BRIAN NAYLOR, HOST:
This is TALK OF THE NATION. I'm Brian Naylor in Washington. Neal Conan is away. Like most prospective parents, Emily Rapp and her husband spent much of her pregnancy poring over parenting guides and planning for their child's future.
But by the time Ronan was nine months old, it was clear that something was wrong. Ronan wasn't developing as expected and a trip to the eye doctor eventually led to a diagnosis of an incurable genetic disease. Most children with that disease, Tay-Sachs, won't live beyond their fourth birthday.
Being a parent of a terminally ill child, Rapp wrote recently in the New York Times, means parenting without a net and without a future. She joins us in a moment.
If this is your story, tell us what helped you. Our number is 800-989-8255. Our email address is TALK@NPR.org and you can join the conversation at our website. Go to NPR.org and click on TALK OF THE NATION.
Later in the program, a former DJ on the future of rock music on the radio.
But first, we start with Emily Rapp. She is a writer and professor of creative writing at Santa Fe University of Art and Design in New Mexico. She joins us now from a studio in Santa Fe.
Emily, thanks for sharing your story with us today.
EMILY RAPP: Thank you for having me.
NAYLOR: Tell us, what did you first do when you first learned of Ronan's diagnosis?
RAPP: It was interesting because, obviously, when I got the news, I was devastated, clearly, and I often tell people that it was almost as if he died that day, in some ways, because we were robbed of a future with him. And then several things happened. I felt a great overwhelming need to reach out to other people who had a similar experience just because it's such an isolating feeling, this kind of grief.
And so I did that and I got in touch with National Tay-Sachs and Allied Diseases Organization and they put me in touch with mothers who started calling me, just calling my number and offering to talk. So my first response was to reach out to other people and see what they had done to survive this experience.
NAYLOR: And, as we heard, you did all of the sort of planning that typical young parents tend to do up until his...
NAYLOR: And, you know, how did the news, you know, transform the way you approached parenting when you found out?
RAPP: It's been a slow kind of evolution over the last nine months since his diagnosis and I feel like it's been a hugely transformative period in my life, maybe the most terrible and beautiful nine months of my whole life because so much of parenting is obviously a future-directed enterprise. And what I started to learn from Ronan was, not only is he sort of free of those expectations, but he encouraged me to sort of love him in the moment, which is actually kind of difficult to do if you follow those sort of traditional or, at least American, parenting advice.
We seem to have found ourselves in a parenting Olympics where parents sort of live through their children. So it was a huge sea change in my perspective and I'm actually very grateful for my son for teaching me that important lesson.
NAYLOR: Just tell us a little bit about Tay-Sachs. How has it affected Ronan?
RAPP: Tay-Sachs is a rare, progressive neurological disease. It's caused by a missing enzyme and it's sort of - it's been commonly associated with the Jewish community, although that's not true anymore. It is not a Jewish disease.
And what ends up happening with children who have Tay-Sachs is that they gradually lose their faculties and their functions and so the first step in terms of prolonging life would be a feeding tube or other interventions.
I also want to say that it's really important for people to know that Tay-Sachs can happen to anybody. It is not a Jewish disease and the NTSAD, the organization I mentioned earlier, has done a great deal of advocacy in this area in terms of carrier screening. And that's something I'm very passionate about because I actually did have the test for Tay-Sachs, but the...
NAYLOR: And they were negative?
RAPP: ..standard - it was negative. The standard prenatal test does not detect all of the mutations.
NAYLOR: So did you feel at all, you know - I don't know. Did you feel betrayed when you found out or felt maybe that the medical community had let you down or that you were the victim of a horrible, horrible joke?
RAPP: I suppose. Yeah. I mean, and I think that anyone who gets a terminal diagnosis for their child feels that often, that sort of, why me, feeling and you feel like a victim. But there's nothing we can do for him. And, in that sense, it's been an experience that's sort of - it's transformed me in the sense that it's made me much more fearless in life. And so I don't feel like a victim of the medical community. I mean, I wish that I had known this information before I had the test, but I certainly don't think I've been wronged in any significant way.
Of course, I wish it hadn't happened, but it has and I understood from the beginning that the only way to survive it was to really just go right into it, accept it and write about it and tell his story. Those are my coping mechanisms and were from the very beginning.
NAYLOR: Yeah. Is it difficult to interact with parents of healthy children? Do you feel at all isolated because of Ronan and Ronan's illness?
RAPP: I don't - sometimes it is, although, you know, I have this amazing parenting group through the Tay-Sachs Foundation and, interestingly, it's probably the only parenting group I've ever felt comfortable in if only because they're not concerned with sort of the banal issues. They know the truth about life. They're in this. You know, their task is loving their child and that's what we talk about.
So I actually don't feel isolated because I've been very connected with parents who - not only do their children have Tay-Sachs, but other fatal diseases, as well. So I have that network of parents.
NAYLOR: And do you find it difficult to talk with other parents, though? I mean, they're talking about their kids doing things that - I don't know - that they would take for granted and that you know that Ronan may never be able to experience? Does it feel alienating, sometimes, or...
RAPP: Sometimes. Although, you know, probably those parents are pretty careful not to have those conversations around me - is what I would say. You know, Ronan is who he is and he's great company. He's a great kid. You know, I can only speak out of my experience, so I suppose there's a little bit of that. It depends on the day.
NAYLOR: Yeah, yeah. What kinds of decisions have you had to make about his care to this point?
RAPP: Nothing at this point, but we have decided to do the nonintervention route because we feel that it would prolong suffering versus giving him a high quality of life. So our goals are centered around making him happy, which is very easy to do, trying to make him feel safe and comfortable and just - you know, we do a lot of sort of sitting with him in our laps and being with him. It's basically just sort of a very peaceful coexistence.
And so far, we haven't had to make those decisions that we will do in the future.
NAYLOR: And you say nonintervention. That means no feeding tubes.
RAPP: That's right.
NAYLOR: And are there other things that you've decided that you're not going to deal with?
RAPP: That's really the first step for most parents and, you know, one thing I will say about the National Tay-Sachs and Allied Diseases Organization is that parents make a whole range of decisions about care for their child and there's no judgment about that within the group. You know, you can only do what you would do for yourself. It's an impossible choice and I really appreciate that about the community that I'm involved with, is that your choice is respected and you're expected to respect the choices of others.
And I can only speak, you know, for my own son, but don't have any judgment about other people's decisions.
NAYLOR: And have people - tell you - how do people respond, you know, when they find out about Ronan? Do they say they, you know, they couldn't imagine facing such a thing or...
RAPP: Yeah. That's the common response. I mean, what I think is interesting about that, when people say I can't imagine, is that the reason that it's so horrific is that they actually can imagine. You know, I have a lot of friends who are parents and they just - you know, a lot of the parenting is about terror of this very thing happening.
RAPP: So I think people actually can imagine, but they maybe don't want to. And I think it's important - I mean, I've - from the very beginning - have been very frank about it and I think that sort of helps people be at ease, but yeah. It's hard to know what to say. I mean, I guess the only thing to - the wrong thing to say would be nothing at all, which, you know, people have different reactions when they hear this news, but, you know...
NAYLOR: And are you and your husband a team on this? You're on the same page? Have you...
RAPP: We are, in terms of care decisions. Absolutely. I mean, we - thankfully, we are and I think we are very united in our care of him and we take good care of him and we enjoy him. We try to understand that, because our moments are limited, we really have to stay aware of what's happening and that can be really sort of exhausting and it can also be incredibly exhilarating. It's kind of a mix.
NAYLOR: And how do you - you mentioned holding Ronan, spend a lot of time with him, I guess, on your lap and close to you. Do you get out much? Do you...
RAPP: Oh, yeah. We take him out. We hike with him. We take him in his stroller. We live in Santa Fe, which is a beautiful place to walk and bike and run and we take him on road trips and take him out to eat. I mean, we take out a lot of places and he's very well known in this town and we have a lot of people who come to visit. We've had people visiting for the last nine months just to meet him and hold him.
And, you know, I just want him to be surrounded by love for his whole life and he might be unique in the sense that he will only have ever experienced unconditional love his entire - for the whole span of his life. So I often think about that when the days are hard.
NAYLOR: Yeah. And this is a difficult question, but you know, how do you think about the future? Have you begun to prepare yourselves for a life without Ronan?
RAPP: We have. I mean, it's really hard, obviously, to do when he's sitting right there and, you know, one of the things that I've tried so hard to do, which is very much against my nature, is to not sort of project into the future, but to really just sit with him and be like, this experience is terrible, but it's also very true. I mean, it's an opportunity to learn something about being a human being, as well as a parent, as well as a partner.
And they maybe aren't opportunities I would choose, but here they are. So we kind of take it day by day because we don't know. We don't know how long he has left, so we try not to project too much into what comes next.
NAYLOR: And you're a writer. Does that help you, in a way, deal with this?
RAPP: It's saved me, I have to say. I started a blog about Ronan three days after he was diagnosed and, believe me, as a writer, it was the last thing I thought would be my coping mechanism, but it has saved me. It's very important. It's one of the hooks into the world that's gotten me through this experience.
NAYLOR: We're talking about the challenges of raising a child with a terminal illness. If this is your story, tell us what helped you. Our number is 800-989-8255. Our email address is TALK@NPR.org. I'm Brian Naylor. This is TALK OF THE NATION from NPR News.
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NAYLOR: This is TALK OF THE NATION from NPR News. I'm Brian Naylor. We're talking with parents about raising terminally ill children. It can be an isolating experience filled with difficult decisions and challenges, but as our guest, Emily Rapp, wrote in the New York Times, there are also rewards. Our experiences have taught us how to parent for the here and now, for the sake of parenting, for the humanity implicit in the act itself. Parenting, I've come to understand, is about loving my child today, now. In fact, for any parent anywhere, that's all there is.
You can find a link to her piece at npr.org. Click on TALK OF THE NATION. If this is your story, tell us what helped you. Our number is 800-989-8255. Our email address is email@example.com.
And let's take a call now from Kaylee(ph), I think in Kansas City. Thanks for joining us.
CALE: I'm sorry, it's Cale(ph).
NAYLOR: I apologize, Cale.
CALE: That's fine. Yeah. My story is a little bit different, but I mean there's difference and similarities. My wife and I lost identical twin daughters. They were born prematurely at 24 weeks and had kind of a compilation of issues, schizencephaly, hydrocephalus and they were monoamniotic, meaning they were in the same amniotic sac and umbilical chords were tangled and things like that.
But, you know, we basically had to decide the same, you know, as your guest did. Do we intervene or not...
CALE: ...when they were born? You know, the surgeries had very little probability of increasing any kind of quality of life or extending life, just - so we chose to let them pass.
NAYLOR: Mm-hmm. So without any intervention.
NAYLOR: Yeah. Well, thank you, Cale. Thanks for sharing that with us. I'd like to bring into the conversation now Jan Wyss. She's a registered nurse who works with parents of terminally and chronically ill children and their families. She manages the Partners for Children Program at San Diego Hospice and joins us from member station KPBS in San Diego.
Welcome to TALK OF THE NATION.
JAN WYSS: Thank you.
NAYLOR: I understand families that connect with your program sometime after their child has received a terminal diagnosis, they've often already had the opportunity to absorb the news, but when parents do first come to you, what's the first thing you tell them?
WYSS: We actually ask a lot of questions. We find out where they're at, where they're at in life, what goals of care they've set for their child, what they're going to need support-wise, advocacy-wise, what it will take to live as normal a life as they can and the most comfortable life they can with that child.
NAYLOR: Emily mentioned the isolation parents like her can feel. Do you see that in your work?
WYSS: I see it on a daily basis and I mentioned to a friend just yesterday that I had a call over the weekend and it was from a mom and that's exactly the reason she called was the isolation. It's hard to tell your family about what's going on. They don't really understand. Your friends don't want to hear you complain all the time. You don't really have time to foster those friendships. It's hard to get away when you're doing 24/7 care with your child. And not knowing how much time you have, you want to make the most quality of that time and not sort of be out and about with friends instead of at home.
NAYLOR: Yeah. And I just want to ask you, Emily, is that - do you feel like your time is really, really valuable now and, you know, you want to spend it as creatively as - doing your job and also, you know, being a mother as much as you can be?
RAPP: I do. I also feel, too, that one of the things that I was sort of committed to at the beginning of this process was to try to live as normal a life as possible and I feel really grateful that I have sort of a planet of friends who have sort of - they call me every day, they send me emails. So I don't actually feel that I'm losing my life as much as I think some people might feel.
RAPP: So, certainly, I think the time with him is very concentrated and is very intense, but at least in Ronan's case, we're not really doing 24/7 care at this point. But I do - you know, everyone's situation is different.
But I also, you know, knew that, if I was going to be able to survive Ronan's death, I would need to make sure that there was something for me after he died. So I'm very conscious of trying to keep those sort of pillars in my life of work and friendship and relationships and family.
NAYLOR: Jan, as a health care professional navigating the health care system, it must be - it's incredibly difficult in the best of times. A parent with a terminally ill child is not going through the best of times. Do you find yourself having to sort of - do you help parents navigate the forms and all of the other things that need to be - the testing and things that need to be done?
WYSS: We do. That's probably what our expertise is at San Diego Hospice is we know what community services are available. We meet parents at appointments, support them, advocate for them and their child, help them understand when they leave the appointment what was discussed, make sure they understand what was - the medical jargon can get so confusing and I think, when you've got an emotional reaction to what the doctor has said, it shuts down your ability to process that. So we're able to help them when they leave that appointment, too, and talk about what happened there. Where do they want to go from here, and support them in the decisions they've made.
NAYLOR: Let's take a call now. Mary, you're on TALK OF THE NATION from San Leandro, California. Thanks for calling.
MARY: Yes. This is Mary.
MARY: Hi. Yeah. I kind of want - listening to Jan speak and the isolation part of the process of having a terminally ill child is one of the frustrating parts, and also trying to decide if your child is going to need a trach and if that's the best option for them.
NAYLOR: And have you found yourself - you know, have you been able to get advice from medical professionals or family members?
MARY: When he was first diagnosed, there wasn't really any support for us. There wasn't any organization that kind of took us and explained everything to us, the process of his illness. I had a son named Brandon(ph) who was diagnosed with Duchenne's muscular dystrophy, which is a life-limited, terminal illness.
And, when we found out, we didn't really have any support and then, once things started changing for him, we were able to get some support through the Muscular Dystrophy Association and through George Mark Children's House in San Leandro, which helped guide us.
And one of the hard parts about having a terminally ill child is you kind of lose your role as the mom and you become a caregiver and you become the doctor. You become his psychologist. You become everything else but the parent to that child because you are so busy taking care of all the medical needs that you don't have time just to sit and hold his hand and cuddle him and just be there for him.
NAYLOR: Mary, I want to ask you one question. Your son was diagnosed at five, as I understand it, and he lived until he was 19.
NAYLOR: You know, that's a 14 year continuum. How did you deal with him growing older and through, I guess, different levels of understanding about what he was facing? Was that difficult?
MARY: I actually have a great husband. Brandon had a great dad. And we just talked about everything and what we really focused on with him getting older - and changing is the quality of life for him. And he loved life so much that, when we were told that he was going to need a trach and be on a ventilator, my husband and I kind of had to sit back and think about that because it was the quality of life. And we knew before that we would not do a trach for him...
MARY: ...because at that point, then his quality of life is going to be limited. But, as he got a little bit older and showed us how much he loved life and how life meant to him and he still - just because he had a trach didn't mean he wasn't going to have any quality of life. But when that moment came, the decision to do it or not do it was hard for us. But, as we discussed it, we decided the best thing for him was to have a trach for him...
NAYLOR: All right.
MARY: ...and being on a ventilator because his quality of life was still good.
NAYLOR: Well, Mary, thanks very much for sharing this with us. I'd like to ask you, Jan, about parents who - everybody has a different story, but children of different ages - do you counsel them how to speak to their children, whether it's age five or age 10 or age 15? There are different levels of understanding, obviously, as children age.
WYSS: That's true. We do. You know, children understand way more than we give them credit for. And sometimes, as parents, we think we're able to hide things or not tell them, but they have it figured out long before we do. So that's probably the first piece. It's just being open and honest with the kids and being there to support them. You know, working with San Diego Hospice, I have a lot of support behind me in helping to find those pieces, and we have counselors that help the kids on our program. But just, you know, those kids are - they know more about their situation and their condition than most kids about anything.
WYSS: So they seem to grow up way too fast.
NAYLOR: What about siblings? How do you help families deal, you know, who have - whose brother may be, you know, facing a terminal illness? What do you tell someone?
WYSS: For siblings we do a lot – a lot of listening, really. We have to be open to where they're at. And sometimes you don't understand whether they're worried about their sibling who's dying and what's going to happen to the sibling, or are they worried that the attention is all going to the sibling and not to them? And so identifying - and both issues are real and appropriate for whatever age they are, but then dealing with what the situation is. Maybe they need parent time away from that sibling, or maybe they need to be included in the activities and the care of the sibling.
NAYLOR: Let's turn to Jim on the line with us from Louisville. Jim, thanks for calling TALK OF THE NATION.
JIM: It's my pleasure. Thank you for the program. And it's Louisville in Colorado.
NAYLOR: There we go.
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JIM: I had to learn that when we moved here.
NAYLOR: You have a child terminally ill?
JIM: Well, we lost our son in '94. He was four years and nine months old at the time, and he was diagnosed at three months with tyrosinemia, which is a metabolic liver disorder. And the only treatment available really at that time was transplant - liver transplant. And we lost him in the process of a liver transplant. And I'd just like to say that - that certainly my heart goes out to all the other parents who've lost children or who face that prospect. But the whole experience for us also taught us the importance of family, community, faith, and sort of the frailty of knowledge.
And I was screened for thalassemia, which is related to Tay-Sachs, and - because I carry the trait - and we found that my wife did not carry that. And going through the experience with Michael with a very different disorder led us to wonder, you know, what would we do if we knew all the possibilities; how would we choose, and we might not have had Michael, who truly was a positive force in our lives and other people's lives.
NAYLOR: Jim, let me just stop to say here for a moment that you're listening to TALK OF THE NATION from NPR News. And Jim, you've had two children since your son's death?
JIM: Yes, we have.
NAYLOR: And was that a difficult decision, or I mean how does that - how does losing a child factor into a decision to have more children?
JIM: That's a great question. And it was - we knew through our time with Michael that we loved being his parents, and it was a terrible decision to know that there was a one-in-four chance that any subsequent pregnancy might result in the same condition for our child. And so we went into it with, you know, great trepidation. Let me just say that friends counseled us along the way that there are many ways to be a parent. And we've been fortunate that our now 16-year-old son and 13-year-old daughter do not have - they didn't have the disease, they don't have the disease, and that one of them, our son, is a carrier of the gene.
NAYLOR: All right. Well, Jim, thanks very much for calling in and best of luck to you.
JIM: Well, thank you.
NAYLOR: Emily Rapp, I want to ask you a very, you know, personal question. But have you...
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NAYLOR: ...thought about - since that's what we're dealing with in this hour, have you thought - after Ronan, have you thought at all about more children?
RAPP: Of course, yeah. I mean, that's something that I very much want. It's much - as the caller stated, it's much more complicated. There are ways to do that in the medical community, to test for Tay-Sachs in utero, et cetera. So those are discussions that haven't been sort of resolved at this stage. But certainly it's something that I think about a great deal, and I love being Ronan's mom. And I like parenting. It's been, you know, this isn't exactly the experience that I expected, but it's definitely one that I value and cherish very much. So, you know, obviously parenting a healthy child would be much different, but I do want that.
NAYLOR: Jan Wyss, I just want to close with you. Families in this situation must begin to make, you know, decisions about end-of-life care, things that, you know, seems so out of step with how we think about being parents. How do you help parents navigate those decisions?
WYSS: Those are really hard decisions, and we start very, very slowly. We give parents a lot of time. You know, unless you've got good rapport built with someone, it's really hard to have those discussions. Those discussions shouldn't happen in emergency rooms. Obviously they have to sometimes. But if parents have thought about things ahead, and so often they have, and then can share their thoughts, they're not always ready to make all of those decisions today, but we'll leave it open.
We talk about the decisions, the consequences, and everyone's talked a lot about quality of life, and that's really where the focus is, is what we'll give them the most comfort, the most palliation of their symptoms and can they live - what's more important, and most of the time it's to live a quality of life. At San Diego Hospice, we value the palliative care and the quality-of-life focus. So you know, those parents feel well supported and able to make decisions. Sometimes they only do make the decision like the one mom said that they knew that they just didn't want to do the feeding tube.
(Unintelligible) Emily said that, that she just doesn't want to do the feeding tube. She hasn't made any decisions beyond that right now. Taking one step at a time as they walk through the diagnosis and the process is all that parents can do.
NAYLOR: I think that's a good point to leave it at. Jan Wyss is a registered nurse and manager of the Partners for Children program at San Diego Hospice. She joined us from member station KPBS in San Diego. Thanks very much.
WYSS: Thank you.
NAYLOR: Emily Rapp is a writer and professor of creative writing at the Sante Fe University of Art and Design. She joined us today from a studio in Santa Fe. Emily, thanks very much for being on TALK OF THE NATION.
RAPP: Thank you. Transcript provided by NPR, Copyright NPR.