In advance of this year’s Sjögren’s Foundation annual patient conference on 29-30 April, AllAfrica’s Tami Hultman spoke with Dr. Nancy Carteron about her work and her book A Body Out of Balance: Understanding and Treating Sjögren’s Syndrome,written with Ruth Fremes, about an underdiagnosed illness that affects millions of people across Africa and elsewhere.
Sjögren’s is thought to be the second most common autoimmune disorder after rheumatoid arthritis. The syndrome is systemic, affecting multiple organs and body parts, from the brain to the toes – making life a daily challenge for many.
Symptoms develop slowly and inconsistently, characterized by ‘flares’ that come and go. Patients – most of whom are women – typically are bumped from specialist to specialist – ophthalmologists, cardiologists, pulmonologists, otolaryngologists, gastroenterologists, gynecologists and neurologists.
Symptoms are diverse and present differently in each person. Among the most common are dry eyes, cloudy vision, dry mouth and difficulty swallowing; dizziness, imbalance and irregular heartbeats; itchy skin, rashes and tingling toes; Reynaud’s syndrome, characterized by cold hands and feet; early fullness, abdominal pain and weight loss; aching muscles; swollen glands; and frequent infections of lungs, urinary tract and sinuses.
Physical examinations are often ‘normal’. Some severely affected Sjögren’s patients never show positive results on any existing laboratory test. Typically, a diagnosis takes years, sometimes after invasive procedures, including exploratory surgeries. Sometimes Sjögren’s is misdiagnosed as multiple sclerosis or fibromyalgia or as idiopathic, meaning symptoms having no known cause.
In North America, Europe and Asia, Sjögren’s is widely recognized, especially by rheumatologists, even though too few know how to diagnose or treat it. Africa – with more widespread deadly diseases such as malaria in many places, and with so many African-trained doctors and nurses recruited to fill the healthcare needs of wealthy countries – has too few of the specialists needed for tackling autoimmune disorders.
A January 2022 article in the Journal of Clinical Epidimiology, which looked at 21 hospital-based studies of Sjögren’s in African nations, says available data suggests a similar rate across Africa as elsewhere in the world. But the authors say: “Extensive and high-quality research is needed”.
Today, African patients’ lack of access to specialized diagnosis and therapy for Sjögren’s is intensified by the crisis of Sars-CoV-2. A pandemic-beset world is benefitting from research, clinical trials, and genetic sequencing by African scientists, which alerts us all to new variants, effective mitigations and approaches, even while inequity of vaccine distribution means that fewer than 12 percent of sub-Saharan Africans have received a single Covid vaccination.
Attention to other diseases has suffered. But Dr. Carteron’s book on Sjögren’s is a resource for both patients and physicians confronted with a confusing array of seemingly unconnected complaints – and few places to turn for answers. It recounts patient stories and suggests ways to cope and how to find expertise.
AllAfrica conversations and interviews are lightly edited for clarity, flow and length.
Why were you drawn to specializing in a disease whose diagnosis is elusive – whose symptoms are chronic yet variable?
Twenty years ago, it was just an extension of being curious. That’s the essence of what got me started in science. In college in the ’70s, it wasn’t a normal path, or at least not with my friends. I was working at [Johns] Hopkins [in Baltimore, Maryland], as a lab technician, when I decided I wanted to pursue an advanced degree. I think it was curiosity; clinical medicine has always been, for me, problem solving.
Medical training puts people into boxes – it’s a kind of pattern recognition. But if those patterns don’t fit together, the system doesn’t know what to do. I work with medical students, and I see it. I think it’s a structural mindset. And that doesn’t help the patient.
You’re a clinician, a rheumatologist. You practice medicine. Was it a challenge, at least early in your career, to avoid the accepted methodology for diagnosing your patient’s problems?
If you have a scientific, bench research [basic research] background, you ask, “What are the facts”? You’re collecting pieces of data, creating a hypothesis, and then testing the hypothesis – and moving forward, seeing if there’s other data. That’s why I don’t think it ever was an obstacle for me.
The obstacles were the time you were allowed with patients – and, more, the financial structure that I ran into being in a private-practice setting – or even now in academics.
But it was fun, right? It’s pretty rewarding to be able to connect the dots, or really help somebody connect the dots, and then either prove or disprove the hypothesis.
By working with a patient, being able to see, overall, the person, brings another dimension. If it’s chronic, it’s frustrating; you can’t ‘fix’ it. But still, what I saw over and over again, was that in about six months or a year, if the hypothesis was right, the focus could become working on the right things to help patients get better and not make things worse. That should be the mission of a healer – to set somebody on the right path.
The other thing that was eye opening is that you could have somebody who would have seen ten to 20 practitioners – and good practitioners at good places with solid training. I was taught at a good program that Sjögren’s was a nuisance disease, just a little dry eye syndrome.
I just had a sense that in my career lifetime there would be insights and understanding into some diseases that there weren’t at that time. I find that if I’m focused on something that’s challenging, but also has value for the community or the greater good, that was what I wanted to do professionally. The frustrating part has not come from the patients; it’s come from the structural healthcare system.
Talk about that more.
When I first got involved with Sjögren’s, the data suggested that it took six years to get a diagnosis, on average. I remember sitting on the board of directors of the Sjögren’s Foundation and saying, “I think this should be a three-month diagnosis. We have the technology, we have the skill set, we just don’t have the interconnectedness”.
In the real world, you know that you’re probably not going to see that happen until there’s an FDA- (Federal Drug Administration) approved drug specifically for Sjögren’s. That drives resources. We also don’t have the therapeutic interventions we should, although there are more now. It’s a shame, but that’s what I’ve experienced.
So much of what you said is similar to what scientists say who have worked for years on polio or malaria or even coronaviruses. When there’s a disease that affects people in wealthy nations, and there are vast profits to be made, vaccine development is breathtakingly fast. It’s not an accident that the World Health Organization has done so much to respond to Covid and to champion vaccine equity. WHO’s Director-General Dr. Tedros is an African – an Ethiopian – epidemiologist. Even so, the structural impediment to curbing Covid globally, when it perhaps could have been contained, are similar, though the disease is very different in being so contagious and deadly to so many.
There’s a lot of overlap. The older you get, you see such a waste of resources that we as different societies make. I always come back to the question, “What happened to just common sense”. You know, so much of this is not rocket science. I saw the public health system in our country starting to disintegrate at the beginning of my training.
Here in San Francisco, we have been fortunate that the HIV experience made the infrastructure in the Bay Area very different than the rest of the country. Public health officials still had some of that mindset with the Coronovirus, but that obviously didn’t translate to the rest of the country.
The HealthWell Foundation helps people with chronic conditions “because no one should go without essential care”.
The healthcare advocacy foundation that I cofounded 15 years ago [the HealthWell Foundation assists people who are under insured with their co-pays and medication bills. Working with insurers at the executive level and with healthcare attorneys, you see the same structural problems. The only way that’s going to change is to have more transparency, so that people across the spectrum can at least see what’s happening.
Because what happens is skewed. The only two countries in the world that advertise prescription drugs on TV are the United States and New Zealand. There’s a reason TV commercials don’t have drug prices!
Maybe it comes back to philosophy. Do you have a philosophy of wanting to preserve your own tiny world? Or do you want to contribute to a wider society?
You mentioned people, often having to see 20 doctors, different specialties trying to understand their diverse symptoms. Does that way of thinking in siloes spill over into public health and international assistance? I once visited a health clinic in Mozambique with the Minister of Health, who wanted to show me that one side was freshly painted and attended by doctors and nurses, while on the other side were people standing waiting in long lines. And he said, “This side is the HIV clinic. Finally international donors woke up to the scourge of AIDS around the world, and we’re getting money for HIV. But we can’t use that for developing our health system. So over here you have pregnant mothers who aren’t getting sufficient prenatal care, because we’re a tiny, poor country, living with the legacy of half a century of Portuguese colonialism.”
Right, it’s all connected. It was so frustrating when I ran my own small business, my medical practice, and had to deal with the finance part. It would cost so much less to have an integrated approach in the beginning. Then, as managed care started to come in the early 90s, I was told by our medical group that I needed to see easier patients, or that I should see Lupus patients only once, when I knew that if I saw them two or three times, I could get the right information to send to their primary [doctor].
It took a little bit more resources, but almost none of my patients ended up in the hospital, because we managed it as an outpatient. So that was less costly.
So I was like, but why don’t you care about that? The answer was because the money comes from two different buckets. And I thought, if physicians don’t care about preventing people from serious illnesses, this is really wrong. I resigned from that group.
I had patients that we had worked really hard on getting them better and back to being productive. And their insurance would change and now the copay on their drug every month was $2,000. And then they got sick again.
Sometimes things are complicated, but often, they’re really simple. Nobody wants to make those decisions, so they push it off to the side. Unless you have all the shareholders and stakeholders willing to really grapple with those difficult questions, that won’t change.
So that leads to the question of what can be done within the system that exists? What should patients and doctors do about a syndrome that overlaps in symptomology with so many other things?
Just being aware, is important, being educated about the diversity of symptoms, so that the thought of Sjögren’s even crosses people’s minds. And then it’s having access to a referral. I think globalization and Coronavirus has allowed a little bit more crossing boundaries, so information can be in patients hands, and they can put together their own care teams.
Twenty years ago it was rare to have patient involvement in their own care. Now that’s more standard. But it’s still siloed, and time is still limited. If you have five or 15 minutes to spend with a complex illness, there’s no way you’re going to get anywhere. You just can’t.
So, what can a doctor do? I think listening to what the patient’s complaints are, and being open and honest with them.
Empowering patients is part of what the Sjögren’s Foundation tries to do, I gather, and what your book tries to do. It’s important to say again that there is fabulous science across Africa. We wouldn’t have known about Omicron for weeks, possibly months, after it emerged, had it not been for scientists in South Africa and Botswana. At the Pasteur Institute in Dakar, Senegal, so much important research has gone on. But that doesn’t mean that a rheumatologist practicing in Botswana or Senegal has the time or the up-to-date information to diagnose and treat a disease like Sjögren’s. Your book is still in print and available online, and there is new research all the time. That’s where the need for awareness comes in – to know what resources there are.
The Sjögren’s Foundation and quarterly [report] – any practitioner, I think, in the world, can get access to that without charge. The patient conferences since Covid have been virtual. Anybody in the world can sign up, although it’s in English. There are several good blogs. Sarah Schaefer, a primary care physician in this country who has Sjögren’s, has a great website with incredible curation of papers. It’s freely available.
There are creative ways to access information from specialists. For example, ultrasound of salivary glands is relatively inexpensive and can be shared on an iPhone. Clinics doing ultrasounds for babies should know they could do ultrasounds for Sjögren’s, if a patient has those symptoms. There’s a reason that you would want to know that somebody pregnant might have Sjögren’s, to manage the risks.
It’s useful to know key questions to ask and that there’s an overlap with chronic viral illnesses such as HIV, hepatitis C and lymphoproliferative disorders that can look like Sjögren’s. It’s important to distinguish the two, to know whether to treat with antivirals or with immune modulating drugs.
Part of a health-care provider’s mission is knowing the patient first, and the context, right? It’s ‘do no harm’. Assessing the facts of what you’re dealing with in whatever part of the world, and then using the knowledge that you have, or have access to and resources you have – then we’ve done much of value.
We’ve been going for a while; I’ve taken a lot of your time. A final question: does Covid change therapies you’d recommend for Sjögren’s now?
Yeah, absolutely. A mainstay of medication for Sjögren’s and some other autoimmune disorders has been Hydroxychloroquine. There’s probably not a risk factor there.
But I’m not sure I would recommend some of the other medications for patients now, because of Covid. For example, even five milligrams or less of prednisone is known to cause increased susceptibility to Covid.
So it’s a complex balancing of needs versus risks?
Well, it seems to me you have found your calling and have ended up in a place where you can help patients with a multidisciplinary approach, which is what is needed.
It’s too bad that the systems are such inhibitors. I feel fortunate to be able to work in a multidisciplinary way, but we see people from across the country who still cannot get a comprehensive workup where they live. So there’s more work to be done. And it’s got to resonate, it’s got to make sense for their other care teams, right?
In the U.S., I think you may see a major advancement with possible funding at a national level. If Sjögren’s does get research grants, we could see more information available to all researchers. There are clinical programs that are in phase two, phase three. Once one of those gets FDA approval, that will drive awareness and interest in Sjögren’s, at least in the U.S.
Dr. Nancy Carteron is a health sciences clinical professor at the Sjögren’s Clinic of the University of California Berkeley and volunteers as a clinical professor of medicine at the University of California San Francisco. She is a Fellow of the American College of Rheumatology and has served on the board of the Sjogren’s Foundation. The HealthWell Foundation she cofounded has awarded over U.S. $2.7 billion in co-payment assistant for under insured patients and helped nearly three-quarters of a million people obtain medications. It has awarded more than $818 million in grants since 2021.