Wanda O’Neal, PhD, Research Associate Professor of Medicine, Department of Medicine, Marsico Lung Institute, UNC Cystic Fibrosis Center, talks about her family history with Cystic Fibrosis and the research being done towards treatment.
Interview conducted by Ivanhoe Broadcast news in October 2017.
Let’s go back to when you were a little girl. First of all you were born into a big family.
O’Neal: My mom had nine children, yeah. It was a big family. But she came from a family of eleven and my dad came from a family of thirteen so they had big families.
Big families.
O’Neal: Big families, lots of cousins.
How many boys, how many girls, and where did you fall in that long row?
O’Neal: There were three girls of the nine so that’s six boys. I was number four; my sisters were older than me.
And this was in North Dakota?
O’Neal: Right. My parents are farmers in North Dakota; a little town called Enderlin, North Dakota which is in the southeastern part of the state. For most of my childhood it was a dairy farm and then became a grain farm toward the end of my time at home.
So obviously your parents had their hands full with so many children and a home but tell us a little bit about what life was like when you were little. With your siblings what was going on with them, were they showing signs of being sick?
O’Neal: Yeah, so I was fourth, my sister Joanne was four years older than me and then my sister Nancy three, my brother Ron maybe one and a half or something and then there was me. And then my brother Bill was born a year and a half after me and then Mike and then Danny and then Curtis and then thirteen years after that or something like that, my youngest brother is thirteen years younger than me was born, Jeff. I don’t remember a lot about my very, very early childhood, I mean nobody really remembers. It must have been relatively normal for me. But from the time that I can remember things, I knew that my sisters and brothers had cystic fibrosis. So one of the very, very first memories that I have is we used to have this phone hanging on the wall back in the days with phone dials. And my dad answered the telephone and I don’t know why I didn’t know this but my sister Nancy had been in the hospital and they said she was in the hospital for pneumonia. They had gotten a phone call about this, that she had died, and I remember gathering around the phone as the family received this news and my mom was there. I remember what they told me at the time I was young, that she choked to death. I don’t know if they thought we just couldn’t grasp it or if they themselves hadn’t grasped what it was. But she passed away. And then what I remember after that is my brother Curtis died. I remembered that because I was in school and they came to get me out of class. And then at that point I think I was old enough to realize that something was going on, something wasn’t right, and my siblings had cystic fibrosis. At some point, I can’t remember exactly when, the diagnosis of cystic fibrosis was made in my family, and at the time they had a sweat test; they still do the sweat test to diagnose CF so because the cystic fibrosis channel doesn’t work in your sweat the salts build up and so your sweat is very salty. They can use that as a measure of whether or not you have CF. So I remember going to the place where they had us all do the sweat test. At that time my sister Joanne and my brother Ron were older than me. My sister Nancy had already passed away. And I had my younger brother (my brother Jeff wasn’t born yet.)
I remember that we all had to go in there and we all had to get our sweat tested. And I remember my mom probably knowing which kids were sick but getting the results one by one by one. My sister Joanne had CF, my brother Ron had CF, I didn’t have CF. My brother Mike had CF, my brother Bill didn’t have CF, my brother Curtis and my brother Danny had CF. And I remember that my mom was crying and I just remember how emotional that was. Something that you sort of already know because the kids are sick and they’re not right. It’s confirmed in that way and that manner.
At that time she had eight kids and she had two healthy kids. Two. Now my brother Jeff was born later and he also does not have CF. So six out of her nine children were diagnosed with CF and several of them on that day. Then after that your life just becomes sort of punctuated by events that happen, by things that happen. With cystic fibrosis, the kids get really sick before they pass away. They’re in the hospital a lot, they get pneumonia a lot, they cough a lot, their lungs go bad over time and eventually their body just can’t handle it. They don’t get enough air, their heart fails and they die. So it’s not sudden right.It’s slow. My mom was not there when my sister Nancy died. She was not in the hospital with my sister and she did not like that. She sort of made a pledge I think to herself that she wasn’t going to let that happen again. They knew enough about CF to know that their kids are not going to live. And they never knew which episode was going to be the one that was their last. Was this the last trip to the hospital, is this the pneumonia they’re not going to survive. So she really took that to heart. When they were at the hospital she was there many, many, many days. I can understand that she didn’t not want to be there because Nancy went so quickly I think everyone was surprised. I mean maybe they weren’t as surprised as me; I was small. But that became part of her life, who was in the hospital at the time and how sick were they. The next child to die was my sister Joanne. She was very, very sick and she was the only sister that I had left,. We were in the same room; we lived in this small farmhouse.
You shared a bedroom.
O’Neal: We shared a bedroom. And she used to have to sleep in the mist tent. We would give her physical therapy treatments. My mom needed help with that sometimes because you were supposed to do them two or three times a day, fifteen minutes. It started out with fists on the chest and she just didn’t have one kid she had to do this with, right. So we would need to do that and she taught us to help her. And then later on they came up with this pad that acted in the same way as us using our hands to hit their backs . The idea is to try to clear out the mucus, to get it out.
I mean, what we know now is the cystic fibrosis gene produces the protein that is required to keep the mucus hydrated. And if the mucus is not hydrated it gets stuck and that normal protective hydrating function of the mucus is lost. Mucus gets stuck and it can’t be cleared, and so even though they cough, the mucus just comes back and bacteria start to grow in it and eventually the lung just succumbs to this constant barrage of bacterial pneumonia infections.
And back then they weren’t really doing transplants.
But that wasn’t an option?
O’Neal: That wasn’t an option at least not one that was available to us at the time. But there were some things that the doctors were trying to do. They were starting to understand that these were infections and that kids were being treated for pancreatic enzyme deficiency so their nutrition was better. They were living longer and you could take them to the hospital and get them an IV antibiotic treatment and they would make it through. They would make it through the infection and live to see another day.
But it was always that dark cloud.
O’Neal: It was always going to come back. So the kids, they were just so brave. They dealt with it every day. And my mom was just so brave; my dad was just so brave. And so I had to be brave. You have to find a way to be brave. So my sister, she was so sick, but then we went to a doctor in Fargo, North Dakota. Probably one of the places that was key for CF back then was the University of Minnesota. So the doctors in Fargo suggested that Joanne go to the University of Minnesota. I remember her going down there; it was a six- or seven-hour drive and it was not easy for my mom to be away. And they weren’t able to help her. She was so sick, but that one time when my mom thought she was stable and wanted to come home. My mom had a sister that she stayed with when she was down there. Her sister lived in the city. She came home for a day and was just relaxing and getting reconnected with my dad and the phone rings. And the nurses told my mom, “Where are you; your daughter needs you.”
I think that my dad thought she was not going to make it through this. He piled my brother Ron and me in the car and my mom and we took off to Minneapolis. He was driving like a maniac and they wanted to get there in time. Well, we made it there and when she saw me, she was glad to see me but she was so sick, she was tiny, she was fifteen and I don’t know what she weighed, eighty pounds, she was so sick. It was just so sad to see her so sick. And when they realized she wasn’t going to survive this episode, she wanted to go back to the hospital in Fargo. So we transferred her up there and she died a few days later.
You got to see her though.
O’Neal: I got to see her, one last time, yes.
For a young child I just can’t even imagine that kind of trauma.
O’Neal: I can’t either, but it was there. It was right there. Then that passed we had the funeral everybody grieved, and then my brother Ron started to get sick. My brother Ron was just a couple years older than me, he was my hero. He was so brave, he had such a good attitude and he was such a hard worker. People just loved him, they just saw such good in him. He never complained, they never complained, they just were brave. He helped my dad on the farm as much as he could and when he couldn’t he felt bad. And he made it to graduation in high school and he gave a speech. Hewas very smart. They were all smart people.
I remember going away to some camp in the summer time and he was in the hospital when I left, and I remember thinking about him the whole time I was there. And it was a bus that brought me back home. And my dad met me when I got home and he said we need to get to the hospital, your brother is not going to live another day. Apparently they knew, they just knew this was the end. So I got in the car for the hour-long drive to the hospital, just balling the whole time, just broken. And we got there and he did the same thing my sister did. He was happy to see me. He smiled at me; he just wanted to see me, just so brave. It wasn’t about him, it was like he thought, “I’m going to give her this one more memory of me.” And then he smiled at me and then he died.
Do you think that maybe your brother, your sister they held on to see you?
O’Neal: I think that they held on to see me. Whether it was for them or whether it was for me. I’m glad they did. But my brother just that smile; it was just so inspiring.
Does it inspire you to this day when you think of Ron in that moment? I know it’s very emotional and traumatic as well.
O’Neal: Yeah, I think about it. If you have a little problem, your day is not going too good, you have a flat tire, you have a cold, right, or you break your toe. While this is happening, somebody so young is taking their last breath. What are you complaining about anyway? Why does this even matter? I mean, I know it matters, things happen to people that matter to them and in the moment you can acutely feel upset about something. But this gives you a long-term perspective on how am I going to handle my life? How am I going to handle the things that come my way?
Yeah, it makes a difference that he smiled at me, it makes a difference that they didn’t complain, it makes a difference that people still remember them for the people that they were, not the disease that they had but the people that they were. They were inspiring to people around them. So you look beyond and I think this is where my mom and dad were really able to do it. They looked beyond the disease and the suffering to the person. They looked to the value of the person, the life itself was valuable despite the cystic fibrosis and the suffering.
It wasn’t about that, it wasn’t about the disease it was about the people—-
O’Neal: It was about the people, who they were and that inspires me, yeah.
Their life had value, their life had value—
O’Neal: Their lives always had value and they still have value. Everyone in my family remembers them, all the extended family remembers. When I go to family reunions they are remembered. My mom is admired for what she’s been able to do. She inspires people just by surviving it, right?
Oh yeah.
O’Neal: Because how do you?
Everyone must have asked that question.
O’Neal: People do, people survive things maybe even worse you know. Imagine all the terrible things that happen in this world. But if you look at her story most people wonder, how did she do that, how did she survive? It’s a very unique story. I think they were able to do it because they saw so much good in them. And they saw how they were able to reflect that goodness out and influence other people around them.
How did it affect you, how do you think it effected the course of your life, the path that you took?
O’Neal: Well completely; it completely affected it. Here I am placed in one of the top, if not the top, cystic fibrosis research centers in the entire world and working with the people who matter in CF research. This whole center, all their colleagues, they matter in the field of CF because they’re the giants of it. They have been working on it for years; they’re geniuses.
They characterized the function of the CF channel back when nobody knew what the heck the CFTR protein did. All of their work set the basis for all of the treatments that are on the market today. Because without knowing that the protein is a channel, you couldn’t have developed the experimental assays to do the high throughput drug screens, you wouldn’t have had any ability to develop those drugs. I find myself in this place completely humbled by it all.
At some point in my twenties, somebody asked me, “What do you want to do with your life?” And I said I want to work on cystic fibrosis. And they said, “Well why aren’t you doing it?” And that was it. That was it. I was like “Okay. You’re right, why am I not doing it and why can’t I do it?”
And so the whole path started, because before that it was like “what am I going to do?” There was always this push. I was in agriculture. I was always interested in agriculture and farming. My undergrad was in agriculture. I went to North Dakota State University and got my degree in plant science, plant pathology. But when that friend just asked me straight out. It was just crystal clear.CF research. There was just nothing else I could do. And the doors just opened and I don’t know why. Why would I? I went to North Dakota State University, when I got down to Texas to go to school there to get my PhD people made fun of that university. You know that’s not Harvard, it’s not big. How did I end up at Baylor with a doctor who was working with people trying to clone the CF gene at the time and one of the most amazing human geneticists in the whole world? how did I end up there? The doors just opened up for me.
Something was guiding you.
O’Neal: Something was guiding me because I didn’t have anything to do with it. I mean if you look back at it it’s like okay it seems like so many people struggle with what they want to do with their life. They’re not satisfied with the path that their life has taken. And if I look at mine, how was I so blessed to become the person whose path was so clear and whose road opened up in front of her so easily to end up where I am. So that’s one of the reasons to agree to talk to you; because I just still honestly don’t understand it.
How it brought you to this moment.
O’Neal: You know, when you look back you go, oh, that makes perfect sense. You had all these siblings die, she was smart she got into science; she had good enough grades to get to Houston to get into graduate school. She worked hard and Dr. Bodet respected that and took her in. And then when it was time for her to move on UNC-Chapel Hill just happened to have an opening for a molecular biologist, and I had the skill sets from my training in Houston to match. And then Dr. Boucher at UNC was willing to take me in and nurture me and keep me. And hopefully make use of me because the people around me are the heroes, they’re the real heroes.
It’s almost as if you couldn’t be doing anything else. It’s hard to put into words.
O’Neal: Sometimes it happens to people, where there’s a moment in their life where things become so clear. I don’t think it happens very often because you see people struggle with this decision or that decision. I was blessed to have that moment of clarity and then to have, like I said, the road open before me. And that road, it’s paved by so many different people so you don’t even really realize how connected things are. What allows me to work here, the Department of Medicine. The Department of Medicine has given me this position and this opportunity and continues to fund me. The UNC Marsico Lung Institute, the people of the United States who give their taxes for research. I mean, I don’t think they get appreciated enough. It’s taxpayers that support the research. The people who give to the Cystic Fibrosis Foundation. The families who have children with CF who care enough to do the CF walks and the CF dinners and donate to the CF Foundation. The CF Foundation can support the research that keeps us all together here. And the people like Mr. Marsico who give of their own wealth to support research here; it’s just a huge community. I’m just amazed that I can live my life and be a part of it.
So you lost five siblings but you have a sibling living now with CF.
Dr. O’Neal: Yes my brother Mike. My brother Mike is 51 and he’s still alive. And it’s awesome and I personally don’t think that cystic fibrosis will be the thing that takes him. And it’s an amazing; his is an amazing story as well. There are genes in the background of everyone that obviously can protect from CF. There’s CF kids who die early and then there’s Mike, people like Mike who live much longer. And Dr. Knowles and I, my colleague, are studying and trying to identify the reasons why someone like Mike who has exactly the same CF mutation would be able to survive to 50. Something is protecting him, something protects his lungs from these nasty infections and by understanding what that might be we might be able to offer that same thing to other CF patients, all CF patients. One of the exciting things that I do with Dr. Knowles is work on that aspect of CF, because I can see it’s real in my family. There’s a real thing that’s protecting Mike that we don’t understand.
So unlocking that part of it could—
O’Neal: Right. Imagine if there’s some protein or some mechanism or something happening in him that’s protecting him that could be added to everyone. That could be a therapeutic target for everyone with CF. You could see how that could improve the life of a CF child and make it almost normal. Now, whether or not that will ever happen, we don’t know. Research is so slow, research is fraught with difficulties.
But you guys are making steps.
O’Neal: We’re making steps yes. We’re making steps and we want to understand it.
Do you expect, do you think this could happen in your lifetime? That you will see a breakthrough—–
O’Neal: The field is already seeing breakthroughs. I mean the Cystic Fibrosis Foundation and all the people that they work with have already generated these drugs that are available for a large proportion of cystic fibrosis patients that we really need to consider as breakthroughs. These are breakthroughs, these were not available even just a few years ago. And everyone is very, very hopeful that they are going to make a huge difference in these children’s lives. It’s a very positive time for CF research.
And you are a huge part of that. I know everyone else is too.
O’Neal: I am a small, tiny little part somewhere in this little office; I’m a tiny little part.
All your siblings are behind you; they’re with you through this process. But I think that they’re a part of pushing you.
O’Neal: I hope so. Because I don’t want to do it alone, I don’t think I could do it alone. So I hope they’re there. Yeah they’re there.
They’re there.
O’Neal: It’s just that too many things have happened right for me, for them not to be there. It’s just too amazing.
It is truly the whole course.
END OF INTERVIEW
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