September is Hydrocephalus Awareness Month

Show Notes

Did you know that September is Hydrocephalus Awareness Month?

Lindsay Clarke, Vice President of Health Education and Advocacy, talks with Diana Gray, President and CEO of the Hydrocephalus Association, about what hydrocephalus is and why an awareness month is so important.

Learn more at www.hydroassoc.org.

Episode Transcript

Lindsay Clarke:

Hi, everyone. Welcome to This is Growing Old, a podcast from the Alliance for Aging Research. My name is Lindsay Clarke, and I’m the Vice President of Health Education and Advocacy at the Alliance for Aging Research. I’m glad to be here hosting this latest podcast and to be sharing important information about hydrocephalus. This is very timely because September is Hydrocephalus Awareness Month. And here to talk to us about what hydrocephalus is and why an awareness month is so important is Diana Gray, President and CEO of Hydrocephalus Association. Diana, welcome to This is Growing Old.

Diana Gray:

Thank you, Lindsay. It’s a pleasure to be here.

Lindsay Clarke:

Well, to start off, can you tell listeners what hydrocephalus is and the mission of the Hydrocephalus Association?

Diana Gray:

Hydrocephalus is actually an abnormal accumulation of cerebrospinal fluid in the cavities of the brain, called the ventricles. And when this cerebrospinal fluid builds up, it causes these ventricles to enlarge. So sometimes we’ve seen babies with big heads. And then the pressure inside the head increases. And so that pressure has to be alleviated. And that means the treatment is brain surgery, unfortunately, to shunt either naturally through something called an ETV where they bore a hole in the third ventricle of the brain, or more commonly in a shunt that helps alleviate that pressure and move that fluid into other parts of body.

Diana Gray:

And you asked about our mission. So our mission, we’ve been around since 1983, and our mission is to find a cure for hydrocephalus and to improve the lives of those who are affected by the condition. I should say that 1 million people in the United States are estimated to have hydrocephalus and anyone at any time can develop hydrocephalus.

Diana Gray:

So it isn’t just… Use the example of a baby with an enlarged head, but really any traumatic event can cause someone to develop hydrocephalus, whether it’s a baby being born and having a brain bleed or an infection or someone getting hit in the head with a baseball, and suddenly there’s a trauma. Someone in our military who has a traumatic brain injury could develop hydrocephalus. And that I know we’re going to talk in a moment about this elderly population that develops something called normal pressure hydrocephalus, which isn’t known very well. So I know we’re going to chat about that in a little bit because dementia is one of the key symptoms.

Lindsay Clarke:

Thank you for that. So you mentioned that it can happen at any age, but my understanding is that the symptoms can differ by age. So can you tell us a little bit about what the symptoms are for older adults and some of the challenges for the condition in older adults in particular?

Diana Gray:

Sure. Yes. So as we talked about earlier, often, there’s a trauma with the younger person who gets hydrocephalus. And so then there’s typically some kind of diagnostic that would indicate that their ventricles are enlarging or there’s pressure, there’s headaches, the person doesn’t feel well, or it’s an emergent situation where we’ve got to fix this right away and save this person.

Diana Gray:

But with normal pressure hydrocephalus, it often mimics other types of dementia. So sadly, it’s estimated that there are about 700,000 of that million people with hydrocephalus who are actually older and have normal pressure hydrocephalus. And they often exhibit a triad of symptoms. So one of them is dementia, one of them is a gait problem, and the third is incontinence. Now you don’t have to have all three to have normal pressure hydrocephalus. But if you do have all three, it’s very indicative that you have normal pressure hydrocephalus. And this typically happens in an elderly population. In fact, it’s estimated that 9 to 14% of people in nursing homes have normal pressure hydrocephalus or NPH. And people 80 or older, probably 5.9%, almost 6% have normal pressure hydrocephalus.

Diana Gray:

So how do you distinguish? How do you distinguish between Parkinson’s, which also has a gait disorder? My father had Parkinson’s. And Alzheimer’s, which also has dementia related to it. And quite honestly, as you age, you may have more than one condition. You could have Alzheimer’s and normal pressure hydrocephalus, which makes it even harder to distinguish. However, often, the very first sign of normal pressure hydrocephalus is this gait disturbance, and it doesn’t necessarily look the same as a Parkinsonian gait problem. For example, you hear about this magnetic… the feet, the person had difficulty getting up and lifting their feet. They’re almost like magnetized to the floor, like magnets are keeping them from walking.

Diana Gray:

So that’s an indicator. And that’s often something that a neurologist or a neurosurgeon would check. If someone came in and was exhibiting and gait disorder, they would check for the type of gait and how quickly they were able to move in addition to checking an MRI to look for enlarged ventricles, which would be indicative of hydrocephalus.

Diana Gray:

Sadly, a lot of elderly folks are just given the diagnosis of dementia and those aren’t checked. I mean, the psychological testing might be similar for dementia of Alzheimer’s or dementia of normal pressure hydrocephalus, although there can be some differences. But it isn’t always the case that an MRI is ordered. And so someone who could have what might be a treatable dementia like normal pressure hydrocephalus where a brain surgery or a shunt could alleviate the symptoms, sometimes very quickly and sometimes over time, they may go undiagnosed. So we really want to get the word out that there is this tradable dementia called normal pressure hydrocephalus.

Lindsay Clarke:

Thank you. That was so incredibly informative. And one of the things that occurred to me as I was learning about hydrocephalus and as you were talking is… and you underscored this, but I just want to bring it back too… is that this under-diagnosis and this undertreatment is such a problem because it could be something that’s treatable and they may be being treated for something that they don’t have. So really thank you so much for sharing that with us. I think it’s so important that you have an awareness day for hydrocephalus. Just echoing again that it’s the September. Why do you believe that an awareness… I’m sorry… month is so important and how can our listeners get involved?

Diana Gray:

Yes. We have the whole month of September for Hydrocephalus Awareness Month, which we call HAM. It’s really critical because of what we just talked about. A lot of people don’t know what hydrocephalus is even when it’s something diagnosed in a child where they know they have it. But when you think about this elderly population who may have something that’s treatable and there’s no awareness, it’s really critical to get the word out that this condition exists. I was surprised when I joined this organization six years ago how few people do know what hydrocephalus is or even how to pronounce it. So we definitely have to get the word out that this condition in many cases is treatable and can resolve these symptoms.

Diana Gray:

The other piece is awareness brings more dollars and support, which can fund more research, which is incredibly important to us because we want a cure. We want better treatments and we want a cure. And so we can’t do that unless we have awareness broader than just the community that’s impacted by hydrocephalus. We need more people in the general population to know about hydrocephalus so that we can garner support for research.

Lindsay Clarke:

Definitely. And at the same time that you are garnering support for research and raising awareness about the disease, you also have a lot of resources and support for people who’ve been diagnosed. Can you tell us a little bit about that?

Diana Gray:

We do. I mean, we were founded on our support and education services even before we had a research program. So someone can go to our website, which we’ve actually recently updated. So there are even far more resources on there than there ever were in terms of living with this condition. Certainly the basics in terms of what it is, how it’s treated, what kinds of questions to ask your doctor. But now, we’ve added more components that talk about living with the condition. And also, this other aspect of the challenge of children who grow up working with a neurosurgeon because they have pediatric hydrocephalus who then transitioned care to working with an adult provider who’s often a neurologist for possibly a neurosurgeon still. But sometimes they have challenges finding a provider. So we have more resources in terms of how to properly transition to new care as a young adult growing out of pediatric hydrocephalus.

Diana Gray:

So also there’s a listening ear as well. There’s someone who answers our phone at (888) 598-3789. You can also reach us at hydroassoc.org on our website. This way you can email us. And those emails, we have a Facebook page. We have a Twitter page, Instagram. All of those social media resources are there and are ways to contact us. But let’s say you do reach us. What do you get? So in addition to actual resources you can read and learn about the condition, we have community networks across the country. And these are other volunteers who have a connection to the condition. Either they’re a child who’s diagnosed or have the condition themselves, and they’re there to host meetings.

Diana Gray:

We also have online meetings that I have to tell you, during the pandemic, as we’ve all gotten used to Zoom and virtual meetings, our community network program has just soared. We’ve increased the number of groups that have happened because people are just so craving connections. And so we actually have more community network meetings and groups than we’ve ever had before when we were hosting them in person live. So that’s a silver lining is that more people are getting this assistance. We also have peer support volunteers. So if someone who has traveled your journey, who can talk with you about their experience, they’re not going to give you medical advice, but they can talk about, “Yeah, I had that. So don’t worry. Yeah. I did this,” or, “I understand what it’s like to find out that I have normal pressure hydrocephalus. And here’s what it’s like on the other side,” or, “Here’s some of the other challenges that I experience.”

Diana Gray:

And then we also host webinars monthly. So that’s another way that someone can find out, sign up to receive messages from us. Once you get on our website, you can get messages from us and you’ll find out about those webinars on different topics. And then probably our biggest educational event we do every other year is something we call HA Connect, and it is our national conference. And last year, we held it virtually because of the pandemic, but we really hope to be in person in July of 2022. We got to get all those booster shots. We’re still holding out hope that this light at the end of the tunnel with the pandemic is really there and that we can host our conference in person in Austin, Texas. And there’s something really powerful about not only coming to sessions with experts and meeting the experts, but also meeting other people who have the condition.

Diana Gray:

I’ve met many people who say they’d never met another person with hydrocephalus until they came to our conference. So that’s really important to know about too. And in January, we will post on our website the full agenda for the conference and people can begin registering. So hopefully I’ve covered everything, but there are a variety of ways that we support people with hydrocephalus. And a lot of people love coming to our walks because that’s a way to meet other people with hydrocephalus. And we’re trying to do those right now live. Some have gone virtual. But on our website, you can find out about how to engage in a walk and meet others and help fundraise for hydrocephalus research and programs.

Lindsay Clarke:

Well, that is amazing. It sounds like you are really supporting people no matter where they are in their journey. And just in case people didn’t catch it, would you tell us your URL again?

Diana Gray:

It’s hydroassoc. So it’s confusing. I wish we could have gotten hydrocephalus.org. H-Y-D-R-O so hydro and then A-S-S-O-C like association. So hydroassoc.org.

Lindsay Clarke:

Great. Thank you. Well, and I would imagine a lot of people who are listening who have been touched by hydrocephalus, either they themselves have been diagnosed or a loved one’s been diagnosed, I’m sure they’re really curious, as am I, to learn a little bit more about the current state of research surrounding treatment and cures. Could you tell us a little bit?

Diana Gray:

I would love to talk about research because that’s something that we’re really passionate about at the Hydrocephalus Association. In fact, many people, once they figure out how to deal with this condition and learn what it is, what they really want to know is how do we make it better? How do we prevent this from happening to other people? Or how do we move towards a cure or better treatments? In fact, one of the things we’re doing right now is something called the community research priorities project. And a lot of other disease conditions have done it. It’s through this model called the James Lind Alliance where you actually survey your community. And it’s a very complicated process from getting thousands of answers down to a survey where you get the top 20, and then it culminates in an actual research or an actual workshop to discuss the top 10 research topics.

Diana Gray:

And you actually debate. Scientists, providers, and people living with the condition and caregivers get together and really debate what are the top 10 challenges. And how this helps us is it helps investigators to know what people who live with the condition really care about. So that’s something exciting we’re doing right now. But when we first started our research program in 2009, there wasn’t a lot of research for hydrocephalus. Our number one goal was to develop the ecosystem to encourage more scientists to get into this field. And if there isn’t funding, scientists don’t get into the field. So we’ve awarded 43 grants since 2009 and we see ourselves as filling the gap between getting National Institutes of Health funding and Department of Defense funding. There’s something called the Congressionally Directed Medical Research Program that you have to advocate to be on the list of conditions. And we do that every year. So those are two federal sources of funding that will fund hydrocephalus research, but you have to already have preliminary data to apply for a federal grant.

Diana Gray:

And so our Innovator Award program helps scientists test a really novel, innovative idea and get data. And that data then helps them to go on to be funded by the NIH or DOD. So our return on investment from our… And I haven’t talked yet about our two research networks, which I will. But we’ve invested about 12 and a half million since that 2009 timeframe where we started our research program, and our return on investment of scientists or networks going on to be funded with bigger dollars is 35 and a half million. And that’s soon to grow because we have another big grant that our adult hydrocephalus clinical research network has been verbally awarded. So hopefully that’ll get closed and we’ll be close to 50 million on our return on investment because of the work that they’re doing.

Diana Gray:

So I can also tell you that we have 10 drugs that are in the process of being investigated. All are very early, but nonetheless, very exciting. One of our scientists, Dr. Bonnie Blazer-Yost, discovered something, a compound that is a TRPV4 antagonist. So it was something that she discovered while doing research for renal disease, that it had an impact on mice and rodents with hydrocephalus. And so she started investigating hydrocephalus and learned that this antagonist in rodent models can decrease CSF, which would then decrease the size of ventricles and could actually be really exciting in terms of controlling the challenge of hydrocephalus, which is this overaccumulation of CSF. So that’s just one example of a drug therapy.

Diana Gray:

Another challenge with hydrocephalus is shunts. So shunts are the primary treatment. Shunt is inserted into the ventricle to reduce the hydrocephalus through reducing the ventricle size. And one of the challenges is sometimes these shunts get obstructed. And so one of the scientists who’s working on a drug to actually reduce the cells from wanting to attach to these membranes in the shunt, which blocks the ability for the cerebrospinal fluid to flow. And so that’s exciting as well. And then there are others. There are scientists that are looking at after a brain bleed. Can we use this treatment therapy to stop hydrocephalus from actually happening after a brain bleed? So all of these different types of looking at compounds to treat hydrocephalus have merit. They’re early, but with more investment in hydrocephalus research, we hope that one day they will actually be in humans and can make a difference for hydrocephalus treatment.

Diana Gray:

The other thing that I didn’t talk about and I should are our two clinical research networks. We have a pediatric clinical research that we call the HCRN and then adult HCRN, hydrocephalus clinical research network. And the HCRN works very much on different… They’re different hospital sites throughout the country, pediatric hospitals sites throughout the country. And these hospitals look at different surgical problems that they can investigate.

Diana Gray:

So through their investigation, they figured out that just by looking at protocols of how you do the surgery, you can reduce shunt infection by 36%. So that’s life-changing for individuals with hydrocephalus because that protocol can keep them out of the OR, which is what we want. And they’re looking at lots of other types of issues. And some of the treatments they look at long-term, follow more long-term to see what the intellectual challenges are post-treatment between shunting and ETB, which I talked about earlier in terms of treatments. And then our adult hydrocephalus clinical research network, both of them have registries, patient registries, so they can study the patients. And they’re obviously anonymized. So no names are affiliated if the way you did with these registries.

Diana Gray:

So the data is useful in terms of the more data you get, you can study the different types of treatments that were used and the different types of hydrocephalus and gain information. But the adult hydrocephalus clinical research network has been doing work more recently on normal pressure hydrocephalus in terms of demonstrating in a randomized control trial that shunting really does prove successful. And you’d think, “Well, yeah. We see that clinically.” We see it clinically all the time that it works. But in order to scientifically prove that it works, you have to do a randomized control trial. So they have done that and they are close to being able to do this on a broader level with an NIH grant that is pending. So that’s very exciting because that will also increase awareness in the medical community about treating hydrocephalus, especially normal pressure hydrocephalus.

Diana Gray:

So, gosh, I’ve talked about a lot of different types of research that we’re doing. And the final thing I would suggest to viewers is we have a patient registry of our own at the Hydrocephalus Association called HAPPIER, which is a way for people to enroll. Again, it’s very highly protected and then anonymized, but it’s a way for you to tell your story in terms of the different kinds of symptoms you’ve experienced having hydrocephalus. Do you have a lot of stomach challenges? Maybe because the shunt tubing is draining in the stomach and how common is that? If we get enough people enrolled in that… We have about 700 right now. But if we get enough people enrolled in our patient registry, then scientists can use that data to also inform the path of the disease.

Diana Gray:

So lots of ways that people can engage with us and certainly support us financially if you want to help increase more research. But if you have hydrocephalus or know someone with hydrocephalus, the patient registry is a really great way to help further research by putting your information in this registry in a very safe way that protects your privacy.

Lindsay Clarke:

That’s fantastic. And it’s clear that the research that you’re funding is already changing people’s lives, and it’s really exciting to hear how much is on the horizon, hopefully. And I look forward to hearing next September about all of the breakthroughs that have happened in the coming year. So thank you for sharing all that. And I think it’s also really important that you underscore the registry. We talk about the Alliance for Aging Research, how important it is for people to participate in research and to share not only for themselves, but for everyone that will be diagnosed after them. So thank you for emphasizing that.

Diana Gray:

Absolutely. And hopefully, I told people how to engage in HAM. I don’t know that I did, but you certainly can use social media or tweet. You can take a selfie of yourself and post it and help us create awareness.

Lindsay Clarke:

That’s great. Right. And so everyone should be thinking about how they can do that come September 1st and obviously visiting your website to learn more.

Diana Gray:

Yes, absolutely.

Lindsay Clarke:

And now switching gears, I wanted to ask you a couple of questions that we actually ask all of our podcast guests. And the first is when you were a kid, what did you imagine that growing older would be like?

Diana Gray:

Well, I love that question because when I was a kid, and I think for most kids, time takes forever and you never imagine that you’re going to grow up. You just want to be older and have independence and be able to make decisions and get on with your life. And then as you age, you find that it goes so fast. So when I was younger, I had a lot of wonderful relationships with grandparents and certainly saw that changes happened to them. And of course, that wasn’t ever going to happen to me because I was younger. I wasn’t going to get old and feel bad or have problems remembering. And I think you learn that pretty quickly as time goes faster as an adult. So I think that’s what I thought, that it would never happen to me.

Lindsay Clarke:

Yeah, no, I think a lot of us feel that way. We witness it, but we don’t realize that we are going to age. But I think on the flip side, there’s a lot of wisdom that comes with aging, and there’s new experiences. So my next question is what do you enjoy most about growing older now?

Diana Gray:

I think that what I enjoy the most is perspective. When I was raising kids… and our kids are all grown now… things happened so fast. Everything was just day-to-day, fast-paced. And it now seems that that has all just flashed by. When you’re in it, you’re working, and you’re raising kids and taking them to activities, you think, “Wow.” We’re in it in the present. But now, in retrospect, it just seems like the time goes so fast.

Diana Gray:

And so for me, the perspective of looking back and looking forward and having this wisdom you talked about, this understanding of the value and importance of each moment, the value of my family, and now having a grandchild who’s three and a half years old and loving that and seeing how absolutely wonderful that is. We have another one on the way that’ll happen early next year. And so for me, it’s valuing the time and importance of each moment. I unfortunately lost both of my parents. They were only 70. One was 70. One was 71. One to Parkinson’s and one to ALS. So both neurological conditions that took them far too soon. And so I miss my mom and dad every day. And so I have lots of great memories with them. But now I really value the moments that I have with the people that are around me.

Lindsay Clarke:

Thank you for sharing that. And I think it’s not that we haven’t heard before that we need to slow down and value things, but I think to understand that even… So I have school age children, and it’s the fast-paced environment. And I don’t have so much control over the pace. But what I can do is enjoy it and value it. So thank you. It’s an important perspective. And Diana, thank you so much for joining us today to share about hydrocephalus and your insights and all of the amazing work that you’re doing. I know I learned a ton and I’m sure our listeners did too.

Diana Gray:

Well, thank you for having me. It was great. It’s always great to share information about this really critical condition. And you’re helping us get the word out. So thank you so much. And thank you for the work that you’re doing.

Lindsay Clarke:

Well, thank you. And thank you everyone for listening to This is Growing Old. Our intro and outro music is City Sunshine by Kevin Macleod. Please stay tuned for new episodes every other Wednesday. You can subscribe on Apple Podcasts, Google Podcasts, Spotify, or anywhere else you listen to podcasts. And please rate and review us if you’re enjoying the show. Thank you for listening to This is Growing Old and have a great day.