Dr. Deborah Friedman at the 2023 Cedars-Sinai Intracranial Hypotension Conference
Dr. Deborah Friedman, neuro-ophthalmologist and headache medicine specialist, presented this talk sharing updates on her quality of life research study at the 2023 Cedars-Sinai Intracranial Hypotension Conference on July 9, 2023. The conference was hosted by Cedars-Sinai with generous support from the Spinal CSF Leak Foundation in Kohala Coast, Hawaii.
Transcript
[00:00:12] I am really pleased to be able to present this data. Our research was funded by the Spinal CSF Leak Foundation. And I’m going to kind of give you two parts. One part we presented at the American Headache Society, and then I have some new hot-off-the-press stuff. So I don’t think I need to tell you guys how disabling this is.
[00:00:29] There have been a couple of studies, both were published in 2022, looking at impact on quality of life and finding that there was significant impact in patients with confirmed leaks. Unemployment, social life, health, well-being, persisting after treatment in nearly 50 percent of people. But we also know that we can’t always make the diagnosis.
[00:00:51] So what we did was a single center, cross-sectional cohort study at our headache and facial pain program at UT Southwestern. We had a waiver of consent and we recruited patients with either a confirmed diagnosis based on ICHD criteria, or people that we clinically suspected of having SIH but we could not confirm the diagnosis, because our experience in clinic was that these people were suffering just as much as the folks we made a confirmed diagnosis on.
[00:01:20] So we identified patients by looking at both ICD-10 codes as well as procedure codes, because previous blood patch actually turned out to have a bigger yield than the procedure codes did, and contacted people. to participate. Everybody completed five validated questionnaires using REDCap. And they were also invited to be interviewed for a suicide screen.
[00:01:47] So the questionnaires that people filled out were the SF-36 for general quality of life; the PHQ-9 for depression; GAD-7 for anxiety; FACIT-Sp-12, which is a a questionnaire that assesses spiritual well-being during chronic illness therapy; and the headache impact test. And then the suicide rating scale was a C-SSRS which is commonly used in research studies.
[00:02:14] So, of the 234 patients that we invited to participate, 106 completed the questionnaires, 95 provided all the information we needed, and that was our analysis cohort. And then of those 95, 67 agreed to be interviewed for the C-SSRS, because it requires an interview. You can’t just fill it out on a piece of paper.
[00:02:36] So, as you can see, as far as the diagnosis status, we had 59 patients with a confirmed diagnosis. 36 did not have a confirmed diagnosis. 73 of those patients still had symptoms for whatever reason. And 22 did not because they were probably effectively treated. It was mostly women average age, about 50, almost overwhelmingly white. And most of the patients were married. 93 of the 95 patients had had at least one blood patch.
[00:03:07] Looking at our results, looking at headache impact, three quarters of patients had severe disability with a median score in the most severe range of the HIT-6. Health-related quality of life was impaired across all the eight domains of the questionnaire. The physical mean score was 30.3. The mental was 36.9. Depression scores, mean was nine, which is not actually as bad as I thought it would be. But 78.5 percent reported symptoms that interfered with their ability to function that were relevant to their depression. And you can see the spread of mild, moderate, moderately severe, and severe depression.
[00:03:51] Anxiety median score was six, which is mild. But there was a quite a range, and a little over a quarter were in the moderate to severe range for anxiety. Spirituality, which has never actually been studied in any headache disorder previously, the median score was 27. And this is actually significantly lower than what was found in the validation studies for this questionnaire, and it was used to validate spirituality in patients with AIDS and cancer. So, our group actually had scores that were significantly lower than people with AIDS and cancer.
[00:04:33] Looking at suicidal ideation, a little over 60 percent expressed some degree of suicidal ideation. 40 percent had previous ideation. About 15 percent had a specific idea of how they would commit suicide, they had a plan. And 25 percent had exhibited previous suicidal behaviors, including suicide attempts and preparatory actions towards suicide. Ideation and behavior were both lower within the three months of our assessment, and fortunately no participant was actively suicidal with a plan in mind. But the C-SSRS was consistent with the question on the PHQ-9 about expressing thoughts of being dead or self-harm within the previous two weeks.
[00:05:26] Interestingly, and I think quite relevant to what we’re talking about just in the past couple of hours, there were no significant differences between participants who had a confirmed diagnosis and a suspected diagnosis. And scores for people who had no SIH symptoms following treatment, or spontaneous resolution, were comparable to the general population.
[00:05:52] But general quality of life scores were markedly inferior to the general population, and lower than reported values for patients who have MS and IIH. So I think that this shows to what a great impact that this disease has on people. This was the first study that looked at suicide, and suicidality, as you saw was pretty prevalent.
[00:06:16] There are lots of limitations. It was a single center study. They were highly educated people. Most were white. They were evaluated by neurologist. They probably were more complex and refractory than the patients that are treated in the community just by virtue of being seen at a quaternary headache center that likes to treat CSF disorders. So definitely there were probably biases of ascertainment; participation, particularly for the C-SSRS; recall; and self-reported diagnosis status. We were not able to get good data about pre-existing depression or anxiety. We tried, we looked at the charts and it just was impossible. As well as headache may have influenced the results. And we also didn’t account for coexisting conditions such as POTS and joint hypermobility.
[00:07:06] But we conclude that the emotional, the physical, and the spiritual impacts of SIH are considerable. Participants scored poorly across all domains of the quality of life measures that we studied. Almost three quarters scored in the most severe HIT-6 category, similar to previous reports. The major impact is probably mostly attributable to limitation of upright time because of the orthostatic headache and the severity of headache. The prevalence of suicidal ideation and behavior was higher in our cohort than what has been reported in migraine and similar to what has been reported in cluster headache. And many patients, when they had the interview, for the C-SSRS expressed the fear that they would never get better. So it just shows us we have a ways to go.
[00:07:57] And then I want to just show you this. That was part one of what we did, because we were working on an SIH impact inventory specific to SIH, and so I just want to show you some of the preliminary findings that we have. Certainly there are a lot of things we haven’t analyzed yet.
[00:08:15] So we asked patients about their symptoms. And almost everyone had headache, neck pain, problems with concentration, and some of the other data we’ve seen at this meeting put brain fog, if you will, down on the bottom of the list. For our people, it was up on the top of the list. Exhaustion, sensitivity to light, balance problems. I mean, you can see the list. But all of the responses were at least, you know, 60 percent of people. And we just took the top 20. We looked, we asked about a lot of different things.
[00:08:46] We asked about what SIH has impacted. I hope you can read the slides. Marital status. Strongly agree. Agree. We’re about 20 to 25 percent each. But there were some people that said it didn’t affect their marital status at all. We do have data about whether it led to divorce or separation and we just haven’t analyzed that yet.
[00:09:07] Affected their parenting ability. I would say most people said yes. The relationship with their children. Definitely. Most people said yes. Affected their ability to work. Yes. Their ability to drive; many about 40 percent said it affected their ability to drive. And about 80 percent said that it affected their ability even to ride in the car as a passenger. Things that we take for granted.
[00:09:35] We looked at the time to diagnosis. And this is in years. Again, this is probably more of a refractory group but our mean time to diagnosis was two years. IQR ranged from half a year to almost five years. And you can see the spread. And then there were people, not an insignificant number, that had waited 5 to 10 years, or even longer than 10 years, to be diagnosed.
[00:09:59] Three quarters of patients had been misdiagnosed. And the most common misdiagnoses, probably not surprisingly, were migraine, chronic migraine; a psychiatric issue in almost a quarter of people; a vestibular issue; or high pressure. And 60 percent of people felt that they had been dismissed by their previous providers.
[00:10:21] As far as treatment, almost all of them had blood patches. That was likely just a bias of how we selected our patients. Mean number of blood patches was four. Months of relief on average was a month and a half. And again, you can see the spread of the duration of effectiveness of blood patches. Percent that said they experienced rebound intracranial hypertension after a blood patch? About 60%. So not not unlike what they found at Duke that we heard earlier today. And 17 percent said they had complete resolution following a blood patch.
[00:10:55] We looked at financial impact, which was reported by 64 people, and that included the medical expenses, what they spent on travel for health care, having to have somebody else come in and clean the house, having to pay for somebody else to drive them around, and even having to have somebody help come in and do personal care. The impact on employment, about 65 percent had worked for wages. And overwhelmingly they indicated that SIH impacted their ability to work, their ability to be promoted. And 65 percent had to stop working because of their symptoms. We had 18 people who were in school, and they all agreed that SIH impacted their ability to attend school or complete their coursework.
[00:11:38] So, so far it looks like there are substantial diagnostic delays and misdiagnoses. Only a less than 20 percent had a lasting effect from blood patches, and SIH seems to affect all aspects of their daily lives. So what we still have left to do is to analyze the findings by their diagnosis and symptom status and report on some of the other variables assessed. And we asked for a lot of stuff. We asked for upright time. We asked did this affect your sex life. And a lot of other things that we thought might be important to people. And then correlate that to our standardized questionnaire.
[00:12:15] So, just many thanks to my corroborators and collaborators. Victor, who is a fourth year medical student who started working on this in a second year of medical school, and he is phenomenal. And he did just more than the lion’s share of the work. And then Dr. Morgan McCreary, the biostatistician who’s working with us. So thank you very much.