pharmacy and healthcare services. To best meet the needs of customers and patients, Walgreens offers a true omnichannel experience, Their platforms bring together physical and digital health care delivery mechanisms. They are supported by the latest technology to deliver high-quality products and services in local communities nationwide.
Alex Gourlay, the Co-Chief Operating Officer at Walgreens, thinks that
“Putting together the family doctor and the local pharmacist with HIPAA-compliant health information and providing really local solutions for patients and customers both physically and digitally will open the front door of health care.”
In the future, he believes that the local pharmacy will focus on health (prescriptions, counseling, and polypharmacy management), well-being (vaccines, blood pressure, and lipids monitoring) as well as some acute illnesses. He hopes that making the pharmacy an important part of the digital front door will increase preventive health by making it more available at lower costs, especially to people of lower socioeconomic resources.
Walgreen is partnering with Village MD to open co-located doctor–led primary care clinics. They explicitly mention targeting chronic disease patients, but we suspect that, as usual, they have diabesity, heart, lung, and kidney disease in their sights, not autoimmune/inflammatory.
It’s quite easy to see how this model could be helpful for autoimmune patients, e.g., early detection of rheumatoid arthritis and thyroid disease, but we will need to see if their plans even focus on this population.
2. CVS/Aetna – from retail Rx to a broader approach to healthcare
CVS/Aetna connects consumers with the health resources of CVS Health in communities across the country as well as among Aetna’s network of providers. The goal is to remove barriers to high-quality care and build lasting relationships with consumers. They hope to make it easier for them to access the information, resources, and services they need to achieve their best health.
CVS Health footprint includes the following:
CVS Health also serves an estimated 39 million people through traditional, voluntary, and consumer-directed health insurance products and related services, including a rapidly expanding Medicare Advantage offering.
Larry Merlo, President, and CEO of CVS Health reflected that
“As a company, we are 20 months into transforming the business with CVS and Aetna coming together. The pandemic became an opportunity to reprioritize elements of the transformation journey to deliver health services, create new solutions, and make them accessible in nontraditional settings.”
He sees a future where they can play a role in fostering reliable public health information. Given the amount of dis- and misinformation around vaccines, the coming massive COVID vaccine rollouts are an opportunity for CVS as a major cold-chain distributor and vaccine dispenser to play a positive role.
Karen Lynch, EVP CVS Health and President of Aetna commented
“We were continually innovating around new products and services while consumer behavior was changing rapidly as the virus was raging. People want to access healthcare digitally. Delivery of more health care in the home than before means we will continue to see transformational changes in healthcare post-pandemic.”
For autoimmune and autoinflammatory patients, making the corner pharmacy more accessible will only be marginally helpful for some routine care (e.g., vaccinations, regular prescriptions). However, home delivery of prescription and OTC products can be a life-saver for autoimmune patients in voluntary or involuntary isolation.
However, if companies like Walgreens and CVS could figure out a more consumer-friendly way to distribute and administer infusion biologic drugs, that could be a game-changer. Getting access to biologics could be part of putting clinics inside the pharmacy that focus on the needs of autoimmune patients by streamlining some of the more routine and repetitive aspects of their care.
As I reflected on pharmacies becoming the digital front door, I wondered if dental offices, which currently see many consumers twice a year for their regular cleanings, will also try to expand their offerings. Dental practices are already screening patients for oral cancer, sleep apnea, and periodontal disease (which can trigger autoimmune disease and flares). Perhaps they can expand this list to offer thyroid screening, nutrition coaching, and other services that relate to the mouth as a gateway to the rest of the body, including to the immune system.
In addition to the expanding role of the pharmacy in care delivery, there are other new competitors. Some examples follow from women’s, family, and behavioral health. Online clinics are experimenting with novel delivery models, using women’s health needs as the digital front door.
Maven is the world’s largest virtual clinic in women’s and family health. They offer on-demand access to over 1,000 women’s and family healthcare providers. They also connect their clients with dedicated care coordinators who personally help users navigate their benefits.
According to Katherine Ryder, the Founder, and CEO of Maven,
“At Maven we owe better care to the next generation and this starts with a focus on family health.”
“one of the silver linings of COVID is that digital health companies with new virtual models to support more holistic and patient-centered care will have a more urgent place in our system today.” In addition, “we are integrating care for parents with care for kids to support the well-being of the entire family.”
Women of childbearing age are at higher risk of autoimmune, autoinflammatory and conditions like chronic fatigue syndrome than any other demographic. With Maven’s focus on family health, they could easily offer screening for autoimmune disorders, including genomic testing (which they are likely doing already for fertility and family planning purposes) to identify those who are at higher risk.
This could be integrated with their intergenerational approach, looking to detect or even prevent chronic immuno-inflammatory conditions in their patients’ children. They could help connect autoimmune patients with specialists or even coach them in lifestyle modification approaches that may reduce their chances of developing disease or at least get them earlier diagnosis and treatment.
kindbody is expanding access to fertility and reproductive health with virtual or in-clinic care for fertility, gynecology, and wellness. With transparent pricing, their website includes pricing information for services such as IVF, egg freezing, and embryo banking, as well as nutrition counseling. Care teams can include an ob-gyn, endocrinologist, physician assistant, and a variety of counselors and coaches.
According to Gina Bartasi, the Founder and CEO of kindbody-
“kindbody is on a mission to increase access to fertility and family-building care for all. 50% of our corporate team identifies as non-caucasian, 45% of our patients are women of color and 15% are GBTQ+ so when we think about creating change, we are really being mindful of today’s social inequity.”
For autoimmune patients, many of whom are women of color with low incomes (a group that faces slower diagnosis and less aggressive care), a logical extension of kindbody’s current offerings could include autoimmune screening as part of genetic testing used for family planning as well as routine thyroid screening for autoimmune thyroiditis, the most common autoimmune diseases among women.
Furthermore, kindbody could help connect autoimmune patients with specialists or even coach them in lifestyle modification approaches that may reduce flares or their chances of developing a disease or at least get them earlier diagnosis and treatment.
brightline is reinventing behavioral health care for children, teens, and their families.They deliver integrated care through innovative technology, virtual behavioral health services, and a collaborative care team focused on supporting children across developmental stages and their families.
Given the ongoing explosion of mental and behavioral health issues among children, teens, and young adults, especially under the stress of COVID-19 lockdowns, the current environment is ripe for a solution like brightline.
According to Naomi Allen, CEO and Co-Founder of brightline, we are
“providing uncommon support to the most common family challenges in behavioral health”
As shown below, brightline offers the broadest multidisciplinary care teams of any of the companies (not just the female/family offerings) profiled in these posts. This represents more silo-busting coordination of specialists than any of the other platforms. As an advocate for autoimmune patients, I am happy to see this innovative approach being used. I’ve provided screenshots to show this competitive advantage.
brightline provides access to a wide variety of providers, including:
Amongst other services, they offer tailored content, telehealth visits, treatment plan tracking, and digital exercises at home.
Particularly noteworthy is their approach to prevention, which includes a yearly pediatric well visit as shown below.
Brightline is an example of diverse multidisciplinary care teams being scaled through digital technology to help children with a wide variety of therapy programs. Better care coordination and specialist collaboration is needed by autoimmune patients, too. What’s more, autoimmune patients, an increasing number of them children, almost always have comorbid mental health issues, primarily depression and anxiety.
It would be a logical extension of the Brightline approach to reach out to their mood disorder patients to see how many in their families suffer from chronic diseases, especially autoimmune, maybe even undiagnosed immuno-inflammatory disorders.
Taking this even further, a platform like this could work for chronic GI conditions with immunological aspects such as the IBDs (inflammatory bowel diseases: Crohn’s and ulcerative colitis). Given what we now know about the gut-brain axis, GI diseases almost always involve a big mental health component.
Specifically, brightline could offer IBD patients access to care teams that include gastroenterologists as well as dieticians, health coaches, and psychologists that could help them better manage their daily lives between regular appointments. Such an approach could help IBD patients avoid flares and complications requiring costly hospitalizations or emergency visits.
In addition, applying brightline’s prevention approach to their population of families could allow earlier identification of IBD, especially in children, when early lifestyle modifications (diet triggers, food sensitivities) might prevent the disease altogether.
The HLTH VRTL conference was an excellent opportunity to capture a glimpse of some of the changes brought about by the COVID-19 pandemic. The sudden, pandemic-triggered the following changes:
2. A shift in payment models to VBC that is accelerating competition for the digital health front door (See above “Competition for the Digital Front Door”).
3. A spurring of a variety of new care delivery models. (See above “New delivery models focus on women’s, family, and behavioral health”).
4. Increasing competition for attracting patients through the digital front door that includes an expanded role of pharmacies, as well as new, digitally enabled, integrated approaches to women’s and family health.
Throughout HLTH VRTL there was almost no mention of autoimmune or other chronic immuno-inflammatory diseases, making it harder to see how these new players might help patients with these conditions.
This omission is telling in terms of where healthcare managers are focusing their efforts. While mental health is now receiving more (well-deserved) attention, autoimmune, autoinflammatory, and conditions like chronic fatigue and post-viral syndromes, easily as costly and as big a cause of human suffering as cancer, are still invisible to most payers and many providers.
Maybe the emergence of post-COVID syndromes (aka long covid) will finally prompt payer and provider interest in chronic immuno-inflammatory disorders in the same way the pandemic & lockdowns have speeded previously slow progress in telehealth and work from home.
There is no one-size-fits-all approach for autoimmune patients and much experimentation is needed, especially since so little has been done outside a handful of small companies that are mostly focused on individual autoimmune diseases.
I hope next year’s HLTH conference will offer more tangible examples of how large and small healthcare companies are using telemedicine, remote patient monitoring, new delivery care models, including VBC reimbursement to better serve the individual daily needs of chronic autoimmune patients.
Elevating and improving the role of primary care through digitally-enabled platforms and salaried physicians in both established delivery systems and new care models could also benefit immuno-inflammatory disease patients. However, this will only happen if payers and providers focus on this huge unmet need/business opportunity.
We still wonder where immune disease specialists: dermatology, rheumatology, gastroenterology, immunology, endocrinology, fit into these models. Are specialists going to become members of coordinated care teams? Or will they be in satellite practices coordinated through digital platforms that help chronically ill patients get the full spectrum of care they need? How will these new models and digital platforms tackle the complexities of chronic autoimmune and autoinflammatory disease diagnosis and treatment?
Such questions would be good ones for HLTH and its participants to focus on for 2021.
This article examines the future of healthcare in a post-COVID-19 world as gleaned from the HLTH VRTL 2020 online mega-conference. It also explores the impact of the future state on the care of chronic inflammatory diseases, my area of focus. As I engaged in virtual sessions and networking, I viewed them through my lenses as a financial analyst, business consultant, and patient.
In keeping with my mission at Your Autoimmunity Connection, I constantly kept one question in mind. How could these post-COVID changes help patients with autoimmune and autoinflammatory conditions?
I love going to conferences, from the anticipation of adventure with my well-worn suitcase and beloved travel clothes. Plus the excitement of meeting new people in the most unlikely locations: bathrooms, hallways, and elevators. For me, conferences have been intermittent opportunities to take the pulse of digital health.
In the past, HLTH has been one of the best such adventures. In 2019, I shared a room with Mette Dyhrberg, founder of Mymee, a great way to collaborate on our joint missions to improve research, diagnosis, and care for autoimmune patients.
This year, 2020, the Year of the COVID-19 pandemic, is different. With safe travel options limited, we are all adjusting to virtual conferences.
I am happy to report that the HLTH VRTL platform execution was superb. During an entire week of online presentations and one-on-one virtual meetings, the platform never crashed. In fact, that dreaded circular buffering icon never once appeared. Kudos to the HLTH VRTL tech team for creating a seamless virtual conference experience.
First impressions are key. HLTH did a great job setting the stage. They started with an inspirational video and followed with an informative statistics video.
Among the myriad of tracks, I found the daily recaps with Andy Slavitt, Leona Wen, and John Brownstein to be useful and informative. These helped me to quickly identify the healthcare delivery trends that I think might be important to chronic autoimmune and autoinflammatory patients.
Below are highlights of selected sessions, framed by my analysis and commentary on how disruptive post-COVID drivers of change might help create a new normal that will better meet the current and future needs of autoimmune patients.
In the “Future of Telehealth,” section of the HLTH Counterpoint Series, Tina Reed, Executive Editor of Fierce Healthcare, moderated contrasting perspectives on the future of telehealth from Roy Schoenberg, President, and CEO of Amwell (NYSE: AMWL), and Mario Schlosser, CEO of Oscar Health.
Amwell is a leading telehealth platform provider in the United States and globally. It connects and enables stakeholders to deliver greater access to more affordable, higher quality care. These include:
With over a decade of experience, Amwell powers telehealth solutions for over 2,000 hospitals and 55 health plan partners with over 36,000 employers, covering over 80 million lives.
Roy Schoenberg, CEO of Amwell, said,
“The coronavirus has shown people that delivering healthcare with technology has a role to play. We have a strong voice for the payer and provider, maybe we are beginning to see the arrival of the patient voice…If we can create a gratifying experience for the people that we serve over the long term that is a winning strategy.”
“There is an important question as to whether these technologies are part of the delivery or the payer side of care.”
He sees a future where group practices are enabled by technology to be more easily accessible to patients while providing great customer service and communication. Rather than viewing telehealth as just another “tool”, he believes that a “home run” would be when:
”Telehealth is a care setting in which traditional medicine is delivered and the home front will be the most coveted care setting.”
Chronic disease patients, especially autoimmune and autoinflammatory disorders patients, need ongoing support between regularly scheduled appointments. The expanding use of telehealth may benefit them.
Telehealth can enable more frequent, convenient, yet less costly encounters from patients’ homes. This is even more true if telehealth is not just replacing PCP encounters but augmenting them with coaching and other staff. Coordinating with specialists is another important feature for chronic and autoimmune disease patients, but that doesn’t seem to be as far advanced.
Oscar Health is a hybrid insurance company, telemedicine platform, and primary care provider, based in the Pacific Northwest. Founded in 2012, Oscar Health was the first direct-to-consumer (DTC) health insurer. It facilitates member engagement with their own full-stack technology platform that handles both the insurance side and the care delivery side of the business. This includes:
Oscar is one of many companies building variations on care teams to improve coordination, delivery, and customer service while controlling costs. These teams, which Oscar calls concierge care guides, can help patients do the following:
This approach: digitally supported personalized care teams led by nurses, coordinated by guides (aka coaches), and physicians. The personalized care teams provide a range of care, including chronic disease plans, which is similar to ideas we’ve proposed from 2014 on to help CID patients navigate their complex ongoing care.
Mario Schlosser, CEO of Oscar Health, told HLTH that the COVID boost to telehealth is a
“huge opportunity to bend the cost curve and increase the number of touchpoints (visits) and therefore reduce the variation in care.”
For autoimmune and autoinflammatory disorders patients, more telehealth visits and remote monitoring of symptoms, medications, etc. between appointments could help them reduce flares, better manage side effects, and avoid costly emergency care and hospitalizations.
Schlosser sees a future where there will be more remotely enabled physician practices that take on the financial risk themselves so that
“the number of physician visits go up, but the price goes down.”
In conclusion, telemedicine and remote patient monitoring are enabling technologies, much needed. However, they have not yet directly focused on autoimmune or autoinflammatory disease care.
Using telehealth to expand access, speed, and the number of encounters, along with the expanded use of care-based team models that include specialists, could potentially benefit autoimmune patients.
Likewise, remote patient monitoring can enable better chronic autoimmune disease care. However, this will only occur if payers, providers, and platform companies finally focus on this invisible opportunity, as costly and complex as cancer.
Value-based care (VBC) offers a new financial foundation for healthcare delivery business model experiments. From the perspective of autoimmune patients, I wonder if the VBC payment models better support the long-term, multiple-modality care coordination that such patients need, but have generally not received from FFS delivery models?
Bryony Winn did a great job setting the stage, asking why this is a good time to discuss “A Push Into Value-Based Care”, as she asserted that:
From my perspective, FFS has not been a good model for chronic disease management, let alone, chronic antiinflammatory, and autoimmune diseases. The key question is, will VBC payment models support providers and practices to provide better care for lifelong chronic inflammatory patients?
The Massachusetts General Physicians Organization (MGPO) is the largest multi-specialty medical group in New England and one of the largest in the U.S.
Dr. Timothy Ferris- CEO of Mass General Physician Organization, said,
“I do not see [hospital-led ACOs vs independent physician practice-led ACOs]as an either-or? [Hospital-led ACOs] have capital that can be redeployed in ways that generate benefits for the system and patients…The key thing is investing in the infrastructure and services that the FFS model does not pay for.”
How can big ACOs compete with new, inexpensive retail FFS offerings? For example, DTC apps such as HIMS, HERS, Keeps, etc. offer one-stop, doc-in-a-box shopping for popular prescription drugs. Other competitors include big point-of-care retailers, like CVS and Walgreens, for acute care, vaccinations, prescriptions, etc.
“I think that integrated delivery systems of the future will have to find a model to deliver low-end services.” (flu shots, etc.) as cheaply as their new competitors. “But most of our spend is not on low-value care, but rather on folks who are very sick.”
In part because many very sick patients present with multiple comorbidities, any provider’s ability to reduce the spending on these sick folks is limited.
“We can show that large integrated delivery systems were able to reduce overall system costs by 2%”.
Dedicated care managers (similar to those tracking hypertension patients) could help autoimmune patients with treatment support needs between regular appointments. They could also help foster more proactive flare-prevention strategies.
Autoimmune and autoinflammatory conditions are costly to manage and not just because of specialty medications. However, avoiding flares and thus even more expensive episodes of emergency care and hospitalization strike me as low-hanging fruit, high-return investments for big hospital systems.
Yet this unmet need is an opportunity that seems generally to have been overlooked. Maybe the emergence of post-COVID syndromes, which look very much like autoimmune reactions, will force big provider systems to come to grips with the unseen epidemic of immunological and inflammatory diseases.
Aledade is a platform company that partners with primary care physicians to help them build and lead their own Accountable Care Organizations (ACOs).
Farzad Mostashari, the co-founder and CEO of Aledade, told us that 6 years ago they bet that independent PCP ACOs would win over hospital-led ACOs. The independents were not burdened with the large financial overhead of hospital systems. Therefore, Aledade saw an opportunity to
“Go with those with the most to gain and the least to lose from VBC.”
As of 2020, Aledade serves 550 practices and 7000 clinicians with $10 billion under management.
“It is working and it’s growing.”
Aledade provides regulatory expertise, technology, data analytics, business transformation services, and upfront capital. In addition to all the other elements, independent practitioners need to succeed in value-based care.
Patients are not seeing reduced care. Mostashari said we can offer “more screening, more immunizations, better blood pressure control.” Patients are getting primary care that is “more accessible, more informed, and more engaged.”
Overall, they claim an 8-13% reduction in the total cost of care. Savings vary by state.
Providing affordable, easy to integrate, off-the-shelf IT infrastructure and other business process support tools as well as data management for primary care docs could enable them to better compete with large hospital systems. This might also enable them to offer more timely, accessible care to chronic disease patients, including autoimmune patients.
This leaves open the question of how specialists, much utilized by autoimmune and chronic inflammatory patients, fit in. Specialist practices have thrived under supply-restricted, high-demand FFS models. And so far have not been eager to participate in digitally-enabled care team practices.
Once more, maybe the pandemic disruption to cash cows like colonoscopy will accelerate the previously sluggish migration of specialists to VBC models.
Telemedicine and remote patient monitoring are two necessary but not sufficient enabling technologies for better autoimmune care delivery. Making care more accessible from patients’ homes through telemedicine, apps and the expanded use of care-based team models could potentially benefit those with chronic immune conditions, who need more frequent touchpoints on their care journeys.
The changing reimbursement landscape, from fragmented, uncoordinated FFS to patient-centered (or at least capitation or episode-of-care coverage) VBC may also better financially support care delivery for autoimmune patients. Especially those patients on specialty meds who need monitoring, management, and support between regular physician and specialist visits.
Such care should also include non-pharmacological modes like physical therapy, exercise coaching, diet management, sleep, and mental health support. Some of these pieces are being incorporated into some of the new care delivery models. However, the chronic disease focus has been on cancer, diabetes, and cardiopulmonary disease, not inflammatory, immunological, autoimmune or autoinflammatory conditions.
The conference focused on bringing virtual primary care into the home or at least to the smartphone. However, I have unanswered questions concerning the role of specialists in these new models. How will team-based care coordination integrate the multiple specialists (e.g. rheumatologist, gastroenterologist, dermatologist, neurologist) many autoimmune patients need to guide chronic care?
I took a pass on keeping the regular follow-up visit with my retinal specialist in April. I said to myself (and anyone else who would listen), there’s no sense keeping the appointment for my rare eye disease because I was not going to accept the recommended treatment. You see, my left eye is afflicted with a progressive, most likely auto-inflammatory process, known as chorioretinitis. It is slowly getting worse.
According to my specialist at Stanford, the next step in my care is big-time immunosuppression with IV methylprednisolone (a steroid) plus IV cyclophosphamide (a chemotherapeutic and immunosuppressive drug). This scared me.
I felt like being immunosuppressed in the middle of the COVID-19 pandemic would be suicide. I also told anyone who would listen, “I would rather be blind than dead.” But the truth is I prefer neither.
Remember, we didn’t know much about the virus at that time except that it was very infectious. And, it makes some people (older folks and those with chronic medical conditions) very, very sick. Some die. And from what I was reading at the time, it is a pretty horrible way to exit this earth.
By June, it looked like the pandemic was slowing down in Northern California. So when my next appointment popped up on my calendar, I decided I would go. This was a big leap for me as I only rarely left the house at that time.
I was nervous, of course, going to a medical setting where people could possibly be exposed to the virus and transmit it. But, I had one N95 mask that one of my sons, a nurse, gave me at the start of the pandemic. (This was way before we knew anything about shortages of these types of masks.)
My husband drove me from Marin County where we live to the Byers Eye Institute, a part of Stanford Healthcare.
The COVID-19 precautions at Byers seemed well thought out. They made everyone enter the building through a single entrance that was guarded by a man with hand-sanitizer and a box of masks. We had to line up six feet apart and enter one-by-one.
We were required to put their mask over the N95s we were already wearing. Our temperature was checked just inside the door. And, only I could enter the patient care area. My husband was relegated to the outer waiting area.
I had to get the usual panel of advanced eye-imaging studies. But I relaxed when I saw that the tech was double-masked and was wearing a face shield. Gradually, I grew confident that Byers was doing everything they should be doing to keep us safe from getting COVID in their clinic.
By the time I got the bad news that my eye disease had indeed progressed, I was able to rationally think about my choices. We initially thought that the inflammation of my eye was due to taking alendronate (Fosamax) for osteoporosis. This is because the timing of me starting the drug and developing the disease was highly suggestive that it could have been the trigger.
It was made more likely because I had tested negative for every known cause of the condition. That made me something you never want to be: an interesting case. I am even the subject of a detailed case study in the Journal of Ophthalmology.
Now, the problem is that the only way to know for sure that the drug was the culprit would be to take it again and see what happens to the disease. This, of course, would be a foolish thing to do.
But there was one other course of action that could be taken before subjecting myself to immunosuppression therapy, however. That was to take a sample of my vitreous (the jelly-like substance in front of the retina that gives the eye its shape) and analyze it for rare or unusual infectious organisms that are not currently known to cause a condition such as mine.
This didn’t seem far-fetched to me as I have traveled in the developing world for years. I once fell in the mud in the jungles of Peru. Who knows what I was exposed to over the years.
Therefore having some sort of exotic infection was not, in my mind, out of the question. Further, I hoped it would be the case as then we would 1) know the cause and 2) be able to take a treatment targeted specifically to that cause.
Aperiomics is a laboratory that specializes in identifying pathogens in various body fluids. They have developed a database of every known, sequenced bacteria, virus, fungus, and parasite in the world.
Then, using something called “deep shotgun metagenomic sequencing”, an advanced technology that can find even small amounts of genetic material in the sample. Using sophisticated algorithms, the sequences of that genetic material is then matched to the database. This can lead to the identification of the causal organism in many cases that had previously gone undiagnosed.
I had interviewed Crystal Icenhour, the founder and CEO about a half a year ago. So I gave her a call to see if this testing could be done in a vitreous sample. They had not done this before but she said she believed it was possible.
I put my ophthalmologist in touch with her so the specimen collection and transfer could be done properly. I wasn’t going to leave this to chance.
Other stories by the same author: COVID-19: The Impact of Our Early Failure to Respond
So, I opted for surgery, a procedure known as a diagnostic vitrectomy. Yes, they remove all or almost all of the vitreous. The surgeon assured me, however, that the body would replace the missing gel with aqueous humor – the liquid in the front part of the eye. He said it wasn’t a risky surgery and, in fact, it only takes about a half-hour to perform.
I arrived at Byers a little nervous but confident I made the right decision. If Aperiomics found some weird infection, that would be a good thing. It could be treated and I would be done with my rare disease.
Again, Byers’s attention to detail with respect to the virus made me feel comfortable. If I am going to get COVID eventually, it was not going to be because of my eye surgery.
I was escorted into the outpatient surgery center and, once again, my husband was relegated to an outside waiting room.
Many different nurses worked on getting me ready for surgery. All were fully PPE’d up.
The nurse anesthetist explained that I was not going to be asleep for the operation. Rather, they were going to use drugs to put me into a twilight state.
And, indeed, I could hear everything that was going on during the surgery, but I pretty much didn’t care. It was pretty strange. I heard the surgeon and his assistant say that I needed to have laser treatment to firm up a weak spot in my retina. I didn’t care about that either.
Later, I could hear my surgeon telling pathology that he was going to take care of mailing the vitreous sample to Aperiomics. I did care about that. After all, why let someone remove your vitreous and then mess up by not ensuring that it arrives at the lab in good shape.
The morning after surgery, I noticed a big bubble with a black rim floating around in my eye. The vision was ok if I positioned my head in a way that allowed the bubble to float out of the field of vision. I also had a huge pupil because I was prescribed dilating drops.
My follow-up visit was uneventful. The surgeon assured me the bubble would be resorbed in time – and it was.
The white of my eye was deep red from pooling of blood below the conjunctiva (the outer lining of the eyeball). I had seen subconjunctival hemorrhages many times when I worked as an emergency physician. So this did not alarm me.
My eye stayed dilated for almost 11 days after I stopped the atropine. But, just like the doctor said, it eventually returned to normal.
Lab tests that had been sent to the Stanford lab started rolling in shortly after the surgery. Everything was negative.
Aperiomics, the outside lab, kept me in the loop on the status of processing my precious specimen. I thought it was a nice touch:
In the end, almost everything was negative: no parasites, no fungi, no viruses. There were small amounts of bacteria, but they likely were contaminants. The report did point out that several of them, however, were known to cause disease in some circumstances. So, I didn’t get the definitive answer I was hoping for.
Obviously, I was disappointed. As I said, I had hoped for a treatable infection even though the ophthalmologist kept telling me an infection was very unlikely because of my clinical course. And, because I never had cells or other signs of infection on my detailed and meticulous eye exams.
One of the choices, the dreaded combo of intravenous (IV) cyclophosphamide (a drug used as chemotherapy and to suppress the immune system) and IV methylprednisolone (a steroid) was still on the table. But this time, new options were offered:
And finally, the doctor suggested trying a course of antibiotics (Septra DS) telling me that sometimes it works in cases like mine. Of course, I opted for that fully aware that I am rolling the dice as the relentless disease keeps on killing off my retinal cells. Luckily, the growth of the inflammatory process is heading away from the macula, the most important area of the eye with respect to vision.
So far, so good.
As you know, in the months after my surgery, the COVID virus went wild again scaring the bejesus out of folks like me worried about having a high risk of a bad outcome. So not being on immunosuppressants right now seems like a good thing. My doctor, however, assured me that other patients are taking those drugs right now and doing just fine.
I will have been on the antibiotics for about a month by the time I go to Byers again. My vision is stable (as always) and I feel great.
I am hoping against hope that we will find that the Septra will miraculously stop the process in its track. If not, this time I may have to bite the bullet and go for the big guns. Fingers crossed.
The bottom line for me is that I was able to actively participate in the decision-making for my eyes. Obviously, I am not a chorioretinitis specialist but I have one of those after all.
I am, however, a specialist in myself. I knew that I had to do everything possible to avoid immunosuppression in the age of COVID. If I end up having to get the dreaded drugs now, at least I know that I did everything within my power to avoid it when I felt it was too big a risk for me.
For me, it was the right plan of action and that is what patient-centered centered care is all about.
Early testing in healthcare has been a hot topic in recent months. For both infectious diseases and countless other health conditions. Timely testing is critical to faster diagnosis, prompter treatment, and, ultimately, better outcomes in many medical conditions, including osteoporosis.
As a physician who has the opportunity to care for patients living with this common, yet serious, disease, I understand first-hand the importance of early testing for osteoporosis. Individuals can have the condition for a long time and not even know it.
Osteoporosis is a silent disease. Patients often have no signs or symptoms until a fracture occurs.
Risk factors for osteoporosis include
Estrogen significantly affects the rate of bone loss. This is why osteoporosis is most common in postmenopausal women.
In the first 5-7 years after menopause, bone breakdown outpaces bone formation and bone loss often accelerates. Up to 20% of bone mass can be lost in a few years.[i]
Moreover, nearly 1 in 2 women over the age of 50 will experience a fragility, or low-impact, fracture.[ii] Unfortunately, there is still a disconnect that these fractures, such as those in the wrist or ankle, could be a warning sign for the disease. As a result, many patients go undiagnosed and untreated.
That’s why if your patients are women over 50, it’s important to prioritize bone health. Early identification and treatment of low bone density is the most effective approach in order to help patients avoid future painful fractures.
A dual-energy X-ray absorptiometry scan, commonly referred to as a DXA scan, is a widely available, reliable bone density test. The test requires no special preparation and typically takes only 5 to 10 minutes.
For the results of the test, patients receive a T-score, which shows how much higher or lower their bone density is than that of a healthy young person, when bones are at their strongest. In general, the lower the bone density, the greater the potential risk of fracture.
Many women wait to obtain a bone density test until they are 65. However, if patients have clinical risk factors for bone loss or have experienced fractures, earlier testing is important.
Many women enter menopause with low bone mass already. Further, for some subsets of women, there is a risk for rapid bone loss of 10-20% over just five years.[iii]
For that reason, in my practice, I typically recommend a bone density test for anyone 50 and older with clinical risk factors. This is especially true for those who have already experienced a fracture.[iv]
The results of a DXA scan, combined with other risk factors including, but not limited to,
will help assess future fracture risk and determine the need for potential treatment.
Lifestyle changes, such as calcium, vitamin D, and fall protection, may be effective for some patients. However, when fracture risk is high, medications are needed.
Fortunately, there is a strong arsenal of treatment options. But, it is important to remember that what’s right for one person may not be right for another.
Prescription osteoporosis treatments come in several forms, including
They can be broken into two categories: antiresorptive and anabolic agents.
Antiresorptive treatments include the following:
These types of medications help slow down the process of bone loss in order to preserve bone strength to reduce the risk of fracture.
Anabolic treatments include drugs such as PTH analogs and sclerostin inhibitors that do more than just maintain the bone that you already have. They help build new bone to reduce the risk of fracture.
Since studies have shown that postmenopausal women who have had a low-impact fracture are 6 times more likely to have another fracture within 1 year[v]. These bone builders, therefore, are an important option to help reduce the risk of fracture or subsequent fractures in this high-risk population.
It’s critical that patients play a role in their treatment decisions. This means that they must be fully educated on all their options, including the benefit-risk profile.
As a first step, I encourage my patients to visit the websites of the companies that make the treatments. And, I advise them to look at the information in an objective way. Patients should also always talk to their doctor to make sure they are balancing the risk and the reward.
As adults age, the impact of fractures can become more serious, increasing the risk of loss of independence and even dying prematurely. In fact, hip fractures have a 20% 1-year mortality in women (higher in men). And they cause 1 out of 5 patients to need care in a nursing home. They are the most serious type of fracture caused by osteoporosis and 75% of all hip fractures happen in women.[vi],[vii] Another recent study found that older adults who suffer a fragility hip fracture have an increased risk for death that lasts for more than 10 years after the fracture.[viii]
Related content: What Happened When I Fell and Broke My Shoulder
All of these statistics reiterate the importance of being an empowered patient if they’ve been diagnosed with postmenopausal osteoporosis. They need to talk with their doctor about their bone health to fully understand their options. And they must choose a treatment where the benefits outweigh the risks.
The disconnect between osteoporosis and related fractures and subsequent treatment gap is alarming. However, there is a tremendous opportunity before us. By empowering more women to obtain bone density tests before a fracture occurs, even when they otherwise have no symptoms, we can ensure they take the necessary steps to protect bones by seeking treatment earlier.
I encourage my fellow physicians to engage in open dialogue with patients. Talking will help them fully understand all of the available postmenopausal osteoporosis treatment options. This means including both antiresorptives, which slow down bone loss, and anabolic medications that are designed to help boost the natural process that builds new bone.
With a concerted effort and greater collaboration between patients, primary care providers and specialists who more regularly treat postmenopausal women, such as rheumatologists and endocrinologists, we can help many more patients make effective treatment decisions earlier and reduce their fracture risk.
[i] National Osteoporosis Foundation – What Women Need to Know. Available at: https://www.nof.org/preventing-fractures/general-facts/what-women-need-to-know/. Accessed 05/26/2020.
[ii] National Osteoporosis Foundation. What is osteoporosis and what causes it? https://www.nof.org/patients/what-is-osteoporosis/. Accessed 05/21/2020.
[iii] Finkelstein J., et al. Bone mineral density changes during the menopause transition in a multiethnic cohort of women. J Clin Endocrinol Metab. 2008 Mar; 93(3): 861–868. Free access available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2266953/. Accessed 05/21/2020.
[iv] National Osteoporosis Foundation. Bone Density Exam/Testing. https://www.nof.org/patients/diagnosis-information/bone-density-examtesting/. Accessed 06/01/2020.
[v] Simonelli C, et al. Evaluation and Management of Osteoporosis Following Hospitalization for Low-Impact Fracture. J Gen Intern Med. 2003 Jan;18(1)17-22. Free access available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1494813/. Accessed 05/21/2020.
[vi] Leibson CL, et.al. Mortality, disability, and nursing home use for persons with and without hip fracture: a population-based study. J Am Geriatr Soc. 2002;50(10):1644‐1650. Accessed 06/01/2020.
[vii] Hyassat, D., et.al. Prevalence and risk factors of osteoporosis among Jordanian postmenopausal women attending the National Center for Diabetes, Endocrinology and Genetics in Jordan. BioRes Open Access. 2017; 6(1): 85–93. Free access available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5515108. Accessed 06/01/2020.
[viii] Tran T., et. al. Persistence of excess mortality following individual nonhip fractures: a relative survival analysis. J Clinical Endo Metabolism. 2018 Sep; 103(9): 3205–3214. Free access available at: https://academic.oup.com/jcem/article/103/9/3Bone 205/4996518. Accessed 05/21/2020.
Systemic sclerosis is also known as scleroderma. It is one of the most fatal of the autoimmune disorders. It affects up to 75,000 people in the United States each year[mfn]Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 2003;29(2):239-54.[/mfn]. An early and accurate diagnosis can be challenging due to the way the disease presents and its rare nature. The objective of this article is to provide a comprehensive overview to aid in the understanding of systemic sclerosis. It includes recommendations for its diagnosis and management, as well as a discussion on the future hope for this disease.
[Note: the term systemic sclerosis and scleroderma will be used interchangeably in this article.]
Systemic sclerosis is a rheumatic autoimmune disorder that affects the skin and internal organs. It is a complex disease driven by inflammation and fibrosis (scarring of tissue). This leads to severe damage and failure of multiple organs including,
Although scleroderma is rare, it is considered one of the most life-threatening rheumatic diseases.
There are two main types of systemic sclerosis: limited and diffuse. They are defined by the extent of skin involved.
Limited is the milder form of skin involvement. It is characterized by fibrosis below the elbows and knees. Generally, limited systemic sclerosis causes less severe fibrosis of internal organs than the more severe form.
In diffuse form, fibrosis affects larger areas of skin including the torso, upper arms, and legs. It often involves internal organs. Diffuse systemic sclerosis can have damaging and harmful effects on the lungs, heart, and kidneys. If not treated promptly, it can lead to poor outcomes[mfn]LeRoy EC, Medsger TA, Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28(7):1573-6.[/mfn], [mfn]Shah AA, Wigley FM. My approach to the treatment of scleroderma. Mayo Clin Proc. 2013;88(4):377-93.[/mfn].
This disease is characterized by immune system activation, vasculopathy, and widespread tissue fibrosis. However, its pathogenesis is still unclear.
The most accepted working hypothesis suggests that a triggering event occurs that causes activation of specific cells of the immune system and extracellular matrix (ECM). These findings are the defining features of connective tissue disease.
The chronic activation of immune cells, inflammatory mediators, and components of the ECM, including fibroblasts, can lead to fibrosis, organ damage, and even loss of organ function[mfn]Katsumoto TR, Whitfield ML, Connolly MK. The pathogenesis of systemic sclerosis. Annu Rev Pathol. 2011;6:509-37[/mfn], [mfn]Asano Y. Systemic sclerosis. J Dermatol. 2018;45(2):128-38.[/mfn], [mfn]Jimenez SA. Role of endothelial to mesenchymal transition in the pathogenesis of the vascular alterations in systemic sclerosis. ISRN Rheumatol. 2013;2013:835948.[/mfn], [mfn]Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007;117(3):557-67.[/mfn].
As mentioned, the exact cause of the disorder is unknown. It is widely accepted that the interaction of several factors leads to the disease , including:
Scleroderma can present at any age. However, it is typically found in people between the ages of 20 and 50 years . Seventy-five percent or more of patients with the disorder are female, suggesting that gender plays a role[mfn]Peoples C, Medsger TA, Jr., Lucas M, Rosario BL, Feghali-Bostwick CA. Gender differences in systemic sclerosis: relationship to clinical features, serologic status, and outcomes. J Scleroderma Relat Disord. 2016;1(2):177-240.[/mfn].
The symptoms of systemic sclerosis can be similar to other autoimmune diseases. This can make accurate diagnosis challenging.
Early clinical signs of the condition can be varied, but often include Raynaud’s phenomenon and gastroesophageal reflux disease (GERD)[mfn]Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-99.[/mfn]. Other early symptoms include inflammatory skin disease, puffy and swollen fingers, musculoskeletal inflammation, and/or fatigue [mfn]Bellando-Randone SG, S.; Marco Matucci-Cerinic, M. Very Early Diagnosis of Systemic Sclerosis. Polish Archives of Internal Medicine. 2012;122:18-23.[/mfn].
Later in the disease course, some patients present with end-organ manifestations, such as pulmonary arterial hypertension [mfn]Domsic RT, Nihtyanova SI, Wisniewski SR, Fine MJ, Lucas M, Kwoh CK, et al. Derivation and validation of a prediction rule for two-year mortality in early diffuse cutaneous systemic sclerosis. Arthritis Rheumatol. 2014;66(6):1616-24.[/mfn], [mfn]Cavagna L, Codullo V, Ghio S, Scire CA, Guzzafame E, Scelsi L, et al. Undiagnosed connective tissue diseases: High prevalence in pulmonary arterial hypertension patients. Medicine (Baltimore). 2016;95(39):e4827.[/mfn]. Lung fibrosis is a common complication of systemic sclerosis. Up to 50% of patients develop interstitial lung disease (ILD). It is the most common cause of mortality in patients [mfn]Tackling systemic sclerosis from all angles. The Lancet Rheumatology. 2020;2(3):e121.[/mfn].
The disorder has both physical and emotional effects on patients living with this condition. It can cause significant disability, disfiguration, and reduced life expectancy ,[mfn]Debois AC, Cacoub P. Systemic sclerosis: An update in 2016. Autoimmun Rev. 2016;15(5):417-26.[/mfn]. Patients often experience a reduced quality of life, complicated by the uncertainty of outcome and development of potentially lethal manifestations.
The prognosis depends on the type, the time of diagnosis, and treatment. With limited disease, a patient’s condition can be stable for years. However, the diffuse form can be fatal if not treated promptly. This is especially true for those who present with pulmonary hypertension, despite currently available treatment options.
Systemic sclerosis is among the most fatal autoimmune disorders. One in four patients dies within eight years of diagnosis, demonstrating the importance of early diagnosis and prompt treatment[mfn]Hao Y, Hudson M, Baron M, Carreira P, Stevens W, Rabusa C, et al. Early Mortality in a Multinational Systemic Sclerosis Inception Cohort. Arthritis Rheumatol. 2017;69(5):1067-77.[/mfn].
Scleroderma is an uncommon disease for most physicians. It is usually diagnosed by a rheumatologist.
Once the disorder is suspected, patients are often assessed by ACR/EULAR (American College of Rheumatology/European League Against Rheumatism) OR VEDOSS (Very Early Diagnosis of Systemic Sclerosis) criteria. As systemic sclerosis presents with heterogeneous clinical manifestations, not all patients diagnosed will fulfill these criteria[mfn]Saketkoo LA, Magnus JH, Doyle MK. The Primary Care Physician in the Early Diagnosis of Systemic Sclerosis: The Cornerstone of Recognition and Hope. American Journal of the Medical Sciences. 2014;347(1):54-63.[/mfn].
Tests are ordered to confirm diagnosis and screen for internal organ involvement, including:
Though gastrointestinal tract involvement is not part of the classification criteria, it is often affected in systemic sclerosis. As such, screening for esophageal disease can be helpful for diagnosis and management.
It is recommended that patients diagnosed with systemic sclerosis be considered for pulmonary function tests. This is because lung fibrosis is a common complication of the disease.
These tests evaluate lung restriction and reduction in diffusion capacity and can allow the initiation of proper therapies. Other tests for heart and lung screening include:
Early diagnosis and screening are critical for the management of the disease. Cases can be misdiagnosed, especially in cases with mild or no skin manifestations.
The disease cannot be cured. However, it is treatable in its early stages.
Primary care physicians often play an important role in recognizing initial symptoms of systemic sclerosis and referring their patients to specialists with expertise in treating the disease, such as rheumatologists.
Delays in referring patients to rheumatologic care, especially for those with moderate-to-severe cases, can be detrimental. Such cases can progress to irreversible end-organ damage that may have been prevented or delayed by early diagnosis and intervention [mfn]Guiducci S, Bellando-Randone S, Matucci-Cerinic M. A New Way of Thinking about Systemic Sclerosis: The Opportunity for a Very Early Diagnosis. Isr Med Assoc J. 2016;18(3-4):141-3.[/mfn].
Available treatments can help improve pain, various disease manifestations, and improve the quality of life for patients with systemic sclerosis. However, there is currently no cure for this chronic disease.
Current approaches to managing systemic sclerosis are organ-specific. They are aimed at responding to the damage driven by inflammation and fibrosis, and not the underlying mechanisms of the disease , [mfn]Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327-39.[/mfn], [mfn]Smith V, Scire CA, Talarico R, Airo P, Alexander T, Allanore Y, et al. Systemic sclerosis: state of the art on clinical practice guidelines. RMD Open. 2018;4(Suppl 1):e000782.[/mfn].
The following table outlines the current treatment options. , [mfn]Eldoma M, Pope J. The contemporary management of systemic sclerosis. Expert Rev Clin Immunol. 2018;14(7):573-82.[/mfn].
|Organ System||Clinical Manifestation||Example Treatments|
|Skin||Scleroderma||-Immunosuppressive therapies (IST) e.g. methotrexate|
|Musculoskeletal||Inflammatory arthritis||-IST e.g. methotrexate|
|Renal||Scleroderma renal crisis||-Angiotensin-converting enzyme inhibitors|
|Gastrointestinal||Gastroesophageal reflux disease (GERD)||-Lifestyle modifications
|Peripheral vascular||Raynaud’s phenomenon
|-Calcium channel blockers
-Phosphodiesterase 5 inhibitors
-Angiotensin II receptor blockers
-Endothelin receptor antagonists
|Pulmonary||Interstitial lung disease (ILD)
Pulmonary arterial hypertension (PAH)
|-Immunosuppressive treatment e.g. mycophenolate mofetil, cyclophosphamide
-Tyrosine kinase inhibitor
-Endothelin receptor antagonists
-Phosphodiesterase 5 inhibitors
-Soluble guanylate cyclase agonists
Inflammatory cardiac disease
|-Drug therapies e.g. angiotensin-converting enzyme inhibitors and diuretics
-IST e.g. corticosteroids and/or cyclophosphamide
Clinical manifestations, such as joint contractures, may require physical and occupational therapy to prevent disability. Other ancillary services may be indicated based on clinical features, such as:
For patients with rapidly progressive disease at risk for organ failure, hematopoietic stem cell transplantation (HSCT) can be considered, but should only be done at high volume expert centers .
As previously mentioned, patients with systemic sclerosis should be referred to specialists, like rheumatologists who are experienced in treating the complex clinical manifestations of this disease. Often, rheumatologists will work together with primary care physicians and other specialists to ensure comprehensive care of the patient , [mfn]Denton CP, Hughes M, Gak N, Vila J, Buch MH, Chakravarty K, et al. BSR and BHPR guideline for the treatment of systemic sclerosis. Rheumatology (Oxford). 2016;55(10):1906-10.[/mfn]. For example, management of gastrointestinal manifestations can be achieved through coordinating care between a gastroenterologist and rheumatologist[mfn]Sakkas LI, Simopoulou T, Daoussis D, Liossis SN, Potamianos S. Intestinal Involvement in Systemic Sclerosis: A Clinical Review. Dig Dis Sci. 2018;63(4):834-44.[/mfn].
Treating systemic sclerosis requires a thoughtful approach carefully tailored to each patient. A new physician resource, www.totalssc.com, addresses the complexity of systemic sclerosis which is helpful for understanding the needs of patients living with this devastating disease.
Systemic sclerosis is a complex disease with many different clinical manifestations. Currently, the only FDA-approved therapy is a tyrosine kinase inhibitor. It slows the decline of pulmonary function in adults with ILD-associated with systemic sclerosis.
Treatments used today only address clinical manifestations and not the underlying drivers of the disease demonstrating a large unmet need.
Research on the treatment of systemic sclerosis is fundamental to improving outcomes for patients. Considerable progress has been made in the understanding of the pathogenesis of this disease in recent years which has led to the development of novel therapeutic candidates.
For example, one therapy in development targets the endocannabinoid system (ECS), which is a regulator of inflammation and fibrosis [mfn]Gonzalez-Mariscal I, Krzysik-Walker SM, Doyle ME, Liu QR, Cimbro R, Santa-Cruz Calvo S, et al. Human CB1 Receptor Isoforms, present in Hepatocytes and beta-cells, are Involved in Regulating Metabolism. Sci Rep. 2016;6:33302.[/mfn]. Research has shown that targeting the cannabinoid receptor type 2 (CB2) on immune cells can reduce inflammation and limit fibrosis. This is a novel approach and if approved could be the first therapy that targets underlying drivers of systemic sclerosis as opposed to its clinical manifestations.
Antifibrotic therapy to treat ILD-associated systemic sclerosis is being actively studied as an add-on to immunosuppressive therapy. Studies have shown that the underlying mechanisms of idiopathic pulmonary fibrosis (IPF) may be similar to those contributing to fibrosis in systemic sclerosis which has led to the investigation of its use in ILD-associated systemic sclerosis [mfn]Volkmann ER, Tashkin DP. Treatment of Systemic Sclerosis-related Interstitial Lung Disease: A Review of Existing and Emerging Therapies. Ann Am Thorac Soc. 2016;13(11):2045-56.[/mfn].
Currently, there are several drug candidates in Phase 3 trials, which is significant given the low incidence of this disease. This indicates that there is growing interest in developing new treatments for systemic sclerosis, providing hope to those affected by the disease.
The Scleroderma Foundation is a national non-profit organization that supports patients and their families, promotes public awareness and education. It also supports research to improve treatment and eventually find a cure for systemic sclerosis.
This foundation offers many tools and resources for new and current patients and their families, including
“The Scleroderma Foundation is proud to be the leading scleroderma patient advocacy organization in the US and the largest one globally,” said Robert Riggs, chief executive officer of the Scleroderma Foundation. “With a network of 19 chapters and 150 support groups around the country, the Scleroderma Foundation is a trusted source of medically-vetted information and community connection.”
Scleroderma specialty centers specialize in the care of patients with systemic sclerosis and related conditions. Physicians at these centers seek to learn more about systemic sclerosis and the best treatment approaches.
Oftentimes, a scleroderma center can help coordinate treatment services for patients in addition to providing access to clinical research opportunities.
What does the future of healthcare offer for chronic autoimmune and inflammatory disease? Having just come back from the Personalized Lifestyle Medical Institute functional medicine thought leaders conference (PLMI), I was curious to find out what HLTH had in store.
Although there were no sessions specifically focused on autoimmune patients, I was inspired nevertheless, when at every meal, the people on my right and left knew someone with an autoimmune disease. It’s clear that chronic inflammatory and autoimmune diseases are slowly increasing in industry awareness.
With 6200 attendees from all over the world attended this year’s HLTH conference: Create Health’s Future. It has quickly emerged as one of the healthcare industry’s leading gatherings.
The conference featured an impressive list of speakers from many sectors, including:
It also attracted researchers and companies focused on a specific area, such as the microbiome, digital therapeutics, care coordination and so much more. This wide assortment of viewpoints with a shared goal of building the future of healthcare provided a rich networking environment for participants.
I attended the conference with my autoimmune patient hat on. I was seeking a sweet spot where consumer wellness and maximizing well-being for people with chronic disease overlap. I was pleased to find some amazing innovations that will be useful for patients with immune-mediated diseases.
My conversations with autoimmune patients have revealed widespread frustration with the lack of care coordination and collaboration between and among their primary providers and various specialists (Rheumatology, Gastroenterology, Immunology, etc.). This is an obvious target for better technology.
Another source of frustration and sub-optimal quality of life is that most autoimmune patients struggle to find appropriate, supportive physical therapy and exercise modalities and programs.
Autoimmune patients are especially likely to suffer from intolerance at exercise levels normal people are comfortable with. Therefore, chronic immuno-inflammatory patients and their providers need more customized and personalized approaches to movement as therapy.
As shown below, Bridge Connector, an integration platform as a service (iPaaS) company, enables development, execution, and governance of workflows connecting any combination of on-premise and cloud-based processes, services, applications and data within individual or across multiple organizations.
Speaking with Jason Raphael, The Chief Delivery Officer of Bridge Connector, “breaking down walls of connection while putting data around the patient. Their soon to be launched product, Destinations, is designed to enable the citizen integrator. His descriptions left me hopeful that tech companies are finally identifying problems that are important to autoimmune patients.
It is the first middleware without coding to create an Integration Platform as a Service. Its customers include hospitals, post-acute facilities, behavioral health, specialty care, and new technology start-ups.
On a related note, many autoimmune patients could use better tech for home exercise and rehab. This is why Kaia’s AI home exercise coach, the Motion Coach ™ excited me. Interestingly, it started as a business-to-consumer model and has transitioned to a business-to-business model, selling to self-insured employers and health plans for chronic low back pain.
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As shown below, the Motion Coach acts similarly to a “virtual physical therapist” correcting and helping to mediate the patient’s home exercise program. An independent randomized clinical trial recently published in Nature found Kaia Health’s app-based exercise therapy for lower back pain (LBP) to be an effective treatment for patients. It could be more effective than a standard strategy of individual physiotherapy sessions paired with online education. More studies are underway to further explore these results, with even larger sample sizes.
There are 3 components to the program, which is a digital version of multimodal pain therapy:
Beyond remote exercise coaches, exciting future technology is the creation/discovery of digital biomarkers, which could track disease progression as well as record snapshots of users’ ability to perform ADL (activities of daily living).
I would love to see Kaia expand to include other movement therapies such as Gyrotonic, Feldenkrais, and others.
For those who prefer in-person exercise, perhaps ClassPass is a viable option to encourage fearless experimentation. ClassPass is a monthly membership program that allows users to attend classes from multiple studios, gyms, and wellness partners.
Like other aggregated marketplaces, it offers users discounted prices, but it’s different from an offering like Groupon because it is focused on habituation, with more variety in wellness choices.
Similar to Kaia’s business model transition, according to Jennie Aberle, the Strategic Accounts Director of ClassPass “exercise has become one component of the larger growing health and wellness trend, so that 2 years ago ClassPass went B2B in corporate wellness.” She continued, “I love fitness and wellness and love the idea that HR leaders are looking for something that works.”
With 26,000 partners in 27 countries, ClassPass makes it easy to find a class either at home or when traveling. Over the years we learned that “location, convenience, variety, and cost are key to making it easy for people to participate in a fitness class.” We would like to see ClassPass extended to an even more diverse selection of studios as well as other studios specializing in small, personalized exercise classes.
After interviewing Bridge Connector, Kaia and ClassPass, I remain hopeful that the emergence of consumer wellness as a trend, will become more relevant to those who manage chronic diseases in the future.
Brief sidebar: Consumer brands and healthcare
Before closing, I must include a brief sidebar on consumer brands and healthcare. One noteworthy session at the conference was a discussion of on-demand personalized health with Lyft, Mastercard, and Bose.
At another time I would have wondered what these consumer brands were doing at a healthcare conference, but with the entrance of Google, Amazon and Berkshire Hathaway into healthcare, it is no longer surprising.
Next year at HLTH, it would be great to see some sessions specifically dedicated to improving care coordination and collaboration for those with immune-mediated diseases. Thus creating customized exercise programs for those dealing with the chronic pain and fatigue associated with these diseases.
Other Articles by this author:
Millions with Autoimmune Disease Need a Better Solution
How Movement Therapy Can Help You Overcome Chronic Pain
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The saying “teamwork makes the dream work” applies to people of all ages and backgrounds. Even Iron Man, awesome as he is on his own, must join forces with the Avengers to battle intergalactic adversaries threatening to destroy the universe.
While the Avengers team is fictitious, it serves as an inspiration. It shows how individuals from different backgrounds with various skill sets can come together to fight for a common cause.
We believe that Avengers-like teamwork and collaboration is needed to reach the audacious goal of Your Autoimmunity Connection to
For years, we and other patient advocates have called for coordinated care between disciplines involved in autoimmune disease. Now, with the incidence and prevalence of autoimmunity on the rise, the most effective way to treat patients is through a broad collaboration. One that includes
I (BF) recently attended the Interdisciplinary Autoimmune Summit 2019 that was held in Chicago, April 5-7. The theme of many of the presentations was interdisciplinary collaboration.
It was extremely heartening to hear a renowned academic, Leonard Calabrese, DO, echoing this call to action. Dr. Calabrese is the Cleveland Clinic Lerner College of Medicine professor and RJ Fasenmyer Chair of the Department of Immunology. He stated that
“we are very subspecialized and we tend to live in silos…Collaboration is important across disciplines in both the basic sciences and clinical practice.”
Interdisciplinary collaboration is essential not only on the clinical side but also in the field of drug development. Tens of years and hundreds of thousands of dollars are invested into research before a drug is discovered, developed, approved, and on the market.
Along the way, pharmaceutical researchers and clinicians often gain a new understanding of molecular pathways or underlying mechanisms of a certain disease. Some of this knowledge may provide insights into other diseases. Interdisciplinary collaboration facilitates this process.
Related content: Understanding Systemic Sclerosis (Scleroderma)
To start off the conference, Dr. Calabrese presented an overview of recent advances in clinical and basic immunology. He drilled down on four different areas as shown below:
Interleukin-1 is the first of what is now a family of 11 chemical messengers (cytokines) that regulate inflammatory processes. It was discovered through studies that explored possible connections between fever and other types of inflammation in animals and humans.
IL-1 occurs in two forms called alpha and beta. It is a powerful pro-inflammatory cytokine. Over decades of study, IL-1 has emerged as a target for drug intervention. The hope was that reducing IL-1 could inhibit processes leading to cardiovascular and other diseases.
C-Reactive Protein (CRP) is a biomarker of inflammation. It is produced by the liver in the presence of any inflammation, including infection and autoimmune diseases such as Rheumatoid Arthritis.
Measures of CRP in the blood are used as a proxy for blocking IL-1, however, the correlation with clinical benefit has been unclear.
Recent research theorized that blocking IL-1 via canakinumab (a monoclonal antibody biological) could reduce cardiovascular events and mortality. However, when tested in the CANTOS trial, this approach fell short of producing significant results.
Regardless, the scientific community did not turn their backs on the IL-1 target. Instead, they looked at inhibition of the IL-1 pathway in various metabolic syndromes. For example, they tested whether IL-1 inhibition could reduce progression from prediabetes to diabetes? Unfortunately, research trials found no statistically significant protection from progression to diabetes.
Research has found that Rheumatoid Arthritis patients have a higher incidence of comorbid lung cancer. Inhibition of IL-1 (as measured by CRP) in Rheumatoid Arthritis patients might mitigate symptoms of arthritis as well as minimize cancer risk. However, clinical trial results are still inconclusive. Calabrese described this as a “cup half empty, cup half full” situation.
Although the definitive benefit of IL-1 inhibition and development or progression of various diseases has yet to be proven, future research may yet demonstrate a link with inflammation or possible associations with inflammatory pathways.
Attached to the ends of each of our chromosomes (as well as in other mammals) are long strands of repetitive nucleotide sequences called telomeres. They protect the chromosome from deteriorating during replication.
Telomeres grow shorter as we age. Genetics play a significant role in telomere shortening as do environmental exposures and individual lifestyle behaviors.
Telomere length offers an easy-to-test, but very general, view of biological (vs chronological) age.
Another molecular pathway that scientists have been analyzing for a better understanding of aging is the mTOR pathway, a molecular complex encoded by the mTOR gene.
The mTOR pathway is involved in regulating many critical cell pathways, including:
Inhibiting mTOR in the appropriate way may increase longevity.
Within the mTOR pathway, there is a pivotal switch that leads to two downstream pathways, mTOR1 and mTOR2.
Inhibition of the mTOR1 complex and increasing mTOR2 results in longer life in mice. While this sounds like an easy fix to lengthen human lifespan, finding a biologic to accomplish this has proven to be much more difficult.
In the search for the perfect molecule that would effectively inhibit mTOR1 and not mTOR2, researchers discovered that mTOR pathway inhibition also
The microbiome is perhaps the latest gold mine of research endeavors. Omics studies and technological advances now allow for better characterization of bacterial strains/species that inhabit our gut.
Fecal transplant is the best-known therapy involving the gut microbiome. In the world of autoimmune diseases, it has been shown to improve symptoms in Inflammatory Bowel Disease (IBD) patients.
Now, researchers have begun to look into how fecal transplants may help fight infections and cancer. Researchers managed to isolate 11 bacterial strains that enhanced cancer checkpoint inhibitor (CPI) therapy in two tumor models in mice.
Further research on the microbiome has produced other noteworthy findings, demonstrating that the human microbiome may be relevant in most, if not all, fields of medicine.
This year’s surprising research findings include:
The Janus Kinases (JAKs) are a family of intracellular tyrosine kinases. They provide transmission signals from cytokines, interferons, and many hormones receptors to the nucleus.
These signals result in the synthesis of many biologically active compounds and changing cell metabolism and function. With this ability to transmit cytokine-related signals, JAKs play a key role in proper function of innate and adaptive immune systems as well as an important role in such pathophysiological processes as hematopoiesis, immune cells development, and many others.
Until recently, JAK inhibitors were known to have safety issues. In fact, one of us (BF) worked at SGX Pharmaceuticals which created an early JAK inhibitor that failed preclinical safety trials.
Yet, as shown below, this year “JAK Inhibitors are Coming of Age” New indications include ankylosing spondylitis, lupus, psoriatic arthritis, and others autoimmune diseases. The drug is available in both oral and topical formulations.
Another topic explored at the conference was the role of mental health providers, psychiatrists in particular, in the management of autoimmune disease. We know that mental disorders are generally approached and treated independently from the rest of the body. Speaker Saundra Jain MA, PsyD, LPC jokes,
However, with recent research that shows mental disorders, such as depression and anxiety, may play a role in inflammation (and maybe vice versa), Jain and her colleague Andrew Laster, MD, FACR discussed the importance of including mental wellness into disease treatment plans.
Understanding the brain-body link in autoimmune disease requires a general understanding of psychoneuroimmunology (PNI). PNI is a convergence of disciplines including
More specifically, the field looks at three systems: nervous, endocrine and immune.
In short, the systems are all bidirectional and are able to communicate with each other through neurotransmitters, hormones, and cytokines. As a result, the presence of psychosocial stressors affecting one system causes a domino effect on all the systems.
It was once thought that the blood-brain barrier (BBB)— a border that separates the circulating blood from the brain and cerebral spinal fluid (CSF) in the central nervous system, was mostly, as the name implies impenetrable to chemicals.
A new understanding of the BBB, however, is that it is a highly selective semi-permeable barrier, allowing only smaller molecules, such as cytokines, access to the brain.
Proinflammatory cytokines are produced by activated macrophages in response to psychosocial stressors. They can then pass through the BBB and activate microglia within the brain.
These microscopic interactions may seem inconsequential. However, research conducted on study participants with major depression found that additional psychosocial stress can induce inflammatory responses through increased levels of IL-6 (cytokines).
Of note, patients with Rheumatoid Arthritis had a significantly higher baseline and stress-induced levels of proinflammatory cytokines compared to healthy controls.
From these findings, Jain and Laster concluded that psychiatrists and other mental health providers, ought to be included in the conversation of interdisciplinary healing teams and mental wellness practices incorporated in treatment plans.
Jain and Laster recommended these five wellness-enhancing behaviors and practices:
Obesity itself is an inflammatory state. However, whether you are obese or not, exercise has been found to alter IL-6 cytokines from their proinflammatory trans-bonded state to their anti-inflammatory cis-bonded state. While some people may choose to hit the gym or go for a five-mile run, those that want to start out at a lower intensity can find more exercise options here.
Sleep deprivation has been shown to increase cytokine production in monocytes and macrophages. Sufficient, restorative sleep has numerous additional benefits on top of cytokine reduction.
While there are many foods that are anti-inflammatory, Dr. Jain discusses the Mediterranean diet as having anti-inflammatory results, lowering levels of CRP, IL-6 and other inflammatory proteins. If dietary restrictions are a concern, perhaps look into other diets that also reduce inflammation.
Related Content: New Medical Nutrition Therapy for Patients with Malabsorption
Mindfulness has been shown to downregulate inflammatory genes. Not to mention, long-term meditation practices may reduce stress reactivity and have therapeutic benefits in chronic inflammatory conditions characterized by neurogenic inflammation.
5. Social Connectedness:
Do friends really matter? Jain references research studies that found people who had more friends and more social network ties also had lower levels of inflammation, fibrinogen, and albumin.
While the above-mentioned wellness behaviors and practices are great, there are many other methods that can also be incorporated in daily life. Practicing good, well-informed oral care is another good way to enhance wellness practices.
By creating and coordinating a multidisciplinary team of specialists, nurses, dieticians, social workers and psychiatrists, a so-called medical home can provide the “one stop shopping’ that inflammatory bowel disease (IBD) patients need.
The idea of a medical home is something that we have been dreaming of ever since 2014, resonating with our findings from our Stanford Medicine X workshop. It puts the patient at the center of care, using home visits, coordinated scheduling, management of pharmaceuticals, even telemedicine and digital tools, to more smoothly coordinate care, prevent emergency visits, improve patient outcomes and satisfaction, all while lowering cost of care to the system. Sounds visionary, doesn’t it?
I was excited to meet Dr. Miguel Regueiro, MD and learn about his pioneering work aiming to incorporate the concept of a medical home for IBD patients.
“For the treatment of IBD patients, we need better communication and collaboration between primary care and the GI docs.”
In addition to using the team approach to support patient care, (above) there are new digital tools, such as remote monitoring and telemedicine, that can act as supportive devices for patients to reach their care team between appointments. (below)
The good news is that not only did IBD patients benefit from this holistic care redesign, but they also experienced cost savings associated with implementing this approach.
The medical home in IBD is currently being applied at the Cleveland Clinic, UPMC, Allegheny Health Network, and UCLA. We can’t wait until next year’s meeting where we hope to hear about the progress of other medical homes in other autoimmune diseases.
Collaboration is needed throughout the entire autoimmune disease management process. This applies to research, diagnosis, treatment, and care management.
Overall, the 2019 Interdisciplinary Autoimmune Summit was an excellent update and overview of the valuable potential of collaboration to reimagine research, diagnosis, and care for autoimmune patients.
Ellen M. Martin was a co-author of the original story.
The Kenneth Rainin Foundation’s mission is simply to enhance life. They accomplish this in three different areas of focus: Arts, Education, and Health. Through these lenses, they support open-minded innovation, moving from problem identification to real action plans. Under their Health lens, the foundation conducts a yearly Innovations Symposium. This year, I was lucky to attend the 2018 Innovations Symposium in San Francisco, CA. For two days, talented people from a multitude of backgrounds presented research, created connections and collaborations and discussed various topics surrounding Inflammatory Bowel Disease (IBD).
The tone of the meeting sets this one apart from many others that I have attended. Every single person I met was deeply committed to improving the lives of all those affected by IBD. I was puzzled as to what brought these folks together until I met Jen Rainin. She brings a rare combination of pure humility and avid curiosity to her work at the Kenneth Rainin Foundation. The organization has given $20 million to IBD research in the hopes of helping researchers move towards enlightened plans of action.
Although every person I met at the conference was fascinating, three stood out as outstanding examples of translating problem identification to actionable plans for IBD.
Dr. Suskind is a pediatric gastroenterologist, is Professor of Pediatrics at Seattle Children’s Hospital and the University of Washington School of Medicine. In addition to his clinical practice, Dr. Suskind is an avid researcher seeking new ways to treat IBD and other gut diseases. His clinical research has shown support for dietary therapies in patients with IBD.[i] He and his team have shown changes in the microbial populations in IBD patients’ guts after diet therapy.[ii] Microbiome dysbiosis in the gut is defined as having decreased species diversity. You can see in this graph that after 12 weeks of diet therapy, certain patients have increased microbial diversity.
The PRODUCE study, a collaboration between Seattle Children’s and Cincinnati Children’s Hospital, is expanding upon Dr. Suskind’s initial studies and is the first large-scale multicenter study on the use of the SCD (specific carbohydrate diet) as an effective treatment for IBD in children.[iii] The PRODUCE study compares a strict SCD to a modified SCD diet in decreasing symptoms and reducing inflammation in IBD subjects. Dr. Suskind is also the founder of NiMBAL, a free website whose mission is to educate and help support families, patients and healthcare providers in incorporating dietary therapy to treat IBD.
Dr. Axelrod got his training in Elementary Particle Physics at Yale, and then Stanford where he completed his postdoc in the same field. He worked for 15 years at Measurex-Honeywell, where he became the Director of Measurement Systems and Hardware Engineering, developing complex measurement products like nuclear and X-ray sensors, before transitioning to the medical device field.
Motivated by a daughter who was diagnosed with Crohn’s Disease at 13, he applied his knowledge of the medical device field and his background in physics to create a novel product– necessity is the mother of invention. The result is a small abdominal patch called the G-Tech Patch System [iv] that harnesses the latest mobile, cloud, and materials technologies to help sense and determine digestive function.
G-tech is like an EKG for one’s gut
Started in 2011, G-Tech offers wireless, wearable patches, that easily conform to the body and run continuously for three days as the patient goes about their normal daily activities. The non-invasive patches read the electrical signals from the GI tract, generated by the muscles of the stomach, small intestine, and colon, acting very much like an EKG for one’s gut.
The patient wears the patches which transmit the signals via Bluetooth to a smartphone app, which in turn uploads it to the cloud. This data is then processed with proprietary algorithms, developed by combining math, physics, cellular biology, and an understanding of the digestive tract, to provide a report for the physician to help diagnose the underlying cause of a patient’s symptoms.
G-Tech’s studies have taught them that each person has a unique “gut-print”, that emerges over 24 hours of recording data. They refer to this period as a “gutbeat” in analogy with the heartbeat. This novel product can help personalize medicine and translate data into specific care for individual patients.
Although it is still in the prototype stage, G-Tech has already presented 12 posters, created 2 medical journal manuscripts, and with the help of the Rainin Foundation, has turned 15 million data points of raw signal into something with meaning.
Dr. Keefer is a GI Health Psychologist and the Director of Psychobehavioral Research at the Icahn School of Medicine at Mount Sinai in New York City, NY. She takes a psychological and behavioral approach to gut diseases, such as IBD, and is a pioneer in providing behavioral therapies to improve gut health for patients [v].
In her current role, Dr. Keefer oversees Psychobehavioral Research for the Division of Gastroenterology and also Co-Directs a patient-centered subspecialty medical home within the Susan and Leonard Feinstein IBD Clinical Center called GRITT-IBD [Gaining Resilience Through Transitions-] which focuses on reducing negative outcomes for patients with IBD by enhancing their resilience with behavioral tools. She remains committed to the development of self-management tools that leverage the strong connection between the brain and the gut to improve outcomes for patients with chronic digestive diseases.
Harnessing positive psychology and Cognitive Behavioral Therapy (CBT), she focuses on resilience, optimism, and social support, to help patients find the ability to self-regulate their wellbeing, happiness, and engagement in life. Dr. Keefer came up with the GRITT scoring system which uses resilience instead of stress as an endpoint.
Current models are reactive, not proactive
Dr. Keefer explained to me, “Current models of psychosocial support in GI practices have been reactive and not proactive.” She, therefore, sought a different path, by utilizing the power of positive psychology to turn the focus away from the negative (stress) to a positive (resilience). Her GRITT scoring system is built around gaining resilience through transition. There are many transitions throughout life: from sick to healthy, from pediatric to adult care, from an old job to a new one etc. By focussing on resilience, you can capture the patient’s strengths in addition to risks and vulnerabilities. In application, measurable variables are tracked with the goal of optimization. A care plan is devised, which has multiple extenders: peer mentoring, telemedicine, pain management, patient advisory board, etc.
She also uses hypnotherapy to address resilience and help IBD patients. In a study that was conducted, she found that patients that received the hypnotherapy were in remission for 78 days longer. She also found that the risk of a flare was two times higher in the control group. NBC News broadcast a video segment on Dr. Keefer and her use of hypnosis for IBD. In addition to her research and therapy, she serves as a team member/advisor for two entrepreneurial companies, GastroGirl and MetaMe health.
One thing that connects these three individuals is that their commitment and passion for their practice and research has resulted in outstanding and innovative products, ideas, and therapies, moving us all forward. With the support of Jen Rainin at the Rainin Foundation and all wonderful innovators, the hopeful future of IBD is happening now.
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[i] Lane, ER, Lee, D, Suskind, DL. “Dietary Therapies in Pediatric Inflammatory Bowel Disease: An Evolving Inflammatory Bowel Disease Paradigm.” Gastroenterology clinics of North America.” Vol 46(4). 2017. 731-744. https://www.ncbi.nlm.nih.gov/pubmed/29173518
[ii] Suskind, David et al. “Clinical and Fecal Microbial Changes With Diet Therapy in Active Inflammatory Bowel Disease. Journal of Clinical Gastroenterology. Vol 52(2). 2018.
[v] Ballou, Sarah, Keefer, Laurie. “Psychological Interventions for Irritable Bowel Syndrome and Inflammatory Bowel Diseases.” Clinical and Translational Gastroenterology. Vol 8(e214). 2017. https://www.nature.com/articles/ctg20166
Ellen Martin and Becca Maliza were also authors on this story. Click the link to read about them here.
There is a big wave of recent research on the oral microbiome and its relationship to systemic health. In this article, we will focus on links between the oral microbiome and the autoimmune disorders, Sjogren’s Syndrome, Lupus, and Rheumatoid Arthritis.
Dysbiosis (an out-of-balance ecosystem of commensal and pathogenic bacteria living in the oral cavity) may be a key factor in a variety of disorders. This includes not only obvious ones like dental caries and periodontal disease, but also systemic illnesses, like cardiovascular disease, chronic obstructive pulmonary disease (COPD), and maybe even cancer (1)(2)(3). This growing body of research offers us a more ecological and holistic understanding of the role of the oral microbiome. It also begs the question:
What other diseases could be triggered by oral dysbiosis?
To answer this question, we first look at autoimmune diseases.
An autoimmune disease is a disorder in which a body’s immune system mistakenly attacks its own healthy cells. For example, in multiple sclerosis, the current understanding is that the patient’s own immune system T-cells mistakenly attack myelin cells that make up the tissue that sheathes motor nerves (axons). This, in turn, triggers a cascade of inflammation that damages not only the myelin sheath but also the cells that produce myelin and the axons themselves. The result is a loss of motor control and even paralysis. Why these people’s T-cells go haywire is not yet fully understood, although genetic susceptibility triggered by viral and other environmental insults (e.g., smoking) is likely.
The classic understanding is that, in these cases, the immune system mistakes a normal protein on its own cells for a foreign antigen and mounts a response against the cells displaying such auto-antigenic triggers. We still have an incomplete picture of how the immune system learns to differentiate its own cells from foreign molecules and how both genetic susceptibility and environmental factors can trigger this self-destructive immune response.
New insights into immune disorders place autoimmune diseases in a larger category of immune-mediated inflammatory diseases (IMID). IMID’s are conditions which result from any abnormal activity of the body’s immune system, from allergic reactions to diabetes. Autoimmune diseases are a subset of IMIDs. They are defined as disorders in which the immune system reacts specifically against its own cells and tissues as if they were pathogens or infected cells. Researchers have also recently developed another sub-category of autoinflammatory diseases…but we’ll leave the specifics of those disorders to another post.
The last few years have seen an increase in awareness of autoimmune diseases as well as a new focus on providing better care and more health solutions for the millions suffering from these disorders. To this end, researchers have been investigating potential causes and associated risk factors that increase individual susceptibility to autoimmune disorders. One of the newest research efforts explores the relationships between the oral microbiome and systemic diseases with a special focus on systemic autoimmune diseases.
The new understanding of the oral microbiome is shaping how we think about dental caries, periodontal and systemic diseases. While the traditional view held that these diseases were caused by a small number of pathogens, we now consider the oral microbiome to be a finely tuned ecosystem, a balanced (or unbalanced) community of microorganisms that mediates not only oral health and disease but also some systemic diseases (5).
So far, three pathways that link oral infections to secondary systemic effects have been proposed:
Related Content: What Everyone Should Know About the Infant Microbiome
A number of autoimmune diseases have been linked to multiple pathogenic factors, including genetic susceptibilities, environmental triggers, and dysregulated immune responses. Dysregulated immune responses may involve over-activated B-cells stimulated by toll-like receptors (TLRs). TLRs are one of a larger category of pattern recognition receptors (PRRs). PPRs have evolved to detect proteins on or secreted by pathogens. They have also been implicated in the production of autoantibodies to nuclear and cytoplasmic autoantigens and the presence of anti‐citrullinated protein antibodies (ACPA) (7)(8). Such dysregulated immune responses can trigger progressive inflammation of certain tissues that manifests in particular autoimmune diseases such as Sjogren’s Syndrome, Systemic Lupus Erythematosus, and Rheumatoid Arthritis.
Common Oral Symptom: Extremely Dry Mouth
Sjogren’s Syndrome is an autoimmune disease that mainly affects the lachrymal (tear) and salivary glands. Thus, common symptoms include dry eyes and a significant decrease in saliva production that can cause difficulty in speaking, eating, and swallowing. Saliva is an important component in the composition of the oral microbiome due to its role in protein precipitation and biofilm formation. Insufficient saliva is associated with high bacterial species counts, as well as the frequent occurrence of caries.
In this disease, cytokines and lymphocytic infiltrates in exocrine glands cause damage that reduces secretion. Activated B-cells and T-cells stimulated by TLRs produce increased levels of inflammatory cytokines, IFN-𝛾 and IL-17, that disrupt epithelial cells in the salivary and lacrimal glands, inhibiting their production of saliva or tears and altering the mucin content. (9).
Common Oral Symptom: Lichenoid Lesions, Lupus Cheilitis
SLE is a complex, multifactorial connective-tissue disease that commonly affects joints and many organ systems including the skin, joints, heart, lungs, kidneys, and nervous system (10). The disease is characterized by the presence of autoantibodies to nuclear and cytoplasmic autoantigens.
Oral symptoms of SLE include lichenoid lesions and lupus cheilitis. Lichenoid lesions resemble a white spider web or film on the inner cheeks, tongue, and roof of the mouth. Lupus cheilitis may appear as a rash on or swelling of the upper and lower lips, sometimes including the surrounding areas of the mouth.
So far there are a couple of proposed mechanisms that link the oral microbiome to SLE. The first suggests that certain viral infections of the mouth, such as the Epstein-Barr Virus (EBV, the pathogen that causes mononucleosis, aka mono) are implicated in SLE pathogenesis. Certain EBV antigens have structural and functional molecular similarities to SLE autoantigens. Impaired EBV-specific T-cell responses in genetically susceptible individuals may trigger autoantibody responses to self-cellular antigens (11). In other words, EBV antigens share molecular similarities to SLE antigens and other cellular components, causing the cells of our acquired immune system–normally the defenders of the body–to mistakenly attack cells free of viral infection.
Another proposed mechanism that links SLE to oral microbiomes is based on recent research that organisms in the blood (blood microbiome) are associated with a number of non-communicable chronic diseases. Although the gut microbiome is the main site of origin for pathogenic microbes that infiltrate the blood, the oral cavity is another source for translocated microbes (12). A high dormant blood microbiome (i.e., the presence of detectable, but not culturable, microbes) is associated with chronic inflammatory diseases, including SLE.
Common Oral Symptom: Presence or Early Onset of Periodontal Disease
Rheumatoid Arthritis is a well-known disease (not to be confused with osteoarthritis, which is not considered autoimmune). Many people are unaware that it is categorized as an autoimmune disease, the abnormal immune reaction triggering inflammation that causes the tissue lining inside of joints to thicken. Not only joints may be affected, but also other tissues, including the valves of the heart. At the molecular level, the presence of autoantibodies, like anti‐citrullinated protein antibodies (ACPAs) contributes to a loss of immune tolerance to self-antigens and is one of the first steps toward inflammation (7).
ACPAs are a group of autoantibodies found in 50-70% of RA patients, but infrequently associated with other diseases or found in healthy individuals, making them uniquely predictive factors for disease pathogenesis. The presence of ACPAs, along with the maturation of ACPA response mechanisms, are associated with the prodrome of the disease that precedes the onset of clinically apparent RA. This preclinical RA is an entire subset of the disease itself, and has been broadly defined and broken down into six phases by the European League Against Rheumatism (EULAR).
What’s also interesting about ACPAs is that they are associated with periodontal infection with P. gingivalis, suggesting that periodontitis could be a significant risk factor for RA. Periodontal disease refers to inflammatory processes in the tissues surrounding the teeth (gums, etc.) in response to bacterial accumulations, or dental plaque, on the teeth (15). Although it originates in the mouth, it has been linked to systemic diseases–more information can be found here. The image below illustrates in further detail a step-wise process of how periodontal disease can lead to chronic inflammation in rheumatoid arthritis.
Source: Current Rheumatology Reports
Autoimmune disease is an umbrella term for more than 100 different illnesses, each presenting a variable array of symptoms. Due to the variability of symptoms and a history of disease definition by body part (joints, nerves, skin) which does not reflect our current understanding of the systemic nature of the immune system, these diseases have been difficult to diagnose and treat.
Recognizing similarities between autoimmune diseases will provide more insight into the pathophysiological processes deranging the immune response.
This will help us understand the interactions of genetic susceptibility and environmental triggers that lead to these disorders.
The mounting research on the oral microbiome and its connection to systemic autoimmune diseases is exciting. Not only could the detection of imbalances in the microbial composition facilitate the early diagnosis of autoimmune diseases, but also correcting these microbial imbalances may have potential as a treatment for autoimmune diseases.
Ellen M. Martin and Hailey Matooka were also authors on this story. You can read about them by clicking the link.
Got gout? You’re in good company given that it impacts over eight million Americans,[i] making it the most common form of inflammatory arthritis. Despite it being common, no one should have to live with gout because, today, we understand what causes it and how to treat it. Yet, despite these advances, many suffer from flares and live with avoidable pain in their joints.
It’s true that diet is a factor in gout, and the public often thinks it’s the only important factor. It’s true that being overweight raises the blood uric acid level (which we call sUA or urate). It’s also true that when we eat protein, one of the products of its breakdown is urate. Foods that have been associated with gout flares include alcohol of all types (with beer possibly being the worst), red meat, high fructose corn syrup (as in regularly-sweetened sodas), shellfish and organ meats such as liver. However, although diet is important in gout, the real cause lies in urate itself and in a person’s genetic tendency to gout.
Urate is the key to understanding gout. When there is too much uric acid, crystals can form and settle in multiple places in the body, especially the joints. High blood urate levels, called hyperuricemia, precede a person’s first gout flare. Many people are naturally disposed to developing hyperuricemia on a genetic basis. People without a hereditary tendency to gout don’t get gout flares even if they eat or drink all the “wrong things.”
Other factors can cause or set off gout beyond genetics and diet. Abnormal kidney function can raise urate levels. Taking diuretics can likewise raise urate levels.
Gout is also complicated by the fact that it is almost always (over 90%) accompanied by at least one other of a group of medical problems, including obesity, heart disease, high cholesterol/triglycerides, diabetes and kidney disease.
With this in mind, rheumatologists treating gout consider both lifestyle changes, diet, and medication therapy, all of which contribute to getting urate levels below the goal of less than 6.0. If a patient can keep urate levels below 6.0, over time gout flares almost always stop happening. Diet and weight loss are usually not enough, and most people need medication. Patience and persistence with therapy are needed!
As was made clear in a survey of 1,000 gout patients and 500 caregivers conducted by the non-profit arthritis advocacy group CreakyJoints®, gout is a disease that is out of control. Patients surveyed experienced an average of eight painful gout attacks per year and more than fifty percent reported their attacks lasted three or more days.[ii] Half of all patients did not report symptoms they experienced to their doctor and nearly one third hid attacks from loved ones.
Having two or more gout attacks per year means that it’s time for a treatment change. Flares can cause patients to have difficulty walking, climbing stairs or sleeping. And a person can develop significant functional disability. [iii]
Caregivers are impacted as well. Of those surveyed, on average, they missed 4.5 days of work to care for their loved one during a flare and the vast majority worry about gout’s impact on their loved one’s physical and emotional health. Certainly, intimacy is impacted when your partner is having pain.
Rheumatologists, primary care providers, nurses and other healthcare professionals have an important role to play in educating people with gout and their loved ones about its causes and management. It starts with setting a target of lowering uric acid levels (sUA) to below 6mg/dl. That’s why it’s important to have the urate level tested in anyone suspected of having gout. Levels should be also it followed over time to make sure it’s kept below 6. Since most people with gout need medication to control urate level, the most important part of their treatment is to stay regularly on the medication and to follow up with their doctor to have the urate levels checked over time. In addition, people with gout can help reduce flares by:
CreakyJoints recently published “Raising the Voice of Patients: The Patient’s Guide to Treating and Managing Gout.” Using easy-to-understand language, these guidelines provide comprehensive education for people living with gout. It’s a resource a family might consult in between appointments so that they know what questions to ask their doctor during the next visit. Written by patients and CreakyJoints staff and medically reviewed by rheumatologists (including myself), the patient guidelines provide an unbiased review of different management strategies, including the different medications, complementary therapies, and alternative therapies. It also suggests approaches to speaking with insurance companies and working with the larger arthritis community to advocate for patient-centered health policy and laws.
When a doctor and a person with gout work closely together, it is entirely possible to keep gout under control. Asking for support is vital. I advise my patients to talk openly about their disease with their family, friends, and employers so that those around them can help when there is a need. Online support is also available at CreakyJoints. Gout flares are not something a person needs to tolerate or expect from their diagnosis. It can often take a year or longer to get gout flares to stop, even with a perfect regimen of gout treatment. However, over the long run, a successful outcome is almost always reached. And a much better quality of life can be achieved.
The gout survey was conducted online among 1,000 gout patients and 500 caregivers of gout patients in March 2017 to gain insight into their perceptions of and experiences with gout. Edelman Intelligence conducted the survey, which was made possible by CreakyJoints, with funding from Ironwood Pharmaceuticals. “Raising the Voice of Patients: The Patient’s Guide to Treating and Managing Gout, is available for free at www.CreakyJoints.org/patientguidelines (among other volumes covering other forms of arthritis) and they were jointly sponsored by Horizon Pharma and Ironwood Pharmaceuticals.
[iii] Fu, t., Cao, H., Yin, R., “Associated factors with functional disability and health-related quality of life in Chinese patients with gout: a case-control study.” BMC Musculoskelet Disord. 2017 Nov 3;18(1):429. doi: 10.1186/s12891-017-1787-7. Accessed on May 23,2018 at https://www.ncbi.nlm.nih.gov/pubmed/29100504
[iv] Choi, HK, Karlson, EW, Willet, W. et. al, “Alcohol intake and risk of incident gout in men: a prospective study”, Lancet, Volume 363, Issue 9417, 17 April 2004, Pages 1277-1281. Accessed on May 23, 2018 at https://www.sciencedirect.com/science/article/pii/S0140673604160005
According to the Arthritis Foundation, arthritis afflicts 54 million U.S. adults and is the leading cause of disability among Americans over the age of 55. Although total joint replacements are very successful and can be life-changing, patients should not rush into surgery if they don’t have to.
Total joint replacements are meant for older patients whose pain is limiting their daily activity. All too often, younger and younger patients are requesting and having their joints replaced. This can lead to severe problems for them in the future.
The artificial knee consists of metal moving on plastic. Like the heel of your shoe, the plastic will wear down over time. In patients over 65, we know replacements last around 15 years. However, the more active you are, the faster the plastic will wear down.
The problem is the plastic debris. Plastic doesn’t break down – as you may know with our world’s environmental problem of plastic accumulation from our garbage. Over time, the plastic debris accumulates in the knee joint.
The more active you are, and the more you weigh, the more plastic debris. The white cells in your body recognize that plastic debris as foreign invaders and try to break it down. The more debris, the greater the number of white cells. Since the white cells can’t break down the plastic, they instead will attack the surrounding bone.
Therefore, when it becomes time to replace the plastic joint, we will also have to replace the bone. That is a significant surgery I don’t enjoy, and I guarantee you won’t either.
The best approach for arthritis of the knee is to wait as long as possible before joint replacement surgery.
There are several steps you can take to deal with your pain before selecting the surgical option. These measures, some of which you can perform without the aid of a medical professional, often significantly lessen the pain and improve your quality of life.
When you’ve tried all of these non-surgical measures and they don’t seem to work any longer, and you are not ready for a rocking chair and shawl, then surgery may be your best option. If surgery is necessary, rapid and successful recovery is possible by having optimized your physical and nutritional health beforehand.
First-time experiences are often marked by an intriguing spirit of curiosity and discovery. This is the excitement I felt as I attended my first FasterCures meeting in San Francisco this month.
For a long time now, there has been a discussion of a cross-disciplinary collaboration in autoimmune disease. Patients want it, as do many healthcare providers, yet still others remain entrenched in a specialist-based disease system, examining disease symptoms and body parts in isolation and losing sight of the bigger immunological picture.
I have been a contributing voice to this conversation, advocating for a shift from the current fragmented system to a more collaborative, holistic approach. I was excited to hear like-minded voices sharing their ideas and innovations on numerous panels at Faster Cures.
We are living through a time of unprecedented technological convergence, with new forms of big data such as genomics, proteomics, and metabolomics, moving understanding from a reductionist model of disease (fixing single causes) to a systems biology model of health (preventing disease, optimizing well being).
The digitization of new data sources suggests that data science must be part of a collaborative research model, as discussed by the panel Medicine Needs Data Scientists. However, this notion is not immune to the dangers of “over-hype.” Atul Butte’s panel eloquently describes how artificial intelligence and machine learning can provide new hope for medical advances, and how we can differentiate this concrete potential from substance-free hype.
Beyond the panels themselves, I felt as if I’d won the jackpot when I discovered the treasure trove of information available on the Faster Cures website. These resources support and enhance many of the same themes I’ve discussed around autoimmunity, including:
One of my main messages as an autoimmunity advocate has been the necessity of moving from problem identification and awareness-spreading to an action plan. FasterCures frames this pivotal discussion as “moving from aspiration to application”.
I refer to much of the stagnancy of progress in autoimmunity as stemming from a “language” problem in healthcare—patients, providers, and researchers are often using different vocabularies.
Claudia Williamson’s panel Health Citizenship: Creating a New Social Compact for Health articulated some of these same issues. Panelists discussed the importance of challenging assumptions and reframing conversation around the role of patients in research. They agreed that humility, active listening, and an open mindset are key to collaboration.
Continuing with a discussion of language differences between different stakeholders, Luke Timmermans’ panel Tech Culture Meets Medical Research took to the stage. Panelists emphasized the concepts of being humble, asking the right questions, and focusing on transparency between patients and providers. Both panels encouraged all of us to move from supporters to problem solvers, whether we are patients, part of patient support systems, or medical professionals.
Pulling these concepts together, I am eager to see the following Venture Philanthropy powerhouses tackle what may be termed an “autoimmune moonshot”:
To learn more about venture philanthropy in medical research, Faster Cures has created the TRAIN Central Station, where foundations that fund research may share their best practices, exchange ideas, and find relevant tools and resources.
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I have been a long-time admirer of the work of Kathy Giusti’s Multiple Myeloma Research Foundation (MMRF), which pioneered collaboration among stakeholders and has been able to bring 10 new multiple myeloma drugs to patients in need of them.
The Michael J. Fox Foundation has a laser focus on finding a cure for Parkinson’s disease. Since 2000, they have funded more than $750M while creating a variety of novel consortia, as shown below:
Another novel approach is being taken by Cohen Veteran Bioscience, currently rethinking how we study diseases of the brain, identify new targets, and advance precision medicine. Their roadmap uses tools that may also be beneficial if applied to autoimmune disease.
Looking at the meeting with deep curiosity from an autoimmune perspective, I discovered specific examples of autoimmune application:
Replays from the entire Faster Cures meeting may be accessed here.
Before attending Faster Cures, I was frustrated by the apparent lack of progress in autoimmune research. Now, walking away from this meeting and analyzing the open-minded, collaborative work of these venture philanthropists, I see a new template for autoimmune research.
What if we took the ideas of venture philanthropy, created consortia similar to the MMRF and the Michael J. Fox Foundation, established collaborators like those of Cohen Bioscience, and applied these approaches to solving “An Autoimmune Moonshot?”
Is anyone out there already working on this approach? If so, please let me know by leaving a comment.
This post was co-authored by Ellen M. Martin