Progress, Not Perfection: Joy’s Journey to Building Confidence in Independence

 Rachel Tierney: I want to thank you so so much for taking the time to share your story, and I'm really excited to hear just kind of how you've navigated living with BBS. From what I know about you, I know that you have accomplished so much, and are such a great resource for everyone around you, and so I'm really excited to hear more about what you have to say. Do you mind introducing yourself a little bit? Your name, how old you are, where you're from

Joy Ravenel: Okay, I'm Joy Ravenel. I'm in my early forties, and I'm a native Coloradian, and I live in downtown Denver.

Rachel Tierney: Awesome. And what do you like to do for fun?

Joy Ravenel: Listen to music, follow sports, cooking competition shows, get together with different friends. I'm back into church full time now. So I'm building up that church family again. I have a pair of season tickets for CU Football – University of Colorado football. So I love taking someone who roots from the other team – so much more fun. But a lot of the times the people I take are willing to go for a good time.

Rachel Tierney: Yeah, that's so fun that you take somebody [rooting] for the opposing team. But I guess you gotta keep things interesting, right?

Joy Ravenel: Yeah, I've only done it a few times, but it's fun.

Rachel Tierney: Yeah, that's awesome. Do you usually win?

Joy Ravenel: Some years, yes – some years, no. It depends on how the team's doing.

Rachel Tierney: How about for work?

Joy Ravenel: So I'm a budget analyst for the Bureau of Reclamation, and I've been there almost 10 and a half years.

Rachel Tierney: Wow. It's really, really cool to see how much you're doing in your personal life, in your work life, all of that. Can you tell me a little bit about when you were diagnosed with BBS?

Joy Ravenel: That was an interesting story. Because back in the late nineties, when I was finally diagnosed with degenerating retinas, we were led to someone who said the closest degenerative retina disease was Bardet Biedl Syndrome. But my learning disability is very mild, and so she didn't give me the diagnosis. We went and saw the head of the Genetics department at University of Colorado, Health Sciences, and she said in her thick Romanian accent, which I can still hear to this day, that I had all the characteristics of Bardet Biedl, but I didn't look like someone [with the syndrome].To get the diagnosis at that point, you had to look if you were in the middle of the bell curve, but I was an outlier. I didn't have kidney issues. I was thin and tall, long, angular face. I had the extra finger at birth which we had removed, and like I said, my learning disability is very mild. It's more on the expressing than taking in the information. And so we were still trying to figure it out. 

Mom self-diagnosed me – my mother, Ruth Dameron – when she saw the paper by Dr. Phil Beales of the 45 different [BBS] characteristics, where I think you were supposed to have, like 3 from column A and 2 from Column B, and I had 3 from column A and 20 from Column B. So Mom self-diagnosed me, but we didn't get the official clinical diagnosis until I was 15 from Dr. Richard Lewis, who we met through the Foundation Fighting Blindness Conference. And so he said, I would like for you and your parents’ blood to go into the Blood Bank for research. And so eventually they found the genes, and eventually we learned, I have BBS1. Which to us, it was nice to know, because it explains why I went through speech and language therapy, occupational therapy for fine motor control, physical therapy for balance issues, vision therapy to correct where I was using one eye for near [sight] and one eye for far [sight]. My eyes always just worked one at a time, so I've never had double vision, just binoculars because of that. I could hop on one foot or the other, or I could hop on both. And when I went and learned how to ski at the age of almost being 5, that helped make my 2 sides of the brain have to work together, because otherwise you're not going to be able to stop.

Rachel Tierney: Wow! And through all this work that you've done, what have you found are the biggest challenges that come along every day? Are there any that are a lot less obvious that nobody would really suspect or know about?

Joy Ravenel: Yeah. I use public transportation to get around places that are further in distance. My office is 12 miles away from my condo on public transportation, and it's a bus and a train which is a long commute – about an hour and a half commute one way. So when it gets the time in late September, early October, where I'm putting in 12-hour days of work – because it's the end of the fiscal year/the start of the next fiscal year, and bringing the books closed from the engineers to then be literally pretty much reopened so that they can continue to do their job on their projects – I don't wanna try to put in a 15-hour day with the 3-hour [commute], so I'll work from my condo during that time period. But I do enjoy going into the office multiple times throughout the week, because I get social interaction, and will find out about coworkers' different likes of sports and things like that, and with my [exceptional] memory, I'm gonna remember the finer details they tell me.

Rachel Tierney: Right. I know you have a fantastic memory.

Joy Ravenel: Yeah, like, today, I was working through the list of jobs that are past due, and I was trying to get status updates, so I was either saying to them where I've already asked him like 3 months ago, “Well, this is what my mental notebook says. Is that still true?” And then, if it was a new one, I say, “Please let me know. So I can update my mental notebook.” Because I don't have any paper notebooks written down of where we are on the status of certain projects, because, well, I can't read paper anymore. I mean, I could have a Word doc open to have it in, but I'm tracking about 700 projects across 2 divisions for about 200 and employees from memory.

Rachel Tierney: Wow! They're definitely lucky to have you, that's for sure.

Joy Ravenel: And they do appreciate everything I do for them. They are amazed when I get things done really quick. I'm a graduate of Metropolitan State University of Denver, the Roadrunners. So I try to deliver my services to the engineers at the speed of a roadrunner, but also be accurate and quick. And that's why I use JAWS – that stands for Job Assistance With Speech. It's a screen reader software. I would say about 15 years ago that was a 4 letter swear word to me because I wasn't ready for it. But accepting that I needed to use it meant my vision got to the point where I needed it, and now I use it all the time for work. I use the Macbook version, for when I do things in the evening like fan fictions and checking email and things like that. 

My coworkers really don't know that I have balance issues, because I've done a lot of therapy to master that, but there are times where my feet could catch an edge, and I can't react fast enough to keep myself upright instead of going down forward so my knees might get scraped up, or palms of my hands might get bruised or scraped.

Rachel Tierney: What helps the most to get through tough days where you feel like you're feeling it a little bit more, or struggling a little bit more than other days. What helps you the most to get through?

Joy Ravenel: I do suffer through seasonal depression, so January is definitely one of my worst months. I think having a job helps me because it tells me Monday through Friday: Joy, you have to get up. You don't feel like you do, but you gotta go show up. And so I do. And I think that helps me, knowing I have things to do, even though I might be feeling down, might be feeling blue. It's like, push through and say, “Okay, I gotta do this. I gotta be there.” Because I know they're leaning on me to help them to be able to do their job, and I'm supporting my other teammates to do, you know, my part of the pie? Shall we say. I don't wanna let them down by not showing up and pulling my weight. Shall we say.

Rachel Tierney: Right, and I love that you have that attitude. I really admire that you don't let anything stop you.

Joy Ravenel: Yeah, that's kind of why even though I have BBS, even though I have a disability, I don't let it stop me from accomplishing my goals, and my dreams! It might take longer to accomplish them. I might have roadblocks that are thrown up, and you gotta figure out workarounds, but I really, truly don't let it stop me from accomplishing what I want to accomplish.

Rachel Tierney: That is so cool, and it's so inspirational. And that's why I'm seriously so grateful that we can have you sit here and talk about it and be open and candid about your life. And you know, I know that there's so many people who are really gonna learn a lot and just be able to see what incredible things you've done, and know that they don't have to let anything stop them either, if they can help it, you know.

Joy Ravenel: I got trained on a white cane way before I needed it, so it was in my toolbox when it was finally time I needed to use it. Because my vision was slowly degenerating, but I really didn't notice much change until I was in my early twenties – it probably has gone some more since then – but the way the rate my eyes are degenerating, it's slow and steady, so I think if you can be able to learn about something before you need to actually use it, it's like they're in your toolbox is kind of helpful.

So growing up in middle school and high school working on building, as I like to call it, my toolbox – knowing how to do your own laundry, cook meals for family, being able to get around by public transportation to see the dentist, or go to a [check-up], or see the eye doctor. I'm grateful I have all those tools now for being able to get to and from places by the bus or train, and I don't need to rely upon my mom for help in that department. I can do what I want to do, and I feel independent and able to do things. And I use the white cane – you know how I use that – and I use keyboard commands, I use streamer software. There's other tools 've been taught throughout different types of training for low vision, like on my microwave. I have rhinestones on certain buttons like on the 30 seconds. So I'm not trying to look at the button to see, to be able to read it. I'm just feeling for that rhinestone.

Rachel Tierney: Is there anything in particular that makes you really proud of yourself?

Joy Ravenel: Yeah, I'm employed full time with benefits. I love what I do. Federal government might be stressful, as this year we have a change of administration, and as we come down our fiscal year pipeline we're in the tail end of the 3rd quarter, which means 4th quarter is about to be here, which just means my workload goes up really high, but I love it because that means my days are going to fly, and I know that we're coming down to the end of a fiscal year, and then the start of a new fiscal year, and it might be stressful, but I love it, and I'm really good at it. I mean, I've had engineers that have given me time-off awards for things I've done for them that allows them to continue to do their job where they can focus on their project of being an electrical or mechanical engineer or a hydrological engineer. Our mission at the Bureau of Reclamation is water and hydropower, so understanding about dams and spillways and turbines and things like that.

Rachel Tierney: It's clear you're very integral to the team getting anything done, so that's seriously awesome. It's very clear also that you're super busy and have a lot going on. What does your support system look like as you're going between work and all of these hobbies and friends and family. Who do you find supports you the most?

Joy Ravenel: I don't think I would get to where I am today if it wasn't for my mom. I mean, she's a rock star, and she's given me the tough love when I needed it. She's helped me by reading out loud like my textbooks in college. She's helped with awkward phone calls with RTD (that's the Public Transportation Company here in Denver). And then, my neighbors in my Condo building. There's 10 units, and they're all willing to assist if I come a-knocking asking, “May I please borrow your eyes?” which is my code phrase for, “Help?” It's a great community, and they're all willing to support me. Definitely. Some of my college friends are good support coworkers. And now that I'm back into church full time, like I said, I'm building up that church family, true support team as well.

Rachel Tierney: It's so clear that you've worked really, really hard to get to where you are, and have a really wonderful support system. What would you tell either a parent whose child just got diagnosed, or somebody who just got diagnosed with BBS and doesn't know where to start?

Joy Ravenel: I would definitely say, make sure you have a support system that is willing to help you get through these different aspects. It's going to be multiple, different pieces of the puzzle. So don't focus on everything at once. Focus on one aspect at a time, like the milestone of maybe your kid is doing the combat corral, and hasn't yet gotten up in her knees and hands yet, or they're on their hands and knees, and they haven't yet got to walk, but you know, just allow them to. They'll get there eventually, but just allow them to go at their pace, and they might reach the milestones just not at the normal speed that, say, someone like a kid that does not have BBS, would. 

Or a grownup that has just found out they have BBS. For a grownup, it probably explains why they've had all these different issues throughout their life, and it's probably just a nice comfort to know, “Oh, that explains why I had to deal with XYZ.” 

I've had a theme song most of my life that is called, “I couldn't do it a year ago, but I can do it now.” It's a great song. It's done by Lois Lafond & the Rockadiles. A theme song I've lived by. I'm a hard worker, so I'm going to work to get to that next milestone. And like, I said at the beginning. I don't allow BBS to stop me from getting to the milestones. It might just take me longer to get there, or we might have to figure out a workaround. Like, I have low vision. Okay, so what are the tools out there that I can use to be able to be successful in my condo and be able to work full time and do my accounting job on the computer? So I do everything on the computer with keyboard commands and screen reader software. And sometimes there might be hiccups because the IT guys have pushed through some update that has undone what my software is working on, or there might be a site or a program that I'm supposed to be using that is not accessible with screen reader and keyboard commands. And we have figured out workarounds for that software program. 

Just be willing to understand, it's not going to be what you thought it was going to be, but you're gonna have a great kid. And yeah, they might not be able to graduate high school, they might not be able to graduate college, they might not be able to live on their own, they might not be able to work, but they also might be able to do all of those things. We never know, because we don't know the diversity of the different symptoms.

So that's one of the ways howI describe BBS. I've had all these different pieces of a puzzle that weren't quite correct until I got diagnosed with my eyes. And that's kind of like the missing piece of the puzzle to say, “Oh, it's Bardet Biedl Syndrome! That explains why you had 16 years at birth, why you had to have jaw surgery lengthen your lower jaw, why it took you forever to say the hard K and the hard G.” Those were the last 2 consonants that took me to figure out why I was dropping consonants when I was much at a younger age, and why I went through all the different types of therapies I've gone through, and things like that.

Rachel Tierney: So through all of that, how has living with BBS shaped your outlook on life?

Joy Ravenel: Well, I think not knowing what we were dealing with in the first X amount of years of my life really did help along the way shape who I am, because we were just getting whatever therapy I needed next to be successful going at shall we say “Joy speed,” and I think having BBS allows me to have an outlook of  “I'll get there eventually!” but it might take longer and might have some roadblocks we've got to go around. Like going through speech and language therapy to be able to talk like this or be able to put together essays for school, or having someone be willing to read out loud 10 hours minimum per week of accounting textbooks, because I was learning that I’m better [at listening] and following along at the same time, so as an audio visual learner that helped for it to stick into my brain. Then that wonderful, incredible mother of mine had to teach the tutors I was working with an adjunct program. They thought that I needed help in understanding the material coming in, but in the other way, it was that I needed help in expressing the knowledge I knew of. I think having BBS allowed for me to develop into who I am: being a hard worker, understanding it might take longer to accomplish it, but I'm going to get there one day.

Rachel Tierney: Yeah. And I feel like that's so true in every aspect of life, whether it's something that you've learned through having BBS or not. And I do have one last question for you, and you can interpret this however you want. If you could tell the world one thing about living with BBS, what would it be?

Joy Ravenel: There's many different symptoms, and not all of us have the same set of symptoms, so it's gonna be harder to find a cure for Bardet Biedl Syndrome. But hopefully we can find maybe a cure for different pieces of the puzzle that maybe one day might help to find a way that would allow for the vision to stop degenerating, allow for people to have better weight management, be able to have better balance, and be able to learn better, or things like that. I really don't know. I'm hopeful that one day we might find a cure, but I don't know. It's a very complex syndrome that affects the whole body, so you gotta figure out a way to fix the message guys, the guys that are on the antenna of the cell phones, to get the right messages.

Rachel Tierney: Absolutely. Is there anything else about living with BBS that you feel is important to share? Or a message you want to get out?

Joy Ravenel: So to parents: When you first hear about Bardet Biedl Syndrome, it's probably scary to you, and you probably think that your kid can’t have a life. Well, I want to tell you that it might take longer to accomplish what the goals might be for your kid, but I'm fully successful. I work full time, and I love what I do. I have a degree in accounting, and that's what I do for work. I calculate numbers all day long, and I'm a happy camper in that department. I go to church. I live on my own. I actually own my condo, so I'm independent and can hire people to come and clean my condo, as I really don't like to clean, and it's a bit hard when you've lost a good chunk of your vision, but I at least straighten up things before she comes, you know, like the dishes are in the dishwasher, you know, dirty clothes are in the hamper, things like that, so that she's only dealing with the main areas of cleaning. I've hired someone else to cook healthy meals for me that I get shipped every week, and all I have to do is put them in the microwave for a couple minutes. I do that throughout the work week, because when I get home my brain is just frazzled and scrambled from all those calculations I've been doing all day long, and I want to eat healthy, of course, because I'm trying to manage the weight, and I don't have the problem of not feeling full. Some of you might have that problem, and I'm hopeful that you can find a way to overcome that. And I'm passionate about all the sports teams I follow. I have my core sports teams, and then I have friends. I've learned what their sports teams are, and I follow those and I track them so that whenever they call me up I can tell them, hey, your team is doing so well. I'm working on rebuilding an awesome church family who are willing to give me a ride home when it's too dark for me. I try to walk, slash, take public transportation to get home. I get involved in a community Bible study group during the week, and things like that. It took me longer to get my college degree. I do have an associate of arts with an emphasis in business and a bachelor's of science in accounting. It did take me 8 years to get it done, but I got it done. And like I said, I work full time.

I really, truly do love my life. And yes, I have BBS. But, like I said at the beginning, I don't allow that to stop me from accomplishing what I want to do.  I think I've had a long part of my [journey] when I wanted to be as close to normal as my peers are. Now, I also understand that I need tools and help and assistance and accommodations to be at that same playing level as my peers.

Rachel Tierney: That was excellently put. Thank you so much, Joy. I really appreciate it, and your willingness to share who you are and your story. It's really amazing, and I'm excited to share it with our community.