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Connecting ALS is a weekly podcast produced by The ALS Association in partnership with CitizenRacecar. We aim to discuss research and technology developments, highlight advocacy efforts, and share the personal stories woven through the community.
Kelly Gwathmey:
It's definitely challenging to understand exactly the contributing factors to this disparity, and racial disparities in diagnostic delay have been reported across many different diseases, but I think a lot of work needs to be done in this area to understand where this disparity is coming from.
Jeremy Holden:
Hello everyone, and welcome to Connecting ALS. I am your host Jeremy Holden. As listeners are all too well aware, the process of confirming an ALS diagnosis is lengthy. It can take on average 10 to 15 months from the time a person first notices symptoms to them receiving a confirmed ALS diagnosis. That means that for many people living with ALS, they are diagnosed after the disease has already progressed. Delays in diagnosing ALS can cause further functional muscle loss, prevent timely access to disability benefits, and lost opportunities for medical interventions that can prove healthcare outcomes.
Reducing diagnostic delays from the patient's initial clinical interaction to confirm diagnosis of ALS in an ALS clinic is critical for early initiation of multidisciplinary care, supportive treatments, and standard of care therapy. Now, myriad factors can drive diagnostic delays. According to research published in the October 2020 issue of the Journal of Neurological Sciences, people often wait three to six months after initial symptom onset before seeing a doctor. Other factors cited in that research that elongate the time to diagnosis include referrals to specialists and misdiagnoses, which often lead to unnecessary surgeries. The researchers also noted that the age of a person at symptom onset could play a role in delayed diagnosis, and we'll share a link to that research in the show notes, so you can learn more about researchers know on what causes delays in diagnosis.
But what other factors can help us understand delays in diagnosing ALS? New research out of Virginia Commonwealth University Health ALS Clinic looked into the role that race could play in diagnostic delays for people living with ALS. I recently talked to Dr. Kelly Gwathmey, Associate Professor of Neurology, the Neuromuscular Division Chair in the Department of Neurology at VCU, about what they found. Dr. Gwathmey, thank you so much for being with us this week on Connecting ALS.
Kelly Gwathmey:
Thank you so much for having me.
Jeremy Holden:
Yeah, it's an important topic. We're pulling together two strands of discourse that we've had in the past on this show. One being racial disparities in health outcomes and access to care, but also time to diagnosis. So, I'm curious, what led your team to hypothesize whether race impacts time to diagnosis for people with ALS?
Kelly Gwathmey:
Thank you, Jeremy, for asking this. Our ALS clinic team noticed that a lot of our Black ALS patients were coming in with much more advanced disease at their initial clinic appointment, which is really what opened our eyes to this potential problem. And when we first meet a patient, we perform not only an exam, but also the ALS Functional Rating Scale as well as the respiratory testing to see what our patient's respiratory numbers are, to understand the severity of the disease. And we were noticing that our Black patients were coming in with a lot more urgent needs, such as they needed durable medical equipment, they needed respiratory equipment, they needed a feeding tube placed, and so on. And so our Black patients would often come in with more advanced disease and not qualify also for certain clinical trials. So, it was just our experience that really drove us to look into this systematically.
Jeremy Holden:
So, you have your hypothesis, you've made some kind of tentative observations. So how did you go about collecting data to test whether what you were observing was in fact, did the data bear that out?
Kelly Gwathmey:
Thank you, Jeremy. This was a retrospective review of all the ALS patients we've been seeing at the VCU Health ALS Clinic going back to 2017. And so we mostly analyzed our patients who were seen for their very first ALS multidisciplinary clinic visit in the VCU system as opposed to patients who were transferring over from another multidisciplinary clinic, because we really wanted to extract the patients that had the most complete datasets. And so what we were looking at was basic demographic data. For example, in this study, we analyzed 174 white patients and 35 Black patients, and we looked at the time from symptom onset to the date of diagnosis, so the diagnostic delay, and then we looked at their initial ALS Functional Rating Scale score as well as their upright forced vital capacity at time of that very first visit.
Jeremy Holden:
And so what did you find?
Kelly Gwathmey:
Sure. So, again, the breakdown of who we studied, I would say it was weighted slightly more towards males in both our white patient group and our Black patient group. And then in keeping with what we all know about ALS, we saw that nearly about 75% of our patients that we analyzed were limb onset and the remainder were bulbar onset. And then in terms of diagnostic delay, it was significantly longer for our Black patients. The average was 15.8 months from symptom onset to diagnosis in our Black patients, and then 12.9 months in our white patients from symptom onset to diagnosis. And then similar trend in the ALS Functional Rating Scale, as you'll recall, the maximum perfect score is 48 points, and so in our Black patients, they were coming in to us with an average score of 33.9 points and our white patients were coming in with an average score of 39.4 points.
And so that was clearly statistically significant both for the diagnostic delay as well as the ALS Functional Rating Scale at time of presentation. So again, similarly with the forced vital capacity tested upright, our Black patients came in with about a 65% forced vital capacity and our white patients had an 81.6% forced vital capacity. Again, statistically significant. And this is quite impactful data if you think about it, that we try to get patients to have their feeding tube placed if they choose to proceed with that with, with a vital capacity at or above 50%. And then for most clinical trials, to qualify, you need a vital capacity somewhere in the 65 to 70% cutoff range. Right there on average, our Black patients who are seeing us for the first time in clinic already were barely qualifying for clinical trials, which is extremely concerning.
Jeremy Holden:
You're looking at different variables that could potentially explain this statistically significant clinically meaningful data. Were you able to look at what role, if any, maybe socioeconomic status played?
Kelly Gwathmey:
I think that's an excellent question also. And right now for our patients, this remains mostly unknown. We recently obtained some additional data and actually have a manuscript submitted looking at this. And as this was a retrospective study, we had access to only a few data points. We knew the distance to the clinic for these patients and we knew the median household income by the residential ZIP code reside. And that was pretty much the best we could do to look at socioeconomic status. So, we found that our Black patients lived closer to clinic and had a higher proportion living in the inner city of Richmond, but in less affluent areas with a lower median household income. So, looking at our Black patients, the average household income, again by ZIP code analysis, was about $55,000 per year compared to our white patients where it was $69,900 per year.
Jeremy Holden:
So, what factors then can we look at or what factors do you see explaining the disparity in time to diagnosis?
Kelly Gwathmey:
It's definitely challenging to understand exactly the contributing factors to this disparity, and racial disparities in diagnostic delay have been reported across many different diseases.
Jeremy Holden:
Sure.
Kelly Gwathmey:
And there are some common themes, right? So, potentially physicians' implicit bias, which is defined as negative behaviors resulting from unconscious attitudes towards particular demographics, and in this case race and ethnicity. Perhaps that's hindering our Black patients from receiving the diagnostic evaluations that they need or perhaps resulting in misdiagnosis. Again, somewhat anecdotally from our experience, a lot of our Black patients ended up being initially thought to have had a stroke or have significant spine problems causing this, which resulted in unnecessary spine surgeries. And then also the concept of structural racism, which is also termed institutionalized racism, and that's characterized by differential access to goods and services and opportunities. So, do our Black patients have less access to healthcare than our white patients? And so that's certainly a concern, but I think a lot of work needs to be done in this area to understand where this disparity is coming from.
Jeremy Holden:
I don't want to get too far ahead of ourselves, but it sounds like a conversation you and I might be having a little bit later this year may touch on some of that, so we can stay tuned for that. The population of the research that we're talking about here today was generally speaking Richmond, surrounding areas, but the footprint of Virginia Commonwealth University. Is there anything unique to Richmond or is this data, can we extrapolate these findings and think that similar outcomes, similar disparities would exist in other regions, other states, other localities?
Kelly Gwathmey:
First I'll say I think the Richmond area is the perfect environment to study this, just because Richmond itself is about 50/50 Black and white population. And so by nature of that, we just have a higher percentage of Black ALS patients than perhaps some other clinics in the nation, and so it's an excellent opportunity. That said, you're asking does this disparity exist elsewhere in our country or elsewhere in the world? And I must admit it's relatively understudied, but there are some similar trends. So for example, the Emory Group in Atlanta, Georgia did look at this in 2021, they published it in the Green Journal, in the Neurology Journal, and they analyzed the patients that they saw in their large multidisciplinary clinic and also found that there was a greater diagnostic delay for the Black patients and lower baseline ALS Functional Rating Scale scores, lower baseline respiratory numbers in the Black patients. So, our data very much mirrors what they've already published, but otherwise, there's not a lot of work or data out there to analyze to understand if this is more of a national trend.
Jeremy Holden:
The kind of classic thing we see in research is, and I don't mean this to be glib, but more research needs to be done. We identified a question that needs to be asked more broadly, right?
Kelly Gwathmey:
Absolutely. And as Black patients in general are significantly underrepresented in clinical research nationally in ALS, I am concerned that one of the reasons we're not getting our Black ALS patients in clinical trials is because they are presenting later in their disease course, and so that's why this work really needs to be done at a national level.
Jeremy Holden:
And I know you have some thoughts on ways we can start tackling this problem, and it's, and I know I alluded to this earlier, but a conversation we'll be having later this year. Very much looking forward to that. Dr. Gwathmey, before I let you go, I know that VCU's Multidisciplinary Clinic was recently named a Certified Treatment Center of Excellence. What impact does that designation have on the work that the clinic is doing and can do going forward?
Kelly Gwathmey:
Yes, thank you, Jeremy. Our VCU Health ALS Clinic team is very honored to have received that designation, and we certainly hope that given this Center of Excellent status, it'll allow us to continue to raise awareness of diagnostic delay that seems to be more negatively impacting our Black ALS patients, at least in our region. And in a time when earlier diagnosis means earlier access to disease-modifying therapies and improved survival, everyone deserves equal access to high-quality care and good patient outcomes.
Jeremy Holden:
Looking forward to the continued great work out of VCU. Dr. Gwathmey, thanks so much for your time this week.
Kelly Gwathmey:
Thank you, Jeremy, for having me.
Jeremy Holden:
I want to thank my guest this week, Dr. Kelly Gwathmey. If you liked this episode, please share it with a friend, and while you're at it, rate and review Connecting ALS wherever you listen to podcasts. It's a great way for us to connect with more listeners. Our production partner for this series is CitizenRacecar. Post-production by Alex Brouwer, production management by Gabriela Montequin, supervised by David Hoffman. That's going to do it for this week. Thanks for tuning in. We'll connect with you again soon.